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  • Title: The spine in diastrophic dysplasia. The surgical arthrodesis of thoracic and lumbar deformities in 21 patients.
    Author: Matsuyama Y, Winter RB, Lonstein JE.
    Journal: Spine (Phila Pa 1976); 1999 Nov 15; 24(22):2325-31. PubMed ID: 10586456.
    Abstract:
    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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