PENTALOGY OF CANTRELL IN TWINS: A CASE REPORT

INTRODUCTION. Pentalogy of Cantrell is a very rare congenital multiple malformation with the poor outcome. Syndrome included the following pentad: abdominal wall defect, a defect of the lower sternum, anterior diaphragm defect, ectopia cordis, as well as a variety of intracardiac anomalies.

In the fifteenth week of the twin pregnancy, ultrasound examination showed multiple anomalies in one, as well as the absence of the nose apex in the other twin. The autopsy of the fetuses was performed after the abortion had been approved by the Ethics Committee. The external examination of the fetus number one showed ectopic heart and omphalocele. The internal examination revealed the existence of sternum cleft, ectopic heart and part of the left lung outside the thoracic cavity, the presence of diaphragmatic hernia with protrusion of abdominal organs, heart with only one large vessel and the horseshoe kidney. The full expression of the Pentalogy of Cantrell was confirmed. The external and internal examination of the other twin showed just agenesis of the nose apex.

Recognition and diagnosis of the syndrome is of the exceptional importance. Proper and timely diagnosis should contribute to good outcome of the pregnancy and delivery.