Images in Clinical Practice

Indian Pediatrics 2002; 39:501

Cantrells Pentalogy

A female baby weighing 2.5 kg was born by Caesarean section to a non-consanguienous Muslim primigravida aged 21 years. The mother was not suffering from any illness related to pregnancy. There was no history of intake of any teratogens or exposure to unusual environment in antenatal period. The baby was cyanosed at with with a 1 min Apgar score of four. On examination, the neonate had an omphalocele (9 cm × 8 cm) with a visible cardiac impulse on its cephalic end, and the umbilical cord attached at the caudal end of the sac (Fig. 1). The thorax was well formed and breath sounds were well heard all over chest, but no heart sound was audible over the precordium. 

Fig. 1. Photograph showing large omphalocele containing heart.

There was a systolic murmur Grade II/VI audible over the apex with radiation upwards. Chest X-ray revealed absence of heart shadow (Fig. 2). Echo-cardiography revealed that there was a single atrium and single ventricle with a diverticulum from its left wall. The baby was referred to a cardio-thoracic surgeon . At surgery, there was a distal sternal cleft, partially absent pericardium and absent central part of diaphragm but both the cupula were well formed. The patient died two days after the surgery. This neonate had all the components of Cantrells anomaly, i.e., sternal defect, diaphragmatic defect, absence of pericardium, abdominal wall defect, and cardiac anomaly.

Fig. 2. Chest X-ray showing absence of heart shadow in the chest.