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KMID : 0357919830170040472
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Korean Journal of Pathology
1983 Volume.17 No. 4 p.472 ~ p.476
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Hydranencephaly (An autopsy case report)
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정진숙/Jin Book Jeong
주종은/홍숙희/지제근/Jong Eun Joo/Seok Hee Hong/Je Geun Chi
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Abstract
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Hydranencephaly is characterized by absence of cerebral hemisphere forming unilocular
sac, intact meninges and a rather normal skull vault. The wall of the sac is composed
of meninges and soma of dense glial tissue and the sac is filled with cerebrospinal fluid.
This anomaly is considered to be the result of secondary destruction of a brain after
having developed to a certain degree of maturation, and accordingly should be
distinguished from anencephaly that is always associated with acrania.
We report a case of hydranencephaly in a still-born female delivered after 38 weeks
gestation from a 33 year-old multipara woman who was uneventful during this
pregnancy. She measured 4.1㎏ in weight and 54.0㎝ in height. There was no anomalies
on external examination except for an enlarged head, 40㎝ in occipito-frontal
circumference. The brain was changed to unilocular sac showing remnants of cerebrum,
midbrain, and cerebellum on the floor of the cranium. The sac was filled with
xan-thochromic cerebrospinal fluid, about 600㏄ in toto, and was walled off by intact
meninges and skull bone. Microscopically, the thin membranous wall of the sac
consisted of leptomeningeal layers closely connected with inner glial tissue. The
cerebellum and remaining brainstem as well as spinal cord were preserved. No other
internal anomalies were found.
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