Macrodystrophia lipomatosa: four case reports

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Macrodystrophia lipomatosa: four case reports

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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Macrodystrophia Lipomatosa

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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On a case of "Macrodystrophia lipomatosa".

Macrodistrophia Lipomatosa is a congenital malformation of rare finding and unknown pathogenic mechanism. The pathology is mainly characterized by the interest of lower extremities and peculiar macroscopic feature is the presence of hyperthrophic fibro-adipose tissue. In our experience the localization to the upper extremities and the presence of uncommon clinical signs show the importance of i...

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MR imaging of macrodystrophia lipomatosa.

Macrodystrophia lipomatosa is a rare nonhereditary congenital form of localised gigantism usually involving the 2nd or 3rd digit of the hand or foot. Pathologically an increase in adipose tissue involving subcutaneous tissue periosteum and bone marrow is present. Typical clinical andradiological appearances are described in this case report. Case Report A 36-year-old female presented with enlar...

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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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ژورنال

عنوان ژورنال: Italian Journal of Pediatrics

سال: 2010

ISSN: 1824-7288

DOI: 10.1186/1824-7288-36-69