The approach and safety of esophageal dilation for treatment of strictures in children with epidermolysis bullosa

Objective: To analyze a large series of esophageal balloon dilations in patients with Epidermolysis Bullosa (EB) to determine procedural approach and frequency of post-endoscopic adverse events (AE). Methods: Retrospective chart review for AE occurrence and clinical outcomes in children and adolescents with EB, age 1–19, who underwent esophageal dilation for esophageal stricture(s) from January 2003- April 2016 at an academic, tertiary care, free-standing children's hospital. The primary outcome measure was occurrence of procedural AEs (defined as events occurring within 72 hours after endoscopic dilation procedure). Results: 231 fluoroscopy-guided esophageal balloon dilation procedures (209 anterograde, 20 retrograde, 2 both) were performed in 24 patients. Strictures were more common in the proximal portion of the esophagus with median stricture location 13 cm from the lips. From 2003–2012, 4.1% of dilations were retrograde. From 2013–2016, 20.2% of dilations were retrograde. AEs attributable to dilation occurred after 10.0% of procedures, and the most common AEs were vomiting, pain, and fever. No esophageal perforations, serious bleeding events, or deaths occurred secondary to dilation. The rate of post-dilation hospitalization was 6.9%. Dilation approach (anterograde versus retrograde) did not impact the likelihood of AEs. Conclusions: The characteristic esophageal lesion in EB is a single, proximal esophageal stricture. EB patients can safely undergo repeat pneumatic esophageal balloon dilations with minimal risk for severe complication. We observed a trend towards increased use of retrograde esophageal dilation. Address correspondence and reprint requests to David E. Brumbaugh, MD, Children's Hospital Colorado, 13123 E 16th Ave, B290, Aurora, CO 80045 (E-mail: David.Brumbaugh@childrenscolorado.org). Received 6 November, 2017 Accepted 23 June, 2018 Sources of Support: Dr. Feinstein is supported in part by the Eunice Kennedy Shriver National Institute of Child Health and Human Development (1K23HD091295–01). Conflicts of Interest: None declared. Supplemental digital content is available for this article. Direct URL citations appear in the printed text, and links to the digital files are provided in the HTML text of this article on the journal's Web site (www.jpgn.org). © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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