Narejo A S, Khan M U, Alotaibi W M, Khan M M
Department of Anesthesia, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Saudi J Anaesth. 2016 Jan-Mar;10(1):110-2. doi: 10.4103/1658-354X.169489.
Epidermolysis bullosa is a group of inherited rare skin disease, characterized by bullae formation in the skin or mucous membranes. The fundamental abnormality is collagen degeneration leads to splitting of various epidermal layers. Dystrophic epidermolysis bullosa (DEB) is one of the major forms of epidermolysis bullosa. These patients often admitted to the hospital for corrective surgeries, change of dressing, contracture release, and skin grafting. Anesthetic management of these cases is always a challenge. We are reporting a case of 5-year-old boy diagnosed as a case of DEB scheduled for upper lip contracture release, skin grafting and debridement of nonhealing scars under anesthesia. In this case, we have focused mainly on the anesthetic management, preparation of the monitoring, transportation, difficulties in establishing the venous accesses, and airway management.
大疱性表皮松解症是一组遗传性罕见皮肤病，其特征是皮肤或黏膜形成水疱。根本异常是胶原蛋白变性导致各表皮层分离。营养不良性大疱性表皮松解症（DEB）是大疱性表皮松解症的主要形式之一。这些患者常因矫正手术、换药、挛缩松解和皮肤移植而住院。这些病例的麻醉管理始终是一项挑战。我们报告一例5岁男孩，诊断为DEB，计划在麻醉下进行上唇挛缩松解、皮肤移植和不愈合瘢痕清创术。在该病例中，我们主要关注了麻醉管理、监测准备、转运、建立静脉通路的困难以及气道管理。
