Tracheal ligation: the dark side of in utero congenital diaphragmatic hernia treatment

Abstract

Currently there are two in utero procedures that have been proposed for the treatment of Congenital diaphragmatic hernia (CDH); reduction of the herniated viscera with repair of the diaphragmatic defect (CDH repair) and stimulation of lung growth by ligation of the fetal trachea (CDH + TL). Recent studies have shown that CDH + TL may result in a significant surfactant deficiency. The aim of this study was to compare the postnatal lung function of these two interventions using the fetal lamb model of CDH. CDH was created in 14 lambs at 78 days' gestation. At 110 days, seven lambs had their trachea ligated through a transverse neck incision and seven had repair of their diaphragmatic defect via a left subcostal incision. At term the lambs were instrumented with the umbilical circulation intact, then delivered and ventilated to a standard protocol for 4 hours. Pulmonary hemodynamics and blood gas levels were measured and compared every 30 minutes. Four lambs in the CDH repair group and five lambs in the CDH + TL group survived to be studied. After the initial data were analyzed, a further group of CDH + TL lambs (n = 4) were studied. In this group a replacement dose of surfactant (Infasurf, Ony Inc, Buffalo, NY) was administered. These initial results cast doubt on tracheal ligation as an in utero therapy for CDH, and indicate that the lung produced by this intervention is not physiologically normal as previously thought. However, the function of these lungs can be normalized if the surfactant deficiency is corrected. If this improvement can be maintained and there is recovery of the endogenous surfactant system, then in utero tracheal ligation may become a viable treatment for fetal CDH.