A local case of pentalogy of cantrell in 2013 (clinical case study)

Qualified as a very rare disease, the Pentalogy of Cantrell comprises a number of defects, such as: anterior abdominal wall defect, lower sternum defect, diaphragmatic and pericardial defects, and congenital heart malformations. The typical form of the pentalogy includes all these five defects. The cases of the complete pentalogy described in the existing literature are only a few. The first description of the pentalogy was made by Cantrell et al. in 1958, who gave an account of 5 cases presented with this abnormality. Cases with only two, three or four of the defects are more frequent. The case we present here involves the 16-year-old A.M.A., pregnant in the 18th gestation week. The fetus is male with bilateral cleavage of lip and palate; low-set ears, congenitally deformed feet (pes equinovarus), absent little toe of the right foot, complete absence of sternum and whole abdominal wall defect. The heart, liver and intestinal loops, as well as the kidneys and urinary bladder are ectopically located outside their corresponding cavities. A lack of pericardium is noted, together with ventricular septal defect, and atrial septal defect. My colleagues and I believe this case is typical for Pentalogy of Cantrell.