title: Twelve hour anaesthesia in a patient with epidermolysis bullosa.
abstract: Epidermolysis bullosa (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 room 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 women with recessive dystrophic epidermolysis bullosa 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.
authors.affiliation: Medical College of Wisconsin, Department of Anesthesiology, Milwaukee 53226, USA.
authors.forename: A E
authors.initials: AE
authors.lastname: Yonker-Sell
authors.forename: L A
authors.initials: LA
authors.lastname: Connolly
mesh_terms: D000328:Adult; D000768:Anesthesia, General / Q000379:methods*; D016108:Epidermolysis Bullosa Dystrophica / Q000150:complications / Q000235:genetics / Q000503:physiopathology*; D005260:Female; D005808:Genes, Recessive; D006225:Hand / Q000601:surgery; D006801:Humans; D009468:Neuromuscular Diseases / Q000150:complications; D011300:Preoperative Care; D012119:Respiration; D017592:Skin Care; D013524:Surgical Flaps / Q000379:methods; D013576:Syndactyly / Q000601:surgery; D013997:Time Factors