Twelve hour anaesthesia in a patient with epidermolysis bullosa

Abstract

Epidermolysis bulbsa (EB), an inherited disorder presents clinically with recurrent cutaneous blister formation with possible involvement of mucous membranes and other organs. The sequelae of this disease pose multiple challenges to the anaesthetist and operating mom team. Recent literature describes several anaesthetic techniques for the short surgical procedures this patient population may undergo. We describe the anaesthetic technique employed in a 28-yr-old woman with recessive dystrophic epidermolysis hullosa who underwent 12 hr reconstructive surgery followed by a review of the literature that includes a recent description of the possible association of EB with at least two distinct neuromuscular diseases. A detailed description of airway and skin management is described in addition to preoperative concerns. We conclude that a prolonged operative procedure can be undertaken successfully in this population with minimal sequelae involving skin integrity and airway management. © 1995 Canadian Anesthesiologists.