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porencephaly

cyst or cavity in the cerebral hemispheres Classification: - type 1 - usually unilateral - results from focal destructive lesions after 12th week of gestation - fetal vascular occlusion - vascular infections - traumatic disorders - birth trauma - type 2 (schizencephalic porencephaly) - usually symmetric & represents a primary defect or arrest in the development of the cerebral ventricles Epidemiology: rare Pathology: - cyst or cavity in the brain may contain CSF Genetics: - associated with defects in COL4A1

Specific

hydrancephaly schizencephaly

General

neural tube defect cephalic malformation

Database Correlations

OMIM 175780

References

  1. OMIM :accession 175780
  2. NINDS Porencephaly Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Porencephaly-Information-Page