The management of bone fractures must achieve both the reduction and stability providence. However, dermatological conditions such as dystrophic epidermolysis bullosa (DEB) for instance can lead to catastrophic events when operating on the patient’s bone fracture. This can lead to wound infections and possible failure of bone healing leading to fracture nonunion. This dermatological disorder leads to heterogenous bullous dermatoses including cutaneous fragility leading to cutaneous bullous formation after exposure to any type of trauma. DEB is a rare inherited form of the disease characterized by the formation of cutaneous bullae. DEB is associated with a genetic mutation of COL7A1 gene that encodes collagen type VII. Due to the rarity and uniqueness of the disease, special modifications due to the challenges faced during the patient care approach were accomplished to prevent any possible harm to the patient. In this study, we propose a case report that is followed by the anesthetic and surgical challenges faced and how they were modified upon.
A 20-year-old female presented to the emergency department with a spiral mid-diaphyseal fracture of the femur after an incidence of falling from bed. The patient was previously diagnosed with dystrophic epidermolysis bullosa which made this case unique and complex. As the patient was examined by the team, her skin was covered with old blisters and wounds that have developed with the consequence of the disease. Due to the sophisticated dermatological condition and the unique presentation, the established multidisciplinary team took a decision to treat the patient with flexible intramedullary nailing in an open versus closed reduction technique, and modifications of the treatment approach were done based on the challenges in this case. The goal was to provide the management while minimizing the risk of infections and complications that would have arisen. The proposed case will set a baseline for the management of similar cases.
We suggest that in order to manage femur shaft fracture in the setting of dystrophic epidermolysis bullosa, modifying the management to avoid the least possible skin harm at any expense while managing the bone fracture is the golden approach.