Author(s)

Elias Amorim¹, Nilo Antunes Sousa Filho¹, Petrucio Abrantes Sarmento², Joseval Silva Lacerda³, Wildel Campos Ferreira³, Paulo Roberto Mocelin⁴, Antonio Vieira Dias Filho⁴, Victor Hugo Dorigo Castilho⁵, Joel Fernando Sodré Bayma Silva⁶, Rejane Albuquerque⁷, Camila Raposo⁷, Marineia Vale⁷

¹Thoracic Surgery Service of the Teaching Hospital of the Federal University of Maranhão, Maranhão, Brazil.
²Thoracic Surgery Service of the Teaching Hospital of the Federal University of Paraiba, Paraiba, Brazil.
³Cardiac Surgery Service of the Teaching Hospital of the Federal University of Maranhão, Maranhão, Brazil.
⁴Plastic Surgery Service of the Teaching Hospital of the Federal University of Maranhão, Maranhão, Brazil.
⁵Anesthesiology Service of the Teaching Hospital of the Federal University of Maranhão, Maranhão, Brazil.
⁶Medical Student-Researcher of the Teaching Hospital of the Federal University of Maranhão, Maranhão, Brazil.
⁷Intensive Therapy Service of the Teaching Hospital of the Federal University of Maranhão, Maranhão, Brazil.

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.

Pentalogy of Cantrell, Agenesis, Diaphragm, Pericardium

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.