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Aim: In pathophysiology of lipoedema, almost exclusively seen in women, lymphatic insufficiency might play a significant role. However, little is known about the pathophysiology of these abnormal localized depositions of body fat. We studied the involvement of the lymphatic system in lipoedema of the type Allen-Hines as well as of Typus Rusticanus Moncorps. Patients, methods: The standard (epifascial pathway) and a modified method (subcutaneous pathway) of lymphoscintigraphy was carried out with 28 patients suffering from lipoedema. Uptake percentages normalized to the injected dose were used as functional quantitative parameters. Visual assessment of both studies were done and scored. Patients with oedema of the legs because of venous insufficiency (Widmer stage II) served as a control group. Results: All patients of the control group and all patients with lipoedema of Typus Rusticanus Moncorps showed a normal standard lymphoscintigraphic study by visual scoring as well as by quantitative outcome. Lymph transport from the subcutaneous fat tissue was significant higher (p <0.012) in the group of patients with lipoedema diagnosed as type Allen-Hines than in Typus Rusticanus Moncorps. Conclusion: Epifascial lymph drainage in patients with lipoedema is not significantly disturbed. However, subcutaneous lymphatic drainage significantly differed in patients with lipoedema of type Rusticanus Moncorps in comparison with type AllenHines hinting at a differing lymphatic pathophysiology.

"Lipedema," a special form of obesity syndrome, represents swelling of the legs due to an increase of subcutaneous adipose tissue. In 12 patients with lipedema of the legs and in 12 healthy subjects (controls), fluorescence microlymphography was performed to visualize the lymphatic capillary network at the dorsum of the foot, at the medial ankle, and at the thigh. Microaneurysm of a lymphatic capillary was defined as a segment exceeding at least twice the minimal individual diameter of the lymphatic vessel. In patients with lipedema, the propagation of the fluorescent dye into the superficial lymphatic network of the skin was not different from the control group (p > 0.05). In all 8 patients with lipedema of the thigh, microaneurysms were found at this site (7.9 +/- 4.7 aneurysms per depicted network) and in 10 of the 11 patients with excessive fat involvement of the lower leg, multiple microlymphatic aneurysms were found at the ankle region. Two obese patients showed lymphatic microaneurysms in the unaffected thigh and in only 4 patients were microaneurysms found at the foot. None of the healthy controls exhibited microlymphatic aneurysms at the foot and ankle, but in one control subject a single microaneurysm was detected in the thigh. Multiple microlymphatic aneurysms of lymphatic capillaries are a consistent finding in the affected skin regions of patients with lipedema. Its significance remains to be elucidated although its occurrence appears to be unique to these patients.

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.

Lipedema never reveals clinical picture of extreme lymphedema-elephantiasis, and skin signs and complications have not been observed. Aim of this paper is to present a case of lipedema with the initial lymphedema in which, after one episode of lymphangiitis and cellulitis, came to the rapid development of lymphedema followed by chyloderma. During the local treatment of extreme chyloderma with excessive exudation, semiocclusive synthetic dressings have been used for moist wound healing. The treatment was completed after 20 weeks with total epithelizsation, without maceration and irritation, without additional spreading of the chyloderma field, without wound infections, with fast and full relief of the pain. Lipedem with extreme lymphedema can be followed by skin complications of lymphedema like chylodermia.

We describe a 52 year-old woman in whom lymphedema primarily of the abdominal wall was superimposed on lipedema resulting in an abdomen of enormous dimensions with marked impairment of ambulation. Treatment consisted of preoperative compression of the legs by an external pneumatic device (Lympha-Press) followed by excision of the lymphedematous abdominal fat pad in conjunction with "debulking" of the right leg. The patient illustrates the extremes of lipedema complicated by lymphedema and the technical difficulties associated with its management.

In order to develop further the criterias of the ultrasonographic diagnosis of the lip- and lymphedema, also in the demarcation of the phlebedema, we examined 100 patients in our practice. In addition to the specific “sonomorphology”of the lip- and lymphedema we found a different reaction of the subcutaneous tissue of the lower leg in painful and not painful lipedemas examined with compression-sonography. A more precise demarcation of the cutis from the subcutis was made possible with a new 13 MHz linear probe. The spontaneous painful lipedema shows a thickened subcutis with increased echogenity and is only compressible at 10 to 20 percent, while the not painful lipedema is compressible at about 50 percent. In patients with lymphedema the typical echoless gaps showed no colour coding and were also not compressible. Especially because of the low time needed, the non-invasive method and the lack of radiation the ultrasonographic description, measurement and compression of the subcutaneous tissue of the lower leg is useful for diagnosis, the objective control of therapeutic strategies and the estimation of the clinical symptoms – especially in cases of lipedema – and also for the etiology – especially in cases of lymphedema. The sonography is superior to the clinical examination and should be the second step in diagnosis of all nonsystemic forms of edemas of the legs.

The anatomical and functional status of the epifascial and subfascial lymphatic compartments was analyzed using two compartment lymphoscintigraphy in five groups of patients (total 55) with various forms of edema of the lower extremities. Digital whole body scintigraphy enabled semiquantitative estimation of radiotracer transport with comparison of lymphatic drainage between those individuals without (normal) and those with leg edema by calculating the uptake of the radiopharmaceutical transported to regional lymph nodes. A visual assessment of the lymphatic drainage pathways of the legs was also performed. In patients with cyclic idiopathic edema, an accelerated rate of lymphatic transport was detected (high lymph volume overload or dynamic insufficiency). In those with venous (phlebo) edemas, high volume lymphatic overload (dynamic insufficiency) of the epifascial compartment was scintigraphically detected by increased tracer uptake in regional nodes. In patients with deep femoral venous occlusion (post-thrombotic syndrome). subfascial lymphatic transport was uniformly markedly reduced (safety valve lymphatic insufficiency). On the other hand, in the epifascial compartment, lymph transport was accelerated. In those patients with recurrent or extensive skin ulceration, lymph transport was reduced. Patients with lipedema (obesity) scintigraphically showed no alteration in lymphatic transport. This study demonstrates that lymphatic drainage is notably affected (except in obesity termed lipedema) in various edemas of the leg. Lymphatic drainage varied depending on the specific compartment and the pathophysiologic mechanism accounting for the edema. Two compartment lymphoscintigraphy is a valuable diagnostic tool for accurate assessment of leg edema of known and unknown origin.

The anatomical and functional status of the epifascial and subfascial lymphatic compartments was analyzed using two compartment lymphoscintigraphy in five groups of patients (total 55) with various forms of edema of the lower extremities. Digital whole body scintigraphy enabled semiquantitative estimation of radiotracer transport with comparison of lymphatic drainage between those individuals without (normal) and those with leg edema by calculating the uptake of the radiopharmaceutical transported to regional lymph nodes. A visual assessment of the lymphatic drainage pathways of the legs was also performed. In patients with cyclic idiopathic edema, an accelerated rate of lymphatic transport was detected (high lymph volume overload or dynamic insufficiency). In those with venous (phlebo) edemas, high volume lymphatic overload (dynamic insufficiency) of the epifascial compartment was scintigraphically detected by increased tracer uptake in regional nodes. In patients with deep femoral venous occlusion (post-thrombotic syndrome). subfascial lymphatic transport was uniformly markedly reduced (safety valve lymphatic insufficiency). On the other hand, in the epifascial compartment, lymph transport was accelerated. In those patients with recurrent or extensive skin ulceration, lymph transport was reduced. Patients with lipedema (obesity) scintigraphically showed no alteration in lymphatic transport. This study demonstrates that lymphatic drainage is notably affected (except in obesity termed lipedema) in various edemas of the leg. Lymphatic drainage varied depending on the specific compartment and the pathophysiologic mechanism accounting for the edema. Two compartment lymphoscintigraphy is a valuable diagnostic tool for accurate assessment of leg edema of known and unknown origin.

Lipoedema is a common but infrequently recognized condition causing bilateral enlargement of the legs in women. Although generally considered to be the result of an abnormal deposition of subcutaneous fat with associated oedema, the precise mechanisms responsible for oedema formation have yet to be fully established. In order to evaluate the possible role of lymphatic or venous dysfunction in the pathogenesis of lipoedema, 10 patients were investigated by photoplethysmography (venous function) and quantitative lymphoscintigraphy (lymphatic function). The results were compared with those from patients with primary lymphoedema and those from healthy volunteers. The results demonstrated minor abnormalities of venous function in only two patients. One patient had moderately impaired lymphatic function in both legs and seven patients had a marginal degree of impairment in one or both legs. However, in none of these cases did the impairment attain the low levels seen in true lymphoedema. Lipoedema appears to be a distinct clinical entity best classified as a lipodystrophy rather than a direct consequence of any primary venous or lymphatic insufficiency.

PURPOSE: Lymphoscintigraphy has emerged as the diagnostic test of choice in patients with suspected lymphedema. To assess the lymphatic circulation of 386 extremities in 188 patients, we prospectively recorded a semiquantitative index of lymphatic transport in addition to visual evaluation of lymphoscintigraphy image patterns. METHODS: Sixty-one male and 127 female patients were studied (mean age 48 years, range 13 to 87 years). Twenty had upper extremity swelling, and 168 had lower extremity swelling. The disease was bilateral in 60 patients. Lymphoscintigraphy was performed by injecting a mean of 503 microCi of technetium 99m-antimony trisulfide colloid subcutaneously into the second interdigital space of the extremity. Time for transport to regional lymph nodes, appearance of lymph vessels and nodes and distribution pattern were scored. These scores were compiled into a modified Kleinhans transport index (TI). To assess the venous circulation, 155 patients underwent evaluation of the venous system by impedance plethysmography, ultrasonography, or contrast venography. RESULTS: The mean TI (+/- SEM) in 79 asymptomatic extremities was 2.6 +/- 0.5, with 66 (83.5%) demonstrating normal lymphoscintigraphy pattern (TI < 5). Patients with clinical diagnosis of lymphedema (n = 124) had a mean TI of 23.8 +/- 1.5; 81.5% of these were greater than 5. Fifty-six patients (30%) had primary and 68 (36%) had secondary lymphedema. (TI of 26 +/- 3.5 and 22.1 +/- 1.9, respectively, p = NS). Patients without any lymphatic transport (TI of 45) were more likely to have cellulitis in their history (p < 0.05). Contrast lymphangiography in six patients correlated with lymphoscintigraphy. Sixty-four patients (34%) had swelling without lymphedema (venous edema, cardiac edema, lipedema, etc.; TI of 1.9 +/- 0.4, p < 0.001). Of the 41 patients with abnormal venous studies, 18 (44%) had an elevated TI. CONCLUSIONS: Semiquantitative evaluation of the lymphatic transport with lymphoscintigraphy reliably depicts abnormalities in the lymphatic circulation. Lymphoscintigraphy excluded lymphedema as a cause of leg swelling in one third of our patients.

The authors assessed the use of magnetic resonance imaging in differentiating lymphedema, phlebedema, and lipedema of the lower limb. They examined 14 patients: five with lipedema, five with lymphedema, and four with phlebedema. T1- and T2-weighted transaxial sequences were performed before administration of gadolinium tetraazacyclododecane-tetraacetic acid (DOTA) and T1-weighted spin-echo sequences were performed after administration of Gd-DOTA in each patient. Images of patients with lipedema showed homogeneously enlarged subcutaneous layers, with no increase in signal intensity at T2-weighted imaging or after Gd-DOTA administration. Patients with phlebedema had areas containing increased amounts of fluid within muscle and subcutaneous fat. In lymphedema, a honeycomb pattern above the fascia between muscle and subcutis was observed, with a marked increase in signal intensity at T2-weighted imaging. After Gd-DOTA administration, there was only a slight increase in signal intensity in the subcutis in lymphedema and phlebedema and a moderate increase in signal intensity in muscle in phlebedema.

The use of a single axial slice through the mid calf in the differential diagnosis of a swollen leg is described. This is a very simple quick non-invasive investigation. Venous obstruction results in an increase in the cross sectional area of the muscle compartment. The subcutaneous fat layer is normally homogeneous; in obesity or lipoedema it is increased but remains homogeneous. In lymphoedema fluid collects in the interstitial spaces which become very prominent on CT images. In chronic lymphoedema a honeycomb pattern is seen as a result of increase in the interstitial tissue due to fibrosis. Popliteal cyst extensions result in fluid collections between muscle planes. Haematomas have higher attenuation, and are intramuscular. The findings in 64 patients and 10 controls are presented and the literature is reviewed.

Twelve patients with primary lymphedema of the lower limb were examined with computed tomography (CT). A characteristic "honeycomb" pattern of the subcutaneous compartment was seen in 10 of these patients. CT scans in nine other patients with swollen leg secondary to chronic venous disease or lipedema did not show this characteristic pattern. CT may be helpful in the differential diagnosis of a swollen leg, thus obviating venography or lymphangiography.

To the Editor.— I do not believe that Stallworth et al (228:1656, 1974) have proven their case for a definite clinical entity of "lipedema" in the patients described.The plasma lipid values seem high, but are of no significance unless the controls were patients of comparable sex, age, and mean weight; this is not stated. The origin of tissue lipid values would similarly have to be a group of middle-aged, overweight patients with fat legs but no symptoms, to make their figures valid.I have seen and treated many patients with a clinical picture identical with that described in their paper. Many of these patients have clinical evidence of past phlebitis; others do not. Both groups have episodes of painful, lumpy swellings in their legs, that may be accompanied by redness, heat, and tenderness in the affected areas. This picture frequently takes many weeks to resolve and may leave some ...