Spinal canal compromise in Proteus syndrome: case report and review of the literature.
A 24-year-old Vietnamese man with Proteus syndrome presented to the emergency department with progressive paraplegia and underwent urgent operative decompression of an angiolipomatous mass compressing the spinal cord. The associated spinal deformity and difficulty in imaging the mass, together with the paucity of discussion regarding the spinal cord sequelae of this rare syndrome in the literature, made this an extremely challenging case. Spinal cord compromise in patients with Proteus syndrome can result from either infiltration of the spinal canal by an angiolipomatous mass or canal narrowing as a result of vertebral hypertrophy. Regardless of the cause, the prognosis for neural recovery remains poor.
Ring
D
D
Children's Hospital, Boston, Massachusetts, USA.
Snyder
B
B
D000328:Adult; D018206:Angiolipoma / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D006801:Humans; D007796:Laminectomy; D008297:Male; D011379:Prognosis; D016715:Proteus Syndrome / Q000150:complications*; D013117:Spinal Cord Compression / Q000150:complications / Q000209:etiology*; D013123:Spinal Fusion; D013125:Spinal Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D013904:Thoracic Vertebrae