International recommendations on neonatal management with congenital diaphragmatic hernia: literature review

Abstract

Congenital diaphragmatic hernia (CDH) is a defect of diaphragm development which is characterized by herniation of the abdominal content into the chest resulting in pulmonary hypoplasia and pulmonary hypertension of various degrees. Mortality and sickness rates among survived children with this pathology remain high all over the world. Prenatal diagnostics of high quality will help to find neonates with severe CDH and promote mobilization of human and material resources for optimal management at the tertiary level of giving medical aid. Attention of specialists in prenatal ultrasound diagnostics should be drawn to the necessity of measuring observed/expected lung(to(head ratio and location of the liver in fetus. Optimal treatment of neonates during preoperative period is focused on mild ventilation, avoidance of oxygen toxicity, baro/volutrauma of a hypoplastic lung and cardiac support. Administration of pulmonary vasodilator therapy including inhaled nitric oxide and extracorporal membrane oxygenation is pathogenetically substantiated for patients with pulmonary hypertension, and surgical correction should be delayed till stabilization of patient's condition. Echocardiography is a critically important diagnostic method. It enables to determine severity of pulmonary hypertension and myocardial functional disorders, to find targeted systemic methods of treatment that improve hemodynamic function or decrease pulmonary vascular resistance. Considering an insufficient level of evidence of the existing international recommendations, large(scale multi(center randomized studies on investigation of the best methods to prevent, diagnose and treat neonates with CDH are essential. No conflict of interest was declared by the authors. Key words: congenital diaphragmatic hernia; neonate; lung-to-head ratio; pulmonary hypertension; extracorporal membrane oxygenation.