Lipedema A Practical Guidebook 2022 - Gestão do Conhecimento (2024)

Prévia do material em texto

123A Practical GuidebookZaher JandaliLucian P. JigaCorrado CampisiEditors LipedemaLipedemaAL GRAWANYZaher Jandali • Lucian P. Jiga Corrado CampisiEditorsLipedemaA Practical GuidebookEditorsZaher JandaliDepartment of Plastic, Aesthetic Reconstructive and Hand SurgeryEvangelical Hospital Oldenburg26131 Oldenburg, Niedersachsen GermanyCorrado CampisiICLAS -RapalloGVM Care & ResearchGenova ItalyLucian P. JigaDepartment of Plastic, Aesthetic Reconstructive and Hand SurgeryEvangelical Hospital Oldenburg26131 Oldenburg, Niedersachsen GermanyTranslation from the German language edition: Lipödem und Lymphödem by Lucian P.Jiga, etal., © Springer-Verlag GmbH Deutschland, ein Teil von Springer Nature 2021. Published by Springer Berlin Heidelberg. All Rights Reserved.ISBN 978-3-030-86716-4 ISBN 978-3-030-86717-1 (eBook)https://doi.org/10.1007/978-3-030-86717-1© Springer Nature Switzerland AG 2022This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed.The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use.The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, expressed or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.This Springer imprint is published by the registered company Springer Nature Switzerland AGThe registered company address is: Gewerbestrasse 11, 6330 Cham, SwitzerlandAL GRAWANYhttps://doi.org/10.1007/978-3-030-86717-1vPrefaceMany women suffer from a painful disproportional tissue distribution in the extrem-ities, especially the legs, and often know that something is wrong with them long before being diagnosed. It is the pain in the legs and arms that leads those affected to embark on a sometimes adventurous search for a diagnosis and possible ways for medical treatment. Lipedema is often misdiagnosed, so usually patients will have to change several doctors before finally confronted with the real diagnosis. As a rule, the first question most of the patients ask after being diagnosed is how the treatment looks like and how long will it take. Ignorance about the condition often fuels fears that the condition will progress rapidly without being able to do anything to stop it. These in turn will have an important negative effect on the patient’s well-being but also generate a major psychological burden affecting the self-esteem and social relations. Aesthetics also play an important role that should not be underestimated.Although knowledge about the disease "lipedema" has improved significantly in recent years, especially since entire health systems have considered giving it more attention, it is often based on incorrect information, assumptions, and facts. One thing is certain: there is a general lack of information on the subject of lipedema. This starts with the scientific side, continues with the specialized knowledge, and ends with the treatment, whether conservative or surgical. Therefore, it is important to us that we approach the topic of "lipedema" systematically and illuminate it from all aspects.We hope, through this book, to offer at least a small contribution towards a much better understanding of this complex disease. Irrespective of this, professional soci-eties, physicians, and centers must continue to work on transparency and knowledge transfer on all fronts in the future. Only in this way can we come closer to the goal of providing the best possible care for those affected.With this book, we would like to provide those affected by lipedema with a prac-tical guide.We wish you an informative read.Niedersachsen, Germany ZaherJandaliNiedersachsen, Germany LucianJigaGenova, Italy CorradoCampisiviiThe Rationale for the BookThe idea for the book had been floating around in our minds for a long time, but as is often the case, there is simply not enough time to implement such projects.The idea finally took form during one of the yearly meetings we organized for our colleagues for education and training in the field of lymphatic and lipedema surgery. I remember well when we talked about the poor transfer of knowledge to those affected. It doesn't even start with the patients; it starts with us, the physicians! There are many colleagues who have never heard of lipedema or lymphedema and are not familiar with the differentiation between these two conditions. What level of knowledge should the patients have about this disease?At the same time, talking about our scientific projects we found out that we do a lot in the field of lymphatic surgery, but quite little to nothing in the field of lipedema, although this issue accounts for a much larger share in our clinical routine. Furthermore, contemplating the actual evidence on lipedema we identified a major gap in both medical and patient-oriented literature.Our motivation for this book was to convey the current state of scientific knowl-edge in terms understandable for our patients and everyone interested to learn more about this condition.AL GRAWANYixAcknowledgementsMost readers see the editors and authors as the creators of a book. As this might be true for the idea and content, completing such a task takes much more than these to achieve. As such, we would like to thank all who helped and supported us from the bottom of our hearts starting with our patients who offered us their unbounded trust, motivating us to write this book. For us, it is a matter of the heart to help patients affected with lipedema.We would like to thank Mrs. Jandali and Mr. Stober (www.svenstober.com) for the great pictures, with the help of which readers will definitely get a much better view of the individual explanations throughout the book.We owe a big thank you to Springer, who believed in our idea and recognized the need for this book. Special mention should be made of the project planner Ms. Kraplow and project manager Ms. Beisel. We have rarely experienced such dedi-cated cooperation.Very special thanks go to our proofreader Mrs. Thürk. From the first moment of cooperation, we felt very well taken care of. The proofreading made the book an easy lecture, giving it a great boost. Thank you for your patience with us. It was certainly a difficult task, which you mastered brilliantly.http://www.svenstober.comxiAbout this BookThe idea to write this book was fueled from many years of experience in dealing with affected patients and their families. With this book, we have set ourselves the goal of giving the reader an in-depth overview on all aspects related to “lipedema” helping you to make your own treatment decisions.At the beginning of the book, the current scientific knowledge is summarized. In doing so, we dispel many half-truths and myths. In a structured way, the cause of lipedema, its clinical picture, and how this disease manifests itself are explained. Finally, its consequences as well as supposedly similar diseases such as pure lymph-edema and obesity are discussed. Furthermore, wein sum-mer. Like Hines, who initially described edema, I checked this out myself in the summer: I measured my leg circumferences right after I got up in the morning and at the end of a long consulting day. I am athletic, healthy, and fit. Yet, at the end of the day, I was able to measure a circumference increase of 3–5mm on average. We are all subject to such minor variations throughout the day. Then there are the additional influencing factors such as weather, activity, and much more.In many countries, knowledge about lipedema has already been consolidated to the extent that it is known that lipedema is not usually accompanied by edema requiring treatment. Therefore, we see the understanding of edema in lipedema as more of a national problem.1 The LipedemaAL GRAWANY24 c In lipedema patients, there is usually no edema in the classical sense. Only very rarely are forms of combined lipedema or lipo- lymphedema seen.CausesLet’s dive a little deeper into the subject of edema. If there is increased pressure in the venous circulatory system, fluid can be forced out of the bloodstream into the surrounding tissue. Most often we see this phenomenon in heart failure, so-called cardiac insufficiency, because the blood backs up in front of the heart, increasing the pressure in the venous part. Varicose veins in the legs may also be responsible for such increased pressure in the venous leg. Regardless of the causes, it is typically the legs that are affected by edema. The reason for this is gravity, which is followed by the fluid in the tissues and is thus deposited at the lowest point of the body.Another cause of edema can be an altered colloid osmotic pressure in the blood. This sounds complicated however the facts are simply explained. To keep a certain amount of fluid in the blood vessels, the blood contains large protein molecules that attract the fluid to them. If the protein molecules are decreased, then the colloid osmotic pressure decreases and the proteins can no longer “hold” the fluid in the blood. The fluid migrates into the surrounding tissue, and edema develops. We often see edema in kidney diesease patients, for example, who lose a lot of protein in their urine. However, the lack of protein can also have another reason, for example, mal-nutrition, liver disease or metabolic disease.Another possible cause of edema is vascular disease. This can result in increased permeability to fluids, which ultimately leads to fluid leakage into the surrounding connective tissue.If a thrombosis (blockage of a blood vessel) develops or if there is a weakness of the venous valves in a leg vein, this also results in a backlog of venous blood with a corresponding increase in pressure in the system. The pressure forces fluid from the blood vessel system into the connective tissue.Medications can also cause edema. Here, blood pressure medications and diuret-ics (water tablets) are at the top of the causative list. All of these edemas can undoubtedly be detected by ultrasound, MRI, or even other examinations (usually a simple indentation of the skin with the thumb is enough).Theory of Increased Capillary PermeabilityThe most widespread theory of edema in lipedema is the theory of increased “capil-lary permeability.” Lipedema is associated with, as the name suggests, increased permeability of the blood vessels (permeability) to proteins and water.This happens at the level of the smallest vessels of the capillaries, a small but crucial section in our blood circulation (Fig.1.16). Our blood circulation consists of arteries, veins and lymphatic vessels. Arteries carry oxygenated blood from the heart to the periphery, organs, brain, and all other tissues. At the capillary level, there is an exchange of oxygen, nutrients and waste products. This naturally results in some fluid transfer from the blood vessels and the capillary bed into the connec-tive tissue.Z. Jandali et al.25If there is increased capillary permeability for proteins (proteins) and water mol-ecules, these pass into the connective tissue. Proteins are very large in relation to water molecules so that we speak here of macromolecules. They bind the water in the connective tissue and in this way cause manifest edema (Fig.1.17).Initially, the lymphatic system can still remove the increased tissue fluid and compensates for the situation. Many people speak here of a high-performance phase of the lymphatic system in the initial stage. However, after a longer period, this leads to an overload of the lymphatic system. This phase is also called the decom-pensation phase. In the further course, fibrosis of the lymphatic vessels occurs, resulting in a loss of inherent elasticity. This results in a further, progressive loss of function, and edema takes its course.Fig. 1.16 Blood circulation of the body1 The LipedemaAL GRAWANY26The capillaries not only become leaky to the proteins; it is suspected that they are then also more vulnerable than “healthy” capillaries. In this so-called microangi-opathy, there is localized damage to the end-stream pathway with partial or com-plete loss of function of the smallest arteries (arterioles) and capillaries, so that they are injured more quickly and bruising can result.The second explanation for the bruises we have just discussed is the theory with the lack of oxygen and the signal to the tissue: “We need more blood vessels.” It should be noted that the new vessels suffer from capillary fragility and are more fragile than healthy vessels. c Those affected by lipedema have a tendency to bruise (hematoma tendency).Progression of lipedema results in further stretching of the lymphatic vessels and the formation of microaneurysms (vascular bulges). However, other studies contra-dict this theory. In our opinion, lipedema is not and does not remain a primary dis-ease of the lymphatic system. c We do not consider lipedema to be a primarily lymphatic disease.Let’s summarize the causes of edema in people suffering from lipedema:As in healthy individuals who do not suffer from a painful fat distribution dis-order, the most common type of edema is orthostatic edema (sagging of tissue fluid caused by the upright gait and gravity). The initial publication of lipedema also described edema in terms of “orthostatic edema,” which occurs in response to position and weight-bearing. However, such edemas have nothing to do with lymphedema. Especially these edemas occur preferentially in the warm sum-mer months.Other causes can be the diseases described above. Of course, a combination of different causes is also possible.Change in capillary permeabilityNormal capillary permeability Increased capillary permeabilityVeinLymphatic vesselCapillary vesselArteryVeinLymphatic vesselCapillary vesselArteryFig. 1.17 Increased permeability of the capillary vesselsZ. Jandali et al.27Obesity-Associated LymphedemaLet’s now go into a little more detail about edema in overweight patients. There is a proven link between obesity and the development of leg edema. Approximately 20% of all German citizens are classified as obese. Among those suffering from edema, the proportion of obese people is significantly higher than in the normal population. Only up to 30% of edema patients have a normal BMI.Here, the ques-tion is certainly justified as to whether obesity or edema is present first since BMI inevitably increases with pronounced edema. We see obesity-associated lymph-edema particularly in patients with a BMI above 40kg/m2.Obesity-associated lymphedema, like lipedema, tends to be a diagnosis of exclu-sion. We typically observe the following circumstances: – Lymphedema is not congenital and develops only in the second half of life. – Lymphedema increases with weight. – The feet are usually not affected, and Stemmer’s sign (a diagnostic skin fold test after its first describer, Robert Stemmer,Sect. 1.9) need not be present because lymphedema tends to occur in the upper part of the lower extremity, that is, the buttocks, hips, upper and lower legs. – We also see this form of edema in the area of fat aprons and the genital region. – Skin changes are regularly observed in advanced stages.The underlying pathological mechanism is represented by a circulatory distur-bance of the lymphatic and venous systems due to the mass and deadweight of the adipose tissue and soft tissue excess. The vessels are “squeezed,” which prevents further transport in the vascular system. c If an increase in edema is observed along with weight gain, then these can often be positively influenced by weight loss.We see edema in lipedema, especially in advanced stages. In these stages, there is usually also very pronounced obesity, so that it can be difficult to find the cause of the actual edema. Unfortunately, it is also not uncommon that water tablet abuse is practiced and thus edema is provoked.In summary, so far, we can state the following reasons for the presence of lipo lymphedema: – Presence of lipedema and lymphedema independently of each other. – Obesity-associated lymphedema. – Edema resulting from very advanced lipedema, even without obesity.At first glance, it is often difficult to distinguish lipedema from lipo-lymphedema (Fig.1.18). What do you think? Is it pure lipedema or lipo-lymphedema?In this case, lipo-lymphedema is indeed present (Fig.1.19). The lower legs and feet can be pressed in with a dent remaining for a long time as a result, which is indicative of lymphedema.1 The LipedemaAL GRAWANY28 c Edema in lipedema is rare.Theory of Edema in the AdipocyteThere is another interesting theory on the subject of “edema in lipedema,” and that is that the edema is IN the adipocyte (the fatty cell) that is, that the fat cells accumu-late and store more water.Fig. 1.19 Evidence of lipo lymphedemaFig. 1.18 Lipedema or combined lipo-lymphedemaZ. Jandali et al.29We have already discussed the cell structure of a fat cell in Sect. 1.2.1. Here we briefly summarize once again: A cell membrane separates the cell from the envi-ronment and within this, all components are surrounded by the cell water, the so-called cytoplasm. As the name “cell water” implies, the cytoplasm consists mainly of water. In the cytoplasm, we find the cell organelles, including the cell nucleus.The fat cell stores fat in a fat vacuole. The ratio between nucleus and cyto-plasm as well as cytoplasm and fat vacuole can be measured and determined. From this ratio, the cytoplasmic content can be measured and evaluated. There is currently no study that we are aware of that describes a difference in cell–cyto-plasm ratio and proves increased water retention in a fat cell in lipedema. In the case of increased water storage, the cytoplasm ratio would be larger (Fig.1.20). Rather, we suspect that a different development occurs: as the adipose tissue and thus the volume of the fat vacuole increases, the ratio of cytoplasm present could decrease.Thus, the claim that a fat cell in lipedema stores water is rather false or at least not proven and thus baseless.How does edema show up clinically? Indentable edema is more likely to be a sign of venous weakness, liver, cardiac, or renal disease. When pressure is applied to nondepressible edema, no dent remains in the tissue. Lymphedema may or may not be compressible, depending on the cause. The swelling or “edema” in classic lipedema is usually not compressible, so we talk about lipo-lymphedema.We would like to show you an example from our practice that speaks against the edema theories presented. We perform many examinations of the lymphatic vessels in our lymphedema patients. For this purpose, we stain the lymphatic vessels to make them visible. By injecting a dye for lymphatic vessels, ICG (indocyanine green), in the area of the feet, we can visualize and examine the lymphatic vessels with the help of an infrared camera after a short waiting period. The dye is first injected into the space between the toes. After a short waiting period, the camera is used to examine the extent to which the lymphatic vessels absorb and remove the dye. In healthy people, the dye is taken up into lymphatic vessels and move along proximally without any sort of diffuse distribution in the tissue. In lymphedema, we regularly see a diffuse cloud or at least severe irregularities in the distribution pat-tern of the dye. We have also performed this examination in lipedema patients and have not seen any irregularities in the ICG uptake. This is purely an observation from our own experience.NucleusCytoplasmaFatFig. 1.20 Water retention in the cell1 The LipedemaAL GRAWANY30 c To determine whether you are suffering from edema, a simple examination is sufficient: press the tissue in the area of your front lower legs deeply. If a dent appears that does not disappear immediately but takes 2–3s or longer, then it is possible that you are indeed suffering from edema, which is a rarity.1.4 PainThe indication of pain in the presence of a fat distribution disorder is THE decisive criterion for the diagnosis of “lipedema.” Pain is the leading symptom of lipedema. It is important to note that related to pain, there is NO scientific evidence as to how the pain is actually triggered. What we do know for sure, however, is that it is located in the subcutaneous adipose tissue. Thousands of women suffer from this chronic pain in the buttocks, hips, legs, arms, and sometimes other regions. At the same time, we know quite different descriptions of pain in lipedema. The classical indicated pains are. – pressure pain – tearing pain – strain pain – touch pain and – pain at rest.It is typical for lipedema to be associated with hypersensitivity to touch and pain.Pain DevelopmentAlthough it has not been proven, the question is how the pain is caused. One theory is that the pain is caused by pressure in the tissues. Increased capillary permeability (permeability of the smallest blood vessels) to water and proteins causes the edema already described in the subcutaneous fat tissue. This is followed by pressure- induced tissue overstretching, which triggers pain via the activation of pain receptors.For comparison, let us look at patients suffering from pure edema due to cardiac insufficiency, medication, or other diseases. These patients report NO form of pain in the areas of edema. Thus, we do not assume that it is the edema that triggers the pain in lipedema. Lymphedema patients also do not usually report pain, at least not in the same way as lipedema patients.The theory is also questionable because there is usually no edema in lipedema. c Pain is not caused by edema.In contrast, the following mechanism is more plausible: In lipedema, there is an undersupply of oxygen in the adipose tissue. This triggers an inflammatory reaction, which in turn triggers vascular sprouting (fragile blood vessels), fibrosis (connective Z. Jandali et al.31tissue proliferation). Furthermore, the lack of oxygen leads to increased fat cell death, which exceeds the natural death in the fat cell cycle. This observation was demonstrated in the laboratory via antibody assays. In the regions of oxygen defi-ciency, there was an increased number of phagocytes, which belong to the white blood cells, the leukocytes. Phagocytes are part of the body’s innate immune response and may also be an indication of an inflammatory response. Other studies suggest an insidious, chronic inflammatory process.Others suggest that excessive fat storage in fat cells causes a stress response, which in turn leads to the release of pro-inflammatory factors from the fat cells themselves and the immune cells in the adipose tissue. No matter how we spin it, everything points to an existing inflammatory response that irritates nerve receptors,which can trigger pain.Described theories are a combination of scientifically proven facts, conjecture and hypotheses. That is all we can offer from our ranks on the subject of pain causation.Complaints in the sense of pain often occur in the later stages. Nevertheless, we see sufferers in very early stages with markedly severe pain. Consequently, pain intensity does not seem to correlate with disease progression. c The visual stage of the disease “lipedema” does not correlate with pain intensity. Although the pain is usually pronounced in later stages, we also see women in early stages with very severe pain.1.5 A Chronic-Progressive Course?Let us turn our attention to another often postulated claim concerning the course of lipedema. Many speak of a chronic-progressive evolution. Chronic-progressive means that the existing lipedema continuously and inexorably worsens. Very often, it is precisely this fear of uncontrolled and permanent progression of lipedema that brings those affected to us. These fears are fueled by Internet portals or media who also propagate a cure through holistic liposuction. This claim is additionally under-lined by extreme patient examples, which we all know from the internet, alongside texts like “Who wants to end up like that” all these rattling the conscience of those affected. This is, of course, pure nonsense.If we break this assertion down into its component parts, we are talking about the words chronic and progressive.Chronic refers to the period. If lipedema with its symptoms is present for more than 6months, we can safely speak of chronic disease.Progressive or progressive means “progressing.” If we put the words “chronic” and “progressive” together again, then this is meant to describe a long-lasting dis-ease in which the manifestation of the symptoms increases continuously over a long period. However, this course does not apply to the disease “lipedema.” However, from our point of view lipedema must be understood as a “disease that progresses chronically in episodes.” What does this small but subtle difference mean?1 The LipedemaAL GRAWANY32Let’s look again at the development of lipedema: lipohypertrophy develops into lipedema (pain is added). Lipedema can remain at this stage for years or forever. Often, there are one or more relapses (hormonal changes), which then aggravate the lipedema. Nevertheless, the fact remains that lipedema does not worsen in every patient. We know some affected people who are 80years old and have been in stage I for what feels like an eternity. It should be noted that the stage does not indicate the severity of the symptoms. c Lipedema is NOT a chronic progressive disease, but a chronic relapsing disease.Nevertheless, in clinical practice, we see massive numbers of patients with lipedema that increase over the years. The reason for this is either the aggravation of the existing hormonal harmony (estrogen-progestin) or progressive obesity, which is wrongly distributed due to the congenital fat distribution disorder aggravating the situation.There is one thing that everyone is sure to agree on: if weight gain occurs in the presence of an existing disproportion in the sense of lipohypertrophy (without pain) or lipedema (with pain), this will always have a negative effect on the affected regions. This fact has been known since its first description in 1940.1.6 Obesity andLipedemaThe number of massively overweighted patients we see in our ambulatory center is steadily increasing. As we will see, there is a link between lipedema and obesity. Worldwide, obesity has increased dramatically in recent years, which also explains the significant increase in lipedema cases.Obesity does not have a good reputation worldwide and is often associated with own failure, lack of discipline, loss of control, and many other prejudices. Many of us know how difficult it is to control our weight or even lose a few more pounds. No one can count on help from the food industry, which contributes quite a bit to the calamity. Many resign themselves over time, and the frustration grows greater and greater with each failed attempt to control weight. We are all human and when we fail—perhaps without realizing it—we look for explanations. When we fail at some-thing, we like to subconsciously explain to ourselves that no one can do it and/or there must be some other reason. If we then encounter the thesis that you can’t lose weight with fat legs, we gladly accept it and feel a sense of relief. For this reason, the statement: “Whoever suffers from lipedema cannot lose weight” has become quite persistent and has even arrived in the professional literature. And that’s exactly how convinced those affected tell us in our consultations—without knowing where this insight comes from. We have not seen any proof for this thesis until today.Of course, we cannot make it so simple for ourselves and will illuminate the con-nection with “weight loss and shape change” in lipedema in detail in Chap. 3, “Treatment of lipedema” and put our statement into perspective.Z. Jandali et al.33Some medical colleagues also use lipedema for themselves. If you don’t know how to treat it, dubbing it “lipedema” comes in handy, true to the motto: “There’s nothing we can do about it—that’s what everyone wants to hear and that’s that.”“I’m not fat, I suffer from lipedema.” I stand behind this statement, as long as we are talking about sufferers who are not extremely obese. For those who are extremely obese, it should correctly read, “I suffer from obesity and lipedema.” Consequently, my first statement applies only to sufferers who are of normal weight or only slightly overweight. A fat distribution disorder in favor of the legs with an otherwise slim figure has nothing to do with “being fat.”Obesity or adiposity (from Latin adeps=fat) is a nutritional and metabolic dis-ease. Obesity is characterized by an increase in body fat above normal levels. Obesity was classified as a disease by the World Health Organization many years ago. There is even talk of a so-called obesity epidemic. c Obesity is a widespread disease.Obesity is not “a few kilos too much,” but a significant excess of body fat tissue. This condition can cause a whole range of diseases that not only significantly reduce the quality of life but also shorten life expectancy.When it comes to obesity, it is important to be able to estimate your own body weight. To do this, one must be able to put the excess weight in perspective. To assess obesity, the BMI (body mass index) has become widely accepted. BMIbody weight [kg]body height body size m�� �� ��2 If the BMI value is around 25kg/m2, there is little risk of developing secondary diseases in the future due to being underweight or overweight. On the other hand, if the BMI is around 27kg/m2, you have the highest life expectancy ever.Did you know that being underweight is much more harmful to our health than being overweight? Being underweight is associated with diseases of the body much faster than being overweight. For a person who is 160cm tall and weighs 60kg, being 50% overweight, that is, a bodyweight of 90kg (BMI 35.19kg/m2), is harm-ful in the long term, but being 50% underweight, that is, a bodyweight of 30kg (BMI 11.72kg/m2), is life-threatening and virtually fatal. From a BMI of 13kg/m2, inpatient treatment is required due to severe malnutrition and danger to life. Anorexia is defined as a BMIoverweight or a more severe form. By definition, we speak of obesity (adiposity) from a BMI of 30kg/m2. All higher BMI values are then only used to distinguish the severity of obesity (Table1.2, Fig.1.21).1 The LipedemaAL GRAWANY34Table 1.2 Weight classes depending on BMI (according to WHO classification)BMI in kg/m2 Type40 Obesity/obesity grade IIIFig. 1.21 ObesityZ. Jandali et al.35A BMI of 30–34kg/m2 is referred to as obesity grade I, a BMI of 35–39kg/m2 is referred to as obesity grade II, and a BMI of >40kg/m2 corresponds to obesity grade III.The higher the obesity, the greater.A BMI of 30–34kg/m2 is referred to as obesity grade I, a BMI of 35–39kg/m2 is referred to as obesity grade II, and a BMI of >40kg/m2 corresponds to obesity grade III.The higher the obesity, the greater the risk for secondary diseases and a short-ened life expectancy.Possible CausesObesity develops whenever energy intake exceeds energy consumption. There can be very different causes for this. c Due to the high number of overweight or obese lipedema sufferers, the number of normal weight sufferers is underrepresented. There is no scientific evidence that lipedema leads to obesity.Very decisive in the development of obesity seems to be the genetic factor, that is, the inherited predisposition, to obesity. Very rarely, only one gene is responsible for present obesity. In these cases, we speak of rare monogenic forms (i.e., one gene). Much more frequently, there is an interaction of several genes (polygenic forms). Each interacting gene then contributes to obesity. Targets of these genes can be appetite control, weight regulation, energy balance, hunger-satiety regulation, and food intake. It is believed (twin studies) that our body weight is about 70% determined by our genes. c Obesity has many underlying causes. Approximately 70% of our body weight is predetermined by our genes.Obesity can also be triggered by metabolic disease. Examples here are an under-active thyroid gland or an overactive adrenal gland. It is equally possible for obesity to occur as a result of surgery on a body gland or the brain. Another reason for weight gain may be medication use. Examples include the use of cortisone, hor-mone preparations, and psychotropic drugs such as antidepressants. c Only in a few sufferers are metabolic diseases, surgery, and medication use responsible for weight gain (estimated at 1in 100 sufferers).Weight and AgeMiddle-aged women are a special case. Almost 2/third of women aged 40–59 and about 3/fourth of women over 60in the United States are overweight with a BMI over 27kg/m2. After the age of 40, there is an average annual weight gain of about 0.7kg. These changes lead to a decrease in both rest- and activity-related energy expenditure. Therefore, aging leads to weight gain unless there is a compensatory change in dietary habits and physical activity.1 The LipedemaAL GRAWANY36In addition, there are symptoms such as mood swings, loss of drive and motiva-tion, sleep disturbances, and the classic complaints of the musculoskeletal system. The preceding symptoms also support further weight gain.There is a change in body fat distribution from the premenopausal gynoid pattern (pear type) to the postmenopausal android pattern (apple type).Fat DistributionApple type and Pear type? That is only figuratively speaking! If we look at the gender-specific characteristics of fat distribution, we often see in men belly- emphasized fat tissue storage with fat deposits around the internal organs, but also in the subcutaneous fat tissue on the abdomen. Here we speak of the central type, the “apple type.” The situation is different in women, in whom the adipose tissue is already located to a greater extent in the buttock–hip area for genetic reasons. This distribution is referred to as the “pear type” (Fig.1.22).The transition from pre- to postmenopausal is often also the time when lipohy-pertrophy first develops, painfully changes to lipedema, or when preexisting lipedema worsens. Lipedema adipose tissue, often referred to as “lip fat,” is often initially found in the bulbous regions of obese individuals. We try to avoid the term Fig. 1.22 Apple and pear typeZ. Jandali et al.37“lipid fat“because it suggests that one can distinguish adipose tissue of lipedema from healthy adipose tissue with the naked eye or via ultrasound, when in fact only some immunohistochemical markers are able to do so. In only slightly overweight and slender women, however, we often find the fat distribution disorder in the area of the entire lower extremity, often as a so-called columnar leg, where the entire leg contour resembles a column (Fig.1.23).The male apple type is much more harmful than the female pear type. Especially dangerous is the abdominal fat, that is, the fat around the organs. Fatification of the abdomen—and this has been scientifically proven—has a det-rimental effect specifically on the function of the internal organs. For women, an abdominal girth of 80 cm or more and for men, 94 cm or more is considered harmful (Fig.1.24). c Waist circumference is associated with the amount of “belly fat” and is closely related to cardiovascular disease.Calorie TurnoverAs already mentioned in Sects. 1.2.3 and 1.2.6, we often do not use as much energy as we consume. The surplus is simply not utilized but is stored in the adipose tissue. Moreover, if one suffers from an estrogen receptor distribution disorder, as is sus-pected in lipedema, even less energy is probably sufficient to store fat.In the past, storing fat was necessary for survival, because we didn’t know when we’d next have something to eat. We built up a bacon coat for the winter as heat insulation and energy storage. Nowadays, this is different. Our food supply is Fig. 1.23 Examples of different column legs1 The LipedemaAL GRAWANY38secure. Nevertheless, our body still thinks and works according to the old principles. Eat whenever possible! For us humans, the development into prosperity simply hap-pened too fast for our behavior to have been able to adapt through evolution. Our eating behavior, thus, lags far behind the current supply. We must therefore actively take countermeasures to change our behavior.Our daily total caloric expenditure is on average 2300kcal and consists of a basal metabolic rate and activity metabolic rate. The basal metabolic rate can be roughly estimated. Multiply the body weight by 24 and you have a very good approximation. c The caloric basal metabolic rate can be roughly estimated using the formula: Bodyweight × 24.But if you want to know more about it here in detail:The basal metabolic rate is the amount of energy required by the body at rest and at indifference temperature (28°C) during 1 day (24h) to maintain its function. It is, so to speak, the “standby turnover.” Of course, many factors play a determining role in the basal metabolic rate, for example, – Age. – Gender. – Bodyweight. – Body size. – Proportion of muscle mass.Fig. 1.24 Circumference measurement at the waistZ. Jandali et al.39 – Ambient temperature and thermal insulation by clothing as well as. – General state of health.To calculate the basal metabolic rate, the “Harris–Benedict formula“has proven itself. The value is not exact, but a very good approximation to the real basal meta-bolic rate. In the basal metabolic rate, the brain, heart, and kidneys have the highest demand for energy.For women: Basal metabolic rate , body weight/ ( ,kcalhkg24655 1 9 6� � � � �� �� 118 4 7, body height ,� � �� � � � � �� �cm age years Although not many men will read it, here the formula for comparison: Basal metabolic ratecal, body weight kg/ ( ,kh2466 47 137� � � � �� ��� � � �� � � � � �� �5 6 8body height cm , age years If we now go into further detail, we can name the energy metabolism of various tissue components (Table1.3). Although the brain accounts for only about 2% of body weight, it consumes quite a lot of our daily basal metabolic rate. We can roughly say that 20% of the basal metabolic rate is consumed by the brain. 500g of brain mass consumes about 110kcal per day. Our brain has an approximate weight of 1500g, which makes a brain calorie basal metabolic rate of 330kcal per day. The special thing about the brain is that it can only burn sugar and takes it directly from food.Kidney tissue consumes about 200kcal per day. One kidney weighs about 130g. 500g of muscle mass consume 6kcal, 500g of fat 2kcal per day. c Our brain burns an average of 330kcal per day and can only utilize sugar.Power ConversionOur activity or power metabolism is the amount of energy that we burn in 1day with our body. This turnover is mainly generated by brain and muscle activity (work, leisure, sport). To calculate the conduction metabolic rate, we use the PAL value (“Physical Activity Level”; Table1.4; Fig.1.25).Table 1.3 Energy metabolism by tissueTissue Mass (g) Consumption per day [kcal]Brain 1500 330Kidney tissue 130 200Muscle tissue 500 6Fat 500 21 The LipedemaAL GRAWANY40For this purpose, the factors are multiplied by the respective number of hours and then added. To obtain a daily average, this number must be divided by 24. To deter-mine the total energy requirement, the daily average is multiplied by the basal meta-bolic rate. The result is the average total energy requirement (Fig.1.26). The daily total calorie requirement thus varies from individual to individual.Let us now take a look at the calories’ consumption of other activities: We assume a person weighing 70kg and the calories consumption within 1h (Table1.5).Energy Intake and Eating BehaviorLet’s look now at energy intake and how we supply ourselves with energy. The “how” may sound strange at first, but our eating habits are more unfavorable than ever before. We are talking here about our eating behavior. We eat on the side, stand-ing up, and often stressed—all negative characteristics.Many eat only in between and unconsciously, for which they should not be blamed. In a big city, the most appetizing treats are held under our noses virtually every 50m. Not only in the pedestrian zones but also on the roadways we find invit-ing fast-food snacks against ravenous appetite. It’s often difficult to resist as we walk by, and if we don’t find anything as we pass by, we subconsciously know that an online ordering food center won’t let us down. This gives us added confidence Table 1.4 PAL valueFactor Activity Example0,95 Sleeping Night rest1,2–1,3 Sitting, lying Seniors, bedridden people1,4–1,5 Sitting, little physical activity Desk workers1,6–1,7 Predominantly sitting, walking, standingPupils, students, bus drivers1,8–1,9 Predominantly walking and standing Salespersons, waiters, mail carriers2,0–2,4 Physical work Construction workers, farmers, loggers, athletesFig. 1.25 PAL valuesZ. Jandali et al.41Fig. 1.26 Calorie requirements by activity levelTable 1.5 Calorie consumption within 1hActivity Kcal/hWeightlifting 224Water gymnastics 149Cycling (moderate) 260Rowing (moderate) 260Cross trainer 335Billiards 93Golf 130Walking (6,4km/h) 167Jogging (12km/h) 465Gardening 167Sleeping 23Cooking 93TV 28In standing in a queue 47Playing with the kids 147Wallpapering/painting 167Computer work 51Counter work 65Forestry worker 2981 The LipedemaAL GRAWANY42that we won’t go hungry. Conveniently, we kill two birds with one stone with deliv-ery services: first, we get the food delivered and don’t have to “pick or hunt” like we used to, because that also used to burn a very large percentage of calories. Second, we don’t even have to go to the trouble of preparing it! What a luxury we live in. What we don’t know is what’s really in the products. Besides flavor enhancers, there are huge amounts of fats and sugars in the products. Therefore, our food is often unbalanced and one-sided. Vegetables, fruits, and other things are completely miss-ing in many such items. Ready-made meals are often no better (Fig.1.27).We haven’t even discussed two major calorie bombs yet: sweets and soft drinks. In Germany, around 31kg of sweets are bought and eaten per capita every year, and Fig. 1.27 Healthy and unhealthy foodZ. Jandali et al.43on average, every German drinks around 133 liters of soft drinks, the liquid sugar bombs, per year. In the USA, on the other hand, 195l are drunk per person. A truly frighteningly high figure.Expectations of one’s own role and social stress are further factors. Eating disor-ders of various kinds are often present. Obesity due to a psychological cause (so- called psychogenic obesity) is not in itself a distinct clinical picture, although a very high number of people suffer from this cause of obesity.However, many of those affected are not even aware of the psychological back-ground of their suffering. Most often, binge eating is used to regulate emotions. Often, people with psychogenic obesity use food to cope with unpleasant or disturb-ing feelings such as loneliness, stress, anger, boredom, overwhelm, sadness, disre-gard, or trauma (whether violent or sexual). Sometimes weight gain is said to unconsciously cause one to acquire a “protective armor” and thus lose attractiveness.In many cases, those affected are internally oversensitive and vulnerable. Eating is used as a way of regulating feelings. Many speak of so-called substitute satisfac-tion in these situations.In addition, there are negative habits: Some eat when they are stressed, others when they are successful, and others eat throughout—whether at work or on the road—a little candy here and a piece of cake there.Did you know that with age the body learns to digest food better? Therefore, the nutrient yield in old age is better than in youth. Another reason why we get by with less food as we age.Finally, we have the suffering topic of smoking. Many people try to quit smok-ing, especially nowadays when smoking is becoming more and more frowned upon and unpopular. The undesirable side effect is an increase in body weight. The exact reasons why we gain weight when we stop smoking are many. In addition to an abolished appetite control, the “substitute drug candy” is often to blame.Energy Consumption and LifestyleAs with energy supply, we are also dealing with a multilayered issue when it comes to energy consumption. Our own lifestyle plays a major role. This is often inactive and passive. Even short distances are covered by a car. Hunting and gathering are long gone. We live in a society of comfort, in which we allow ourselves to be entertained.Psychosocial factors also play a role here. The influence of an intact family (nuclear family) and intact family life, as well as friends and circle of friends, are important for our activity.Do you walk the stairs or take the elevator? Even up to the fifth floor? What about sports? Do you go to the gym or have you only been paying your contributions for years?Our very own biology doesn’t help us to keep our weight either because in prin-ciple we only have two programs inside us. One program tells us to eat whenever we find something to eat (we just discussed that). The second program tells us to con-serve as much energy as possible, that is, to move minimally to stay at maximum 1 The LipedemaAL GRAWANY44strength. That’s why we feel so comfortable “chilling.” In the past, at least, we burned energy hunting and gathering. Today we reach for the tablet or cell phone. We prefer cars and other means of transportation and are happy to leave the bike behind. For the most part, walking no longerfinds a place in our fast-paced times. Our workplaces often involve a lot of sitting, and you can already tell we’re “chill-ing through life.” One could cite many more examples here that would underline our “chill mentality.”In addition, there is the psyche, which we will discuss in detail in the following section. The background is that lipedema sufferers, who suffer from chronic pain, exclusion, and strong dissatisfaction with their appearance, are often plagued by depressive mood or actual depression.1.7 Complaints andEffects ofLipedemaWe have already talked about pathomechanism, which is still unknown to us, and obesity, which often occurs in combination. Now it is time to talk about the symp-toms and the course of the disease “lipedema.” Since lipedema can be a chameleon, there are statements we can make in general, but likewise, there are always exceptions.Typically, lipohypertrophy starts in the area of the lower extremities (incl. Buttocks and hips). Accordingly, it is often seen at the first moment merely visually: “Something is wrong here.” Subsequently, those affected notice a feeling of heavi-ness in the legs and only then, in the further course of the disease and the later stages, there is a feeling of tension and pain in the area of the affected regions.Most often we see that lipedema starts in the lower half of the body and then moves to the upper part of the body. c Typically, lipedema begins after the onset of lipohypertrophy in the lower extremity. Only in the later stages can the upper extremity be affected.We see a wide range of sensitivity to touch and touch pain in our patients. For many, it is even the smallest touches and strokes that can trigger an unpleasant feel-ing or even pain. This often leads to a withdrawal from the own relationship with the resulting further conflict-building sites.From our clinical experience, further typical complaints are feeling tension or pain in the sense of pressure pain, touch pain, or tearing pain. Mostly the complaints occur during the day and bring restlessness in the legs. In many cases, the pain is most pronounced in the evening. If there is temporary or permanent edema, then a worsening of the symptoms with an increase in edema during the day is often reported.The curious thing about our current system is that if you suffer from a fat distri-bution disorder, you are only recognized as “sick” if you have pain. If one has no pain, one is considered healthy. In fact, one is treated only when one reaches a Z. Jandali et al.45certain visual stage, but this has nothing to do with pain. If one does not see any-thing visually or if one can only rudimentarily recognize a fat distribution disorder and the patient states severe pain, she is still considered healthy (or a spinner, which is even worse). But even if both criteria apply, that is, a fat distribution disorder is associated with pain and the patient is overweight, she will not have an easy time with treatment.We see very many affected persons—whether normal or overweight—who do not suffer from pain but a restriction of their mobility. Regardless of the visual stage, in our view, a restriction of movement is pathological above a certain level (Fig.1.28).It is often deforming fat pads on the inner thighs and knees (Fig.1.29) that cause restricted movement. In addition to a leg malposition, these lead to increased Fig. 1.28 Movement restriction due to excess skin and adipose tissue1 The LipedemaAL GRAWANY46sweating, redness, and itching. Often, the affected persons cannot do sports and are less resilient both at work and in their private lives. If skin flaps are present, moist chambers propagating fungal infections can form in these intercutaneous furrows.It is unfair if we recognize a fat distribution disorder as a disease only with pain. There are definitely complaints that can be associated with a fat distribution disor-der and thus also have a disease value (and we have not yet talked about the psycho-logical burden).Affected persons with a fat distribution disorder without pain, but with a restric-tion of movement due to the fat masses and skin flaps, are entitled to treatment like everyone else. Certainly, one must ask oneself which treatment would be the right one in these cases. For this, an individual therapy plan must be created.Be it lipohypertrophy or lipedema: many affected people also suffer psychologi-cally from the consequences of their external appearance. Often, this aspect is given far too little attention! From the very beginning, however, we have to distinguish between a burden limiting the feeling of being alive or the quality of life and a mani-fest, severe psychological disorder.Many, especially physicians and administrators such as health insurers, know only one or the other. We have a different view of things; from our perspective, there are many intermediate levels.The lightest form of a psychological burden (and in our eyes, it is not even a burden) is the subliminal dissatisfaction concerning external body appearance caused by lipedema or lipohypertrophy. We are not talking here about a body dys-morphic disorder, which is present when there is a pathological perception of one’s body image, but about psychologically stable women who are simply a little more than dissatisfied with one or another part of their body.Fig. 1.29 Deforming fat pads/lipedema type 4 stage IIIZ. Jandali et al.47If we go one step further, those affected often describe a “discomfort” with the external appearance. Although the “problem” is not always completely comprehen-sible objectively, it is recognizable and identifiable. Those affected are somewhat more isolated but still very active socially, however refusing to go to the swimming pools or the beach, so places where they are forced to expose their body.Higher suffering pressure usually exists in patients with a comprehensible objec-tive disproportion. Those affected often withdraw in social life and also in relation-ships. A depressive mood and mood swings are often described.In the case of pronounced deformities, we often see a very strong restriction of the quality of life up to withdrawal from the relationship and the social environ-ment. A very high level of suffering prevails, the entire life is restricted, with depres-sion and other mental illnesses requiring specialized treatment.These psychological components now have two effects: One is a lack of drive and thus a withdrawal from sports and general activities. Less exercise automati-cally reduces the daily calorie consumption, but at the same time, there is another effect, the reduction of muscle mass having a negative effect on the daily caloric basal metabolic rate. The second effect is the so-called “frustration” or “emotional” eating. In this case, food is used to process emotions—a vicious circle that is diffi-cult to break, and one that nutritional psychology deals with intensively. Sufferers often reduce themselves to their lipohypertrophy or lipedema and feel stigmatized accordingly. “Everyone stares at me and thinks I’m just fat.”In all of the described mental states, the affected persons can additionally suffer from pain. However, let’s assume that there is no pain, neither in the legs nor in the arms. Now everyone must decide for themselves to what extent we have a social responsibility to also take care of these affected persons and to reintegrate them into our society.Let us now deal with manifest lipedema patients. More than 60% of women with lipedema have a mental illness. These psychological conditions are not nec-essarily related to lipedema but have usually arisen independently of it before-hand. The reasons for such mental illnesses are often other, for example, physical illnesses, motivational disorders, burnout, depression, trauma in the sense of vio-lence and sexual abuse, family situations suchas separations, personality disor-ders, and so on. Often the underlying illness is an eating disorder. This is often disregarded or at least the aspect of “eating disorder” is often not taken seri-ously enough.Here, a mixture often takes place and lipedema is blamed for this situation. Only in a small proportion ofthe body. We count painful fat, outside the regions of hips, arms and legs as atypical lipedema. Many colleagues do not consider lipedema of the but-tocks as lipedema. We see it differently. The buttock region is certainly a region that is often painfully affected and forms a unit especially with the hip and upper thigh region. c By atypical lipedema, we mean lipedema that is not visible at first glance or painful adipose tissue in atypical regions. The term “atypical lipedema” has been coined by us.We would like to comment on the current type and stage classification.As we have already noted, the type and stage classification is merely descriptive. Neither the intensity of pain nor the extent of movement restriction nor the psycho-logical burden (manifest mental illness excluded) is taken into account.Unfortunately, in Germany, the staging is used to establish a medical justification for surgical therapy. This is a major problem. It is also difficult that in this classifica-tion a stage applies to the entire body. However, it may be that the disease progres-sion in the area of the thighs corresponds to a stage III, but in the area of the lower legs to a stage II or vice versa.So, we see that the classification is not quite as simple as it seems at first glance. As with many other diseases, lipedema is not only black and white but also gray, sometimes even light gray and dark gray.It is precisely this gray area that represents a major challenge in the treatment of lipedema. The many intermediate and special forms must be given appropriate indi-vidual consideration in therapy. It would therefore make more sense to use a clas-sification that takes more account of the pathological changes.In an effort to improve all these, we have developed our own classification. In addition to the classic types already described, there are buttock-only, hip-only, thigh-only, and lower leg-only types. Even though in our daily clinical routine these are rather rare, we see affected persons who only have complaints in the upper or lower arms or the whole arm. Therefore, we distinguish each individual body region Z. Jandali et al.53from one another in our classification (Fig.1.33). Depending on the affected region, a simple type code can be created. This means that we can always see exactly which regions are affected.By combining the numbers and letters, it is then possible to clearly determine exactly which regions are affected by lipedema. If, for example, both legs are com-pletely affected and both upper arms are affected, we speak of type VI/A, which is actually quite simple and straightforward. This classification makes it possible to combine very many variations of the affected areas.In our opinion, the current classification of stages also needs to be revised. There are indeed women who have strong or more severe symptoms in the early stages of I. Buttock II. Hip III. ThightIV. Knee V. Lower leg VI. Complete legA. Upper arm B. Lower arm C. Completed armFig. 1.33 New, individual type classification1 The LipedemaAL GRAWANY54lipedema than other patients in a higher stage. Unfortunately, this fact is not taken into account in the current staging, which severely limits its validity. c The current type and stage classification should be revised.In addition, the staging is spongy and not concrete enough. The transitions between the stages leave too much room for discussion.Therefore, we postulate not only a new type of classification but also a new stage classification, which we collect using a 21-page questionnaire tailored to lipedema and an associated score system. This results in a concrete diagnosis and stage of all affected regions. However, even our staging is only as good as the main symptom of the disease, pain, can be objectively assessed. Despite questionnaires, this is extremely difficult. The pain (P=pain) is then indicated using the score of 0–10 per region. We will go into more detail on establishing the diagnosis in Sect. 1.9.The stage also includes the regional manifestation of lipedema or lipohypertro-phy. For this purpose, we use a scale from + to +++ depending on the severity for the respective region. Furthermore, our staging classification indicates the extent of edema (E=edema) and restriction of movement (D=disability), each with a value of 0–4 (Table1.9). This staging is hardly suitable for everyday use, but it is recom-mended for medical documentation and better analysis of lipedema.Lipedema of the hip/thigh and lower leg and arms could be classified as follows: II +/P3/E0/D0—III +++/P8/E0/D1—IV ++/P0/E0/D0−A +/P2/E0/D0. The inter-pretation is presented in Table1.10.Table 1.10 Example classification of lipedema in the hip/thigh, lower leg, and arm areaClassificationRegional manifestation Pain P Edema EMovement restriction DII +/P3/E0/D0 HipLittle pronouncedLevel 3(lipedema, since >0)No edema NoneIII +++/P8/E0/D1 Upper legStrongly pronouncedLevel 8(lipedema, since >0)No edema LittleIV ++/P0/E0/D0 Lower legModerately pronouncedNone(no lipedema, since 0)No edema NoneA +/P2/E0/D0 ArmsLittle pronouncedLevel 2(lipedema, since >0)No edema NoneTable 1.9 New, individual stage classificationSymptom Abbreviation ScaleRegional severity – + to +++Intensity of pain P (pain) 1–10Extent of edema E (edema) 1–4Extent of movement restriction D (disability) 1–4Z. Jandali et al.55In this way, each region can be described in detail. For the sake of simplicity, we will continue to use the classic classification of lipedema in the remainder of the book.Another fundamental aspect that needs to be discussed is the naming of the dis-ease. The name “lipedema” is unfortunately only partially appropriate for the clini-cal picture, as it is always associated with the presence of edema, which is not true in most cases.The word “edema” often quickly establishes a close relationship to lymphedema. However, this is a completely different and independent clinical picture with no relationship to lipedema. The conclusion derived from this that lipedema is treated like lymphedema is also incorrect. The rare case of lipo-lymphoedema must be considered separately.The call for a new name is not new and is also advocated by other experts in this field. Let’s take a look at our neighboring countries and see what they have to say on the subject:This is how our colleagues in the United Kingdom view the issue in their guide-lines, “The management of lipoedema” (https://www.lipoedema.co.uk/):Lipoedema is predominantly a chronic adipose tissue disorder (the word lipoedema means 'fat swelling'), with clinically apparent oedema due to fluid accumulation in the tissues occurring as a secondary feature in some individuals (Todd, 2010; Herbst, 2012a; Reich- Schupke etal, 2013; Herbst etal, 2015). Although most commonly called lipoedema, the condition has a variety of other names.Meaningfully translated, this means:Lipedema is predominantly a chronic adipose tissue disorder (the word lipedema means "fat swelling"), with clinically apparent edema in the sense of fluid accumulation in the tis-sues occurring as a secondary feature in some individuals (Todd, 2010; Herbst, 2012a; Reich-Schupke etal, 2013; Herbst etal, 2015). Although the condition is most commonly referred to as lipedema, it has a number of other names.The Lipedema Guidelines in the Netherlands can be found on the Internet at the following address: https://nvdv.nl/. We take the liberty of reprinting this text passage:A major obstacle is the limited available scientific literature, along with inconsistency con-cerning the diagnosis of lipedema. The condition is characterized by symmetrical accumu-lation of fat tissue with typical clinical characteristics, generally in the extremities (more detail provided later). Lipedema is an unfortunate term, as it often evokes the idea ofswell-ing due to fluid accumulation. However, it refers to swelling—in the sense of an increase in volume—due to increased fat tissue.Meaningfully translated, this means:A major problem in lipedema is the poor scientific situation in terms of literature and incon-sistencies in diagnosis. The condition is characterized by a symmetrical accumulation of adipose tissue with typical clinical features, preferably in the extremities (more details later). Lipedema is an unfortunate term because it often evokes the idea of swelling due to fluid accumulation. However, it is a swelling—in the sense of an increase in volume—due to increased adipose tissue.1 The LipedemaAL GRAWANYhttps://www.lipoedema.co.uk/https://www.lipoedema.co.uk/https://nvdv.nl/56 c Conclusion: Lipedema should not be called lipedema, but rather adipose tissue disease.Lipedema is simply a fat disease. This group of fat diseases should then be sub-divided into subcategories, each of which has its own disease value (Table1.11). Lipo-lymphedema would not be affected by this and would remain as such.1.9 DiagnosisBy a diagnosis, we understand the determination and naming of a disease. In the diagnosis phase, we work toward establishing and naming the disease. c By a diagnosis we mean the identification and naming of a disease.With lipedema, the phase of diagnosis can be comparatively quite long. At least this is true for many lipedema sufferers we see in our practice and tell their story of suffering. Often it is because those affected do not know where and to whom they should present themselves. In addition, many colleagues are not familiar with the clinical picture and in many cases make an incorrect diagnosis or no diagnosis at all.Diagnostic CriteriaAs we have already indicated in Sect. 1.8, lipedema has a decisive disadvantage compared with other diseases: there are no objectifiable diagnostic criteria for lipedema. This means that no examination is available with which lipedema can be detected with certainty. There is neither an objective imaging nor an objective laboratory value nor an objective fine tissue examination (examination of a tissue sample) nor any other objective parameter that can undoubtedly diagnose lipedema.In this context, we speak of so-called soft and hard parameters. Soft parameters, in contrast to objective parameters, are criteria that can be interpreted in different ways. The soft parameters thus offer a large scope for discussion.For a better understanding of this issue, the following examples may help: 1. You stumble and fall on your right hand. Because you are in pain and your hand is also swollen, you consult a doctor. In this case, an X-ray of your right hand is taken for diagnostic purposes. If the X-ray shows a fracture, it is certain that the bone is broken. There is no need to discuss this.Table 1.11 Classification of adipose tissue diseasesForm TypePainful type Lipalgia or lipodolorosa (chronica)Movement-restricting types without pain Functional fat distribution disorderPainless type Lipohypertrophy/fat distribution disorderZ. Jandali et al.57 2. You suffer from shortness of breath and visit your family doctor, who will arrange various examinations. An ultrasound examination could then reveal, for example, that you are suffering from cardiac insufficiency. This also leaves rather little room for discussion.In both examples, the diagnosis is confirmed by an objective procedure that can-not be challenged.The situation is different with lipedema, where the objective criteria are com-pletely lacking. This is a major problem—a problem of traceability, a problem of recognition, a problem of billing, and a problem of differentiation from other diseases. But the problem is not just objective diagnosability. Rather, the difficul-ties begin with understanding the disease. To date, we know very little about the causes, development, or reasons for the development and progression of lipedema. A therapeutic approach aimed at curing the disease therefore simply does not exist.Due to the fact that these objective diagnostic criteria do not exist, there can also be no exact figures in terms of frequency and new occurrence of the disease. The figures that sometimes appear in the press are only rough estimates.Very often we experience in our clinical everyday life that lipedema is misdiag-nosed. On the one hand, this means that a diagnosed lipedema is not present in the affected person at all, or that an actual lipedema is not recognized as such. This is usually difficult for sufferers of both situations to understand. If we can confirm the diagnosis, we often see enormous relief in the patients. It is worse when we have to say that it may not be lipedema after all.In general, the earlier a diagnosis is made, the sooner treatment can be initiated. So if you think you suffer from lipedema, you should see an “expert.” How to find such a specialist, you will read in the further course.The path to diagnosis can vary greatly from physician to physician. In our prac-tice, in front of a case of suspected lipedema, we proceed by collecting 1. the medical history (anamnesis) of the person concerned. This step is fol-lowed by, 2. an observation (inspection) of the affected parts of the body and, 3. a physical examination (palpation).In all three parts of this protocol, there are typical constellations that speak for or against lipedema. The procedure is supported and recorded by a questionnaire.Medical HistoryTo begin with, we ask you what is the driving force behind your introduction to us. The answers to this question turn out to be very diverse. For many of those affected, the feeling of fear of suffering from a fat distribution disorder is at the forefront because they have noticed changes in shape that are atypical for themselves. Others have had chronic, almost unbearable pain for years. The range is really very wide. Classically, those affected report that a visible fat distribution disorder has already 1 The LipedemaAL GRAWANY58set in with the onset of puberty. For many, this is still painless at that time, for others it is already painful. c A fat distribution disorder/lipedema usually begins with puberty.Of particular interest to us is exactly which areas are affected. Here, too, there are definitely differences. While some complain of complaints in the legs only, others report pain in the legs and arms. For others, only the hip–buttock region is affected, or even a completely different region of the body.A relapsing course of the disease is often described. However, this is not always the case. We also see, for example, older patients in an initial stage in which they have been for several years without observing any progression. Very typical are relapses in phases of hormonal change. This also fits the theory of hormonal depen-dence. These include puberty, pregnancy, and the onset of menopause.Furthermore, it is interesting for us to know whether your mother, grandmother, or other relatives have or had similar complaints. It is not uncommon for a fat dis-tribution disorder to be inherited.Even if you have already described your complaints to us in detail, we will usu-ally ask you a few more questions. Not because we have not listened to you, but because there are some core questions that are essential for our and your diagnosis. Some of these questions revolve around the pain issue. Namely, if there is a painless fat distribution disorder, then by definition we are talking about simple lipohyper-trophy. If it is lipohypertrophy with pain, then by definition it is lipedema. It is therefore not a myth that lipohypertrophy is distinguished from lipedema solely by the presence of pain.The pain itself is usually described as rather nonspecific. However, both the qual-ity and intensity of the pain are known to exhibit a wide range. This is exactly why we haveour lipedema questionnaire. c Our lipedema survey form can be downloaded at http://www.lipold.de.Sample questions include: What type of pain is present? Pressure pain, tension pain, touch pain, or tearing pain? Does the pain tend to occur at rest or with exer-tion? Does the pain increase during the day? Is there also pain at rest?The pain symptomatology plays a major role for us, as the type and intensity of the pain often indicate certain other diseases that should be ruled out—even if lipedema is suspected. c By definition, a painless fat distribution disorder is not lipedema and is referred to as lipohypertrophy.In addition, regular feedback on the success of each treatment is important for us to be able to constantly adapt and improve it. The questioning of pain before and after an operation thus also serves our own quality assurance.Z. Jandali et al.http://www.lipold.de/59To record pain, we use a so-called visual analog scale in our questionnaire (Fig.1.34). The visual analog scale (VAS is used to record subjective attitudes, in this case, pain.However, the VAS can also be recorded in practice, for example, using a pain slider or in writing. In the written version, a horizontal bar is usually given, on which a cross is placed according to the extent of the sensation queried. Either we ask explicitly for a pain value (0=no pain, 10=strongest pain) or we use the template.With the stencil, the rating is done on the scale from one extreme (far left) to the opposite extreme (far right). Often equipped with a slider, this can be slid over the printed numbers from 0 to 10.However, not only the intensity and quality of the pain play a role for us. The question of the duration of the pain is also relevant for us. From a period of about 6months, we speak of chronic pain. Whereby the period for “chronic” is defined somewhat differently and generally lies between 3 and 12months. Ultimately, the decisive factor for our treatment strategy is to weigh up whether pain or dissatisfac-tion with the external appearance is the main issue. Filtering this out is, as you can imagine, a balancing act.In addition to pain, we are also interested in any kind of disturbed sensibility in the affected parts of the body. Is there a strong sensitivity to touch? For example, do you have a feeling of tension or heaviness in your legs or arms?What about bruises? Do you bruise quickly? Although bruising in lipedema is controversial, we raise this symptom as a possible indication of lipedema.What about water retention in the sense of edema? When do you observe this phenomenon and how does it behave during the course of the day? Does it improve or worsen during the day? How it responds to which treatment?Fig. 1.34 Example question from our lipedema survey questionnaire1 The LipedemaAL GRAWANY60We also ask about subjective physical limitations. To what extent is there a restriction of movement and thus a reduced performance capacity that prevents you from mastering your everyday life without complaints? Do you experience restric-tions at work? What about sports?Finally, to get a good overall view of the history of the disease, we also ask how satisfied you are with your body image, what stresses you experience as a result of this and how you deal with your situation. In the further conversation, we also go into your course of the disease and your previous therapy.InspectionNow that we have talked in detail about the entire complaints, your examination follows. During the examination, we determine to what extent we can objectively understand the fat distribution disorder described by you. It is important to look at the entire body, including the arms, hands, feet, and the entire torso area. It is impor-tant not to look at the affected regions in isolation, but to form a relationship between the different regions.In the case of a combination of fat distribution disorder and obesity, it is neces-sary to determine exactly which disorder is the main problem. This is crucial for the therapeutic approach. To objectify this relationship, there are parameters that help us. We will discuss these later in this section.But how obvious or how disproportionate does a fat distribution disorder have to be for us to speak of lipedema? Can lipedema still be in the foreground if obesity is pronounced, or should perhaps the obesity be treated first?These key issues, along with pain symptomatology, are the central questions that need to be answered in terms of the best possible treatment. c Lipohypertrophy/fat distribution disorder in favor of the extremities is a relatively sure sign of lipedema if pain symptoms are present at the same time.Difficult to assess is atypical lipedema, especially borderline cases, at both ends of the extremes. For example, there are patients in whom lipedema is ruled out because it is not outwardly obvious or is only very mild, but the patients neverthe-less suffer from lipedema. The same applies to very obese patients in whom the accompanying obesity may rule out lipedema from the outset for the inexperienced observer. This is a very unsatisfactory situation for both groups. Here it is necessary to make the correct diagnosis with a great deal of tact and experience. c There are forms of lipedema that exist WITHOUT an obvious fat distribution disorder, especially in very slim affected individuals.Another element we need to pay attention to is the skin. Questions we need to ask ourselves here are: What is the skin texture? Have flaps of skin already formed? Sometimes we see a so-called collar- or muff formation on arms and legs. This phe-nomenon describes an abrupt jump in caliber from the affected to the unaffected area, which then causes the external appearance of a collar or muff (Fig.1.35).Z. Jandali et al.61But beware. Skin-soft tissue excess, as defined for stage III, can also occur in patients after massive weight loss. We see a significant number of patients after gastric bypass and massive weight loss in which the clinical appearance resembles stage III lipedema, although there was never any discomfort or disproportionate fat distribution disorder.Palpation ExaminationThis is followed by a physical examination. Softness and turgor of the tissue? Is palpation painful?To check for the presence of edema, we first examine the pressure-related behav-ior of the tissue. We distinguish between edema that can be pushed away and edema that cannot be pushed away (the name “pushable” is unfamiliar, but it is the correct term for it). Edema that can be pushed away responds to pressure. Usually, this pres-sure is applied by the thumb or another finger during the examination. If the skin of an area of edema is depressed with the thumb, a “dent” or depression will remain even after the thumb is removed. This takes some time (longer than 2–3s) to level out to skin level again. In the early stages, edema can usually be pushed away, whereas in the advanced stages it cannot be pushed away due to the associated tis-sue changes and thus no “dent” remains in the tissue (Fig.1.36).After checking the impressionability, we examine the hands and feet. The aim here is to determine whether the backs of the feet and/or hands are also affected. The so-called Stemmer’s sign gives us a clue. To do this, an attempt is made with the index finger and thumb to lift a fold of skin over the second or third toe or the dor-sum of the foot. If an edema component is present, no skin fold can be lifted off and the Stemmer’s sign is positive (Fig.1.37). If no edema component is present, then a skin fold can be lifted off and Stemmer’s sign is negative. If Stemmer’s sign is posi-tive and a fold cannot be lifted off the dorsum of the foot, there is certainly NO pure lipedema. It could be lymphedema, lipo-lymphedema, or edema of a different etiology.Fig. 1.35 Collar formation at the transitiondiscuss the current classification we use of lipedema as we would like it to be.We then enter into our actual core topic, the treatment of lipedema. In addition to the aspects of the timing of a treatment, we deal with the different conservative and surgical options. At the end, we provide the reader with our recommendations for treatment.All the above are of course only rough headings of the topics that await you in this book. Look forward to exciting details and in-depth expertise to help you sharpen your view on the topic of “lipedema.”After reading this book you will be familiar with all actual treatment options for lipedema. In particular, through the information you will discover in this book, you will be given the chance to weigh up the advantages and disadvantages of each individual procedure according to your own symptoms. We are confident that by reading this guide, your level of knowledge and your self-confidence will increase, making the next visit to your doctor a “walk in the park” instead of a chaotic uncon-trolled input of unknown information.However, this book is neither thought as a substitute for a doctor appointment and since medicine is in a constant state of development nor as a complete or up to date reference for lipedema. Likewise, each chapter reflects the opinion of its respective authors.This book is NOT a scientific publication. It targets mainly the “non-medical readership,” who by reading it should gain a thorough understanding of this painful fat tissue disease.AL GRAWANYxiiNo before-and-after pictures of operations are shown in this book, so as not to give the impression that advertising for surgical measures is being carried out. If before-and-after images are shown, they are 3D illustrations and not actual images.Enjoy reading.About this BookxiiiContents 1 The Lipedema . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1Zaher Jandali, Benedikt Merwart, and Lucian Jiga 1.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1 1.2 Causes and Emergence. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4 1.2.1 Adipose Tissue . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5 1.2.2 Science in Detail . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9 1.2.3 Hormone Activity of the Adipose Tissue . . . . . . . . . . . . . . . . 12 1.2.4 Lipohypertrophy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17 1.2.5 Theory of Microvascular Disruption and Lymphatic Interaction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21 1.2.6 Uncontrolled Fat Tissue Proliferation . . . . . . . . . . . . . . . . . . 21 1.3 The Edema . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 23 1.4 Pain . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30 1.5 A Chronic-Progressive Course? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31 1.6 Obesity and Lipedema . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32 1.7 Complaints and Effects of Lipedema . . . . . . . . . . . . . . . . . . . . . . . . . 44 1.8 Appearance, Stages, Classifications, and Course . . . . . . . . . . . . . . . . 48 1.9 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 56 2 The Lymphedema . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 69Corrado Campisi, Lucian Jiga, and Zaher Jandali 2.1 Anatomy and Functioning of the Lymphatic System . . . . . . . . . . . . 69 2.2 Causes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 73 2.3 Clinical Appearance . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 75 2.4 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 76 2.5 Conservative Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 78 2.6 Surgical Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 81 2.6.1 Restorative/Reconstructive Surgery . . . . . . . . . . . . . . . . . . . . 82 2.6.2 Tissue Removal Measures . . . . . . . . . . . . . . . . . . . . . . . . . . . 85 2.7 Similarities and Differences of Lipedema and Lymphedema . . . . . . 88AL GRAWANYxiv 3 Treatment of Lipedema . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 95Zaher Jandali, Benedikt Merwart, Ralf Weise, Angel Pecorelli Capozzi, and Lucian Jiga 3.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 95 3.2 Measures for Weight Stabilization . . . . . . . . . . . . . . . . . . . . . . . . . . . 98 3.2.1 Conservative Measures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 98 3.2.2 Surgical Measures for Weight Stabilization . . . . . . . . . . . . . . 101 3.3 Complex Physical Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 107 3.4 Complex Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 111 3.5 The Individual Therapy Plan . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 114 3.6 The Liposuction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 115 3.6.1 General Information . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 115 3.6.2 Techniques and Shapes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 117 3.6.3 Liposuction Volume . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 123 3.7 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 130 3.7.1 Requirements and Preparations . . . . . . . . . . . . . . . . . . . . . . . 130 3.7.2 Liposuction Procedure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 136 3.7.3 Aftercare . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 140 3.7.4 Success and Long-Term Prospects . . . . . . . . . . . . . . . . . . . . . 146 3.7.5 Consequences and Risks . . . . . . . . . . . . . . . . . . . . . . . . . . . . 150 3.7.6 Course of the Complex-Operative Therapy Plan . . . . . . . . . . 160 3.8 Treatment Example . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166 3.9 Cost Absorption . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 167 3.10 Autologous Fat Grafting . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 171 4 Body Contouring Surgery After Extensive Liposuction and Weight Loss . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 177Zaher Jandali, Benedikt Merwart, and Lucian Jiga 4.1 Medical Indication for Tightening of Excess Skin and Soft Tissues . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 177 4.2 Noninvasive and Minimally Invasive Tightening Methods . . . . . . . . 178 4.3 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 180 4.4 Thigh Lift . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 181 4.5 Upper Arm Lift . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 186 4.6 Buttock Lift . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 190 4.7 Tightening of the Lower Torso Wall . . . . . . . . . . . . . . . . . . . . . . . . . 191 5 Additional Information about Treatment . . . .between the lower leg and foot with different degrees of lipedema1 The LipedemaAL GRAWANY62To examine the painfulness, we can use the so-called pinch test. It should be said in advance that the test is widely used but not very specific. It merely tests the extent to which there is increased sensitivity to pain in the affected areas. For this purpose, a healthy and supposedly diseased area is pinched at the same time. The pinching is performed with moderate force. In the case of lipedema, those affected usually feel more pain in the diseased area than in the healthy area. c The pinch test is not very specific.Fig. 1.37 Edema test by attempting to lift off a skin fold (Stemmer positive)Fig. 1.36 Edema test by indentationZ. Jandali et al.63When evaluating this examination method, it must be noted that this is a purely subjective test procedure. Here again, we encounter the problem that the subjective perception of pain offers no possibility of objectifiability. Despite the limited evalu-ability, the test can serve as another piece to complete the puzzle.Imaging DiagnosticsAs we have already stated, there are no apparative diagnostics that either prove or exclude lipedema with certainty. Since other conditions besides lipedema and lymphedema can be associated with swelling of the legs or arms, we may send you to other specialists to have certain tests performed. It is important to rule out other conditions in advance or, if necessary, to treat them. One example is chronic venous insufficiency, a vein disease that can cause edema of the legs even in its early stages. So, in the case of swollen legs, an evaluation by a phlebologist, angiologist or vas-cular surgeon is often recommended as well. Often, an ultrasound examination is then performed to examine the vessels. c To rule out other causes for the symptoms, a presentation to a phlebologist, angiologist, or vascular surgeon should be made.An ultrasound examination can also be used to measure skin thickness. Many colleagues speak of a “thicker” skin in lipedema, which can be detected by ultra-sound. In a 2018 study by Hirsch etal. (Halle, Germany), there were no differ-ences regarding skin thickness in lipedema patients compared to healthy or obese individuals. However, a thickening of the skin is often seen in lymphedema patients, so that a skin layer thickness measurement can be useful in these patients.Others speak of larger fat cells on ultrasound. However, there is no evidence for this, especially when comparing lipedema areas with those of purely obese people.Even though we use a high-resolution ultrasound machine on a daily basis to plan other surgical procedures, we do not see any additional benefits of using an ultrasound machine in lipedema diagnosis.To further differentiate between lipedema and lymphedema, we sometimes perform an infrared camera imaging of the lymphatic vessels, which in border-line cases, provides us with decisive information about the cause of the swell-ing. For this purpose, we inject a dye in the area of the interdigital or interfinger furrow. After a waiting period of a few minutes, we use a special infrared cam-era to view superficial lymphatic vessels and how they transport the dye away. While the examination works very well in the early stages of lipedema, we are limited in advanced stages. This is because this method can only visualize very superficial lymphatic vessels. Since we do not usually see concomitant lymph-edema in lipedema, we do not regularly use this examination method for this purpose.Continuing imaging studies such as lymphoscintigraphy and lymphangio-MRI also do not provide any benefit in the diagnosis of lipedema.1 The LipedemaAL GRAWANY64DiagnosisLet us now turn to how we consolidate and finalize our diagnosis. We have gathered a lot of information in the conversation and during the examination and now want to substantiate this with objective data. As we already know, this is not always easy.Especially in the case of a fat distribution disorder or lipedema, in which subjec-tive factors largely determine the symptoms, we must try to find objective factors and criteria that help us make a diagnosis. Also, with the knowledge that these fac-tors are not an expression of the actual pain intensity, movement restriction, and restriction of the quality of life and that there is the atypical lipedema.In terms of body weight, we roughly distinguish five groups (with many transi-tional stages), so-called body shapes (Fig.1.38).The five body shapes for the affected people with lipohypertrophy or lipedema are – Underweight. – normal body weight, – slightly increased body weight, – strongly increased body weight and, – extremely overweight.Affected persons who are underweight are basically not seen at all. Each of these groups must be treated differently—not fundamentally differently but in a gradu-ated manner (see Sect. 3.4, The individual therapy plan).We already talked about the BMI (body mass index) for the classification of obesity in Sect. 1.3. Unfortunately, the BMI only takes into account pure weight as well as the pure height and does not do justice to different body compositions. For example, athletes with a lot of muscle mass and little fat can have an increased BMI value, just like obese people.If we look at the number of lipedema sufferers in the group of the severely and extremely obese, we can roughly say: the higher the body weight, the more sufferers of lipohypertrophy and lipedema are found in these groups. This supports the thesis that in the case of a multifactorial genesis of lipedema (i.e., it is triggered by various causes) obesity is one of the causes.A large group of lipedema sufferers are not overweight per se, although they are led to believe so by an increased BMI.This happens when the fat distribution disor-der is very pronounced. Therefore, it is enormously important in these sufferers that we do not use BMI alone or even BMI at all to assess lipedema.Bodyshape 1 Bodyshape 2 Bodyshape 3 Bodyshape 4 Bodyshape 5Fig. 1.38 Five different body shapesZ. Jandali et al.65BMI is a parameter/index for evaluating body weight in relation to height, noth-ing more. c BMI, body mass index or also called body measurement index, is an index for evaluating body weight in relation to body size. BMI �� ��� ��m kgl m2 2 The BMI is given in the unit of measurement kg/m2. The body mass m is divided by the body height l2, where m, the body mass, is given in kilograms and l, the body height, is given in meters.However, the BMI helps to get a rough orientation: rather normal weight, rather slight overweight, rather strong overweight. This can certainly be derived from the BMI. If you have a present, clearly visible fat distribution disorder with pain in these regions, a tendency to bruise and a BMI of up to 34kg/m2, the presence of lipedema is more likely than not. However, if you suffer from a significant fat dis-tribution disorder, you should not use the BMI value as a reference. This will then not apply to you. There are other values that can be consulted instead of BMI.These include: 1. waist–hip ratio (WHR), 2. waist-to-height ratio (WHtR). 3. circumference and volume measurements of the extremities.Thereby, to get a quick impression, the Waist–Hip Ratio and Waist-to-Height- Ratio are interesting.Waist–Hip RatioThe Waist–Hip Ratio (WHR) relates the circumference of the waist to the circum-ference of the hips. The number, that is, the ratio, is given as a simple number with-out a dimension or unit.To obtain the waist–hip ratio, the smallest waist circumference (centrally between the lowest rib and the upper edge of the pelvis) and the hip circumference in the area of the largest circumference are measured in a standing position with the arms rest-ing against the body (Fig.1.39). WHRwaist circumferencehipcircumference�� � �� � �u ml m For example, the waist-to-hip ratio for a waist circumference of 80cm and a hip circumference of 100cm=0.8. WHRwaist cmhip cm�� �� ��T 801000 8, 1 The LipedemaAL GRAWANY66Normal values are considered to be a WHR 0.85in women and>1.0in men. The situation is different when there is a disproportion, as in lipedema, in favor of the hips. The more pronounced the disproportion, the smaller the WHR. WHR �� �� ��Twaist cmhip cm801300 69, c The greater the disproportion between the waist and hips, the smaller the waist-to-hip ratio.Thus, the WHR can be used to assess forms of lipohypertrophy or lipedema that are particularly localized around the hips and buttocks. Thus, if one has an increased BMI but a small WHR, this should be taken as a sign of disproportion.Fig. 1.39 Waist–hip ratioZ. Jandali et al.67Waist-to-Height Ratio (WHtR)The waist-to-height ratio is another tool for assessing the severity of lipedema or lipohypertrophy. This measure is particularly suitable for assessing body fat distri-bution in affected individuals who have a marked fat distribution disorder without underlying obesity (Table1.12). It is also suitable for assessing health risks. WHtRu ml m�� � �� � �waistheight WHtR �� �� ��waist cmheight cm801700 47, Example in lipedema patients: WHtR �� �� ��waist cmheight cm901700 53, c If there is a disproportion as in lipedema in favor of the hips with a normal waist, a WHtR should be in the normal range. Even if the BMI should be high and the WHtR is in the normal range, this speaks for a fat distribution disorder and not for obesity.Circumference and Volume Measurement of the ExtremitiesA circumference and volume measurement can also be useful, especially to record a progression. When measuring the circumference, we recommend measuring the hips, waist, torso alongside the inframammary line, at the very top of the upper arm, in the middle of the upper arm, at the level of the crook of the elbow and in the area of the middle forearm, at wrist level and the circumference of the hand when the hand is stretched out flat. On the leg, analogously, in the area of the thigh close to the body, at mid-height, at knee height, in the area of the lower leg close to the body, at ankle height, and in the area of the middle foot. There are also standard tables with marked locations where the measurements should be taken. We have our own proto-col for these values, which has become established over the years. In Fig.1.40 you can see the measurement regions. Of course, both arms and legs must be measured.There are also volume measurements, for example, with the aid of modern 3D cameras, via water displacement, and many more, which would go beyond the scope of this article.Table 1.12 Reference values of the WHtRAge Critical range0,540–50 0,5–0,6>50 >0,61 The LipedemaAL GRAWANY68From all the data collected above, the interview, the examination, the palpation findings, the data on BMI, WHR, and WHtR, we finally derive the diagnosis.If you were to ask us what we would like to see in the future, it would be simpler and better diagnostics. But what could better diagnostics look like? If it were pos-sible, for example, to send a sample of subcutaneous fat tissue or even a blood sample to a laboratory and detect lipedema via a test, that would be perfect. What would be the consequences if such objective diagnostics were possible? The health insurance companies would certainly reevaluate the disease and cover the costs of treatment due to the reliable diagnosability.What needs to be done until then? We have to work with the tools at our disposal, support science, and try to establish standards.Fig. 1.40 Circumference measurement on arms and legsZ. Jandali et al.69© Springer Nature Switzerland AG 2022Z. Jandali et al. (eds.), Lipedema, https://doi.org/10.1007/978-3-030-86717-1_2C. Campisi (*) Adjunct Professor University of Catania. Plastic, Reconstructive and Aesthetic Surgery, Lymphatic Surgery and Microsurgery, Private Consultant Genoa, Milan, Rapallo, Reggio Emilia, Genova, Italye-mail: corrado.campisi@campisiandpartners.com L. Jiga · Z. Jandali Department of Plastic, Aesthetic, Reconstructive and Hand Surgery, Evangelical Hospital Oldenburg, Oldenburg, Niedersachsen, Germanye-mail: dr@jandali.de2The LymphedemaCorradoCampisi, LucianJiga, andZaherJandaliIn this chapter, we will look at both the similarities, but mainly the differences between lipedema and lymphedema.The differences result from the different causes of both diseases. These differ-ences are not always clear to the layperson and are often difficult to understand due to the medically complex interrelationships. It should become clear that, despite some similarities, these are two completely different clinical pictures, which differ significantly from each other in their development, symptoms, diagnosis, and therapy.However, notwithstanding these differences, the problem is up to now under dis-cussion, and related research is in progress, considering moreover the potentially compromising lymphatic drainage in dysfunctional adipose tissue and the possible, even if relatively rare, evolution of the initial pure lipedema to the lipo-lymphedema state, depending, of course, on lipedema worsening staging.2.1 Anatomy andFunctioning oftheLymphatic SystemOur body has two vital transport systems. In addition to our blood circulation, which transports oxygen from the lungs to the tissues and carbon dioxide back, supplies our cells with nutrients and acts as a transport route for hormones, components of blood clotting, and defense, there is a second, almost parallel transport route in our body. This is the so-called lymphatic system (syn. Lymphatic system). While the AL GRAWANYhttp://crossmark.crossref.org/dialog/?doi=10.1007/978-3-030-86717-1_2&domain=pdfhttps://doi.org/10.1007/978-3-030-86717-1_2#DOImailto:corrado.campisi@campisiandpartners.commailto:dr@jandali.de70function and structure of the blood circulation are usually well known, the lym-phatic system is far less familiar to most people. In the following, we want to change that and give you an understanding of the basic features of the lymphatic system.As already mentioned, the lymphatic system is also a transport system of our body. On the one hand, it serves to remove fluid from the tissues and thus keep the fluid balance in equilibrium, and on the other hand, it serves as a means of transport for so-called lymphatic substances. These include proteins, fats, bacteria, viruses, and foreign bodies that cannot be absorbed by the capillaries of the blood vessels due to their size. Our lymphatic system is therefore often disparagingly referred to as the “body’s garbage disposal system.” In addition, the lymphatic system has an indispensable task in immune defense.To fulfill all these tasks, this unique and highly specialized organ system of our body has a very special structure (Fig.2.1). On the one hand, it consists of the lym-phatic vascular system, on the other hand of the lymphatic organs. The latter, in turn, can be divided into primary and secondary lymphatic organs.The primary lymphoid organs are responsible for the formation and maturation of progenitor cells into mature immune cells. These include the thymus and bone marrow. The secondary lymphoid organs are where the contact between the mature immune cells and antigens takes place. In addition to the lymph nodes, they include special tissues in the gastrointestinal tract (mucosa-associated lymphoid tissue), the pharynx (pharyngeal, palatine, and lingual tonsils), and the spleen.Primary Lymphatic OrgansThe primary lymphatic organs include the bone marrow, which is locatedinside all bones, and the thymus, a small gland located in the upper mediastinum. This is where the formation and maturation of special defense cells called lymphocytes or “white blood cells” takes place. The formation of all lymphocytes begins in the bone marrow. Depending on whether their maturation into functional defense cells also takes place in the bone marrow or the thymus gland, a distinction is made between B (“bone marrow”) and T (“thymus”) lymphocytes. B and T lymphocytes fulfill various tasks of the body’s defense system. These include the production of antibodies or the recognition and destruction of viruses or degenerated cells (e.g., cancer cells).Secondary Lymphatic OrgansSecondary lymphoid organs include the spleen, lymph nodes (Fig.2.2), and mucosa- associated lymphoid tissue. In these tissues, the so-called antigen presentation and recognition take place. In a sense, the lymphocytes are taught here against which antigens they have to act and how.The lymphatic vascular system begins with the initial lymphatic vessels, often referred to as lymphatic capillaries or lymphatic collectors. They are the smallest sections and begin as a network between the capillaries of the blood circulation in the intercellular space of organs or the skin. The diameter of such a lymphatic capil-lary is about 50μm, which is about 10 times larger than that of a blood capillary. Their task is to absorb tissue fluid and dissolved substances. From here, the lymph C. Campisi et al.71is transported to the larger lymphatic vessels. They are formed by the union of sev-eral lymphatic capillaries into so-called precollectors. Several lymph nodes are interposed in these and basically serve as a filtering station. A special feature of the lymphatic vessels is the many interposed valves that facilitate the transport of the lymph and prevent backflow (Fig.2.3).Several lymphatic vessels then unite to form the lymphatic trunks, which are usually arranged in pairs. In these lymph trunks, the lymph fluid is collected in each case from a specific region of the body. From here, it is drained into the left and right vein angles via the lymphatic ducts, which form the last section of the lymphatic pathway, and is thus fed into the bloodstream. With the exception of the lymphatic vessels that drain lymph from the right arm and right head and neck region, all lym-phatic vessels converge into the main lymphatic trunk (thoracic duct). This eventu-ally drains into the left subclavian vein. Lymph vessels from the right arm and the Fig. 2.1 Structure of the lymphatic system2 The LymphedemaAL GRAWANY72Fig. 2.2 Cross section of a lymph nodeFig. 2.3 Cross section of a lymphatic valveC. Campisi et al.73right head and neck region, on the other hand, drain into the right lymphatic duct (Ductus lymphaticus dexter), which in turn ends in the right subclavian vein.Due to this special architecture, the lymphatic system essentially fulfills three different tasks.Firstly, it acts as a transport system. Every day, approx. 2–3L of the ultrafiltrated interstitial fluid is reintroduced into the bloodstream. The transport takes place on the one hand passively by contraction of the surrounding skeletal muscles, and on the other hand actively by a peristaltic movement of the muscles built into parts of the lymphatic vessels. The interposed valves prevent the lymph from flowing back (Fig.2.3).Secondly, the lymphatic vascular system fulfills an important function in the body’s defense system. Through the intermediate lymph nodes, pathogens that can easily penetrate the lymphatic vascular system due to the high permeability of the lymphatic capillaries can be freed. Furthermore, the lymphatic vascular system serves as a transport medium for lymphocytes.Thirdly, it is the task of the lymphatic vascular system to absorb or transport lipids (fats). Glycerol and fatty acids are absorbed via special lymphatic vessels of the gastrointestinal tract, the so-called chyle vessels, which also have a very high permeability. This allows lipids to be supplied directly to adipose tissue and muscle without first passing through the liver. Due to the high fat content after passage through the digestive tract, the lymph changes its appearance here from clear to milky turbid. In addition to the high fat content, the lymph contains numerous plasma proteins, coagulation factors, and fibrinogen, as well as cellular components (mainly lymphocytes).Interestingly, the central nervous system is left out of the lymphatic system to protect the brain. This extends from the outside only as far as the meninges. However, there is an indirect connection to the lymphatic system via the brain’s own disposal system.2.2 CausesLymphedema is a complex clinical condition. Approximately 140–250 million peo-ple worldwide suffer from it. It is caused by a lack of transport capacity of the lym-phatic system, which means that the interstitial fluid (tissue fluid between the cells) can no longer be adequately removed, resulting in a backlog of lymph in the inter-cellular spaces. This appears as a visible and palpable accumulation of fluid, which is accompanied by fibrosis of the tissue (the connective tissue loses its functional properties) and excessive storage of fatty tissue, especially in advanced stages. The lower and upper extremities are most frequently affected. Based on the cause, pri-mary (congenital) is distinguished from secondary (acquired) lymphedema.Primary Lymphedema/Congenital LymphedemaPrimary lymphedema, which describes the rarer form of lymphedema, is a congeni-tal disorder of the lymphatic vascular system or the lymph nodes, which are either not formed at all or are formed incorrectly.2 The LymphedemaAL GRAWANY74 c In primary lymphedema, the lymphatic vascular system is congenitally disturbed.Most often, there is hypoplasia (lack of formation) of the lymphatic vascular system or a reduced number of lymphatic vessels in a particular region of the body. The lymphatic drainage of the lower extremity is most frequently affected. Less commonly, valvular disorders may also occur. Primary lymphedema occurs spo-radically in about 97% of cases and is therefore not inherited. The primary form usually manifests itself with the onset of puberty and can occur unilaterally or bilat-erally. Women are affected about twice as often as men.Secondary Lymphedema/Acquired LymphedemaThis is an acquired damage of the lymphatic system, as a result of which there is a disturbed outflow of the lymph. c Secondary lymphedema is caused by acquired damage to the lymphatic system.Common causes are accidents, tumor diseases or inflammation of the lymphatic vessels, chronic venous insufficiency (chronic venous congestion), and diabetes mel-litus. The most common cause worldwide is lymphatic filariasis, an infectious disease caused by the so-called nematode (Wuchereria Bancrofti). In our latitudes, however, this disease plays a minor role. Mostly it is caused by treatments such as surgery, radia-tion, or the removal of lymph nodes in the course of tumor surgery. The most common example of this is probably secondary lymphedema of the upper extremity (arms) after lymphonodectomy (removal of lymph nodes) from the axilla in breast cancer (Fig.2.4).The risk of developing lymphedema of the arm ranges from 9 to 41% in cases of radical lymph node removal and 4–10% in cases where only the sentinel lymph node was removed. Somewhat insidious is the usually delayed onset of lymphedema after surgery; several months to years often elapse. In breast cancer-associated lymphedema, the first symptoms appear on average 8months after axillary dissec-tion (lymph node removal from the axilla), 75% during the first 3years. Once onset, the subsequent course of secondary lymphedema is usually highly variable. Some patients may experience only mild, painless, and nonprogressiveswelling that does not require therapy, while others may experience rapid progression associated with a severe reduction in quality of life. Unfortunately, if left untreated, the progressive course is the rule rather than the exception. c The incidence for the occurrence of lymphedema of the arm ranges from 9 to 41% in cases of radical lymph node removal and 4–10% in cases where only the sentinel lymph node was removed.The same applies to the lower extremity as to the upper extremity. Lymphedema can also occur in the head and neck or genital area after lymph node removal. Thus, lymphedema is not limited to the extremities. In addition, there are many reports C. Campisi et al.75from EBM literature confirming that also secondary lymphedemas related to lymph node removal, often recognize congenital dysfunctional and/or dysplastic latent impairments of the underlined loco-regional lymphatic system.Malignant lymphedema is a subgroup of secondary lymphedema. In this case, either progressive tumor grows itself or metastasis leads to obstruction of the lym-phatic vessels.2.3 Clinical AppearanceDepending on its expression and classification, lymphedema can be divided, accord-ing to the updated ISL Consensus Document, into three different stages (modified by Campisi Staging, 2009), each of which is accompanied by a more or less charac-teristic appearance.Fig. 2.4 Lymphedema in the area of the left arm after breast cancer2 The LymphedemaAL GRAWANY76In the latency stage (stage 0), no symptoms appear yet. It is characterized by reversible, subthreshold edema.In stage I, a visible and palpable doughy-soft swelling can already be detected. However, this can usually be reversed spontaneously by elevation. Smaller fibro-sclerotic tissue changes (changes from functional tissue to connective tissue with a resulting loss of function) may occur in isolated cases.Stage II is already characterized by marked fibrosclerotic changes and the prolif-eration of fatty tissue. The palpation changes from formerly doughy-soft to rather hard. At this stage, elevation no longer leads to spontaneous regression. Whereas in stage I, it is still easy to “press in” a dent in the edema area, this is hardly possible in stage II.Stage III shows the maximum expression of lymphedema. Extensive fibroscle-rotic changes and often massive fatty tissue proliferation are evident, which severely restrict natural movement. As edema progresses, the skin tends to develop eczema, erysipelas, or vesicles. Symptomatically, pain, feelings of tension, and a character-istic feeling of heaviness of the affected body part usually occur in the early stages (Fig.2.5).2.4 DiagnosisThe medical history is of decisive importance and the first step in the basic diagno-sis of lymphedema. Past operations, past infections, tumor diseases as well as vas-cular diseases or skin changes that may have seemed insignificant up to now, in combination with the corresponding symptoms, already provide initial indications of the presence of lymphedema. A positive family history can also be a further clue.Furthermore, the assessment of the clinical appearance is elementary, because the external examination can provide information at an early stage as to whether the complaints described are lymphedema or not. In this context, attention is paid to the localization of the swelling and any differences in circumference, which can be just as decisive as the assessment of the skin in terms of color, skin changes, tempera-ture, and texture.The third decisive step in the initial lymphedema diagnosis is the palpation find-ings. This is similar to the examination in lipedema. We also check the Stemmer’s sign here. It is considered a reliable feature for detecting the presence of lymph-edema. However, some caution is required here. A negative Stemmer’s sign does not necessarily rule out lymphedema. Palpation also includes the assessment of lymph nodes with their size, consistency, displaceability, and tenderness. Palpation also checks for edema consistency and reliability. c A negative Stemmer’s sign does not rule out lymphedema.To confirm the diagnosis and to discuss the extent and location of the damage to the lymphatic system in more detail, various other diagnostic procedures can help. C. Campisi et al.77Among the imaging procedures, we distinguish morphological from functional diagnostics. Sonography, MRI, and indirect lymphangiography provide information about morphology. Function can be assessed by functional lymph scintigraphy (Campisi et al. 2019; Villa et al. 2019) and fluorescence microlymphography. Indocyanine green lymphangiography (ICG) is also increasingly used.We perform an apparative diagnosis by means of an indocyanine green lymphan-giography. A liquid, fluorescent dye is injected into the patient’s skin. This is absorbed by the lymphatic vessels and transported away (Fig.2.6). The figure shows the cloudy dye injection site in the area between the toes and the good removal of the lymph via the well-illustrated, linear superficial–subdermal lymphatic vessels. The fluorescent dye can be seen and assessed via an infrared camera connected to a monitor (Figs.2.7 and 2.8) (Campisi et al. 2018; 2020).Fig. 2.5 Stage III lymphedema in the left leg after pelvic lymph node removal2 The LymphedemaAL GRAWANY782.5 Conservative TherapyLymphedema is a chronic and usually progressive disease that requires long-term treatment (Fig.2.9). Therapy includes both conservative and surgical measures and aims to prevent the progression of the disease and alleviate existing symptoms.Before recommending any therapy, we conduct a medical history survey. Likewise, each patient is thoroughly examined and any additional diagnostics are also performed. Together with the results of the medical history, diagnostics, and examination, we are able to offer patients a treatment plan that is individually tai-lored to their condition.The basis of conservative lymphedema therapy is based on a combination ther-apy of manual lymphatic drainage (MLD), compression therapy, movement exer-cises, and skin care developed as early as the 1970s. The complex therapy consisting of these four components is summarized under the term “Complex Physical Decongestive Therapy“ (CPD). CPD is considered the gold standard and the first- choice therapy for lymphedema. c CPD (complex physical decongestive therapy) is considered the gold standard and the therapy of the first choice.Manual Lymphatic DrainageManual lymphatic drainage (MLD)is a special form of physiotherapeutic treat-ment in which the removal of lymph is promoted with targeted hand movements. On the one hand, the accumulation of fluid from the interstitium toward the lymph capillaries is supported in this way, and on the other hand, the self-trans-port within the lymph vessels is stimulated, which favors the removal of fur-ther lymph.Fig. 2.6 ICGC. Campisi et al.79In addition to manual lymphatic drainage, appliance-based lymphatic drainage can be performed. Special devices can perform lymphatic drainage at home via an adapted peristaltic compression cuff. There are different providers here, although the devices work according to the same principle. These devices are available from the relevant providers via a prescription after requesting cost coverage from the health insurance company.Compression TherapyCompression therapy is also an important component of CPD.The affected part of the body is wrapped with bandages. The pressure applied from the outside supports the drainage of the lymph, and the pressure decreases toward the trunk to ensure a directed lymph flow. For lymphedema, we recommend flat-knit compression gar-ments made to measure, including the hands and feet.Fig. 2.7 Lymphedema in the area of the arm2 The LymphedemaAL GRAWANY80Fig. 2.8 Lymphovenous (1) and multiple lymphovenousanastomosis (2)Fig. 2.9 Clinical images of planning and relocation of lymphatic vesselsC. Campisi et al.81Movement ExercisesTargeted movement exercises are designed to increase lymph drainage. Among other things, the natural activation of the muscle pump thus promotes passive lymph transport. It is important that an individually tailored therapy program be developed for each affected person.A new advanced specific protocol is under assessment, in Genoa, named BioCircuit, with tailored exercises by an exclusive Computer Assisted Technology.Skin CareDaily skin care is an indispensable pillar of CPD for lymphedema patients. Due to the usually compromised natural skin barrier, the skin is significantly more suscep-tible to infections. Especially via furrows and rhagades (smallest tears in the skin) germs can penetrate the body and cause severe infections. Bacteria have an easy time spreading due to the defective lymphatic system and poor metabolism in the region. c For those affected by lymphedema, skin care is essential to prevent serious infections.CPD is divided into two phases. In the first, so-called decongestion phase, the existing edema is to be reduced as much as possible. In this stage, daily treatments are carried out by means of manual lymphatic drainage, compression therapy by means of wrapping, movement exercises, and skin care. The bandage is worn con-tinuously, except during treatment. Depending on the stage, this phase can last sev-eral weeks.Once the edema has been reduced as much as possible, the maintenance phase begins. The main purpose of this second phase is to maintain or improve the results already achieved. At the beginning of this phase, a flat-knitted compression stocking is fitted. Unlike the circular knitted stocking, this stocking is not stretchable in all directions and therefore provides better compression. In the maintenance phase, the therapy concept also consists of manual lymphatic drainage, compression treat-ment, movement exercises, and skin care. However, lymphatic drainage usually, in the earliest stages (IB, IIA), no longer has to be performed daily, but from 3 to 4 times a week to 1–2 times every 14days, depending on the extent. Wrapping, in these cases, is also now only done on the day of therapy. On any other day, the com-pression stocking can be worn. Often, the initial treatment takes place within the framework of a rehabilitation measure in a clinic specialized for this purpose.2.6 Surgical TherapyTo understand the therapy of lymphedema, it must be noted that lymphedema is often a chronically progressive disease. It is associated primarily with the sometimes mas-sive accumulation of lymphatic fluid, fibrosclerotic tissue changes, massive fat tissue proliferation, and ultimately also the destruction of lymphatic pathways.2 The LymphedemaAL GRAWANY82As described above, each patient receives an individual therapy plan. Although often, in advanced stages (IIB, IIIA, and IIIB), a long-term treatment for lymph-edema/elephantiasis, CPD has established itself as the gold standard in therapy. The prerequisites for the success of this cure are lifelong implementation and strong compliance on the part of each patient.If CPD does not achieve the desired success, various surgical options are avail-able in addition to conservative therapy, with the goal of preventing the progression of edema, reducing excessive volume, and improving the aesthetics and function of the affected region.In principle, a distinction is made between two different approaches. On the one hand, there is the restoration (reconstruction) of lymphatic drainage. This can be achieved, for example, by means of lympho-lymphatic bypasses, lympho-venous anastomoses, performed as lymphatico-venular superficial scattered microanasto-moses or as single-site multiple deep and superficial lymphatic-venous anastomo-ses, or with the so-called vascularized lymph node transfer. Yes, you are right: this sounds complicated, but we will explain these complicated terms in a moment. In contrast, there are resecting (= tissue-removing) procedures that aim purely to reduce mass or volume. These include liposuction and excision (simply cutting away) of the diseased tissue.Let us first discuss the reconstructive options for lymphedema treatment. In these surgical measures, we distinguish techniques, – based on surgical treatment of the lymphatic vessels, – transplant the lymph nodes, and, – which aim to resprout lymphatic vessels.2.6.1 Restorative/Reconstructive SurgeryAdvances in the field of surgery have now made it possible to identify microscopic lymphatic vessels and assess their quality during surgery. This opens up the possi-bility of at least partially restoring impaired lymphatic drainage. We would like to explain various techniques to you below.Lympholymphatic BypassThis technique can be used, in selected cases, to bridge individual sections with restricted lymphatic drainage. For this purpose, an endogenous lymphatic vessel (or vein) is removed from a region of the body not affected by lymphatic drainage. This lymphatic vessel is then connected to functional sections of the lymphatic vessel system both far from the body and close to the body of the drainage disturbance. The lower the stage of lymphedema, the more promising this technique is. Individual studies have demonstrated up to 80% reduction in the circumference of the affected limb. A disadvantage is that in isolated cases lymphedema may occur in the region of the removed lymphatic vessel.C. Campisi et al.83Lymphovenous AnastomosesThese methods represent today the most frequent microsurgical techniques applied in clinical practice for lymphedema treatment.A direct connection between the lymphatic system and venous circulation is also established here. The advantage over lymphovenous bypass is that the removal of a donor vessel can be dispensed with. In fact, with this technique, lymphatic vessels are connected to smaller veins in the immediate vicinity. Individual studies describe a subjective improvement in symptoms in up to 95% of patients after such an inter-vention. The great advantage of this scar-saving technique is the comparatively less traumatic procedure, which significantly reduces the perioperative risk. In isolated cases, wound healing disorders or the formation of lymphatic fistulas may occur.The figure (Fig. 2.11) schematically shows a (1) lymphovenous anastomosis (LVA) and (2) multiple lymphovenous anastomosis (MLVA). The difference between LVA and MLVA is that in MLVA, a single lymphatic vessel is not con-nected 1:1 with a vein, but several lymphatic vessels are inverted into a vein and connected. In Campisi’s experience single-site MLVA is performed at the inguinal crural region for lower limb lymphedema, and at third medium-superior of the volar surface of the arm for upper limb lymphedema. In addition, Campisi does not use a cross skin incision along the limb, except to approach the inguinal crural region.Figure 2.9 shows a lymphedema patient in our operating room. Images 2–4 were taken with 50x microscope magnification. Lymphatic vessels were visualized via injection of indocyanine green (1). Subsequently, the lymphatic vessels and veins were visualized. The yellow plastic arrows point to the veins, and the blue–green colored vessels are the lymphatic vessels that absorbed the dye from the tissue (2). For a lymphatic vessel to be sutured, we (Jandali–Jigas’s Technique) cannulate it with a hair-thin thread (3). In picture (4) the completed detour of the lymphatic ves-sels into veins can be seen.Vascularized Lymph Node TransplantationOne of the newer techniques of lymphedema therapy is free vascularized lymph node transfer (Fig.2.10). The principle is the removal of a lymph node package, including its vascularization, from an unaffected part of the patient’s own body and the trans-plantation of this package into the area of lymphedema. In theory, two different mechanisms triggered by this procedure are thought to improve lymphatic drainage. Firstly, the transplanted lymph nodes act as a kind of sponge, so to speak, sucking up the lymph in the region and directing it toward the lymphatic vessel. On the other hand, the lymph node transplantation results in a new lymph vessel sprouting in the area surrounding the lymph node. The newly formed lymphatic vessels then carry the lymph to the lymph node, and from there, further, metabolization occurs via the vein of the lymph node into the venous circulation. Several body regions can be used as donor sites. The groin region, the chin, and neck area or the area above the collarbone and lymph nodes from the abdominal cavity have become established. We most fre-quently remove lymph nodes from the abdomen, as the risk of suffering lymphedema as a result of the removal is significantly reduced in this area.2 The LymphedemaAL GRAWANY84However, research is in progress to establish the potential risk of malignant degeneration in the transfer site, reported in some recent articles, due to the growth factors locally induced by lymph node transfer on the immune altered lymphedema-tous tissue, with lymphangiosarcoma (like Stewart–Treves Syndrome) or carcinoma frightful implant. That is why, on the ethical, deontological, and medicolegal points of view, this terrific event, even if exceptional, would be considered and clearly explained to the patient, acquiring his/her informed consensus.Das LYMPHA-Prinzip (Lymphatic Microsurgery Preventive Healing Approach)An example of lymphedema treatment that we perform very frequently is the com-bined treatment of breast reconstruction after breast removal and lymphedema treat-ment after lymph node removal from the armpit. For breast reconstruction, the transfer of excess skin-fat tissue from the abdomen is performed. In addition, a lymph node package is relocated from the groin to the armpit. Finally, the removed tissue is connected to the local blood supply in the recipient area.Fig. 2.10 Clinical images of planning and relocation of lymphatic vesselsa b cFig. 2.11 The LYMPHA principleC. Campisi et al.85Due to the anatomy of the upper extremity lymphatic drainage, lymphedema often develops on the arm of the affected side after breast removal in combination with lymph node removal. The removal of multiple lymph nodes in combination with the injury to multiple lymph vessels from surgery can be compensated for to some degree, but are also eventually exhausted.Due to the progress in the field of microsurgery in the course of the last years, it is nowadays possible for us to identify these damaged lymphatic vessels and to restore them immediately. On the one hand, this can be done by directly suturing the injured lymph vessels or by connecting the lymph vessel to a vein in the surround-ing area.In practical terms, this means that a trained plastic surgeon joins the operation during the lymph node removal from the armpit and diverts the injured lymph ves-sels directly into veins (i.e., as a precaution). This extends the total operation time by 30min and requires good cooperation between oncological and plastic surgery teams. Studies have shown that this significantly reduces the risk of lymphedema.In Fig.2.11, we show the principle of LYMPHA operation. (A) Before tumor removal. The dark dot above the nipple represents the tumor, green the lymphatic vessels. (B) As (A), but with veins. (C) After tumor and lymph node removal and redirection of lymph vessels into draining veins.2.6.2 Tissue Removal MeasuresLiposuctionWe will discuss liposuction in detail in Chap. 3, Treatment of lipedema. Its primary purpose is to reduce the increase in subcutaneous fatty tissue observed in advanced lymphedema. Although it primarily leads to a reduction in circumference and an associated improvement in function and aesthetics of the affected limb, studies also show a marked improvement in lymphatic drainage after surgery. There appear to be several explanations for this: First, tissue injury during surgery could result in con-nections between lymphatic vessels and veins, allowing lymph to be delivered directly to the bloodstream. On the other hand, injuries to the respective muscle fasciae could promote drainage of the lymph from the superficial to the deep lym-phatic vascular system, which actually makes more sense.In summary, liposuction for the treatment of lymphedema is a comparatively low-risk procedure, only if performed by lymph vessel sparing procedure, on the guide of the fluorescent ICG microlymphography. In this way, the risk of aggravat-ing lymphedema during surgery by injuring remaining, functional lymphatic ves-sels is quite low if the technique is correctly adopted. On the contrary, if liposuction is performed without this kind of lymph vessel procedure, due to the total lymph vessel debulking, the consequent heavy charge is the permanent and mandatorily need to continue wearing compression garments for the rest of the patient’s life. Then, debulking blind liposuction is not a measure that restores lymphatic drainage, and therefore is more likely to be noted as a fallback option. In Campisi’s experi-ence, as a matter of fact, lymph vessel sparing selective liposuction (by this author 2 The LymphedemaAL GRAWANY86preferably named fibro-lipo-lymph-aspiration, to avoid any possible confusion with the blind liposuction) is regularly performed as a sequential complementary thera-peutic procedure, after MLVA microsurgery, for the effective treatment of advanced lymphedema.In any case, plastic surgeons today have to be very careful in applying liposuc-tion, even if for only aesthetic indications, and have to be skilled to respect lymph vessels during this procedure.Overall, the pre- and postoperative care, as well as the surgical procedure, differs from liposuction for lipedema. For example, before surgery, the edema must be minimized as best as possible. Likewise, compression must be worn much more consistently after surgery.To be updated on this topic concerning relationships between lymphatics and lipedema, both on theoretic and on practical points of view, it is mandatory to explain that, although assuming the specific differences between lipedema, lymphedema, and the relatively rare lipo-lymphedema, there are recent review articles in which is underlined that “expanding adipocytes produce some lym-phangiogenic factors, such as VEGFC, which may induce lymphatic hyperplasia. Lastly, in hypoxic environments, hypoxia-inducible factor 1 enhances fibrosis, thus potentially compromising lymphatic drainage in dysfunctional adipose tis-sue. Taking into account these findings, research is still needed to clarify whether a persistent and progressive damage of the microlymphatic vessels because of adipose tissue expansion, rather than a primary lymphatic defect, may be respon-sible for the lipo- lymphedema state” (Buso, Mazzolai etal., Obesity, 2019: 27, 10, 1567–1576).In Campisi’s recent experience there is an exemplary case of a lipedema, initially diagnosed by other specialists as a pure lipedema, in which superficial and deep lymphoscintigraphy, according to the Genoa protocol (with the additional calcula-tion of the transport index), showed a latent functional and clinical impairment of the lymphatic circulation, allowing us to perform the proper tailored treatment by MLVA at the inguinal crural region.To conclude, approaching lipedema surgery, the skilled plastic surgeon must be sure that the lymphatic system is functionally intact and, in any case, it has to be respected during the surgical procedure.Tissue Removal/Debridement (Charles Procedure)Radical removal of the areas affected by lymphedema (skin and subcutaneous tis-sue) is nowadays reserved exclusively for the mostsevere forms of lymphedema. The aim of this technique is to remove the skin with all underlying fatty tissue down to the respective muscle skin and then cover it with a skin graft. However, the improved function of this procedure is offset by the unsightly result and the high surgical risk (Fig.2.12).If it were now a matter of explaining how we make our decision as to when to use which procedure, it would go a little too far. If surgical reconstruction is indicated, C. Campisi et al.87we make our decision based on the individual lymphatic vessel status and the over-all medical history. Currently, according to Jandali‘s procedure, lymph node trans-plants are most frequently performed, followed by lymphovenous “shorts”, obtaining better results with lymph node transplantation than with lymphovenous detour. In Jandali‘s experience, the majority of cases (except for lymphedema fol-lowing axillary lymph node removal after breast cancer), is treated by harvesting lymph node packages from the gastric region. The removal is performed laparo-scopically through only three small incisions. The subsequent transplantation usu-ally takes place quite quickly within 2–4h.According to Campisi’s experience, the majority of cases is treated by MLVA (alone, in the stages I A-B and II A), and followed by sequential fibro-lipo-lymph- aspiration with lymph vessel sparing procedure for stages II B, III A–B.Fig. 2.12 Principle of the Charles operation2 The LymphedemaAL GRAWANY882.7 Similarities andDifferences ofLipedema andLymphedemaIt is not uncommon for lipedema to be referred to as lymphedema or other diseases or to be confused with them. As explained in detail in the previous sections, these are fundamentally different clinical pictures whose causes, diagnostic possibilities, and therapies largely differ greatly from one another.Gender DistributionLet’s first take a look at the gender distribution of the two diseases. This is where we see the most obvious difference in our daily clinical routine. Lipedema manifests itself almost exclusively in women. The cause of this remains unknown, despite increasing research in this area. Hormonal influences are considered to be the deci-sive factor. The frequent onset in phases of hormonal change, such as puberty, as well as the aggravation during pregnancy or even the late onset during menopause, speak in favor of this. If lipedema occurs in men, it is usually associated with other diseases (e.g., cirrhosis of the liver, hypogonadism) or is a side effect of hormonally active therapies (e.g., therapy of prostate carcinoma). Lymphedema, on the other hand, affects both men and women.CauseIn contrast to lipedema, lymphedema has a tangible cause, namely either congenital (primary) or acquired (secondary) damage to the lymphatic transport system. This damage can, of course, vary greatly in its origin and severity. It should be noted that lymphedema (at least primary lymphedema) also occurs much more frequently in women than in men. Both diseases show a familial accumulation. c Lipedema occurs almost exclusively in women, while lymphedema occurs in both men and women.Affected AreasA further difference can be seen in the external appearance of both clinical pictures. Although lipedema and lymphedema may look the same at first glance, since they share the symptom of swollen legs, ultimately a closer look at the affected areas usually reveals clear differences. While the swelling in lipedema always affects both legs or both arms, in lymphedema often only one leg or arm is affected by the increase in circumference. If in rare cases of lymphedema, swelling occurs on both sides, one side is usually more affected than the other. Feet and hands also show a clear difference in both clinical pictures. While the feet and hands are excluded from the swelling in lipedema, the backs of the feet and hands are usually clearly swollen in lymphedema (Fig.2.13). This can be verified by a positive Stemmer’s sign.On the surface, lipedema is often accompanied by obesity, which places an increasing burden on those affected. Lymphedema, on the other hand, usually occurs independently of obesity, at least in the early stages.C. Campisi et al.89PalpationDuring a closer examination in the course of our clinical examination, clear differ-ences in the palpation findings of both diseases can also be observed. Lipedema appears mostly soft, often described as doughy or spongy. When the tissue is pressed in, no dent remains.The tissue is similarly soft in early stages of lymphedema (stages 1 and 2). If we press the soft edema in lymphedema, a visible dent remains. In advanced lymph-edema, where longer term edema deposits lead to increased collagen formation and thus to hardening of the tissue, pressing in is no longer possible.Consequently, dents can only be depressed in “soft” lymphedema (early stages), but not in “soft” lipedema. In the late stages of lymphedema, the tissue is too hard to be depressed.Skin LesionsFurther differences can be seen when looking at the skin of lipedema and lymph-edema. The externally visible skin changes in lipedema are caused by the prolifera-tion of fatty tissue under the skin without any actual structural change in the skin structure. In the early stages, the skin appears finely knotty, while a more coarse- knotty appearance occurs in later stages.In lymphedema, on the other hand, the protein-rich edema in advanced stages leads to structural changes in the skin and subcutis. Initially, there is a thickening of both the subcutis (i.e., the lower skin) and the cutis (skin), which is caused on the one hand by an increase in the subcutaneous fatty tissue and on the other hand by an increase in the connective tissue. Furthermore, trophic changes occur in the upper-most skin layer, the epidermis. The changes range from dry skin to hyperkeratosis, excessive growth of the uppermost skin layer, the already described hardening (pachyderma), often also called elephant skin, to the formation of areal skin tumors (papillomatosis), lymphatic vesicles, and ulcers.Ultimately, the aforementioned skin changes in lymphedema, in combination with the impaired removal of lymphatic fluid, lead to damage to the natural skin barrier, which results in a significantly higher incidence of infections (e.g., erysip-elas or cellulitis) in lymphedema than in lipedema.ComplaintsLet us now take a closer look at the complaints of both clinical pictures described by those affected. Here too, in addition to a few similarities, there are major Fig. 2.13 Swollen back of the hand in lymphedema2 The LymphedemaAL GRAWANY90differences between lipedema and lymphedema. It should be mentioned that the symptoms listed below represent only a cross section of the symptoms described by those affected. The occurrence is sometimes subject to strong individual variations.Having said this, let’s start with the similarities: First of all, what both diseases have in common is that the symptoms that occur significantly restrict the quality of life in everyday life and at work. For example, the increase in circumference can lead to restricted movement, which occurs in advanced stages of both diseases and is not infrequently so severe that the natural gait pattern appears to be significantly impeded.In the case of lipedema, pain clearly dominates the symptoms of most of those affected. It should be clarified here that the currently valid staging does not take into account the painfulness of this disease. As a result, there is no direct correlation between stage and pain. This means that patients with stage I lipedema can subjec-tively experience significantly more pain than, for example, patients with stage III lipedema, but the same is also true the other way around—we have already dis-cussed this topic several times in this context. Pain is described much less frequently inlymphedema and is more likely to occur in advanced stages or the case of com-plications such as erysipelas or cellulitis. The everyday limitations of lymphedema are mostly caused by the sensation of tension and heaviness.Those affected by lipedema often report a strong sensitivity to touch, which is described rather rarely in lymphedema. The tendency to hematoma formation after minor trauma is also more likely to be attributed to lipedema than to lymphedema.Apart from the obvious symptoms, sufferers of both conditions have to contend with severe restrictions in their everyday lives. For example, the usually one-sided swelling of lymphedema causes unexpected problems when buying clothes. Pants or tops usually have to be purchased several sizes too large to accommodate the one- sided swelling. Furthermore, the clothing often constricts the skin, which often causes problems due to the poor quality of the skin. c In lipedema, the pain usually dominates the symptoms. These occur regardless of the respective stage. In lymphedema, these are usually only found in advanced stages.ObjectivityAnother striking difference is the objectifiability of both diseases. The basic diagnos-tic procedure, which is the same for both diseases and consists of anamnesis (ques-tioning), inspection (examination) and palpation (palpation), allows the trained and experienced examiner to draw clear conclusions about the clinical picture. The dis-tinction between lipedema and lymphedema seems to be relatively easy to make. But what about the distinction between lipohypertrophy and lipedema, whose only dif-ference is the painfulness of the affected areas in lipedema? In this case, the examiner is solely dependent on the description of the affected person and can only make the diagnosis on the basis of his experience, examination, and statements of the affected person. Further diagnostics are simply not available when diagnosing lipedema. Unfortunately, the disease cannot be objectified in comparison to lymphedema, which makes it difficult for it to be recognized as a health insurance benefit. In the C. Campisi et al.91case of lymphedema, on the other hand, we have numerous, so-called advanced diag-nostic procedures at our disposal. In addition to morphological imaging procedures (MRI, CT, and ultrasound), these also include functional diagnostics (functional lymphoscintigraphy, indocyanine green lymphography) and various genetic tests. c Lymphedema is objectifiable as a disease, lipedema is not.TreatmentAlthough we will discuss therapy, especially that for lipedema, in detail in subse-quent chapters, we will briefly discuss similarities and differences in both conditions.Despite the commonality of CPE as a conservative therapeutic approach, it must again be made clear that lipedema and lymphedema are completely different clini-cal pictures. This is also the reason for the different therapy.In lipedema, CPE only leads to an improvement of symptoms in some cases; a complete alleviation of symptoms by the combination of compression and lym-phatic drainage is almost impossible. Particularly with regard to manual lymphatic drainage in lipedema, there is no proven effect. The only remaining therapeutic approach is liposuction, which, in our experience, greatly alleviates or completely eliminates the symptoms in almost all patients. It should be noted that even if the symptoms are completely reduced after surgical treatment of lipedema, it cannot be said that the disease has been cured. Also, a recurrence of the disease cannot be excluded by surgical therapy. The disease is therefore not curable.In principle, CPD can achieve a significant improvement in the symptoms of lymphedema. In contrast to lipedema, manual lymphatic drainage represents a cen-tral point of therapy. It is also important to distinguish between congenital and acquired lymphedema. In addition to CPD, we now have numerous microsurgical procedures at our disposal, which can increasingly lead to a complete and, above all, permanent reduction in symptoms and even cure.The differences between lipedema and lymphedema are shown in Fig.2.14.We do not want to leave unmentioned that there are also mixed pictures of these diseases among themselves or with other diseases. Lipo-lymphoedema and phlebo- lymphoedema should be mentioned in particular. We have already discussed obesity.As the name suggests, lipolymphedema is a mixed form of lipedema and lymph-edema. In some patients, lymphedema also develops during the course of lipedema (Fig.2.15). In addition to the typical symptoms of lipedema, there are also symptoms of lymphedema that are not usually found in lipedema. An example of this is the swol-len backs of the feet or hands or edema on the lower legs that can be pushed away. In contrast to pure lymphedema, the edema in lipo-lymphedema is usually symmetrical.In Fig.2.16 marked lipo-lymphoedema after massive weight loss is shown.Phlebo-lymphedema is a combination of venous disease and lymphedema. It is caused by chronic venous insufficiency, a venous outflow obstruction that can be caused, for example, by varicose veins, phlebothrombosis, or arteriovenous malfor-mations. This damage to the veins can result in the blood not returning properly from the periphery of the body back to the heart. The blood backs up, so to speak, and presses fluid out of the veins into the surrounding tissue.2 The LymphedemaAL GRAWANY92As a consequence, increased tissue fluid is produced here as well, which—simi-lar to lipo-lymphoedema—leads to an overload of the lymphatic vascular system. In phlebo-lymphoedema, too, early treatment of the underlying disease is indispens-able. Manual lymphatic drainage and compression therapy are prescribed as sup-portive measures.Fig. 2.14 Differences between lipedema and lymphedemaFig. 2.15 Dorsal edema of the foot in lymphedemaC. Campisi et al.93There is another very impressive observation from our daily surgical routine when we compare lipedema and lymphedema. When we take a patient with lymph-edema to the operating room and make a skin incision, tissue water (edema) escapes from the wound immediately after penetration of the skin with the scalpel. Occasionally, this edema discharge can be observed through the skin suture into the dressing for several days after the operation. The situation is completely different in lipedema. Although a small incision must be made through the skin at the beginning of every liposuction procedure, we have never seen even a drop of tissue water leak out in lipedema in the past 15years.Basic differences and similarities between lipedema and lymphedema are sum-marized here. 1. Lipedema and lymphedema are two entirely different conditions, both of which cause swollen legs.Fig. 2.16 Lipo- lymphedema after massive weight loss2 The LymphedemaAL GRAWANY94 2. Lipedema occurs almost exclusively in women, while lymphedema occurs in both men and women. 3. Lipedema always shows a symmetrical swelling. Lymphedema often shows asymmetric swelling of an arm or leg. 4. In lymphedema, the backs of the hands and/or feet are also affected by swelling, but not lipedema. 5. Stemmer’s sign is negative in lipedema but positive in lymphedema. 6. The swelling in lipedema feels soft, in advanced lymphedema, it is hard and bulging. 7. In lipedema, the pain usually dominates the symptoms. It occurs regardless of the respective stage. In lymphedema, pain is usually observed only in advanced stages. 8. Common complications of advanced lymphedema are erysipelas (erysipelas) and cellulitis. These do not usually occur in lipedema. 9. A tendency to hematoma formation is observed in lipedema, even after minor trauma, but not in lymphedema. 10. Lymphedema is caused by a disorder of the lymphatic transport system. The cause of the development of lipedema is not clear to date. 11. Obesity can leadto the development of lymphedema. In lipedema, it can have a negative effect on progression and also be associated with it. 12. Lymphedema can usually be diagnosed causally with diagnostic imaging pro-cedures. There is no apparative examination that is conclusive for lipedema. 13. Both diseases may or may not progress. Whether in what time frame or to what extent the diseases progress cannot be predicted for both lipedema and lymphedema. 14. Manual lymphatic drainage is a core element of lymphedema therapy. In lipedema, it usually has no lasting effect. 15. In both diseases, it is important to treat not only the physical effects but also the psychological stress.ReferencesCampisi CC, Villa G et al (2019) Rationale for the study of the deep subfascial lymphatic ves-sels during lymphoscintigraphy for the diagnosis of peripheral lymphedema. Clin Nucl Med 44:91–98Campisi C, Ryan M, Campisi CS, Boccardo F, Campisi CC (2018) Lymphatic Venous Anastomosis Applied in the Surgical Management of Peripheral Lymphedema: from Prophylaxis to Advanced Disease. In H-C Chen, P Ciudad, S-H Chen, YB Tang. Lymphedema Surgical Approaches and Specific Topics (Chapter 6, pp. 55–70). Elsevier. 2nd editionCampisi C, Marlys W et al (2020) Matching Primary with Secondary Lymphedemas across Lymphatic Surgery in Genoa Italy from 1973 until time of Covid 19. Italian Journal of Vascular and Endovascular Surgery, Minerva Medica 2021(March);28(1):25–41.Villa G, Campisi CC, Campisi C et al (2019) Procedural recommendations for lymphoscintigra-phy in the diagnosis of peripheral lymphedema: the Genoa Protocol. Nuclear Medicine and Molecular Imaging 53:47–56C. Campisi et al.95© Springer Nature Switzerland AG 2022Z. Jandali et al. (eds.), Lipedema, https://doi.org/10.1007/978-3-030-86717-1_3Z. Jandali (*) · B. Merwart · L. Jiga Department of Plastic, Aesthetic, Reconstructive and Hand Surgery, Evangelical Hospital Oldenburg, Oldenburg, Niedersachsen, Germanye-mail: dr@jandali.de R. Weise Klinik für Allgemein- und Visceralchirurgie, St. Marienhospital Friesoythe gemeinnützige GmbH, Friesoythe, Germany A. P. Capozzi PLATINUM MEDICAL CENTER, SLP, Carrer Sant Elíes, entresuelo 115, Barcelona, Spain3Treatment ofLipedemaZaherJandali, BenediktMerwart, RalfWeise, AngelPecorelliCapozzi, andLucianJiga3.1 IntroductionIn our opinion, lipedema has serious consequences for those affected. In addition to the pain, which is often responsible for a high level of suffering, mobility and over-all resilience are often significantly limited. The heavy weight-bearing associated with lipedema can lead to painful wear and tear in the hip and knee joints at an early stage. Those affected withdraw, feel misunderstood, marginalized, or even discrimi-nated against. These social and psychosocial components have a major impact. We have an obligation not only to treat the pain but also to reintegrate those affected into social life. We must help to make possible a life free of psychological pressure.It is important that all doctors take the complaints of those affected very seri-ously and do not simply continue the “fat leg principle” as before. It is not enough to tell sufferers that weight loss is the right thing to do and that they should just eat less. If it were that simple, sufferers would have already done it. Sufferers need to feel taken seriously and well cared for.We see a colorful landscape of different players with different training, orienta-tion, and interest in the treatment of lipedema. We see that the market is highly competitive, with oral and maxillofacial surgeons, orthopedic surgeons, gynecolo-gists, vascular surgeons, and dermatologists presenting themselves as experts in lipedema. This is not at all objectionable, as everyone can contribute something to AL GRAWANYhttp://crossmark.crossref.org/dialog/?doi=10.1007/978-3-030-86717-1_3&domain=pdfhttps://doi.org/10.1007/978-3-030-86717-1_3#DOImailto:dr@jandali.de96the cure and research from their area of expertise. However, what makes one won-der greatly is that few are concerned with conservative treatment and instead turn to surgical liposuction.Quite aggressive advertising is used for this. To outdo each other, there is a real power struggle with tempting offers containing false statements. It can almost be described as “normal” when doctors hire professional bloggers to specifically advertise for them. When we read on the tempting web portals: “Lipedema is cur-able,” “We cure lipedema,” or “We suck off all the fatty tissue, with us no fat remains,” we wonder what is going wrong. On the point, some drive it then, which want to generate a patient influx with the stoking of fears: “If you do not let your-selves be sucked off, then it becomes always worse.“ This statement is then under-lined by the publication of rare lipedema pictures, which are to be seen as the final stage and the evil to be expected. We are particularly uncomfortable with bloggers and hired laymen working under the guise of nonprofit.We will tell you how to find a good, reputable, and practically trained doctor in Chap. 5. Even we, the authors of the book, are not the measure of all things, but we will try to give you an honest picture of the nevertheless opaque situation and the pronounced conflict of interests of the individual players. Don’t fall for it. “We will cure your lipedema,” sounds good, but it is not true.We even go a small step further. In Germany, for example, even politicians have put on the shoe of wanting to take care of lipedema sufferers—an approach that is very much to be welcomed. However, politics has placed itself in a nest of nettles. Instead of promoting and supporting research, liposuction in certain stages of lipedema is now covered by health insurance in Germany. From our point of view, this is a gross and negligent injustice for all those who do not fall into the stages. In addition, the stage-dependent surgical indication, based on a purely descriptive clas-sification of stages, can hardly be surpassed in nonsense and cannot be verified by any objective means. Here, too, politicians, driven by bloggers and clueless laymen, only want to put themselves in the center of events and do not want to take care of the affected persons in a sustainable way.We stand for evidence in medicine, and in lipedema, there is simply almost none. This also applies to all our statements, which are based on experience and very few good studies. Those who work at the grassroots level are the real heroes in our view. These include the doctors in research who are working on this issue with few finan-cial resources, the self-help groups with their tireless efforts to support those affected, and the many honest doctors who do not have a microphone in front of their mouths all day to communicate. The truth of it all is, as always, somewhere in between.When we think about what good treatment of patients with lipedema looks like, we have to take a holistic view of the patient and the options available to us. In addi-tion to the therapy of the pain, which should be in the foreground, any existing secondary diseases must also be treated. This may include obesity or mental ill-nesses such as depression.Let’s talk about the treatment options. The basic prerequisite for us as treatment providers is that the desire for treatment comes from you personally. Only then will Z. Jandali et al.97you also have the necessary motivation and discipline to experience long-term success.Just a few months ago, a mother and her daughter came to see me. Right from the start, the mother had the floor and began by saying that she had seen a report on television and had known right away that she herself and also her daughter would suffer from lipedema. She had always wondered why she looked the way she did and wanted to spare her young daughter these thoughts. When asked how her daugh-tersaw it, she said that she had noticed for a long time that her proportions were not quite right either, but that it had never bothered her. Neither she nor her mother had any pain. There is no lipedema, but a simple lipohypertrophy. The young daughter does not wish to undergo treatment. It, therefore, makes no sense to treat the daugh-ter. It would make more sense to possibly treat the mother, but then for aesthetic reasons and not on the basis of lipedema.The path of any treatment is complex, whether conservative or surgical. In most cases, existing pain can be treated well by whatever method. Certainly, there are also rare therapy-refractory courses (courses that do not respond to therapy), but ulti-mately our experience shows that almost all sufferers can be relieved of their pain. Perhaps not always with the measures that the affected person would like, but there are possibilities. The outside can also always be positively influenced. It just depends on what goals the sufferers have, how realistic they are, and what they are willing to put in to achieve the goals. A statement like “I was told that no one can help me” is certainly only understandable in the case of severe secondary illnesses or other spe-cial reasons. Other questions include medical necessity and who will pay the costs.Let’s take another look at the group of lipedema sufferers (Fig.3.1). Since more than 50% of lipedema sufferers also suffer from morbid obesity, long-term treat-ment of obesity is often a crucial component in the treatment of lipedema. The pri-mary goal is to prevent further weight gain or, in the best case, to bring about weight reduction, depending on the initial weight.Different treatment aspects can be considered for the treatment of lipedema. These are in detail: 1. Conservative treatment. a. Weight stabilization (nutritional therapy and lifestyle adjustment). b. Compression treatment. c. Manual lymphatic drainage (only if edema is present). d. Apparative lymphatic drainage (only if edema is present). e. Exercise therapy. 2. Complex surgical treatment. a. Weight stabilization (conservative or surgical). b. Liposuction for lipedema treatment. c. Lifting operations according to need and medical necessity.Since we find weight stabilization in all treatment concepts, we will first discuss it. If you have an extreme fat distribution disorder and are of normal weight or only slightly to moderately overweight, you can skip Sect. 3.2.3 Treatment ofLipedemaAL GRAWANY983.2 Measures forWeight StabilizationIn weight stabilization measures, we distinguish conservative from surgical mea-sures. Let’s start with the general and conservative measures.3.2.1 Conservative MeasuresIn this context, it seems important to us to point out that the change of diet should be clearly distinguished from a diet. Perhaps you have already been on many unsuc-cessful diets. If you still have doubts in this regard, let us tell you: Diets are NOT the right way! But why? Often the problem lies in the way we lose weight. Low-calorie diets often do not lead to weight loss, but weight gain in the aftermath. What is the mechanism? With diets where we only eat 1000–1200kcal per day, the pounds may well fall off at the beginning of such a diet, but as soon as the desired weight is reached (or not), the well-known yo-yo effect occurs.The reason for this is that we initially lose mainly muscle mass. Lack of muscle mass leads to a reduction in basal metabolic rate. With the same food intake as before the diet, there is a continuous weight gain due to the now lower muscle mass. There is further scientific evidence as to why weight increases after dieting. The blood leptin level after a low kilojoule diet decreases. The low leptin level causes an increase in appetite. At the same time, metabolism slows down. The increase in appetite and slowing of metabolism can support this yo-yo effect.Fig. 3.1 Normal to severely overweight body shapes (without fat distribution disorder)Z. Jandali et al.99 c We do not recommend dieting.The repeated weight gain after diets has also given rise to the popular opinion that lipedema is diet-resistant. This cannot be generalized in this way. In general, the lower the obesity and the greater the disproportion, the smaller the effect of weight loss on the areas affected by lipedema. In normal-weight lipedema sufferers, weight loss will accordingly bring only a small change, sometimes only visible at a sec-ond glance. c We do not agree with the common opinion that areas affected by lipedema cannot be influenced by weight loss.The explanation for this probably lies in the altered hormonal responsiveness and signal processing of the fat cells. The diseased adipose tissue appears to be more resistant to weight loss than the adipose tissue in other regions. It decreases dispro-portionately to the residual adipose tissue during weight loss. But again, it’s differ-ent for overweight or severely obese people. Here, a prolonged and permanent weight loss is certainly accompanied by an overall decrease of the excess adipose tissue, including the areas of disproportion and the painful areas of lipedema. In many cases, weight loss is also accompanied by a significant improvement in pain symptoms, sometimes more, sometimes less pronounced. One of the most common statements made by sufferers is: “When I lose weight, it’s everywhere but on my legs and arms. “We do not agree with this, as we must again take into account the different starting conditions. c Massive weight loss in sufferers with a BMI >40 kg/m2 is often accompanied by relief of the pain associated with lipedema.Let’s now ask the loaded question: If a large percentage of lipedema sufferers also suffer from obesity, how well can they lose weight on their own? The answer is: very poorly! About 95% of all self-directed diet attempts in sufferers with a BMI >40kg/m2 fail. That’s a statement you have to come to terms with first. So if you have lipedema and a BMI >40kg/m2, it is very likely that you will not be able to lose weight on your own. You need professional help to treat your obesity.Whenever we feel that there is significant obesity, we recommend treating it as a first step. Depending on the degree of obesity, there are different approaches to treat it.This book is not a nutritional guide, but lipedema is associated with overweight or obesity in the majority of patients. Therefore, we cannot hide the issue therapeu-tically and have to deal with it. This is not about dieting, but about changing lifestyle habits and eating behavior. We will be brief at this point, as there are separate books and guidebooks for this.The causes of obesity are complex and not easy to break down. In the first step, it is important to visualize your ideal “I” internally. What characteristics should your ideal “I” have? This is about behaviors, attitudes (e.g., about food and 3 Treatment ofLipedemaAL GRAWANY100exercise), and where you see your body. How is the ideal “me” different from your current, present “me”? What are the lifestyle habits you would like to have?After a little self-reflection, you will find your answer. You should recognize and implement the challenges that this poses to you. In the next step, it is important to summarize the experiences you have made so far. Regardless of your success, it is the journey that counts. Don’t value your results so far as failures, but as steps that bring you closer to your goal. Methods that don’t work for you bring you closer to those that do.What measures do we recommend when we talk about changing eating habits? There are thousands of different ways. But this much from our side: we do not rec-ommend a dietary change that should work purely by reducing the number of calo-ries. To be successful, an adjustment must be made in four key areas of life: Nutrition, fitness, lifestyle, and psychology. . . . . . . . . . . . . . . . . . . . 199Zaher Jandali, Benedikt Merwart, and Lucian Jiga 5.1 Possibilities and Limits of Plastic Surgery . . . . . . . . . . . . . . . . . . . . 199 5.2 How to Find the Right Doctor? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 200 5.3 Presentation to the Plastic Surgeon . . . . . . . . . . . . . . . . . . . . . . . . . . 201 Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 205ContentsxvAbout the AuthorsCorradoCampisi, MD, Ph.D., MRM is a plastic, reconstructive, and aesthetic surgeon based in Genoa, Italy (GVM Care & Research: ICLAS—Rapallo, Genoa; Salus Hospital—Reggio Emilia; Maria Pia Hospital—Turin), and an Adjunct Professor of Plastic Surgery at the University of Catania, Italy. He com-pleted his Ph.D. in Experimental Surgery and Microsurgery at the University of Pavia, Italy, and his Master's Degree in Reconstructive Microsurgery at the UAB in Barcelona, Spain (Reconstructive Microsurgery European School—RMES). He is an Executive Committee Member of the International Society of Lymphology (ISL) and will host the 23rd ISL World Congress in Turin in 2023. He has pub-lished numerous scientific papers and contributed to several books on the surgical treatment of lymph-edema. The Genoa Lymphedema Clinic is interna-tionally known and receives patients from all over the world.Angel Pecorelli Capozzi, M.D. is a specialist in plastic and reconstructive surgery. Dr. Pecorelli com-pleted his undergraduate and six-year residency in Venezuela. Three years of his six-year residency were in the Department of Oral and Maxillofacial Surgery at the Dr. Miguel Perez Carreño Clinic, Caracas-Venezuela. Dr. Pecorelli completed his post-graduate studies with a focus on aesthetic and nonin-vasive treatments and physiological aging medicine. Dr. Pecorelli is the owner of Platinum Medical Center in Barcelona, Spain. Plastic & Reconstructive Surgery Specialist Specialization Postgraduate in Cosmetic & Aesthetic Medicine Specialization AL GRAWANYxviPostgraduate in Physiological Aging Medicine Member of the Ibero-Latin American Federation of Plastic Surgery (FILACP) Member of the Venezuelan Society of Plastic Reconstructive Aesthetic and Maxillo-Facial Surgery (SVCPREM) CEO at Platinum Medical Center (Barcelona, Spain) Board of Director of Spanish Society of Facial Plastic Surgery (SECPF) Vice-President of the Latin-American Society of Facial Aesthetic Surgery (SOLAFACE) International Educator in the Spanish Society of Facial Plastic Surgery (SECPF) Member of the Spanish Society of Cosmetic Surgery and Medicine (SEMCC) E-Mail: pecorellicapozziad@icloud.com Web: www.platinumbarcelona.comZaher Jandali, M.D. graduated in 2006 at the University Medical Center Hamburg-Eppendorf (UKE) in Hamburg as medical doctor. In 2012 he completed his training as consultant in plastic and aesthetic surgery in the Department of Plastic, Aesthetic, Reconstructive and Hand Surgery at the Asklepios Clinic in Hamburg-Wandsbek. Since day one, Dr. Jandali has been intensively involved with the topic of “lipedema.” Early on, he began giving lectures to those affected and interested, as well as to patient support groups. This was followed by lectures on this topic at national and international congresses. Since 2016, Dr. Jandali occupies the chair position of the Clinic for Plastic, Reconstructive, Aesthetic and Hand Surgery at the Evangelical Hospital in Oldenburg (Lower Saxony). Dr. Jandali further devel-oped several surgical techniques for lipedema, com-bining different approaches to achieve better and safer results. Since 2007, his main focus has been on the treatment of lipedema and lymphedema. He per-forms liposuction using a unique technique optimized for lipedema. Dr. Jandali also focuses on reconstruc-tive surgery after weight loss, as well as aesthetic and reconstructive microsurgery. Dr. Jandali is a member of the following professional societies: DGPRÄC, German Society of Plastic, Reconstructive and Aesthetic Surgeons DGH, German Society for Hand Surgery WSRM, World Society of Reconstructive Microsurgery www.jandali.de www.lipold.deAbout the Authorshttp://pecorellicapozziad@icloud.comhttp://www.platinumbarcelona.comhttp://www.jandali.de/https://www.lipold.dexviiLucianJiga, M.D. completed his medical studies at the Victor Babes University of Medicine and Pharmacy Timisoara in Romania. In 2002, he moved to the Ruprecht- Karls- University of Heidelberg for pursuing a research fellowship that led him to suc-cessfully defend his doctoral thesis three years later. After his stay in Heidelberg, in 2005 Dr. Jiga moved back to Timisoara to the University Clinic for Vascular Surgery and Reconstructive Microsurgery. Starting in 2009, he occupied the section chair posi-tion as Associate Professor in the Department of Reconstructive Microsurgery. In 2013, Dr. Jiga returned to Germany as a senior consultant of the Clinic for Plastic, Aesthetic, Reconstructive and Hand Surgery at the Evangelisches Krankenhaus in Oldenburg. Since 2016 he shares here the chair of department position with Dr. Jandali. Dr. Jiga looks back on a large number of international lectures and scientific publications. In addition to the treatment of lipedema, his clinical work focuses on reconstructive surgery after breast cancer and after weight loss, the treatment of lymphedema, microsurgical reconstruc-tive surgery, especially to preserve extremities, and complex hand surgery. Dr. Jiga is a member of the following professional societies: DGPRÄC, German Society of Plastic, Reconstructive and Aesthetic Surgeons DGH, German Society for Hand Surgery WSRM, World Society of Reconstructive Microsurgery TTS, The Transplantation Society.BenediktMerwart completed his medical studies at Heinrich Heine University in Düsseldorf, Germany, graduating in 2015. He discovered his interest in the treatment and therapy of lipedema and lymphedema early on in his training, which he began in 2015 at the Clinic for Plastic, Aesthetic, Reconstructive and Hand Surgery at the Evangelisches Krankenhaus under the direction of Dr. med. Z.Jandali and Dr. med. L.P.Jiga. Mr. Merwart regularly lectures on this topic for affected individuals and self-help groups as well as at national and international professional congresses. His other areas of interest include breast reconstruc-tion after breast cancer treatment and restoration of body shape after massive weight loss. Mr. Merwart is the author of several scientific publications and books.About the AuthorsAL GRAWANYxviiiRalfWeise, M.D. is a specialist in general, visceral and special visceral surgery, a specialist in surgical proctology, certified in minimally invasive surgery (CAMIC), and a specialist in nutritional medicine. Since 2006, he has been the head physician of the Clinic for General and Visceral Surgery at St.-Marien-Hospital in Friesoythe. In 2007, he founded the North-West Obesity Center, which was certified as a Center of Excellence in 2011 and as a Reference Center in 2015. In 2017, the Obesity Center cele-brated its 10th anniversary. To date, more than 3,500 obese patients have been treated in Friesoythe and more than 1,700 metabolic interventions have been performed. Dr. Weise has served as medical director at St.-Marien-Hospital in Friesoythe since 2013. He has given numerous lectures and presentations at international congresses and has published articles on obesity in four books. www.weiseoperiert.de www.adipositas- zentrum- nord- west.deAbout the Authorshttp://www.weiseoperiert.de/http://www.adipositas-zentrum-nord-west.de/http://www.adipositas-zentrum-nord-west.de/1© Springer Nature Switzerland AG 2022Z. Jandali et al. (eds.), Lipedema, https://doi.org/10.1007/978-3-030-86717-1_1Z. Jandali (*) · B. Merwart(Table3.1).The need for additional psychological co-treatment should be discussed with each patient and initiated if necessary.Did you know that there are many concepts that attribute incredible weight loss potential to the vital substance-rich fresh diet? It is even possible for someone suf-fering from obesity to lose weight on a hypercaloric diet, as long as the diet contains enough vital substances to make this reduction.Table 3.1 Measures per key area for a lasting change in dietArea MeasureNutrition Refrain from fats as far as possibleReduce carbohydrates/sugar to the maximum (avoid sweets, soft drinks, dough products such as bread or spaghetti)Conduct your dietary changes in a way that you don’t think of them as diets, but as long-term dietary changesExercise at least 20min daily with an increase to 30minTo do this, find a sport that you enjoy (alternate muscle building/cardio workouts)Fitness Increase daily activityWalk pathsLifestyle Avoid elevatorsYour work must leave you room for cooking, sports, and balanceRethink your sleep patternsSurround yourself with healthy living peopleIf the people around you oppose you, hinder you, or slow you down, think about these relationshipsAnalyze imbalances related to work, family, relationship, and financesThink positivePsychology Reduce stress to avoid relapseLove yourself and othersHave grace toward yourself. Don’t give up right away if you don’t reach the goal once. Nobody is perfectWrite in a diary. This helps to reflectThink long term. Short-term goals do not lead to the desired resultZ. Jandali et al.1013.2.2 Surgical Measures forWeight StabilizationRalf WeiseBecause conservative measures have a high failure rate for weight loss, surgical measures for weight stabilization have been at the forefront for some time. Patients with morbid obesity are high-risk patients. The demanding treatment requires close cooperation of many disciplines, which can only be guaranteed at specialized centers.Several months usually pass from the first contact to the implementation of an operative procedure, during which numerous examinations and treatments as well as organizational measures take place. For the planning and implementation of such a demanding concept, structured treatment paths as well as an institution that takes over the management of the patients and the organization have proven their worth. Such an institution for the treatment of patients with obesity-related diseases is called an obesity center.Obesity centers at large urban hospitals or university hospitals usually have all the necessary specialist groups under one roof. In contrast, obesity centers in smaller hospitals, some of which are located in rural areas, often have to rely on external cooperation partners. In these centers with a large catchment area, follow-up care can also be organized close to home at external locations. Both concepts are possi-ble and can demonstrate comparably good long-term results.Numerous studies and follow-up observations over the past decades have shown that a lifelong change in lifestyle only succeeds in absolutely exceptional cases without professional help. However, even with professional support, weight regain often occurs after treatment.When the nonsurgical approaches to the treatment of morbid obesity remain unsuccessful and have been exhausted, it is time to consider so-called metabolic interventions to support the therapy. As the name suggests, these are surgeries that serve to treat the secondary diseases of obesity, metabolic disorders. The additional reduction of body weight is a means to an end.Based on numerous study results, there is worldwide agreement among experts on when surgery should be offered. The current German guidelines are also based on this scheme.Surgical therapy for obesity is used when all nonsurgical treatment options have failed to achieve the treatment goal and one of the following constellations is present: 1. BMI between 30 and 35kg/m2 in patients with particular severity of concomitant and secondary diseases of obesity, 2. BMI between 35 and 40kg/m2 with secondary diseases (e.g., diabetes mellitus, hypertension), 3. BMI of 40kg/m2 or higher.From a BMI of 50kg/m2, there is an absolute reason to include a surgical proce-dure in the treatment plan.3 Treatment ofLipedemaAL GRAWANY102Before undergoing metabolic surgery, every patient should participate in a prepa-ration program. There is a whole range of different surgical procedures for the treat-ment of obesity, i.e. metabolic interventions, which are used worldwide. Many of these procedures are performed only sporadically in some centers. Their long-term benefit is often not sufficiently proven. Therefore, only the currently established procedures will be presented here. In advance, you will see the normal anatomy shown in Fig.3.2.All interventions are based on two effects, which are used either alone or in combination: – Reduction of the volume of ingested food by reducing the size of the stomach. – Reduction of absorption of food components in the small intestine due to bypass-ing of the small intestine.Which surgical procedure is the right one must be decided on an individual basis. The choice of surgical procedure depends, among other things, on the patient’s eat-ing habits, underlying diseases of the gastrointestinal tract, and occupational and lifestyle habits.Adjustable Gastric BandA plastic band is placed around the upper part of the stomach (Fig.3.3). This band is connected via a tube to a can which is positioned under the skin. By puncturing this can with a special needle, the size of the band can be adjusted. Thus, the patient’s personal setting, his “green zone,” must be found in several sessions.The number of gastric band implantations has declined sharply in Europe in recent years, while this procedure was first approved in the United States in 2010 and initially experienced a real boom there.Even though this is a quick, low-risk surgical procedure, many complications, most of them minor, often occur as the procedure progresses. These range from slip-ping of the band to infections and ingrowth of the band into the stomach. In LiverGall bladderColonStomachDuodenumSmall intestineFig. 3.2 The normal anatomy of the gastrointestinal tractZ. Jandali et al.103addition, the cooperation of the patient and regular follow-up care are particularly important with this procedure in order to achieve long-lasting good results.Many gastric bands must therefore be removed again in the course of life. Since the problem of obesity then persists, another procedure usually has to be performed.Gastric Tube (Sleeve Resection)The entire length of the stomach is closed and cut, leaving only a narrow tube (Fig.3.4). In contrast to other metabolic procedures, a large part (approx. 2/3) of the stomach must be removed. During the procedure, a relatively wide gastric tube is placed in the stomach so that the gastric tube does not become too narrow.Gastric sleeve formation has been increasingly used to treat morbid obesity for about 15years. In 2017, over 60% of metabolic procedures in Germany were sleeve resections. Originally, gastric sleeve was a precursor to performing a more complex procedure. However, it has become increasingly established as a treatment proce-dure in its own right in recent years.This procedure is only suitable to a limited extent for patients with heartburn (a so-called reflux disease). Recent studies on this topic are currently causing a critical rethinking of this surgical technique.Gastric BypassThe stomach is separated in the upper region (Fig.3.5), leaving only a small resid-ual stomach for the passage of food. For the onward passage of the food, the small intestine is cut at a certain point and connected to the small stomach. The previously severed small intestine,which is attached to the large stomach remnant, is recon-nected to the small intestine at a lower point.Fig. 3.3 Adjustable gastric band with port system3 Treatment ofLipedemaAL GRAWANY104Fig. 3.4 Sleeve resectionFig. 3.5 Gastric bypassZ. Jandali et al.105As a rule, this procedure achieves a weight reduction of 50–70% of excess weight. It combines stomach reduction with small intestine bypass.The first described insertion of a gastric bypass for the treatment of morbid obe-sity took place in 1966 by MD Mason in the United States. Since then, this proce-dure has become established, initially in North America and in recent years also in many other countries.The gastric bypass procedure is the best compromise for many patients because of the low risk of surgery and a high quality of life, as well as its excellent impact on secondary diseases. Under the regular intake of vitamins and trace elements, there is only a small risk of developing malnutrition.Mini Bypass (Omega Loop)The stomach is severed in the middle region, leaving only a relatively small residual stomach for the passage of food (Fig.3.6). To pass the food on, a loop of small intestine is connected to the side of the small stomach at a specific point.It combines a stomach reduction with a bypass of the small intestine. Some sub-stances in food, especially fats and carbohydrates, are no longer absorbed by the intestine to the same extent as before the operation.The Omega Loop is a relatively new procedure and has been used for several years with increasing numbers of operations. Even though it is now an established procedure, its position in the 2020 round of metabolic interventions has not yet been defined with certainty.Duodenal SwitchAfter creation of a tubular stomach (sleeve), the duodenum is cut behind the stom-ach outlet. For the unobstructed passage of food, a connection is made between the Fig. 3.6 Omega loop3 Treatment ofLipedemaAL GRAWANY106stomach and small intestine after the small intestine has been cut. The remaining loose end of the small intestine, which carries crucial digestive juices, is connected to the colon relatively close to the point where the small intestine joins the colon (Fig.3.7).As a rule, this procedure achieves a weight reduction of more than 70% of excess weight. In contrast to gastric bypass, the gastric pylorus is preserved, which reduces the risk of food falling out. It is occasionally performed as a second surgical treat-ment step after sleeve resection.With a reduction of over 70% in excess weight and excellent control of concomi-tant diseases, patients with a duodenal switch have the best long-term outcomes.SADI-SThe duodenal switch procedure is surgically demanding with a significantly increased complication rate. An alternative procedure has therefore been developed in which a gastric tube is also formed. In contrast to the duodenal switch, a loop of small intestine is connected laterally to the duodenum beyond the gastric outlet to pass the food (Fig.3.8). The operation times and the risk of complications are thus significantly lower than with the duodenal switch.The relatively radical small bowel bypasses of the last two procedures lead more frequently than the other procedures to malnutrition and occasionally to foul- smelling fatty stools. They are therefore considered reserve procedures for particu-larly severe courses of disease.Fig. 3.7 Duodenal switchZ. Jandali et al.1073.3 Complex Physical TherapyLet’s talk about the complex-physical therapy of lipedema. It consists of the follow-ing components: – Compression treatment. – Manual lymphatic drainage (only if edema is present). – Apparative lymphatic drainage (only if edema is present). – Exercise Therapy. – Rehabilitation treatment.Certainly, complex-physical therapy is an important component of treatment, and we have often observed an improvement through a change in lifestyle with appropriate dietary changes and exercise in combination with complex-physical therapy, but rather only discretely pronounced. In addition, we have not yet seen lipedema regress via physical treatment.Compression TreatmentNormally, compression treatment is given to reduce edema, i.e. typically in lymph-edema and lipo-lymphedema. By reducing the edema, there is a decrease in symp-toms. In lipedema, the situation is different, since in the majority of cases there is no edema. Consequently, the edema is not the reason for the compression treatment. There is evidence for a reduced pain stimulus transmission triggered by the Fig. 3.8 SADI-S3 Treatment ofLipedemaAL GRAWANY108compression treatment and thus pain reduction. In addition, the compression gar-ment gives a feeling of stability.The question that often arises is: flat knitted or circular knitted compression stockings? Circular or flat knitted refers to the manufacturing process. Circular knitted stockings are made seamless and spiral. They are more elastic and give more than flat knitted ones. Flat knitted stockings are made of more robust material and with a seam. Flat knitted stockings can exert pressure over a wider area, are more individually adjustable, and are also suitable for difficult conditions.Venous disorders are usually treated with round-knitted compression stockings. In contrast, patients with edema are more likely to receive flat-knit compression stockings. Flat-knit compression stockings have also proven effective for lipedema. Compression stockings are available in different compression classes. We distin-guish between compression classes 1–4, with compression class 1 being the lightest and 4 the strongest (Table3.2).In compression garments, we distinguish between custom-made and prefabri-cated compression garments. In addition, different forms of compression garments are available. Classic for lipedema are stockings in a long form and—much rarer—in a short form (Figs.3.9 and Fig.3.10).However, most often we see compression tights. From our experience, we have the best feedback from tights (including the hips), as they fit quite stably and pro-vide good comfort. The form of compression used depends on the areas and how pronounced the lipedema is.A compression garment for lipedema is available through a prescription from a panel physician, including a change of care. All those who do not suffer from clini-cal edema and start compression treatment will notice a reduction in circumference when wearing compression garments. In combination with the loss of inherent elas-ticity of the compression pants, a replacement fitting is necessary after approxi-mately 6months—possibly earlier for the first fitting.Due to the individual characteristics, we recommend custom-made flat-knitted compression stockings class II in the form of pantyhose. Most medical supply stores will measure you individually for this. Sleeves or boleros (jackets) are available for the arms. Here we rather recommend the use of boleros. c For lipedema, we recommend a supply of flat-knitted compression stockings class II.Table 3.2 Compression stocking classesCompression class Strength Compression in kPA Compression in mmHGI Easy 2.4–2.8 18–21II Medium 3.1–4.3 23–32III Powerful 4.5–6.1 34–46IV Very strong >6.5 >49Z. Jandali et al.109Manual Lymphatic DrainageIf there is no edema, no drainage of this edema can be responsible for pain relief. However, many state that lymphatic drainage relieves pain and does good. So what is the positive effect of lymphatic drainage? The positive effect of lymphatic drain-age probably lies more in the attention experienced through therapy. The closeness to the therapist, the recognition of the disease, and accompanying processing. The therapist is there for you, listens and is a constant contact person for your illness. Those affected can often switch off and “shut down” in the process. c Even iflymphatic drainage has positive effects, they are not due to the treatment of the disease.Apparative Lymphatic DrainageApparatus lymphatic drainage, also known as apparatus intermittent compression (AIK) or intermittent pneumatic compression (IPC), is often colloquially referred to as “lymphomat” and is not indicated for classic lipedema. Apparative lymphatic drainage is regularly used in decongestive therapy for lymphatic or venous diseases. If lipo-lymphedema exists, lymphatic drainage by means of an apparatus makes sense.Fig. 3.9 Compression stockings short (lower leg stockings)3 Treatment ofLipedemaAL GRAWANY110This is how apparative lymphatic drainage works: A double-walled cuff consist-ing of several air chambers are applied to the legs or arms and connected to a pro-grammed compressor. The compressor regulates the air supply and discharge as well as air pressure in the cuff. The device generates pressure up to 200mmHg. The pumping cycles are programmed to cause decongestion. The cuff pumps up first away from the body and then the overlapping air chambers. The chambers remain filled until the last chamber reaches the set pressure. Then simultaneous deflation occurs and a new cycle begins.The devices are available in practices and for home use. A prescription is issued by the doctor. The medical supply store then checks whether the costs are covered Fig. 3.10 Compression tights longZ. Jandali et al.111by the health insurance company and finally provides the device. This method also uses external pressure to support the veins and lymphatic vessels. c Apparative decongestive therapy is not a basic therapy for lipedema and is reserved for diseases with an edema component.Exercise TherapyAs an essential component, we would like to mention exercise therapy. By this we mean an increase in activity and muscle building. Initially, exercise in and around water, e.g. aqua walking and aqua gymnastics, is certainly advisable due to the reduction of the patient’s own weight in the water and simultaneous water resis-tance. Water-based exercise therapy is gentle on the joints and sustainable. It is also important here that not only fitness but also musculature is built up.There are experts and further literature for movement therapy to which we would like to refer at this point. A highly recommended program for exercise and fitness in general is, for example, the so-called Sei-stolz-auf-dich concept (www.seistolza-ufdich.de).Rehabilitation TreatmentInitial treatment with a holistic approach often takes place during inpatient rehabili-tation treatment (cure). Here, depending on the concept, the psyche and body are strengthened. Furthermore, nutritional counseling and decongestion therapy are often provided. Certainly, rehab is more indicated for those affected with edema than for those without, since the initial edema therapy requires constant and daily treatment.3.4 Complex Surgical TreatmentIn the past, surgical therapy for lipedema was always in the background and was the last therapeutic option, even for us surgeons. I still remember my first day of plastic surgery training. One of my first patients in the consultation was actually a lipedema patient. At that time, metabolic surgery was still in its infancy, and we often per-formed so-called megaliposuctions in which many liters of fatty tissue were suc-tioned out (up to over 20L of pure fatty tissue in one operation). This is a strategy that no one would approve of today. However, these experiences and complications have shaped us in retrospect and influenced our current standard of care. Combined surgical and conservative treatment, as we perform it today, was not standard back then.Surgical therapy (Fig.3.11) has now emerged from the shadow of purely conser-vative treatment, and we have long since ceased to say that purely conservative treatment must be exhausted in order to initiate surgical therapy. It depends on the treatment goal, your wishes, and medical aspects.Conservatively oriented clinics often don’t leave a good hair on liposuction and lump all surgical concepts together, although both sides are noticeably working to 3 Treatment ofLipedemaAL GRAWANYhttp://www.seistolzaufdich.dehttp://www.seistolzaufdich.de112overcome the hurdles and understand and acknowledge each other’s concepts—apart from some lymphatic therapists who still believe that lifelong lymphatic drain-age therapy is the golden path. At this point, we ask patients who hold this conviction to pause for a moment and reflect on how much they are benefiting from their man-ual therapy in the long term and whether compression is the right path for them.Our treatment strategy of complex surgical treatment is completely unknown to many. Complex because liposuction as a surgical measure is one of many compo-nents that lead to success (Fig.3.12).Since we often make initial diagnoses and initiate conservative treatment ini-tially (decisive for the insurance applications), we can estimate quite well how much success the conservative treatment brings according to our experience. After 6months we evaluate our initiated treatment consisting of compression (formerly in combination with lymphatic drainage) and nutritional or exercise therapy. We have rarely seen long-term positive courses of conservative treatments. It should be men-tioned that these results are observational and not from a blinded scientific study.It is possible, of course, that many patients who have received purely conserva-tive treatment externally do not find their way to us because they no longer have any complaints, and consequently only those who respond poorly to conservative treat-ment present to us. However, this contradicts the initial diagnostic treatment we perform. We have no other possible explanations for these observations. Certainly, we have also seen patients who have improved only by compression treatment and a change in their lifestyle, including diet. However, this is rather the exception than the rule. c Lipedema is not curable by either conservative or surgical therapy.We are now of the opinion that conservative therapy has its justification, but is not the only option. It should certainly not be exhausted for years, only to be replaced by surgical therapy. We often see a lasting effect with our concept of complex- surgical treatment. It is important for us to make an honest indication and to see who is suitable for surgical intervention and who is not.Fig. 3.11 Performing liposuction using the waterjet-assisted techniqueZ. Jandali et al.113 c We often see a lasting effect through our concept of complex surgical treatment.If the accusation comes that surgeons naturally only want to operate, we have to face it and can—until science is ready—only give our experience and opinion based on it to the best of our knowledge and belief. Regardless of the treatment, our pri-mary goal is to treat your pain. Other goals are to promote your mobility and to support you so that you can do again everything that you were no longer able to do due to the changes caused by lipedema—always with the best possible aesthetic result in mind.Of course, we also want you to be satisfied with your external appearance and body contour. We always keep this aspect in mind during the surgical therapy of lipedema. This also means that we not only remove disturbing fatty tissue, but also treat any excess skin that may occur during large-volume liposuction. It does not make sense to suction out disturbing and painful fatty tissue from you, but then leave you to fend for yourself with excess and hanging skin. Unfortunately, this is exactly the problem we see very often, because many of the doctors who are not trained in plastic surgery do not offer tightening surgery. Therefore, tight-ening surgery is also an important selection criterion for choosing a plastic andFig. 3.12 Liposuction as a building block of the treatment plant3 Treatment ofLipedemaAL GRAWANY114aesthetic surgery specialist. You can find out more about how to find a suitable doctor in Chap. 5. c Especially if you suffer from pronounced lipedema, you should look for a practitioner who also performs tightening surgery. Ask if their practitioner also performs thigh lifts and upper arm lifts. Ask to see results of these tightening surgeries.A much advertised claim is that the previous need for lymphatic drainage after liposuction no longer exists. The first question to ask here, of course, is: Was lym-phatic drainage actually necessary before? Probably rather not. So here professional providers of liposuction take up a previously unnecessary wrong treatment in order to advertise that it is no longer necessary afterwards.Even if lipedema appears complex, it can be broken down into individual com-ponents. Therefore, liposuction makes absolute sense in many constellations. If liposuction is performed in our clinic, this is always done according to a previously prepared therapy plan—whether in the case of overweight patients with a change in diet and exercise therapy or in the case of morbid obesity with prior weight reduc-tion through bariatric surgery. All measures are building blocks of our complex surgical treatment. c In most cases, liposuction is only part of the lipedema treatment.Liposuction can only be successful if all criteria for a successful treatment are met in advance. These include, among other things, a stable weight, the necessary compliance, well-controlled physical or psychological concomitant diseases, if present, and last but not least realistic expectations of the result.3.5 The Individual Therapy PlanEach of us is different. There is a huge variety of body shapes and equally different manifestations of lipedema. Therefore, we create an individual therapy plan for each affected person.What does our complex surgical treatment for lipedema look like in detail? In the run-up, we often, but not necessarily, perform a purely conservative treatment for at least 6months. This is mainly due to the fact that the health insurance companies require this procedure for an application for reimbursement. During this time, other underlying diseases should be excluded (e.g. venous disease). For the initial treatment, we recommend flat-knitted compression stockings of class 2. If there is confirmed edema in the sense of lipo-lymphedema, supportive manual lymphatic drainage may be useful.If your parameters and body constitution speak for the existence of significant obesity (also taking into account the WHR and WHtR), the treatment of lipedema is always preceded by weight reduction. This means in 95% of massively overweight Z. Jandali et al.115cases a metabolic intervention in the sense of gastrointestinal surgery in a special-ized center for obese people. We cannot and do not want to use BMI values for patients with lipedema, but must decide on a case-by-case basis. There is certainly an indication for surgery if the BMI is clearly >40kg/m2. However, surgery is often indicated even if lipedema is not severe and the BMI issaline solution into the tissue. This special saline solution (also known as tumescent solution in technical jargon) con-sists of saline, a local anesthetic for pain suppression, adrenaline, and a buffer (solu-tion for balancing the acid-base balance in the tissue; Fig.3.14). Due to the local anesthetic of the solution, liposuction can be performed very well only with the special saline solution without general anesthesia.By introducing the special saline solution, three main effects are achieved: 1. Local anesthesia eliminates the sensation of pain. 2. The adrenaline in the solution causes blood vessels to constrict, resulting in much less bleeding into the tissue. 3. Flushing the fat tissue with this solution loosens it up and prepares it for liposuction.Fig. 3.14 Preparing the tumescent solution3 Treatment ofLipedemaAL GRAWANY118Depending on how much special saline solution (amount in ml) is introduced into the fatty tissue, the common suction methods are also called wet (“wet”), super wet (“super wet”), and tumescent methods. The tumescent and super-wet methods introduce a lot of fluid into the tissue. Just to understand the quantities: The super- wet method puts about as much special saline solution into the body as fat tissue is to be removed (1:1). The situation is different with the tumescent method. Here, two to three times as much fluid is brought into the tissue as fatty tissue is to be removed. We recommend the tumescent or super-wet method, but each surgeon has his or her own preference.The Dry technique, which completely avoids the use of tumescent solution, is not recommended. This technique causes severe tissue trauma and often leads to unsightly dents and ripples. Moreover, uncontrolled bleeding and very severe scar-ring may occur.We recommend the classic wet method, which represents a good middle ground. With this method, not too much liquid is introduced and an assessment of the pos-sible result is still possible. c We recommend liposuction with the tumescent or super-wet method and strongly advise against dry liposuction without fluid (dry method).Technically apparatively we distinguish different systems with which we can perform liposuction. We distinguish classic liposuction from water jet–assisted lipo-suction (WAL), power or vibration assisted liposuction (PAL), ultrasound liposuc-tion, laser liposuction, and many others, the list of which would go beyond the scope of this article. However, we would like to go into more detail about some of the techniques mentioned.As mentioned, all techniques involve loosening of the fatty tissue via the special saline solution (tumescent solution). From a scientific point of view, none of the techniques described below can be said to be superior. Rather, each practitioner has his or her own personal technique. With regard to the possible forms of anesthesia, the methods do not differ. All of them are possible in a local partial or general anes-thesia. We will come to the forms of anesthesia later on.In our clinic, most of the techniques listed below are used with the various sys-tems. Due to the diverse systems available, an individual adaptation to the respec-tive patient can be made.Fig. 3.15 Different cannula of different systemsZ. Jandali et al.119 c From our point of view, the method or system of liposuction is secondary. More important are the training and experience of the surgeon.For each system, there are different cannulas with which the fat is aspirated (Fig.3.15). We distinguish between cannulas with small diameters of 2–3mm and very large-lumen cannulas with 5–6 mm. We also distinguish between cannulas with many or few openings and straight from curved cannulas. Some cannulas are more rigid than others are slightly more flexible. Another decisive difference is the sharpness of the holes, so some cannulas have very sharp holes and can thus almost cut and others are very blunt.In the case of large-volume liposuction, large-lumen cannulas tend to be used in the operation at the beginning, and thinner cannulas are then used as the operation progresses. The thinner cannulas are thus used more for definition and fine contouring.Before we discuss the different systems, let’s briefly look at the manual tech-nique. Many differentiate between purely aesthetic liposuction and liposuction for lipedema in terms of manual technique. The distinction should be made on the basis of lymphatic vessel sparing and non-lymphatic vessel sparing. It is said that in lipedema liposuction is performed only parallel to the lymphatic vessels and in aes-thetic liposuction also in a so-called criss-cross technique.In the initial chapters, we talked about the anatomy of lymphatic vessels. Let us now imagine a moving liposuction cannula in close proximity to a lymphatic vessel. If we suction parallel to the lymphatic vessels, they can certainly be injured less quickly than if we perform liposuction at right angles to the lymphatic vessels. We view the entire discussion about parallel and nonparallel suctioning to the lymphatic vessels in aesthetic procedures and in lipedema as critical. In our opinion, the lym-phatic vessels must always be handled gently and considerately, regardless of whether lipedema is involved or not. It is not so much a question of the direction of suction, but rather how sharp the cannulas are (by this we mean the sharpness of the holes) that are used. Sharp cannulas cut the tissue and thus also lymphatic vessels. It is therefore important to avoid sharp cannulas.Finally, let’s talk about negative pressure during liposuction. All techniques require negative pressure to suction off the fat cells. The interaction of negative pressure, cannula, and cannula sharpness is particularly important. The more nega-tive the negative pressure, the more aggressively the fat tissue is suctioned out. Liposuction is performed at −100 to −1000mbar. Relatively little fatty tissue is suctioned at −100mbar and a great deal at −1000mbar. But the negative pressure does not only have an effect on the fat cells; of course, it also has an effect on the surrounding tissue. If we work with a very negative pressure, injury to the surround-ing tissue is more likely, because the tissue is sucked in more. So if we change one component (negative pressure, cannula holes, cannula sharpness), it will affect the entire liposuction procedure. Here it is important to achieve a harmonious interac-tion of all components.3 Treatment ofLipedemaAL GRAWANY120Classic LiposuctionIn classic liposuction, rigid, straight, or curved cannulas are used (Fig.3.16). It is the classic image as we know it from television. Apart from a system that creates suction, no other aids are used.Classic liposuction still has its justification today. It works perfectly and is the most frequently used method worldwide. However, it is possible to make life much easier for the surgeon by using machine support, e.g., with the systems presented below (Fig.3.17). Nevertheless, we continue to use classical liposuction, especially for small, circumscribed liposuctions to obtain fat tissue for autologous fat transplantation.The disadvantage of classic liposuction is that we see significant fibrosis of the fatty tissue, especially in advanced courses. The more advanced fibrosis is, the more “troublesome” liposuction is. The same is aggravated after liposuction, the internal fibrosis and scarring continue to increase. Once an area has been liposuctioned, it is difficult to perform a second liposuction in that area. Therefore, various devices have been established to “simplify” liposuction. In the following, we will discuss some of these supporting devices.Fig. 3.16 Classic cannulas for liposuctionFig. 3.17 WAL + PAL + classic liposuctionZ. Jandali et al.121Water Jet Assisted LiposuctionWater jet–assisted liposuction is also called Bodyjetliposuction (from the name of the device that is used) or high-pressure water jet liposuction. WAL has been around for over 10years, and it has found its permanent place in liposuction procedures. In WAL, a special device called a bodyjet is used. The special thing about this is that in this form of liposuction a water jet continuously assists (assists) the liposuction, hence the name. The liposuction is performed through a double-lumen cannula. Liposuction is performed via the outer large-lumen portion, while the smaller inner portion of the cannula is used for simultaneous injection of tumescent solution (Fig.3.18).In all forms of liposuction, the special saline solution is introduced into the fatty tissue at the beginning of liposuction, usually with quite a large volume (tumescent or super-wet method). This is different in the WAL method. Here, although tumes-cent solution is also infiltrated at the beginning of liposuction, it is in a much smaller volume, which is only sufficient for anesthesia and initial preparation of the fat tis-sue, as the continuous water jet makes its own contribution to further anesthesia and fat cell release during liposuction. This gives a small advantage to aesthetic liposuc-tion procedures where little fat tissue is present and consequently little is to be suctioned. The inexperienced surgeon can better judge the contours if not too much tumescent solution has been infiltrated.One advantage of WAL is the simultaneous flushing out of the fat cells during liposuction. This is supposed to be gentler and less traumatic for the tissue, but also for the triggered fat cells. In the case of autologous fat transplantation, this method is preferred because fewer stem cells are to be destroyed than with other methods. We also use WAL in addition to PAL for second or third liposuction when areas have already been liposuctioned. Many refer to WAL as new and innovative, which it is not. In our opinion, the WAL method has been replaced by the PAL method in many areas. c The WAL method is one of our standard methods of liposuction, along with PAL and classic liposuction.Fig. 3.18 Water-jet assisted liposuction (WAL)3 Treatment ofLipedemaAL GRAWANY122Power-Assisted LiposuctionVibration-assisted liposuction is also known as power-assisted liposuction or PAL.The word “power” already gives a lot away. The system does indeed have “power” in it and is one of my personal favorites among liposuction systems.Unlike the Bodyjet, which is produced by only one company for WAL, there are significantly more manufacturers offering a PAL system in the power-assisted sys-tems segment. The main difference to waterjet-assisted liposuction is that the can-nulas of the PAL can either only infiltrate or only suction, but not both at the same time. Consequently, with this technique, it is necessary to infiltrate first and then suction in a very classic way.The principle behind PAL is to release the fat cells from the tissue complex with vibrational energy. The surgeon holds a handpiece with a “motor” in his hand that triggers vibrations (Fig.3.19). Different cannulas can then be attached to this hand-piece. The vibration generated, although somewhat more powerful, is similar to that of the well-known ultrasonic vibration toothbrushes. The fat cells are aspirated simultaneously with the release.The PAL method is gentle on tissue and particularly suitable for large-volume liposuction. But fine contouring can also be performed very well with the PAL method. I personally prefer the PAL method to most other methods for first, second, and third liposuctions. It is also very well suited for autologous fat transplants and offers the best results in our hands with WAL.In Fig.3.19 you can see a PAL system for liposuction. c PAL suction is one of the most suitable techniques for the treatment of lipedema.Ultrasound Assisted LiposuctionUltrasound-assisted liposuction (UAL)is also a long-established method that has found some enthusiasts. It was established in Europe in the 1990s and is not widely used. It uses low-energy ultrasound, and liposuction is performed over 3 substeps. First is the infiltration of the tumescent solution, the second step is the release of the Fig. 3.19 Handle (motor) of power-assisted liposuctionZ. Jandali et al.123fat cells from the connective tissue complex via the ultrasound probe, and finally, in the third and final step, the detached fat cells are suctioned out. Of course, nerves, blood vessels, and skin remain unharmed during ultrasound-assisted liposuction. Some colleagues consider this form of liposuction less suitable for autologous fat grafting, although clinical studies show that fat tissue suctioned by UAL can also be used for autologous fat grafting.Manufacturers of UAL devices see a great advantage of UAL in body contouringor body sculpting. In this case, the body is particularly defined during suction, such as a six pack on the abdomen, which is then simulated by fat. But all other methods can also be used very well for body sculpting; it is just a question of the method.Laser-Assisted LiposuctionIn laser-assisted liposuction, the fat cells are destroyed by a laser that simultane-ously spares the surrounding connective tissue. The heat generated by the laser stimulates collagen synthesis in the connective tissue, which should lead to tissue shrinkage and associated tightening effect.Especially this method requires an experienced surgeon. In the past years, we have seen some patients with unsightly dents and skin defects after burns caused by such laser liposuction. In our opinion, the propagated tissue shrinkage by the laser can also be achieved by using other techniques with specific cannulas or by using tightening devices (Sect. 4.2). This feature of laser liposuction should therefore not be the decisive selection criterion for this form of liposuction. If you are interested in laser liposuction, seek out a truly experienced surgeon who has been performing laser liposuction for some time. The results are usually the same with skilled sur-geons regardless of the technique used.3.6.3 Liposuction VolumeOne of the questions we are most frequently asked, apart from the technique used, is the maximum number of liters of fatty tissue we can aspirate in one operation (Fig.3.20). In order to answer this question in a comprehensible way, we must first take a closer look at the “liter figure.”As already explained, before liposuction, a special saline solution (tumescent solution) is introduced into the tissue to prepare the fatty tissue for liposuction. During liposuction, fat cells and portions of the tumescent solution are suctioned out. The remainder of the tumescent solution is eventually absorbed by the body (Fig. 3.21). The “suction bag” now contains the so-called lipoaspirate as a fat- tumescent solution mixture. c The lipoaspirate is a mixture of liquid and fatty tissue.Quantity SpecificationThere are two ways of specifying the quantity. Either the total amount of lipoaspi-rate or the pure fat amount is indicated. If you want to specify the pure amount of 3 Treatment ofLipedemaAL GRAWANY124Fig. 3.20 Lipoaspirate after waterjet-assisted liposuctionPrinciple of liposuctionliposuction cannulacannula openingFat (Superficial)fat (deep)aspirated fatFig. 3.21 Principle of liposuctionZ. Jandali et al.125fat, you must allow time for the lipoaspirate to settle from the water. Since fat has a lower density than water, the fat settles in the upper phase and water in the lower phase. Thus, the pure amount of fat tissue can be indicated. But even then, there is still a certain amount of fluid between the fat cells, although not much. The longer you give the lipoaspirate time, the more accurate the pure adipose tissue quantity can be indicated.Unfortunately, there is no standard for specifying the volumesaspirated. It is best to ask your surgeon whether the figure is for pure adipose tissue or for lipoaspirate (fat + liquid), so that you know how to interpret the figures.Depending on which method is used for liposuction and how small or large the cannulas are, the total amount of liposuction varies. Liposuction with very small cannulas (WAL or PAL) results in a lower amount of pure fat tissue than with large- lumen cannulas (classic liposuction). It is most likely due to the fact that the cells are separated more and no conglomerates are suctioned, which are more condensed.We have often seen that only the total volume is given in surgery reports. The pure fat volume should always be stated so that it is comparable and others do not stand out with a misleading “I suction more.” We always state our liposuction vol-umes as pure fat volumes after an appropriate “settling time” of about 20min. c Liposuction volume should always be reported as fat-only to ensure consistent documentation.In Fig.3.22, we see different lipoaspirates with different proportions of fatty and aqueous phase. Aspirated quantities of fatty tissue vary from a few milliliters a b c dFig. 3.22 Different lipoaspirates. A, B fat phase (1) and water phase (2); C, D almost exclusively pure fat tissue (without 1) and only low water phase (2)3 Treatment ofLipedemaAL GRAWANY126(Fig.3.23), such as in cosmetic procedures on the face (neck or cheek suction) or for use as a filler for autologous fat transplants for wrinkle treatment, to over 10L of pure fat in so-called megaliposuctions. The term megaliposuction is not defined and is very vague. Many understand a megaliposuction as a total lipoaspirate quantity (fat and liquid) of more than 5L.For us, megaliposuctions are defined as a suc-tioned, pure fat quantity of more than 10L.Safe Suction VolumeThe scientific landscape is divided on the safety of liposuction. In America, pure fat amounts of 3–5% of one’s own body weight are indicated as safe. In Europe, scien-tific papers show a safe amount of suction of 8–10% of one’s own body weight.This means that for a body weight of 80kg, liposuction of 3% of body weight results in a pure amount of fatty tissue of 2.4L, and for a suction amount of 10% of body weight, 8L.Thus, quite a large variable is shown when it comes to the amount of fat tissue suctioned. Table 3.3 shows an example of the different quantities depending on body weight. c There is no consensus on how much fat tissue should be suctioned out in a low-risk surgery.How do such different statements regarding the maximum suction volume come about? This is simply a matter of safety and risk minimization. In general, the less fatty tissue that is aspirated, the lower the risk. We would like to go into this problem in a little more detail.Fig. 3.23 Approximately 20 mL of fat for autologous fat graftingTable 3.3 Safe fat aspirate by aspiration amount and body weightBody weight (kg)3% Fat aspirate (L)5% Fat aspirate (L)8% Fat aspirate (L)10% Fat aspirate (L)70 2.1 3.5 5.6 7.080 2.4 4.0 6.4 8.090 2.7 4.5 7.2 9.0100 3.0 5.0 8.0 10.0110 3.3 5.5 8.8 11.0Z. Jandali et al.127Aspect Tumescent SolutionThe more volume is to be suctioned, the more tumescent solution is introduced into the tissue. The special saline solution often contains lidocaine as a local anesthetic. Above a certain concentration, this can lead to an oxygen deficiency in the body, which is due to the fact that the drug alters (oxidizes) the red blood pigment “hemo-globin,” resulting in so-called methemoglobin. Due to its higher binding power to oxygen, it deprives it from hemoglobin, and there is an oxygen deficiency in our body. Symptoms are the classic signs such as headache, fatigue, shortness of breath, lethargy, and blue discoloration of the skin and mucous membrane.We and many other practitioners therefore resort to an alternative local anesthetic to avoid this problem. Others do not use a local anesthetic at all and only perform the treatments under general anesthesia and without any addition of local anesthetics. We feel that the local anesthetic in the tumescent solution is a good and sensible pain treatment for the first hours after the operation. Therefore, in our opinion, a certain amount of local anesthetic should always be used to ensure pain relief after surgery.Aspect DilutionDuring liposuction, the introduction of the tumescent solution, which is absorbed by the body in large proportions, results in a dilution effect of the blood. Blood consists of about half liquid blood plasma and half blood cells or blood corpuscles. The percentage of blood cells in relation to the total blood volume is called hematocrit. Since the red blood cells, the so-called erythrocytes, make up approx. 99% of the blood cells and ensure oxygen transport in the body, a low hematocrit value indicates a low proportion of red blood cells and thus a low oxygen transport capacity (Fig. 3.24). Consequences of a too low hematocrit can be dizziness, Fig. 3.24 Blood composition (hematocrit)3 Treatment ofLipedemaAL GRAWANY128nausea, pallor, circulatory weakness, lack of strength, loss of appetite, malaise, and headache. c The hematocrit value indicates the proportion of red blood cells in the total blood volume. The normal value for women is a hematocrit of 37–45%. c If there is too much fluid or too few red blood cells in the blood, this is called dilution. Dilution is indicated by a lowered hematocrit value (HK or Hkt value).Aspect BleedingOf course, bleeding occurs in any surgery, both during and after the procedure. Bleeding has historically always been the main problem with liposuction. They have limited liposuction for a very long time. The larger the procedure, the higher the risk of bleeding becomes. In surgery with a proper skin incision, you would see the source of bleeding and perform hemostasis via cauterization of the blood ves-sels. However, this is not possible with liposuction because we operate with long cannulas through a small incision of about 5mm. Therefore, the “adrenaline” in the special saline solution is indispensable. It acts on the blood vessels and causes them to contract. This has two effects: First, the small blood vessels close their lumen (the opening where blood flows through). Second, the blood vessels take up less space in the tissue, and injury with the suction cannulas is reduced. We know pretty much when and how well the epinephrine is working, because as soon as the epinephrine kicks in, the skin turns completely white as a sign of decreased blood flow, also known as the blanching effect (whitening) in medical jargon. But also during the operation we can see how the lipoaspirate looks in the tube. If it is golden yellow, then there is little to no blood mixed in. If it is red or reddish, there is more blood mixed in with the lipoaspirate. Nevertheless, there is always some blood loss.Blood loss is accompanied by a drop in the hemoglobin level. Hemoglobin (Hb) is the red blood pigment and a protein of red blood cells (erythrocytes). The most important task of hemoglobin is the transport of oxygen and removal of carbon dioxide in the blood. c Blood loss is accompanied by a drop in hemoglobin levels.The hemoglobin value depends on age and gender. Women have a normal value range of 12–16g/dL.If bleeding occurs, the Hb value drops because the body is unable to produce new red blood cells quickly enough. Similarly, dilution (overhy-dration with tumescent solution) leads to a lowered Hb level. This means that over-hydration with tumescent solution causes a drop in hematocrit and a drop in hemoglobin, and the drop in hemoglobin is also aggravated by a real loss of erythrocytes.Thus, the greater the blood loss and the higher the dilution, the more likely are reduced performance, shortness of breath (initially only during exertion,then also at rest), palpitations and ringing in the ears, pale skin and mucous membrane. If Z. Jandali et al.129blood loss is too great, a blood transfusion must be given. In the worst case, one can bleed to death from it.How much blood you actually lose depends on various factors. Physical factors, technical aspects, and surgical factors. Physical factors include how good one’s blood clotting is, how fit and well one’s body works, whether one suffers from high blood pressure, and whether there is any family history of clotting disorders (which often do not warrant liposuction), etc. Like anyone who focuses on liposuction, we have our own technique. Depending on whether we operate under general or local anesthesia, we use an adapted composition of tumescent solution. Especially in the case of high-volume liposuction, we have modified it to further reduce the compli-cations mentioned, such as dilution, side effects of local anesthetics, and bleeding.Time ExpenditureAccording to the effort required, the time needed for liposuction varies from 30min to over 4h. At the same time, liposuction in a very slim lady who is undergoing treatment for purely aesthetic reasons or for atypical lipedema can take much longer than a 10-L megaliposuction, with a suctioned pure fat volume of 2–3L.It depends on the actual effort and how much care the region needs at the moment of treatment. For example, the lower leg region is considered time-consuming, the upper back region is considered difficult to perform and bloody compared to other regions (there are more powerful blood vessels in this region). Thus, each region has its own characteristics, which is also reflected as another factor on the aspect of time. Recent studies show that the surgical risk of complications increases with the duration of surgery. For most liposuction procedures we need at least 2h of pure operation time with quite a lot of experience, but often 2.5–3h, sometimes even longer. With suf-ficient experience, the pure operation time can be kept relatively short and effective. Very often liposuction is performed not only by one surgeon, but by a whole team. A well-coordinated team therefore brings advantages here. Do not compare your liposuctioned fat amounts with others, as the conditions are different and the time required differs depending on the region and the patient. Liposuction of the hips is difficult to compare with liposuction of the lower legs.Outpatient–Inpatient?Liposuction we generally perform on an outpatient basis up to a volume of 3–4L.Many colleagues, especially those who operate in the practice, handle this somewhat differently. In some cases, they aspirate more volume and maintain a follow-up period of at least 12h before discharge. There are certainly many ways to combine safety and patient comfort here. Our experience has shown that inpatient treatment for larger volumes has many advantages. The biggest advantage is that you can be monitored clinically (see how you are doing) and accordingly you can be supported in all your needs. There can also be infusion treatments and checks on blood values. Often you will lose fluid through the small incisions for liposuction afterwards, which is often uncomfortable at home. If you remain in the clinic, you can leave the leaking tumescent solution in the hospital without having to deal with it at home. If complications arise, someone will be there to help you. Those affected 3 Treatment ofLipedemaAL GRAWANY130say it is relaxing when you don’t feel pressure to go home right after surgery. Likewise, follow-up is simplified during the first few days. We will see you every day without you having to be brought to or picked up from an office. Thus, we rec-ommend to treat liposuction from 3 to 4L inpatient for one night. The more volu-minous the liposuction is, the longer a stay should be planned, as the physical adjustment reactions then usually take a little longer. For liposuction of 8L or more of pure fat, we recommend a stay of 2–3days. From 10L it should be 3days or longer. It should be pointed out again that we are talking about pure lipoaspirate quantities. c For liposuction from 3 to 4L, patients should stay in the hospital for at least 12h.3.7 ConclusionIn summary, we consider the discussion of maximum liposuction amounts to be dif-ficult, as many factors must be taken into account. Experience with different tech-niques over a long time is also valuable here. When we say that we often suction fat volumes of much more than 10L, it does not mean that everyone can do it on every-one. It depends on the overall constellation. We think that a large volume liposuc-tion in a specialized center is associated with a lower risk than a “smaller liposuction” in untrained hands.From our experience, we can say that about 10% of liposuctions are performed with a volume below 4L of pure fat. In 50% of the patients, liposuction is per-formed with 4–8L and in 40% of the cases, liposuction is performed with more than 8L of pure fat volume. c The most common amounts aspirated for lipedema settle between 4 and 8L of pure fat per surgery. c Caution. Do not allow yourself to be “recruited” by colleagues with statements such as: “With us, there is no post-operative bleeding or hemorrhaging” or “Surgery without Hb drop.” These statements are not serious. Liposuction is always associated with risks.3.7.1 Requirements andPreparationsThe following tips and information apply in principle to all liposuction procedures. Nevertheless, it should be noted that, for example, liposuction with 500mL of suc-tioned fat on the abdomen has both a completely different course and different risk potential than liposuction of 12L of pure fat on the legs. A very small liposuction will have almost no impact on your well-being. You will pretty much certainly not have any dizziness, nor will you have any major bruising or significant blood loss. On the other hand, with very extensive liposuction, the wound areas created are Z. Jandali et al.131much larger. The anesthesia time is longer and the risk for complications is higher. Also, your recovery time after the procedure will be longer than after small, circum-scribed liposuction. If your tissue is very fibrotic (connective tissue remodeling), liposuction is often more traumatic.If you have morbid obesity and the treatment concept intends a combined treat-ment of obesity and lipedema, the obesity is usually treated first, by whatever options. Before the liposuction procedures, it would be helpful to reach the targeted weight as much as possible. Optimal would be a stable target weight for at least 6months, better 1year. This is certainly not a normal weight, as the lipedema com-ponent will not have disappeared.Even if weight loss fails, it does not mean that all options have been exhausted or that liposuction per se is not possible. A solution approach can be found for you. If you do not suffer from severe obesity, the issue is not relevant for you.For every operation, it is important to create the best possible starting conditions for an uncomplicated procedure and the subsequent healing process. All secondary diseases should be optimally controlled. If you suffer from secondary diseases, ask your attending physician how he or she assesses the condition of your secondary diseases and whether there are possibilities for further optimization. An example here would be a blood sugar disease (diabetes) and high blood pressure.No matter what body constitution you have: Exercise regularly and activate your body metabolism through physical activity. This will strengthen your heart and cir-culatory system in the long term. It is the same with your muscle balance. A healthy muscle balance for the phase during and after the operation is helpful. This allows the body to process the operation better, and the recovery phaseafter the operation is significantly shortened. In addition, the overall tissue perfusion is much better in physically fit people than in nonathletic ones. You can thus significantly reduce the risk of complications through your own initiative. Unfortunately, many people who are about to have an operation think: “Sport? That’s too late for me now. “ Be told that it is never too late for that. Even if you start exercising two to three times a week only 8 or 6weeks before surgery, it will have a positive effect on your treatment and psyche. c Before surgery, make sure to adjust all secondary diseases in the best possible way. Try to make your body fit for the operation by exercising.We have already discussed the topic of nutrition, and a balanced and healthy diet with an adequate supply of proteins, unsaturated fats, and important trace elements as well as vitamins is indispensable for a balanced metabolism in connection with an upcoming operation.Smoking should be absolutely stopped before surgery, especially before liposuc-tion! Smokers have much higher risks for the operation itself, but also for the course after the operation. It has been proven that smokers require higher doses of anesthet-ics and painkillers. Compared to nonsmokers, smokers have about six times the risk of pulmonary circulation complications after surgery. Examples would include pul-monary embolism or pneumonia. After surgery, smokers have about a three- to 3 Treatment ofLipedemaAL GRAWANY132sixfold higher risk of wound healing problems and wound infections. The risk of thrombosis is also substantially increased.Smokers suffer more often from diseases triggered by tobacco consumption such as cardiovascular diseases, respiratory diseases (e.g. chronic obstructive pulmonary disease, asthma), diabetes, and many others. All these diseases have a negative effect on the course after liposuction. Especially in the combination of liposuction plus smoking, the mentioned risk of thrombosis is a problem. Stopping smoking, the earlier the better, always has a positive effect on the operation, the recovery period, and the success of the operation (Fig.3.25).The immune system regenerates already within 4–6weeks. Bronchial secretions and lungs recover after 6–8weeks. Wound healing disorders occur less frequently if you stop smoking 4–6weeks before surgery. If you do not manage to stop smoking, you should at least radically reduce your tobacco consumption. Even if the effect is not equal to smoking cessation and is less effective, at least a small risk reduction for surgery can be achieved by doing so.Shortly before the operation (1–2days) to give up the glow stick, unfortunately, no longer really brings much, but is still better than to continue to smoke, because the oxygen supply in the tissue is measurably better in comparison. c Smoking cessation should definitely be attempted, preferably at least 8weeks prior to surgery.We already talked about the blood values and the hemoglobin level. In order for our body to produce functional red blood cells, it is necessary that our iron balance is balanced and that our iron reserves are well filled before an operation in order to be able to guarantee a problem-free new production of red blood cells. Now, is it a must to have a blood count check before any liposuction procedure and also check the iron storage levels? No, it is only a must if serious diseases are known. In some cases, we still recommend a blood count check, and in general there is nothing to be said against a check before a procedure if you want to be on the safe side.If necessary, check your hemoglobin value (the hematocrit is lowered in anemia) and pay attention to the serum ferritin value. This value shows us the degree of fill-ing of the iron store. If the iron balance is not balanced, it takes some time to IT’S NEVER TOO LATE TO TAKE THE FIRST STEP!STOP SMOKINGComplications12 hours4 weeks6-8 weeksoxygen supplyself-cleaningmechanism of thebronchiWound healingFig. 3.25 Stop smokingZ. Jandali et al.133replenish the iron stores. In this case we recommend a long-term intake of iron supplements with appropriate control 4–8weeks before surgery.As an important building block for building and maintaining red blood cells, consumption and replenishment of the iron balance takes place. If an operation is performed and blood is lost naturally during the procedure, this automatically leads to increased iron consumption in order to produce new blood cells. This can lead to a relevant supply bottleneck of red blood cells and thus to surgery-related anemia. Therefore, it makes sense to pay attention to one’s iron balance before surgery and to support it, for example, with iron-rich foods and, if necessary, with iron supple-ments from the health food store. We regularly recommend a herbal tonic with organically bound iron to our patients to prevent iron deficiency. c Talk to your family doctor; if necessary, a blood count check is recommended before liposuction. We recommend preventing iron deficiency with a herbal tonic and organically bound iron.At the time of surgery, it is important that you are healthy. You should not have a cold, diarrhea, or other illnesses. In case you fall ill before the operation, be fair to yourself and the surgeons and inform them in time. Otherwise, you are taking an incalculable risk, the consequences of which will affect you.You should also not suffer from a local infection. Unfortunately, this occurs more often, especially with chronically rubbing together skin soft tissues as in stage III or with sufferers of acne inversa. If such an “infected pimple,” open wound, or weep-ing redness is present shortly before or on the day of the operation, discuss it with your attending physician, even if the operation has to be postponed because of it. In principle, doctors do not do this to annoy you, but only for the sake of your health. An open wound or an acute abscess increases the risk of tissue infection, which does not have to be taken because this is not an emergency operation.If you are taking a cortisone preparation due to a lung, rheumatic or other illness, it should be clarified in advance whether this can be discontinued. Cortisone can impair and delay wound healing. Discuss all your medications with us, including homeopathic and nonprescription medications. Avoid alcohol and any form of drugs prior to liposuction.Do not take any blood-thinning medication before surgery. If you are dependent on blood thinning medication due to medical conditions, discuss this with your surgeon. We do not perform liposuction procedures while taking blood thinners. Some “headache pills” also contain blood thinners. Pay attention to this! c Do not take any blood-thinning medications or homeopathic remedies before liposuction.Please do not take homeopathic remedies before surgery. This also includes arnica. Although arnica has many positive properties, depending on the dosage, quite different effects result, and neither thrombosis through activation of clotting 3 Treatment ofLipedemaAL GRAWANY134nor “blood thinning” in the sense of a tendency to bleed is desired. Therefore, the use of homeopathic remedies should be postponed until the days after surgery.Before the procedure, already plan the time after the procedure. Do you have everything you need in the household? Is someone there for you after liposuction? Is everything ready so that you can easily get to things after the surgery? Do all the shopping in advance and maybe get some nice books or DVDs to pass the time.Whether outpatient or inpatient treatment, organize reliable transportation home. We have seen patients come to the clinic in their own car after very complex aspira-tions and assume that they will be able to drive home again. However, this is not the case. You should not drive a vehicle eitherafter extensive liposuctions or after cir-cumscribed, small liposuctions.To find out approximately how long you will be out of action, ask your practitio-ner in advance. Plan sufficient time for the downtime after the surgery. We give you a rough overview of downtime below, as we know it from our daily clinical routine. Of course, downtime is different for every patient and depends on how fit you are, how the surgery went, whether you tolerated the anesthesia well, etc. In addition, it depends on the profession and the activity in the profession. In Table3.4, we have the total downtime (TAZ), which is the time you should really rest. After that, you can return to light exertion and simple activities, but you should refrain from sports or physically demanding work.Despite this information, the courses can be very different. It is therefore better to plan a little more time than too little. If necessary, you should arrange with your employer for a sheltered job, that would certainly be a good option. If you have a family and children, this must also be organized.Do you normally wear compression stockings and are affected by lipedema? If so, consistent compression should be worn before surgery, especially if you also suffer from an edema component. c If you suffer from lipedema with an edema component, you should also wear your compression before liposuction.Remember to change your compression supplies. Usually, the hospital will give you compression garments that are meant to be worn for the first period after sur-gery. Alternatively, you can wear your old compression garments after surgery. Get heparin ointment (60,000IU) ahead of time to treat hematomas (bruises) after sur-gery. Rub the heparin ointment two to three times a day for 10–14days.After surgery, we recommend decongestant medications on a plant basis (homeo-pathics). For example, the active ingredient bromelanin has proven effective. This is Table 3.4 Average total downtime (TAZ) after liposuctionIntervention Number of exhausted liters TAZ in daysSmall outpatient liposuction 1–2 2–3Suction on the legs and abdomen Approx. 3 3–4Medium suction 5 7–14Very large extractions >8 >21Z. Jandali et al.135available under various product names. Discuss its use with your doctor and obtain the product as a capsule from the pharmacy before the operation.After liposuction, we recommend lymphatic drainage treatments. We will dis-cuss the details in the course, but you can organize the appointments in advance.If legs or arms are suctioned, then get enough pillows for elevation. Get old tow-els or pads for home to place under the suctioned areas, as fluid will continue to leak from the puncture sites for some time.What you Should Consider Before Liposuction 1. Most importantly, you should go into liposuction in the best possible health and absolutely fit. 2. All underlying and secondary diseases such as diabetes, high blood pres-sure, etc. should be optimally controlled. 3. Do sports and activate your body metabolism. Your own initiative is required here. 4. Stop smoking! Smokers have significantly higher risks during surgery. Especially during liposuction, the risk of thrombosis is increased by tobacco consumption. 5. Make sure you eat a balanced diet. 6. If necessary, have your iron balance and hemoglobin checked. Serum fer-ritin and hemoglobin levels should be noted. 7. You must be healthy at the time of surgery. If you fall ill, please cancel the surgery in time. 8. Discuss all of your medications with your practitioner. 9. Cortisone preparations should be paused if possible. Consult your practitioner. 10. Do not take any blood-thinning medications before surgery, including homeopathic remedies. 11. Avoid alcohol and drugs before liposuction. 12. Before the procedure, already plan the time after the procedure. Do you have everything you need in the household? Is someone there for you after the operation? 13. Arrange transportation to surgery and return home. 14. Plan enough time off (vacation). 15. If you want to return to work early, take care of a gentle workplace. 16. If you have children, organize a support. 17. If you normally wear compression stockings, wear them consistently before surgery. 18. Think about alternate supply. 19. We recommend the use of heparin ointment after surgery 2 to 3 times a day. 20. We recommend the use of decongestant medications, such as bromelain. 21. Organize appointments for lymphatic drainage well in advance. 22. If legs or arms are suctioned, get enough pillows to elevate them.Get old towels to place underneath at home. Fluid will continue to leak from the puncture sites for some time.3 Treatment ofLipedemaAL GRAWANY1363.7.2 Liposuction ProcedureIn summary, before liposuction, an introduction, examination, consultation, surgical explanation, and clarification of all administrative matters take place. The day before the surgery you should shower and put on fresh clothes. Do not put cream on your skin after showering, as we do our markings with a marking pen and it does not adhere well to creamed skin. Remove all earrings, piercings, and artificial nails. If you are having surgery in the pubic region, you should shave on the day of surgery. Arrive at surgery fasting, even if surgery is scheduled under a local anesthetic. Do not take any medications on your own that day.AnesthesiaLiposuction can be performed in different anesthesia procedures. Most often, lipo-suction is performed under local anesthesia alone or under local anesthesia in com-bination with so-called analgesia (also known as twilight sleep). In the case of analgosedation, the patient’s consciousness is dampened while being sedated and deprived of pain by medication. Analgesia does not involve intubation (insertion of a breathing tube into the airway) or mechanical ventilation. We prefer and recom-mend, whenever possible, local anesthesia in combination with twilight sleep. This combination is a very pleasant form of anesthesia for you, because you are virtually unaware of the whole procedure and yet do not have the risks of a general anes-thetic. In addition to local anesthesia and the combination with twilight sleep, lipo-suction is often performed under general anesthesia. c For lipedema, we usually recommend liposuction under general anesthesia.When it comes to the topic of lipedema and high-volume liposuction, we very often advise general anesthesia. It is undisputed that with general anesthesia the risk is increased compared to analgosedation and local anesthesia. However, in recent years, these risks of general anesthesia have been reduced more and more. In con-trast, comparatively more local anesthetic must be given for high-volume liposuc-tion under local anesthesia than under general anesthesia. This also increases the risks described above. Weighing up the advantages and disadvantages as well as the risks, general anesthesia is absolutely preferable to analgesia and local anesthesia for high-volume liposuction.Another aspect of liposuction is the positioning in the operation. Local anesthe-sia or analgosedation is an advantage for dynamic liposuction, where the position-ing in the surgery often has to be changed (e.g., side-side-back position). A long prone position for liposuction of the backs of the legs or torso is not quite as com-fortable under local anesthesia. Also, analgesia is rather impractical because the anesthesiologist does not have controlled access to the airways in this position. For this reason, we prefer to perform liposuction on lipedema patients under general anesthesia.Z. Jandali et al.137ProcedureSurely you are wondering how exactly liposuction is performed. Whether you are an outpatient or inpatient, you come to the clinic one day before or on the day of the operation. On the day of the operation, as with most plastic surgery· L. Jiga Department of Plastic, Aesthetic, Reconstructive and Hand Surgery, Evangelical Hospital Oldenburg, Oldenburg, Niedersachsen, Germanye-mail: dr@jandali.de1The LipedemaZaherJandali, BenediktMerwart, andLucianJiga1.1 IntroductionLipedema is recognized as a disease, yet it is trivialized by many colleagues, fre-quently being used as a “way out diagnosis,” according to the motto: “If we don’t find anything and you have thick legs, then you have lipedema.” Patients with lipedema suffer from a serious illness that is responsible for significant suffering. The complex clinical picture of lipedema cannot simply be reduced to painful legs and arms. It cannot be equated with “thick legs” or “thick arms,” nor can all thick legs and arms be attributed to lipedema. It is therefore important to make an accu-rate diagnosis clearly distinguishing lipedema from other diseases (Fig.1.1).Perhaps you are a patient yourself, with high hopes of being cured, reading this book to learn more about your disease. If so, you match the profile of the majority of patients addressing us for possible treatment. Unfortunately, after their first appointment, most of these patients are faced with the fact that such expectations are sometimes far from what is actually possible. Either social media or physicians often fuel such discrepancies. Particularly inexperienced colleagues allow them-selves, as a display of unsustainable knowledge “in front of the camera,” to bring the topic “lipedema” into the public eye with questionable advances just for the sake of media attention. Despite several existing claims on lipedema being a curable dis-ease, there is, unfortunately, no cure for it to date. However, with the right measures, the suffering can be sustainably alleviated and quality of life restored.We have deliberately chosen these provocative introductory words to draw atten-tion to the lack of acceptance of “lipedema” as a disease. If you really want to under-stand lipedema, you first have to find out the state of knowledge about this disease. Thus, we would first briefly discuss the history and origin of the term “lipedema.”AL GRAWANYhttp://crossmark.crossref.org/dialog/?doi=10.1007/978-3-030-86717-1_1&domain=pdfhttps://doi.org/10.1007/978-3-030-86717-1_1#DOImailto:dr@jandali.de2The Term “Lipedema”The term “lipedema” comes from the ancient Greek term “fat swelling.” It is com-posed of the two words λίπος, lípos, “fat” and the word οἴδημα, oídēma, “swell-ing.” Synonyms for the term lipedema include pillar leg, lipalgia, adipoalgia, lipo-fat disease, lipohypertrophia dolorosa, and adiposity dolorosa of the arms and legs. In addition to these synonyms, there are many other terms of the same category that should NOT be used either because having a different meaning or no meaning at all. The examples are lipohypertrophy, breeches syndrome, lipidosis, fat-leg syndrome, lipdem, fatty leg, hyperplasia dolorosa, lipohyperplasia dolo-rosa, zonal obesity, and others (Table1.1). Several of these terms will be explained in the book, as they are directly related to lipedema or should be distinguished from it.Fig. 1.1 Typical clinical picture of moderately pronounced lipedemaZ. Jandali et al.3 c We have long advocated abandoning the term lipedema and using the simple umbrella term “adipose tissue disease” with the sub-terms “lipalgia” or the term we introduced, “lipodolorosa (chronica).”History of the DiseaseLet us first look back at the history of the disease “lipedema” and what we have learned from the past. The first ones to describe lipedema were the physicians E.V.Allen and E.H.Hines in 1940 publishing the first scientific texts on the subject of “lipedema” in 1940, 1951, and 1952. Many scientific papers, websites and col-leagues still refer to the rather old first descriptions. We roughly summarize the studies below to reflect their core statements.The first of the three publications from 1940 is entitled “Lipedema of the legs: a syndrome characterized by fat legs and orthostatic edema” and describes a clinical syndrome that is often very distressing for those affected and could only be observed in women. Accordingly, the main complaints were swelling and an increase in adi-pose tissue volume and water retention in the area of the buttocks and legs. The swelling below the knee joint occurs predominantly when one is on one’s feet a lot or during warm weather. Pain in the legs is also common. Furthermore, the syn-drome is associated with a gradual increase in body weight. Unlike obesity, in which food intake exceeds the body’s caloric needs, the increase in subcutaneous fat only on the buttocks and legs is not easily explained. The edema of the affected persons results from a passage of fluid from the blood into the tissue. If there was a fat dis-tribution disorder in favor of the lower extremities without obesity, dietary measures would have no chance of success.The second publication from 1951 with the very similar title “Lipedema of the legs; a syndrome characterized by fat legs and edema” describes lipedema as a pro-gressive disease with orthostatic swelling of the legs. Compared to lymphedema, a decrease in leg swelling will not be facilitated by lying down. In this observational study,119 lipedema patients were presented, the observations from the first publica-tion from 1940 being confirmed.Only 1 year later, Hines published another article on lipedema with the title “Lipedema and physiologic edema.” Here, he continues to speak of adipose tissue proliferation and water accumulation in lipedema. What was new was the exclusion of the feet region in the description of fatty deposits or edema. The absence of edema in the feet was explained by tight-fitting shoes that could have prevented it.Table 1.1 Actual and apparent synonyms of lipedemaActual synonym Apparent synonymPainful column leg Fat legLipalgia Lipdema, lipidosisAdipoalgia, Adipoalgesia Fat-leg syndromeLipohypertrophia dolorosa LipohypertrophyObesity dolorosa Lipohyperplasia dolorosaPainful lipedema syndrome Hyperplasia dolorosaAdiposis dolorosa Zonal obesity1 The LipedemaAL GRAWANY4These three studies were therefore the “birth” of lipedema as a disease and are still quoted today. Many of the facts described here have retained their validity, but there are also some aspects that we now see differently. One example of this is the term “progressive” disease, which we discuss in more detail in Sect. 1.5. c Cited facts about lipedema often come from rather older studies and do not meet current scientific standards.Let’s get to one of the biggest misconceptions about lipedema:“Lipedema is a condition associated with lymphedema.”This statement is wrong and has been outdated for a long time. Regrettably, how-ever, this has yet to be understood by those affected, by the press and a significant number of scientific papers.Rare cases in which there a combination of lipedema and lymphedema can be diagnosed clinically make the exception to this claim. Moreover, the outdated dis-ease name suggests that it is edema, which in the true sense, it is not but rather pathologically distributed fat tissue. c The first three publications on lipedema are: c Allen EV, Hines EA (1940) Lipedema of the legs: a syndrome characterized by fat legs and orthostatic edema. Proc Staff Meet Mayo Clin. 15: 184–187. c Wold LE, Hines EA Jr., Allen EV (1951) Lipedema of the legs; a syndrome characterized by fat legs and edema. Ann Intern Med 34(5): 1243–1250. c Hines EA Jr. (1952) Lipedema and “Physiologic” Edema. Proc Staff Meet Mayo Clin 27(1): 7–9. c Lipedema is a real disease that must be taken seriously.1.2 Causes andEmergenceIn this section, among other things, we want to go on causal research and talk about the pathophysiology of lipedema. We will not only discuss our own knowledge but also go into those facts that are unfortunatelyoperations, it begins with a drawing and surgical planning (Fig.3.26).This is also the right time to carry out photo documentation so that a before-and- after comparison is possible if required. Another nursing admission is made, and then in the course of the day we go to the operating room.Depending on the treatment areas and the agreed form of anesthesia, either the induction of anesthesia and then the disinfection of your body takes place in the a1 a2 bFig. 3.26 Example drawing. A1, A2 Lipedema type III stage II; B Lipedema type IV stage II3 Treatment ofLipedemaAL GRAWANY138operating room or the other way around. First, you are washed or disinfected while standing and then the anesthesia or local anesthesia is induced.When and why does washing off (disinfection) take place while standing? Imagine that we are planning liposuction of the front and back of your legs (i.e., including the abdomen and back). The front and back of the legs as well as the abdo-men and back are to be disinfected. If you are already asleep, this is possible, but unnecessarily complicated and time-consuming. Therefore, many liposuction pro-cedures like to wash off while you are standing. Specifically, this means that you will be undressed and washed off or disinfected while standing before the anesthe-sia is administered. You will then lie down on an operating table covered with a sterile cloth. There you will be further covered with sterile drapes. Then the anes-thesia is induced or local anesthesia is applied—depending on what has been agreed.In some practices and clinics, local anesthesia is administered on a surgical couch using quite small needles. After the onset of the effect and before the surgery starts, washing off and covering are done. This is also a feasible way. We recom-mend performing liposuction in a proper operating room and not in an old converted living room or similar. Sterile conditions must be maintained. c We recommend performing liposuction in a real operating room.Let’s assume a classic procedure: You come into the operating room, anesthesia is induced, and then the hips, legs, and arms are washed off and covered with sterile drapes. Then it’s on to the operation itself. According to the agreement before the operation or according to the conditions in the operation, the approx. 5mm large accesses for the introduction of the special saline solution (tumescent solution) are placed. After an exposure time of about 20min, liposuction is started. In the first pass, the coarse fat pads are usually suctioned off, if they are present. This is done with relatively thick cannulas (5 and 6mm). After this, fine contouring is performed in several passes (4- and 3-mm cannulas). Whether waterjet-assisted or power- assisted liposuction is performed is irrelevant for the procedure (Figs. 3.27 and 3.28). After sufficient thinning and ensuring symmetry, the skin incisions are closed by suturing at the end of the operation (Figs.3.29 and 3.30).Fig. 3.27 Setting for liposuction with WAL and PALZ. Jandali et al.139Whether and how many punctures are closed varies from plastic surgeon to plastic surgeon. Some colleagues do not suture the puncture sites at all so that the remains of the saline solution introduced can drain out unhindered. We tend to suture some of the small incisions with dissolving suture material and leave others open. We are moving more and more toward inserting drains. We remove these as soon as the flow rate is less than 30mL over 24h. At the end of the operation, sometimes there is taping and application of the compression a bFig. 3.28 Suctioned thighs during (a) and at the end (b) of the operationFig. 3.29 At the end of liposuction without lower legFig. 3.30 After liposuction of the arms3 Treatment ofLipedemaAL GRAWANY140garment or wrapping for compression treatment. But some form of compression garment should usually be applied to help counteract swelling and bruising. To be fair, there are colleagues who do not use compression and do not have poor results. We always forgo compression when there is a contraindication, a reason why compression should not be applied. Examples are severely wrinkled skin with constrictions or allergic reactions to the material of the compression garment.3.7.3 AftercareAfter the operation, you will first be taken to the recovery room to wake up and from there to the ward. If liposuction was planned as an outpatient procedure, you can be driven home after rest and recovery. After surgery, fluid may leak from the small puncture sites. These may also be mixed with blood. The amount of fluid leakage should continuously recede and stop within the first 1–2days, if not please consult your physician. In the clinic, we keep absorbent pads for you in bed and change them as needed. We also recommend that you use such pads at home. On the Internet, you can find a number of different pads that are suitable for this purpose. Sometimes old towels are absolutely sufficient for this purpose, depending on the volume of fluid leakage.PainkillerThey are provided with painkillers according to a standard regimen (after querying allergies and intolerances) and receive further adjusted doses of painkillers as needed. Often, NSAIDs, so-called nonsteroidal anti-inflammatory drugs, are suffi-cient for pain treatment; a well-known active ingredient is ibuprofen. When taking NSAIDs, it is important to have stomach protection, such as omeprazole. For dos-age and intake, ask your doctor or pharmacist. Affected persons who have had meta-bolic surgery are often not allowed to take NSAIDs and must switch to other medications. But let me tell you: Liposuction is usually not very painful.On the Day of the Surgery 1. Shower extensively before the operation and put on fresh clothes. This can reduce body contamination with bacteria. 2. Do not apply cream after showering, the marking pen will not hold as well. 3. Refrain from wearing makeup, nail polish, and piercings. 4. Please remove all artificial nails before surgery. 5. In case of surgery on the intimate area, we ask you to shave the area. 6. Do not take sedatives on your own. 7. Discuss in advance which medications you can and cannot take on the day of surgery. 8. Come fasting on the day of surgery (depending on the type of anesthesia). 9. Wear comfortable, loose-fitting clothing on the day of surgery. Make sure that you can easily take off and put on the clothes.Z. Jandali et al.141Fluid BalanceYou may get up as soon as you feel like it after liposuction. For the first time, please call a specialist nurse, because there may be circulation problems after liposuction. It is important to drink plenty of fluids after liposuction to help your circulation. This is because the body first fills the suctioned cavities with fluid. In professional circles we call this “volume redistribution.” We try to avoid infusions after the pro-cedure as much as possible. c Drink as much as possible in the first days after liposuction. c The shift of fluid into the tissues occurs despite the compression garment, which is designed to counteract swelling.If you do not drink enough, then water may be removed from the blood, it simply thickens. If the swelling of the leg is added to this, the blood flow back to the heart is inhibited by a squeezing of the blood vessels. In combination with an insufficiently functioning muscle pump of the lower legs due to too little move-ment (immobilization), the risk of deep vein thrombosis with the danger of developing a pulmonary embolism increases. By drinking and, if necessary, infu-sions, we can help the fluid balance—the only thing missing is the support of the muscle pump in the lower legs. You can support this with your own exercises by moving your feet to the tip of your nose while lying down and then stretch-ing them.To counteract thrombosis, you willbe given a drug in syringe form for thrombo-sis prophylaxis. It is usually a low-molecular-weight heparin preparation that is injected once daily. You will receive the first injection on the day of surgery. The injection is given into the subcutaneous fat tissue and into an area that has not been suctioned. In the case of liposuction of the legs, it is recommended to inject into the subcutaneous fatty tissue of the legs and vice versa. We advise performing thrombo-sis prophylaxis for at least 1week. Please discuss this with your attending physi-cian. Iron supplements should be continued, if necessary until the iron balance has normalized.If the legs have been suctioned, it is advisable to keep the legs elevated (e.g., on a pillow) at the beginning consistently and then later in the course in between, so that the swelling and bruising can be transported away to the body more easily. A flexion in the knee joints of more than 80° is counterproductive for the venous return of the blood to the heart. A bend in the knees of about 45° is ideal. This can be achieved well with one or more pillows on the sofa or bed. c Keep elevating the legs/arms every now and then to counteract the swelling.Lymphatic DrainageWe recommend starting early with lymphatic drainage. Lymphatic drainage is a manual treatment in which excess tissue water is massaged out of the tissue. Usually, lymphatic drainage is offered and performed by physiotherapists, occupational 3 Treatment ofLipedemaAL GRAWANY142therapists, or lymphatic therapists. This can be started early after 2–3days. It is important that the lymphatic drainage is painless and that you do not have any cir-culatory problems. If you feel pain during lymphatic drainage, you should give the internal wounds a few more days to recover and try a new attempt with lymphatic drainage a few days later. One hour of lymphatic drainage costs between 45 and 60 euros, depending on the region. Ideally, lymphatic drainage should be repeated two to three times a week and continued until most of the swelling has subsided. In case of extreme lipedema, it is recommended to start immediately and to perform it every day. In general, lymphatic drainage is not obligatory after liposuction, but the swell-ing goes down faster with lymphatic drainage.BruisesThe bruises disappear within 2–3weeks, although a slight blue tinge may still be visible. This completely recedes in the following 2–3weeks. The initial hardening of the suctioned areas slowly dissolves and should be completely gone after 4–6months at the latest. Massaging the bruises 1 to 2 times a day, carefully at first with heparin ointment (60,000IU ointment), helps the bruises to dissolve quickly, reduces the swelling, and dissolves the scars that form early. In our opinion, the massage is more important than the ointment. To further support the reduction of swelling, we recommend taking bromelain. Bromelain is extracted from pineapple, it has a decongestant and anti-inflammatory effect.There is controversy about the necessity of antibiotic treatment in terms of pro-phylaxis, although science considers a single administration to be useful. Depending on the extent of the operation, we continue prophylactic antibiotics for a few days (1–3) in case of extensive liposuction.Compression UnderwearAfter the compression garment is applied in the surgery, it should be worn consis-tently for 23h a day for at least 8weeks. Swelling after liposuction increases in the first 2 to 3days and has its maximum extension about the third day. After that, the swelling process begins, at first quickly, then more and more slowly. Most of the swelling is reduced after 6–8weeks. Any remaining residual swelling then subsides from the third to the sixth month. If the cost of the surgery is covered by health insurance, you may be given a prescription for a second supply of compres-sion garments. Otherwise, a second suit can be purchased at a medical supply store. A control of the suctioned areas is performed as needed. We check them on the first or second day after the operation, depending on the extent of the liposuction. c The compression garment should be worn for 23h a day.The First ShowerA frequent question is: “When can I start showering again?” Here there are quite different statements and opinions in professional circles. We recommend the first Z. Jandali et al.143shower after 2–3days, but depending on how you feel, it is also possible earlier. The following things should be taken into account: When you want to take off the com-pression garment for the first time, for example, for showering, we recommend that you do it in a lying position (bed, sofa, or similar). Beforehand, you should have eaten something light and small and drunk enough throughout the entire period. Furthermore, it is important that a person helps you or is at least in the immediate vicinity, as opening the compression garment can cause circulatory weakness. Likewise, increased fluid may enter the connective tissue of the suctioned areas, resulting in a volume shift in your fluid balance. Dizziness, “black before the eyes” and/or nausea may occur. Therefore, it is good to have someone by your side to help you if needed.After removing the compression suit, you should first sit carefully on the edge of the bed and wait briefly so that the circulation can adapt to this position. After a few minutes, you then move to a standing position. Before you start walking directly, stand still for a short time and again wait for your body’s adaptation reaction. Only start walking when your body gives you the “ok” signal.Keep showers very short in the first few days and do not shower too hot, as this can also hit the circulation. Showering may be done with shampoo and shower gel before quickly exiting the shower. Dry off and put on a fresh change of clothes. The old suit may be washed in the meantime. We have often heard that some people shower with their suits on, but we do not recommend this. For showering in the first days, shower plasters can be stuck on the wounds. After showering, the shower plasters should be removed.Follow-Up ChecksAfter discharge from the hospital, further treatment will take place on an outpatient basis with your plastic surgeon. Depending on the surgery and length of inpatient stay, you should initially see your doctor weekly or as needed. After the third week, we recommend longer intervals between check-ups—every 2–3weeks if still neces-sary, then after 8weeks and after 6–9months for the final checkup. If you have any problems, questions, or complaints, we recommend seeing your surgeon promptly. You should not carry any worries with you. Your small wounds are usually covered with a band-aid. Change the plasters every 1–2days at first. Once the wounds are closed, you will not need any more plasters.MovementEarly mobilization is important to get the circulation going. You should also gradu-ally increase light physical activity. Our motto for this is: move yes, strain no. You should refrain from swimming for at least 4–6weeks if you have extensive suction. Light sporting activities such as walking are possible after 2–3weeks. Excessive sports should also wait 4–6weeks.Depending on the occupational activity and the amount extracted, work can be resumed after a few days or weeks. In the case of extensive suctioning, you will need at least 3–4weeks to return to your daily work routine.3 Treatment ofLipedemaAL GRAWANY144ScarsDepending on the surgeon, the small incisions (skin punctures) may or may not have been sutured. If they have been sutured, the suture material may be self- dissolving or non-self-dissolving. If the suture material is non-self-dissolving, the sutures must be removed after 10–12days. Talk to your doctor about this.The calling card of a plastic surgeon is the scar that remains in the area of visibleskin after each surgical procedure. In liposuction, generally only very small skin incisions are made. However, not all of them are usually closed, which can leave widened scars. Scars can be very different. Ideally, a scar is inconspicuous, flat, pale, fine, soft, and painless. In the opposite case, the scar may be conspicuous, raised, reddened, widened, hardened, and painful. Weather sensitivity is also a typi-cal scar complaint. In addition to uncomplicated scar healing or scar maturation with a desired beautiful scar, we distinguish pathological forms of scars, which are called “hypertrophic” or “keloid” scars.The following factors can influence scar healing: – Individual congenital disposition of the scar healing process. – Localization and positioning of the scar. – Technique of suturing the wound or leaving it open. – Tension of the scar during wound closure. – Suture material. – Immobilization of the scar for healing. – Wound care and condition. – Compression and silicone pads. – Sun exposure. – Scar massage.One of the best and most effective means of scar treatment (by scar is meant the wound suture after the last crust has fallen off) is treatment with silicone and com-pression. Silicone has been tested in many studies as an effective means of prevent-ing and treating scarring.The mechanism of action has not yet been conclusively clarified. One important mechanism of action appears to be water vapor transmission (water vapor release). Others suspect a change in the charge in the scar. Normal skin has a transmission of about 8.5g/m2 of water vapor per hour. Hypertrophic scars and poorly healing scars have a much lower transmission of about 4.5g/m2. The silicone (foil or gel) creates a seal, which positively influences the water vapor emission of the scar. Silicone gel is applied thinly one to two times a day. Silicone foils remain on the scars under compression for at least 6months to 2years. The silicone film should be washed off daily with normal hand soap, and the silicone overlay must be boiled for 5min once a week. Good silicone products have a very long shelf life. c As long as a scar is still reddened and raised, the scar is in the active maturation phase and is organizing itself. In this phase, the scar can still be treated very well with conservative measures such as silicone overlay and compression.Z. Jandali et al.145Protect your scar from sun rays. UV rays can cause pigmentation disorders and also hypertrophic scars. Therefore, we recommend avoiding sun radiation or apply-ing sunscreen with at least factor 30 to the scar region. The sunscreen should be applied for at least 1year.Active scar massage can prevent unsightly scarring. After initial healing of the scar, daily scar massage can make the scar softer and smoother. Massage improves blood circulation to the scar. This positively affects the fluid metabolism of the scar and the collagen of the scar becomes softer, elastic, and flexible, lowering the initial raised level of the scar.We recommend you three massage directions: circular, horizontal, and vertical massage. It is possible to use a moisturizing lotion to improve lubrication. Likewise, it is possible to massage the silicone gel while applying it. In doing so, bring two to three fingers together and perform circular movements with light pressure on the scar in the circular massage. In the horizontal method, you massage the scar from one end to the other. In the vertical massage, light up and down movements are used. You should alternate between these different techniques during the massage.Aftercare after Liposuction 1. At home, use absorbent pads or old towels to soak up any leaking tumes-cent solution. 2. You will receive an adapted pain therapy. 3. The first time you get up (with the compression on), it is best to do it accompanied as follows: From lying down, you first move to sitting and remain seated at the edge of the bed for a short time. Only when you can cope well with this do you stand up carefully and remain standing in front of the bed for a moment. Then you can take your first steps. 4. Drink enough fluids. 5. Perform daily thromboprophylaxis for at least 1week. 6. Perform thromboprophylaxis exercises in bed and standing. 7. Continue taking iron supplements after surgery until your blood levels stabilize. 8. If legs or arms have been suctioned, place them high (preferably on pil-lows) after liposuction. 9. Lymphatic drainage is allowed immediately after surgery but is usually tolerable from the fourth to seventh day. Lymphatic drainage should be painless. 10. Massage bruises with heparin ointment (60,000IU) daily in the morning and evening, for which they briefly remove the compression garment. 11. We recommend taking decongestant medication, for example, with the active ingredient bromelain. 12. It is usually not necessary to take antibiotics after surgery.3 Treatment ofLipedemaAL GRAWANY1463.7.4 Success andLong-Term ProspectsAll sufferers are interested in the lasting effect of liposuction. But is liposuction for lipedema really sustainable? There is no general answer to this question. As almost always, it depends on the initial situation. If there is pronounced obesity at the beginning of treatment, which is not treated, and there is no treatment plan for obe-sity, then liposuction is unlikely to be crowned with long-term success. It is exactly these patients who report in the aftermath that immediately after the surgery it starts to “proliferate” on the abdomen or in other regions. However, it does not proliferate, the excess calories are deposited differently than before the operation due to the changed fat cell count and fat precursor cell count and their distribution.In cases of only mild obesity involving fat distribution disorder and nonobese sufferers, a simple lifestyle and dietary change combined with liposuction as defined by our complex surgical treatment may be the key to long-term success.There is almost always a significant improvement or even a complete decrease in pain after treatment. However, depending on whom you ask, different opinions pre-vail about the effectiveness of liposuction, especially in the long-term course. Freedom from complaints after liposuction is stated quite differently in various studies from 100 to 70 to 20%. No study stands out qualitatively enough to say that this is the study that can be trusted. 13. Consistently wear your compression garment 23h a day if possible. In case of constriction or discomfort, it should be taken off. In such a case, consult your doctor. We recommend wearing the garment for at least 8weeks. 14. A control of the suctioned areas is carried out as required and agreed upon. 15. If you were discharged with a drain, you will need to see your practitioner or physician closely until it is removed. 16. The first time you take off the compression completely (usually after 1–3days) and then want to stand up, take off the compression while lying down, and then proceed as for the first time standing up. 17. You may shower after the operation. Due to hygienic principles, never shower with the compression on. This must be removed beforehand. Shower for the first time with a companion. We recommend shower plas-ters at the beginning. 18. Change the plasters every 1–2days. Once the wounds are closed, you no longer need plasters. 19. Exercise yes, strain no! As soon as it is possible, you should mobilize and move a lot; however, strain should be avoided. 20. In case of severe pain (especially in the calf region), shortness of breath, or shortness of breath, immediately present yourself to us or the attending physician. 21. Use silicone gel for scar treatment and massage your scar. 22. Protect your scars from harmful sunlight with UV protection.Z. Jandali et al.147From our point of view, liposuction for lipedema oreven for the existence of pure lipohypertrophy has a really very good prognosis to be long-term and sustainable. With successful treatment, one certainly improves first of all the physical com-plaints including the mobility and the ability to bear weight, but there is also a sig-nificant improvement of the body shape. The improved body shape almost always leads to mental stabilization, an increase in self-confidence, and more participation in social and working life. To answer the question of sustainability: the indication and the therapy plan must be right. c When properly indicated, the results of liposuction are usually long- term and sustainable.If there is a complete reduction in the symptoms of lipedema as a result of weight loss, conservative therapy or complex surgical treatment alone, many people speak of lipedema in remission. Remission because those affected continue to carry the predisposition to lipedema. It can therefore never be ruled out that removed fatty tissue will come back in the same place and lead to renewed lipedema symptoms. No one can guarantee success.More often than a recurrence or worsening of lipedema, we are told by affected women that they could really lose weight for the first time after liposuction. We do not know in detail what is responsible for this, but certainly, the breaking of the hormonal regulatory circuit of the adipose tissue plays a major role. Likewise, intrinsic, newfound motivation certainly plays an important role. Liposuction removes not rigid but hormone-active adipose tissue.The success of liposuction varies depending on the region. We have some regions that we refer to internally as “problem regions “ and “difficult to suction.” The front of the knee (Fig.3.31) and lower leg are particularly worthy of mention here.The problem with knee joints is that the excess fat in this region is highly sep-tated. These septa make liposuction enormously difficult. Furthermore, there is often connective tissue fibrosis in this area. However, there are two additional fac-tors: (1) the skin recedes more poorly in this area than in other regions and (2.) the remaining excess skin overlaps the knee joint due to the firm connective tissue fibers around the knee joint.Another “problem region” is the front and back of the lower leg. Due to the fre-quently advanced fibrosis here as well, the tissue cannot always be removed as desired and residual tissue swelling remains (Fig.3.32).The last problem region is the waist–abdomen–hip transition. In particular, if only the hips and extremities are suctioned without shaping the waist and abdominal region, a harmonious transition is often not possible. However, this is purely an aesthetic aspect.Often women affected by lipedema also suffer from cellulite (please do not con-fuse it with cellulitis! Cellulitis, in contrast to cellulite, is a bacterial inflammation of the subcutaneous connective tissue). Cellulite is caused by the female sex hor-mone estrogen and appears as so-called orange peel skin with dents and waves, especially on the buttocks and hips. Cellulite in itself is not a disease and does not cause pain on its own.3 Treatment ofLipedemaAL GRAWANY148As we read in Sect. 1.2.1, the subcutaneous fat tissue is separated from each other by connective tissue fibers (septa). In contrast to men, the septa are arranged differently in women. In women, the rather fine connective tissue septa run perpen-dicular to the skin surface, whereas in men they run parallel and crosswise. This makes men’s skin more robust and stable, while women’s skin is softer and more flexible, which is advantageous during pregnancy, for example. Overall, however, this makes women’s skin less resistant to the underlying fatty tissue.As a result, the fatty tissue can push up to the upper layers of the skin and cause bulging. Liposuction results in several effects on cellulite, which can be treated positively in the long term. The causing fat pads are removed and the connective tissue fibers are slightly injured. This leads to scarring and stabilization of the fat pads. These two effects lead to a regular improvement of the skin appearance after liposuction (Fig.3.33). We do not show before and after pictures due to the law on Fig. 3.31 Problem zone knee joint regionZ. Jandali et al.149Fig. 3.32 Problem zone lower legFig. 3.33 Results of liposuction for lipedema3 Treatment ofLipedemaAL GRAWANY150advertising of medical products. However, these can be shown to you during a per-sonal consultation with your doctor.3.7.5 Consequences andRisksWe have already talked about the positive aspects of liposuction. But what about the consequences and risks? Depending on how much fatty tissue is suctioned out, there is a risk of tissue slackening, that is, sagging of the skin. This is where our skin plays a central role. The skin is our largest human, organ and, in addition to its barrier function against environmental influences, has many other functions, such as tem-perature and water balance regulation. The skin consists of different layers, roughly subdivided, the epidermis and the dermis or corium (Fig.3.34).Simplified, the structure of the skin (Fig.3.34) can be imagined as many indi-vidual skin cells, which individually look like a brick, lying next to and on top of each other like a brick facade. The cells grow from the depths toward the surface, undergo a maturation process, and are shed on the surface as skin scales.The basis of skin elasticity is collagen fibers and elastin. These are also known as fibrous protein and are located in the dermis (deep layer of skin), run parallel to the surface of the body, and together form a matrix (network compound). The combina-tion of elastin and collagen results in the elasticity and tear resistance of the tissue. However, this elasticity and tensile strength are finite and therefore limited. If this matrix is overstretched and destroyed, the stretch marks we are all familiar with will appear. In technical jargon, these are also called “striae distensae.” Externally, these areas of overstretching appear as blue-reddish stripes. The coloration is the result of an on-site inflammatory reaction caused by the tearing. The skin at the tearing site becomes thinner than in other areas so that the small skin vessels show through and are responsible for a change in color in this region. To some extent, these streaks fade over time, but the overused skin has lost the ability to regress. After illustrating Architecture of the skineqidermisdermisconnective tissuefatmuscleblood vesselfat fasciamuscle fasciaFig. 3.34 Structure of the skinZ. Jandali et al.151this mechanism of tearing, it also becomes understandable that a cream or lotion cannot really help repair the complex.If volume removal occurs in the subcutaneous fat tissue, the outer mantle (the skin) must adapt to the new volume. The extent to which your skin recedes is very individual. Here, the inherited skin quality with its regression property certainly plays a very central role. Surprisingly, in some patients, the skin recedes massively and very well (in Fig.3.35, we show a good skin shrinkage result after liposuction of many liters on the legs), in others even a small loss of volume is sufficient for the formation of sagging skin.You may wonder whether there are any measures that can positively influence the desired skin shrinkage process through external factors. Exercise and nutrition play a central role here. Exercise promotes tissue circulation and supports the natural metabolic balance in the long term. Likewise, a balanced diet rich in vitamins is an essential basic building block for the regeneration of skin and tissue. Fig. 3.35 Result after liposuction3 Treatment ofLipedemaAL GRAWANY152You should avoid smoking at all costs. Smoking causes the skin to age faster andreduces the skin’s metabolism. Likewise, excessive sun exposure is harmful to your skin. Massaging the skin with oils and creams rich in vitamins (e.g., vitamins A, B3, C, E, and H) is quite useful. However, we see the positive effect more in the local massage and increase in tissue circulation than through the active ingredients per se.What can we say in conclusion about the probability of skin shrinkage after lipo-suction for lipedema? Depending on the type of skin and the region suctioned, the skin behaves differently and therefore recedes differently well, but it is not possible to predict with certainty what it will actually be like against the background of the above-mentioned reasons. c The elasticity of the skin is finite. After reaching the elasticity limit, stretch marks, wrinkles, and sagging skin develop. Each region of the body reacts differently.In general, the inner thighs quickly begin to sag. In contrast, the outer sides of the hips and upper arms compensate for sagging quite well, up to a certain extent.It is not always easy to strike a balance between residual fat, hanging skin, and aesthetics, although in lipedema we recommend and usually perform maximum fat reduction because of the existing pain (Fig.3.36).Fig. 3.36 Sagging skin on abdomen and legs after liposuction of 30L of pure adipose tissue on legs, abdomen, arms, and back over three sessionsZ. Jandali et al.153Why are we even talking about the issue of sagging skin? Sagging and wrinkled skin can also cause problems. Skin irritation and restricted movement are the most frequently expressed and seen complaints. Areas of skin that lie on top of each other cause heat to build up and, in the absence of air circulation, a moist chamber is formed, disrupting the natural skin barrier. In the worst case, redness or sores can be the result.Excess “skin flaps,” especially on the upper arms and thighs, can restrict natural movement in everyday life and especially during sports. The excess lags behind the arm and leg movement due to its inherent inertia.None of those affected likes to undress in front of others and “present” their excess skin. This results in the exclusion of many activities, for example, swim-ming, sunbathing on the beach, or even just wearing short fashionable clothes. Likewise, in many sufferers, the partnership suffers and not in a small way. We speak of social isolation, psychological stress, and withdrawal from the social envi-ronment with isolation.Important: You should talk about possible tissue sagging after surgery with your doctor before liposuction. Most plastic surgeons address this topic on their own initiative, while other groups of doctors, who do not perform lifting operations themselves, sometimes like to leave this topic out. Don’t be fobbed off with phrases like “it almost never happens” or “we’ll talk about it then.”In patients who are fairly certain to experience skin sagging, we already include these tightening operations in our initial therapy plan to create transparency. Overall, although we perform tightening surgeries very frequently, they are rather rare in lipedema patients compared to patients after massive weight loss due to gastric surgery. We include the group of extremely obese lipedema patients in the group of patients after massive weight loss. Even if lipedema is still worth treating after weight loss, skin slackening due to the mass is still in the foreground.Let’s move on to the risks. Every surgeon who operates also has complications. If he doesn’t have any, he doesn’t operate or he fibs. Good complication manage-ment is an important quality feature of a good specialist.The so-called clarification meeting must take place at least 24hours before the operation. Even if you have signed this clarification, you can withdraw from the operation at any time without stating a reason. As a rule, you must not incur any costs by canceling the operation, regardless of whether the costs of the operation are borne by the health insurance company or by yourself.The risks listed here are only an excerpt of possible surgical risks. Your attending physician must discuss any special risks you may have in detail with you before the operation.First of all, we will go into very general risks. Every operation that involves anesthesia is associated with an anesthetic risk. This will be explained to you in detail by the anesthesiologist during the anesthesia consultation.General surgical risks are as follows: Despite the greatest care, blood vessels can be injured during liposuction. Some injuries can never be avoided, but they should be as minimal as possible. After the procedure, there is always further fluid leakage through the puncture sites. Often the fluid is diluted with blood. If there is more 3 Treatment ofLipedemaAL GRAWANY154bleeding after the operation, they form larger and extensive bruises. As described in Section 0, postoperative bleeding and/or thinning of the blood may necessitate a blood transfusion. In our case, it happens about once per year that a blood transfu-sion is necessary, despite very high to extremely high lipoaspirate volumes.A distinction must be made between how extensive and how voluminous liposuc-tion is performed. Let us consider the following actual scenario with two different liposuction procedures: Patient 1, normal weight with very mild visually pronounced lipedema but severe pain, Patient 2 with extreme lipedema, mild obesity, and moderate pain. Patient 1 receives liposuction on her arms, legs, front and back, that is, very extensive liposuction with a suction volume of 3.5L of pure fatty tissue. Patient 2 receives liposuction only on the front of the legs (11.8L of pure adipose tissue). Of course, the second patient has significantly more fat deposits than the other, and one would theoretically have to list further details such as body size, general condition, and many more, at this point, but we will refrain from doing so here. In short, patient 1 (3.5L) experienced greater blood loss due to areal hemorrhage combined with greater dilution. The consequences were dizziness, ringing in the ears, headache, nausea, pal-pitations, and general weakness. Hb was 6mg/dl and hematocrit was 20%. A blood transfusion was necessary. The subject was quite weak on her feet for 1week; the other got up on the day of surgery, had a perfect course, and went home 2days later. This means that it often depends on the extent of liposuction and not on the amount. c The volume aspirated alone does not reflect the risk.Despite high-quality controls of blood products, a blood transfusion is associated with the risk, albeit very low, of transmission of infectious diseases. Other risks of blood transfusion include allergic shock reactions, chills, headaches, muscle cramps, fever, and fatigue. Autologous blood donation and subsequent administration of autologous blood are no longer performed. We consider the risk of blood transfu-sion during liposuction to be very low.In addition to extensive hemorrhage, we distinguish acute local hemorrhage. This can happen when a larger blood vessel is injured. In my entire career, I have experienced this one time. In the affected person, liposuction for the planned upper arm lift was performed in one session. Through the tube, we could see a clear unusual blood leakage and then found an injured blood vessel through the incision of the upper arm lift. Even if no lift had been planned, we would have had to make an incision to stop the bleeding.Bruising occurs in most liposuction procedures, that is a fact. Other risks are dents and waves in the sense of irregularities of the suctioned areas. These may also require correction if severe. The risk of dents and ripples is greater in very volumi-nous liposuction procedures than in others.Liposuction can theoretically injure lymphatic vessels, especially if sharp can-nulas are used. I personally have not seen any liposuction-induced lymphedema—neither in our lymphedema consultation nor in our lipedema consultation.Occasionally, there is circumscribed loss of sensation due to injury to small cuta-neous nerves. These sensory losses usually regenerate slowly over 2years.Z. Jandali et al.155Extremely rarely do we see infections or healing disorders of the wound and even more rarely they spread. In the worst case, these could develop into blood poisoning. This would be a serious complication requiring inpatient or even inten-sive care treatment.Circumscribed or local wound healing disorders are usually treated conserva-tively, that is, without surgery. Treatment includes regular wound cleansing, appli-cation of disinfecting ointment, and application of clean dressings. An example of disinfecting ointment is Lavanid gel. However, the “brand” is not decisive, but the ingredient should be polyhexanide 0.04% or 0.02%. In case of infection or wound healing disorder, an antibiotic in tablet form may be recommended. You should fol-low this recommendation without fail. c If there is a wound-healing problem, see your plastic surgeon and discuss wound treatment.A complication that we see more frequently with very extensive liposuction and almost exclusively in the area of the outer thighs isaccumulation of wound water, so-called seromas. Often the problem resolves itself, but sometimes we have to puncture the seromas in the consultation. Occasionally we insert drains. Up to now, we have always managed to get all seromas under control. To be able to answer the legitimate question as to why the seromas only occur on the outer sides of the thighs, we must first look at Fig.3.37. Here you can see that exactly in the outer thigh region there are no lymphatic channels and therefore no lymphatic collectors. Therefore, tissue water that accumulates there can be poorly removed. An accumu-lation of tissue water (seroma) can therefore develop preferentially in this region.With every operation, there is a risk of suffering a thrombosis or pulmonary embolism during or after the operation. In thrombosis, the blood thickens after sur-gery due to the loss of blood or fluid. This causes local swelling that squeezes blood vessels and reduces blood flow back to the heart. Blood vessel injury and activation of the body’s clotting system also play a role in the development of thrombosis. In addition, being bedridden causes the circulatory system to shut down. This and other reasons are responsible for the development of thrombosis. If thrombosis occurs, it is usually in the veins of the lower leg. From there, a thrombus can detach from the vessel and travel via the heart to the lungs. The migration of the thrombus to the lungs occurs via the pulmonary artery, hence the name “pulmonary artery embolism.” Once in the lung, the thrombus—depending on whether it blocks a large or small vessel—cuts off that area of the lung from its function of supplying the blood with oxygen. A pulmonary embolism is an emergency that requires intensive medical care and, in the worst case, can result in death. To prevent thrombosis, you should drink enough to counteract blood thickening.Another important goal is to improve venous return to the heart. This is possible via the following physical measures: – Compression suit/compression stockings. – Wrap dressing. – Pneumatic leg cuffs.3 Treatment ofLipedemaAL GRAWANY156 – Legs up. – Early mobilization. – Exercise therapy, such as bed exercises.You will be put on the compression suit after the surgery in the operating room, sometimes with an additional wrapping bandage. In bed, your legs should be ele-vated. After the operation, it is important that you mobilize as soon as possible. As soon as you can sit at the edge of the bed without any problems, they should move to standing as early as possible, first under escort and then alone. Stop short of the bed and listen to your body. It will tell you if you can already take your first steps, first at room level and then at ward level. Walking is of immense importance, as it Fig. 3.37 SeromesZ. Jandali et al.157not only stimulates circulation but also activates the muscle pump in the calves. The muscle pump in the calves supports a return flow of blood from the legs to the heart.But also movement exercises in bed help to prevent thrombosis. For this purpose, we would like to give you two exercises that you can do alone in bed.Exercise number 1 (Fig.3.38): You lie relaxed in bed with your arms placed loosely beside your body. Your legs are stretched out in bed and the tips of your toes point upwards. Now bend and stretch your toes as shown in Fig. 3.38 15 times.Exercise number 2 (Fig.3.39): As soon as you can sit, sit on a chair and place your feet on the floor. Press the heels of both feet firmly on the floor and lift the tops of the feet vigorously. Keep the tips of your toes up briefly. Then roll over the tips of your toes with pressure. Repeat this exercise 15 times as well. Try to do the exer-cises several times a day.Fig. 3.38 Exercise for thrombosis prophylaxisSummary thrombosis prophylaxis – Take in sufficient fluids (drinking, infusions). – Movement and exercises for the muscles of the lower legs. – Prophylaxis injection for at least 4days. – Compression garments.3 Treatment ofLipedemaAL GRAWANY158Fig. 3.39 Exercise for thrombosis prophylaxisSymptoms of Possible Thrombosis – Increasing swelling of the legs with a feeling of heaviness and/or tension. – Pain in the leg, often especially in the lower legs in the area of the calves (pressure pain). – Overheating or blue discoloration of the skin. c If you have symptoms that make you suspect you may have a thrombosis, please present to your doctor or an emergency room immediately.A thrombosis can develop into a pulmonary embolism with far-reaching consequences, such as intensive medical treatment or, in the worst case, death. A patient dying during liposuction is an extremely rare complication, and we Z. Jandali et al.159Fig. 3.40 Triflow exercise: Inserting the mouthpiecedon’t want to scare you. However, the rare complications must also be addressed, because no one can rule out a complication in advance.“Take a deep breath” is the motto. After a procedure, pain can be respon-sible for your inability to breathe properly. This can lead to lung ventilation problems or, even worse, pneumonia. But not only deep breathing, but also exercises with the so-called Triflow help to ventilate the lungs well. c Ask the hospital for a Triflow to do your breathing exercises.To perform the lung ventilation exercise with the Triflow, place the mouth-piece of the Triflow in your mouth (Fig.3.40), seal the mouthpiece with your lips and inhale so deeply that the balls slowly rise up one after the other (Fig.3.41). It is better to hold only one or two balls up for as long as possible than to hastily try to pull all three balls up. It is best to repeat this exercise every hour, trying to pull the balls up more than 5 times. Rotate the Triflow and exhale. Likewise, try to keep all the balls up as long as possible. c If you have trouble breathing or any of the symptoms listed in the overview, please present to your doctor or an emergency room immediately.This was an excerpt of possible complications and risks. Before surgery, a detailed discussion will take place in which you will be informed about these and other risks.3 Treatment ofLipedemaAL GRAWANY1603.7.6 Course oftheComplex-Operative Therapy PlanMany people wonder in which order the liposuctions is performed. Let us now go into the sequence of the complex surgical therapy plan and especially the sequence of the planned liposuctions integrated into it.The complex-surgical treatment plan includes: 1. Conservative treatment of lipedema withcompression therapy, if necessary lym-phatic drainage, if edema should be present (optional). 2. Weight analysis and, if necessary, treatment of concomitant obesity (from life-style modification to metabolic surgery). 3. Liposuctions for the treatment of lipedema. 4. Tightening surgery for foreseeable, physically limiting skin-soft tissue excess.In the following, we will discuss point 3, how the individual liposuctions proceed.Fig. 3.41 Triflow exercise: take a deep breathPossible Symptoms of Pulmonary Artery Embolism – Shortness of breath when inhaling or exhaling, possibly accelerated breathing. – Pain when breathing and chest pain, which may also radiate. – Palpitations. – Anxiety, possibly with sweating. – Dizziness, fatigue, fainting. – Cough and/or hemoptysis. – Unusual sounds when breathing.Z. Jandali et al.161Many providers of liposuction for lipedema have their own concept of how and in what sequence liposuction is performed. We have our own names for the different procedures: the “bottom-up concept,” the “outside-in concept,” the “holistic suction concept,” the “lipedema compression concept” and the concept of SRL liposuction, systematic-regional liposuction.“Bottom-up” ConceptIn the “bottom-up“concept, the lower parts of the body are suctioned first and the upper part of the body in subsequent operations. An example would be suctioning the lower legs and knee region in one operation; the thighs would be suctioned in a second and finally the hips in a third.“From the outside in” ConceptIn the “from the outside to the inside” concept, liposuction of the outer sides is per-formed in a first operation and liposuction of the inner sides in another operation. An example here would be liposuction of the lower legs, knees, thighs, and outer sides of the hips first. In the follow-up surgery, the inner sides of the lower leg, knee, and thigh would be suctioned.“Concept of Holistic Suction”.The “concept of holistic liposuction” suctions all body areas, as the theory is that lipedema “migrates” after liposuction and then affects other areas.“Lipedema Compression Concept”The “lipedema compression concept” is interpreted differently. Many suction all areas “somewhat” and again and again in different interventions until a satisfactory result is achieved.“SRL Liposuction Concept”.SRL stands for “systematic regional liposuction.” This concept has emerged after many years of searching for the best approach for us and many plastic surgery col-leagues. It includes, among other things, the systematic working off of the affected regions, the sequence of procedures, and the technique of how liposuction is to be performed. It does not matter whether we perform WAL, PAL, or other liposuction. c SRL stands for “systematic-regional liposuction” in which we try to close regions if possible and not suction them twice.To understand our concept, it is necessary to consider many individual factors. It is necessary to consider how the anatomy is, how liposuction works and is per-formed, what happens in the body as a result of liposuction and what the healing process is like. Our concept of systematic regional liposuction (SRL liposuction), due to the reasons listed above, always tries to close off regions as much as possible to avoid second and third liposuction of a region. At the same time, we must not disregard aesthetics and must continuously suction the transitions as well. This does not mean that small corrections here and there or even second aspirations cannot 3 Treatment ofLipedemaAL GRAWANY162occur, but we try to completely aspirate and close a region in one procedure if possible.But now it still depends on the fat deposits present. If these exceed an appropriate volume, secondary liposuction must be performed in any case. However, in about 70% of cases, we can avoid this.If we look at a classic case and see that lipedema is significantly located in the hip–thigh region and lower legs, often with a moderate expression, we can reach all affected areas in two liposuctions. One liposuction would then be performed in the supine position and another in the prone position. c Supine position means that you lie on your back during the operation. In the prone position, it is the other way around accordingly.During these individual liposuctions, we suction off a maximum amount of fatty tissue according to medically justifiable criteria. Of course, transitional areas are always suctioned as well, to obtain a harmonious transition between the regions as far as possible. It is not possible to suction off the entire fatty tissue, that is, com-pletely, without leaving any fatty tissue behind. Those colleagues who advertise or even make these statements must be questioned very critically. However, as little as possible fatty tissue should remain. c A statement like “we can remove all the fatty tissue or all the diseased fatty tissue” is not serious. Give a wide berth to such users.In the first operation in the supine position, we finally get good access to the outer sides of the hips, the thighs (front, outer, and inner sides) and also the inner sides of the knees. If there is also lipedema on the lower legs, we can treat the front, outer and inner sides of the lower legs as well. This is done until we reach our reasonable vol-ume limits or the maximum duration of surgery (for volume limits Section 0).The second operation is liposuction in the prone position. In this session, the outer sides of the hips (buttocks if necessary) are again treated and completed as a transitional region. The same applies to the inner thighs. Suction is applied to the backs of the thighs and the transitional region of the inner sides of the knees and the inner and outer sides of the lower legs. We often pay special attention to the lower leg region in this operation. This region, which is the most challenging to suction, requires highly precise work and a lot of experience.Depending on the extent of the first liposuction, the second liposuction can fol-low at the earliest after 2 months, better after 3–6 months. A too short interval between liposuctions is not recommended for medium to pronounced liposuctions, since the physical strain is quite high and the bruises and scarring need time to recede. Likewise, personal–social aspects such as career and family should also be taken into account.If the affected person also suffers from lipedema in the arms, the arms could be combined with liposuction of the backs, but only if it fits from the circumference. Otherwise, the arms would have to be liposuctioned in a separate procedure.Z. Jandali et al.163A three-stage treatment plan is shown in Fig.3.42. In the first step, liposuction of the fronts is performed, in the second step, liposuction of the backs is performed, and in the third step, liposuction of the arms is performed.We recommend an interval of at least 2–3months between two operations involv-ing the same transition areas. If liposuction of an area is planned more than once due to a strong manifestation of lipedema, we recommend an interval of at least 6months between the operations.Some colleagues recommend whole-body liposuction to treat lipedema, that is, liposuction of all areas of the body, because otherwise, lipedema will spread else-where on the body. On the one hand, it is nonsensical to argue this way; on the other hand, the approach makes perfect sense, and always when liposuction is used as the sole surgical component. Especially in the case of obese people, the approach is understandable, but it does not promise long-lasting success.We also very often advise liposuction of more regions than just the arms, but-tocks, hips, and legs. However, the overall treatment plan must be correct, and the recommendation of extensive liposuction is purely for aesthetic reasons, and we stand by that. Through extensive liposuction,including the back area, flanks, abdo-men, and lateral thoracic wall, we can shape an aesthetic and sophisticated body contour beyond the goal of pure pain treatment. c We often recommend suctioning other areas of the body for purely aesthetic reasons.In Table3.5 you will find a few examples of typical liposuction plans to help you understand our planning. Although the treatment plan is fixed, it is flexible in the course and can be readjusted after each procedure. Sometimes this reduces the Fig. 3.42 Flowchart of liposuction3 Treatment ofLipedemaAL GRAWANY164Table 3.5 Typical liposuction plansStage Location Quantity Extraction areaLipedema type 4 stage I (hips, legs, and arms)Supine position1 HipsOuter, anterior, and inner thighsFront and inner sides of the kneeLower leg front, outer, and inner sidesProne position1 HipsOuter, back, and inner thighsInside kneeBack, outer, and inner sides of the lower legPoorLipedema type 3 stage II (hips and legs)Supine position1 HipsOuter, anterior, and inner thighsFront and inner sides of the kneeLower leg front, outer, and inner sidesProne position1 HipsOuter, back, and inner thighsInside kneeBack, outer, and inner sides of the lower legLipedema type 3 stage IIISupine position2 or moreHipsOuter, anterior, and inner thighsFront and inner sides of the kneeLower leg front, outer, and inner sidesThe regions can be further subdivided per OP.Prone position2 or moreHipsOuter, back, and inner thighsInside kneeBack, outer, and inner sides of the lower legThe regions can be further divided per surgery. We would alternate between front and then back suction every 2–3months.Lipedema type III stage II plus abdominal region, flanks, and backSupine position1–2 HipsOuter, anterior and inner thighsFront and inner sides of the kneeLower leg front, outer and inner sidesThe regions can be further subdivided per OP.Prone position1–2 HipsOuter, back, and inner thighsInside kneeBack, outer and inner sides of the lower legPlus liposuction of buttocks, back, and flanks if necessary.The regions can be further subdivided per OP.Supine position1 BellyFlanksZ. Jandali et al.165number of liposuctions planned as the procedure progresses. Any necessary tighten-ing operations are performed after 6months.A very frequently asked question is when is the best time for surgerywhen plan-ning to have children. There are different opinions on this and we do not have a general answer. The fear of further aggravation of lipedema is justified and is fre-quently observed. However, we have also observed worsening of lipedema after liposuction and subsequent pregnancy.We hold the following opinion: A desire to have children always comes first. For those who do not want to have children for fear of aggravation, we would like to point out that this fear is unjustified, as much can be corrected and achieved afterwards.It is, therefore, necessary to consider performing liposuction before pregnancy or afterwards. The aim of liposuction before pregnancy would be to remove as much fatty tissue as possible so that there is no significant increase in volume during preg-nancy. Liposuction after pregnancy would have the advantage of removing areas that may have worsened.Following thoughts: – Not every pregnancy is associated with an exacerbation (probability is high, however). – Despite liposuction in advance, there may be a significant increase in lipedema. – Pregnancy often leads to significant tissue sagging, whether with or without lipo-suction beforehand. – Much more important than liposuction in advance is a perfect diet and lifestyle during pregnancy. There are special courses and concepts for this. – After the completion of the childbearing process, a holistic approach to body shape correction, including lipedema treatment, can be created if needed.Table 3.5 (continued)Stage Location Quantity Extraction areaLipedema type IV stage II plus abdominal region, flanks, and backSupine position1–2 HipsOuter, anterior, and inner thighsFront and inner sides of the kneeLower leg front, outer, and inner sidesThe regions can be further subdivided per OP.Prone position1–2 HipsOuter, back, and inner thighsInside kneeBack, outer, and inner sides of the lower legPlus liposuction of buttocks, back, and flanks if necessary.The regions can be further subdivided per OP.Supine position1 BellyFlanksSupine position1 Arms (with liposuction on the abdomen and flanks, if necessary).3 Treatment ofLipedemaAL GRAWANY166My personal opinion: If pregnancy is planned within the next 1–3years, I would rather not recommend liposuction in advance. If the time is unknown or in the dis-tant future, we recommend lipedema treatment in advance. Finally, it is also a ques-tion of cost coverage and financial means.Conclusion on Surgical Liposuction Treatment for Lipedema Neither conser-vative nor surgical treatment can cure lipedema. We do not share the classic recom-mendation that conservative therapy should be exhausted and now consider surgical therapy to be the treatment of choice for the treatment of lipedema, but only with the inclusion of possible obesity in the sense of complex surgical treatment with an individual therapy plan. Likewise, the complex surgical treatment also includes a forward-looking assessment of the impending change in body shape and requires mandatory consideration of possible tightening operations.3.8 Treatment ExampleIn the following, we would like to tell you a very classic lipedema patient story: The 36-year-old patient presented for the first time in our outpatient consultation. She reported suffering from a disproportion in favor of the legs and arms since puberty—initially very discreet and only noticeable to herself, but then increasingly. The first attempts at dieting failed, and there was a slow weight gain, almost always after dieting. In addition to the disproportion described, the affected person reported that she experienced pain under stress and later also at rest. At the slightest bump, she suffered a bruise. The disproportion became more pronounced over time. Likewise, the pain worsened. All further attempts to maintain and control the body weight failed. On the contrary, there was a creeping weight gain. The pregnancy of the first and the second child led to a significant aggravation. Especially the pain would have been almost unbearable by now. Many visits to the doctor followed, all of which were disappointing and unsuccessful. Finally, she presented to a vascular surgeon colleague in private practice. The diagnosis was made: lipedema.Further examination of the vascular system revealed no evidence of disease. A colleague from the vascular surgery department referred the patient to our consulta-tion. When the patient presented to our clinic, she was in sheer despair. Compression treatment and consistent lymphatic drainage were unable to achieve any significant improvement.On the day of the examination, body weight was 98kg with a height of 1.67m. The examination showed clinically a very pronounced lipedema of type IV stage 2.We talked to the patient about the possible options and showed her the advan-tages and disadvantages and risks of surgical and conservative treatment. First, we filed an application for cost coverage, which was rejected by several instances. The patient did not want to take legal action because of the poor prospects of success.Our surgical treatment plan included liposuction of the front of the lower extrem-ity (hips, thighs, knee region, and lower legs) and liposuction of the back of the lower extremity. In another session, liposuction of the arms (upper arms and Z. Jandali et al.167forearms) was also planned. If there was tissue sagging in the inner thigh area, a thigh lift could be considered (this was unlikelyat the time). In addition, the patient wanted liposuction of the abdomen and flank region (we combined this with lipo-suction of the arms).Finally, the planned procedures were performed (with the exception of the thigh lift). During the three liposuction procedures, nearly 28L of fat were removed using our own technique. After each of the procedures, the patient stayed with us in the clinic for 3days. Dizziness and nausea were not reported, there was only a slight decrease in the Hb value (hemoglobin). On each of the following days, the patient was able to move freely on the ward level. Of course, there were bruises and indu-rated areas. Lymphatic drainage was started early, and in time the initial swelling and bruising subsided.The further course was unspectacular. The soft tissues recovered well. The pain was already virtually gone after the first liposuction in the surgical regions—as it was at the end of the treatments. The pain was completely gone. The patient gave up compression only slowly after 6months. The aesthetic result was very good (for the patient and also from our point of view). The patient thanked us with a basket of chocolates at the end of the treatment. Another control will follow in 1year.3.9 Cost AbsorptionReimbursement for treatment, whether outpatient or inpatient, requires that the con-dition and treatment be recognized by the health insurance system.Lipedema is a recognized disease and is listed as a medical diagnosis in the International Statistical Classification of Diseases and Related Health Problems (ICD).In the German version of the current ICD-10, lipedema is coded E88.2x.Classification according to ICD-10-GM.E88.20 Lipedema stage I (Fig.3.43 left)E88.21 Lipedema stage II (Fig.3.43 center)E88.22 Lipedema stage III (Fig.3.43 right)E88.28 Other or unspecified lipedemaThus, one part of the requirement for cost coverage is met. The other part, namely the treatment, is somewhat more problematic in this case. Conservative treatment in the sense of compression treatment and lymphatic drainage are usually covered by health insurance for life. Unfortunately, when it comes to liposuction, the situation is different. Liposuction (liposuction) is not recognized as a measure for the treat-ment of diseases and thus also of lipedema.To test the effectiveness, the Federal Joint Committee (G-BA) has commissioned a clinical trial in which liposuction (surgical liposuction) has been compared with the standard nonsurgical treatment of lipedema since the beginning of 2020. During the trial study, the costs of liposuction will be borne by the statutory health insur-ance funds.3 Treatment ofLipedemaAL GRAWANY168Women with lipedema in all stages (I–III) in the leg region can participate in the study. The basic prerequisite for participation is that there has not been sufficient relief of symptoms under conservative measures. There are a number of inclusion and exclusion criteria that we do not wish to discuss further here, as the inclusion deadline is Dec. 31, 2019. The aim of the trial study is to assess the potential of the method in the indication to include the method in the benefits catalog of the statu-tory health insurance (SHI) if the result is positive—a ray of hope for all patients who cannot participate in the trial study. We ourselves are participating as a study center, but we strongly criticize the study design. Through the study, we all hope to gain more insight into the extent to which liposuction has a positive effect on the symptoms of lipedema.However, there are two other options through which liposuction is possible as a health insurance benefit.Fig. 3.43 Stages of lipedemaZ. Jandali et al.169 – Since 2020, liposuction in severe cases has become a statutory health insurance benefit. By “in severe cases” it means the presence of stage III.We have already discussed in detail that this criterion makes no sense, because stage III says noth-ing about the pain, nor about the suffering pressure, nor any other form of impair-ment. The fact that stage III liposuction has been included in SHI-accredited care was decided by the Federal Joint Committee (G-BA). The service has been included for a limited period until December 31, 2024. By then, the findings from the above-mentioned trial study, which is to assess the effect of liposuction in all stages of lipedema, should be available. – Cost absorption by the health insurance fund is also possible via a so-called indi-vidual case decision. The individual case decision is an administrative act based on separate circumstances. An individual case decision is requested from the health insurance fund. An application includes a medical report and an initially informal request formulated by you. As with all measures for which the health insurance fund is to cover the costs, there must be a medical indication.Surely you have already read the term “medical indication” and if this is not the case, you will certainly encounter it in the course of the application process. Indication in itself means “indication of cure.” It simply means which measure, for example, an operation or a drug, is the right one for the therapy of disease. The word “medical” means that the treatment is for an actual illness.In the case of lipedema, pain or restriction of movement would be the reason for treatment. The treatment of facial wrinkles with Botox® (botulinum toxin A) or by a facelift would be a cosmetic (=aesthetic) indication, since there is no physical or health impairment here. Consequently, it is a treatment of a condition which, by definition, is not a disease. These treatments are also referred to as IGeL services (individual health services).A medical indication exists in the case of a disease or disfigurement within the meaning of the Fifth Social Code (SGB V). This means that the health insurance fund will bear the costs of treatment for those affected. This means that in the case of a medical indication, the health insurance fund must bear the treatment costs.Actually, the health insurance company would have to cover the costs of treat-ment per se, provided that the doctor sees a pathological change. Before an eye, intestinal, or foot operation, the health insurance company is not asked either, since no one gets the idea that these could be “desired operations.” This is different for lipedema or tightening operations. Here, the view of the health insurance companies often differs from that of the plastic surgeons who provide a medical indication. The health insurance companies very often refuse to reimburse the treatment costs after the treatment has taken place. The health insurance company argues that there was no pathological condition and that the operations were scheduled, not acute. Therefore, the health insurer should have been asked in advance whether, in its opinion, there was a medical indication and the operation could be charged to the health insurer.3 Treatment ofLipedemaAL GRAWANY170Against this background, the individual case application for cost coverage must be made in advance. Since many employees at health insurance companies are pure clerks and do not have the competence to assess medical facts, the health insurance company often helps itself with the medical service of the health insurance com-pany (MDK).Thus, health insurance usually decides only after obtaining an expert opinion from the MDK.The MDK either decides on the basis of files, which is very difficult in the case of these operations, or it conducts an expert opinion.When are you entitled to have costs covered? Section 7 sentence 1 of the German Social Code Book V states: “Insured persons are entitled to health treatment if it is necessary in order to recognize or cure an illness, to prevent its aggravation or to alleviate symptoms of illness.” c Specialist lawyer T. Werner has written the very interestingChap. 6 with all the necessary information on medical indications and questions of cost coverage. I can only warmly recommend this to you.In the case of psychological problems, according to case law, treatment with psychotherapy, or psychiatry is indicated as a priority. Therefore, you should never apply for cost coverage on the basis of psychological stress; a rejection would be inevitable.In reality, medical indications are far less tangible than the legal text would sug-gest. The assessments by the MDK are so varied that we can no longer even make a prognosis as to whether costs will be covered. This most often has to do with the existing or lacking qualification of the physicians of the medical service in this par-ticular field. How well trained is a trauma surgeon, internist, or gynecologist to decide if liposuction is the right treatment? We have read very questionable and astonishing decisions by the MDK in our daily clinical practice. In addition, the health insurance company does not always follow the recommendation of the MDK, which it does not have to per se.Costs for operations for which there is no medical justification are not covered by health insurance. These are essentially aesthetic operations. The person con-cerned must pay for these. In contrast to medically justified operations, where the entire treatment, including treatment of complications, is fully covered by the health The Medical Service of the Health Insurance Fund (MDK) is an institution that advises health insurance funds on medical issues, among other things. Doctors from various specialities are permanently employed by the Medical Service and, to put it simply, work as “experts.” In the rarest of cases, the MDK can call on the services of a plastic surgeon. Therefore, doctors from other fields often deal with plastic surgery issues, which unfortunately often leads to incomprehensible decisions. But here, too, the MDK and the physi-cians work continuously to achieve comprehensible decisions.Z. Jandali et al.https://doi.org/10.1007/978-3-030-86717-1_6171insurance, the affected person will share in the costs of complications after aesthetic operations, depending on their income. To exclude this risk of co-payment, a so- called follow-up cost insurance can be concluded. In addition, operations that are not medically indicated are charged with VAT. c Medically justified interventions are operations whose costs are charged to the health insurance companies. Aesthetic procedures (operations without medical indication) are charged to the patient. The costs of follow-up operations or complications must then be borne by the patient. To avoid this risk, a special follow-up insurance policy can be taken out for this purpose before the operation.After the refusal of cost coverage or if there is no medical indication, liposuction can also be performed as a self-pay service. The costs of liposuction depend on the number of regions to be treated during the operation as well as the difficulty of the operation, form of anesthesia, duration of anesthesia, length of stay, visits, and fol-low- up checks. It also plays a role whether the treatment is outpatient or inpatient in the hospital. Liposuction starts at about 1000 euros plus VAT with local anesthesia and a small circumscribed area. A hip–thigh liposuction on an outpatient basis under twilight sleep anesthesia costs about 4500–6500 Euro plus VAT.For extensive liposuction of the hip, thigh, and lower leg region, costs between 5500 and 7500 Euro plus VAT have to be calculated.Other costs that are added to a self-pay service are follow-up insurance. If a complication occurs during a self-pay service, your health insurance company may charge you a share of the costs or refuse to cover the costs altogether. With follow- up cost insurance, you can protect yourself against this financial risk.3.10 Autologous Fat GraftingAngel Pecorelli Capozzi, Zaher JandaliYou may think that this chapter is rather unusual for a lipedema book. However, we performautologous fat grafting in about every tenth lipedema treatment. This corresponds to about 10% of cases and is not entirely insignificant.Liposuctioned fat can be used very well for autologous fat grafting. Autologous fat grafting is also called “lipofilling.” c Autologous fat grafting is also called lipofilling.In this process, the extracted fat is washed, processed, and transplanted. The self- transplanted fat grows on site to about 70% and remains in place forever. About 30% of the fat cells die after transplantation. c In autologous fat grafting, the extracted fat is washed, processed, and transplanted.3 Treatment ofLipedemaAL GRAWANY172For several years, autologous fat grafting (autologous fat transfer, lipofilling) has enjoyed increasing popularity. Most often, autologous fat transplantation is used for volume augmentation and tissue rejuvenation. Volume augmentation is often per-formed for breast augmentation in cases of congenital or acquired volume defi-ciency. When we talk about an acquired volume deficit in the breast area, we are talking about changes in the breast that occur due to the natural aging process, or weight loss. However, volume loss also occurs in the face during the aging process, as well as aging and sagging of the skin. The loss of volume in the face occurs due to a reduction of fatty tissue and bone, as well as sagging of the soft tissues due to gravity.Over the years, various synthetic fillers and implants have been used to treat these volume deficits. Each of the substances had its advantages and disadvantages, and the practitioners always had to make compromises due to the often not insignifi-cant disadvantages. In the past, liposuction procedures were traumatic, and fat cells were destroyed during liposuction. This is no longer the case today. Nowadays, liposuction procedures, whether WAL, PAL, or standard, are very gentle procedures and the fat cells obtained as a result have good vitality. As a result, fatty tissue has proven to be an ideal filler for the above-mentioned indications. But not only that: fatty tissue offers much more. As we have read in the introductory chapters, we also find stem cells and growth factors in adipose tissue. We can extract these stem cells and growth factors and deliver them to the skin to support collagen synthesis, improve blood circulation and improve skin quality. Here we are already in the middle of the topic of regenerative medicine. However, we most often use lipofilling for breast augmentation and wrinkle treatment. c Possible applications are own fat breast augmentation, facial wrinkle treatment, lip modeling, and many more.Autologous fat grafting is divided into two parts. First, liposuction is performed, followed by fat grafting. In all techniques, the suctioned fat must be collected in a special canister or system for fat grafting (Fig.3.44).After repeated cleaning and, if necessary, processing of the fatty tissue, it is drawn up into syringes and can be injected into the recipient area, for example, the breast (Fig.3.45).Roughly speaking, we distinguish between three types of fat that are suitable for transplantation: In addition to macro fat, which is the simple fat that we harvest with our suction cannulas, there is also micro fat and nano fat. The macro fat, as shown in Fig.3.46, we use, for example, for breast or buttock augmentation, but also the correction of dents or irregularities. In macro fat, the fat particles are larger than 1.5mm, on average about 2–3 or 4mm. The fat tissue is obtained with fine cannulas. c Roughly speaking, we distinguish three types of fat for autologous fat grafting: macro fat, micro fat, and nano fat.Z. Jandali et al.173Fig. 3.45 Drawn-up fat syringes for transplantationFig. 3.46 Macro fat when inserted into the breast for breast augmentationFig. 3.44 The collected greasefar too often sold as truths. You can expect an exciting potpourri of scientifically proven facts, hypotheses, suppositions, half-truths as well as insights gained from observations.The term “pathophysiology” is composed of two terms. The word “pathology” comes from the ancient Greek πάθος, páthos, meaning “disease,” and λόγος, lógos, the doctrine. Pathology, then, means the doctrine of disease, the “doctrine of afflic-tions.” The word physiology is also composed of two words and comes from the ancient Greek: φύσις, phýsis, ‘nature’ and λόγος, lógos, ‘doctrine’. Physiology is consequently the study of what is normal or healthy. Pathophysiology thus describes Z. Jandali et al.5which functional mechanisms lead to pathological changes and how the sick body functions.Before we turn to the pathophysiology, we must briefly discuss the disease trig-gering factors. To date, the trigger mechanism for a fat distribution disorder is unknown. Also, why lipohypertrophy that it can be an inherited condition (the lit-erature speaks of an accumulation of up to 60% in first-degree relatives). In addi-tion, there are factors such as hormonal balance and lifestyle. But one thing is certain: lipedema takes place in or around the adipose tissue. That is why we are taking a closer look at the adipose tissue. c Triggers of lipohypertrophy and lipedema are unknown.1.2.1 Adipose TissueWhen talking about adipose tissue, we distinguish between “storage fat” and “con-stitutional” fat. We find the constitutional fat mainly in the area of organs such as the kidney, but also in our extremities, such as the hands and feet. In the area of the heel, it serves, among other things, to absorb shocks when walking, thus the fat having here a purely mechanical function. The storage fat is the classic subcutaneous adi-pose tissue, which acts as an energy storage reserve and “cold insulation” or insulator.TypesWe distinguish a total of three types of adipose tissue: white, beige, and brown adi-pose tissue (Fig.1.2). In the context of lipedema, only the white adipose tissue, which has the function of storage or depot fat, is of interest to us.However, before we take a closer look at white adipose tissue, for the sake of completeness we will briefly discuss the other two forms.Until 2009, it was assumed that brown adipose tissue was only present in babies. However, a study showed that adults also have a proportion—albeit very small—of brown fat. Brown fat has the property that it can generate heat. This occurs in the so-called mitochondria, which work like small power plants within the cells. This Fig. 1.2 White, beige, and brown adipose tissue1 The LipedemaAL GRAWANY6form of adipose tissue is very common in the animal kingdom, especially in animals that hibernate so it will help them to quickly raise their body temperature as soon as they wake up. Adults, on the other hand, have only limited brown fat depots.Beige adipose tissue is found sporadically between the white adipose tissue. The function is not conclusively clarified; however, heat production is also discussed.Let us now turn to the white adipose tissue. We find this as subcutaneous adipose tissue all over the body and thus also in the regions where lipedema takes place.The subcutaneous fat tissue, where lipedema takes place, consists of white adi-pose tissue.Adipose tissue is a form of connective tissue and consists, among other things, of fat cells, the so-called adipocytes. One can imagine adipose tissue as a sponge in which the “holes” are filled with adipocytes. The adipocytes are surrounded by many other different cells, scaffolding fibers and blood vessels and are combined within the surrounding tissue into small conglomerates, so-called lobules. Also located in the adipocyte environment are the progenitor cells of the adult adipocyte, which we will look at in more detail later in this section.Depending on the body region, the subcutaneous fat tissue is structured differently. The total layer of subcutaneous fat tissue is divided into two compartments by a fat fascia (connective tissue plate): superficial and deep. The superficial compartment contains predominantly finer fat cells, while the deep compartment contains larger fat cells or fat conglomerates, each in proportion to the respective region. This means that, for example, the conglomerates of the deep fat layer on the buttocks are much larger than those on the forearm. In Fig.1.3, we see an example of such a structure.The white adipose tissue has different functions. In addition to its function as a metabolic organ, it mainly acts as a storage or depot fat. In addition, it can act as insulating fat to protect against heat loss and as a buffer zone as well as a protective layer in the form of building fat (kidney-bearing fat, sole of foot, eye). c The largest proportion of white adipose tissue is found in the subcutaneous adipose tissue.Structure and FunctionSimilar to the way human skin acts as a barrier to the environment, a fat cell (adipocyte) is separated from the environment by a cell membrane (cell wall). Fig. 1.3 Adipose tissue buildup in the subcutaneous fat tissueZ. Jandali et al.7This cell wall contains a number of different interfaces to which messenger sub-stances can dock and trigger cell actions. We call these interface receptors. Examples are estrogen, insulin, and adrenaline receptors. In lipedema, receptors seem to play a central role. Therefore, we will go into this topic again in more detail in Sect. 1.2.3.The fat cell, like any cell, has a “basic equipment,” that is, a typical cell structure with a nucleus, where the genetic information (DNA) is located, with its energy providing mitochondrial apparatus and so on. Within the fat cell are the cell organ-elles, surrounded by the cytoplasm with the basic water-based structure of the cell, the cytosol. These are responsible for different functions of the cell.The special feature of each white fat cell is its function of storing fat. The fat inside the cell is not limited by any wall or similar. The so-called lipid droplet (the fat content in the fat cell) is delimited in the cell only by a light-colored fringe (delimiting vimentin filaments) visible under the microscope. Depending on whether one or more fat droplets are found in the fat cell, we distinguish univacuolar (one fat droplet) from multivacuolar fat (several fat droplets) whereas white adipose tissue is predominantly univacuolar fat.In Fig.1.4 we show an example of a univacuolar fat cell with a typical, volumi-nous lipid droplet accounting for about 95% of the cell volume, pushing the nucleus to the cell membrane forming a so-called signet ring structure of the cell nucleus.The energy balance of our body is subject to a constant dynamic. We distinguish between an anabolic and a catabolic phase. During the anabolic phase, the body’s own energy storage components are built up under a certain energy consumption. For fat cells, this means that the lipid droplet is built up as a fat store in an anabolic phase and broken down accordingly in a catabolic phase.A buildup and storage of fatty acids in a fat cell is only possible when there is an energetic surplus. The storage of energy in fat form is only possible through two mechanisms:Fig. 1.4 Univacular fat cell with typical giant lipid droplet1 The LipedemaAL GRAWANY8 1. absorption and storage of fats via food intake or, 2. body’s own production of fatty acids, for example, by carbohydrates, a process is also known as fatty acid synthesis.The breakdown of fatty acids and the resulting energy is referred to as lipolysis. The buildup and breakdown of fat are hormonally controlled, the hormones insulin and adrenaline play being the key players in this process.Did you know that a fat cell has a limited lifespan and is subject to a life cycle? Fat cells are continuously built up andis drawn up from the collection container via syringes3 Treatment ofLipedemaAL GRAWANY174If the fat conglomerates are broken up, then we obtain micro fat (Fig.3.47). The micro fat particles have a diameter of about 1mm and are aspirated through special micro fat cannulas with a hole diameter of 1mm. The micro fat is particularly suit-able for facial wrinkle treatment.When we talk about nano fat, we are basically no longer talking about adipose tissue per se, but only about the stem cells found in adipose tissue. Nano fat is all about regenerative medicine. Nano fat is produced via a process in which we first break up the fat conglomerates into tiny particles and finally further process the entire fat emulsion through a filter. In the end, we have an aqueous-yellowish emul-sion that no longer contains intact fat cells, but instead contains a great many stem cells with regenerative potential. We use nano fat most frequently for sustainable improvement of the skin’s appearance (Fig.3.48).The exact mechanism of fat cell survival after transplantation is not yet fully understood. We would like to briefly present one of the common theories: After the fat cells are removed from the donor region and transplanted into the recipient region, a race against time begins for each individual fat cell- death versus survival. For the survival of the transplanted fat cell, the supply of oxygen and nutrients is indispensable. Since the fat cells do not have their own blood vessels, they must be supplied via diffusion until the fat cells have reconnected to the local vascular net-work and can be nourished via it. This diffusion provides an initially sufficient sup-ply for most of the cells.It should be noted that the higher the oxygen content of the recipient tissue, the better the growth rate of the fat cells. Negative influencing factors such as smoking and excessive pressure on the cells should therefore be strictly avoided. Clinically, an average survival rate of transplanted fat cells of about 60–70% is observed. c Autologous fat grafting in smokers more often shows a poor fat cell attachment rate. Poorer tissue perfusion and a lower oxygen supply in the recipient tissue are responsible for this.Fig. 3.47 After autologous fat grafting to the breast with previously severe asymmetry and bilateral hypovolemiaZ. Jandali et al.175In addition, it is important to avoid too much pressure on the transplanted area. Pressure from the outside causes reduced blood flow, which in turn causes the cells to die. This means, for example, that you should not wear a compression bra or tight bandage at first after a breast augmentation. You can wear a light sports bra that does not exert much pressure from the second week. Likewise, you should not lie on your stomach after a breast augmentation with your own fat. Too much internal pressure due to “overfilling” with fat cells can also lead to fat cell death. Warmth, on the other hand, has a positive effect on fat cell survival. The warmer the recipient area, the better the blood circulation in this area.The most important factor for the growth of as many fat cells as possible is the technique of transplantation. If fat transplantation is performed with an excessive amount of fat tissue, too much internal pressure is exerted on the transplanted cells. On the one hand, this impairs the blood supply to the resident cells, and on the other Fig. 3.48 Micro and nano grease3 Treatment ofLipedemaAL GRAWANY176hand, the diffusion necessary to supply nutrients to the transplanted cells is impaired, so that both groups of cells may die.For the reasons just mentioned, fat grafting to the breast, for example, can some-times be performed with only 100mL, sometimes with up to 400mL or more per breast. So how much fat can be transplanted depends on how much well perfused, localized tissue is available. If you naturally have little subcutaneous fat in the breast area, very little fat can be transplanted in the first few grafts. In this first transplanta-tion, the surgeon creates a so-called graft store with the fat grafts for the following transplantations. If the tissue thickness in the breast is sufficiently good, a larger volume can be transplanted directly in the first procedure.Due to the volume limitation during surgery, breast augmentation is performed, depending on the target size and in several sessions. As a rule, one to three sessions are planned. This step-by-step procedure to ensure the success of the therapy is called sequential autologous fat grafting.The most frequently asked question when it comes to the topic of autologous fat is whether lipedema then develops in the breast with a halting growth of the fat cells. From our many years of experience, we can answer this question with a clear no. If, for example, breast augmentation has been performed using autologous fat transplantation, the breast will behave quite naturally afterwards. If you gain weight, the breast volume increases. If you lose weight, the breast volume decreases. Painful breasts after autologous fat transplantation have not yet been described to us. Unfortunately, there is not enough scientific work in this area either. It also seems that the painfulness has more to do with the location of the fat cell than with the fat cell itself, which makes sense.Z. Jandali et al.177© Springer Nature Switzerland AG 2022Z. Jandali et al. (eds.), Lipedema, https://doi.org/10.1007/978-3-030-86717-1_4Z. Jandali (*) · B. Merwart · L. Jiga Department of Plastic, Aesthetic, Reconstructive and Hand Surgery, Evangelical Hospital Oldenburg, Oldenburg, Niedersachsen, Germanye-mail: dr@jandali.de4Body Contouring Surgery After Extensive Liposuction andWeight LossZaherJandali, BenediktMerwart, andLucianJigaThe last component of our complex surgical treatment plan for lipedema is the treat-ment of any excess skin-soft tissue. In many patients, these measures are not neces-sary, however, in certain cases, there is a medical indication for body shape restoration. This is especially true for patients with stage 3 (or according to our classification +++) or with massive weight loss associated with lipedema. The most common surgical corrections in such patients are the thigh and upper arm lifts, but also abdominoplasty, circular torso, and buttock lifts.4.1 Medical Indication forTightening ofExcess Skin andSoft TissuesA medical indication for treatment exists in the case of a specific disease or disfig-urement. This means that the health insurance must or should bear the treat-ment costs.Skin irritation is the most frequently expressed complaint. Heat accumulates due to skin surfaces lying on top of each other, and in the absence of air circulation, moist chamber forms, disrupting the natural skin barrier. This favors the formation of chronic open wounds with fungal and bacterial infections. These are usually found in the area of the inner thighs, armpits, and the fold of the abdominal wall. The continuous rubbing taking place in these areas can lead to real sores and pain and can be responsible for the use of pain medication.The excess “skin flaps,” especially on the upper arms and thighs (Fig.4.1), result in a restriction of natural movement in everyday life and especially during sport. The excessive soft tissues and skin will lag behind at each arm and leg movement AL GRAWANYhttp://crossmark.crossref.org/dialog/?doi=10.1007/978-3-030-86717-1_4&domain=pdfhttps://doi.org/10.1007/978-3-030-86717-1_4#DOImailto:dr@jandali.de178due to their inherent inertia. Here, the restrictions range from “somewhat disturb-ing,” which certainly cannot be applied as a justification for a medical indication, to “I can hardly move” or “sport is not possible per se.” The chronic sweating and the associated unpleasant body odor lead to social withdrawal and the feeling of stigmatization.broken down by the body. An adult adipocyte grows from a fat precursor cell, a so-called preadipocyte, which in turn grows from a connective tissue precursor cell. You are probably wondering now how a fat cell knows that it is to become a fat cell? For the differentiation or development of such a fat cell, several intricate messenger compounds and processes are required. Explained simply, there is something like a program that is played leading to the production of all the necessary messengers so that the precursor cells understand that they need to turn into a fat cell. When the life of the fat cell is over, it dies and is degraded. A new fat cell develops in its place (Fig.1.5).If we take another look at the structure of adipose tissue, we have to imagine a convolute of fat cells, blood vessels, and other connective tissue cells. The so-called precursor cells (stem cells) are attached to the small blood vessels, from which new, young fat cells can develop by means of appropriate signals. In the process, they take up appropriate triglycerides (fatty acids) from the blood, through which they build up your fat droplet.Body fat tissue can expand from 2–3% to over 60–70% of the body volume. A normal weight man has a fat tissue percentage of about 10–20%, a woman about 15–25%.In childhood and adolescence, there is an increase in the absolute number of fat cells, but in adulthood, their numbers remain under physiological conditions Fig. 1.5 The picture shows the theoretical life cycle of a fat cellZ. Jandali et al.9unchanged. Weight gain will lead to an increase in the size of the existing fat cells whereas, in the case of weight loss, the opposite happens. In these cases, we speak of hyperplasia and hypertrophy of fat cells. Hyperplasia stands for the proliferation of fat cells (an increase of their absolute numbers) and hypertrophy stands for a pure increase in the volume of each individual fat cell. c The number of adult fat cells increases until puberty without significant change afterwards. What can still change in adulthood is the volume of the individual fat cells.Even after massive weight loss following bariatric surgery, it was shown that the absolute fat cell count did not change significantly.About 8.4% of all fat cells renew themselves each year. It is assumed that each fat cell has a lifespan of about 1year. In total, a normal average person with 13.5kg of adipose tissue has about 40 billion fat cells.A fat cell can store a maximum of 1μg of fat (= 1 millionth of a gram=10−6g). 1g of fat has about 7 calories. c 1kg of fat tissue has 7000 calories. If you wanted to lose 1kg of fat in a week, you would therefore have to save 1000 calories a day (at constant body weight).Due to our physical storage and depot fat, humans are able to go several days without food. c One cubic centimeter of fat (equivalent to the volume of 1mL of water) weighs 0.94g. 1L of fat is therefore equivalent to 940g.In humans, there are different types of fat storage depending on gender. We speak of a gender-typical fat distribution, in a variable range that is genetically determined (Fig.1.6).A study on the structure of adipose tissue of lipedema patients as compared to and healthy individuals (both groups were of normal weight), revealed enlarged fat cells in the lipedema group.1.2.2 Science inDetailFor the understanding and treatment of disease, science is the measure of all things. For this reason, we need to look at the scientific data on lipedema to delve further into the topic of pathophysiology. However, not all science is the same. We distin-guish different qualities of scientific papers. The measure of the quality of a scien-tific paper is measured by a level of evidence. A good paper has a high degree of evidence and thus also a high content of truth of its statements. The basis of a good paper starts in the planning phase with the basic framework, the study design. If the 1 The LipedemaAL GRAWANY10design of the study is not well planned, no meaningful result will emerge at the end of the work.Such examples are observational studies that focus mainly on one single ques-tion and not on a certain intervention (e.g., how pain perception changes in lipedema patients after surgery) aimed to improve outcomes. Thus, the overall results of such a study, regardless of being retrospective or prospective remain of limited relevance for the reader. It is why, one should choose carefully the information sources, be aware of the relevance of each information, and not believe in everything that’s written.Purely scientifically, we do not know much about lipedema. The scientific papers that exist on lipedema are rather poor in terms of scientific quality. Most of the stud-ies we found are purely descriptive or observational, underpinned by conclusions and references of other equally poor studies. Experimental studies on lipedema are extremely rare to find. The few studies that do exist use questionable study designs, small numbers of cases, or fail to show statistically significant results.Anyone who still has doubts about this shocking reality should independently perform the same search on the subject lipedema. As such, affirmations like: “Lipedema is curable” or “The diseased fat cells store water instead of fat” Fig. 1.6 Gender-typical fat distribution in men (apple type) and women (pear type)Z. Jandali et al.11appearing either on websites or other media, are grossly negligent and without any scientific fundament. Updating a patient about the actual stand of knowledge in lipedema is a tedious but mandatory step before initiating the talk on possible treat-ment options. As a consequence, many patients leave after the first consultation dissatisfied, because of being confronted with an uncomfortable truth. This fact is also frustrating for the physician, his efforts to provide such information in a reason-able way to his patients resemble at times a battle against windmills.Let us first look at the general data situation. If we search for the term “lipedema” in one of the largest scientific portals (PubMed), we currently (01/2020) find a total of 197 literature hits, although this number says nothing about the really correct hit rate on the topic and certainly nothing about the quality of the studies. If we search for the term “lymphedema,” there are 15,203 hits.It is claimed that up to every tenth woman suffers from lipedema. We have simi-lar disease figures with breast cancer. Although breast cancer, which can have fatal consequences, is not comparable to lipedema, the 399,260 hits when entering “breast cancer” in the search mask demonstrate how underrepresented lipedema is in the scientific literature. This is also clear in the case of lymphedema, which occurs much less frequently than lipedema, but has many times more hits.Of the 197 hits in the literature about lipedema, we believe that about 80% have major relevance issues. The remaining 20% include good studies in terms of struc-ture and design, but none could be found in which the following questions are clearly answered: 1. How is the disease triggered? 2. What is the mechanism of the disease? 3. How can lipedema be diagnosed with certainty? 4. How can the disease be cured?Consequently, we are largely dealing with observations and assertions in these studies. Unfortunately, and this is currently a problem, many statements from these studies have managed to be recognized as truths and facts. To our regret, this recog-nition has occurred not only among patients and their families but also among many physicians who propagate these findings instead of questioning them. Almost every patient referred to us with the diagnosis of lipedema will say “I have lipedema and water in my legs.” This statement is more than questionable since lipedema primar-ily has nothing to do with water retention. As alreadydescribed in Sect. 1.2.1, a fat cell consists largely of lipids and only to a small extent of water, which is present in the form of the cytosol. There are rare exceptions, which we will discuss in more detail in Sect. 1.3.An interesting phenomenon: Germany is at the forefront of scientific publica-tions on lipedema. At this point, however, we are only looking at the absolute num-ber of publications and not the quality. In addition, Germany is also a pioneer in scientifically unfounded statements on this topic.Guidelines for diseases are often published by professional societies or an asso-ciation of professional societies. This is also the case with lipedema. These 1 The LipedemaAL GRAWANY12guidelines are available to all on the Internet and, like studies, are divided into dif-ferent quality classes. The lipedema guideline is a so-called S1 guideline with only a “recommendation” character. Here, too, there is a lack of scientific impact because the guideline reflects the opinions of various experts. There are no objective criteria that make one an expert or not. Just as we have not been tested as experts, but con-sider ourselves to be. c Anyone can download the current guidelines for the treatment of lipedema from the AWMF website at https://www.awmf.org/1.2.3 Hormone Activity oftheAdipose TissueThe hormonal activity of the adipose tissue is an often underestimated aspect of lipedema. Adipose tissue has long since ceased to be regarded as an inactive, inert tissue, being much more than a mere “fat store.” It is an active and also endocrine organ. The metabolism of adipose tissue affects the entire body. Adipose tissue is capable of synthesizing and releasing hormones but is also sensitive to hormones such as estrogen and insulin.Since the onset of lipedema often coincides with the onset of a hormonal change phase, an estrogen-regulated disorder is assumed. Whether this is inherited polygen-etically (i.e., a change resulting from several gene changes) or monogenetically is still a subject of research. Based on our clinical observation and the stories of the affected persons, a hormonal connection is also comprehensible from our point of view.EstrogenWe have already talked about the life cycle as well as the metabolism of fat cells. Another theory, which has been researched with scientifically sound and compre-hensible foundations, focuses precisely on this metabolism and the control of the fat cell by estrogens. Estrogens are recognized by two different receptors of the fat cell. We distinguish the estrogen receptor α (alpha) from the estrogen receptor β (beta). Both receptors trigger different metabolic mechanisms in the fat cell. At the same time, these receptors are also found on many other tissue cells; we will focus here and now on the fat cell. In the schematic representation, we have omitted other receptors for simplicity (Fig.1.7).Fig. 1.7 Schematic representation of estrogen receptors on a fat cellZ. Jandali et al.https://www.awmf.org/13The α-receptor is significantly responsible for energy balance (buildup and breakdown of fat volume), the inflammatory response in adipose tissue and fibrosis. c Fibrosis is a pathological proliferation of connective tissue that results from increased collagen synthesis. Fibrosed connective tissue is hardened and functionless. If, for example, lung tissue turns fibrotic, this part is no longer available for gas exchange.If the estrogen concentration is reduced (e.g., postmenopausally), abdominal obesity develops with an increased risk of metabolic syndrome. This development can be counteracted, for example, by estrogen replacement therapy.In addition to the concentration of estrogen in the bloodstream, an incorrect (pathological) distribution pattern of α- and β-estrogen receptors on fat cells can program “fat gain” or “fat loss” (Fig.1.8).The α-estrogen receptor mostly triggers positive metabolic processes. By this, we mean an improvement in insulin sensitivity and glucose tolerance. The receptor counteracts fat accumulation, which is why it is attributed a weight-reducing prop-erty. Overall, its triggered metabolic cascades lead to a normalization of fat and sugar balance.To explain: Each fat cell carries an individual number of α- and β-estrogen recep-tors. Their distribution determines how a fat cell responds to caloric excess (Fig.1.9). Many of these conclusions have emerged from animal studies. If these theories apply to humans, then this theory would be a reasonable explanation of why lipedema develops in some women and not in others. Indeed, if the α-receptor were increased and the β-receptor decreased on a fat cell, adipose tissue would be degraded; the other way around, the fat gain would occur.Fig. 1.8 Schematic maldistribution of estrogen receptors that can lead to weight gain1 The LipedemaAL GRAWANY14 c Adipose tissue is hormone-active and hormone-sensitive. In lipedema, a maldistribution of estrogen receptors in the area of the affected regions is assumed.In addition, altered estrogen receptor patterns in the brain are discussed as caus-ative for impaired appetite.Estrogen and ProgesteroneThe counterpart of estrogen is progesterone. Progesterone is a so-called corpus luteum hormone and the most important representative of the progestins, which—as the name suggests—are produced in the corpus luteum of the ovaries. The balance between progesterone and estrogen is of enormous importance for our body. If there is a disbalance, important body functions such as weight regulation become unbalanced.Since estrogen production in adipose tissue occurs through the conversion of androgens (male sex hormones) into estrogen, too much adipose tissue leads to estrogen overproduction. This acts on the own estrogen receptors and at the same time causes a further imbalance to progesterone.If the estrogen level is permanently elevated compared to the progesterone level due to a lot of existing adipose tissue, overeating, or due to an underproduction of progesterone in the ovaries, there will be a continuous weight gain. The situation is similar during menopause. Although there is a lack of estrogen, at the same time Development of Lipedemafood intakeLipohypertrophyPainLipedemaHypoxia (oxygen deficiency)Angiogenesis (vascularization)Inflammatory responseFibrosis (connective tissue scarring)+Estrogen Receptor (Beta)“upregulation”“downregulation”Estrogen Receptor (Alfa)Hypertrophy of the fat cellAndrogens --> EstrogensFig. 1.9 Illustration of lipedema developmentZ. Jandali et al.15hardly any progesterone is produced due to the absence of ovulation, so that there is also a disbalance. c When there is an excess of estrogen, we speak of estrogen dominance. c In women over 40, there is additional energy saving due to a failure of ovulation. If egg production is reduced or comes to a complete standstill, the body saves up to 300kcal a day 1week before the period.InsulinInsulin is also an important hormone for fat metabolism. But what is insulin and what does it do exactly? Insulin is a hormone that is produced and released by the pancreas. A release of insulin occurs with food intake which in turn will regulate the sugars extracted from food to can be taken from the bloodstream into our body cells.It works according to the lock-and-key principle. Insulin is the key that docks with a receptor on the cell surface and can then introduce sugar molecules into the cell.If obesity is present, this can lead to so-called insulin resistance. This means that the insulin signal no longer has its intended effect. In principle, more and more insulin release leads to overstimulation of the receptors. The overstimulation causes the insulin receptors to down-regulate (“down-regulate”). As a result, the cells can no longer respond adequately to insulin secretion. The pancreas in turntries to compensate for this by overproducing insulin, which can be up to 15 times the original amount of insulin. Eventually, however, the pancreas gives up and stops producing insulin. The result is a chronically elevated blood glucose level, type II diabetes. c The consequence of insulin resistance is the so-called “diabetes”=diabetes type II.The result of insulin resistance is further weight gain through the following mechanisms: no longer enough glucose reaches the cells because the insulin key can no longer open the gate for sugar (glucose). In the brain, too, insulin can induce appetite suppression, which does not occur when there is resistance. Thus, there is a lack of central inhibition of appetite. This can cause you to get a strong craving for sugary foods and then logically follow that, supporting the vicious cycle. Researchers made an interesting discovery in this area. They found that the amount of belly fat increases in particular the risk of insulin resistance by up to 80%. In comparison, fat in other parts of the body only increases the risk of this by 50–60%.Excessive blood glucose levels are extremely harmful to the body in the long term. Particularly susceptible to “hyperglycemia” are the small and smallest blood vessels, which undergo pathological changes. This results in circulatory disorders, for example, in the kidneys and the eye, but also in vascular changes in large blood vessels, such as those in the legs (PAD—peripheral arterial occlusive disease) which will, in turn, increase the risk of heart disease and stroke.1 The LipedemaAL GRAWANY16 c If the symptoms occur c elevated blood glucose level, c Severe overweight. c High blood pressure and. c Lipid metabolism disorders. c together, we speak of a “metabolic syndrome.”As suspected, the known risk factors for developing diabetes are obesity due to overeating, heredity, stress, and lack of exercise. Generally speaking, when a body is “fed” glucose, it causes a shift in metabolism. Fat breakdown is slowed down and the synthesis of new fat is pushed.LeptinAnother hormone produced by fat cells is leptin. In healthy people, leptin reduces appetite. Unfortunately, this is not the case in people with an increased amount of fat. Although leptin levels are higher than in normal-weight individuals due to the mass of adipose tissue, the brains of obese people respond to leptin more poorly than those of healthy individuals. We call this condition, analogous to insulin, leptin resistance. Why the brain fails to reach the full leptin message is still a subject of research. Due to leptin resistance, a feeling of satiety is often not achieved despite sumptuous meals, and the appetite remains. This leads to an unstoppable urge to eat, which accelerates the vicious circle.GhrelinGhrelin is the hunger hormone par excellence, a growth hormone that is produced predominantly in the stomach, but also to a small extent in the pancreas. Via the bloodstream, it reaches our central mainboard (brain) and interacts with the hunger center and the pineal gland. Ghrelin stimulates the pineal gland to secrete the growth hormone somatotropin. A deficiency of somatotropin in an adult is associated with obesity, decreased life expectancy, reduced muscle mass, and decreased bone den-sity. Before food intake and during periods of fasting, serum ghrelin levels in the blood increase. Normally, food intake results in suppression of ghrelin release. Curiously, one would think otherwise low ghrelin secretion occurs in obesity, how-ever, this is still under investigation, one possible theory suggests that obese people are more sensitive to the hormone ghrelin. c It is reasonable to assume that hormonal changes can trigger or aggravate lipedema.Basically, we must note that lipedema is often associated with obesity and obe-sity is often associated with lipedema. c Adipose tissue is very hormone-active.The adipose tissue in lipedema is normal adipose tissue in terms of its rough structure. However, it is still special, because due to altered receptors on the fat cell Z. Jandali et al.17surface (still a subject of research), adipose tissue in lipedema behaves differently than “normal” adipose tissue—and this is undisputed.1.2.4 LipohypertrophyA widely accepted theory on the development of lipedema is that of “lipohypertro-phy.” From our point of view, this is a theory that makes sense in many patients but at the same time disregards a number of patients with atypical lipedema (Fig.1.10). Affected persons with atypical lipedema often have severe pain and above-average suffering.The theory of “lipohypertrophy” states the following: The basic prerequisite for lipedema to develop is the prior presence of lipohypertrophy. Lipohypertrophy is a multifactorial fat distribution disorder of the buttocks, hips, legs, and/or arms. Only one or several different regions may be affected. Heredity plays a central role in the development of lipohypertrophy, as do environmental influences, diet, and lifestyle habits. The approach with the estrogen receptor distribution disorder is particularly Fig. 1.10 A lipedema of an affected person not recognizable at first sight1 The LipedemaAL GRAWANY18comprehensible in lipohypertrophy. Lipohypertrophy is by definition always painless. c Fat distribution disorder (lipohypertrophy) is multifactorial; especially the hereditary component seems to play a major role. Lipohypertrophy is not accompanied by pain.Lipohypertrophy varies greatly from individual to individual and, in extreme cases, can be such that the waist is extremely slender and the legs very voluminous (Fig.1.11). From a purely external point of view, lipohypertrophy cannot be easily distinguished from lipedema.Figure 1.12 shows a typical clinical picture of lipohypertrophy.Lipedema then develops from this lipohypertrophy. Externally, it can look abso-lutely identical, but as soon as pain is added as a leading symptom to lipohypertro-phy, we speak of lipedema. In this theory, therefore, lipedema does not develop directly, but develops out of lipohypertrophy, which by definition is painless.At the other extreme are very slim women who have a barely visible fat distribu-tion disorder. Many outsiders, who are not familiar with the subject, wrongly con-demn these women. Often these women search for a perceived eternity until the correct diagnosis is made.We refer to the lipedema in these women as “atypical lipedema,” since visible lipohypertrophy is only indicated or completely absent, but the typical pain due to the adipose tissue is still present. c A fat distribution disorder simply means a disturbed distribution of fat tissue on the body and, by definition, is not associated with pain.What is the difference between lipohypertrophy and lipedema? The most impor-tant distinguishing feature is the symptomatology in terms of pain.The lady in the picture may have lipohypertrophy or she may have lipedema. Typically, these findings are often pure lipohypertrophy without pain. In this case (Fig.1.13), the affected person stated pain, which by definition makes it lipedema.Fig. 1.11 Exemplary manifestation of lipohypertrophyZ. Jandali et al.19 c If someone suffers from a disproportion in favor of buttocks, hips, legs, and/or arms without pain, then it is per se a “lipohypertrophy,” that is, a pure fat distribution disorder. c If the same person has complaints in the sense of pressure, touch, rest or stress pain, then we call this condition “lipedema.”Fig. 1.12 Clinical example of lipohypertrophyFig. 1.13 Lipohypertrophy or lipedema?1 The LipedemaAL GRAWANY20The risk that initially painless lipohypertrophy will develop into painful lipedema increases with the further development of the disproportion, that is, a further increase in fat tissue. The further increase can have different causes, for example, a furtherhormonal change with downstream metabolic changes or also a pure calorie surplus due to malnutrition can be responsible for it. We will discuss why the pain develops in Sect. 1.4.Regardless of whether or not someone suffers from a genetic predisposition to a fat distribution disorder, hormonal transition phases are often associated with weight gain. Perhaps you have experienced it yourself. You are like many women in whom pregnancy and often the onset of menopause lead to an increase in body weight. In the case of a fat distribution disorder, however, the fat tissue is then distributed dif-ferently. Thus, simply a further continuous weight gain can also lead to a further expression of the disproportion and the lipohypertrophy can turn into a painful lipedema.In Fig. 1.14, we want to show you the range of different manifestations of lipedema.In each affected person, lipohypertrophy is objectively present whether lipedema can only be determined by including the subjective “pain.”Very often we see that in the internet portals and literature a certain type of lipedema is described as “typical lipedema.” We consider such a description to be misleading. Considering the wide range of different manifestations of lipedema, it is difficult to speak of “a typical lipedema” in our view. In fact, all women in the pictures shown in Fig.1.14 complained about “pain” and thus suffer from lipedema by definition.Almost all theories agree that in lipedema individual fat cells increase. Some experts from different fields—and we do not share their opinion—postulate that there is also an increase in the total number of fat cells. Advocates of this theory also argue that the preliminary stage of lipedema should not be called “lipohyper-trophy.” Rather, the preliminary stage of lipedema should be referred to as “latency stage lipedema syndrome” (i.e., not yet erupted). We consider this view to be rather absurd since substantial scientifical evidence exists on the fact that there is no, and if only very subordinate, increase in the absolute number of fat cells in lipedema.Fig. 1.14 Different manifestations of lipedemaZ. Jandali et al.21 c If a cell (e.g., fat cell) increases in volume, we speak in the technical jargon of “hypertrophy.” If there is an increase in the absolute number of cells, we speak of “hyperplasia.”1.2.5 Theory ofMicrovascular Disruption andLymphatic InteractionTo understand the development of lipedema, two main theories must be considered: one is the theory of microvascular dysfunction, the other is the theory of lymphatic interaction.In microvascular disorders, it is assumed that an increase in fat cell volume causes an undersupply of oxygen to the tissue. The oxygen deficiency is a stimulus for new blood vessel formation because the body wants to counteract the deficiency with new vessel sprouting. This results in malformed capillaries, which should explain the tendency to hematoma.The theory of lymphatic interaction states that the lymphatic and capillary vas-cular systems act incorrectly, resulting in the formation of edema. Local messengers and degradation products produced by metabolic processes interact with the local fat cells, resulting in a slow change of the tissue. The result is hypertrophy (enlarge-ment) of the fat cell and fibrosis (proliferation and hardening due to increased col-lagen synthesis) of the connective tissue. Likewise, a chronic, subliminal inflammatory reaction of the tissue is said to play a role, which is mainly responsi-ble for fibrosis.We can confirm from our clinical observation that fibrosis does indeed occur (Fig.1.15). During liposuction procedures, we see clear differences in tissue quality between patients or even body areas and whether liposuction is “easy” or rather “laborious” during the performance.If we look at pure lymphedema, we often see an increase in subcutaneous fat tis-sue here as well, which would argue in favor of this theory.1.2.6 Uncontrolled Fat Tissue ProliferationWe often read and hear that the adipose tissue in lipedema would “proliferate.” The adipose tissue is said to virtually take on a life of its own and inexorably increase in volume, no matter what. For this book, we looked for scientific evidence of this uncontrolled growth and proliferation of adipose tissue but found no solid proof.There is not even a rudimentary basis of argumentation for this. In medicine, we are only familiar with uncontrolled growth in tumor tissue (benign or malignant tis-sue proliferation). In lipedema, however, we are not dealing with a tumor disease, but with storage of excess energy in the form of fat in fat cells. The peculiarity, in our opinion, is that in classic lipedema there is a fat distribution disorder, regardless of the possible causes, and therefore there is the well-known visual fat distribution disorder. An uncontrolled growth would be accompanied by consumption of the 1 The LipedemaAL GRAWANY22existing energy stores and not by a buildup of energy stores as in lipedema. We see this in the sad courses of advanced cancer, where the last reserves are drained from the body and it gradually undergoes degradation.There is scientific work that has observed that sufferers with lipedema may con-tinue to gain weight despite dieting and a calorie-deficient diet of around 10%. This is a very interesting statement that certainly requires further clarification. c Adipose tissue in lipohypertrophy and or lipedema does not proliferate uncontrollably in the sense of becoming independent.It is certainly quite comfortable to say that it is uncontrolled growth, especially when countless diet attempts have failed and weight gain continues to occur. It is Fig. 1.15 Clinical picture of lymphedema with malapposed lymphatic vessels on the left sideZ. Jandali et al.23not the fault of the patient, because there is a complex malfunctioning system underlying the weight gain. When this has been understood, therapy can be successful.1.3 The EdemaLet us now turn to the central point of discussion, the edema in lipedema. Colleagues, therapists and industry representatives still say today: “In lipedema, there is edema.“ Some claim this out of ignorance, others for monetary reasons because there is whole machinery behind the care of lipedema sufferers.But what actually is edema, how does it develop, and how can we detect it? Colloquially, edema is often referred to as water retention. By definition, edemas are fluid deposits in the tissue. Many people first think of lymphedema. In brief, lymphedema is caused by a pathological change in the lymphatic system, for exam-ple, due to malpositioned lymphatic channels or surgically removed lymph nodes. This causes lymph to leak into the surrounding tissue, resulting in an increase in volume in the sense of lymphedema.Depending on the disease, edema has different compositions. The main compo-nents of edema are water and proteins, in principle similar to blood plasma. If there is high protein content, we speak of protein-rich edema (so-called exudate), if there is a low protein concentration, we speak of protein-poor edema (so-called transudate).Let’s look again at lipedema and what we already know: Lipedema manifests itself in a circumscribed, symmetrically localized subcutaneous fat tissue prolifera-tion, disturbed in distribution, in favor of the buttocks, hips, legs, and arms. In addi-tion to this fat distribution disorder, edema may occur in rare cases; we then speak of lip-lymphedema or lipo-lymphedema.The term “lipedema” has held up valiantly since it was first described in 1941. Unfortunately, the term was already unfavorable at that time because the first described was orthostatic edema (edema caused by gravity, usually in women due to prolonged standing or sitting). Perhaps you are familiar with this? This widespread edema also occurs in many healthy people, especially