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The primary lymphedema is a pathological interstitial accumulation of lymphatic fluid. Lower limbs are mainly affected by primary lymphedema. It is caused by a hereditary lack or complete absence of lymphatic vessel systeme. Final complications of chronic limb lymphedema include »elephantiasis«, lymphatic ulceration and malignant degeneration. Secondary lymphedema, obesity, phlebedema and lipedema are most important differential diagnoses.Treatment of primary lymphedema includes early manual lymph drainages, decrease in weight, exercise, prevention or therapy of skin infections. Up to the present primary lymhedema cannot be cured in causality.

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.

Ich habe 2002 in Denver die Operation des Lipodems und den Vorschlag zu ihrer Standardisierung vorgestellt. Heute konnen wir die Follow-up-Untersuchungen der Patienten besprechen. Alle Patienten wurden mittels indirekter Lymphszintigraphie und indirekter Lymphangiographie vor- und nachuntersucht. Die Ergebnisse zeigen eindeutig, dass die Liposuktion beim Lipodem zu einer Normalisierung des Lymphflusses fuhren. Die Schmerzhaftigkeit des Krankheitsbildes Lipohypertrophia dolorosa wird drastisch verbessert. Manuelle Lymphdrainage und Kompression, die bislang bei diesen Patientinnen an Armen und Beinen lebenslang durchgefuhrt werden mussten, werden in der Frequenz mindestens verringert. In den meisten Fallen konnte vollig darauf verzichtet werden. Ich werde eine Variation der Operation in Lagerung und Zugangsweg vorstellen. Wir operieren heute die Arme und Beine in insgesamt zwei Sitzungen. Der Stellenwert der postoperativen manuellen Lymphdrainage und der lang andauernden Kompression durch Garments soll im besonderen noch einmal unterstreichen, dass es sich bei der Operation des Lipodems nicht um eine ausschlieslich asthetische, sondern in erster Linie um eine medizinische Indikation handelt.

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.

BACKGROUND: Lipedema is a condition characterized by diffuse, bilaterally symmetrical, painful swelling of the legs and buttocks. Microscopically, there are dermal and septal edema, adipocyte degeneration, and numerous mast cells, features held in common with lipedematous alopecia. CASE REPORT: We present the case of a 60-year-old woman with a long history of bilateral leg masses with microscopic features of lipedema. In addition, elastic-fiber changes typical of pseudoxanthoma elasticum (PXE) were discovered within the subcutaneous septa in three separate specimens obtained from an affected extremity. The patient did not have other clinical findings of PXE, although there was a history of both hypertension and congestive heart failure. CONCLUSION: This tumefactive presentation of lipedema has not been previously described. Regarding the elastic-tissue abnormalities, the patient could have either a subclinical form of PXE, perhaps predisposing to lipedema, or secondary elastic-tissue changes resulting from the massive edema. If the latter is the case, then this could represent an unusual manifestation of localized acquired cutaneous PXE (calcific elastosis).

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.

PURPOSE: To evaluate the accuracy of computed tomography (CT) scan imaging in distinguishing lymphedema from deep venous thrombosis (DVT) and lipodystrophy (lipedema) in patients with swollen legs. MATERIAL AND METHODS: CT scans of the lower limbs were performed in 55 patients with 76 swollen legs (44 lymphedemas, 12 DVT and 20 lipedemas). Thirty-four normal contralateral legs were also similarly evaluated. Primary lymphedema was verified by lymphography or lymphoscintigraphy, whereas secondary lymphedema was documented by a typical clinical history. DVT was established by ultrasound Doppler imaging. The diagnosis of lipedema was made with bilateral swollen legs where lymphoscintigraphy and Doppler examination were both unremarkable. Qualitative CT analysis was based on skin thickening, subcutaneous edema accumulation with a honeycombed pattern, and muscle compartment enlargement. RESULTS: Sensitivity and specificity of CT scan for the diagnosis of lymphedema was 93 and 100%, respectively; for lipedema it was 95 and 100%, respectively; andfor DVT it was 91 and 99%, respectively. Skin thickening was found in 42 lymphedemas (95%), in 9 DVT (75%), and in 2 lipedemas (16%). Subcutaneous edema accumulation was demonstrated in 42 legs (95%) with lymphedema and in 5 (42%) with DVT but in none with lipedema. A honeycombed pattern was present only in lymphedema (18 legs or 41%); muscle enlargement was present in all patients with DVT, in no patient with lipedema, and in 4 (9%) with lymphedema. CONCLUSION: Edema accumulation is readily demonstrated with plain CT scan and is not present in lipedema. Specific CT features of the subcutaneous fat and muscle compartments allow accurate differentiation between lymphedema and DVT.

OBJECTIVES: The purpose of this study was to define the relationships between the short saphenous vein (SSV) and the fasciae of the leg, including the muscular fascia (MF) and the membranous layer (ML) of the subcutaneous tissue. METHODS: Fascial relationships of the SSV were evaluated by means of dissection in 30 cadaveric limbs and by means of duplex sonography in 270 healthy limbs from living subjects. RESULTS: All along the leg, the SSV courses in a flat compartment delimited by the MF and the ML. Neither results from dissection nor results from sonographic examination demonstrated piercing of the MF by the SSV. A hyperechoic lamina similar to a ligament connects the SSV to the fasciae by which it is encased. An SSV tributary and collateral vessels course out of this space and are devoid of any fascial wrapping. CONCLUSIONS: The SSV does not correspond to the classical description of a "superficial" vein. In fact, from the anatomical point of view, the SSV is an interfascial vein, because it is encased by two connective fasciae, just like the greater saphenous vein. Fascial relationships of the SSV suggest that muscular contraction potentially influences the caliber and hemodynamics of the SSV. In addition, the ML is arranged as a sort of mechanical shield that could counteract dilative pathologic conditions in varicose limbs.