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KMID : 0357919800140030056
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Korean Journal of Pathology
1980 Volume.14 No. 3 p.56 ~ p.61
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Exencephalus (Two autopay cases report)
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Abstract
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Exencephalus is a variant of anencephaly. It probably sits morphologically between
classic anencephaly and encephaloele. A significant portion of prosencephalon can be
recognized in the head where vertex structure is regularly missing. Diencephalon and
rhombencephalon are fairly well preserved in almost every case. Microscopically
isocortical lamination is present and cerebral vessels are relatively well developed.
Recently authors experienced two autopsy cases of these rare anomalies.
The first case was a product after 29 weeks gestation from a 26 years old female who
had a history of carbon monoxide poisioning during the ear]y gestation period. The baby
died 2 hours after delivery. She weighed 1.02㎏. and measured 47.5㎝. in height. She had
typical massive exencephalic acrania along with facial and phalangeal anomalies. The
exencephalic mass was 9.0×5.0×4.0㎝. and weighed 120gm. Structures suggestive of
gyri and sulci were noted, and these structures were confirmed by microscopic
examination.
The second case was a product after 30 weeks gestation from a 25 years old female
who had no noticeable abnormalities during early gestation period. The baby died soon
after delivery. He weighed 1.2㎏. and measured 53㎝. in height. He also had typical
massive exencephalic acrania. The exencephalic mass was 8.0×6.0×4.0㎝. and was
150gm. in weight. Structures of gyri and sulci were noted, along with opendyma-lined
thalamic structure.
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