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Extended Laminectomy for Spinal Stenosis in Achondroplasia

  • Elizabeth Streeten
  • Sumio Uematsu
  • Orest Hurko
  • Steven Kopits
  • Edmund Murphy
  • Reed Pyeritz
Part of the Basic Life Sciences book series (BLSC, volume 48)

Abstract

Achondroplasia is the most common chondrodysplasia, an autosomal dominant condition with an incidence of 17–29 per million (1). The basic defect is in endochondral bone formation, the cause of which is unknown (2). The abnormal bone growth leads to the typical skeletal features of rhizomelic limb shortening, frontal bossing, lumbar lordosis and narrowing of the spinal canal. Neurological complications have been attributed to the skeletal abnormalities since the early 1900’s, when the narrowed lumbar canal was recognized as a cause of paraplegia (3). In 1925, Donath and Vogl (4) provided the first detailed description of the structural changes in the vertebral column resulting in neurological complications. Their autopsy studies revealed a decrease in size of the thoracic spinal canal in achondroplasts due to shortened vertebral bodies and pedicles. Later studies have demonstrated a reduction in size of the entire spinal canal (5), with caudal tapering (6) and stenosis of nerve root canals (7). The spinal cord and cauda equina are normal in size (6,8). In addition, the intervertebral discs are congenitally hyperplastic and tend to bulge laterally and posteriorly (6).

Keywords

Spinal Stenosis Lumbar Spinal Stenosis Somatosensory Evoke Potential Johns Hopkins Medical Institution Post Void Residual 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Plenum Press, New York 1988

Authors and Affiliations

  • Elizabeth Streeten
    • 1
  • Sumio Uematsu
    • 2
  • Orest Hurko
    • 3
  • Steven Kopits
    • 4
  • Edmund Murphy
    • 1
  • Reed Pyeritz
    • 1
  1. 1.Division of Medical GeneticsJohns Hopkins Medical InstitutionBaltimoreUSA
  2. 2.Dept. of NeurosurgeryJohns Hopkins Medical InstitutionBaltimoreUSA
  3. 3.Depts. of Medicine; NeurologyJohns Hopkins Medical InstitutionBaltimoreUSA
  4. 4.Orthopedic SurgeryJohns Hopkins Medical InstitutionBaltimoreUSA

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