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CC BY 4.0 · Rev Bras Ginecol Obstet 2019; 41(05): 352-356
DOI: 10.1055/s-0039-1679878
Case Report
Thieme Revinter Publicações Ltda Rio de Janeiro, Brazil

Ectopia Cordis Associated with Pentalogy of Cantrell—A Case Report

Ectopia cordis associada à pentalogia de Cantrell—relato de caso
José Mauro Madi 
1   Department of Obstetrics and Gynecology, Faculty of Medicine, Universidade de Caxias do Sul, Caxias do Sul, RS, Brazil
,
José Roberto Festugatto
2   Department of Radiology, Hospital Geral de Caxias do Sul, Universidade de Caxias do Sul, Caxias do Sul, RS, Brazil
,
Matheus Rizzon
2   Department of Radiology, Hospital Geral de Caxias do Sul, Universidade de Caxias do Sul, Caxias do Sul, RS, Brazil
,
Ana Paula Agostini
3   Department of Pediatrics and Neonatology, Faculty of Medicine, Universidade de Caxias do Sul, Caxias do Sul, RS, Brazil
,
Breno Fauth de Araújo
3   Department of Pediatrics and Neonatology, Faculty of Medicine, Universidade de Caxias do Sul, Caxias do Sul, RS, Brazil
,
Rosa Maria Rahmi Garcia
4   Department of Endocrinology, Faculty of Medicine, Universidade de Caxias do Sul, Caxias do Sul, RS, Brazil
› Author Affiliations
Further Information

Publication History

10 August 2018

17 January 2019

Publication Date:
25 March 2019 (online)

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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 al[2] 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.

Resumo

A pentalogia de Cantrell (PC) é uma rara anomalia congênita caracterizada por alterações nas estruturas medianas mesodérmicas e doenças cardíacas congênitas, cursando muitas vezes com um mau prognóstico. Em 1958, Cantrell et al[2] definiram o espectro completo da síndrome com as seguintes anomalias: defeitos do diafragma anterior, da parte inferior do esterno, da região supraumbilical e parede abdominal, do pericárdio diafragmático, e várias anormalidades congênitas intracardíacas. O presente relato relaciona-se a um caso de ectopia cordis associado à PC e à importância da participação de uma equipe multidisciplinar no acompanhamento da doença.

Keywords

ectopia cordis - pentalogy of Cantrell - abdominal wall defects - congenital heart defects - umbilical hernia

Palavras-chave

ectopia cordis - pentalogia de Cantrell - defeitos da parede abdominal - defeitos congênitos cardíacos - hérnia umbilical
 

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