Cantrell Syndrome (Thoracoabdominal Ectopia Cordis; Anomalous Umbilical Cord; Diaphragmatic, Pericardial and Intracardiac Defects) in the Pig (Sus scrofa domesticus).

Abstract

Pentalogy of Cantrell (PC) is a complex body wall defect, involving the abdominal wall, sternum, diaphragm, pericardium and heart. We report on six stillborn piglets with anomalous umbilical cord (UC), cranio-umbilical abdominal defect, cleft sternum, incomplete diaphragm and pericardium, ectopia cordis and intracardiac anomalies. Anomalous UC was defined as a single umbilical artery (SUA) and/or short cord, or as an UC with atypical coiling pattern. The embryonic period, in which all the anomalies found in these piglets develops, coincides with that of UC formation in the pig. We propose that anomalous UC should be considered a sixth defect in Cantrell syndrome (CS), considering that the insult leading to the classical malformations of PC and UC abnormalities is the same or that the sequence of malformations itself may alter the early fetoplacental blood flow and therefore the normal development of the UC angioarchitecture. CS has not been reported previously in animals.