Korean J Obstet Gynecol.
2004 Dec;47(12):2490-2493.
A Case of Cantrell's Pentalogy with Anencephaly Diagnosed by Prenatal Ultrasonography
- Affiliations
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- 1Department of Obstetrics and Gynecology, Wonju Medical College, Yonsei University, Wonju, Korea.
- 2Department of Pathology, Pochon CHA University College of Medicine, Pochon, Korea.
Abstract
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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.