The spine in diastrophic dysplasia. The surgical arthrodesis of thoracic and lumbar deformities in 21 patients

Y Matsuyama, R B Winter, J E Lonstein
Spine 1999 November 15, 24 (22): 2325-31

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 degrees/97 degrees) with a true kyphosis at the junction of the two scolioses (101 degrees). 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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