WHAT YOU NEED TO KNOW
Lipoedema: clinical aspects and treatment.
Thomas WITTE MD, Falk-Christian HECK MD.
LipoClinic Dr. Heck, Zeppelinstr. 321, D - 45470 Mülheim an der Ruhr, firstname.lastname@example.org
Lipoedema is a high-impact demographic disease whose characteristics have often been highlighted.
Liposuction is - independent of the health insurance company's judgement - the only method that can reduce pathological fatty tissue, providing patients with great and lasting benefits.
Keywords: lipoedema, liposuction, quality of life, LTA (local tumescent anaesthesia), WAL (Waterjet Assisted Liposuction).
Lipoedema was first named in 1940 in the medical literature by Allen and Hines (1).
The disease is now fully recognized and listed in the WHO International Classification of Diseases (ICD) with its own code (E88.xx) (20).
Today, the register in medical databases is long when searching for lipoedema.
The "Deutsche Gesellschaft für Phlebologie" (German Society for Phlebology) has even developed its own guidelines on the subject. (8).
The causes of the disease have not yet been fully explored.
- To summarize, it is assumed that the female hormone estrogen, due to increased production of growth factors, leads to an increase and enlargement of fat cells (29,30).
- The large increase in fat cells leads to a lack of oxygen in the tissue, so that some of the fat cells die (31).
This in turn leads to chronic inflammation, which in turn leads to tissue damage.
-In addition, there is a higher permeability of the vessel wall, which allows more lymph to pass through the tissue, which can no longer be evacuated by the lymphatic system that has remained intact (called insufficiency due to the large volume of lymph to be evacuated).
- This leads to the typical feeling of pressure and tension felt by patients.
- In addition, due to the increased sensitivity of the pain receptors, the tissue changes and causes spontaneous pain, which patients often complain about.
- There is also a tendency for haematomas induced by a high fragility of the capillary vessels (35).
It is assumed that the prevalence in the female population is 8-17% (11,18,23).
For France alone, this corresponds to a total of about 5 million women affected.
The disease affects men only very rarely.
And is associated in most cases with hormonal imbalance.
Currently, there are only a few individual reports on this subject (4,10).
Very often, it is a coincidence that leads to the diagnosis of lipoedema.
- Patients recognize their symptoms, for example, through television reports and comments from those around them about the strange appearance of their legs.
- Although the disease has been known for 80 years, knowledge about lipedema is still very underdeveloped in many places.
- Even in countries such as France, Germany, Spain or Belgium, there are only a few specialists dedicated to the subject.
The main symptom is pain (33).
- Pain from pressure, touch, spontaneous, tension or movement can occur simultaneously or independently of each other.
- The tendency to non-traumatic hematomas persists (5,28).
- The physical transformation of the arms and legs also promotes stigmatization of patients.
- It appears symmetrically and bilaterally.
- Normally, the feet and hands are not affected.
- Initially, there is no dimpling of the Achilles heel (10).
During the course of the disease, the legs develop into a post with a clear demarcation at the wrists and ankles. A pronounced fat sleeve may appear at these places (Photo N.1) as well as small fat deposits typical of the malleolus (10,13). Very often, a fatty area below the knee develops.
- At a later stage, the altered morphology and weight gain in the legs can lead to deformity of the lower limb joints and arthrosis (Fig. N. 2) (39).
- At an early stage, there is an increase and concentration of fat in the arms.
- Later, there may be very pronounced deposits in the elbow and forearm, with the exception of a triangular area at the wrist on the inside.
The stage of the disease course is determined by the clinical appearance.
Three stages are defined. As the course is very individual, it is not possible to make a prognosis. (7).
Photo N. 1 Typical post legs with pronounced formation of greasy sleeves at the ankles.
Photo N. 2 Deformation of joints (genu valgum) due to long-standing lipoedema.
In contrast to obesity, lipoedema does not respond at all or very much to attempts to lose weight (1,13,34).
Stemmer's sign (skin of the second toe no longer folds when pinched), which represents the boundary between lymphedema and lipolymphedema, is negative (13,37,42).
Currently, there are no devices available to reliably diagnose lipoedema. Ultrasound, magnetic resonance imaging (MRI), or computerized tomography, can only reveal large or condensed fat deposits (22,8,25,3,27).
The diagnosis is made using clinical criteria (21,33).
Even the histological analysis of pathological cells is not specific, as Stutz showed in 2009 on the basis of 30 patients with lipoedema who underwent liposuction using the WAL method (40).
The packaging was primarily composed of lipocytes. In the case of pathological subcutaneous fat cell multiplication, it is not clear whether it is adipocyte hypertrophy, hyperplasia, or a combination of both (33).
The clinical diagnosis is based on the history, which shows that lipoedema occurs most often at puberty, early pregnancy or menopause (1,43). Very often, it is a positive family history, although the literature speaks of a range from 16 to 64%, thus with relative reliability (1,16,38). In 97% of cases, it is the legs that are affected and in 31% the arms (19). In our opinion, both areas, legs and arms, are always affected, but the development in the arms is often less or occurs later.
As with diagnosis, there are still few centers specializing in the treatment of lipedema. This leads to results of very different quality. In order to obtain the best possible result, it is advisable to refer patients to a center specialised in lipoedema surgery.
For many years it was considered the best treatment option (12,19,41) the combination of :
- physical decongestion through the use of straight, flat-knit compression garments,
- the practice of manual lymphatic drainage
- weight normalization
- and if possible to supplement with a domestic intermittent compression device.
These measures mainly lead to a reduction in oedema, but do not reduce pathological fat cells. Although physical decongestion is recommended in existing guidelines, there are, however, no evidence-based studies (7,41).
Patients' quality of life is greatly reduced (9) because they must:
- wear custom-made compression garments for life,
- and do regular manual lymphatic drainage.
Since 2004, liposuction in the treatment of lipoedema has been practiced with great success (23,32).
In this area, the 30-year-old LTA method (local tumescent anaesthesia) is increasingly being replaced by the more modern WAL method (water jet assisted liposuction).
Comparative studies show that the WAL method causes less pain and swelling and that the patient can return to work more quickly.
In order to work efficiently and ensure maximum patient safety, 2 to 3 operations are required for the lower limbs. The arms are normally done in one operation.
The number of complications following this method using a standardized protocol is very low, in the order of 1-2%, (36).
Standardization of treatment allows very good results to be reproduced (19) (Abb. 3 and 4). Our team was the first in the world to publish in 2018 a standardized operating protocol that includes not only the operation, but also the pre- and post-operative follow-up.
- For example, to relieve tissue congestion in preparation for the operation and in the postoperative period, it is imperative that patients wear straight, flat-knit compression tights.
- Only these compression garments - thanks to their dynamic properties - contribute to active tissue decongestion.
- Liposuction of the legs and arms can be performed in 3 or 4 operations.
This depends on the starting weight of the patient, the amount of fat and the distribution of fat cells.
When the volume to be removed in the thighs and buttocks is too large, then 2 operations are required.
- For this purpose we only operate using the WAL technique (water jet assisted liposuction), which is currently the most modern method in the treatment of lipoedema.
- In separate operations, the legs, thighs and buttocks as well as the arms are decompressed in a circular fashion. (Photos N° 3,4,5,6,7).
Multiple studies confirm the effectiveness of liposuction in the treatment of lipoedema.
Dutch guidelines state that abnormal fat tissue can only be removed by surgery (15). In the "S1" guidelines of the German Society of Phlebologysurgery is named as a treatment option for all stages of lipoedema (7).
Other studies not only confirm the benefits of liposuction but also show positive long-term effects (2,5,26,36,44). In summary, all studies show a significant reduction in pain, on average 70%.
All the studies done so far are monocentric and non-randomized studies.
The quality of life of patients who have undergone liposuction to treat lipoedema has increased significantly.
- This is what Frambach says in a study of 164 patients reviewed in consultation between 4 and 8 years after the operation (14). Most of the painful symptoms had disappeared, as had the feeling of pressure, the tendency to oedema and reduced mobility. In addition, there was no increase in subcutaneous fat tissue in these patients.
- Our own study, which is still ongoing, confirms the very good results and highlights improvements in various criteria (pain, swelling, reduced quality of life or work capacity, etc.) in 77% to 96% of cases following liposuction with the WAL method.
- Finally, liposuction can help eliminate the need to wear compression garments and lymphatic drainage (36).
Photo N.3 Stage II lipoedema in the legs prior to surgery.
Photo N.4. After leg lipo-decompression. A total of 15 liters of fat removed in 3 operations.
Today - both in Germany and in France - cost coverage by the health insurance company is only carried out in exceptional cases, while the financial possibilities of patients are becoming increasingly restricted.
In Germany, a prospective, multicenter, randomized study is currently underway to prove the benefits of liposuction.
The results will not be known until 2024. Only then will it be decided whether liposuction is covered by the health insurance company or not.
Photo N. 5 Stage II lipoedema.
Beginning of the operation before the injection of the tumescent solution.
Photo N. 6 Status at the end of the operation.Liposuction of 2.3 l of pure fat.
Photo N. 7 WAL technical.
Thanks to the jet of water coming out of the cannula, the fat cells are lifted off in
softness of the fabrics. In the same gesture, the pathological cells then detached
are sucked through the other opening of the same cannula.
Allen EU, Hines EA: Lipedema of the legs: A syndrome characterized by fat legs and orthostatic edema. Proc Staff Mayo Clin 1940; 15: 184-7.
Baumgartner A: Long-term benefit of liposuction in patients with lipoedema: a follow-up study after an average of 4 and 8 years. Br J Dermatol 2016; 174 (5): 1061-1067.
Breu FX et al: Neue Ergebnisse der duplexsonographischen Diagnostik des Lip- und Lymphödems. Kompressionssonographie mit einer neuen 13-MHz-Linearsonde. Phlebologie 2000; 29: 124-175.
Chen, S. G., Hsu, S. D., Chen, T. M., Wang, H. J. Painful fat syndrome in a male patient. Br J Plast Surg 2004; 57(3):282-286.
Cornely ME: Dicker durch Fett oder Wasser - Lipohyperplasia dolorosa vs. Lymphödem. Hautarzt 2010; 61: 873 - 879
Dadras M, Mallinger PJ, Corterier CC, Theodosiadi S, Ghods M: Liposuction in the Treatment of Lipedema: A Longitudinal Study. Arch Plast Surg 2017; 44: 324-331.
Deutsche Gesellschaft für Phlebologie: S1-Leitinie Lipödem 10/ 2015. awmf.org
Dimakakos PB et al: MRI and ultrasonografic findings in the investigation of lymphoedema and lipoedema. Int Surg 1997; 82: 411-416.
Dudek JE, Bialaszek W, Ostaszewski P: Quality of life in woman with lipoedema: a contextual behavioral approach. Quality of Life Research 2016; 25 (2): 401-408.
Fife CE et al: Lipedema: A frequently misdiagnosed and misunderstood fatty deposition syndrome. Adv Skin Wound Care 2010; 23: 81-92.
Földi, E., and Foldi, M. (2006) Lipedema. In Földi's Textbook of Lymphology (Foldi, M., and Földi, E., eds) pp. 417-427, Elsevier GmbH, Munich, Germany.
Földi M, Földi E, Kubik S. Lehrbuch der Lymphologie. Stuttgart, New York: Gustav Fischer 2005.
Forner-Cordero I et al: Lipedema: an overview of its clinical manifestations, diagnosis and treatment of the disproportional fatty deposition syndrome - systematic review. Clinical Obesity 2012; 2: 86-95.
Frambach, Y et al. Long-term results of liposuction in patients with lipedema. Presented at: 23rd World Congress of Dermatology; Vancouver, British Columbia 2015 June: 8-13.
Halk AB: First Dutch guidelines on lipedema using the iternational classification of functioning, disability and health. Phlebology 2016.
Harwood CA et al: Lymphatic and venous function in lipedema. Br J Dermatol 1996; 143: 1-6.
Heck FC, Witte T: Standards in der Lipödemchirurgie. CHAZ (2018) 19: 320-325.
Herpertz, U: Krankheitsspektrum des Lipödems an einer Lymphologischen Fachklinik - Erscheinungsformen, Mischbilder und Behandlungsmöglichkeiten. vasomed 1997; 301-307
Herpertz U. Ödeme und Lymphdrainage. Diagnose and Therapy. Lehrbuch der Ödematologie. 5. Aufl. Stuttgart: Schattauer 2014.
ICD-10-GM Version 2017 Onlineversion; dimdi.de
Kröger K: Lymphoedema and lipoedema of the extremities. Vasa 2008; 37: 39-51.
Marshall M, Schwahn-Schreiber C: Lymph-, Lip- und Phlebödem. Differenzialdiagnostische Abklärung mittels hochauflösender Duplexsonographie. Gefässchirurgie 2008; 3: 204-212.
Meier-Vollrath I, Schmeller W: Lipoedema - current status, new perspectives. J Dtsch Dermatol Ges 2004; 2 (3): 181-186.
Meier-Vollrath, I., Schneider, W., and Schmeller, W. (2005) Lipödem: Verbesserte Lebensqualität durch Therapiekombination. Dtsch Ärzteblatt 102, A1061-1067.
Monnin-Delhom ED et al: High resolution unenhanced computed tomography in patients with swollen legs. Lymphology 2001; 35: 121-128
Rapprich S: Liposuction is an effective treatment for lipedema - results of a study with 25 patients. J Dtsch Dermatol Ges 2011; 9 (1): 33-40.
Reich-Schupke S, Altmeyer P, Stücker M: Thick legs - not always lipedema. J Ger Society Dermatol 2012.
Shin BW: Lipedema, a rare disease. Ann Rehabil Med 2011; 35: 922-927.
Szel E, Kemeny L, Groma G, Szolnoky G: Pathophysiological dilemmas of lipedema. Med Hypotheses 2014; 83 (5): 599-606.
Siems W, Gune T, Voss P, Brenke R: Anti-fibrosclerotic effects of shock wave therapy in lipedema and cellulitis. Biofactors 2005; 24: 275-282.
Suga H, Araki J, Aoi N et al: Adipose tissue remodeling in lipedema: adipocyte death an concurrent regeneration. J Cutan Pathol 2009; 36: 1293-1298.
Schmeller W, Meier-Vollrath I: Tumescent liposuction: a new and successful therapy for lipedema. J Cutan Med Surg 2006; 10 (1): 7-10.
Schmeller W, Meier-Vollrath I: Lipödem - Aktuelles zu einem weitgehend unbekannten Krankheitsbild. Akt Dermatol 2007; 33: 1-10.
Schmeller W, Meier-Vollrath I: Pain in lipedema - an approach. LymphForsch 2008; 12: 7-11.
Schmeller W, Meier-Vollrath I: Lipödem - Moderne Diagnostik und Therapie. Gefäßchirurgie 2009; 14: 516-522.
Schmeller W: Tumescent liposuction in lipoedema yields good long-term results. Br J Dermatol 2012; 166 (1): 161-168.
Stemmer R, Stemmer's sign-possibilities and limits of clinical diagnosis of lymphedema. Wien Med Wochenschr. 1999; 149 (2-4): 85-6.
Strößenreuther RHK: Lipödem und andere Erkrankungen des Fettgewebs. Viavital Verlag 2009, Köln.
Stutz J: Liposuction of Lipedema for Prevention of Later Joint Complications. Vasomed Journal 2011; 23: 62-66.
Stutz JJ: Water jet-assisted liposuction for patients with lipoedema: histologic and immunohistologic analysis of the aspirates of 30 lipoedema patients. Aesthetic Plast Surg 2009; 33 (2): 153-162.
Wagner S: Lymphedema and lipedema - an overview of conservative treatment. Vasa 2011; 40: 271-297.
Warren Peled A, Kappos EA: Lipedema: diagnosis and management challanges. Int J Womens Health 2016; 8: 389-395.
Wienert V, Földi E et al: Lipoedema guidelines of the German society for Phlebology. Phlebologie 2009; 38: 164-167.
Wollina U, Heinig B: Treatment of lipedema by low-volume micro-cannular liposuction in tumescent anesthesia: Results in 111 patients. Dermatologic Therapy. 2019;32:e12820.