Pentalogia de Cantrell: relato de caso

Introduction: Pentalogy of Cantrell is a rare disorder described by Cantrell in 1958 and characterized by heart anomalies, involving defects of the diaphragm, abdominal wall, supraumbilical region and pericardium. Methods: We report a case of the disease that presented with agenesis of the sternum and partial absence of costal cartilage, treated by a multidisciplinary team. Results: The patient underwent median sternotomy. An enlarged heart, compromising the pulmonary trunk and the right ventricle, and a left superior vena cava were identified. The right atrium was opened and an interventricular communication of around 10 mm in diameter was seen through the tricuspid valve. Following the repair of the heart defects, chondroplasty and placement of biological mesh (Bioway of Gore) were performed. Conclusion: A Successful surgical treatment for Cantrell Syndrome includes correction of the cardiac malformation, a good repair of thoracoabdominal wall.

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Pentalogia de Cantrell: relato de caso

Abstract: RESUMO: Os autores descrevem o tratamento cirúrgico de um caso de pentalogia de Cantrell, rara anomalia congênita, caracterizada por defeitos da parede abdominal, terço inferior do esterno, diafragma, pericárdio e coração.

Cited by 2 publications

References 1 publication

Être mère d’un enfant malade chronique : une approche en recherche biographique

Être mère d’un enfant malade chronique : une approche en recherche biographique

Pentalogy of Cantrell with Sternum Agenesis—A Case Report

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