Ectopia cordis: A case report of pre-surgical care in resource-limited setting

Int J Surg Case Rep. 2021 Jun:83:105965. doi: 10.1016/j.ijscr.2021.105965. Epub 2021 May 12.

Abstract

Introduction and importance: Ectopia cordis is a rare congenital malformation of thoracic midline fusion that presents as location of the heart outside the open chest cavity. This presents as a surgical emergency and demands early and specialized intervention. Particularly in resource-limited settings, where prenatal ultrasonography screening is not done, these children are often born in facilities without the capability of managing such conditions definitively, necessitating them to be referred to a specialized centre. At lower health facilities, the challenge is in ensuring that the child is kept stable and protected from infection until they can reach a centre with the facilities required for care. This report describes the management give to such a child until they were successfully handed over to a cardiac institute.

Case presentation: We present a newborn male baby delivered at term to a mother from a low socio-economic background with his heart and abdominal viscera outside the thoracic and abdominal cavity. Despite presenting at a centre without cardiac surgery facilities or cardiologists, they were sustained until referral.

Clinical discussion: Ectopia cordis is a rare congenital anomaly characterized by defect in the fusion of the anterior chest wall resulting in the abnormal extra-thoracic location of the heart. Five types exist; cervical type with worst prognosis, attempts can be made to re-locate the heart and close the thoracic defect surgically.

Conclusion: Even with limited resources, it is possible to provide the basic care necessary to sustain a child with this complex anomaly until definitive management can be provided.

Keywords: Cantrell's pentalogy; Case report; Ectopia cordis; Tanzania.