Anesthetic Management of Adults With Epidermolysis Bullosa

Abstract

Epidermolysis bullosa (EB) is a group of rare, inherited diseases characterized by skin fragility and multiorgan system involvement that presents many anesthetic challenges. Although the literature regarding anesthetic management focuses primarily on the pediatric population, as life expectancy improves, adult patients with EB are more frequently undergoing anesthesia in nonpediatric hospital settings. Safe anesthetic management of adult patients with EB requires familiarity with the complex and heterogeneous nature of this disease, especially with regard to complications that may worsen during adulthood. General, neuraxial, and regional anesthetics have all been used safely in patients with EB. A thorough preoperative evaluation is essential. Preoperative testing should be guided by EB subtype, clinical manifestations, and extracutaneous complications. Advanced planning and multidisciplinary coordination are necessary with regard to timing and operative plan. Meticulous preparation of the operating room and education of all perioperative staff members is critical. Intraoperatively, utmost care must be taken to avoid all adhesives, shear forces, and friction to the skin and mucosa. Special precautions must be taken with patient positioning, and standard anesthesia monitors must be modified. Airway management is often difficult, and progressive airway deterioration can occur in adults with EB over time. A smooth induction, emergence, and postoperative course are necessary to minimize blister formation from excess patient movement. With careful planning, preparation, and precautions, adult patients with EB can safely undergo anesthesia.

Transnasal Humidified Rapid-Insufflation Ventilatory Exchange for Difficult Airway Management in Adults With Recessive Dystrophic Epidermolysis Bullosa: A Case Series

“…Among the multiple anesthetic considerations for patients with recessive dystrophic epidermolysis bullosa, airway management is regarded as the most complex and challenging, especially as patients become adults. 6,7 A case series from 2010 describes airway deterioration in a series of adult patients with dystrophic epidermolysis bullosa who had previously undergone tracheal intubation without difficulty as children. 4 After cycles of injury and fibrosis, adults with recessive dystrophic epidermolysis bullosa may develop microstomia, limited mandibular protrusion, neck contractures, and distorted airway anatomy, all increasing risk for difficult airways.…”

“…In all cases, intravenous access was established, and adhesive-free modified standard monitors were used. 2,6 According to our protocol (Supplemental Content 1, Supplemental Digital Content, http://links.lww.com/AACR/A494), THRIVE administered via Optiflow (Fischer and Paykel Healthcare) was used for preoxygenation and apneic oxygenation to avoid skin trauma associated with bag-mask ventilation. The THRIVE nasal cannula was lubricated, padded with petrolatum gauze, and applied at a flow rate of 10 to 30 L/min as tolerated for preoxygenation.…”

“…A & A PrACtiCe established, and adhesive-free modified standard monitors were used. 2,6 According to our protocol (Supplemental Content 1, Supplemental Digital Content, http://links.lww. com/AACR/A494), THRIVE administered via Optiflow (Fischer and Paykel Healthcare) was used for preoxygenation and apneic oxygenation to avoid skin trauma associated with bag-mask ventilation.…”

Anesthesia Management of the Lower Limb Amputation in an Adult Patient with Recessive Dystrophic Epidermolysis Bullosa

“…These disorders result from abnormal or absent structural proteins that cause weak or absent connections between the epithelium and underlying layers of the skin and mucosa [1,2]. In addition to blistering and chronic wounds, there are several extra cutaneous manifestations of EB such as microstomia, laryngeal stenosis, cardiomyopathy, esophageal stricture, gastroesophageal reflux, anemia and chronic pain [3]. Common surgical procedures in adult EB patients are esophageal dilation, contracture release, cesarian section and squamous cell carcinoma resection.…”

Lower-Leg Amputation Performed Under Regional Anesthesia in a Patient with Epidermolysis Bullosa: A Case Report

“…Epidermolysis bullosa (EB) is a type of hereditary skin disease characterized by extracutaneous manifestations of skin fragility and blistering, first reported by Von Hebra in 1870 [ 1 , 2 ]. The overall prevalence of EB varies by region, but is reported to be 4–11 cases per million [ 3 ]. EB is a rare condition in which eruption of blisters on the skin and mucous membranes can be caused by the slightest mechanical stimulus.…”