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Images in Clinical Practice Indian Pediatrics 2003; 40:363-364 |
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Macrodystrophia Lipomatosa |
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Twelve-year-old female patient presented with hypertrophy of second toe of right foot since birth and was gradually increasing in size (Fig. 1). There was no family history of hypertrophy of limbs, no history of pigmentation and seizures. The patient was otherwise normal. X-ray foot revealed localized gigantism of right second toe and its digits, there was hypertrophy of fibroadipose tissue of right second toe. (Fig. 2). A diagnosis of Macrodystrophia lipomatosa was made.
Macrodystrophia lipomatosa is a congenital form of macrodactyly seen at birth. It is a progressive condition characterized by an increase in mesenchymal elements, especially the fibro adipose tissue. The abnormality is always unilateral, and usually in the distribution of the median nerve (upper extremity) and the planter nerve (lower extremity). Secondary degenerative arthro-pathy may be evident in young adults and limitation of joint movement is associated. Radiographic examinations demonstrate macrodactyly including the soft tissue and the bone. The trabeculae are normal. T1-weighted coronal MR image shows marked prolifera-tion of the adipose tissue. A high signal linear structure with low signal rim along the involved area may correspond to a thickened nerve.
Macrodystrophia lipomatosa should be differentiated from other causes of congenital macrodactyly e.g., lymphangioma, heman-gioma, Klippel-Trenaunay-Weber syndrome, Ollier’s disease and Proteus syndrome. Anoop Verma,
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