ANESTHETIC MANAGEMENT OF PATIENTS WITH EP IDERMOLYSIS-BULLOSA UNDERGOING SURGERY OF THE HAND

Objective: To report on the anaesthetic management with axillary block of patients suffering from recessive dystrophic epidermolysis bullosa (RDEB), undergoing repetitive surgery of the hand. Study design: Retr ospective analysis of a case series. Patients: Twenty-two patients, in cluding 11 children less than 8-year-old and with a body weight under 20 kg, treated since 1988, were considered. Methods: For surgery, incl uding usually three stages at a 7-day interval, an axillary block was placed when feasible, after oral premedication midazolam (0.1-0.2 mg . kg(-1)) with a 25 gauge needle in patients of less than 30 kg of body weight and 22 gauge beyond. The local anaesthetic mixture included 2% lidocaine (5-10 mg . kg(-1)) and 0.5% bupivacaine (2-3 mg . kg(-1)). A catheter for repetitive injections had not been inserted. For childr en less than 10-year-old a parent was present in the theater during al l the course of operation. Results: Between 1988 and 1995, 22 patients underwent 160 operative interventions on 54 hands. Regional anaesthes ia was used in 142 cases, including 140 axillary and 2 interscalene bl ocks. General anaesthesia was only required in 20 cases, either alone or associated with regional anaesthesia. The success rate of axillary blocks was 98%. Discussion : For surgery of the hand in patients with RDEB, we switched in 1988 from general anaesthesia with ketamine to ax illary block, even in young children, as it is closer to the no-touch principle, which is essential to prevent from blistering. In compariso n to general anaesthesia, regional anaesthesia raises neither the prob lems of airway and vascular access, nor those of instrumental monitori ng. The main factors of success with regional anaesthesia are technica l skills, expertise in the management of patients with RDEB and parent al presence in the operating room which makes the procedure less stres sful far children.