Cantrell’s pentalogy: long-term survival and repair of a large abdominal wall defect

Abstract

Cantrell's pentalogy is a congenital defect, described more than 50 years ago by Cantrell, Haller and Ravitch. Long term survival of patients with Cantrell's pentalogy is extremely rare. We report a case of pentalogy of Cantrell class 1, that showed sternal cleft, left anterolateral diaphragmatic hernia, thoraco-abdominal ectopia cordis, diaphragmatic pericardial defect, patent ductus arteriosus and a large omphalocele. Initial surgery was done in his first day of life, obtaining partial correction of the defect and allowing survival with good quality of life. At the age of 18 years, he showed an abdominal wall defect measuring 22x24 cm, with near-total eventration of the liver and small bowel. Surgical correction consisted on visceral partial reduction and abdominal wall closing with a microporous polytetrafluoroethylene mesh and dermal flaps. Judicious combination of reconstructive techniques obtained long term survival free of symptoms and excellent aesthetic results.

Pentalogy of Cantrell

“…There was no prenatal diagnosis, and this is common in developing countries with resource constraints. [ 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.…”

“…[ 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 ]…”

“…Prenatally, termination of pregnancy could be an option. [ 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.…”

“…Staged repair with the use of polytetrafluoroethylene has also been used. [ 4 ] Mesh augmentation is a viable option where there are insufficient tissues for cover and to offer more strength to the anterior abdominal wall like in case 1. This has been employed in previous reports.…”

“…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.…”