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Anesthesiology pp 421–428Cite as

Anesthesia for Epidermolysis Bullosa

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Abstract

Epidermolysis Bullosa (EB) is a heterogeneous group of mechanobullous disorders of the skin. EB is caused by 1 of 13 genes responsible for mechanical adhesion between the epidermis and the dermis. EB can have far-reaching effects, involving multiple organ systems in addition to the skin. Although more than 20 phenotypes have been identified, there are three major types of EB: epidermolysis bullosa simplex; junctional epidermolysis bullosa; and recessive dystrophic epidermolysis bullosa. While they all have cutaneous features in common, the systemic manifestations vary. Depending on the type and severity of the disease, anesthesia in these patients can be challenging. A comprehensive and multi-disciplinary plan is necessary to ensure safe and effective perioperative anesthetic care. From transferring the patient to the operating room table, to placing patient monitors, to vascular access, to airway management, each step requires meticulous attention to detail. While airway management can present a significant risk in the perioperative setting, with careful preoperative evaluation and a thoughtful approach, it can be performed safely. Understanding the different types, pathophysiology, and anesthetic considerations EB patients enables better long-term outcomes for these patients who require repeated gastrointestinal, dermatologic, and airway interventions throughout their lives.

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Author information

Authors and Affiliations

  1. Anesthesia and Pediatrics, Cincinnati Childrens Hospital Medical Center, Cincinnati, OH, USA

    Eric Wittkugel M.D. & Ali Kandil D.O., M.P.H.

Authors
  1. Eric Wittkugel M.D.
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  2. Ali Kandil D.O., M.P.H.
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Corresponding author

Correspondence to Eric Wittkugel M.D. .

Editor information

Editors and Affiliations

  1. Hospital University Pennsylvania, Perelman School of Medicine, Philadelphia, Pennsylvania, USA

    Basavana G. Goudra

  2. Department of Anesthesiology, Emory University, Atlanta, Georgia, USA

    Michael Duggan

  3. Department of Anesthesiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA

    Vidya Chidambaran

  4. University Hospitals Birmingham, Birmingham, United Kingdom

    Hari Prasad Krovvidi Venkata

  5. Emory University Hospital, Atlanta, Georgia, USA

    Elizabeth Duggan

  6. University of Alabama at Birmingham, Birmingham, Alabama, USA

    Mark Powell

  7. All India Institute of Medical Sciences (AIIMS), New Delhi, Delhi, India

    Preet Mohinder Singh

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Wittkugel, E., Kandil, A. (2018). Anesthesia for Epidermolysis Bullosa. In: Goudra, B., et al. Anesthesiology. Springer, Cham. https://doi.org/10.1007/978-3-319-74766-8_44

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  • DOI: https://doi.org/10.1007/978-3-319-74766-8_44

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-74765-1

  • Online ISBN: 978-3-319-74766-8

  • eBook Packages: MedicineMedicine (R0)

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