Partial Cantrell’s Pentalogy

A 12-month-old boy with a history of repeated respiratory infections was referred to our university hospital for evaluation of a pulsating vascular channel at the midline of the anterior abdominal wall. Clinical examination revealed a systolic murmur heard all over the chest with maximum intensity at the left sternal border. Abdominal examination revealed a thick pulsating vascular channel at the midline of the anterior abdominal wall extending down to the umbilicus, together with diastasis of the recti. Echocardiography revealed a large perimembranous ventricular septal defect (VSD) and failed to delineate the nature of the pulsating vascular channel.

Multislice computed tomography with intravascular contrast performed with a 64-slice scanner revealed the presence of a ventricular diverticulum originating from the apex of the left ventricle and extending down to the level of the umbilicus. In addition, pericardial and diaphragmatic defects were detected (Figures 1 -3). These findings are consistent with a partial presentation of Cantrell’s pentalogy ¹ with presence of pericardial defect, diaphragmatic defect, abdominal wall defect (here, diastasis of recti), and a ventricular diverticulum (here with a VSD, a common association), but there is no sternal defect.

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Figure 1.

Oblique section of computed tomography of chest and abdomen showing ventricular diverticulum (arrow) extending down to the umbilicus.

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Figure 2.

Sagittal section of computed tomography of the chest and abdomen showing ventricular diverticulum (arrow) extending down to the umbilicus.

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Figure 3.

Computed tomographic image showing origin of diverticulum form the apex of the left ventricle.

Elective surgery was planned, but the patient failed to appear on the appointed date. Efforts to reach the family, who live in a remote rural area, have been unsuccessful. No follow-up information is available.