Epidermolysis Bullosa

The anesthetic management of children with epidermolysis bullosa (EB) presents unique challenges to the pediatric anesthesiologist. Preoperative planning includes anticipation of a potentially difficult airway, focused protection of fragile skin and mucous membranes, and special consideration in the placement of standard monitors and intravenous access. Additionally, this chapter highlights the natural history and common procedures that may be performed in a child with EB; this will help prepare the anesthesiologist and the patient for a smooth anesthetic course. This chapter presents the case study of a 10-year-old girl with a medical history of recessive dystrophic EB, esophageal strictures, and poor nutritional status, who presents for esophageal dilation and percutaneous endoscopic gastrostomy tube placement.