Anderson, S.H.C.; Doig, L.; Meenan, J.
April 2003
Gut;Apr2003 Supplement 1, Vol. 52, pA45
Academic Journal
Background: Epidermolysis bullosa (EB) is a rare inherited disorder of the stratified squamous epithelium, characterised by blistering and scarring following minor trauma. Patients with the most severe forms, particularly dystrophic EB, develop bullae, inflammation, and scarring of the oesophagus following the ingestion of solid food. This results in dysphogia in the first two decades of life, severely compromising the ability to eat. There is no specific medical treatment for the disease and maintaining an adequate nutritional intake is often a central aim. Methods: We report the results of 53 adults and children with FB and oesophageal strictures who were treated with endoscopic balloon dilatation. The procedure was performed using propofol anaesthesia and "through the scope" balloons. Results: The median age at the time of index endoscopy was 16 years (range 3 to 61 years). A median of 2 barium studies (range 0 to 16) were performed per patient, identifying strictures at a median of 20 cm from the incisors (range 15 to 29 cm). 75% of patients had a single stricture (median = 1, range 1 to 6 strictures). Recurrent strictures tended to occur in the same position. The total number of dilatations performed was 182—the median number of dilatations per patient was 2, over a mean follow up period of 3.5 years. The median interval between dilatations was 18 months. The median balloon size used was 45 Fr (range 42-56 Fr). All but three patients had an improvement in the dysphagia score. The median change in weight following the procedure was an increase of 2.6 kg (p<0.0001) over a median 29 days. Apart from self-limiting odynophagia in 3 patients, there were no other post-procedural complications and no oesophageal perforations. Conclusion: Endoscopic balloon dilatation is a safe and effective treatment for the oesophageal strictures of EB, producing a long-term relief of dysphagia and an improvement in the nutritional status in the majority of patients.


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