Approach and Safety of Esophageal Dilation for Treatment of Strictures in Children With Epidermolysis Bullosa

Anderson, Bradley; Feinstein, James A.; Krämer, Robert; Narkewicz, Michael R.; Bruckner, Anna L.; Brumbaugh, David

doi:10.1097/mpg.0000000000002106

Cited by 10 publications

(6 citation statements)

References 27 publications

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“…This occurs most frequently in RDEB and rarely in KEB and EBS [4,11]. In case of (sub) total esophageal obstruction detected on an esophagogram, immediate referral to an EB reference center to perform a fluoroscopically-guided balloon esophageal dilatation, followed by oral or IV steroids (as dexamethasone 1 mg/kg twice a day for 3 days) or soluble prednisolone in equivalent dosage) is recommended [7,12,27] Evaluation of nutritional status and dietary recommendations [22,23] Management should be individualized depending on each patient's presentation and characteristics…”

Section: Acute Esophageal Obstruction Definitionmentioning

confidence: 99%

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Emergency management in epidermolysis bullosa: consensus clinical recommendations from the European reference network for rare skin diseases

Mellerio

Hachem

Bellon

et al. 2020

Orphanet J Rare Dis

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Epidermolysis bullosa (EB) comprises a group of genetic disorders with the hallmark of fragility of the skin and mucosal surfaces. The severity of different types of EB varies markedly as does the occurrence of extra-cutaneous involvement and complications. A number of emergency situations may occur in the context of EB including obstruction to oral intake from oral or esophageal blisters or scarring, acute airway obstruction, acute urinary retention, sepsis and corneal erosions. Whilst general management principles apply in each of these settings, specific considerations are essential in managing EB to avoid undue trauma or damage to delicate tissues. These recommendations have been developed from a literature review and consensus from experts of the European Network for Rare Skin Disorders (ERN-Skin) to aid decision-making and optimize clinical care by non-EB expert health professionals encountering emergency situations in babies, children and adults with EB.

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Section: Acute Esophageal Obstruction Definitionmentioning

confidence: 99%

“…6 Pediatric Ophthalmology Department, Children's Hospital, University Hospital Brno, Brno, Czech Republic. 7 Paediatric Dermatology Department, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania. 8 Endoscopy and Digestive Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.…”

Section: Fundingmentioning

confidence: 99%

Emergency management in epidermolysis bullosa: consensus clinical recommendations from the European reference network for rare skin diseases

Mellerio

Hachem

Bellon

et al. 2020

Orphanet J Rare Dis

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“…Each method could be performed using an antegrade approach, insertion of the guiding wire and balloon through the mouth or retrograde through a gastrostomy tube. The fluoroscopic method is the most frequently reported management strategy for EB patients (5)(6)(7)11,17) as it involves minimal instrumentation, reducing the risk of mechanical shearing trauma. Furthermore, balloon size is not limited by the caliber of an endoscope, thus allowing for maximum dilation and hypothetically decreasing the need for subsequent dilations (11).…”

Section: Discussionmentioning

confidence: 99%

Outcomes and Predictors for Re‐stenosis of Esophageal Stricture in Epidermolysis Bullosa

Pope

Mansour

Berseneva

et al. 2020

J. pediatr. gastroenterol. nutr.

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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: χ2 = 4.293, P = 0.038, hazard ratio [HR] = 1.294 (95% confidence interval [CI]: 1.014--1.652 and 3 vs 1 stricture:χ2 = 7.986, P = 0.005, HR = 1.785 [95% CI: 1.194, 2.667]) and a long (≥1 cm) segment stricture (χ2 = 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 2 = 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.

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“…EB‐associated esophageal strictures in the proximal area may arise, resulting in progressive dysphagia and requiring esophageal balloon dilatations (Image 1.3). 16 This has an impact on dental care. Prescriptions need to consider the patient's ability to swallow.…”

Section: Chapter 1: General Information On Epidermolysis Bullosa For mentioning

confidence: 99%

Clinical practice guidelines: Oral health care for children and adults living with epidermolysis bullosa

Krämer

Lucas

Gamboa

et al. 2020

Special Care in Dentistry

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Background: Inherited epidermolysis bullosa (EB) is a genetic disorder characterized by skin fragility and unique oral features. Aims: To provide (a) a complete review of the oral manifestations in those living with each type of inherited EB, (b) the current best practices for managing oral health care of people living with EB, (c) the current best practices on dental implant-based oral rehabilitation for patients with recessive dystrophic EB (RDEB), and (d) the current best practice for managing local anesthesia, This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

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Approach and Safety of Esophageal Dilation for Treatment of Strictures in Children With Epidermolysis Bullosa

Abstract: The characteristic esophageal lesion in EB is a single, proximal esophageal stricture. EB patients can safely undergo repeat pneumatic esophageal balloon dilations with minimal risk for severe complication. We observed a trend towards increased use of retrograde esophageal dilation.

Cited by 10 publications

References 27 publications

Emergency management in epidermolysis bullosa: consensus clinical recommendations from the European reference network for rare skin diseases

Emergency management in epidermolysis bullosa: consensus clinical recommendations from the European reference network for rare skin diseases

Outcomes and Predictors for Re‐stenosis of Esophageal Stricture in Epidermolysis Bullosa

Clinical practice guidelines: Oral health care for children and adults living with epidermolysis bullosa

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