The Spine in Diastrophic Dysplasia: The Surgical Arthrodesis of Thoracic and Lumbar Deformities in 21 Patients

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Study Design.Retrospective chart and radiographic film review.Objectives.To discern the deformity problems in diastrophic dysplasia and to report our results in surgical treatment.Summary of Background Data.Due to the rarity of the problem, the literature is very scanty as to the indications for surgery or the best technique.Methods.Analysis of radiographic film for scoliosis, kyphosis, lordosis, and decompensation before surgery, after surgery, and at follow-up. Analysis of charts for complications and problems.Results.The most common deformity pattern was a double thoracic kyphosis (79°/97°) with a true kyphosis at the junction of the two scolioses (101°). Combined anterior-posterior arthrodesis gave the best results.Conclusions.Very severe deformity can occur in children with diastrophic dysplasia, even at a young age. Prompt anterior-posterior arthrodesis can prevent catastrophic deformity.

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