| A case of Prune Belly syndrome associated with holoprosencephaly. |
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Myung Hee Youn1, Mi Sung Jeong1, Hyung Ja Park1, Shinna Kim1, Keum Min Park2, Song Ja Chin2 |
1Department of Pediatrics, Dong Boo City Hospital, Seoul, Korea
2Department of Pathology, Dong Boo City Hospital, Seoul, Korea |
| Holoprosencephaly 와 동반된
Prune Belly 증후군 1례 |
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윤명희1, 정미성1, 박형자1, 김신나1, 박금민2, 진송자2 |
1서울시립 동부병원 소아과
2서울시립 동부병원 병리과 |
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Received: 12 February 1991 • Accepted: 15 April 1991 |
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| Abstract |
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This is brief report of a case of prune belly syndrome associated with holoprosencephaly in one-day
old neonate.
At birth, physical examination revealed skull bone defect, anophthalmia, severe hypotelorism,
arhinia with proboscis, micrognathia, webbed neck, severely dilated abdomen with thin wall, agenesis
of external urogenital organs and anus, equinovalgus and genu valgus.
Autopsy findings were alobar holoprosencephaly with hydrocephalus, hypoplasia of lung and ribs,
markedly dilated cystis (14x11.5x6cm) with hypertrophied muscular layer of 1.2cm in thickness,
mild hydroureter, polycystic kidney (Potter type II and IV), colonic atresia and agenesis of gallblad-
der, pancreas and internal genital organs. |
| Key Words:
Prune belly syndrome, Alobar holoprosencephaly |
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