Upward transtentorial herniation following frontoorbital advancement for syndromic craniosynostosis: Case report

Vivek Mehta, Joshua Bakhsheshian, Amir H. Dorafshar, Edward S. Ahn

Research output: Contribution to journalArticlepeer-review

Abstract

The authors describe the case of a boy with Muenke syndrome, an autosomal dominant disorder associated with craniosynostosis. The family history was significant for syndromic craniosynostosis in the patient's maternal grandmother, who died in adulthood after a craniofacial reconstruction. The patient, her grandson, underwent craniofacial reconstruction surgery at the age of 9 months and developed upward transtentorial herniation. Imaging findings revealed remote cerebellar hemorrhage after a large quantity of supratentorial CSF was drained during postoperative Day 1. The clinical course was further complicated by cerebral sinus thrombosis, which was diagnosed after a fourth surgical procedure. Upward transtentorial herniation can occur when a significant increase in intracranial pressure in the posterior fossa causes displacement of the central lobule and superior surfaces of the cerebellum upward through the incisura tentorii. This is a rare but well-documented phenomenon that commonly occurs in the setting of an expansive posterior fossa lesion or excessive supratentorial CSF loss. To help clinicians recognize and prevent this rare but potentially fatal complication, the authors review the postulated mechanisms by which this process may occur.

Original languageEnglish (US)
Article numberE8
JournalNeurosurgical focus
Volume38
Issue number5
DOIs
StatePublished - Jan 1 2015

Keywords

  • Herniation
  • Intracranial pressure
  • Remote intracranial hemorrhage

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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