[Anesthetic management in a patient with Cornelia de Lange syndrome]

Abstract

An eight-year-old boy with Cornelia de Lange syndrome underwent left inguinal hernioplasty and orchiopexy under general anesthesia. The patient with Cornelia de Lange syndrome had severe primordial growth failure with muscle-skeletal system such as cleft palate, micrognathia, and micromelia of the extremities and mental retardation as well as characteristic faces such as deep supercilia, etc. We suspected difficulty of endotracheal intubation due to this syndrome. Anesthesia was induced with intravenous injection of atropine 0.1 mg and ketamine 10 mg followed by inhalation of nitrous oxide 3 l x min(-1), oxygen 3 l x min(-1), and sevoflurane 5% without any muscle relaxant. Although his neck and temporomandibular joint were stiff, his trachea was intubated orally without difficulty with a 4.5 mm ID tracheal tube using a Macintosh laryngoscope. Anesthesia was maintained uneventfully by bolus intravenous injection of ketamine 5 mg and inhalation of oxygen and sevoflurane 2-3% with mechanical ventilation. The anesthetic management in a patient with Cornelia de Lange syndrome should be carried out with careful preoperative evaluation of physical status, and especially the difficult endotracheal intubation should be kept in mind. Induction of general anesthesia with injection of ketamine followed by inhalation of sevoflurane without muscle relaxant is a safe method in Cornelia de Lange syndrome.