Congenital malformations of various sorts account for a large percentage of childhood hydrocephalus but associated Craniovertebral junction (CVJ) anomaly has not been reported earlier. Though the anaesthetic concerns for isolated arrested hydrocephalus and CVJ anomaly has been reported but the concomitant occurrence of both and its anaesthetic implications is not mentioned in literature. Here we present the anaesthetic management of a child with arrested hydrocephalus along with CVJ anomaly leading to compression of cervicomedullary junction and myelopathy scheduled for decompression and fixation of craniovertebral junction.

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