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Brachial plexus anesthesia in eight patients with recessive dystrophic epidermolysis bullosa.

Recessive dystrophic epidermolysis bullosa (RDEB) is a rare (1;300,000) (1) chronic disorder of ectodermal structures. It is classified as a mechanobullous disorder (2), characterized by disintegration of the subepidermal connective tissue, with frictional separation of the epidermis by a split in the dermis, at a level below the dermoepidermal junction (3). This results in bullae formation after trauma, friction, or pressure. Originally described by Von Hebra (4), epidermolysis bullosa was further elucidated by Fox (5) and by Koebner (6) and more recently by Gedde-Dahl (3) and by Briggaman (7). The 16 forms of epidermolysis bullosa have since been categorized by genetic, clinical, and histologic criteria (3). RDEB is one form that is of special interest to anesthesiologists because of the involvement of mucus membranes. This presents a challenge to the anesthesiologist to maintain patency of the airway during an anesthetic without damaging epithelial surfaces. This problem has confronted anesthesiolgists for decades; various techniques of general inhalation anesthesia have been proposed (8-14) with unsatisfactory results. As a result, ketamine (15-18) has emerged as a popular alternative to inhalational anesthesia for these patients, because its respiratory stimulation can minimize the need for instrumentation of the airway. The use of spinal, epidural, (19,20) and brachial plexus anesthesia (21) has been reported, but only in single patients. We present a series of eight patients given axillary block for repair of pseudosyndactyly secondary to RDEB.