Epidermolysis bullosa (EB) is a group of rare genetic disorders that arise from at least 19 gene mutations for proteins that are involved in skin integrity. EB affects 1 out of 20,000 births in the United States and results in fragile skin that easily blisters from any minor friction or mechanical trauma. Other organ systems can also be seriously affected. The chronic skin inflammation and infections also places patients at risk for developing squamous cell carcinoma. Unfortunately, there is currently no cure for EB. The standard of care is supportive therapy and includes daily wound care, specialized dressings, and pain control. Because of the wide spectrum of systemic symptoms, there are numerous imaging findings that can be seen in patients with EB. These radiologic features can be categorized by body system including: 1) cardiac; 2) respiratory; 3) gastrointestinal; 4) genitourinary; 5) musculoskeletal; and 6) prenatal/fetal. There are various precautions that must be taken when performing any type of imaging study or anesthetic procedure for a patient with EB. These include but are not limited to: avoiding adhesives on the skin, providing special care when moving a patient, supporting pressure points on the imaging table, using large amounts of sterile water-based gel for ultrasound probes, and taking special care to protect the patient’s skin, airway and oral cavity during anesthesia or sedation events. The purpose of this poster is to: 1) review the various clinical presentations, pathologies, and associated imaging findings involved in EB, 2) examine imaging and anesthetic concerns when dealing with patients with EB, and 3) discuss the imaging and anesthesia approach used when evaluating EB patients.
SPR 2019 Annual Meeting & Postgraduate Course