Abstract

BUENROSTRO-ZARAGOZA, Gloria et al. Pentalogy of Cantrell: A case report. Bol. Med. Hosp. Infant. Mex. [online]. 2008, vol.65, n.5, pp.376-380. ISSN 1665-1146.

Introduction. The pentalogy of Cantrell is a infrequent congenital syndrome. There are 90 cases reported in the literature, described by Cantrell-Heller-Ravitch and characterized by hernia of the anterior diaphragm, omphalocele, diaphragmatic pericardium, congenital heart defect and in the lower sternum. Case report. Female newborn delivered at term, product of the 4th pregnancy from a 36 year-old mother with history of smoking and alcoholism, asthma treated with salbutamol during pregnancy, urogenital tract infection treated, preeclampsia in the 4th month of gestation non-treated. Diagnosis by ultrasound at 35.2 weeks of pregnancy of omphalocele and ectopia cordis; abdominal delivery of the newborn with the defects described above, associated with intracardiac lesions: pulmonary atresia, vascular transposition, ventricular septal defect and atrial septal defect. The infant died on the 5th day. Conclusion. The survival rate in cases reported in literature is rare and depends on the complexity of the cardiac defect.

Keywords : Hereditary diseases; Pentalogy of Cantrell; abnormalities; hernia umbilical; omphalocele; ectopia cordis; midline wall defects.