Anesthetic Management and Outcomes of Patients With Epidermolysis Bullosa: Experience at a Tertiary Referral Center

Abstract

BACKGROUND: Epidermolysis bullosa (EB) is a group of rare epithelial disorders caused by abnormal or absent structural proteins at the epidermal-dermal junction. As a result, patients experience blisters and wounds from mild shearing forces. Some forms of EB are complicated by resultant scarring and contractures. The perioperative anesthetic management of patients with EB is complex and requires a systems-based approach to limit harm. We reviewed our experience with providing general anesthesia to patients at our tertiary EB referral center, including adverse events related to anesthetic care, outcomes in the immediate perioperative period, and details of anesthetic management. METHODS: We retrospectively reviewed the charts of all patients with EB anesthetized at the Children's Hospital Colorado between January 2011 and December 2016. A subset of pediatric anesthesiologists cared for all patients using a standardized clinical care pathway. Patient demographics, detailed anesthetic methods, immediate perioperative outcomes, and adverse events were characterized. RESULTS: Over a 6-year period, 37 patients underwent 202 general anesthetics. Most patients (75.7%) had dystrophic EB (DEB). Female patients comprised 48.6%. The majority (56.7%) traveled >50 miles to receive care, and many (35.1%) traveled >150 miles for their care. Common adaptations to care included avoidance of electrocardiogram leads (88.6%) and temperature probes (91.6%). Nasal fiberoptic intubation (n = 160) was performed, or natural airway/mask (n = 27) was maintained for most patients. Supraglottic devices were not used for airway management during any of the anesthetics. Anesthesia preparation time was longer (average 25.8 minutes [standard deviation {SD} = 12.7]) than our average institutional time (14 minutes). Succinylcholine was never used, and nondepolarizing muscle relaxants were used in only 1.5% of patient encounters. Blood was transfused in 16.3% of cases and iron infused in 24.8%. Average length of stay in the postanesthesia care unit was comparable to our institutional average (average 40.1 [SD = 28.6] vs 39 minutes). New skin or mucosal injury occurred in 8 encounters (4%), and desaturation occurred in 43 cases (21.3%). There were no major adverse events. CONCLUSIONS: By using a specialized team and a standardized clinical care pathway, our institution was able to minimize adverse events caused by the anesthetic and surgical care of patients with EB. We recommend natural airway or nasal fiberoptic airway management, meticulous avoidance of shear stress on the skin, and a multidisciplinary approach to care. Supportive therapy such as perioperative blood transfusions and iron infusions are feasible for the treatment of chronic anemia in this population.

Anesthesia Management of the Lower Limb Amputation in an Adult Patient with Recessive Dystrophic Epidermolysis Bullosa

“…These patients will likely have a very limited mouth opening, dental caries, and restriction of neck movement owing to recurring contractures. Even so, safe airway management can be achieved if carefully prepared [12]. Limited mouth opening, dental caries, tongue fixation and skin lesions on the face of this patient suggest that his endotracheal intubation is a challenging task.…”

“…However, the skin of his spine and buttocks is ulcerated and infected, which is a contraindication to regional anesthesia for lower extremity. Supraglottic airway is dismissed due to microstomia, intraoral scarring and ankyloglossia [12]. General anesthesia with endotracheal intubation was ultimately adopted due to the operative duration and risk of aspiration.…”

Consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa

“…Balloon dilation can be safely repeated without the risk of severe complications such as esophageal perforations or death [ 35 ]. For further discussion of procedural anesthetic management for people living with EB during esophageal dilatation, the panel refers the reader to the articles by Peterson et al [ 36 ] and by Gottschalk, et al [ 37 ].…”

Lower-Leg Amputation Performed Under Regional Anesthesia in a Patient with Epidermolysis Bullosa: A Case Report

“…On the other hand, there are reports that no serious tracheal intubation-related complications occurred [ 11 – 13 ]. It is possible that the pseudostratified columnar epithelium that covers the trachea and most parts of the larynx except the false vocal cords and parts of the epiglottis makes them less susceptible to friction-induced blisters than the oral cavity and pharynx, which are composed of multilayered squamous epithelium [ 14 ].…”