Ondine’s curse: clinical presentation with diaphragmatic pacing and spontaneous respiratory recovery. Illustrative case

Alexander J. Schupper, Alex Devarajan, Dong Seok Lee, Enrique Perez, Raj K. Shrivastava

Research output: Contribution to journalArticlepeer-review

Abstract

BACKGROUND The complexity of posterior fossa surgery can often lead to rare complications due to the anatomy involved. Vestibular schwannoma resection is a common pathology in the posterior fossa, often requiring surgical intervention. Given the proximity of this space to the brainstem, cranial nerve VII/VIII complex, and posterior inferior cerebellar artery (PICA), neurovascular complications are not infrequent. A rare vascular complication from this surgical approach is a lateral medullary infarction from injury to the lateral medullary segment of the proximal PICA, leading to central hypoventilation syndrome (CHS). OBSERVATIONS This report presents a unique case of a 51-year-old man who underwent a retrosigmoid craniectomy for resection of a vestibular schwannoma. Following surgery, the patient was unable to be weaned off the ventilator and was noted to become apneic while he slept, a clinical picture consistent with Ondine’scurse. LESSONS This report discusses the anatomical considerations of this surgical corridor leading to this complication and the management of a patient with acquired Ondine’s curse and reviews the scarce literature on this uncommon cause of acquired CHS.

Original languageEnglish
Article numberCASE233
JournalJournal of Neurosurgery: Case Lessons
Volume5
Issue number21
DOIs
StatePublished - 2023

Keywords

  • Ondine’s curse
  • central hypoventilation syndrome
  • diaphragmatic pacing
  • posterior fossa
  • skull base surgery

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