Management of nerve compression in multiple hereditary exostoses: a report of two cases and review of the literature

Abstract

Multiple hereditary exostoses (MHE) is a rare autosomal dominant condition that results in the growth of cartilage-capped prominences that often cause nerve compression and injury. Many patients suffer from continued and debilitating chronic pain which leads some to advocate avoiding surgical intervention in patients with multiple hereditary exostoses. We present a review of the literature as well as a case series at our institution in order to evaluate the role of surgery in multiple hereditary exostoses. We searched the literature for reports of patients with multiple hereditary exostoses undergoing surgery for nerve compression. We then reviewed the recent experience at our institution which revealed two patients with multiple hereditary exostoses. Our literature search revealed that there have been several case series and retrospective analyses in the literature that assess the benefit of surgery in the case of nerve compression caused by exostoses. The majority of these reports are of solitary exostoses. Few reports expand on the role of surgery in patients with multiple hereditary exostoses suffering from nerve compressions secondary to bony overgrowth. A recent review of the experience at our institution revealed two patients with multiple hereditary exostoses who together underwent a total of four surgeries for treatment of peripheral nerve compression resulting in pain or weakness. Postoperative evaluation revealed improvement in pain and/or motor strength following each operation. Based on our experience and literature review, we advocate that nerve compression in selected individuals with multiple hereditary exostoses that results in neurological injury should be considered for nerve decompression and resection of the offending exostosis.