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Korean Journal of Obstetrics & Gynecology 2004;47(12):2490-2493.
Published online December 1, 2004.
A Case of Cantrell's Pentalogy with Anencephaly Diagnosed by Prenatal Ultrasonography.
Hyoung Chan Cho, In Bai Chung, Seong Jin Choi, Yong Hee Lee, Jong Won Ha, Jung Ju Lee
1Department of Obstetrics and Gynecology, Wonju Medical College, Yonsei University, Wonju, Korea.
2Department of Pathology, Pochon CHA University College of Medicine, Pochon, Korea.
Abstract
Pentalogy of Cantrell was very rare congenital anomaly and represent a regional prevalence of 5.5/1 million live births. The syndrome, Cantrell's pentalogy is characterized by defects in the lower sternum with ectopia cordis, anterior diaphragm defects, midline supraumbilical abdominal wall defects, defects in the diaphragmatic pericardium, and various intracardiac defects. Anencephaly involves acrania with a degenerated mass of brain tissue. Combination of both conditions are extremely rare. We experienced a case of Cantrell's pentalogy with anencephaly detected by ultrasonography at 15 weeks 1 day menstural age and confirmed at autopsy. Thus we report a case with brief review of the literature.
Key Words: Cantrell's Pentalogy, Anencephaly, Ectopia cordis, Omphalocele


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