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

Due to the cutaneous and mucosal fragility associated with epidermolys is 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 a ll these procedures may worsen, sometimes dramatically, the lesions in these young patients, still in a precarious health state. Basing on p ublished studies and their own experience, the authors have used in th ese patients a combined locoregional and general anaesthesia. The latt er was obtained with isoflurane, administered in the non intubated and spontaneously breathing patient through a closed surgical isolation c ontainer (Vi-Drape(R)), including the patient's head and ventilated wi th a ventilator generating a PEEP for long procedures. The results obt ained during 9 procedures in 3 children are reported and discussed. Fo r several shorter procedures (for example wound dressing), intramuscul ar ketamine was used.