Ref Number = PITIKA-ASPR0250
Ni Nyoman Anik Cindi Yuliastini, I Made Kardana, I Wayan Darma Artana, Putu Junara Putra, Made Sukmawati  
Objective: Pentalogy of Cantrell (PC) is a rare and multifactorial anomaly mark by the coexistence of omphalocele and ectopia cordis. PC comprises of the following five characteristics: midline, upper abdominal wall disorder; defect of lower sternum; anterior diaphragmatic defect; pericardial abnormality; congenital abnormalities of the heart. This syndrome Classified into class I occurrence of all defects, class II occurrence of 4 defects with intracardiac and ventral abdominal wall abnormalities definitely present, and class III incomplete expression of the disorders showing various combinations of defects, although sternal anomalies are definitely present. The incidence is estimated at 5.5 – 7.9 per 1 million live births. Unfortunately, the mortality rate is quite high with minimum survival rate even with surgical reconstruction.
Case: A one-day old baby was referred from Lombok, because suspected of PC with neonatal pneumonia. The baby born through section cesarean with mild asphyxia, birth weight was 3000 gram and body length was 48 cm. There is no family and pregnancy risk factor found. The multidisciplinary team consists of neonatologist, pediatric cardiologist, and the pediatric surgeon was teamed up to handle this case. There were slightly abnormalities in complete blood counts. Thorax and abdominal X-ray showed a suspicion of pneumonia but this is not supported with our physical examination result. Echocardiography was done by pediatric cardiologist and found that some intracardiac defect such as Taussig Bing anomaly, single atrium, mild valvular pulmonary stenosis, mild tricuspid regurgitation, and mesocardia. Genetic testing and further radiologic examination other than plain X-ray are not done in this case. There is no further intervention for this patient other than regular echocardiography and antibiotic topical treatment. 
Conclusion: Knowing pentalogy of Cantrell since antenatal is an important thing to discuss the management after birth even though the mortality rate is still high after surgical reconstruction.
Keywords: Pentalogy of Cantrell, genetic testing
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