Long-term outcomes for resected congenital lobar emphysema: Follow-up recommendations for primary care providers
Loading...
Date
2021-05-15
Authors
Wolfe, Emma
Journal Title
Journal ISSN
Volume Title
Publisher
Abstract
Congenital lobar emphysema (CLE) is a rare lung anomaly characterized by
hyperinflation of one or more pulmonary lobes. It most often presents within the first six monthsof-
life with respiratory failure. Treatment depends on disease severity, with resection of the
affected lobe(s) necessary for children with severe respiratory compromise. Children with
resected CLE may be followed by their surgeon, but more commonly, the long-term care of these
patients falls to primary care providers, including physician assistants. As CLE is rare, primary
care providers may not have the experience to assess and treat long-term outcomes as these
patients grow up. The purpose of this study was to summarize the existing literature about the
long-term outcomes of children with resected CLE and create a best practice guideline for the
follow-up of patients after CLE resection. A literature search was performed, which revealed the
31 eligible references included in this review. The outcomes described in the references were
grouped into five themes: survival, respiratory, musculoskeletal, anthropometric, and general
well-being. Overall, the literature suggested that these children do well in terms of survival,
growth and development, and general well-being. However, there are areas that primary care
providers should screen for complications. Musculoskeletal complications such as pectus
excavatum and scoliosis are more prevalent in children with resected CLE than in the general
population. Additionally, children with resected CLE may be at increased risk of small airway
obstruction and diminished, but not significant, pulmonary function.
Description
Keywords
congenital lobar emphysema:, primary care providers, Long-term outcomes, recommendations, CLE