Case Report: Macrodystrophia lipomatosa - Illustration of two cases

Abstract

Macrodystrophia lipomatosa is a rare cause of congenital macrodactyly, characterized by progressive proliferation of all mesenchymal elements, with a disproportionate increase in fibroadipose tissue. This developmental anomaly is reportedly more common in the foot than in the hand, with a predilection for the plantar and median nerve distribution. We present two cases of MDL of the hand, one of which had an unusual nerve territory distribution, making clinical diagnosis difficult. Preoperative diagnosis was however made on the basis of radiography and MRI and was later confirmed on surgery.