Elena Pope1, Mark Mansour2, Maria Berseneva3, Carmen Liy-Wong1, Julio Salas4, Ignacia Fuentes5,6, Maria Joao Yubero5,7, Francis Palisson5, Anna Martinez8, Jemima Mellerio8, Irene Lara-Corrales1, Anes Yang9, Dedee Murrell9, Mauricio Torres-Pradilla10, Anne Lucky11. 1. The Hospital for Sick Children and University of Toronto, Ontario. 2. Schulich School of Medicine & Dentistry, Western University, London, Canada. 3. University of Toronto. 4. WellMedic, Rio de la Plata and DEBRA, Mexico. 5. Fundación DEBRA Chile. 6. Centro de Genética y Genómica, Facultad de Medicina, Clínica Alemana Universidad del Desarrollo. 7. Facultad de Medicina Clínica Alemana - Universidad del Desarrollo, Santiago, Chile. 8. Great Ormond Street Hospital NHS Foundation Trust, London, UK. 9. Department of Dermatology, St George Hospital, University of NSW, Sydney, Australia. 10. Fundacion Universitaria de Ciencias de la Salud and Hospital de San Jose, Bogota, Colombia. 11. Cincinnati Epidermolysis Bullosa Center, Cincinnati Children's Hospital, Cincinnati, OH.
Abstract
BACKGROUND: 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] = 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: χ = 4.293, P = 0.038, hazard ratio [HR] = 1.294 (95% confidence interval [CI]: 1.014--1.652 and 3 vs 1 stricture:χ = 7.986, P = 0.005, HR = 1.785 [95% CI: 1.194, 2.667]) and a long (≥1 cm) segment stricture (χ = 4.599, P = 0.032, HR = 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; χ = 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.
BACKGROUND: 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] = 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: χ = 4.293, P = 0.038, hazard ratio [HR] = 1.294 (95% confidence interval [CI]: 1.014--1.652 and 3 vs 1 stricture:χ = 7.986, P = 0.005, HR = 1.785 [95% CI: 1.194, 2.667]) and a long (≥1 cm) segment stricture (χ = 4.599, P = 0.032, HR = 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; χ = 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.
Authors: Andrew M Veitch; Franco Radaelli; Raza Alikhan; Jean-Marc Dumonceau; Diane Eaton; Jo Jerrome; Will Lester; David Nylander; Mo Thoufeeq; Geoffroy Vanbiervliet; James R Wilkinson; Jeanin E van Hooft Journal: Endoscopy Date: 2021-08-06 Impact factor: 10.093
Authors: Andrew M Veitch; Franco Radaelli; Raza Alikhan; Jean Marc Dumonceau; Diane Eaton; Jo Jerrome; Will Lester; David Nylander; Mo Thoufeeq; Geoffroy Vanbiervliet; James R Wilkinson; Jeanin E Van Hooft Journal: Gut Date: 2021-09 Impact factor: 23.059