Cantrell pentalogy. Case report

Abstract

Pentalogy of Cantrell is a rare disease, with peculiar clinical, anatomical and embryological characteristics. This entity is a thoracoabdominal variety of ectopia cordis in which five anomalies are associated: epigastric defect of the supraumbilical abdominal midline, defect of the lower third of the sternum, deficiency of the anterior segment of the diaphragm, pericardial defects and congenital heart malformations. Ventricular and atrial septal defect is found in half of the cases and can be associated with other extracardiac anomalies such as spina bifida, various malformations of the central nervous system, Meckel's diverticulum, polysplenia, among others. The diagnosis is made by fetal ultrasonography in the first trimester of pregnancy; In incomplete cases, helical tomography and magnetic resonance imaging complement the diagnosis. The treatment is surgical and in several stages, the main thing is to protect the exposed viscera and correct the congenital heart disease. The results are still controversial. The prognosis of the disease depends on the extent of the defect, early diagnosis and perioperative management. We consider that Pentalogy of Cantrell is a complex disease in which the multidisciplinary intervention of the health team is necessary to reach the precise diagnosis and decide the best treatment. Our objectives were: to know the characteristic radiological findings of the pathology; identify all the disorders present in patients who suffer from it and their association with the discovery of other malformations.