Document Detail

Odontoid hypoplasia with vertebral cervical subluxation and ventriculomegaly in metatropic dysplasia.
MedLine Citation:
PMID:  2915283     Owner:  NLM     Status:  MEDLINE    
Our experience with 12 patients with metatropic dysplasia has demonstrated two important and treatable complications: odontoid hypoplasia with subluxation of the first and second cervical vertebrae, and ventriculomegaly. Hypoplasia and lack of ossification of the odontoid process were noted in all cases. Subluxation of these two vertebrae was demonstrated in all six patients who had lateral flexion-extension radiographs; three had subluxation even in a neutral position, and sudden odontoid dislocation developed in another after a simple fall. Four individuals have had surgical fusion of the cervical vertebrae; one child died suddenly, 1 week before scheduled surgery. In the three patients in whom computed tomography scans of the head were obtained, enlarged ventricles were found; one had symptomatic increased intracranial pressure and required a shunt. We recommend that odontoid hypoplasia be evaluated in all patients with metatropic dysplasia. If subluxation is proved, atlantoaxial fusion should be performed before damage to the cervical part of the spinal cord results. Serial head circumference measurements and evaluation for hydrocephalus are also recommended.
M Shohat; R Lachman; D L Rimoin
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  The Journal of pediatrics     Volume:  114     ISSN:  0022-3476     ISO Abbreviation:  J. Pediatr.     Publication Date:  1989 Feb 
Date Detail:
Created Date:  1989-03-17     Completed Date:  1989-03-17     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  0375410     Medline TA:  J Pediatr     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  239-43     Citation Subset:  AIM; IM    
Department of Pediatrics, Cedars-Sinai Medical Center, Los Angeles, CA 90048.
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MeSH Terms
Atlanto-Axial Joint / injuries*,  radiography
Axis / pathology*
Child, Preschool
Dislocations / complications,  etiology*,  radiography
Dwarfism / complications*,  pathology
Infant, Newborn
Odontoid Process / pathology*
Spinal Cord Compression / etiology
Grant Support

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