StayCurrentMD · Pentalogy of Cantrell
Article1 min read·Published Apr 2019Older

Pentalogy of Cantrell

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Article · Apr 2019 · 1 min read

In brief

In brief

Pentalogy of Cantrell comprises five congenital defects affecting the heart, pericardium, diaphragm, sternum, and abdominal wall. This review examines diagnostic approaches and surgical management strategies, emphasizing the importance of early identification and coordinated multidisciplinary care for optimal outcomes.

Written by the GCMD Library team from the article.

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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