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Cervical Spinal Stenosis in Metatropic Dysplasia

Leet, Arabella I. MD*; Sampath, Jayanth S. FRCSEd, FRCS (Tr&Orth), MSc; Scott, Charles I. Jr MD; MacKenzie, William G. MD

Journal of Pediatric Orthopaedics: May-June 2006 - Volume 26 - Issue 3 - p 347-352
doi: 10.1097/01.bpo.0000214923.95314.ad
Spine: Original Article
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Abstract: Metatropic dysplasia is a rare skeletal dysplasia characterized by rapid collapse of the thoracolumbar spine into kyphoscoliosis. Other spinal anomalies associated with metatropic dysplasia include odontoid hypoplasia and atlantoaxial instability leading to cervical myelopathy. Children with metatropic dysplasia evaluated at our institution for spinal deformity showed evidence of cervical stenosis with or without associated cord compression. Magnetic resonance imaging was found to demonstrate these changes. The association of cervical spinal stenosis and metatropic dysplasia has not been previously described. This has significant treatment implications, because decompression over the stenotic segments should be considered in conjunction with spinal fusion for treatment of odontoid hypoplasia or atlantoaxial instability. A retrospective review of 13 cases of metatropic dysplasia was performed. Despite the challenges provided by this patient population, the chance to halt or reverse neurological dysfunction and improve deformity necessitates prompt surgical intervention.

From the *Department of Orthopedic Surgery, The Johns Hopkins Hospital, Baltimore, MD; †Department of Pediatric Orthopaedics, Booth Hall Children's Hospital, Manchester, UK; and ‡Department of Orthopedics, Alfred I. duPont Hospital for Children, Nemours Children's Clinic, Wilmington, DE.

This investigation was performed at the Alfred I. duPont Hospital for Children, Nemours Children's Clinic in Wilmington, DE.

No author received financial support for this study.

Reprints: William G. Mackenzie, MD, Department of Orthopaedics, Alfred I. duPont Hospital for Children, PO Box 269, Wilmington, DE 19899. (e-mail: wmackenz@nemours.org).

© 2006 Lippincott Williams & Wilkins, Inc.