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

Autor:	Brooks Peterson M; From the Department of Anesthesiology, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado., Strupp KM; From the Department of Anesthesiology, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado., Brockel MA; From the Department of Anesthesiology, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado., Wilder MS; Department of Anesthesiology and Critical Care, Rady Children's Hospital, San Diego, California., Zieg J; From the Department of Anesthesiology, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado.; Department of Anesthesiology and Critical Care, Rady Children's Hospital, San Diego, California.; Department of Dermatology, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado.; Department of Biostatistics and Informatics, University of Colorado-Anschutz Medical Campus, Aurora, Colorado.; Department of Anesthesiology, Nemours Children's Hospital, University of Central Florida, Orlando, Florida., Bruckner AL; Department of Dermatology, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado., Kaizer AM; Department of Biostatistics and Informatics, University of Colorado-Anschutz Medical Campus, Aurora, Colorado., Szolnoki JM; Department of Anesthesiology, Nemours Children's Hospital, University of Central Florida, Orlando, Florida.
Jazyk:	angličtina
Zdroj:	Anesthesia and analgesia [Anesth Analg] 2022 Apr 01; Vol. 134 (4), pp. 810-821.
DOI:	10.1213/ANE.0000000000005749
Abstrakt:	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. Competing Interests: The authors declare no conflicts of interest. (Copyright © 2021 International Anesthesia Research Society.)
Databáze:	MEDLINE
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