Current Concepts in the Management of Congenital Diaphragmatic Hernia in Infants

Abstract

The therapeutic approach to congenital diaphragmatic hernia (CDH) has shifted from one of immediate repair to management of pulmonary hypertension, physiologic stabilization, and delayed surgical repair. Lung hypoplasia, remodeled pulmonary vasculature, and ventricular dysfunction all contribute to the high morbidity and mortality associated with CDH. In addition, genetic syndromes associated with CDH can increase the incidence of serious anomalies and hence impact survival. Prenatal and postnatal management of infants with CDH is challenging in the best of circumstances and need multidisciplinary teams for optimal outcomes. However, advances using ultrasound and fetal MRI can predict prognosis and survival and plan for postnatal management. Survival rates for patients with CDH have increased for the past decade with better management at resuscitation; implementation of gentle ventilation strategies; and medical management of pulmonary hypertension, physiologic stabilization, and extracorporeal membrane oxygenation. However, follow-up of these infants for long-term morbidities is essential for optimal outcomes after discharge. . Lung hypoplasia and pulmonary hypertension are key factors which contribute to the morbidity and mortality associated with CDH.

Hepatopulmonary fusion associated with right-sided congenital diaphragmatic hernia: management of this rare anomaly and a review of the literature

“…Some authors have defined these criteria as stable mean blood pressure for gestational age, preductal oxygen saturation in the range of 85%–95% on ≤50% oxygen, urine output >2 cc/kg/h, and serum lactate levels <3 mmol/l. 24 Pulmonary artery pressure also determines stability. Some surgeons operate only if normal measures are maintained for at least 24 to 48 h based on echocardiography because they believe that pulmonary hypertension is a major risk of mortality.…”

A clinical study on congenital diaphragmatic hernia in neonates: Our institutional experience

“…Lung hypoplasia is the major determinant of survival, and the degree of pulmonary hypoplasia correlates with the severity of PPHN. [ 9 ]…”

“…The presence of liver herniation into the chest (liver up) is considered to be a poor prognostic indicator. [ 8 9 10 15 ] Some studies have reported higher mortality among the right-sided CDH[ 12 13 14 15 16 17 ] whereas others did not find a statistical difference in mortality rate when compared to left-sided defects. [ 15 18 ]…”

The Outcome of Operated Neonates with an Isolated Congenital Diaphragmatic Hernia in a Limited Resource Scenario

“…Deep sedation and neuromuscular blockade were provided selectively in the event of higher ventilation or oxygen requirements. [ 6 7 ] The aim was to achieve preductal SaO 2 of 80%–95%, postductal SaO 2 > 70%, arterial pCO 2 between 45 and 60 mm Hg, and a pH of 7.25–7.40. [ 7 8 9 10 ] Oxygen supplementation was titrated to achieve a preductal saturation of 85%–95%.…”

“…[ 6 7 ] The aim was to achieve preductal SaO 2 of 80%–95%, postductal SaO 2 > 70%, arterial pCO 2 between 45 and 60 mm Hg, and a pH of 7.25–7.40. [ 7 8 9 10 ] Oxygen supplementation was titrated to achieve a preductal saturation of 85%–95%. The preferred ventilation strategy was pressure-cycled ventilator with initial continuous mandatory ventilation, followed by intermittent mandatory ventilation, peak inspiratory pressure (PIP) <25 cm H 2 O, positive end-expiratory pressure (PEEP) of 3–5 cm H 2 O, and ventilator rate of 40–60/min.…”