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Pentalogy of cantrell in Ile-Ife: A case report and review of the literature
O Ugowe1, O Afeniforo1, B Adeyefa2, JA Okeniyi3, EA Adejuyigbe4
1 Department of Pediatrics, Neonatal Unit, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Nigeria
2 Department of Pediatrics, Pediatric Cardiology Unit, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Nigeria
3 Department of Pediatrics, Pediatric Cardiology Unit, Obafemi Awolowo University Teaching Hospitals Complex; Department of Pediatrics and Child Health, Obafemi Awolowo University, Ile-Ife, Nigeria
4 Department of Pediatrics, Neonatal Unit, Obafemi Awolowo University Teaching Hospitals Complex; Department of Pediatrics and Child Health, Obafemi Awolowo University, Ile-Ife, Nigeria
Correspondence Address:
Dr. O Ugowe
Department of Pediatrics, Neonatal Unit, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife
Nigeria
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/njhs.njhs_45_20
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Pentalogy of Cantrell is a rare congenital malformation. The aetiology is not well understood, but genetic and sporadic forms have been reported. It is a combination of five congenital defects: the heart, pericardium, diaphragm, sternum and anterior abdominal wall. Only a few cases of pentalogy of Cantrell have been reported in Nigeria. Prenatal diagnosis is possible at the beginning of the second trimester using ultrasonography. Success in the management of this condition requires expertise and a multidisciplinary approach. We report the case of a 7-day-old boy, who was referred to our hospital in Ile-Ife with a chest wall defect at birth and ectopia cordis, diaphragmatic hernia seen on ultrasound and multiple heart defects seen on echocardiography. The diagnosis of pentalogy of Cantrell was made. The severity of the cardiac defects in this case and the late referral contributed to the poor outcome of the reported case. Other difficulties in the management of this case peculiar to our environment are highlighted.
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