Anaesthesia for correction of oesophageal stricture in a patient with recessive epidermolysis bullosa dystrophica: case report.

Epidermolysis bullosa dystrophica is a rare hereditary disorder which presents significant anaesthetic problems. These include malnutrition and anaemia; electrolyte imbalance in severe cases; renal failure and amyloidosis in progressive disease; association with porphyria; a history of steroid therapy. Technical problems associated with the necessity to avoid trauma to the skin and mucous membranes include those related to management of the airway and avoidance of regional techniques. Venepuncture may be difficult and oesophageal stricture increases the risk of regurgitation and aspiration. Anaesthetic management of a patient with epidermolysis bullosa dystrophica with oesophageal stricture presenting for colonic interposition is described and the problems associated with this disease are discussed.