• S S Dalvie, M H Noordeen, and A Vellodi.
• Great Ormond Street Hospital for Children, London, United Kingdom.
• Spine. 2001 Dec 1; 26 (23): E539-41.

Study DesignA case series of seven children who had a thoracolumbar gibbus related to mucopolysaccharidosis treated with anterior instrumentation were reported retrospectively.ObjectiveTo describe a new technique for treating progressive thoracolumbar kyphosis in children with mucopolysaccharidosis.Summary Of Background DataManagement of this condition is not well represented in the literature. Isolated reports on the surgical management of this disorder appear, but there is no previous report of correction performed anteriorly.MethodsSeven patients underwent anterior instrumentation for correction of a thoracolumbar gibbus not arrested by brace treatment. Preoperative kyphosis ranged from 42 degrees to 64 degrees (average, 52.5 degrees ). Data on all seven patients were collected prospectively. The technique and its principles are described.ResultsA good correction of the kyphosis was obtained, with postoperative angles of 3 degrees to 29 degrees (average, 15 degrees ), and maintained through the follow-up period. There were no complications from the procedure.ConclusionAnterior instrumented correction and fusion of the spine is effective in treating thoracolumbar kyphosis associated with mucopolysaccharidosis.

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