The Pentalogy of Cantrell is a rare congenital disorder characterized by a combination of five specific anatomical abnormalities. It affects the development of the chest, abdominal wall, and heart. The condition is named after the physician who first described it, Dr. Cantrell.
The five main features of the Pentalogy of Cantrell include:
- Midline defect of the lower sternum: This refers to a gap or split in the breastbone, which can vary in severity.
- Defect in the anterior diaphragm: The diaphragm, a muscle that separates the chest and abdominal cavities, may have a hole or weakness.
- Defect in the diaphragmatic pericardium: The pericardium, a sac that surrounds the heart, may be incomplete or absent.
- Malformation of the heart: Individuals with this condition often have congenital heart defects, such as ventricular septal defects or abnormalities in the heart's positioning.
- Abdominal wall defects: The abdominal muscles and tissues may be underdeveloped or have openings, leading to herniation of organs.
Due to the complexity of this condition, treatment typically involves a multidisciplinary approach involving pediatric surgeons, cardiologists, and other specialists. Surgical interventions are often required to repair the various defects and improve the affected individual's overall health and quality of life.