Abstract
Management of congenital diaphragmatic hernia (CDH) remains challenging. Despite advances in technologic and therapeutic strategies such as high-frequency mechanical ventilation, inhaled nitric oxide (iNO), and delayed surgical repair, the condition continues to carry a substantial mortality risk. In recent years, with increasing prenatal identification of fetuses affected by CDH, more effort has been directed toward strategies allowing in utero intervention, with the goal of improving survival. Unfortunately, a universally successful fetal treatment for CDH remains elusive. Over the past several decades, collective knowledge has markedly increased regarding the range of short- and long-term morbidities experienced by CDH survivors, and medical care has evolved as understanding of the disease has progressed. Affected patients demand and deserve a multidisciplinary approach, both in the inpatient arena and follow-up setting, with experts in neonatology, surgery, pulmonology, cardiology, and neurodevelopment working together to optimize outcomes. This article reviews the basic pathophysiology behind CDH, describes recent updates in the field, and outlines the long-term outcomes for these fascinating and complex patients.
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