Prenatal diagnosis of congenital diaphragmatic hernia and pulmonary hypoplasia and therapeutic strategy

Abstract

The outcome of fetuses with congenital diaphragmatic hernia (CDH) has been reported to be fatal when pulmonary hypoplasia (PH) is severe. As an indicator of PH, we attempted to measure the lung-thorax transverse area ratio (L/T) using ultrasonic echography. Immediate postnatal surgery was performed using high-frequency oscillatory ventilation (HFOV) and sometimes followed by extracorporeal membrane oxygenation (ECMO). Eighteen fetuses were treated and 14 survived. L/T correlated well with the best preductal arterial blood gas data before surgical reduction during manual ventilation and HFOV, while preductal PO2 and alveolar-arterial oxygen differences from patients managed with HFOV were better than those in patients with manual ventilation. Although L/T also correlated with the duration of O2 therapy and hospitalization in survivors without major anomalies, there was no significant difference between L/T in survivors and non-survivors. Because delayed institution of ECMO and complications related to ECMO management seemed to be a major cause of death in non-survivors, the unsalvageable L/T due to PH was estimated to be below 0.06 for HFOV and below 0.1 for conventional ventilation based on the correlation between L/T and preductal PO2. These results suggest that L/T is a useful indicator of PH in patients with CDH and also that HFOV is advantageous in treating CDH with PH. The advantage of prenatal diagnosis in predicting unsalvageable L/Ts, should be considered in the therapeutic strategy.