Interdisciplinary management and anesthetic nursing care for a pediatric patient with epidermolysis bullosa

Epidermolysis Bullosa (EB) is a rare disease characterized by the formation of blisters and vesicles on the skin and mucous membranes. There are 4 types: simple, junctional, dystrophic, and Kindler syndrome. They can have serious complications such as difficult airway, syndactyly, wound superinfection, or squamous cell carcinoma.

We present the case of a 6-year-old male patient with severe generalized recessive dystrophic EB, surgically intervened for syndactyly in the left upper limb and cures in both lower limbs.

These patients have a high risk of suffering adverse events related to the surgical process secondary to the fragility of their tegumentary system, mucous membranes, and noble organs. For this reason, prior to the surgical procedure, an anamnesis and planning was performed, confirming that the patient had a difficult airway as evidenced by previous anesthesia and by the Mallampati scale, a mouth opening greater than or equal to 3 cm and a reduced degree of head-neck mobility. Therefore, minimally invasive measures were taken such as a single venoclysis and maintenance of spontaneous breathing and control of this through nasal glasses with capnography and pulse oximetry. An alternative plan was considered in case these measures failed. In addition, special care was applied, such as the use of dressings and vaseline for skin protection, avoiding friction in mobilizations, pain management with intravenous analgesia and nerve blocks, and anxiolysis through family accompaniment.