Department of Anaesthesiology and Intensive Care, University Hospital Muenster, Muenster, Germany.
Curr Opin Anaesthesiol. 2010 Aug;23(4):518-22. doi: 10.1097/ACO.0b013e32833bb50b.
Dystrophic epidermolysis bullosa is a rare disease mainly affecting small children. They often have to undergo different surgical procedures, for example balloon dilatation following esophageal strictures to maintain oral intake and prevent malnutrition.
So far, no specific treatment is available for patients with any forms of epidermolysis bullosa. However, more and more minimal invasive surgical techniques are used to manage symptoms of the disease, for example endoscopic balloon dilatation for severe esophageal strictures.
In this article implications for the anesthetic management of children with dystrophic epidermolysis bullosa are described. General anesthesia and sedation techniques are feasible if specific prerequisites are fulfilled. The team providing anesthesia and endoscopy must be familiar with the nature and disabilities of this disease. Coordinated care of the involved disciplines is crucial for all planned procedures regarding perioperative management.
营养不良型大疱性表皮松解症是一种罕见疾病,主要影响幼儿。他们经常需要接受不同的手术,例如食管狭窄后的球囊扩张术,以维持口服摄入并预防营养不良。
迄今为止,任何形式的大疱性表皮松解症患者都没有特定的治疗方法。然而,越来越多的微创外科技术被用于治疗疾病的症状,例如内镜球囊扩张术用于治疗严重的食管狭窄。
本文描述了营养不良型大疱性表皮松解症患儿的麻醉管理要点。如果满足特定的前提条件,则可以进行全身麻醉和镇静技术。提供麻醉和内镜的团队必须熟悉这种疾病的性质和残疾。所有涉及围手术期管理的计划手术都需要相关学科的协调护理。