Pentalogy of Cantrell with Sternum Agenesis—A Case Report

• 作者: Antonio Vieira Dias Filho Camila Raposo Elias Amorim Joel Fernando Sodré Bayma Silva Joseval Silva Lacerda Marineia Vale Nilo Antunes Sousa Filho Paulo Roberto Mocelin Petrucio Abrantes Sarmento Rejane Albuquerque Victor Hugo Dorigo Castilho Wildel Campos Ferreira
• 来源期刊: 胸外科期刊(英文)
• 学科: 医学
• 关键词: Pentalogy of Cantrell AGENESIS DIAPHRAGM PERICARDIUM
• 年，卷（期）: 2020,（1）
• 页码范围: 1-5
• 页数: 5页
• 分类号: R54

摘要

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.