RELATO DE CASO: PENTALOGIA DE CANTRELL

Abstract

Cantrell's Pentalogy is a rare and complex congenital syndrome, first described in 1958, characterized by a combination of structural malformations: absence of the lower third of the sternum, cardiac ectopia, congenital heart disease, omphalocele, and diaphragm defects. This condition results from an interruption in embryonic development and presents significant challenges for diagnosis and management. This case report aims to analyze a specific presentation of Cantrell's Pentalogy, addressing clinical manifestation, diagnostic challenges, and adopted treatment strategies. We report the case of a female newborn, born at 38 weeks and 4 days of gestation, with a birth weight of 2,970 grams and Apgar scores of 9 at 1 minute and 10 at 5 minutes. The patient presented with extensive failure in the abdominal wall musculature, associated with an omphalocele and cardiac anomalies, including interventricular septal defect and patent ductus arteriosus. The evaluation was complemented by imaging studies, including X-ray, echocardiography, ultrasonography, and tomography. The initial treatment was predominantly clinical. The omphalocele was managed with hydrocolloid dressings, while cardiac malformations were handled conservatively, in coordination with the cardiac surgery team. The patient responded well to the treatment, showing favorable clinical evolution and well-tolerated initiation of oral feeding. Elective surgical correction of the omphalocele is planned to occur between 6 and 9 months of age, after full stabilization of the patient's clinical conditions. This report highlights the importance of early diagnosis and a multidisciplinary approach in managing Cantrell's Pentalogy.

Keywords: Cantrell's Pentalogy, omphalocele, cardiac anomalies, congenital syndrome, multidisciplinary approach.