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Ondine's curse: clinical presentation with diaphragmatic pacing and spontaneous respiratory recovery. Illustrative case.
J Neurosurg Case Lessons 2023 May 23
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's curse.
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.
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's curse.
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.
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