Combined inhalation anesthesia with sevoflurane and dexmedetomidine during oral cavity sanitation in a child with Cornelia de Lange syndrome

Cornelia de Lange syndrome is a rare hereditary orphan disease characterized by multiple dysembryogenetic stigmata, including musculoskeletal abnormalities, maxillofacial changes, neurologic disorders, and ocular involvement. A 13-year-old girl (body weight 54 kg, height 149 cm) was admitted to the dental clinic Stomatologiya NK (Nizhnekamsk) for oral cavity sanitation. The patient’s medical history included spastic tetraparesis, hydrocephalus with intracranial hypertension, and delayed psychomotor and speech development associated with Cornelia de Lange syndrome. No premedication was given before treatment. Anesthesia induction with sevoflurane was performed using a bolus technique. By the tenth breath, the child lost consciousness. The excitation stage occurred 45 s after the start of inhalation of the anesthetic gas mixture and lasted no more than 15 s. After peripheral venous catheterization, intravenous administration of 0.1% atropine sulfate (0.01 mg/kg), dexamethasone (0.1 mg/kg), and 1% propofol (2 mg/kg) was performed. Tracheal intubation was achieved on the second attempt using a guide (Mallampati class III; Cormack–Lehane grade 3), followed by initiation of pressure-controlled mechanical ventilation with transition to low-flow anesthesia: oxygen–air mixture 1 L/min and sevoflurane 2.5 vol% (minimum alveolar concentration, 1). During sevoflurane administration, continuous infusion of dexmedetomidine was maintained at 0.7 µg/(kg × h). After 20 minutes from the start of dexmedetomidine infusion, the sevoflurane dose was reduced to 1–1.2 vol% (minimum alveolar concentration, 0.4–0.5). During treatment, local infiltration anesthesia was not used. Fifteen minutes before the end of the procedure, paracetamol was administered intravenously at 15 mg/kg. Dexmedetomidine infusion was discontinued 15 min before completion of the dental treatment. Five minutes after discontinuation of sevoflurane, tracheal extubation was performed without complications, after which the patient was transferred to the recovery room. Muscle relaxants and opioid analgesics were not administered at any stage of anesthesia. Infusion therapy was not administered. Fifteen minutes after transfer to the recovery room, the consciousness score according to the Ramsay Sedation Scale was 2 points. One hour after completion of treatment, the patient was discharged home. The duration of anesthesia was 2 h 40 min, and that of the dental procedure was 2 h 30 min. This clinical case demonstrates the supra-additive effect of the combined use of sevoflurane and dexmedetomidine at subtherapeutic doses, providing adequate anesthesia at all stages of the dental intervention without local anesthesia. The case demonstrates the adequacy and sufficiency of the selected anesthesia technique, which may be further evaluated and validated in a larger patient cohort, including those with neurologic impairment.