Abstract
Macrodystrophia lipomatosa is a rare, non-hereditary congenital anomaly characterized by localised gigantism in the form of macrodactyly affecting a part of limb and rarely entire limb due to overgrowth of all mesenchymal elements. Radiological and pathological hallmark is the disproportionate fibroadipose tissue proliferation in subcutaneous tissue, nerve sheaths, and periosteum that lead to soft tissue and bony enlargement. We present the case of a twenty years old female who presented with history of gradual enlargement of the medial aspect of right hand along ulnar nerve distribution since birth. MRI showed hypertrophy of bones and soft tissue with fatty proliferation, leading to the diagnosis of macrodystrophia lipomatosa.
Highlights
Macrodystrophia lipomatosa is a rare, non-hereditary congenital anomaly characterized by localised gigantism in the form of macrodactyly affecting a part of limb and rarely entire limb due to overgrowth of all mesenchymal elements
Macrodystrophia lipomatosa comes to clinical attention mostly because of cosmetic reasons, and rarely due to development of neurovascular compression or mechanical problems like secondary degenerative joint disease
Macrodystrophia lipomatosa (MDL) of hand is more common along median nerve distribution; we report a rare case of MDL in a 20 years old female involving ring and little fingers of right hand with predominant ulnar nerve distribution
Summary
Macrodystrophia lipomatosa is a rare, non-hereditary congenital anomaly characterized by localised gigantism in the form of macrodactyly affecting a part of limb and rarely entire limb due to overgrowth of all mesenchymal elements. Is a congenital progressive gigantism of the hand and foot, which may be local or may involve the entire limb. Magnetic resonance imaging (MRI) is considered pathognomonic of MDL and helps to differentiate it from other causes of localized gigantism [1]. MDL of hand is more common along median nerve distribution; we report a rare case of MDL in a 20 years old female involving ring and little fingers of right hand with predominant ulnar nerve distribution.
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