Pentalogy of Cantrell and anaesthesia: a case report

Abstract

Pentalogy of Cantrell, a rare congenital thoracoabdominal disruption, consists of a supraumbilical abdominal wall defect, lower sternal defect, agenesis of the anterior portion of the diaphragm, an absence of the diaphragmatic part of the pericardium, and a malformation of the heart, typically as an ectopia cordis. This case report presents a female neonate, 35 weeks postconception, weighing 2080 g who presented on day four of life for anaesthesia. She had the five anatomical defects characteristic of Pentalogy of Cantrell.

Keywords: abdominal wall defects, anaesthesia, congenital heart disease, ectopia cordis, extrathoracic heart, Pentalogy of Cantrell, perioperative management, transfer difficulties in South Africa