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CC BY 4.0 · Rev Bras Ginecol Obstet 2019; 41(05): 352-356
DOI: 10.1055/s-0039-1679878
DOI: 10.1055/s-0039-1679878
Case Report
Ectopia Cordis Associated with Pentalogy of Cantrell—A Case Report
Ectopia cordis associada à pentalogia de Cantrell—relato de casoFurther Information
Publication History
10 August 2018
17 January 2019
Publication Date:
25 March 2019 (online)
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.
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