[Anesthesia for hand surgery in patients with bullous epidermolysis]

Epidermolysis bullosa is a rare genetic disease, characterized by the formation of bullae in the skin and squamous epithelium following minimal trauma. The majority of surgical indications specific to this disease concern autosomal recessive dystrophic epidermolysis bullosa (RDEB). Hand surgery is one of the fields of surgery involved. The cicatricial course of the lesions results in retraction and pseudosyndactylization of the fingers, sometimes leading to complete destruction of the hand. Since 1988, we have treated 23 patients, including 11 children under the age of 8 years and weighing less than 20 kg. 185 procedures were performed by two anaesthetists, using regional plexus blocks in 157 cases and general anaesthesia in only 28 cases. This series is comparable in number, to that reported by the London team, which prefers general anaesthesia. Regional anaesthesia has been previously used, but only following general anaesthetic induction. Our approach, based on the experience of a hand surgery center, is in marked opposition with this standard paediatric approach. Regional anaesthetic techniques, particularly in the upper limb, present many advantages in addition to their feasibility in small children: efficacy, simplicity, postoperative analgesia, and outpatient comfort. In the context of the specificities and difficulties encountered in the management of these patients suffering from epidermolysis bullosa, the authors demonstrate the value of first-line regional anaesthesia, even in very young children (less than 3 years). The presence of a parent in the operating room is a valuable aid, which is still insufficiently used, but which should be used with conviction for the benefit of all concerned.