Endoscopic third ventriculostomy in hydrocephalus associated with achondroplasia: Report of 3 cases

Dale Swift, Laszlo Nagy, Brian Robertson

Research output: Contribution to journalArticle

16 Scopus citations

Abstract

Hydrocephalus in patients with achondroplasia is thought to be due to increased dural sinus venous pressure resulting from narrowing of the jugular foramen. In this setting, where hydrocephalus is presumed to be "vascular" in origin and therefore communicating, endoscopic third ventriculostomy (ETV) would seem contraindicated. The authors describe 3 patients in whom ETV was successfully performed, resulting in MR imaging-documented decreases in ventricle size. The patients were 11 months, 33 months, and 13 years at the time of surgery. All patients had serial preoperative MR images demonstrating progressive hydrocephalus in a "triventricular" pattern with a small fourth ventricle but an open aqueduct. All patients had undergone suboccipital decompression for foramen magnum stenosis prior to the treatment of hydrocephalus. Preoperative retrograde venography revealed variable pressure gradients across the jugular foramen. It is postulated that the increase in intracranial venous pressure resulting from jugular foramen stenosis may lead to disproportionate venous engorgement of the cerebellum and some degree of obstructive hydrocephalus amenable to ETV. The authors discuss the role of suboccipital decompression in the progression of hydrocephalus in patients with achondroplasia.

Original languageEnglish (US)
Pages (from-to)73-81
Number of pages9
JournalJournal of Neurosurgery: Pediatrics
Volume9
Issue number1
DOIs
StatePublished - Jan 1 2012

Keywords

  • Achondroplasia
  • Endoscopic third ventriculostomy
  • Hydrocephalus

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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