Congenital Diaphragmatic Hernia

Abstract

Over the last two decades there has been a constant improvement in the understanding of the pathophysiology of Congenital Diaphragmatic Hernia (CDH) and its management. However, the ideal treatment remains elusive. The earlier management strategy of immediate surgery is replaced by the principle of physiological stabilisation and delayed surgery. Conventional mechanical ventilatory techniques, with high pressures and hyperventilation to reverse ductal shunting and cause alkalinization, are being questioned because of the risks of barotrauma and consequent broncho-pulmonary dysplasia. It has also been shown that paralysis with pancuronium bromide for patients on conventional mechanical ventilation results in increased incidence of sensorineural hearing loss in childhood survivors of CDH. With the introduction of the concept of permissive hypercapnia and high frequency oscillation ventilation, the complications of pulmonary barotrauma are circumvented. Although ECMO therapy is invasive, yet has improved survival by about 15% independently, especially in critically ill infants who have the predictive mortality rate of more than 80%. Further insights into the pathophysiology of CDH and the introduction of less invasive therapeutic techniques in the form of high frequency oscillation ventilation, inhalation nitric oxide, surfactant, and perfluorocarbon liquid ventilation may even make the need for ECMO redundant.

Early fetoscopic tracheal occlusion for extremely severe pulmonary hypoplasia in isolated congenital diaphragmatic hernia: preliminary results

“…Congenital diaphragmatic hernia (CDH) occurs in approximately one in 3000 live births and can be accurately diagnosed by early second‐trimester ultrasound examination. Severe forms of isolated CDH are associated with high neonatal mortality and morbidity due to extremely reduced lung volumes, liver herniation and decreased pulmonary vascularization.…”

Congenital diaphragmatic hernia—does the presence of a hernia sac improve outcome? A systematic review of published studies

“…Interestingly, we observed that CDH cases with hernia sac were more often associated with liver herniation though the difference(s) were not statistically significant vs infants with no hernia sac. Intrathoracic liver herniation is considered to be a strong prognostic marker in foetal CDH and associated with poor outcome(s) [7,10,21]. In our current study, we found that more than half of the CDH cases we analysed with a hernia sac had reportedly liver herniation and yet a very high survival rate of almost 93%.…”

“…Despite current advances in neonatal intensive care, CDH is still associated with an unacceptably high mortality and morbidity [4][5][6]. Risk factors for poor prognosis currently include liver herniation ('liver up'), large defect size (type 'C' and 'D' lesions) and aberrant pulmonary developmental biology [6][7][8][9][10].…”

Permissive Hypercapnia in the Management of Congenital Diaphragmatic Hernia: Our Institutional Experience

“…Traditionally, it has been believed that extrinsic compression from abdominal contents on the developing lung results in pulmonary hypoplasia and persistent pulmonary hypertension. 1–3 Recent research, however, suggests that aberrations in pulmonary development itself may be causative in CDH. These studies suggest that the affected lung is intrinsically abnormal and may compromise the development of the diaphragm, resulting in a hernia.…”

“…Traditional methods of therapy involved an aggressive ventilator management strategy in which high peak inspiratory pressures (PIP), hyperoxygenation, and alkalization via hyperventilation and administration of sodium bicarbonate were used to maintain a postductal arterial oxygen pressure (PaO 2 ) of >90%, pH >7.2, and partial pressure of CO 2 (PaCO 2 ) within normal physiologic parameters. 1,6,7,10,11 The ventilatory pressures and volumes, and oxygenation required to achieve these goals, were recognized as causing lung trauma that was a significant source of morbidity and mortality in this population. Postmortem analysis of patients treated with aggressive ventilator management showed distinct evidence of barotrauma.…”