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

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 settin...

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Detalles Bibliográficos
Autores principales: Lodhia, Jay, Chipongo, Hillary, Chilonga, Kondo, Mchaile, Deborah, Mbwasi, Ronald, Philemon, Rune
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8141755/
https://www.ncbi.nlm.nih.gov/pubmed/34000488
http://dx.doi.org/10.1016/j.ijscr.2021.105965
Descripción
Sumario: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.