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J Child Neurol. 2016 Mar;31(3):309-20. doi: 10.1177/0883073815592222. Epub 2015 Jul 16.

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

Author information

1
Department of Neurology, University of Washington, Seattle, WA, USA Center for Integrative Brain Research, Seattle Children's Research Institute, Seattle, WA, USA hmtully@uw.edu.
2
Department of Radiology, University of Washington, Seattle, WA, USA.
3
Department of Biostatistics, University of Washington, Seattle, WA, USA.
4
Department of Pediatrics, University of Washington, Seattle, WA, USA.
5
Department of Neurological Surgery, University of Washington, Seattle, WA, USA Center for Integrative Brain Research, Seattle Children's Research Institute, Seattle, WA, USA.
6
Department of Pediatrics, University of Washington, Seattle, WA, USA Center for Integrative Brain Research, Seattle Children's Research Institute, Seattle, WA, USA.
7
Department of Neurology, University of Washington, Seattle, WA, USA Department of Pediatrics, University of Washington, Seattle, WA, USA Center for Integrative Brain Research, Seattle Children's Research Institute, Seattle, WA, USA.

Abstract

Few systematic assessments of developmental forms of hydrocephalus exist. We reviewed magnetic resonance images (MRIs) and clinical records of patients with infancy-onset hydrocephalus. Among 411 infants, 236 had hydrocephalus with no recognizable extrinsic cause. These children were assigned to 1 of 5 subtypes and compared on the basis of clinical characteristics and developmental and surgical outcomes. At an average age of 5.3 years, 72% of children were walking independently and 87% could eat by mouth; in addition, 18% had epilepsy. Distinct patterns of associated malformations and syndromes were observed within each subtype. On average, children with aqueductal obstruction, cysts, and encephaloceles had worse clinical outcomes than those with other forms of developmental hydrocephalus. Overall, 53% of surgically treated patients experienced at least 1 shunt failure, but hydrocephalus associated with posterior fossa crowding required fewer shunt revisions. We conclude that each subtype of developmental hydrocephalus is associated with distinct clinical characteristics, syndromology, and outcomes, suggesting differences in underlying mechanisms.

KEYWORDS:

aqueductal stenosis; encephaloceles; hydrocephalus; myelomeningocele

PMID:
26184484
PMCID:
PMC4990005
[Available on 2017-03-01]
DOI:
10.1177/0883073815592222
[Indexed for MEDLINE]
Free PMC Article

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