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Modeling Posthemorrhagic Hydrocephalus of Prematurity in Rats
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Published on: March 28, 2025

Hydranencephaly.

S Pant1, G Kaur, J K De

  • 1Department of Obstetrics and Gynaecology, Manipal College of Medical Sciences. sadippant@hotmail.com

Kathmandu University Medical Journal (KUMJ)
|January 7, 2011
PubMed
Summary
This summary is machine-generated.

Hydranencephaly is a rare congenital brain condition where cerebral hemispheres are replaced by fluid. Early symptoms can mimic normal development, delaying diagnosis and offering a poor prognosis.

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Area of Science:

  • Neurology
  • Developmental Biology
  • Pediatrics

Background:

  • Hydranencephaly is a rare congenital neurological disorder.
  • It involves the replacement of cerebral hemispheres with cerebrospinal fluid and glial tissue.
  • Normal embryogenesis of the telencephalon is suggested by well-formed meninges and skull.

Observation:

  • Infants may present with normal head size and spontaneous reflexes at birth.
  • Initial behavior can appear relatively normal, potentially delaying diagnosis.
  • Clinical features include intact brainstem reflexes but absent higher cortical activity.

Findings:

  • Bilateral internal carotid artery occlusion in utero is a potential cause.
  • Symptoms include irritability, increased muscle tone, seizures, and hydrocephalus.
  • Other findings encompass visual impairment, deafness, spastic quadriparesis, and intellectual deficits.

Implications:

  • The condition has no definitive treatment.
  • The prognosis for affected children is generally poor, with high mortality before age one.
  • Delayed diagnosis due to initially normal behavior impacts management and outcomes.