SURGERY OF EPIDERMOLYSIS DYSTROPHICA BULLOSA IN CHILDREN - VALUE OF COMBINED GENERAL AND LOCOREGIONAL ANESTHESIA

Due to the cutaneous and mucosal fragility associated with epidermolysis bullosa, this disease is a source of various practical problems for the anaesthesiologist concerning the surgical posture, the monitoring of vital functions, the airways control and the vascular access, as all these procedures may worsen, sometimes dramatically, the lesions in these young patients, still in a precarious health state. Basing on published studies and their own experience, the authors have used in these patients a combined locoregional and general anaesthesia. The latter was obtained with isoflurane, administered in the non intubated and spontaneously breathing patient through a closed surgical isolation container (Vi-Drape(R)), including the patient's head and ventilated with a ventilator generating a PEEP for long procedures. The results obtained during 9 procedures in 3 children are reported and discussed. For several shorter procedures (for example wound dressing), intramuscular ketamine was used.