Efficacy and safety of endoscopic dilation of esophageal strictures in epidermolysis bullosa

Title

Efficacy and safety of endoscopic dilation of esophageal strictures in epidermolysis bullosa

Creator

Anderson S H C; Meenan J; Williams K N; Eady R A J; Prinja H; Chappiti U; Doig L; Thompson R P H

Publisher

Gastrointestinal Endoscopy

Date

2004

Subject

adolescent; dysphagia; major clinical study; priority journal; follow up; scoring system; school child; human; article; female; male; adult; morbidity; treatment outcome; balloon dilatation; barium; body weight; contrast enhancement; endoscopic therapy; epidermolysis bullosa/th [Therapy]; epidermolysis bullosa; esophagus dilatation; esophagus stricture/th [Therapy]; nutritional status; propofol; feeding difficulties; surgical intervention; endoscopic balloon dilatation

Description

Background: Epidermolysis bullosa is a rare genetically determined disorder of the stratified squamous epithelium. Patients with the most severe forms develop scarring of the esophagus after ingestion of food. This results in dysphagia, which severely compromises the ability to eat. Maintenance of adequate nutritional intake is a central aim, but the most appropriate method is unknown. Methods: The results of endoscopic through-the-scope balloon dilation under propofol anesthesia in 53 patients with epidermolysis bullosa and esophageal strictures are reported. Results: Seventy-five percent of patients had a single stricture (range 1 to 6 strictures), most often in the proximal esophagus (median 20 cm from incisors). A total of 182 dilations were performed (median two per patient) over a median follow-up period of 3.5 years. For all but 3 patients, there was an improvement in the dysphagia score. There was a mean increase in weight after the procedure of 2.9 kg: 95% CI[2.0, 3.8]; p < 0.001, over a median 29 days. There was no significant post-procedure morbidity. Conclusions: Endoscopic balloon dilation is a safe and effective treatment for the esophageal strictures of epidermolysis bullosa. In the majority of patients, dilation relieves dysphagia and improves nutritional status.

Rights

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Citation

Anderson S H C; Meenan J; Williams K N; Eady R A J; Prinja H; Chappiti U; Doig L; Thompson R P H, “Efficacy and safety of endoscopic dilation of esophageal strictures in epidermolysis bullosa,” Pediatric Palliative Care Library, accessed October 18, 2021, https://pedpalascnetlibrary.omeka.net/items/show/16576.

Social Bookmarking