Perioperative Anesthetic Management of a Child With Dystrophic Epidermolysis Bullosa Undergoing Hand Contracture...

Dystrophic epidermolysis bullosa (DEB) is a rare inherited disorder characterized by extreme skin and mucosal fragility, posing significant anesthetic challenges. We report the perioperative management of an 11-year-old boy with severe DEB and microstomia undergoing hand contracture release under general anesthesia. He presented with mitten-hand deformities, restricted mouth opening (1.2 cm), limited neck extension, anemia, and difficult intravenous access. Preoperative optimization included a blood transfusion. Meticulous precautions were taken to prevent skin trauma, including padded positioning, modification of monitoring techniques, and hydrocolloid dressings beneath adhesives. Inhalational induction with sevoflurane was performed while maintaining spontaneous ventilation. Fiberoptic intubation was unsuccessful due to restricted oropharyngeal space; tracheal intubation was achieved using a C-MAC video laryngoscope with continuous para-oxygenation through a size 5 nasal airway gently inserted into the child's left nostril. A tourniquet was avoided. Anesthesia and recovery were uneventful. This case highlights the importance of careful planning, gentle tissue handling, airway preparedness, and individualized anesthetic strategies to minimize perioperative complications in children with DEB.