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Congenital hydrocephalus is a condition present at birth characterized by abnormal accumulation of cerebrospinal fluid (CSF) within the brain's ventricular system, leading to increased intracranial pressure and potential brain damage. This condition affects approximately 1-2 per 1,000 live births and can result from various developmental abnormalities affecting CSF production, circulation, or absorption.
The condition develops due to disruption in normal CSF dynamics, including overproduction of CSF, obstruction of CSF flow through ventricular pathways, or impaired CSF absorption at the arachnoid granulations. Common causes include neural tube defects, aqueductal stenosis, Chiari malformations, and genetic mutations affecting brain development, leading to progressive ventricular dilatation and increased intracranial pressure that can damage surrounding brain tissue.
Diagnosis involves recognizing key clinical signs such as macrocephaly, bulging fontanelles, developmental delays, and neurological symptoms, confirmed through neuroimaging studies like ultrasound or MRI showing ventricular enlargement. Early identification is crucial as untreated hydrocephalus can lead to permanent neurological deficits, making prompt surgical intervention with ventriculoperitoneal shunting or endoscopic third ventriculostomy essential for optimal outcomes and normal neurodevelopment.
Key imaging focus: Ventricular dilation, transependymal flow, Evans index