Compression Myelopathy in Proteus Syndrome

Abstract

Proteus syndrome is manifested by a disturbance of cell growth including benign tumors under the skin, overgrowth of the body, more on one side (hemihypertrophy), overgrowth of the fingers (macrodactyly) and spinal deformaties.

Abstracted from Skovby et al. (1993)

Updates

Abstract from Yamamoto et al. (2012)

Proteus syndrome is a rare, sporadic, hamartomatous disorder manifesting with multifocal overgrowth of tissue. The features seem to develop most often during childhood. Vertebral overgrowth with severe spinal canal stenosis is unusual, although scoliosis with abnormal vertebral bodies is one of the typical features of Proteus syndrome. We report a case of Proteus syndrome with severe spinal canal stenosis, scoliosis, cervical kyphosis, and thoracic deformity with airway obstruction because of asymmetrical overgrowth of vertebrae and ribs associated with a tethered cord, lipomas, strawberry hemangioma, flat nasal bridge, and bilateral hypoplasty of the first metatarsal bones with hyperplasty of soft tissue.

Reference

Yamamoto A, Kikuchi Y, Yuzurihara M, Kubota M, O’uchi T. A case of Proteus syndrome with severe spinal canal stenosis, scoliosis, and thoracic deformity associated with tethered cord. Jpn J Radiol. 2012;30(4):336–9.