Cervicomedullary decompression for foramen magnum stenosis in achondroplasia

Carlos A. Bagley, Jonathan A. Pindrik, Markus J. Bookland, Joaquin Q. Camara-Quintana, Benjamin S. Carson

Research output: Contribution to journalArticle

Abstract

Object. Achondroplasia is the most common hereditary form of dwarfism, and is characterized by short stature, macrocephaly, and a myriad of skeletal abnormalities. In the pediatric population, stenosis and compression at the level of the cervicomedullary junction commonly occurs. The goal in this study was to assess the outcomes in children with achondroplasia who underwent cervicomedullary decompression. Methods. Forty-three pediatric patients with heterozygous achondroplasia and foramen magnum stenosis underwent 45 cervicomedullary decompressions at the authors' institution over an 11-year period. After surgical decompression, complete resolution or partial improvement in the preoperative symptoms was observed in all patients. There were no deaths in the treated patients. The surgical morbidity rate was low and usually consisted of a cerebrospinal fluid (CSF) leak in patients in whom the dura mater had been opened (either intentionally or accidentally). This problem was successfully managed in all cases with local measures (wound oversewing) or CSF diversion. Conclusions. In this review the authors demonstrate that decompression of the cervicomedullary junction in the setting of achondroplasia may be accomplished safely with significant clinical benefit and minimal morbidity.

Original languageEnglish (US)
Pages (from-to)166-172
Number of pages7
JournalJournal of neurosurgery
Volume104 PEDIATRICS
Issue numberSUPPL. 3
StatePublished - Mar 1 2006

Keywords

  • Achondroplasia
  • Cervicomedullary compression
  • Decompression surgery
  • Foramen magnum
  • Pediatric neurosurgery

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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  • Cite this

    Bagley, C. A., Pindrik, J. A., Bookland, M. J., Camara-Quintana, J. Q., & Carson, B. S. (2006). Cervicomedullary decompression for foramen magnum stenosis in achondroplasia. Journal of neurosurgery, 104 PEDIATRICS(SUPPL. 3), 166-172.