Craniocervical decompression for cervicomedullary compression in pediatric patients with achondroplasia

J. Aryanpur, O. Hurko, C. Francomano, H. Wang, B. Carson

Research output: Contribution to journalArticle

Abstract

The congenital osseous abnormalities associated with achondroplasia include stenosis of the foramen magnum and the upper cervical spinal canal. In the pediatric achondroplastic patients, such stenosis may lead to cervicomedullary compression with serious sequelae, including paresis, hypertonia, delayed motor milestones, and respiratory compromise. Using a standardized protocol the authors have treated 15 young achondroplastic patients with documented cervicomedullary compression by craniocervical decompression and duroplasty. Following this procedure, significant improvement in presenting neurological or respiratory complaints was noted in all patients. The mortality rate in this series was zero. The major cause of morbidity associated with this procedure was perioperative cerebrospinal fluid (CSF) leakage from the surgical wound, presumably related to coexisting abnormalities of CSF dynamics. This problem was successfully managed by temporary or, when necessary, permanent CSF diversion procedures. It is concluded that craniocervical decompression is an effective and safe treatment for young achondroplastic patients with cervicomedullary compression.

Original languageEnglish (US)
Pages (from-to)375-382
Number of pages8
JournalJournal of neurosurgery
Volume73
Issue number3
DOIs
StatePublished - Jan 1 1990

Keywords

  • achondroplasia
  • children
  • craniocervical decompression
  • hydrocephalus
  • spinal compression

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

Fingerprint Dive into the research topics of 'Craniocervical decompression for cervicomedullary compression in pediatric patients with achondroplasia'. Together they form a unique fingerprint.

  • Cite this