Scoliosis in arthrogryposis multiplex congenita

Fourteen of 50 patients studied with arthrogryposis also had scoliosis, an incidence of 28%. Eight patients developed a severe curve associated with functional disability and poor health. Three patients were unable to walk independently, and 2 died in childhood. Seven patients had congenital scoliosis; 4 had long "C" neuromuscular curves, and 3 had scoliosis associated with pelvic obliquity caused by unilateral hip dislocation or contracture. The natural history of congenital and neuromuscular curves was progression to an extreme and rigid scoliosis. The authors urge others to look for scoliosis in their patients with arthrogryposis and to anticipate its progression.