Pentalogy of Cantrell in a resource-limited setting: A case report

Abstract

Introduction

Pentalogy of Cantrell is a rare congenital condition involving defects in the abdominal wall, sternum, diaphragm, pericardium, and heart. This report adds to the scarce literature by presenting a unique case from a resource-limited setting.

Case presentation

An 8-h-old male neonate was admitted to the NICU with a thoracoabdominal defect observed at birth. Delivered in transit at term to a healthy mother with no prenatal anomalies identified, the neonate exhibited a pulsatile mass from the xiphisternum to the umbilicus, covered by a thin membrane with visible cardiac activity. Imaging and clinical evaluation confirmed herniation of the heart and liver through the defect, consistent with ectopia cordis and omphalocele, accompanied by a sternal defect and diaphragmatic communication. Echocardiography revealed severe intracardiac anomalies, including a single ventricle, single atrium, truncus arteriosus, and mild pericardial effusion. Additionally, a meningomyelocele was identified alongside bilateral clubfoot deformities. Initial management included stabilization with fluids, antibiotics, oxygen, and protective dressings for the exposed structures. The neonate experienced cardiac arrest on the third day of admission, and surgical intervention was not performed.

Conclusion

Prenatal screening and prompt multidisciplinary intervention are essential to improve outcomes in cases of Pentalogy of Cantrell.