Combination of Ketamine with Dexmedetomidine Infusion as Non-operating Room Anesthesia/Opioid-Free Anaesthesia for Oesophagial Dilatation in a Paediatric Patient of Epidermolysis Bullosa Dystrophica: A Case Report

Abstract

Introduction: Dystrophic Epidermolysis Bullosa (DEB) poses major challenges, especially for anesthetic management. The main challenge faced is a pediatric patient with a difficult airway requiring general anesthesia. Any airway manipulation leads to immediate and significant trauma with bullae formation, thereby necessitating the need for postoperative ventilation and prolonged hospital stay. It is advisable to avoid any airway interventions, if possible. Case Presentation: An 11-year-old male child (weighing 15 kg) presented with non-bilious vomiting for 3 - 4 days and dysphagia for solids for 2 - 3 months. Personal history revealed the diagnosis to be a case of DEB at the age of 10 years (genetic testing) with persistent iron deficiency anemia. The patient underwent esophageal dilatation 6 months ago under general anesthesia (propofol, fentanyl on spontaneous respiration), requiring blood transfusion and an intensive care unit (ICU) stay for a day after the procedure. Dexmedetomidine (Dex) injection was started as a low-dose infusion (0.2 µg/kg) 10 minutes before the start of the procedure, followed by (0.2 µg/kg/hour). Before upper gastrointestinal (GI) scope insertion, ketamine injection (0.66 mg/kg) 10 mg intravenous (IV) bolus was given. Upper GI endoscopy and esophageal dilatation were performed in half an hour without any complications. Dexmedetomidine infusion was stopped after the removal of the scope, and the patient awakened within 3 - 4 minutes. He remained hemodynamically stable throughout the entire procedure. Recovery was uneventful, and the patient was obeying commands with no signs of respiratory depression, postoperative nausea/vomiting (PONV), or pain. Vital signs remained stable in the post-anesthesia care unit (PACU), and then the patient was moved to the ward. Conclusions: To date, there is no reported case of DEB being successfully managed with only two drugs, dexmedetomidine infusion and a single dose of ketamine for maintaining spontaneous ventilation (opioid-free anesthesia/OFA) with no airway intervention/manipulation, especially in non-operating room anesthesia (NORA) setting.