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Anesthesiology pp 421-428 | Cite as

Anesthesia for Epidermolysis Bullosa

  • Eric Wittkugel
  • Ali Kandil
Chapter

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.

Keywords

Epidermolysis bullosa Anesthesia Vascular access Airway management Fiberoptic intubation Cardiomyopathy 

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Anesthesia and PediatricsCincinnati Childrens Hospital Medical CenterCincinnatiUSA

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