Scoliosis in arthrogryposis multiplex congenita.

ABSTRACT

Scoliosis was evident in eighteen of eighty-eight patients with arthrogryposis multiplex congenita. The predominant pattern of spinal deformity was a structural thoracolumbar double curve that extended to the sacrum and was associated with pelvic obliquity and lumbar hyperlordosis. Significant contractures about the hips, dislocation of the hip, or both were present in all patients but one. Most of the curves were progressive and they became rigid and fixed at an early age. There was progression of the pelvic obliquity coincident with progression of the curve. Treatment by corrective casts or a Milwaukee brace was ineffective and if surgical treatment directed at the pelvic obliquity did not correct that deformity, spine fusion to the sacrum appeared necessary.