Anesthetic management in a patient with epidermolysis bullosa [Epidermolizis Büllozali Hastada Anestezik Yönetim]

This presentation includes anesthetic approach to a patient with temporomandibular joint involvement due to dystrophic epidermolysis bullosa, who received brachial plexus block with axillary approach under ketamine and midazolam sedation for web-plasty operation performed for pseudosyndactylia. The patient was a 12 year-old male. He was scheduled to be operated in orthopedics and traumatology clinic for pseudosyndactylia associated with dystrophic epidermolysis bullosa. His mouth opening was limited to 1.5 cm due to temporomandibular joint involvement The patient received 0.7 mg kg1 of midazolam prior to the operation, and 1 mg kg1 of ketamine and 10 µg kg-1 of atropine before the brachial plexus block. Axillary artery was palpated and then block was applied with a nerve stimulator at 1 mA, with a frequency of 2 Hz of frequency and a velocity of 0.1 ms using a 24 G, 25 mm stimuplex needle, and motor responses of the median, radial, musculocutaneous and ulnar nerves were sought.. Local anesthetic solution was applied following observation of the motor response maintained after lowering the stimulating current from 0.5 to 0.3 mA. The patient received 0.25 % of bupivacaine (10 mL) + 1 % of prilocaine (10 mL), ie. a total of 20 mL local anesthetic was applied. In conclusion, careful attention should be paid not to traumatize the skin and mucosal membranes in epidermolysis bullosa patients. Difficult airway might be encountered and anticipated, so due preparation in advance will be necessary in surgical interventions and anesthesia applications for these patients. Choice of anesthesia should be planned according to the condition of the patient and to the type of the surgical intervention planned.