Anesthetic management of pediatric patients with inherited epidermolysis bullosa undergoing lysis and reconstructive surgery of both hands adhesions

Abstract

Twenty-five pediatric patients (10 males, 15 females) with inherited epidermolysis bullosa, aged 3-16 yr, weighing 11-29 kg, of American Society of Anesthesiologists physical statusⅠ or Ⅱ, underwent lysis and reconstructive surgery of both hands adhesions from July 2015 to April 2017 in our hospital.Ketamine 4-6 mg/kg and atropine 0.01 mg/kg were intramuscularly injected at 20 min before admission to the operating room.Oxygen 2 L/min was inhaled by mask after admission to the operating room.Anesthesia was induced by IV injection of midazolam 0.08 mg/kg and ketamine 2 mg/kg.Pediatric patients kept spontaneous breathing.Anesthesia was maintained by IV infusion of propofol 5 mg·kg-1·min-1 and remifentanil 0.05-0.08 μg·kg-1·min-1.Mepitel was used to protect skin during surgery, and sodium potassium magnesium calcium and glucose injection was intravenously infused to replace the physiological requirement, blood loss and continued loss.Anesthesia time was (130±43) min, surgery time (107±42) min, the amount of intraoperative fluid infused (352±120) ml, blood loss (29±10) ml and emergence time (15±6) min.Intraoperative respiratory depression was found in 5 cases (20%); postoperative new bullae occured in 3 cases, and among the 3 cases, one developed on the back and the other two on the buttocks.No cardiovascular events occurred during and after surgery in this study. Key words: Epidermolysis bullosa; Anesthesia