DOI: https://doi.org/10.36347/sasjm.2026.v12i11.002

Pages: 1142-1146

Dystrophic epidermolysis bullosa is a rare inherited disorder in which cutaneous and mucosal fragility complicates perioperative care. Upper gastrointestinal endoscopy presents particular challenges because airway access is shared and instrumentation can injure fragile tissues. We describe a 20-year-old woman with hereditary dystrophic epidermolysis bullosa referred for esophagogastroduodenoscopy because of persistent dysphagia despite three previous dilation procedures without symptomatic benefit. Contrast imaging demonstrated severe distal esophageal narrowing extending to the cardia. Propofol was administered by target-controlled infusion using the Schnider pharmacokinetic model, titrated to a Modified Observer’s Assessment of Alertness/Sedation score of 1–2. Supplemental oxygen was delivered through nasal cannulae incorporating capnography. A videolaryngoscope and flexible bronchoscope were prepared, with tracheotomy equipment immediately available. Recovery was uneventful. A focused narrative review examines airway assessment, aspiration risk, respiratory surveillance, atraumatic monitoring, and tissue protection. This clinical experience illustrates a sedation strategy supported by advance airway-rescue preparation; it does not establish its comparative safety. Perioperative planning should balance the risks of instrumentation against those of an unprotected airway, while maintaining reliable oxygenation, ventilation, and cardiovascular surveillance.