Introduction: The reported incidence of scoliosis in arthrogryposis varies from 30% to 67% and, in most cases, the curves progresses rapidly and becomes stiff from early age.
Objectives: The Authors report six cases of scoliosis in arthrogryposis to assess the role of surgical treatment.
Materials and Methods: Six cases (3 males, 3 females; mean age at surgery 13.2 years) with arthrogryposis multiplex congenita associated with the characteristic amyoplasia were reviewed: they were operated on for scoliosis at the Spine Surgery Department of the Rizzoli Orthopaedic Institute between 1987 and 2008.
Surgery was performed using the Harrington-Luque instrumentation (2 cases), the Luque system (1), a hybrid segmental technique with hooks and screws (1) and spinal anchoring with pedicle screws (2).
Results: The patients were clinically and radiologically reviewed at a mean follow-up of 4.2 years, ± 2.7 (range, 1 to 9 years). Three minor postoperative complications were encountered; a long-term complication was seen only in one case and required instrumentation removal. Surgery was successful in the other 5 cases, where solid arthrodesis was achieved and no significant curve progression was observed at follow-up.
Conclusions: The experience acquired on the present case series leads the authors to assert that prompt action should be taken when treating such aggressive forms of scoliosis.
Significance: In case of mild spinal deformities in arthrogryposis, brace treatment should be attempted, the evolution of the curves being unpredictable; however, when the curve exceeds 40° and presents with marked hyperkyphosis, hyperlordosis or pelvic obliquity, surgery should not be delayed.