Cantrell pentalogy: report of a severe, fatal case in Bouaké (Côte d'Ivoire)

Abstract

Introduction: Cantrell's pentalogy corresponds to a defect in umbilical ring closure involving the supra-umbilical portion of the abdominal wall, secondary to an anomaly in mesodermal development during the first stage of embryonic development. Observation: This was a full-term female neonate referred to the Neonatology Department of Bouaké University Hospital at nine (9) hours of age for congenital malformation. The pregnancy was poorly monitored, with no prenatal check-up. Birth parameters were: weight 2750g, height 48cm APGAR: 7-8 at 1 and 5 minutes, CP: 33cm. On admission, the patient was pink, eupneic and had a normal neurological examination. She presented with a chest wall defect revealing a beating mass reminiscent of the heart, with the apex pointing upwards towards the chin. There was no serous membrane on the outer surface of the heart. This thoracic malformation was accompanied by a defect in the abdominal wall, revealing a supraumbilical mass suggestive of an omphalocele. Cardiac ultrasound revealed a malformed heart with a single right ventricle and rudimentary left heart chambers. The parents were discharged against medical advice at 4 days of age. The newborn died on the 9th day at home. Conclusion: Pentalogy of Cantrell is an extremely rare congenital malformation whose prognosis depends on the severity of the associated cardiac anomaly, hence the importance of multidisciplinary management.