TITLE:
Pentalogy of Cantrell with Sternum Agenesis—A Case Report
AUTHORS:
Elias Amorim, Nilo Antunes Sousa Filho, Petrucio Abrantes Sarmento, Joseval Silva Lacerda, Wildel Campos Ferreira, Paulo Roberto Mocelin, Antonio Vieira Dias Filho, Victor Hugo Dorigo Castilho, Joel Fernando Sodré Bayma Silva, Rejane Albuquerque, Camila Raposo, Marineia Vale
KEYWORDS:
Pentalogy of Cantrell, Agenesis, Diaphragm, Pericardium
JOURNAL NAME:
Open Journal of Thoracic Surgery,
Vol.10 No.1,
January
20,
2020
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.