Traitement anténatal de la hernie de coupole diaphragmatique : le point en 2008

Abstract

Despite advances in neonatal care, the associated mortality in isolated congenital diaphragmatic hernia remains in the order of 30%. Death is mainly due to consecutive pulmonary hypoplasia and severe associated pulmonary hypertension. This statement led to the implement of fetal therapy in order to improve fetal lung development. The first phase of fetal surgery consisted in open repair with one-stage surgical correction of the anatomic defect. It was followed by tracheal occlusion technique based on the decrease egress of lung fluid in order to improve lung growth. Initial approach of tracheal occlusion used clips on the trachea. It is now performed with intra-tracheal inflatable balloon. The challenge of such prenatal treatment is to establish accurate prenatal prognosis factors in order to offer this therapeutic in the subgroup of patients in whom outcome of postnatal treatment remains dismal. Current factors used for prenatal prognosis evaluation are thoracic liver position and lung-to-head ratio (LHR). The balloon is inserted at 26 to 28 weeks and removed at 34 weeks. First results are encouraging. However, many patients developed premature prelabour rupture of the membrane and paediatric data are sparse. Only few patients were currently treated and long term evaluation is needed. A powered multicentric study is needed to determine the value of fetal tracheal occlusion in the management of fetuses with isolated severe congenital diaphragmatic hernia.