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Bilateral open lip schizencephaly.

Turyalai Hakimi1, Khalid Mohammad Qasem2

  • 1Department of Pediatric Surgery, Kabul University of Medical Science, Maiwand Teaching Hospital, Kabul, Afghanistan.

Annals of Medicine and Surgery (2012)
|January 26, 2022
PubMed
Summary
This summary is machine-generated.

Schizencephaly, a CNS disorder, involves abnormal cerebral clefts. A case of bilateral open lip schizencephaly with neurological issues improved after a ventriculo-peritoneal shunt procedure.

Keywords:
CleftClosed lipDevelopmental disorderOpen lipSchizencephalyVentriculomegaly

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Area of Science:

  • Neurology
  • Developmental Biology
  • Pediatrics

Background:

  • Schizencephaly is a rare central nervous system (CNS) developmental disorder.
  • It is characterized by abnormal clefts extending from the lateral ventricles to the cerebral cortex.
  • Etiologies include genetic factors, teratogen exposure, viral infections, and advanced maternal age.

Observation:

  • A case of bilateral open lip schizencephaly presented with neurological deficits.
  • The patient exhibited increased intracranial pressure (ICP) secondary to ventriculomegaly.
  • Clinical presentation included significant neurological impairments.

Findings:

  • The patient underwent a ventriculo-peritoneal (V-P) shunt procedure.
  • Post-operative follow-up revealed considerable clinical improvement.
  • The V-P shunt effectively managed the increased ICP and associated neurological symptoms.

Implications:

  • This case highlights the efficacy of V-P shunting in managing complex schizencephaly.
  • Early intervention with CSF diversion may improve neurological outcomes in affected individuals.
  • Further research into the genetic and environmental factors of schizencephaly is warranted.