Lipedema consists of 5 major types, with types 1 to 3 being the most common (Table 3). It is important to note that individual patients can present with a mixture of types. Like many diseases, lipedema has a tendency to progress over time such that disease severity can be described in stages. There are currently 4 reported stages of lipedema: stage 1 involves an even skin surface with an enlarged hypodermis, stage 2 involves an uneven skin pattern with the development of a nodular or mass-like appearance of subcutaneous fat, lipomas, and/or angiolipomas, stage 3 involves large growths of nodular fat causing severe contour deformity of the thighs and around the knee, and stage 4 involves the presence of lipolymphedema.2,3
Because of their similarities, and even though lipedema is believed to be more common, it can be considered a disease along the spectrum of rare adipose disorders, or adipose tissue disorders, which includes familial multiple lipomatosis, Madelung’s disease, and Dercum’s disease.3 At present, little is known about whether each of these diseases represents a truly unique disorder or rather a variation of a common underlying molecular phenomenon of adipocyte growth and hyperplasia. From a clinical perspective, lipedema can be distinguished from familial multiple lipomatosis, Madelung’s disease, and Dercum’s disease because of the hallmark shape of the lipedema subcutaneous adipose tissue; relative sparing of the face, neck, and trunk; and fatty tumors in the subcutaneous adipose tissue that do not dominate the presentation as in the other rare adipose disorders.3,9
PATHOLOGY OF LIPEDEMA
For a disease with many unknowns, the simpler question may be, what do we know? From a histologic perspective, the initial swelling in lipedema is a result of both adipocyte hypertrophy and hyperplasia.10 In addition to enlarged adipocytes, there is thickening of the interstitium with the presence of increased interstitial fluid, secondary to elevated hydrostatic pressure.11–13 Although interstitial fluid is increased, at least in early stages, the lymphatic system seems to be functioning normally.14 Thus, the “edema” from lipedema at this stage is likely secondary to overwhelming the lymphatic pump, rather than a true dysfunction within the lymphatics themselves. However, as lipedema progresses, the lymphatic channels begin to stretch and dilate with development of many “microaneurysms” that have a tendency to leak. These leaking microaneurysms combine with the increased interstitial fluid to result in late-stage lipolymphedema.14–18
In addition to adipocyte hypertrophy, interstitial thickening, and lymphatic changes, the subdermal vascular plexus also undergoes conformational changes consistent with a microangiopathy. This microangiopathy results in capillary fragility, and leakage, which corresponds to the easy bruising and telangiectasias seen in lipedema patients.19,20 There also appears to be an influx of inflammatory cells within the interstitium, which may contribute to the signaling mechanisms involved with adipocyte hypertrophy.3
Because of its relatively unknown status in the medical community, there is a paucity of conclusive, supportive data regarding effective treatments of this disease. Based on the link between lymphatic dysfunction and adipocyte hypertrophy, and the progression to lipolymphedema in late stages of disease, it is therefore not surprising that treatments aimed at supporting lymphatic flow are useful adjuncts in managing lipedema; this includes complete decongestive therapy (which combines compression garments, manual lymphatic drainage, movement therapy, dietary recommendations, and skin care) and the use of home sequential pneumatic compression devices (eg, Flexitouch System; Tactile Medical, Minneapolis, Minn.; or Lymphapress Optimal; Lympha Press USA, Freehold, N.J.). Several literature reports have demonstrated the improvements in edema, lymph drainage, and capillary fragility with these measures.3
Although lipedema fat is resistant to lifestyle modifications, there is evidence to support the positive effects of exercise, particularly aquatic therapy, and lifestyle change on lymphedema, lymph flow, and overall health.3 Patients with late-stage lipedema, or significant pain, are often sedentary because of immobility and also subsequently develop lifestyle-induced obesity. Lifestyle modifications in these patients will therefore treat their obesity; however, the lipedema fat will remain.
As a result of the lack of psychosocial support for women with lipedema, they often suffer from psychosocial disorders, including depression, anxiety, and eating disorders. Therefore, proper counseling and treatment of these conditions are important.3
Additional medical treatment options that have had reported success in treating lipedema, or lymphedema, include beta-adrenergic agonists, corticosteroids, diuretics, flavonoids, and selenium.3 It is recommended that the use of these options be considered and managed by someone who regularly prescribes them, the patient’s primary care physician who can monitor them regularly, or a physician who is knowledgeable about lipedema and lymphedema.
From a surgical perspective, the least invasive means of removing the painful fat of lipedema is through the use of suction lipectomy. It is important to note, however, that the techniques employed for lipectomy of lipedema fat are different from the techniques used for cosmetic liposuction.21–25 Specifically, the techniques employed for lipedema liposuction utilize devices that remove fat in a gentler manner, such as the vibrating cannula associated with power-assisted liposuction or water-assisted liposuction. Reports have indicated that these methods may damage less lymphatic tissue than traditional techniques and therefore result in “lymphatic-sparing” adipose removal.22 Likewise, most surgeons treating lipedema with liposuction utilize specialized cannulas and orient the cannulas longitudinally within the lipedema subcutaneous adipose tissue to minimize potential injury to lymphatics. In addition, the volume of wetting solution used in these patients is often much less than that used in traditional liposuction. Although the literature on lipedema treatment is limited, there are several promising reports, largely from Germany and Europe, detailing the effectiveness of liposuction for the treatment of lipedema. Stutz and Krahl,22 Rapprich et al,23,24 and Schmeller et al25 have published extensive reports on the utility of their specialized liposuction techniques in not only reducing the volume of lipedema fat but also slowing progression of disease, reducing lipedema pain, reducing altered gait and loss of mobility, and improving quality of life in these patients. Likewise, in my own series of patients, I have seen similar improvements in patient’s mobility and ability to exercise without pain and to sit or stand for long periods of time without tenderness or heaviness.
The use of defined liposuction techniques and specialized pre- and postsurgical protocols in this patient population cannot be overstated, as the techniques are specifically utilized to minimize the risk of developing postoperative lymphedema.22 This is especially true in patients with late-stage lipedema who already have some clinical evidence of lymphatic compromise. In preparation for surgery, and postoperatively, it is also important to continue with lymphatic support measures, such as compression and complete decongestive therapy. These adjunctive treatments help prime the lymphatics for drainage of any postoperative edema and residual tumescent fluid utilized during the lipectomy procedure. Likewise, it is also important that patients continue with healthy lifestyle choices, including good eating habits and exercise. Although these lifestyle measures will not necessarily affect the lipedema fat, per se, they will improve the patients overall health and wellness before and after the procedures.
One of the greatest difficulties with treatment of lipedema is insurance coverage for the surgical treatment. Unfortunately, the combination of misconceptions about obesity and lipedema, along with the use of a code for suction lipectomy, which is often employed for cosmetic indications, has resulted in almost universal insurance denials. Hopefully, with increased awareness about this common disease, and also a greater understanding for the reconstructive nature of suction lipectomy techniques employed for removing lipedema subcutaneous adipose tissue, the women who suffer from lipedema will be able to receive treatment without the personal financial burden that currently exists.
Lipedema represents a common medical condition with many misconceptions. It is rarely taught in medical school and/or residency training, and as a result of this educational gap, a large population of patients suffering from this condition are forced to endure a plethora of unnecessary consultations, laboratory and radiological studies, and the psychosocial distress of being told it is their fault. With an incidence that may affect nearly 1 in 9 adult women, it is important to generate appropriate awareness, conduct additional research, and identify better diagnostic and treatment modalities so these women can obtain the care that they need and deserve.
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Copyright © 2016 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of The American Society of Plastic Surgeons. All rights reserved.
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