[Scoliosis in arachnodactyly].
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Biomedical subjects
Publications and source records attributed to P Stagnara.
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Most severe scoliosis and kyphoscoliosis are not unfrequent in European countries. The purpose of their treatment is not only cosmetic but, often, vital. Patients are hypotrophic and their vital capacities usually very poor. Since 1952, the authors have treated (partial, reduction and fusion) 183 patients: with curves above 100 degrees (101 degrees-203 degrees). Reduction was obtained by distraction cast or halo-traction. Surgical treatment (one or two stages) used Harrington rods and fusion with a large amount of iliac bone. These procedures are dangerous for the medullary function. Controls must be performed during the operative distraction. Follow up studies demonstrated the stability of the obtained reductions.
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Scoliosis is a complication of spinal lesions in neurofibromatosis, and leads frequently to major deformities with dislocations. The authors conducted a critical study of 31 cases surgically treated during the 1954-73 period of time. The techniques of spine straightening and of arthrodesis were recalled. The authors conclude that in case of moderate deformity (less than 100 degrees) Harrington's operation was satisfactory enough when correctly conducted. In case of severe dislocation and kyphosis, straightening was obtained after long lasting casts and halo tractions. Then posterior fusion using Harrington's rods had usually to be completed by anterior, transthoracic fusion for better solidity.
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