American journal of diseases of children (1960)
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Three children had hydrocephalus associated with myelomeningocele and the Arnold-Chiari malformation. They all experienced neck pain and opisthotonos, followed by the sudden onset of respiratory arrest as a result of the malfunction of a CSF shunt. There were no changes in pupillary response, and each patient remained alert until shortly before the respiratory arrest. ⋯ The mechanism of acute respiratory arrest seems to be acute compression of the brainstem as a result of hydrocephalus. The supratentorial pressure is direct on the posterior fossa structures through the enlarged tentorial opening, which is one of the characteristics of the brain in the Arnold-Chiari malformation. Sudden respiratory arrest, a life-threatening complication, is a result of a malfunction of the CSF shunt in children with myelomeningocele and requires prompt surgical decompression.