Pentalogy of Cantrell: A Report of Three Cases

Pentalogy of cantrell is a rare upper midline syndromethat may present in association with anomalies outside thetorso. the pentad — the supraumbilical body wall defect,sternal defect, deficiency of the anterior diaphragm, defectof the diaphragmatic pericardium, and the intracardiacanomalies — was first described by cantrell et al., in 1958.the defect is said to be more common in males, and survivalis dependent on the cardiac malformations and on thedegree of completeness of the syndrome. we report threecases of cantrell’s pentalogy managed in our unit. two ofthe patients were females and one a male. all were seen atperipheral health centers before being referred to us. ageat presentation for the girls was 18 hours and 36 hours,respectively, the boy presented at the age of six weeks. all oftheir parents were unschooled manual workers. all patientspresented with a defect in the supraumbilical body wall, bifidsternum, and a visible cardiac impulse. we were unableto do echocardiography to rule out intracardiac anomaliesin the three patients. the thin membranous covering ofthe epigastrium in the female patients was managedconservatively. both female patients were dischargedagainst medical advice as requested by their parents, dueto financial constraints. the male patient was lost to followup after two clinic visits. a multidisciplinary approach to themanagement of this syndrome is recommended.