Lipofibromatous Hamartoma of the Median Nerve in Patients with Macrodactyly: Diagnosis and Treatment of a Rare Disease Causing Carpal Tunnel Syndrome

Background: Lipofibromatous hamartoma or Macrodystrophia lipomatosa is a very rare benign peripheral nerve tumor most commonly affecting median nerve in young adults. It is a form of local gigantism characterised by disproportionate increase of adipose tissue and mesenchymal elements in neural distribution. Case: An 18yr old female patient with complaints of macrodactyly without any neurological symptoms was diagnosed with lipofibromatous hamartoma based on the operative findings which included enlargement of median nerve and neurovascular bundle of fingers. This enlargement was due to fibro-fatty tissue infiltration in nerves. Result: The treatment involves carpal tunnel release to decompress the median nerve, excision of the redundant tissue around the nerves and debulking of soft tissue component. Discussion: The predominant association of median nerve in this condition is unexplainable. The associations of Lipofibromatous hamartoma of median nerve are macrodatyly or macrodactylia fibrolipomatosis, ectopic calcification of soft tissues, bone exostoses, subcutaneous lipomas and vascular tumors.