2024/12/30 by Kouadio Richard A ., Alexise A ., Christelle A . +3
#Côte d’Ivoire #Pentalogy of Cantrell #cardiac ectopy #congenital malformation #ectopie cardiaque #malformation congénitale #pentalogie de Cantrell
paper · doi:10.71566/pist-rmp-194612
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 Hospi[1]tal 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. Toxoplasma serology came back positive, and syphilitic and rubel[1]la serologies were negative. Karyotype could not be performed. Cardiac ultrasound revealed a malformed hear a single right ventricle and rudimentary left heart chambers. A thoracic CT scan for sternal and dia[1]phragmatic malformations could not be performed. The parents were discharged against medical ad[1]vice 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.