Twenty Years of Progress in Congenital Diaphragmatic Hernia at the University of Florida

Abstract

Over the past 20 years the clinical paradigms underlying the care of children with congenital diaphragmatic hernia (CDH) have undergone profound changes. The purpose of this work is to provide an historic review of research and clinical studies related to CDH at the University of Florida (UF) and Shands Children's Hospital during the chairmanship of Edward M. Copeland, III, M.D. and to present our current clinical results. During Dr Copeland's tenure survival for newborns symptomatic with CDH treated at UF/Shands Children's Hospital has improved from less than 20 per cent to 85 per cent. Clinical observations have suggested and research studies at UF using a fetal lamb model have confirmed that fetal distress can occur late in gestation, which may predispose infants with CDH to pulmonary hypertension. However, our patient experience has confirmed that the most significant cause of mortality in human infants is not pulmonary hypertension but iatrogenic injury to their hypoplastic lungs. Strict avoidance of barotrauma in these babies has been the most important clinical advance during these two decades. Significant clinical and research problems remain including defining optimal prenatal care, management of complications during the first few weeks of life, and development of strategies to accelerate lung growth. Dramatic improvements in survival have resulted in children who manifest a number of clinical problems that were not evident when most of these patients died early in infancy. Our experience at Shand's Children's Hospital/UF indicates that feeding problems, respiratory infections, and management of subtle or overt neurologic complications may become major issues for some of these survivors and their families.