Background: Pentalogy of Cantrell (PC) is a rare anomaly, and the incidence varies from 5.5 to 7.9 per million live births. The components are defined as a defect of the lower sternum, a midline supraumbilical thoracoabdominal wall defect, a deficiency of the diaphragmatic pericardium, a deficiency of the anterior diaphragm, and congenital cardiac anomalies. The pathognomonic characteristic of this syndrome is the coexistence of omphalocele and ectopia cordis. Diagnosis is possible by antenatal or physical examination, and other tests should be performed to detect other anomalies, including the heart's anomaly. Surgical treatment is complex, and various operative techniques often require a staged approach. The mortality rate is quite high, with a survival rate of less than 5%.
Case presentation: Two-day-old male infant was complained that his heart had been outside his chest wall since birth without covering by skin or sternum. He was born on aterm through section cesarean with a vigorous baby condition. Section cesarean was chosen because his mother has Human Immunodeficiency Virus (HIV)—no family history with similar symptoms. While pregnant, the mother regularly checks with the midwife and fetal ultrasonography only once during the third trimester but did not find the defect, no history of drug abuse, teratogen exposure, smoking, alcohol, or consanguinity. The midline defect extended from the lower part of the sternum completely. Echocardiography found defects such as classic Tetralogy of Fallot, mild atrial regurgitation, mild mitral regurgitation, mild tricuspid regurgitation, and suspect mass at left atrium (LA). The patients underwent surgery by covering the heart with a silo bag. Three days after the procedure, the patient's condition worsened and death because of the sepsis.
Conclusion: Prenatal diagnosis is important in guiding the surgical team to estimate the prognosis and reduce a postnatal management strategy. Early management can provide the basic care necessary to keep the patients until definitive management can be provided.