Endoscopic third ventriculostomy in hydrocephalus associated with achondroplasia

Report of 3 cases

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  • 1 Neurosurgeons for Children and
  • 3 Perot Family Center for the Care of Brain & Nerve Injuries, Children's Medical Center, Dallas; and
  • 2 Department of Pediatric Neurosurgery, Texas Tech University Health Science Center, Lubbock, Texas
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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.

Abbreviations used in this paper: CTH = cerebellar tonsillar herniation; ETV = endoscopic third ventriculostomy; ICP = intracranial pressure; VP = ventriculoperitoneal.

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Contributor Notes

Address correspondence to: Dale Swift, M.D., P.O. Box 0205, Dallas, Texas 75235-0205. email: dswift@nsfc.us.

Please include this information when citing this paper: DOI: 10.3171/2011.10.PEDS1169.

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