The anaesthetic management of patients with dystrophic epidermolysis bullosa. A review of 44 patients over a 10 year period.

The case notes and anaesthetic charts of 44 patients with dystrophic epidermolysis bullosa were reviewed retrospectively. A total of 390 general anaesthetics were performed for 469 surgical procedures over a 10-year period. Procedures included repair of syndactyly, dilatation of oesophageal strictures, extraction of teeth, excision of skin tumours and other procedures related to dystrophic epidermolysis bullosa. Anaemia, thrombocytosis and hypoalbuminaemia was found in many patients pre-operatively. Damage to skin or mucosa occurred peri-operatively. Four patients developed bullae around the head and neck, two patients developed oral or pharyngeal bullae and three patients developed conjunctival abrasions including one with a new corneal ulcer. There were no reports of laryngeal bullae and no bullae associated with intramuscular injections or the use of limb tourniquets. Thirty-one patients (70%) had evidence of oesophageal strictures and six regurgitated peri-operatively. In all cases there was a history of oesophageal disease. In 10 patients (23%) intubation of the trachea was difficult. It was carried out by various methods including blind nasal and fibreoptic intubation or by using the laryngeal mask airway. One patient requiring a series of general anaesthetics had a tracheostomy formed after fibreoptic intubation. Previous reports have suggested problems with the use of suxamethonium, nondepolarising muscle relaxants and thiopentone. These agents were all used in many of our patients without any adverse effects. There was one intra-operative death in a 35-year-old woman having an oesophageal dilatation which was complicated by oesophageal rupture.