Korean J Obstet Gynecol.  2004 Dec;47(12):2490-2493.

A Case of Cantrell's Pentalogy with Anencephaly Diagnosed by Prenatal Ultrasonography

Affiliations
  • 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.

Keyword

Cantrell's Pentalogy; Anencephaly; Ectopia cordis; Omphalocele

MeSH Terms

Abdominal Wall
Anencephaly*
Autopsy
Brain
Diaphragm
Ectopia Cordis
Hernia, Umbilical
Live Birth
Neural Tube Defects
Pentalogy of Cantrell*
Pericardium
Prevalence
Sternum
Ultrasonography
Ultrasonography, Prenatal*
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