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Epidermolysis Bullosa (EB) is characterized by the presence of extremely fragile skin and painful blister formations in the skin. We present the anesthetic management of 15-years-old, twin sisters with EB during the syndactyly operation. Premedication was done with 0.5 mg/kg nasal midazolam. Anesthesia induction was performed with sevoflurane without touching the facial masks to the skin. After intravenous 2 mgkg-1 ketamine and 50 mg fentanyl, anesthesia was maintained by intravenous 1 mg/kg/hr ketamine and 0.1 mg/kg/min remifentanil. We injected 2 mg intravenous morfin for postoperative pain control. Ketamine with remifentanil seems to be effective, simple, and safe for airway management and practical way to provide general anesthesia and analgesia of EB.

Successful use of ketamin combined with remifentanil in two patients with epidermolysis bullosa