Perinatology.  2022 Jun;33(2):73-81. 10.14734/PN.2022.33.2.73.

Multidisciplinary Management of Congenital Diaphragmatic Hernia: from Womb to Home

• Lee BS

• KMID: 2531061
• DOI: http://doi.org/10.14734/PN.2022.33.2.73

Abstract

Despite recent advances in prenatal diagnosis and newborn treatment strategies, the survival rate of congenital diaphragmatic hernia (CDH) has not changed much in the range of 60% to 80% over the past 20 years. Obstetricians, pediatricians, and pediatric surgeons play a pivotal role in prognosis pre diction, perioperative stabilization, and diaphragmatic repair, respectively. The two cardinal underlying pathologies are lung hypoplasia and persistent pulmonary hypertension, which sometimes lead to early mortality and long-term cardiopulmonary sequelae even after timely treatment in the newborn period. The observed/expected lung-to-head ratio in antenatal ultrasound is a strong predictor of mortality, and provides guidance in family counseling on critical decisions such as in utero transfer and extracorporeal membrane oxygenation (ECMO). In the CDH, the efficacy of pulmonary vasodi lators including inhaled nitric oxide is unclear, and hemodynamic support should be individually tailored based on invasive monitoring and echocardiography as well. ECMO plays a definite role as a last resort to rescue CDH. However, the implementation of ECMO can be a miraculous life-saving bridge to a surgical correction, but rather can be a meaningless life-sustaining machine. Controversy continues over the timing and technique of surgical repair to reduce mortality and postoperative recurrence. Although late death of CDH is rare after hospital discharge, a significant proportion of patients experience a wide range of long-term burden of associated morbidities including feeding difficulties, pulmonary dysfunction, reduced exercise capacity, and neurodevelopmental impairment. Recent clinical trials on fetal tracheal occlusion therapy are promising in that it turns out to improve the survival rate of severe CDH.

Keyword

Congenital diaphragmatic hernia; Pulmonary hypertension; Extracorporeal membrane oxygenation; Fetal therapies