Cervical spinal cord compression in hereditary multiple exostoses

Abstract

Spinal cord compression is an extremely serious complication of hereditary multiple exostoses (HME). A case of HME with compression of the cervical spinal cord is reported. Complete recovery following surgery was achieved. A review of the relevant literature revealed 51 previous cases of HME with cord/cauda equina compression. Most patients were under 30 years of age with more men affected than women. The family history was positive in 60%. The cervical and thoracic areas were predominantly affected, with the symptoms usually developing slowly. Recovery following surgery is to be expected in the majority of cases. In patients with HME and suffering from neurological symptoms, the possibility of spinal cord compression should be considered. Prompt diagnosis and surgical excision provide the best prognosis.

Cervical osteochondroma as a cause of spinal cord compression in a patient with hereditary multiple exostoses: Computed tomography and magnetic resonance imaging findings

“…The family history is positive in approximately 60%, with autosomal dominant inheritance 5 . The exostoses usually involve long bones, and they seldom occur in the axial skeleton, occurring in only 7–9% of patients with HME 1,3–6 . The exostoses grow slowly during childhood and adolescence and growth ceases with skeletal maturation.…”

“…However, spinal cord compression resulting from osteochondroma is an extremely serious and unusual complication of HME. Possible symptoms resulting from compression include myelopathy, paraplegia, paraesthesia, paresis and radiculopathy 3–10 …”

“…Spinal cord compression should be considered in a patient who develops neurological deficit and has established HME. It generally occurs in young adults in the second and third decades, with more men affected than women 1,4,5,8 …”

Multiple peripheral nerve compressions related to malignantly transformed hereditary multiple exostoses

“…Most reported cases involved the cervical and thoracic region, and the majority presented with symptoms suggestive of a slowly growing spinal cord tumor. 5,12,18 In a review of the literature available in 1951, Slepian and Hamby 13 found only 21 reported cases of neurologic complications in over 1000 cases. We believe that predilection of the involvement site may be related to the low incidence of neuropathy in patients with osteochondroma.…”

Solitary Osteochondroma Arising from the Dorsal Vertebral Spinous Process

“…Osteochondromas rarely affect the spine; 1.3% to 4.1% of solitary osteochondromas arise within the spine, and approximately 9% of patients with multiple osteochondromas have spinal lesions. [3][4][5] Understanding embryological development of spine could also conjecture on other etiological hypothesis. The complete growth of the vertebral column is completed in adolescence by secondary ossification centers at the spinous process, transverse process, articular process, and end plate of vertebral body.…”