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Child's Nervous System

, Volume 22, Issue 9, pp 1212–1214 | Cite as

Congenital cervical instability in a patient with camptomelic dysplasia

  • Gregory P. Lekovic
  • Harold L. Rekate
  • Curtis A. Dickman
  • Margaret Pearson
Case Report

Abstract

Introduction

Camptomelic dysplasia (CD) is a rare autosomal dominant skeletal dysplasia classically characterized by bent bones of the extremities, tracheobronchial narrowing, thoracic kyphoscoliosis, and various degrees of phenotypic sex reversal. Most patients die of complications in infancy, although long-term survivors have been reported.

Case report

We report a case of CD complicated by incomplete ossification of the cervical vertebral pedicles, resulting in congenital cervical instability and kyphosis. Closed reduction was performed, and the patient was fitted with a customized cervical orthosis.

Finding

He subsequently developed a complete spinal cord injury at the kyphotic level. This underscores the grim prognosis associated with neonatal cervical spinal instability.

Keywords

Camptomelic dysplasia Cervical instability Skeletal dysplasia 

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Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • Gregory P. Lekovic
    • 1
  • Harold L. Rekate
    • 1
    • 3
  • Curtis A. Dickman
    • 1
  • Margaret Pearson
    • 2
  1. 1.Division of Neurological Surgery, Barrow Neurological InstituteSt. Joseph’s Hospital and Medical CenterPhoenixUSA
  2. 2.Neonatology AssociatesPhoenixUSA
  3. 3.Neuroscience PublicationsBarrow Neurological InstitutePhoenixUSA

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