The anaesthetic management of patients with dystrophic epidermolysis bullosa

Abstract

SummaryThe case notes and anaesthetic charts of 44 patients with dystrophic epidermolysis bullosa were reviewed retrospectively. A total qf 390 general anaesthetics were performed for 469 Key wordsComplications; dystrophic epidermolysis bullosa. Anaesthesia.Dystrophic epidermolysis bullosa (DEB) is a rare, hereditary mucocutaneous disorder whose primary feature is the formation of blisters following minor shearing trauma to the skin. Treatment of DEB involves surgery which has a number of challenging implications for the anaesthetist.In the last 30 years there have been 17 case reports of the anaesthetic management of epidermolysis bullosa (EB) but only two series (both retrospective). One looked solely at airway management during anaesthesia [I] and the other was a review of the anaesthetic management of eight patients [2].St. Thomas' Hospital (which includes the Institute of Dermatology) has been a national referral centre for EB for 15 years and so has built up a large experience of the surgical and anaesthetic management. Patients and methodsThe case notes of patients with DEB who had surgery at St. Thomas' or the Lister Hospital, London SWl , were scrutinised to determine their anaesthetic management and to record any complications that had arisen. Results PatientsFrom 1982 to January 1992, 44 patients (26 female, 18 male) with DEB had a total of 390 anaesthetics given by 20 anaesthetists. Forty patients were diagnosed as having autosomal recessive DEB and four patients had either dominant DEB or localised recessive DEB.The age range of the patients at the time of operation is shown in Figure 1. The youngest patient to have a general anaesthetic was one year old and the oldest 48 years. Surgical procedures

Fluoroscopically Guided Endoluminal Balloon Dilatation of Esophageal Stricture Due to Epidermolysis Bullosa Dystrophica

“…Although the results of both types of surgical procedures are reported as successful, they are both major surgical procedures with considerable morbidity and mortality and should be performed in extensive esophageal involvement [5,7]. General anesthesia, which is mandatory during surgical procedures or endoscopic bougienage, is another problem in RDEB because of upper respiratory tract involvement and risk of perforation during intubation [14].…”

General anesthesia in a patient with dystrophic epidermolysis bullosa

“…Second, total intravenous anesthesia with spontaneous breathing preserved was another possible choice. Griffin and Mayou [3] reported that only 7% of 469 surgical patients with DEB received intravenous ketamine anesthesia and 3% received propofol. However, they did not describe the anesthetic methods in detail.…”

Twelve hour anaesthesia in a patient with epidermolysis bullosa

“…Where intubation is deemed difficult, an awake oral or nasal fibreoptic intubation is appropriate. 21 The use of a laryngeal mask airway (LMA) has been described. 21 This technique could potentially cause damage to the pharyngeal mucosa.…”

“…21 The use of a laryngeal mask airway (LMA) has been described. 21 This technique could potentially cause damage to the pharyngeal mucosa. If an LMA is to be used, it should be very well lubricated and inserted with care to avoid mucosal damage.…”

“…Certain authors suggest that barbiturates should be used with caution in patients with EB although barbiturates have been used without incident including previous anaesthetics in our case. 21 The use of propofol has been used in patients with EB.…”