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Pediatric Radiology

, Volume 27, Issue 4, pp 289–294 | Cite as

Lumbar gibbus in storage diseases and bone dysplasias

  • Terry L. Levin
  • W. E. Berdon
  • Ralph S. Lachman
  • Kwame Anyane-Yeboa
  • Carrie Ruzal-Shapiro
  • David P. Roye Jr.

Abstract

Objective. The objective of this study was to review the problem of lumbar gibbus in children with storage diseases and bone dysplasias utilizing plain films and MR imaging. Materials and methods. Clinical histories and radiographic images in five patients with storage diseases [four mucopolysaccharidosis (MPS) and one mucolipidosis] and two with achondroplasia were reviewed. The International Skeletal Dysplasia Registry (Los Angeles, Calif.), surveyed for all patients with lumbar gibbus and skeletal dysplasias, provided 12 additional cases. Results. All patients had localized gibbus of the upper lumbar spine, characterized by anterior wedging and posterior displacement of the vertebrae at the apex of the curve, producing a beaked appearance. The curve, exaggerated in the sitting or standing position, was most severe in the two patients with MPS-IV (one of whom died). Both developed severe neurologic signs and symptoms requiring surgical intervention. In four patients, MR images demonstrated the apex of the curve to be at or below the conus. Two patients demonstrated anterior herniation of the intervertebral discs at the apex of the curve, though the signal intensity of the intervertebral discs was normal. Conclusion. Lumbar gibbus has important neurologic and orthopedic implications, and is most severe in patients with MPS. The etiology of the gibbus with vertebral beaking is multifactorial and includes poor truncal muscle tone, weight-bearing forces, growth disturbance and anterior disc herniation. The curve is generally at or below the conus. Neurologic complications are unusual, although orthopedic problems can arise. Due to their longer survival, patients with achondroplasia or Morquio's disease are more vulnerable to eventual gibbus-related musculoskeletal complications.

Keywords

Intervertebral Disc Disc Herniation Storage Disease Skeletal Dysplasia Growth Disturbance 
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

© Springer-Verlag Berlin Heidelberg 1997

Authors and Affiliations

  • Terry L. Levin
    • 1
  • W. E. Berdon
    • 1
  • Ralph S. Lachman
    • 2
  • Kwame Anyane-Yeboa
    • 3
  • Carrie Ruzal-Shapiro
    • 1
  • David P. Roye Jr.
    • 4
  1. 1.Department of Radiology, Division of Pediatric Radiology, Columbia-Presbyterian Medical Center, Babies & Children's Hospital of New York, 3959 Broadway, New York, NY 10032, USAUS
  2. 2.International Skeletal Dysplasia Registry, Los Angeles, California, USAUS
  3. 3.Department of Pediatrics, Columbia-Presbyterian Medical Center, Babies & Children's Hospital of New York, New York, USAUS
  4. 4.Department of Orthopedic Surgery, Columbia-Presbyterian Medical Center, Babies & Children's Hospital of New York, New York, USAUS

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