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Lipoedema: Improved Quality of Life by Combination of Therapies Lipoedema is a chronic progressive disease with increased fat volumes mainly of the legs, causing body disproportion together with orthostatic oedema, pain and bruising. Early diagnosis can be achieved by clinical symptoms. Complex physical therapy with manual lymphatic drainage, compression and physiotherapy reduces oedema and complaints. Surgical therapy with liposuction reduces circumscribed fat volumes, improves body proportions and diminishes oedema and bruising. Following surgery physical therapy can be diminished in intensity and number. Nowadays the combination of physiotherapy and liposuction is considered the optimal treatment regime which can tremendously improve the patient's quality of life.

Both generalized and localized edema needs to be submitted to a differential diagnostic investigation. In the case of edema affecting the lower extremities, in particular the Stemmer sign which is the inability to tent the skin at the dorsum of the toes is a useful distinguishing aid. If there is acute unilateral swelling of a leg, other processes with diffuse space-consuming processes need to be distinguished from deep venous thrombosis and secondary lymphedema. Chronic bilateral leg edema is usually due to a venous flowoff obstruction (stasis edema). Less commonly, lipedema or a primary lymphedema may be responsible for the swelling.

Painful fat syndrome or lipoedema is a distinct clinical condition, characterised by bilateral and symmetrical enlargement of the upper and lower leg with tenderness, but excluding the feet. Lipoedema occurs almost exclusively in females, and no male patient has been reported in the literature published in English. We report on an extremely rare presentation of lipoedema in a male patient. A thorough study based on the case history, physical manifestations, and magnetic resonance imaging (MRI) provided valuable clues for a differential diagnosis. Conservative treatment using weight reduction, compression-stocking application, and diuretic therapy was not effective. Tumescent liposuction with postoperative pressure garments provided a satisfactory treatment.

As the science of wound healing has evolved over the past two decades, so has awareness of the "hidden epidemic" of lymphedema. Substantial information has been accumulated regarding the pathophysiology and therapy of lymphedema. Until recently, the relationship between wound healing and the negative effects of associated peri-wound lymphedema has received little attention. Identifying wound-related lymph stasis and safe mobilization of the fluid are fundamentals that must be addressed for proper therapy. Experience gained from the successful treatment of primary and secondary lymphedema has proven very useful in the applications to wound-related lymphedema. The mobilization of lymph fluid from the peri-wound area with the use of reasoned compression is essential for proper therapy of the open wound, as are appropriate bandage selection and safeguards for bandage application.

HYPOTHESIS: The causes and management of lower limb lymphedema in the Western population are different from those in the developing world. OBJECTIVE: To look at the differential diagnosis, methods of investigation, and available treatments for lower limb lymphedema in the West. DATA SOURCE: A PubMed search was conducted for the years 1980-2002 with the keyword "lymphedema." English language and human subject abstracts only were analyzed, and only those articles dealing with lower limb lymphedema were further reviewed. Other articles were extracted from cross-referencing. RESULTS: Four hundred twenty-five review articles pertaining to lymphedema were initially examined. This review summarizes the findings of relevant articles along with our own practice regarding the management of lymphedema. CONCLUSIONS: The common differential diagnosis in Western patients with lower limb swelling is secondary lymphedema, venous disease, lipedema, and adverse reaction to ipsilateral limb surgery. Lymphedema can be confirmed by a lymphoscintigram, computed tomography, magnetic resonance imaging, or ultrasound. The lymphatic anatomy is demonstrated with lymphoscintigraphy, which is particularly indicated if surgical intervention is being considered. The treatment of choice for lymphedema is multidisciplinary. In the first instance, combined physical therapy should be commenced (complete decongestive therapy), with surgery reserved for a small number of cases.

Venous oedema, which occurs almost exclusively in the legs, develops due to increased venous pressure following valvular insufficiency, and is marked by blue discolouration, varicosities and, in chronic forms, by brown discolouration of the skin resulting from the deposition of haemosiderin. It is possible to confirm the condition by phlebological instrumental diagnosis. Primary lymphoedema also almost always occurs in the legs. It is caused by underdevelopment of lymphatic vessels, but the skin colour remains normal. It is recognisable by Stemmer’s sign and characteristic thickening of the skin over the toes resulting from subcutaneous protein fibrosis. Lymphoscintigraphy may be required for diagnosis in rare cases. Venous oedema and lymphoedema are found in both sexes, uni- or bilaterally. If bilaterally then usually asymmetrical. In contrast, lipoedema occurs in women only, thickening is always symmetrical, and the skin has a normal colour as in lymphoedema. The predisposing condition for lipoedema is lipohypertrophy of the extremities, a congenital accumulation of adipose tissue in the extremities that results in a disproportionate physical form with a relatively slim trunk. In one third of cases lipoedema is also observed in the arms. In contrast to lymphoedema, the hands and feet characteristically remain free of thickening and oedema.

Diagnosis and treatment of the <<Lipedema Syndrome>> are now as ever connected with problems for the general physcician, but also for the experts in lymphology. Numerous open questions regarding etiology, diagnosis, and course of the disease as new therapetic concepts have lead to the idea, to include that subject again as one of the main topics into the program of the annual meeting of the German Society of Lymphology

15% of the patients of a special clinic for lymphological diseases had a lipedema. In 97% it was located in the legs and in 31% also in the arms. In 66% it was located only in the legs and in 3% only in the arms. Combinations of lipedema of the leg with phlebedemas have been seen in 2% and with a lymphedema in 1%. The differential diagnosis to lipohypertrophy, adiposis and lymphedema is given. The therapy with liposuction and physical therapy of edema, combination of manual lymphatic drainage and compression, will be discussed.

Early terms of lymphostasis in lipedema can be detected with lymphoscintigraphy. A normal examination almost certainly excludes a lymphatic component. Indirect lymphography is only used to rule out morphological abnormalities of lymph vessels. If a lymphoscintigraphic study is normal indirect lymphography is not indicated.

The "Lipedema" or "Fatedema" is conditioned by a slight mechanical obstruction of the small lymphatic vessels by the increasing pressure of the growing fat tissue. This lymphostasis in a normal lymphatic vessel system arises only with women and always symmetrically and conducts to typical complaints. Therapeutically, apart from loss in weight, only lymph drainage therapy is in a position to remove the complaints of edema.

In a review of 250 cases of lymphedema of the lower extremity, 9 patients were noted to share unique similarities in their history and physical findings. Although these patients had mild swelling in their pretibial areas and were all referred with a diagnosis of lymphedema of the legs, their findings differed significantly from the usual patient with either congenital or acquired lymphedema. Notably, the lower extremity swelling was always bilateral and symmetrical in nature and never involved the feet. Skin changes characteristic of lymphedema were not found, and consistent fat pads were present anterior to the lateral malleoli in each patient. These findings are representative of a clinical entity known as lipedema, which is distinct from lymphedema and for which treatment may be different.