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

Abstract

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.

Keywords: Cantrell; case report; neonate twin; pentalogy; surgical treatment.

Figure 1

Appearance and surgical anatomy. (A) Chest and abdomen appearance and cardiac apex position. (B) The anatomy showed severe dysplasia of the aortic arch. (C) Delayed chest closure after the first operation. (D) Chest and abdomen incision after three operations. This image is published with the patient’s parents’ consent. AO, aorta; PA, pulmonary artery; CoA, coarctation of aorta; RV, right ventricle.

Video 1

The apex beating point is located at the lower edge of the sternum median xiphoid process, with intact skin and partial defect of upper abdominal muscle.

Figure 2

The results of Echocardiography on admission. (A) Great artery short axis view showed VSD. (B) Four-chamber heart section view showed ASD. (C) The transverse aortic arch was hypoplastic with coarctation. (D) Color Doppler indicates that the blood flow velocity in aortic arch, especially in aortic isthmus, was accelerated. VSD, ventricular septal defect; ASD, atrial septal defect; CoA, coarctation of aorta.

Figure 3

The CT angiography before surgery. (A) Enhanced cardiac CT and three-dimensional reconstruction of sternum and ribs showed incomplete sternum ossification, and the lower sternum is missing. (B) Enhanced cardiac CT cross section showed that the lower sternum and muscular layer of abdominal wall were defective. The heart protruded forward. (C) Enhanced cardiac CT oblique sagittal position showed that the apex moves down to the abdomen. (D) Aortic arch hypoplasia and aortic coarctation were shown. CT, computed tomography.

Figure 4

The CT angiography after surgery. (A) Enhanced cardiac CT and three-dimensional reconstruction showed the frontal view of the heart one year after surgery. (B) The posterior view of the heart is showed. (C) The sagittal view shows good morphology of the aortic arch. CT, computed tomography.