A Novel Nasal Continuous Positive Airway Pressure-Assisted Approach for Airway Management in a Patient With Dystrophic Epidermolysis Bullosa: A Case Report

Dystrophic epidermolysis bullosa (DEB) is a severe hereditary subtype of epidermolysis bullosa caused by mutations in the collagen gene. It is characterized by extreme fragility of the skin and mucous membranes, where even minimal shear stress can induce blister formation. Airway management in DEB patients is challenging because of oral scarring, limited mouth opening, and mucosal vulnerability. We report on the successful use of a modified nasopharyngeal continuous positive airway pressure (CPAP) technique for airway management. A 13-year-old boy (125 cm, 21 kg) with recessive DEB was scheduled for dental extraction under general anesthesia. Chronic labial scarring limited mouth opening to 1 cm, making oral intubation impossible. Anesthesia was induced with intravenous midazolam and fentanyl, followed by sevoflurane inhalation while maintaining spontaneous breathing. A small-diameter endotracheal tube (3.0 mm ID) was inserted through the left nostril into the pharynx and connected to the anesthesia circuit. A 3.0 mm bronchoscope was inserted through the right nostril. With the mouth closed, CPAP at 5 cm H₂O and inspiratory pressure at 15 cm H₂O were applied, expanding the pharyngeal space and improving glottic visibility. After confirming a stable airway, rocuronium (12 mg) and fentanyl (50 µg) were administered. A 5.0 mm spiral silicone tube was advanced over the bronchoscope and successfully inserted on the first attempt. Oxygen saturation remained at 100% during induction and surgery, and extubation was uneventful with no airway blister formation. Nasopharyngeal CPAP using a small-diameter endotracheal tube provided continuous oxygenation, improved visualization, and allowed for safe use of muscle relaxants, resulting in atraumatic, first-attempt intubation. This modified approach may be a valuable option for complex pediatric airway management in DEB.