Natural history of a complex congenital diaphragmatic hernia

Abstract

The main complication in the case of congenital diaphragmatic hernia (CDH), which results from the invasion of the abdominal viscera in the thoracic cavity, is represented by the insufficient development of the pulmonary parenchyma (pulmonary hypoplasia, with the increase of the vascular resistance, and finally pulmonary hypertension). Besides pulmonary hypertension, the fetus can also develop cardiac failure in various degrees. Usually, this occurs when the arterial canal closes. We present a completely prenatal diagnosed case of left diaphragmatic hernia of the stomach, liver, and intestinal loops associated with left pulmonary agenesis and stenosis of arterial duct. The important right shifting of the heart in the right hemithorax detected on second trimester ultrasound screening has complicated in the third trimester with pericarditis, pleurisy and heart failure. The magnetic resonance imaging (MRI) examination at 31 weeks of gestation confirmed the ultrasound findings and offered more detailed prognostic elements of the anomaly. Autopsy of the stillbirth sustained MRI diagnosis regarding the absence of the left lung parenchyma and stenosis of the arterial duct. The natural history of this complex malformation underlies the importance of the collaboration of multiple specialists in maternal-fetal medicine, fast fetal MRI, fetal therapy, prenatal genetics, and fetal pathology.