Ectopia Cordis Associated with Pentalogy of Cantrell—A Case Report

Abstract

Pentalogy of Cantrell (PC) is a rare congenital anomaly characterized by changes in the mesodermal median structures and congenital heart disease, often with a poor prognosis. In 1958, Cantrell et al2 defined the full spectrum of the syndrome with the following anomalies: defects of the anterior diaphragm, of the lower part of the sternum, of the supraumbilical region and the abdominal wall, of the diaphragmatic pericardium, and various intracardiac congenital abnormalities. The present report describes a case of ectopia cordis associated with PC and the importance of the participation of a multidisciplinary team in the treatment of this condition.

Pentalogy of cantrell: Reconstructive challenges of two complete cases in a resource-constrained setting

“…[ 4 ] Prenatal diagnosis informs the route of delivery and operative intervention in a specialist center. [ 5 6 ] Postnatal diagnosis of POC is clinical; however, the details of composite anomalies are aided with ultrasonography, echocardiography, computerized tomographic scanning, and MRI. [ 3 4 7 ]…”

“…[ 4 ] Postnatally, repairs could be single or multistaged, immediate, or delayed. [ 4 5 6 7 8 ] Case 1 had a repair at 1 year, whereas case 2 had an immediate repair on the 4 th day of life.…”

“…This has been employed in previous reports. [ 4 5 7 8 ] Arturo Leonardo et al . [ 8 ] augmented mesh repair and cutaneous flaps with resection of costal cartilages to be able to accommodate the heart within the thoracic cavity.…”

A propósito de un caso: Resolución quirúrgica en dos tiempos de Pentalogía de Cantrell

“…El diagnóstico temprano de esta patología, así como el seguimiento durante el embarazo, la planificación de una cesárea en un centro de alto nivel y la aproximación quirúrgica inmediata con un equipo multidisciplinario, son componentes clave en el manejo integral de pacientes con Pentalogía de Cantrell [7].…”

The Unusual Journey of a Pericardial Drainage Catheter in Pentalogy of Cantrell

“…PC has a very high mortality rate with prognosis depending on the severity of the congenital defects. The most distinctive defects in patients with PC are omphalocele, which has an independent estimated incidence of approximately 1 in 4000 births, and ectopia cordis, whose estimated incidence is 1 to 65 in 200,000 births [ 3 , 4 ]. The pericardium is absent in approximately 75% of PC with associated intracardiac defects [ 5 ].…”