to prove efficiency and safety of balloon dilatation in esophageal stricture’s treatment in children with epidermolysis bullosa.Methods
retrospectively we reviewed the charts of 19 patients with epidermolysis bullosa aged 4–17 years who had dysphagia symptoms and esophageal stricture, confirmed on contrast x-ray examination of the upper gastrointestinal tract. All patients were undergone fluoroscopically guided balloon dilatation of esophageal stricture. The first endpoint was a technical success of the procedure, esophageal lumen retrieve and relief dysphagia symptoms. The second endpoin was reintervention rate. Statistical analysis was conducted in Statistica 7.0 (USA). To assess statistical significance were used student’s t-test. Reliable considered differences with a probability of at least 95% (p<0.05).Results
19 patients with recessive dystrophic epidermolisis bullosa, dysphagia and esophageal stricture which was radiology verified, were treated with fluoroscopically guided balloon dilatation. The technical success rate was 100%. The mean follow-up time was 8.45±4,02 months (range, 4–19). The major part of the strictures was situated in the upper (10/21 [47.6%]) and middle (10/21 [47.6%]) esophageal segment, 1 stricture was in lower esophageal segment. Almost all of them were short focal strictures (less than 3 cm) III-IV grade [mean diameter 2,8 mm]by the Gallinger Yu.L., Godzhello E.A. endoscopic classification. One patient had a multiple strictures (1 in the upper esophageal segment, 1 in the middle esophageal segment) and one patient with inverse form of epidermolisis bullosa had a long stricture (more than 5 cm) which required 2 balloon dilatations during 1 procedure. After 12 months in patient with inverse form of epidermolisis bullosa stricture with symptoms of dysphagia relapsed and was successfully treated by reintervention. The stricture had the same characteristics as a previous case. Relapses in other cases were not occur and reintervention rate was 5,2% (1/19). Balloon diameter ranged from 8 to 18 mm. The postprocedural dysphagia score (0,32 [95% CI,0,09–0,55]) was significantly lower than baseline (2.95 [95% CI, 2,59–3,31]) (p<0,0001). Full expansion of the stricture’s“waist” was achieved in all cases. No procedural-related complications were detected. In 16% (3/19) of cases showed rise in body temperature on the day of surgery to febrile values which were cropped by NSAIDs. Almost all patients within 3–4 days had a slight pain and discomfort when swallowing, but it was not an obstacle for adequate oral feeding and gradual increase in enteral feeding with the expansion of the diet.