Lipofibromatous hamartoma of the median nerve: a comprehensive review and systematic approach to evaluation, diagnosis, and treatment.

Many modalities exist for diagnosing and treating lipofibromatous hamartoma (LFH), with no clear consensus. This is the first comprehensive study to review the existing literature on LFH of the median nerve and to suggest a systematic approach to its diagnosis and treatment. An electronic and manual search was conducted on Medline, Embase, Google Scholar, Current Contents, and Science Citation Index for original and review articles in English or French, from 1946 to November 2012. After 2 levels of screening, 106 references containing case reports were retained. Data extraction included patient demographics, clinical information, diagnostic modalities, treatment, and follow-up. A total of 180 cases were reported in the literature. One third of patients had associated macrodactyly (32%). Gender distribution is equal in LFH with or without macrodactyly, with most patients (71%) presenting before age 30 years. The main presenting symptom is an enlargement (88%) over the volar forearm, wrist, or hand, with or without digital hypertrophy, followed by paresthesia (39%). A soft, mobile, nontender, nonfluctuant mass with variable degree of compressive median neuropathy is found on physical examination. Biopsy, which reveals abundant mature fat cells and fibrous connective tissue infiltrating between nerve fascicles and the space between the epineurium and the perineurium, is not necessary because the pathognomonic features of the mass on magnetic resonance imaging offer an accurate diagnosis. Treatment of nerve compression symptoms and macrodactyly should be addressed separately. Carpal tunnel release is the mainstay of treatment for neuropathy, and ray or digital amputation, wedge osteotomy, middle phalangectomy with arthroplasty, and epiphysiodesis are suggested options in the management of macrodactyly. Based on our review of the literature, we propose an algorithm for the diagnosis and treatment of LFH of the median nerve with or without macrodactyly.