encephalocele

• congenital
  a/w
  • agenesis of corpus callosum
  • Arnold-Chiari malformation
  • heterotopic gray matter
  • Dandy-Walker cyst
  • fused frontal horns (holoprosencephaly?)
  • craniolacunae

• acquired
  • skull fx with leptomeningeal cyst
  • surgery

• site: occipital midline (75%), fronto-ethmoidal (15%), parietal
• elevated alpha fetoprotein (aFP)

• DDx: cystic hygroma
• part of Meckel-Gruber syndrome

© 2002, C.E. Kahn, Jr.
2002-04-18