Pediatric Hydrocephalus
Pediatric hydrocephalus is the enlargement of the brain ventricles and a rise in intracranial pressure caused by excess accumulation of cerebrospinal fluid (CSF). In infants, rapid head circumference growth is typical. Treatment usually restores CSF balance with a ventriculoperitoneal shunt or endoscopic third ventriculostomy.
마지막 업데이트: 2026-06-07
Definition
Hydrocephalus is the excessive accumulation of cerebrospinal fluid (CSF) in the ventricles, with raised intracranial pressure, resulting from a disturbed balance of CSF production, circulation and absorption. CSF is produced by the choroid plexus, circulates through the ventricular system and is absorbed from the subarachnoid space into the venous circulation. In children, disruption of this balance leads to ventricular enlargement and increased pressure.
Types and Causes
Hydrocephalus arises through two main mechanisms. In obstructive (non-communicating) hydrocephalus there is a blockage along the CSF pathway (for example aqueductal stenosis, tumor or cyst). In communicating hydrocephalus, absorption is impaired (after hemorrhage or meningitis) or, rarely, production is increased. Common causes in children include congenital malformations (aqueductal stenosis, Dandy-Walker, Chiari malformation), intraventricular hemorrhage in premature infants, infections and posterior fossa tumors.
Symptoms
Because the fontanelles are open in infants, findings include rapid head circumference growth (macrocephaly), a tense, bulging fontanelle, separation of the cranial sutures, irritability, feeding difficulty and downward deviation of the eyes (the 'sunset' sign). In older children the rigid skull causes pressure to rise quickly, producing headache that is worse in the morning, vomiting, double vision, balance and gait disturbance, drowsiness and attention problems.
Diagnosis
Imaging is central to diagnosis. Computed tomography (CT) gives rapid information in emergencies and shows ventricular enlargement. Magnetic resonance imaging (MRI) provides more detailed anatomy and reveals the cause of obstruction; a CSF flow study (phase-contrast MRI) helps locate the blockage and predict the likelihood of success with endoscopic treatment. In infants with an open fontanelle, cranial ultrasound and head circumference monitoring are used, and fundoscopy looks for papilledema.
Treatment
The aim of treatment is to restore CSF balance and prevent brain injury. The ventriculoperitoneal (VP) shunt is the most commonly used method; a catheter and a pressure-regulating valve divert CSF from the ventricle to the abdominal cavity and usually remain in place long term. Endoscopic third ventriculostomy (ETV) is an alternative to shunting, particularly in obstructive hydrocephalus, creating an opening in the floor of the third ventricle to redirect CSF to the basal cisterns. In tumor-related cases, tumor removal may resolve the hydrocephalus. In acute symptomatic cases, a temporary external ventricular drain may be placed.
Shunt Follow-up and Course
Although shunts are effective, they may block, become infected or cause over- or under-drainage over time, so revision may be needed and regular follow-up is important. Programmable valves allow pressure adjustment. With early diagnosis and appropriate treatment many children develop normally; the risk of neurological impairment rises with delayed diagnosis or recurrent shunt problems. Outcomes depend on the underlying cause and the patient, and none can be guaranteed.
참고 문헌
- Greenberg MS. Greenberg's Handbook of Neurosurgery. 10th ed. Thieme; 2023:426-451.
- Winn HR, ed. Youmans Neurological Surgery. 6th ed. Saunders; 2011:1987-1992.
- Kulkarni AV, Riva-Cambrin J, Browd SR, et al; Hydrocephalus Clinical Research Network. Endoscopic third ventriculostomy and choroid plexus cauterization in infants with hydrocephalus. J Neurosurg Pediatr. 2018;21(3):214-223.
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