Cystic fibrosis: a surgical matter?

J Pediatr Surg. 2014 May;49(5):753-8. doi: 10.1016/j.jpedsurg.2014.02.089. Epub 2014 Mar 29.

Abstract

Background: Gastroesophageal reflux disease (GERD) is very common in patients with chronic lung diseases. We evaluated the incidence of GERD in young patients with cystic fibrosis (CF) and defined the characteristics of gastroesophageal reflux episodes analyzed by pH-multichannel intraluminal impedance (pH-MII) and esophagogastric scintigraphy.

Patients and methods: Since 2010, 31 patients with CF underwent pH-MII. Scintigraphy and upper endoscopy were performed in positive GERD patients. Forced expiratory volume in 1 second (FEV1%) predicted was detected.

Results: pH-MII was positive in 17/31 (54.8%) patients (mean age: 12.4 years; range: 4-17 years). pH monitoring detected an average of 64.6 acid reflux events 4.4 episodes >5 minutes in duration. The DeMeester score was 38.5. Impedance identified a mean number of reflux episodes of 66 (65.2% acid; 32% weakly acidic; 2.8% nonacidic), 28% of which reached the proximal esophagus. Esophageal transit and gastric emptying were delayed in 6/13 (46.1%) and in 5/15 (33.3%) cases, respectively. No differences were found in lung function between positive and negative GERD patients (P=0.88).

Conclusions: Pediatric patients with CF have a high incidence of GERD with acidic events. These patients should be investigated with pH-MII and scintigraphy in order to make an early diagnosis and determine the most appropriate follow-up.

Keywords: Cystic fibrosis; Fundoplication; GERD; Gastric scintigraphy; pH-multichannel intraluminal impedance.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / physiopathology
  • Endoscopy, Gastrointestinal
  • Esophageal pH Monitoring
  • Esophagus / diagnostic imaging
  • Esophagus / physiopathology
  • Female
  • Forced Expiratory Volume
  • Gastroesophageal Reflux / diagnosis*
  • Gastroesophageal Reflux / physiopathology
  • Gastroesophageal Reflux / surgery*
  • Humans
  • Male
  • Manometry
  • Radionuclide Imaging
  • Stomach / diagnostic imaging
  • Stomach / physiopathology