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

Abstract

Epidermolysis bullosa (EB) is a family of hereditary skin disorders resulting from abnormal or absent structural proteins that cause weak or absent connections between the epithelium and underlying layers of the skin and mucosa, which occurs in approximately 20 per 1 million live births. Extreme skin fragility, extracutaneous manifestations such as microstomia and laryngeal stenosis, and little experience accumulated make anesthesia management quite a challenge. Here we report the anesthesia management of the lower limb amputation in an adult patient who is the first EB patient admitted to our department during the past 30 years. The patient went through the peri-anesthesia period without emergency ventilation disorder and new bullous formation after adequate evaluation and careful planning, which may suggest that the patient with EB can safely undergo anesthesia if served meticulously enough even in non-EB specialized institutions.