Pediatric Hydrocephalus: A Systematic Synthesis of Pathophysiological Mechanisms, Diagnostic Strategies, and Treatment Paradigms

Abstract

Pediatric hydrocephalus remains one of the most challenging conditions in pediatric neurosurgery. Although traditionally attributed to elevated intracranial pressure secondary to cerebrospinal fluid (CSF) obstruction, accumulating evidence suggests that it represents a broader disturbance of CSF dynamics, brain compliance, and neurodevelopment. This review aims to synthesize contemporary data on the etiological spectrum, underlying mechanisms, diagnostic approaches, and current treatment strategies in children with hydrocephalus.

A systematic search of PubMed, EMBASE, and Scopus was performed for studies published between January 2003 and January 2026. Search terms included “pediatric hydrocephalus,” “CSF dynamics,” “neonatal ultrasound,” “endoscopic third ventriculostomy,” and “low-pressure hydrocephalus.” Studies involving patients younger than 18 years were eligible. Systematic reviews, meta-analyses, cohort studies, and clinical series addressing pathophysiology, diagnosis, or management were included. Studies limited to adults, animal models, or non-English publications were excluded. Two reviewers independently screened and extracted data. Sixty-three studies met inclusion criteria and were analyzed using thematic synthesis aligned with PRISMA recommendations.

The literature reflects a gradual shift from a pressure-based model of hydrocephalus toward a dynamic framework emphasizing brain compliance and CSF circulation. Congenital hydrocephalus is increasingly linked to genetic and developmental abnormalities, whereas acquired forms remain strongly associated with intraventricular hemorrhage, infection, and tumor-related obstruction. Advances in cranial ultrasonography and magnetic resonance imaging have improved early detection and etiological characterization, particularly in neonates. Ventriculoperitoneal shunting continues to represent the most widely used treatment modality; however, endoscopic third ventriculostomy, with or without choroid plexus cauterization, has demonstrated favorable outcomes in selected cases. Low- and negative-pressure hydrocephalus has emerged as a distinct clinical entity related to altered tissue compliance and requires individualized management.

Current evidence supports viewing pediatric hydrocephalus as a heterogeneous and dynamic disorder rather than a purely pressure-driven condition. Improved understanding of CSF physiology, brain biomechanics, and genetic contributions may facilitate more individualized treatment strategies and enhance long-term neurodevelopmental outcomes.