An Autopsy Case of Holoprosencephaly

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An autopsy case of holoprosencephaly was reported. Her birth weight was 2800 g and head circumference, 31.5 cm. She had median cleftlip, ocular hypotelorism and flat nose. Then stridor appeared at 3 weeks of age and tonic convulsion of extremities at 9 weeks of age. On examination the knee and ankle reflexes were hyperactive. She had normal chromosomes. She died at 1 year 8 months of age. The gross findings of the brain at necropsy were as follows. 1) Cerebral hemispheres were less developed than the other areas, especially in frontal and occipital lobes. 2) The interhemispheric fissure was interrupted anteriorly and the both lateral ventricles were in communication. 3) The primary sulci of hemispheres were present. 4) The anterrior circulation derived from 3 arterial trunks ; 1 anterior cerebral artery and 2 middle cerebral arteries. There was no abnormality in the vertebro-basilar system. Considering from many other previous reports, we could devide the anterior circulation of holoprosencephaly into three types as below. 1) two anterior cerebral arteries and two middle cerebral arteries 2) one anterior cerebral artery and two middle cerebral arteries 3) two middle cerebral arteries alone
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