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Outcome of 100 consecutive dilatations of esophageal stenosis due to Epidermolysis Bullosa Dystrophica

Esophageal stenosis in the upper third of the esophagus is a common complication to Epidermolysis Bullosa Dystrophica (EBD). Patients often have a recurring need of dilatations. The patients are as… Click to show full abstract

Esophageal stenosis in the upper third of the esophagus is a common complication to Epidermolysis Bullosa Dystrophica (EBD). Patients often have a recurring need of dilatations. The patients are as a rule malnourished and brittle. Surgical care of these patients is centralized to one hospital in Sweden since 2024. The charts of all patients with EBD that had undergone esophageal balloon dilatations at Akademiska sjukhuset, Uppsala, during 2017-2025 were scrutinized. Data regarding surgeon, operative time, total operating room (OR) time, width of the stenosis, complications as well as age and weight was extracted. Fifteen patients had undergone a total of 100 dilatations. The mean age at dilatation was 13,9 years (2,3-29,9) and the mean weight was 30,7 kg (9,9-50). Five patients had undergone ten or more dilatations (one had undergone 30). Ten dilatations had to be done in retrograde fashion through a gastrostomy-canal since it was impossible to intubate the esophagus from above. Mean width of the stenosis when presenting with symptoms was 2,5mm (0-6). In one case it was impossible to dilate the esophagus due to an elongated near total stenosis. The main surgeon was the same in 91 cases. No surgical complications occurred. The anesthesia was often complex, but no major complications occurred. Mean operative time was 26,7min (10-116) and mean total OR time was 97,9min (51-250). Balloon dilatations of esophageal stenosis of EBD-patients is safe in experienced hands. The long total OR time mirrors the complexity when caring for these patients.

Keywords: epidermolysis bullosa; time; bullosa dystrophica; dilatations esophageal; stenosis; esophageal stenosis

Journal Title: British Journal of Surgery
Year Published: 2025

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