Evaluation of pulmonary hypoplasia with congenital diaphragmatic hernia

Congenital diaphragmatic hernia (CDH) is a developmental defect of the diaphragm with herniation of abdominal viscera into the thorax. The morbidity and mortality in cases is caused primarily by pulmonary hypoplasia. A prenatal diagnosis of CDH can be established by ultrasound, but ultrasound cannot detect pulmonary hypoplasia with certainty. We evaluated pulmonary hypoplasia by the simple method of lung intensity. The subjects were eight fetuses. In four fetuses, the lungs showed low intensity, and these fetuses all had pulmonary hypoplasia. Two of the four fetuses with high intensity showed a good prognosis, however, the other two fetuses did not. One of the two cases with poor prognosis had complex congenital heart disease, and the other died of sepsis and pulmonary hypertension. A high signal did not always promise a favorable prognosis, probably because accompanying abnormalities dominated the prognosis. MR assessment of lung intensity in fetuses with CDH may be useful in evaluating the severity of pulmonary hypoplasia. Low signal of the lung may reflect pulmonary hypoplasia and suggests a poor prognosis. (author)