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Diffuse choroid plexus hyperplasia: an under-diagnosed cause of hydrocephalus in children?
Azian Abd Aziz1, Lee Coleman, Andrew Morokoff
1Department of Medical Imaging, The Royal Children's Hospital Melbourne, Flemington Road, Parkville, Victoria, 3052, Australia.
Insights
Diffuse villous hyperplasia of the choroid plexus is a rare cause of hydrocephalus in children. Surgical resection of the hyperplastic choroid plexus is the recommended treatment, not shunt insertion.
Area of Science:
- Pediatric Neurology
- Neurosurgery
- Histopathology
Background:
- Hydrocephalus is a common neurological disorder in children with diverse etiologies.
- Advancements in imaging, like MRI, aid in identifying rarer causes.
- Congenital aqueduct stenosis was the initial diagnosis for the patient's hydrocephalus.
Observation:
- An 11-year-old boy presented with shunt dysfunction.
- Imaging revealed enlarged choroid plexuses but did not confirm aqueduct stenosis.
- Cerebrospinal fluid (CSF) overproduction was confirmed via externalized ventricular drain.
Findings:
- Diffuse villous hyperplasia of the choroid plexus was surgically resected.
- Histological examination confirmed choroid plexus hyperplasia.
- This finding challenged the initial diagnosis of aqueduct stenosis.
Implications:
- Choroid plexus hyperplasia represents a distinct cause of pediatric hydrocephalus.
- Neurosurgical management should prioritize resection of hyperplastic choroid plexus over shunt placement.
- Accurate diagnosis is crucial for effective treatment of hydrocephalus.
Abstract:
Hydrocephalus is a common neurological disorder in children and the result of a variety of causes. However, with the advancement of imaging modalities, particularly MRI, previously reported rarer causes of hydrocephalus in children are now being more readily appreciated. We report an 11-year-old boy with diffuse villous hyperplasia of the choroid plexus. He had a ventriculo-peritoneal (VP) shunt in-situ and a prior diagnosis from infancy of congenital aqueduct stenosis as the cause of his hydrocephalus. His current presentation was with further shunt dysfunction. CT and MRI demonstrated enlarged choroid plexuses but did not confirm aqueduct stenosis. CSF overproduction was demonstrated from the externalized ventricular drain. The enlarged choroid plexuses were surgically resected and histology confirmed choroid plexus hyperplasia. Identification of choroid plexus hyperplasia is important since the neurosurgical management of hydrocephalus is not VP shunt insertion, but resection of the hyperplastic choroid plexus.
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