Fluoroscopically guided dilation of esophageal strictures in patients with dystrophic epidermolysis bullosa: long-term results.

[dystrophic epidermolysis bullosa]

The purpose of this study was to investigate the immediate and long -term outcomes after fluoroscopically guided balloon dilation of esophageal strictures in a series of patients with dystrophic epidermolysis bullosa (DEB ).Between 2005 and 2011 , the medical records of all patients with DEB treated with fluoroscopically guided balloon dilation of esophageal strictures were included in the study and retrospectively analyzed . The indication for treatment was dysphagia attributed to at least one radiologically verified esophageal stricture . The primary endpoints of the study included procedural technical success , clinical improvement assessed with a 0 -4 dysphagia score , and major complication rate . Secondary endpoints were patient survival and reintervention rates .Nineteen consecutively registered patients with DEB (age range , 10 -51 years ; mean , 30 ± 12 .2 years ) and dysphagia due to esophageal strictures were treated with fluoroscopically guided balloon dilation . In total , 90 procedures and 121 dilations were performed to manage 28 lesions . Balloon diameter ranged from 8 to 18 mm . The mean follow-up time was 47 .51 ± 16 .64 months (range , 17 -73 months ). The technical success rate was 96 .7 % (87 /90 ). There were no major complications . The mean reintervention rate was 1 .19 dilations per patient per year , and the postprocedural dysphagia score (0 .72 [95 % CI , 0 .56 -0 .87 ]) was significantly lower than baseline (2 .50 [95 % CI 2 .35 -2 .65 ]) (p < 0 .001 ).Repeated fluoroscopically guided balloon dilation is safe and effective for the management of dysphagia caused by esophageal strictures in DEB . Use of this technique was associated with marked clinical improvement in dysphagia and satisfactory long -term reintervention rates with no major complications .