Hydrocephalus is one of the most common congenital abnormalities affecting the nervous system, occurring in 0.3 to 2.5 per 1,000 live births. It results from obstruction of cerebrospinal fluid (CSF) pathways by a diverse range of developmental, genetic, and acquired abnormalities and can have negative consequences on the neurodevelopmental outcome of affected neonates. Historically, hydrocephalus was diagnosed after birth and managed with a shunt procedure; however, with the advent of advanced antenatal imaging techniques, it may now be detected and treated before delivery in some individuals. Moreover, surgical options for the treatment of hydrocephalus have increased over the past few decades, and temporary CSF diversion may prevent the need for permanent shunt placement. Posthemorrhagic hydrocephalus is the most common cause of hydrocephalus in the preterm newborn, but the timing of surgical intervention for this condition remains controversial. Recent evidence suggests that earlier, rather than later, intervention may have some benefits, but more data are needed to adequately inform clinical practice. Regardless of etiology or timing of diagnosis, a thorough understanding of the natural history of hydrocephalus and the range of treatment options available is needed for parental counseling, prognostication, and appropriate surgical management.
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