Congenital Diaphragmatic Hernia

Abstract

This is a collective review of the literatures about congenital diaphragmatic hernia. Advances over the years have changed the way in which congenital diaphragmatic hernia is diagnosed and treated. If the diagnosis is made before birth, the mother should be transported to a tertiary hospital. Martenal transport of the fetus is much safer than the baby. But despite advances in infant transport and intensive care, congenital diaphragmatic hernia (CDH) still poses a serious threat to life during the neonatal period. Understanding the pathophysiology of congenital diaphragmatic hernia and its devastating effects on the lungs' bronchial and vascular development assists the physician in rational management during the perioperative period. Patients require immediate and appropriate stabilization. CDH is not necessarily a surgical emergency because repair does not allow expansion of the lung and does not improve the clinical picture. Improvements in ventilatory support and pharmacologic management of pulmonary hypertension have allowed surgeons to delay repair until circulatory hemodynamics, electrolytes, and oxygenation can be optimized. Surgery may actually worsen pulmonary mechanics and lead to pulmonary hypertension. Successful treatment demands prompt diagnosis, informed preoperative assessment and preparation, carefully timed surgical repair, and aggressive postoperative intensive care with a thorough understanding of the role of ECMO. In the future, early fetal intervention or lung transplantation may be included as standard therapy for patient with severe lung hypoplasia, but now these areas remain controversial and experimental. Clinical experience is limited.