Open Journal of Thoracic Surgery

Volume 10, Issue 1 (March 2020)

ISSN Print: 2164-3059   ISSN Online: 2164-3067

Google-based Impact Factor: 0.26  Citations  

Pentalogy of Cantrell with Sternum Agenesis—A Case Report

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DOI: 10.4236/ojts.2020.101001    318 Downloads   659 Views  

ABSTRACT

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 multi-disciplinary 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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Amorim, E. , Filho, N. , Sarmento, P. , Lacerda, J. , Ferreira, W. , Mocelin, P. , Filho, A. , Castilho, V. , Silva, J. , Albuquerque, R. , Raposo, C. and Vale, M. (2020) Pentalogy of Cantrell with Sternum Agenesis—A Case Report. Open Journal of Thoracic Surgery, 10, 1-5. doi: 10.4236/ojts.2020.101001.

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