title: [Surgical treatment of esophageal stenosis with recessive dystrophic epidermolysis bullosa--esophageal dilatation using balloon catheter method].
abstract: We report the details of our treatment of 5 patients with recessive dystrophic epidermolysis bullosa (RDEB). Good results were obtained by esophageal dilatation using the balloon catheter method. Prior to the operation, we corrected the patients' nutritional status with oral supplementation of Hinex-R as well as intravenous drip infusion of Intra-lipid, based on the nutrition index obtained by body weight, serum albumin, rapid turnover proteins, and blood minimum essence. After sufficient anesthesia of the nasopharyngeal mucosa with xylocain spray, we inserted a Microvasive Rigiflex Balloon Dilator into the stenotic region while scanning the upper GI. Then we dilated gradually by barium injection. The patients' condition improved after this treatment and follow up X-ray examination revealed no recurrence of stenosis. There were several advantages to this method: 1) It was safer and could be done repeatedly. 2) It caused less stress for patients. 3) Its effectiveness appeared rapidly. We analyzed the frequency and severity of esophageal stenosis of 12 cases with EB in our department. Ten out of 12 cases complained of subjective esophageal symptoms. Esophageal stenosis was observed in all 8 cases examined by esophagogram. We concluded that this surgical procedure for esophageal stenosis was as important as digit reconstruction in the management of RDEB.
authors.forename: H
authors.initials: H
authors.lastname: Yaguchi
authors.forename: J
authors.initials: J
authors.lastname: Miura
authors.forename: K
authors.initials: K
authors.lastname: Naito
authors.forename: M
authors.initials: M
authors.lastname: Manabe
authors.forename: H
authors.initials: H
authors.lastname: Ogawa
authors.forename: T
authors.initials: T
authors.lastname: Amagai
authors.forename: Y
authors.initials: Y
authors.lastname: Sanada
authors.forename: Y
authors.initials: Y
authors.lastname: Hirai
mesh_terms: D000293:Adolescent; D000328:Adult; D002404:Catheterization; D004820:Epidermolysis Bullosa / Q000150:complications*; D004940:Esophageal Stenosis / Q000209:etiology / Q000628:therapy*; D005260:Female; D006801:Humans; D008297:Male; D009748:Nutrition Disorders / Q000150:complications