Monomelic macrodystrophia lipomatosa. A case report.
There are many reports of macrodystrophia lipomatosa involving the hands or feet, but here we report an unusual case where the whole of the left lower limb was affected.
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There are many reports of macrodystrophia lipomatosa involving the hands or feet, but here we report an unusual case where the whole of the left lower limb was affected.
The development of tendolipomatosis has been studied both by light and scanning electron microscopy in the tendons of healthy persons killed in accidents. Lipid cells are rarely seen in the intact tendon. In the early stages of tendolipomatosis lipid cells are found deep and singly but in the later phases lipocytes are seen to disrupt the continuity of collagen fibres and tendon bundles and so reduce the strength of the tendon.
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A 20-month-old girl showed typical clinical signs of Farber disease: hoarseness since birth, and periarticular subcutaneous painful nodules. Complete deficiency of acid ceramidase activity was found in cultured skin fibroblasts. An electron microscopic examination of a dermal nodule disclosed pathognomonic tubular inclusions in histiocytes. In epidermal cells zebra-body-like and needle-like lysosomal inclusions were found. Their ultrastructure is different from that of the intrahistiocytic lysosomal inclusions. Probably three clinical types of Farber disease may be distinguished according to the symptomatology and the course of the disease: a severe type, an intermediate type and a relatively mild type. The activity of acid ceramidase does not correlate with prognosis of the disease, while a correlation between first appearance of dermal nodules and clinical course appears likely.
We examined 62 patients (72 hands) with carpal tunnel syndrome (CTS) by magnetic resonance imaging (MRI) of the carpal tunnel and latency measurements of the median nerve. In 32 of 72 hands a probable causative lesion of the CTS was identified by MRI, for example tenosynovitis, a cyst-like structure, or an aberrant muscle. The MRI findings were confirmed by surgery in 16 of 24 hands, slightly corrected in 5, and not substantiated in 3. In 65 of 72 hands, MRI disclosed pathology of the median nerve, most prominently an enlargement of the nerve at the level of the os pisiforme, a finding not seen during surgery. Oedema of the nerve was found in 14 of 72 hands. The distal latencies were prolonged in 62 of 72 hands. The sensory latencies correlated significantly with the MRI-determined cross-sectional area of the nerve at the level of the distal radius. The lack of other correlations suggests that partly independent features of the nerve lesion are demonstrated in each method or that the sensitivity and specificity of both methods are limited. Further experience with MRI in CTS is desirable. At present, the practical use of MRI in CTS should be restricted to special diagnostic problems such as carpal tunnel syndromes which do not respond adequately to conservative or surgical treatment.
A patient complaining of epigastric distress was found to have multiple gastroduodenal lipomas. Although the roentgenologic findings were both bizzare and initially confusing, close scrutinization of the abnormalities suggested a correct diagnosis.
We report on a male epileptic patient, presently 27 years old, who has suffered complex-partial attacks for 19 years. Under treatment with carbamazepine the seizures were completely controlled. In addition, the patient exhibited partial hypopituitarism. CT and MRI revealed the presence of 2 lipomas, one located within the optico-chiasmatic cistern and the other one in the medial temporal lobe. To our knowledge, this combination of the generally rare lesions has not been described yet.
A patient with gastric ulceration and hemorrhage due to multiple antral gastric submucosal lipomas is presented. The number and close apposition of the lipomas resulted in loss of the usual gastroscopic and radiologic findings associated with these benign tumors. Complications arising from both singly occurring and multiple gastric lipomas are discussed.
The author, a pioneer in originating the technique of suction lipectomy, discusses in detail the pathophysiology of the fat cell and fat metabolism. In addition, he describes the surgical means by which, since 1977, he has popularized this innovative new approach to the treatment of unsightly fat deposits in many sites in the body, including the thighs, buttocks, abdomen, hips, arms, ankles, breasts, and submental regions.
Widening of the duodenal sweep on upper GI series is a sign of mass in the pancreatic head. A series of patients was examined in which the widening of the duodenal sweep was solely due to fatty infiltration of the peripancreatic space. Because CT established the diagnosis, more invasive procedures were obviated.
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The Proteus syndrome is a recently delineated group of skeletal and mesodermal malformations. Its characteristics include hemihypertrophy and fatty/lymphangiomatous masses. This description outlines the imaging sequences available to the radiologist. It is mainly concerned with the use of magnetic resonance imaging. This shows the extent of the mesodermal malformation with particular reference to the extent of intra abdominal infiltration.
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The leg is a very important aesthetic unit along with the face, neck, décolleté and hands. In addition to individual and inherited factors, which define the appearance and shape of the legs, multiple changes may occur during life which are cosmetically disturbing and may influence individual attractiveness. These changes offer dermato-phlebologists a wide spectrum of possibilities to intervene. Sclerotherapy, laser procedures, surgical approaches such as liposuction, conventional and endovasal phlebosurgery and physical therapy can all be beneficial.
The degree of lipodermatosclerosis in chronic venous insufficiency was determined by high-resolution 20 MHz ultrasound. For comparison, skin biopsies were taken and examined by light microscopy. With increasingly extensive sclerosis, ultrasound showed increasing thickness of the dermis with a poorly defined border from the subcutaneous fat. In addition, an echolucent band appeared in the upper dermis, corresponding to regions of glomerular capillaries in histology. In our experience, high-resolution ultrasound examination allows rapid and objective quantification of lipodermatosclerosis.