Care of the Patient with Epidermolysis Bullosa

AbstractPurpose of ReviewThis review highlights advances in care of patients with severe epidermolysis bullosa (EB) that impact the anesthesiologist. Topics covered include EB molecular genetics, preoperative assessment, intra- and postoperative anesthetic care. Related topics include advances in common procedures performed on EB patients and new clinical practice guidelines.Recent FindingsThe latest EB nomenclature has largely eliminated eponyms. Molecular classification has revealed direct links to cardiomyopathy in several subtypes. Other molecular/genetic links to phenotypic associations such as muscular dystrophy and pyloric/jejunal atresia have been elucidated. Data supports fluoroscopic-guided guided balloon dilation of esophageal strictures and factors associated with recurrent stricture have been elucidated. Minimally invasive gastrostomy tubes can be placed either laparoscopically or using a percutaneous image-guided technique. THRIVE has been used successfully to facilitate fiberoptic intubation in children and adults with EB. Clinical practice guidelines have been established for many facets of EB patient care and clinical trials for potentially curative therapies are currently enrolling patients.SummaryLittle research exists on anesthetic techniques in EB patients, and few high-quality, high-powered studies support decision-making in the general or specific care of these patients. The International Dystrophic Epidermolysis Bullosa Research Association (DEBRA) has...
Source: Current Anesthesiology Reports - Category: Anesthesiology Source Type: research