Cantrell's syndrome.

Cantrell ́s syndrome is characterized by: 1) Omphalocele 2) Sternal cleft 3) Anterior diaphragmatic hernia 4) Ectopia cordis 5) Intracardiac defects With an incidence of 5 to 8 cases per million of newborn babies, this syndrome is caused by mesodermal defects in early stages of the embrionary period. There are two ways of presentation: complete and incomplete. The incomplete way is when the heart is covered by skin, pericardium or both of them. In the complete way, the sternum is absent or it has a wide defect. There is no parietal pericardium and the heart is totally out of the thorax with its apex upwards and there is an important reduction in the size of the thoracic cavity. Generally, with omphalocele. 80% of the cases with incomplete way present congenital heart defect and in the complete way this percentage is 100%. Truncoconal abnormalities are the most usual. The studied case had interventricular communication (IVC) and the emergence of the great vessels was difficult to observe.