Pulmonary hypoplasia(PH) commonly occurs in association with
oligohydramnios and other congenital anomalies, especially
congenital diaphragmatic hernia (CDH). Pulmonary hypoplasia is an important factor, as persistent
pulmonary hypertension, in the prognosis of CDH. In some reports, there is a decrement of
pulmonary surfactant in PH accompanying CDH. Recently, there are some reports that exogenous
pulmonary surfactant therapy is effective in experimental animal model and neonatal respiratory distress with PH. We report a case of a 5 day-old male neonate, who had shown
dyspnea and diagnosed as left pulmonary hypoplasia accompanying CDH. The CDH was surgically treated and the ipsilateral PH, with intratracheal administration of exogenous
pulmonary surfactant postoperatively. After exogenous
pulmonary surfactant application, the left lung volume was increased on chest roentgenogram and lung perfusion scan findings, and there was an improvement in oxygenation and clinical manifestations. We suggest that postoperative exogenous
pulmonary surfactant replacement
therapy is effective in the case of PH and further trials are needed to clarify the optimal dose and timing of supplementation of
surfactant for treatment of infants with PH accompanying CDH.