Bilateral fibrolipomatous hamartoma of the median nerve with macrocheiria and late-onset nerve entrapment syndrome.

The case of a 46-year-old man is reported who presented with an extraordinary constellation of fibrolipomatous hamartomas of the right ulnar and both median nerves, with a right-sided giant hand ("macrocheiria") due to enlarged bones and subcutaneous tissue, and unusual late manifestation of nerve entrapment at the right wrist. Magnetic resonance imaging of the hypertrophied nerves disclosed large nerve fascicles surrounded by fibrous tissue, which allowed preoperative diagnosis.