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

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.

Anesthetic Management of a Patient With Epidermolysis Bullosa Requiring Major Orthopedic Surgery: A Case Report.

Epidermolysis bullosa (EB) encompasses a wide spectrum of rare genetic disorders in which an abnormality in collagen leads to loss or absence of normal intracellular bridges. Friction or shear forces on the skin and mucosa result in blister, bullae, and scar formation. We present our experience in the management of a patient with EB who required multiple procedures for squamous cell carcinoma of the left arm, including forequarter amputation. We describe the anesthetic challenges in caring for a patient with EB undergoing major orthopedic surgery.

First steps in validating the pediatric anesthesia parent satisfaction (PAPS) survey.

BACKGROUND: In the last few decades, patient satisfaction has become a critical component of quality assessment and is being incorporated into payment for performance plans. However, assessment of satisfaction with anesthesia services is problematic and few validated satisfaction tools have been published. Assessing parent satisfaction with pediatric anesthesia services is even more challenging. OBJECTIVE: Our aim was to develop, implement, and start validating a set of survey questions that evaluate parental satisfaction with the pediatric anesthesia services in order to identify strengths and potential areas for improvement. METHODS: The Pediatric Anesthesia Parent Satisfaction (PAPS) survey contained 17 questions adapted from the American Society of Anesthesiologists Committee on Outcomes and Performance Measures. With consent of the hospital quality improvement committee, the PAPS survey was randomly administered on iPads to 250 English-speaking parents in the main operating room and procedure center postanesthesia care units prior to discharge. Statistical analyses including descriptive statistics, exploratory factor analysis, Cronbach's alpha and Raykov's rho coefficients, composite scale creation, correlations between items, composite scores, and overall satisfaction measures were used to provide evidence for a subset of validity and reliability types. RESULTS AND CONCLUSIONS: The PAPS survey is a short and simple tool for evaluation of parent satisfaction with pediatric anesthesia services and provides some evidence for validity and reliability. The majority (greater than 95%) of parents reported were satisfied or very satisfied with the care provided by the pediatric anesthesia department.