Abstract

Hydrocephalus (HC) is the most common condition treated by pediatric neurosurgeons. The etiologies of HC span a spectrum of congenital and acquired causes, which all result in pathologic accumulation of cerebrospinal fluid (CSF) and elevated intracranial pressure (ICP). Common congenital causes of HC are aqueductal stenosis, myelomeningocele, Dandy-Walker malformation, and other generalized malformations of brain development. Acquired causes of HC are most commonly associated with intraventricular hemorrhage (IVH) of prematurity, infection, brain tumor, or trauma. Ultrasonography and magnetic resonance imaging are the diagnostic tools of choice for HC. More recently, advanced imaging modalities such as diffusion tensor imaging are used to correlate and predict neurocognitive outcome of HC. The mainstay treatment of HC is placement of ventriculoperitoneal shunts; however, given the associated lifetime comorbidities associated with shunts, alternative treatments for HC are increasingly adopted in the current management strategy of HC. Prenatal closure of myelomeningocele has been shown to decrease the incidence of HC. Endoscopic third ventriculostomy (ETV) has a high rate of success in older patients with obstructive etiologies. ETV with choroid plexus cauterization (CPC) is being investigated for infants with low success rate with ETV alone. Yet, many areas of uncertainty remain regarding the optimal treatment of HC. For example, it is unclear whether a larger ventricle size of ETV-treated HC has an impact on neurocognitive development or whether decreasing clot burden in IVH of prematurity could decrease the need for shunting. Moving forward, prospective multicenter trials would be needed to address these pressing questions.

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