Case Reports
Lipofibromatous Hamartoma of a Digital Nerve
Am J Orthop. 2008 August;37(8):E146-E148
Author and Disclosure Information
Quanjun Cui, MD, A. Bobby Chhabra, MD, Brian M. Leo, MD, and Michael E. Pannunzio, MD
Dr. Cui is Assistant Professor, Division Head of Orthopaedic Trauma, University of Virginia Health System, Charlottesville, Virginia. He was Resident, Department of Orthopaedic Surgery, University of Virginia School of Medicine, at the time the article was written.
Dr. Chhabra is Associate Professor of Orthopaedic and Plastic Surgery, Division Head of Hand and Upper Extremity Surgery, University of Virginia Health System, Charlottesville, Virginia.
Dr. Leo is Associate Staff, Department of Orthopaedic Surgery, Cleveland Clinic Florida, Weston, Florida.
Dr. Pannunzio is Clinical Associate Professor, Department of Orthopaedic Surgery, Indiana University, Reconstructive Hand Surgeons of Indiana, Carmel, Indiana.
Lipofibromatous hamartoma of a nerve is a gross enlargement of the nerve caused by large proportions of fat and fibrous tissue, with epineural and perineural proliferation.1-5 More than 90 cases have been described in the English-language literature.1-5 Although most commonly reported in the median and digital nerves, the lesion also occurs in the radial,6,7 ulnar,8,9 sciatic,10 cranial,11 and plantar12 nerves. The etiology of this benign tumor is unknown but is considered by some authors to be congenital.1,2,12
The mass is usually slow-growing and may cause nerve compression resulting in sensory change, pain, and loss of motor function.1-12 Patients usually present before adulthood. Biopsy has been needed to confirm the diagnosis and continues to be the standard approach to establish the diagnosis; however, recent reports suggest that the magnetic resonance imaging (MRI) appearance of this entity is pathognomonic.4,13 Treatment of lipofibromatous hamartoma is controversial. Recommendations include decompression of the carpal tunnel (when the lesion involves the median nerve at the wrist), decompression and debulking of the fibrofatty sheath, microsurgical dissection of the neural elements, excision of the involved nerve segment with or without nerve grafting, and observation for asymptomatic patients.1-15
We present the case of a manual laborer with lipofibromatous hamartoma of a digital nerve with normal sensory and motor function before surgery but with an enlarging first web space mass altering the dexterity and functional use of the hand. The patient was treated with incisional biopsy and debulking of the tumor to improve first web space depth and digital range of motion. After presenting this case, we review the literature.