The ESTHI Trial is a multi-center randomized controlled trial (RCT) comparing endoscopic third ventriculostomy with choroid plexus cauterization (ETV+CPC) and shunt in infants with hydrocephalus. The study will leverage the infrastructure of the Hydrocephalus Clinical Research Network (HCRN), a committed group of 14 leading North American pediatric neurosurgical centers with a long track-record of successful collaborative clinical research and RCTs in hydrocephalus. Optimal cognitive outcome is the primary concern of families and will, therefore, be the primary outcome. Assessment of dMRI, a validated, non-invasive method of measuring white matter microstructural integrity and structural connectivity in the developing brain, will provide further insight into the developmental consequences of these two treatments. The results of the RCT will help families determine the optimal treatment of hydrocephalus for their child.
Inclusion Criteria: 1. Corrected age <104 weeks and 0 days, AND 2. Child is ≥ 37 weeks post menstrual age, AND 3. Child must have symptomatic hydrocephalus, defined as: Ventriculomegaly on MRI (frontal-occipital horn ratio (FOR) >0.45, which approximates "moderate ventriculomegaly"), and at least one of the following: - Head circumference >98th percentile for corrected age with either bulging fontanelle or splayed sutures - Upgaze paresis/palsy (sundowning) - CSF leak - Papilledema - Tense pseudomeningocele or tense fluid along a track - Vomiting or irritability, with no other attributable cause - Bradycardias or apneas, with no other attributable cause - Intracranial pressure (ICP) monitoring showing persistent elevation of pressure with or without plateau waves AND 4. No prior history of shunt insertion or endoscopic third ventriculostomy (ETV) procedure (previous temporization devices and/or external ventricular drains permissible) Exclusion Criteria: 1. Hydrocephalus due to intraventricular hemorrhage in a child born before 37 weeks gestational age; OR 2. Anatomy not suitable for ETV+CPC or anteriorly placed ventriculoperitoneal shunt defined as: - Moderate to severe prepontine adhesions on steady state free precession (SSFP) or T2 weighted fast (turbo) spin echo (FSE/TSE) MRI, which includes the following sequences: FIESTA, FIESTA-C, TrueFISP, CISS, Balanced FFE (bFFE), CUBE, SPACE, VISTA, IsoFSE, and 3D MVOX - Closure of one or both foramina of Monro - Thick floor of third ventricle (≥ 3mm) - Narrow third ventricle (<5mm) - Presence of scalp, bone, or ventricular lesions that make placement of an anterior shunt impracticable; OR 3. Underlying condition with a high chance of mortality within 12 months; OR 4. Hydrocephalus with loculated CSF compartments; OR 5. Peritoneal cavity not suitable for distal shunt placement; OR 6. Active CSF infection; OR 7. Hydranencephaly; OR 8. Child requires an intraventricular procedure (e.g. endoscopic biopsy) in addition to the initial first-time permanent procedure for the treatment of hydrocephalus.