Two Hundred Thirty-Six Children With Developmental Hydrocephalus: Causes and Clinical Consequences

Area of Science:

• Pediatric Neurology
• Developmental Biology
• Medical Imaging

Background:

• Hydrocephalus is a common condition in infants.
• Systematic assessments of developmental hydrocephalus are limited.
• Infancy-onset hydrocephalus requires detailed classification for understanding outcomes.

Purpose of the Study:

• To systematically assess developmental forms of hydrocephalus.
• To classify infancy-onset hydrocephalus into subtypes.
• To compare clinical characteristics and outcomes across subtypes.

Main Methods:

• Review of magnetic resonance images (MRIs) and clinical records.
• Analysis of 411 infants with infancy-onset hydrocephalus.
• Assignment of 236 infants to 5 subtypes based on hydrocephalus without extrinsic cause.

Main Results:

• 72% of children walked independently and 87% could eat by mouth by age 5.3.
• 18% of children developed epilepsy.
• Subtypes with aqueductal obstruction, cysts, and encephaloceles showed worse outcomes.
• 53% of surgically treated patients had shunt failure; posterior fossa crowding required fewer revisions.

Conclusions:

• Each subtype of developmental hydrocephalus presents distinct clinical features and syndromology.
• Subtyping aids in predicting developmental and surgical outcomes.
• Distinct subtypes suggest varied underlying pathological mechanisms.