Abstract
Introduction Gastroesophageal reflux disease (GERD) is associated with accelerated decline in
lung health in children with cystic fibrosis (CF). Thus, antireflux surgery (ARS)
is offered to a selected CF cohort with refractory GERD, but outcomes remain poorly
investigated. This study aimed to determine the incidence of GERD in children with
CF and to evaluate complications and outcomes of ARS.
Materials and Methods A systematic literature-based search was conducted using various online databases
according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.
The number of GERD cases in pediatric CF cohorts who underwent diagnostic investigation(s)
was recorded. Data on postoperative complications and outcomes (including symptoms,
lung function, and nutritional status) following ARS were analyzed.
Results Ten articles (n = 289 patients) met the defined inclusion criteria (51% male; age range, 0.5 month–36
years). The overall incidence of GERD was 46% (range, 19–81%), derived from seven
studies (n = 212 patients). Four publications (n = 82 patients) reported on ARS due to uncontrolled GERD. All ARSs were Nissen fundoplication
(majority with gastrostomy placement). Major postoperative complications occurred
in 15 (18%) patients, two required redo-ARS. Median follow-up time was 2 years (range,
3 months–6 years); 59% showed symptom improvement, and pulmonary exacerbations and
decline in lung function were reduced. Nutritional status mainly improved in milder
CF cases. There were no deaths related to ARS.
Conclusion Approximately half of pediatric CF patients have GERD. Published data for children
with CF are limited and heterogeneous in terms of GERD diagnosis and outcomes following
ARS. However, ARS has shown to slow the deterioration of lung function in CF.
Keywords gastroesophageal reflux - cystic fibrosis - fundoplication - antireflux surgery -
pediatric