[General anesthesia for a patient with Cornelia de Lange syndrome]

Cornelia de Lange syndrome involves anomalies in cardio-vascular and musculo-skeletal systems, and mental retardation. In addition, a patient with this syndrome shows a peculiar look. A 22-year-old woman with Cornelia de Lange syndrome underwent general anesthesia twice. She has a small mouth, thin lips, a short neck, short limbs, and stiffness of the neck and some joints associated with slight mental retardation. She was scheduled for arthroscopy and then for rotational acetabular osteotomy and valgus osteotomy. The maximum distance between the upper and lower incisors was 34 mm, when she opened her mouth. Anesthesia was induced with sevoflurane increased slowly to 7.0% in oxygen 6 l.min-1 in both procedures. After the administration of 4% lidocaine 3.5 ml into the pharynx, orotracheal intubation was attempted, but was not successful. Then blind naso-tracheal intubation was performed successfully under spontaneous respiration under sevoflurane-oxygen inhalation. The induction of anesthesia with sevoflurane under spontaneous respiration was useful for blind naso-tracheal intubation in a case with difficult intubation such as in Cornelia de Lange syndrome.