Diagnóstico antenatal de malformação adenomatoide cística congênita unilateral associada à síndrome de Taussig-Bing: relato de caso

Abstract

AIMS: To report a rare case of antenatal diagnosis of unilateral congenital cystic adenomatoid malformation associated with Taussig-Bing syndrome. CASE DESCRIPTION: Patient aged 40 years, four pregnancies with three previous vaginal deliveries, black skin, married, housewife, without prior conditions, was referred to the Fetal Medicine Unit of the University Hospital of Santa Maria due to changes in the thoracic region of the fetus found in routine obstetric ultrasound examination performed at 30 weeks and three days of gestation. At the Doppler ultrasound assessment performed in the reference center, perimembranous ventricular septal defect and transposition of the great vessels were found, characterizing the Taussig-Bing syndrome. Additionally, a unilateral pulmonary congenital cystic adenomatoid malformation type 2 was found. The medical staff decided by a cesarean delivery in a referral center for cardiology, with 37 weeks of gestation. The neonate, weighing 3 kg, presented since the first hours acute respiratory distress with severe cyanosis, and underwent lobectomy. The neonate died within two weeks of life due to respiratory failure during the performance of a surgical procedure to correct the cardiac malformation. CONCLUSIONS: There is no description in the literature of association between Taussig-Bing syndrome and congenital cystic adenomatoid malformation. Early ultrasound diagnosis and monitoring in a Fetal Medicine unit is important for planning pre and post-natal interventions and family counseling by a multiprofessional team.