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Open Access Case Report

Surgical treatment of scoliosis in a rare disease: arthrogryposis

Tiziana Greggi1*, Konstantinos Martikos1, Emanuela Pipitone1, Francesco Lolli1, Francesco Vommaro1, Elena Maredi1, Stefano Cervellati2 and Mario Di Silvestre1

Author Affiliations

1 Spine Surgery Division, Rizzoli Orthopaedic Institute, Bologna, Italy

2 Scoliosis Center, Hesperia Hospital, Modena, Italy

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Scoliosis 2010, 5:24  doi:10.1186/1748-7161-5-24

Published: 9 November 2010

Abstract

Background

The reported incidence of scoliosis in arthrogryposis varies from 30% to 67% and, in most cases, the curves progress rapidly and become stiff from early age.

The authors report six cases of scoliosis in arthrogryposis to assess the role of surgical treatment.

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 authors' Spine Surgery Department 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 pulmonary complication was seen in one case after reintervention and was successfully resolved after 10 days. 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 with the present case series leads the authors to assert that prompt action should be taken when treating such aggressive forms of scoliosis. 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.