Macrodystrophia Lipomatosa: Clinico-patho-radiological Correlation
نویسندگان
چکیده
Macrodystrophia lipomatosa is a rare congenital nonhereditary developmental anomaly. It is characterized by hamartomatous proliferation of the soft tissue leading to disproportionate enlargement of the limbs and digits. Since it leads to diagnostic dilemma, it has to be differentiated from various other conditions as they differ in course, prognosis, complications, and treatment. Herein, we present two cases with localized gigantism and discuss the various differential diagnoses and need for clinico-patho-radilogical correlation for diagnosis of this rare entity.
منابع مشابه
Macrodystrophia Lipomatosa: A rare presentation
Macrodystrophia lipomatosa is a rare congenital nonhereditary mesenchymal hamartomatous malformation resulting in localized gigantism of parts of extremities that manifests clinically as macrodactyly or megalodactyly. Radiological and Pathological hallmark is the disproportionate fibroadipose tissue proliferation in subcutaneous tissue, nerve sheaths, and periosteum that leads to soft tissue an...
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Macrodystrophia lipomatosa is a rare congenital form of localized gigantism characterized by an increase in all mesenchymal elements, particularly fibroadipose tissue. MR imaging can be used to examine the soft tissue and detect the fibrous and fatty hypertrophy that characterizes this condition and also diagnose an associated fibrolipomatous hamartoma of the median or plantar nerve. We des...
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remaining three ulnar digits appeared normal. Magnetic resonance imaging (MRI) revealed characteristics of macrodystrophia lipomatosa such as enlargement and marked proliferation of the adipose tissues of the left hand, especially the radial aspect. MRI also demonstrated excessive fibrofatty tissue around the affected digits. This tissue has the same signal characteristics as fat on MRI, that i...
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Macrodystrophia lipomatosa is a rare entity that is mostly diagnosed in children. It has been very rarely reported in adults. Here, we describe the X-ray and computed tomography findings in a case of macrodystrophia lipomatosa in an elderly female presenting with an enlarged second toe since birth and bony outgrowths causing pressure effects and cosmetic problems.
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AIM Macrodystrophia lipomatosa is a rare cause of gigantism of limb which can be confused with other common causes like congenital lymphedema. It presents usually with loss of function and cosmetic problems. Four cases are described with emphasis on clinical presentation, differential diagnoses, imaging and treatment options. METHODS & RESULTS Four patients of macrodystrophia lipomatosa were ...
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