Growing Up With Developmental Lung Diseases: A Review for the Adult Pulmonologist

Abstract

Topic importance: Improved childhood management strategies have decreased the early life morbidity and mortality of severe developmental lung disorders, such that an increasing number of individuals ultimately transition care to adult pulmonologists for management. Alternatively, individuals with milder malformations may present in adulthood or may have an increased risk for the development of more common adult pulmonary comorbidities (eg, COPD, pulmonary hypertension). As such, there is a critical need for adult pulmonologists to understand how developmental lung disorders may impact respiratory morbidity over the lifespan.

Review findings: There is growing recognition that abnormal lung development contributes to lung disease across the lifespan. This article reviews commonly encountered complex developmental lung diseases, including bronchopulmonary dysplasia, congenital diaphragmatic hernia, Down syndrome, and representative isolated airway and vascular anomalies. Their childhood and adult clinical presentations and comorbidities will be discussed.

Summary: This review aims to provide the adult pulmonologist with a framework for evaluating and managing individuals with developmental lung diseases.

Keywords: Down syndrome; bronchopulmonary dysplasia; congenital diaphragmatic hernia; developmental lung disease; obstructive lung disease.