Kabuki Sendromlu Hastada Anestezi Yönetimi

Abstract

Kabuki Syndrome is a rare syndrome with multiple congenital malformations. The facial features of the patients resemble the make-up of traditional Japanese theater players. This is why it is called Kabuki Make-up Syndrome. Syndrome is characterized by craniofacial dysmorphic disorder, skeletal-muscle anomalies, dermatological anomalies, congenital heart defects, urogenital anomalies, anorectal anomalies, epilepsy, joint laxity, dental anomalies, high palate, ophthalmologic anomalies and mental retardation. Difficult airway, cardiovascular problems, malignant hyperthermia, hypotonia, genitourinary abnormalities and epilepsia accompanied with this syndrome may increase the risk associated with general anesthesia. For this reason, anesthetic management of these cases are specific. İn this case report we present our anesthesia management in a 7-yearold boy with Kabuki's syndrome with Mallampati score class III, micrognathia, muscle weakness and bicuspid aortic valve to be performed due to dental surgery and phimosis.