Outcomes and Predictors for Re-stenosis of Esophageal Stricture in Epidermolysis Bullosa: A Multicenter Cohort Study

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dc.contributor.authorPope, Elena
dc.contributor.authorMansour, Mark
dc.contributor.authorBerseneva, María
dc.contributor.authorLiy-Wong, Carmen
dc.contributor.authorSalas, Julio
dc.contributor.authorFuentes, Ignacia
dc.contributor.authorYubero, Maria Joao
dc.contributor.authorPalisson, Francis
dc.contributor.authorMartinez, Anna
dc.contributor.authorMellerio, Jemima
dc.contributor.authorLara-Corrales, Irene
dc.contributor.authorYang, Anes
dc.contributor.authorMurrell, Dedee
dc.contributor.authorTorres-Pradilla, Mauricio
dc.contributor.authorLucky, Anne
dc.date.accessioned2021-08-06T14:44:29Z
dc.date.available2021-08-06T14:44:29Z
dc.date.issued2020-09
dc.description.abstractBackground: Esophageal strictures are the common gastrointestinal complications in patients with epidermolysis bullosa (EB) requiring dilation. There is limited information on the best type of intervention, outcomes, and predictors for re-stenosis. Objectives: We aimed to investigate the frequency, clinical presentation of esophageal strictures in EB patients, and to ascertain the predictors of re-stenosis. Methods: We conducted a retrospective, multicenter cohort study involving 7 specialized, international EB centers on patients who were 0 to 50 years of age. Descriptive statistics and hazard risks for re-stenosis were calculated. Results: We identified 125 patients with 497 esophageal stricture episodes over a mean period of observation of 17 (standard deviation [SD]¼ 11.91) years. Dilations were attempted in 90.74% of episodes, using guided fluoroscopy 45.23%, retrograde endoscopy 33.04%, and antegrade endoscopy 19.07%. Successful dilation was accomplished in 99.33% of attempts. Patients experienced a median of 2 (interquartile range [IQR]: 1–7) stricture episodes with a median interval between dilations of 7 (IQR: 4–12) months. Predictors for re-stenosis included: number of strictures (2 vs 1 stricture:x2¼ 4.293,P¼ 0.038, hazard ratio [HR]¼ 1.294 (95% confidence interval [CI]: 1.014–1.652 and 3 vs 1 stricture:x2¼ 7.986, P¼ 0.005, HR¼ 1.785 [95% CI: 1.194, 2.667]) and a long (1 cm) segment stricture (x2¼ 4.599, P¼ 0.032, HR¼ 1.347 (95% CI: 1.026– 1.769). Complications were more common with the endoscopic approach (8/86, antegrade endoscopy; 2 /149, retrograde endoscopy vs 2/204, fluoroscopy; x2¼ 17.39, P-value <0.000). Conclusions: We found excellent dilation outcomes irrespective of the dilation procedure; however, with higher complications in the endoscopic approach. Long (>1 cm) segment involvement and multiple locations were predictive of stricture reoccurrence.es
dc.identifier.citationJournal of Pediatric Gastroenterology and Nutrition , 2020 september, vol. 71, n° 3: 310–314es
dc.identifier.urihttps://doi.org/10.1097/MPG.0000000000002820es
dc.identifier.urihttp://hdl.handle.net/11447/4244
dc.language.isoenes
dc.subjectEpidermolysis bullosaes
dc.subjectEsophageal dilationes
dc.subjectEsophageal strictureses
dc.titleOutcomes and Predictors for Re-stenosis of Esophageal Stricture in Epidermolysis Bullosa: A Multicenter Cohort Studyes
dc.typeArticlees

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