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KMID : 0361719930040020247
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Korean Journal of perinatology
1993 Volume.4 No. 2 p.247 ~ p.255
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Three Cases of Potter Syndrome
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정경원
박혜성/박창서/박찬용/조성진/박인서
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Abstract
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Potter syndrome is a collection of fetal malformation associated with renal agenesis, pulmonary hypoplasia and characteristic facial appearance.
We experienced three neonates diagnosed by autopsy as Potter syndrome.
@EN The results were as follows:
@EN 1. The incidence of Potter syndrome were 3 cases/1,800 birth(0.17%).
2. The mean survival time of fetus was 16 hours after delivery.
3. Three cases were revealed lower hight of fundus compared with there gestational age and oligohydramnios at the time of diagnosis on sonography.
4. The maternal serum alpha-fetoprotein revealed lower level in 1 cases and elevated level in 2 cases.
5. Antenatal sonography revealed absence of both kidney in 2 cases.
6. For fetal sex, male was 2 cases, female was 1 cases.
7. The autopsy showed bilateral renal agenesis in 2 cases and unilateral renal agenesis in 1 cases.
Three cases all revealed bilateral pulmonary hypoplasia.
8. Chromosomal study revealed normal.
In spite of it's rare incidence, it's clinical presentatio and prognosis are so produnced that deserve to attention to appropriate diagnosis and genetic counseling.
We report 3 cases of these unusual anomalies with review of literatures.
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KEYWORD
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