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¾È¸é ±âÇüÀ» µ¿¹ÝÇÏÁö ¾ÊÀº îïðûÒà(Holotelencephaly) -1 ºÎ°Ë Áõ·Ê- Holotelencephaly, Alobar type, without Facial Anomaly (an autopsy case)

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ÁÖÁ¾Àº/Jong Eun Joo ÀÌÁ¤Èñ/ÀÓÀμ÷/°íÀÏÇâ/Jung Hee Lee/In Sook Lim/Ill Hyang Ko

Abstract


This case was a stillborn male infant to 26 years old multiparous mother, who was
born after 41 weeks of gestation. During this gestational period, the mother was careful
and uneventful. Antenatal roentgenogram of simple abdomen revealed severe
hydrocephalic appearance and craniotomy followed by induced vaginal delivery was
performed. This infant was 3.1§¸ in weight and 50§¯ in height, showing enlarged head
with increased occipitofrontal length of 15.0§¯ There were no anomalies in face,
extremities and others on external examination.
In internal examination, a typical alobar holotelenceptaly was observed without other
extracranial anomalies except for Meckel's diverticulum. In the past history of the
mother, she delivered first baby, male, 2 years ago. The baby was dead within several
hours after birth and was anencephaly with mongoloid feature and short extremities.
Reviewing of literatures and pathogenesis of the anomaly have been discussed.

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