Outcomes and Predictors for Re-stenosis of Esophageal Stricture in Epidermolysis Bullosa: A Multicenter Cohort Study.
J Pediatr Gastroenterol Nutr
; 71(3): 310-314, 2020 09.
Article
en En
| MEDLINE
| ID: mdl-32833391
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]â=â11.91) 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 χâ=â4.293, Pâ=â0.038, hazard ratio [HR]â=â1.294 (95% confidence interval [CI] 1.014--1.652 and 3 vs 1 strictureχâ=â7.986, Pâ=â0.005, HRâ=â1.785 [95% CI 1.194, 2.667]) and a long (≥1âcm) segment stricture (χâ=â4.599, Pâ=â0.032, HRâ=â1.347 (95% CI 1.026--1.769). Complications were more common with the endoscopic approach (8/86, antegrade endoscopy; 2â/149, retrograde endoscopy vs 2/204, fluoroscopy; χâ=â17.39, P-value <0.000).CONCLUSIONS:
We found excellent dilation outcomes irrespective of the dilation procedure; however, with higher complications in the endoscopic approach. Long (>1âcm) segment involvement and multiple locations were predictive of stricture reoccurrence.
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Epidermólisis Ampollosa
/
Estenosis Esofágica
Tipo de estudio:
Etiology_studies
/
Incidence_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Límite:
Humans
Idioma:
En
Revista:
J Pediatr Gastroenterol Nutr
Año:
2020
Tipo del documento:
Article