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Disorders of the Spine in Diastrophic DwarfismA Discussion of Nine Patients and Review of the Literature*
Daniel Bethem, M.D.; Robert B. Winter, M.D.; Lowell Lutter, M.D.
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Supported in part by the Medical Education and Research Association of Gillette Children's Hospital.
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The Journal of Bone and Joint Surgery, Incorporated
J Bone Joint Surg Am, 1980 Jun 01;62(4):529-536
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In this series of nine patients with diastrophic dwarfism, multiple problems with the spine were noted. Scoliosis was present in all patients and usually became rapidly progressive in the preadolescent years. The scoliosis had a strong tendency to become severe and rigid, with a large rotatory component. Early brace treatment appeared to have merit. Cervical kyphosis was noted in four patients; it resolved in three patients, but progressed in one to a 130-degree deformity, quadriplegia, and death. All patients in this series had spina bifida occulta in the cervical spine. Six of the nine patients demonstrated a narrowed interpedicular distance below the third lumbar vertebra, but none had spinal stenosis.

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