Pentalogy of Cantrell

Abstract

A 2410-g infant was born at 38 weeks' gestation after an uneventful pregnancy and labor. The child was noted to be cyanotic and to have a 4-cm substernal omphalocele, which was pulsatile. The sternum was thought to be bifid inferiorly. Plain radiographs of the chest and abdomen (Figure 1 DENOUEMENT AND DISCUSSION Pentalogy of CantrellThe abdominal sonogram and MRI demonstrated an omphalocele containing a small portion of liver, bowel loops, and a trilobed tubular structure with arterial flow that could be traced directly to a diverticulum arising from the cardiac apex. These studies also demonstrated the bifid sternum and defect in the diaphragm through which the ventricular diverticulum passed into the omphalocele. The cardiac sonogram demonstrated tricuspid and pulmonic atresia with a hypoplastic right heart. A diagnosis of pentalogy of Cantrell was made based on these findings. The patient was taken to the operating room. A trilobed left ventricular diverticulum was resected from the single left ventricle. A right Blalock-Taussig shunt was performed and the omphalocele repaired.This lesion, previously called thoracoabdominal ectopia cordis, was described in detail by Cantrell in 1958 and henceforth referred to as the pentalogy or pentad of Cantrell. 1,2 The pentalogy of Cantrell consists of (a) a midline supraumbilical anterior abdominal wall defect, (b) a cleft of the lower sternum, (c) a crescentic shape anterior diaphragmatic defect, (d) a defect of diaphragmatic pericardium allowing pericardial±peritoneum communication, and

Prolonged prostaglandin E1 therapy in a neonate with pulmonary atresia and ventricular septal defect and the development of antral foveolar hyperplasia and hypertrophic pyloric stenosis

“…The development of abdominal distension in a patient treated with a similar cumulative dose to ours has previously been reported (8). Total doses in excess of 1560 μg/kg have been implicated in the development of focal foveolar hyperplasia (2). However, as seen above, cumulative doses at which symptoms developed were very different from case to case, which is why we believe that future research should focus more on the association between total doses of prostaglandin E1 administered and the development of specific side effects of treatment.…”