Anaesthesia Recommendations for Patients Suffering from Kabuki Syndrome

Abstract

Kabuki syndrome (KS) is a rare genetic disorder whose main clinical signs are multiple organ abnormalities and mental retardation. Niikawa et al. and Kuroki et al simultaneously described this syndrome in a group of patients in 1981 [1,2]. Genetic transmission of KS is autosomal dominant in more than 50% of patients with an estimated incidence of 1 in 32,000 [3]. Niikawa et al. suggested the name of 'Kabuki make-up syndrome" because the facial features of these patients were like the make-up actors in a Kabuki dance-drama in traditional Japanese theatre [1]. The facial features are characterized by an eversion of the lower lateral eyelid with long palpebral fissures, arched eyebrows, short columella, depressed nasal tip, and prominent ears.