Anesthesiologist's Concerns About Dandy-Walker Syndrome: Airway Management, Muscle Relaxants, and Train-of-Four Monitoring of Neuromuscular Blockade.

Abstract

Dandy-Walker syndrome is a rare congenital malformation of the posterior fossa that presents unique anesthetic challenges, including difficult airway management, altered consciousness, hydrocephalus, and potential for prolonged postoperative ventilation. Hydrocephalus, the most common finding, can complicate ventilation and intubation, whereas brainstem involvement, along with agenesis of the corpus callosum, pontine hypoplasia, and distortion of the medullary respiratory centers, may contribute to prolonged postoperative ventilation. Anesthetic management in such cases requires thorough airway assessment, preparation for potential airway difficulties, intracranial pressure monitoring and control, and total intravenous anesthesia (TIVA) to facilitate rapid emergence. We describe the case of a 3-year-old male with macrocephaly, movement disorders, delayed cognitive development, and altered mental status, scheduled for ventriculoperitoneal shunt placement under TIVA without muscle relaxants, with careful airway preparation and intracranial pressure control. To our knowledge, this is the first reported case of Dandy-Walker syndrome managed in this manner.