Yonker-Sell A E, Connolly L A
Medical College of Wisconsin, Department of Anesthesiology, Milwaukee 53226, USA.
Can J Anaesth. 1995 Aug;42(8):735-9. doi: 10.1007/BF03012674.
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.
大疱性表皮松解症(EB)是一种遗传性疾病,临床上表现为反复出现皮肤水疱,可能累及黏膜和其他器官。该疾病的后遗症给麻醉医生和手术室团队带来了多重挑战。近期文献描述了针对这类患者可能接受的短时间外科手术的几种麻醉技术。我们描述了一名28岁患有隐性营养不良型大疱性表皮松解症的女性患者所采用的麻醉技术,该患者接受了长达12小时的重建手术,随后回顾了相关文献,其中包括近期对EB与至少两种不同神经肌肉疾病可能关联的描述。除了术前注意事项外,还详细描述了气道和皮肤管理。我们得出结论,在这类患者中可以成功进行长时间的手术操作,且对皮肤完整性和气道管理的后遗症最小。