Surgical treatment of neonatal Cantrell pentalogy: a case report and literature review

BACKGROUND: Pentalogy of Cantrell (PC) is a congenital multiple malformation consisting of midline supraumbilical thoracoabdominal wall defects, anterior and pericardial diaphragm defects, lower sternum defects, ectopia cordis and various intracardiac anomalies, that present a distinctive challenge for care-providers and surgeons. CASE DESCRIPTION: Cases of PC in twin pregnancies is rare. We report a new born infant, weighing 2,400 g, the younger twin. After birth, he was transferred to the Children’s Hospital of Soochow University due to the prenatal echocardiography indicated that he has ventricular septal defect (VSD), atrial septal defect (ASD) and aortic arch hypoplasia. From the appearance, the lower end of sternum is suspiciously missing, the apical beating point is located at the lower edge of the median xiphoid process of sternum, and the upper abdominal muscle below the beating area is defective. He has the clinical and imaging features of complete type of PC. Three surgical operations were performed in the neonatal period, including end-to-side anastomosis of aortic arch under cardiopulmonary bypass (CPB), patch repair of VSD, repair of ASD, ligation of ductus arteriosus, correction of cardiac vascular malformation and repair of chest and abdominal wall defect, and the postoperative recoveries went smoothly. CONCLUSIONS: PC is a rare congenital dysplasia, and its condition is complex. Our case shows that surgery is an effective treatment method, and the prognosis is related to the complexity of malformations.