Preventing Perioperative Complications of Epidermolysis Bullosa
Epidermolysis bullosa is a rare genetic mechanobullous disorder characterized by excessive fragility of skin and mucous membranes. Shearing forces and friction lead to intradermal fluid accumulation and resultant bullae formation. Complications of epidermolysis bullosa include skin infection, malnutrition, failure to thrive, esophageal strictures, tracheal stenosis, chronic pain, and high opioid tolerance. Children with epidermolysis bullosa will undergo multiple surgeries throughout their lifetimes, most commonly for wound care, extensive dressing changes, correction of pseudosyndactyly and joint contractures, and esophageal dilation. Improper management of these patients intraoperatively can have devastating consequences, including new blister formation. Shearing and compressive forces should be minimized as much as possible to avoid creating new lesions. This disease poses several unique challenges to the anesthesiologist, including monitoring, positioning, airway management, intravenous access, and pain control. The goals of safe anesthetic management include preventing formation of new lesions, optimizing pain control, and avoiding airway trauma, especially at induction and emergence. Clear communication among all staff members regarding safety of patient positioning is crucial. Intraoperative and postoperative analgesia can also be challenging, as patients may be tolerant to narcotics or suffer side effects such as nausea, constipation, and pruritus. Multimodal anesthesia is recommended to minimize narcotic use and provide a smooth and safe emergence.
KeywordsEpidermolysis bullosa, Monitoring, Positioning, Airway management, Intravenous access, Pain control Chronic pain Multimodal analgesia Non-opioid analgesia Dexmedetomidine
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