Iohom G, Lyons B
Department of Anaesthesia, Our Lady's Hospital for Sick Children, Dublin, Ireland.
Eur J Anaesthesiol. 2001 Nov;18(11):745-54. doi: 10.1046/j.1365-2346.2001.00916.x.
Epidermolysis bullosa is a rare, genetically determined disorder characterized by excessive susceptibility of the skin and mucosa to separate from the underlying tissues after mechanical trauma. Patients suffering from this disease may have multiple medical problems, but the risk of anaesthesia is attributed mainly to oropharyngeal involvement; airway management may be hazardous and preservation of mucosa and skin integrity problematical. There is a paucity of data in the literature concerning the perioperative management of children with epidermolysis bullosa. We present our experience of managing 54 general anaesthetics (and two local anaesthetics) over the past 20 years.
The case notes of 16 children with epidermolysis bullosa were reviewed. The children underwent surgical procedures under local or general anaesthesia over a 20-year period.
Fifty-four general and two local anaesthetics were administered for 58 procedures in 10 children (mean age 12.6 years). Surgical procedures included oesophageal dilatation (24), insertion/revision of gastrostomy (16), dental procedures (10), hand surgery (2), skin biopsy (2) and others (4). Anaesthesia was induced by inhalation in 73.4% of patients and the airway was maintained with an endotracheal tube in 64.8%. Monitoring of anaesthesia was performed with pulse oximetry (89%), whereas electrocardiography and non-invasive blood pressure monitoring were used in 16.6% of cases each. The mean duration of anaesthesia was 64 min. Tracheal intubation was difficult in two of the five children who were intubated. Mucocutaneous blistering occurred in three children, otherwise there was no attributable morbidity.
With maximal skin and mucous membrane protection, anaesthesia in children with epidermolysis bullosa may be undertaken with few sequelae.
大疱性表皮松解症是一种罕见的、由基因决定的疾病，其特征为皮肤和黏膜在受到机械性创伤后极易与下方组织分离。患有这种疾病的患者可能有多种医疗问题，但麻醉风险主要归因于口咽受累；气道管理可能具有危险性，且黏膜和皮肤完整性的维护存在问题。关于大疱性表皮松解症患儿围手术期管理的文献资料匮乏。我们介绍过去20年中我们对54例全身麻醉（以及2例局部麻醉）的管理经验。
回顾了16例大疱性表皮松解症患儿的病历。这些患儿在20年期间接受了局部或全身麻醉下的外科手术。
10例患儿（平均年龄12.6岁）接受了58次手术，共实施了54例全身麻醉和2例局部麻醉。外科手术包括食管扩张（24例）、胃造口术置入/修复（16例）、牙科手术（10例）、手部手术（2例）、皮肤活检（2例）及其他手术（4例）。73.4%的患者通过吸入诱导麻醉，64.8%的患者通过气管内插管维持气道。89%的病例采用脉搏血氧饱和度监测麻醉，而心电图和无创血压监测分别用于16.6%的病例。平均麻醉持续时间为64分钟。在接受插管的5名儿童中，有2名插管困难。3名儿童出现了皮肤黏膜水疱，除此之外没有其他可归因的并发症。
在给予最大程度的皮肤和黏膜保护的情况下，大疱性表皮松解症患儿的麻醉可取得较少的后遗症。
