Anesthetic management in a patient with Kindler′s syndrome

A 35-year-old male with pan-anterior urethral stricture was scheduled to undergo perineal urethrostomy. He was a known case of Kindler's syndrome since infancy. He was having a history of blister formation, extensive poikiloderma and progressive cutaneous atrophy since childhood. He had a tendency of trauma-induced blisters with clear or hemorrhagic contents that healed with scarring. The fingers were sclerodermiform with dystrophic nails and inability to completely clench the fist. Airway examination revealed thyromental distance of 7 cm with limited neck extension, limited mouth opening and mallampatti class III with a fixed large tongue. He was reported as grade IV Cormack and Lehane laryngoscopic on previous anesthesia exposure. We described the anesthetic management of such case on guidelines for epidermolysis bullosa. In the operating room, an 18-G cannula was secured in the right upper limb using Coban(™) Wrap. The T-piece of the cannula was than inserted into the slit and the tape was wrapped around the extremity. The ECG electrodes were placed on the limbs and fixed with Coban(™). Noninvasive blood pressure cuff was applied over the wrap after wrapping the arm with Webril(®) cotton. Oral fiberoptic tracheal intubation was done after lubricating the laryngoscope generously with a water-based lubricant with 7-mm endotracheal tube. Surgery proceeded without any complication. After reversing the residual neuromuscular block, trachea was extubated once the patient became awake. He was kept in the postanesthesia care unit for 2 hours and then shifted to urology ward.