Pentalogy of Cantrell

Abstract

Publication date: Available online 9 April 2019

Source: Seminars in Pediatric Surgery

Author(s): Adele P. Williams, Raoud Marayati, Elizabeth A. Beierle

Abstract

Pentalogy of Cantrell is a constellation of five congenital defects that pose a unique challenge for surgeons. Defects of the heart, pericardium, diaphragm, sternum, and anterior abdominal wall are pathognomonic. Although the incidence of this defect is small, it is critical to identify it in a timely fashion in order to adequately address all aspects. Early diagnosis, supportive care, and strategic surgical planning with a multidisciplinary team are all key components in managing patients with Pentalogy of Cantrell. In this text we sought to explore the evolution of both the understanding and treatment for this complex entity and provide current recommendations to today's pediatric caregivers.

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