Pentalogy of Cantrell: Case Report With Review of the Literature.

Journal Article (Review;Journal Article)

Background

Pentalogy of Cantrell is a rare, congenital disorder characterized by lower sternal defects, diaphragmatic defect, pericardial defect, supraumbilical abdominal wall abnormalities, and/or intracardiac defects. The collective defects result from failure of either differentiation or migration of mesenchymal or mesodermal structures during the embryonic phase of development. Mortality of the disease complex is high, and treatment, when appropriate, revolves around surgical correction of the associated defects.

Purpose

This article presents a case of pentalogy of Cantrell and examines the literature to report the most current evidence relative to the embryology and pathophysiology. In addition, the methods of pre- and postnatal diagnosis, management, and prognostic indicators are examined.

Methods/search strategy

Case report was gathered from the medical records and is provided as it occurred. The literature was searched for evidence of best management strategies as well as care implications for families.

Findings/results

A female newborn was delivered at (Equation is included in full-text article.)weeks' gestation secondary to premature onset of labor. Prenatal ultrasonography identified an abdominal wall defect, diaphragmatic hernia, sternal defect, ventricular septal defect, and open neural tube defect. Examination immediately after delivery confirmed prenatal findings and a diagnosis of pentalogy of Cantrell was assigned.

Implications for practice

Patients with the diagnosis of pentalogy of Cantrell should receive antenatal counseling relative to mortality and morbidity risks. An interprofessional approach in the immediate timeframe after delivery facilitates timely diagnostics and offers families prompt confirmation of antenatal findings.

Implications for research

Future research can focus on further elucidating genetic etiologies of pentalogy of Cantrell.

Full Text

Duke Authors

Cited Authors

  • Jnah, AJ; Newberry, DM; England, A

Published Date

  • August 2015

Published In

Volume / Issue

  • 15 / 4

Start / End Page

  • 261 - 268

PubMed ID

  • 26225594

Electronic International Standard Serial Number (EISSN)

  • 1536-0911

International Standard Serial Number (ISSN)

  • 1536-0903

Digital Object Identifier (DOI)

  • 10.1097/anc.0000000000000209

Language

  • eng