title: Successful surgical repair of pentalogy of cantrell at 14 months of age.
abstract: Pentalogy of Cantrell with ectopia cordis is a rare congenital anomaly, first described in 1958 by Cantrell, has a reported incidence of around 5-10 cases per one million live births with wide variety of clinical presentations. We are reporting a child with ectopia cordis along with cleft lower sternum, upper abdominal wall defect, ectopic umbilicus and diaphragmatic defect. Echocardiography in first month of life revealed a restrictive perimembranous ventricular septal defect and a small patent Foramen Ovale, both closed spontaneously in infancy. CT angiography at 10 months of age revealed a defect in the thoracic and abdominal walls along with herniation of left ventricular apex into epigastrium. The two ventriculi formed a tail that looked like a crocodile. This patient underwent surgical correction at our institution at 14 months of age and recovered well with no residual issue.
authors.affiliation: Department of Paediatric Cardiology, Armed Forces Institute of Cardiology (AFIC) and National Institute of Heart Diseases (NIHD), Rawalpindi.
authors.forename: Khurram
authors.initials: K
authors.lastname: Akhtar
authors.affiliation: Department of Paediatric Cardiology, Armed Forces Institute of Cardiology (AFIC) and National Institute of Heart Diseases (NIHD), Rawalpindi.
authors.forename: Mehboob
authors.initials: M
authors.lastname: Sultan
authors.affiliation: Department of Paediatric Cardiology, Armed Forces Institute of Cardiology (AFIC) and National Institute of Heart Diseases (NIHD), Rawalpindi.
authors.forename: Waqar
authors.initials: W
authors.lastname: Ahmed
authors.affiliation: Department of Paediatric Cardiology, Armed Forces Institute of Cardiology (AFIC) and National Institute of Heart Diseases (NIHD), Rawalpindi.
authors.forename: Maad
authors.initials: M
authors.lastname: Ullah
authors.affiliation: Department of Paediatric Cardiology, Armed Forces Institute of Cardiology (AFIC) and National Institute of Heart Diseases (NIHD), Rawalpindi.
authors.forename: Nadeem
authors.initials: N
authors.lastname: Sadiq
mesh_terms: D006348:Cardiac Surgical Procedures; D004452:Echocardiography; D054083:Ectopia Cordis / Q000175:diagnosis / Q000601:surgery*; D006330:Heart Defects, Congenital / Q000601:surgery; D006345:Heart Septal Defects, Ventricular / Q000000981:diagnostic imaging / Q000601:surgery*; D006554:Hernia, Umbilical / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D058502:Pentalogy of Cantrell / Q000175:diagnosis / Q000601:surgery*; D013249:Sternum / Q000002:abnormalities / Q000601:surgery*; D016896:Treatment Outcome