Esophageal Stricture
ICD-10 K22.2ICD-11 DA20.0.2

Treatment of Esophageal Stricture in Epidermolysis Bullosa

Clinical Scenario

Esophageal stricture is a recognised complication in patients with epidermolysis bullosa — a rare, inherited syndrome characterised by mucocutaneous fragility and blister formation due to defects in proteins involved in cellular integrity and adhesion. Strictures in this context occur most commonly in the proximal esophagus, and the inherent tissue fragility of the condition significantly shapes the management approach.

Management Approach

Care in this setting requires particular attention to the vulnerability of the esophageal mucosa. Nutritional support forms the cornerstone of management. Stricture-directed intervention involves dilation techniques, though the choice between approaches — and whether endoscopic methods are appropriate — depends on factors specific to the individual patient. Full protocol details, including the complete stepwise regimen and indication-specific selection criteria, are available via the link below.

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References

Epidermolysis bullosa is a rare, inherited syndrome leading to mucocutaneous fragility and blister formation due to defects in proteins involved in cellular integrity and adhesion.

Nutritional support is the mainstay of management in patients with this condition.

While endoscopic dilation techniques can usually be used for stricture management, this can potentially lead to iatrogenic sheer stress esophageal injuries, resulting in additional blistering and progression of strictures over time.

Thus, in some cases, use of nonendoscopic fluoroscopically guided hydrostatic balloon has been utilized, allowing for better assessment of size and severity of the stricture prior to dilation.

DOI: 10.1016/j.giec.2025.02.002

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