Epidermolysis Bullosa – An anaesthetic challenge

Epidermolysis bullosa (EB) is a rare genetic disorder characterized by blisters of skin and mucosa as a result of friction or minor trauma. EB can be of three forms: Epidermolysis Simplex (EBS), Junctional Epidermolysis Bullosa (JEB) and Dystrophic (DEB). Management of EB poses a challenge to the anaesthetist. We report a case of a 4-year-old girl who was diagnosed with DEB at birth. She was found to have an oesophageal stricture and was posted for balloon dilatation of the oesophagus. Airway examination revealed mouth opening of 1.5-2 fingers breadth with adequate neck movements. Difficult airway was anticipated. General anaesthesia was administered, and intubation was achieved using Airtraq video laryngoscope. Care was taken not to cause further trauma to the skin and mucosa in the perioperative period.