Macrodystrophia Lipomatosa: A rare presentation

Received: November 08, 2010
Accepted: December 02, 2010
Ref: Kachewar SG, Sankaye SB, Kulkarni DS. Macrodystrophia lipomatosa: a rare presentation. Internet J Med Update. 2011 Jul;6(2):65-8.

MACRODYSTROPHIA LIPOMATOSA: A RARE PRESENTATION

Sushil G. Kachewar* MD DNB, Smita B. Sankaye† MD and Devidas S. Kulkarni* MD

*Department of Radiodiagnosis, †Department of Pathology, Rural Medical College, PIMS, Loni, Maharashtra, India

(Corresponding Author: Dr. Sushil G. Kachewar, Department of Radiodiagnosis, Rural Medical College, PIMS, Loni, Maharashtra, India; Mobile: +9921160357, Email: sushilkachewar@hotmail.com)

ABSTRACT

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 and bony enlargement. As imaging is the key to early diagnosis, findings on various modalities like plain radiographs, CT scan and MRI are highlighted here.

KEY WORDS: Macrodystrophia lipomatosa; CT; MRI

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