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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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MINNEAPOLIS, MINNESOTA
Supported in part by the Medical Education and Research Association of Gillette Children's Hospital.
437 Portage Trail, Cuyahoga Falls, Ohio 44221.
606 24th Avenue South, Minneapolis, Minnesota 55454.
1661 St. Anthony Avenue, St. Paul, Minnesota 55104.

The Journal of Bone and Joint Surgery, Incorporated
J Bone Joint Surg Am, 1980 Jun 01;62(4):529-536
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Abstract

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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    These activities have been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of the American Academy of Orthopaedic Surgeons and The Journal of Bone and Joint Surgery, Inc. The American Academy of Orthopaedic Surgeons is accredited by the ACCME to provide continuing medical education for physicians.
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