Macrodactyly of the right ring finger due to macrodystrophia lipomatosa: Pathologic and imaging characteristics
نویسندگان
چکیده
Introduction: Macrodystrophia lipomatosa (MDL) is a non-hereditary congenital developmental anomaly characterized by localized gigantism due to overgrowth of all mesenchymal elements. Case Report: The authors describe a case of macrodystrophia lipomatosa (MDL) with involvement of the median nerve in a 10-year-old girl and review the pathologic, radiographic, ultrasonographic and MR imaging characteristics. Conclusion: Ultrasonography and MR imaging are pathognomonic for MDL and reliably distinguish it from other forms of localized congenital gigantism. However, since ultrasonography has the advantages of much lower cost, portability and easy accessibility, we propose its use as the imaging modality of choice in the initial diagnostic approach of congenital localized gigantism.
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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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 is a sporadic, nonhereditary developmental anomaly and a rare form of congenital localized gigantism. It is characterized by the proliferation of all the mesenchymal elements of a digit or digits, and a disproportionate increase of fibroadipose tissue involving the nerve sheath, muscle, periosteum and bone marrow. In the present study, a 9-month-old boy was referred t...
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