Fibrolipomatous hamartoma emerging from the median nerve associated with macrodystrophia lipomatosa

Fibrolipomatous hamartoma with macrodystrophia lipomatosa is a rare congenital condition characterised by a benign enlargement of the peripheral nerves and surrounding fibro-adipose tissues. A 7-year-old child was referred to our plastic surgery clinic with complaints of a massively enlarged right middle finger, a moderately enlarged index finger and a puffy hand. He had palpable, nontender enlargements of the fibro-adipose tissues (13 x 6 x 4 cm and 8 x 4 x 2 cm) extending to the palm and the wrist. They were slowly growing and had been noticed since birth. The diagnosis was based on magnetic resonance imaging. Functional and cosmetic considerations are important in such cases, and multiple debulking surgeries are often required.