Anesthesia management in Kabuki make-up syndrome.

TitleAnesthesia management in Kabuki make-up syndrome.
Publication TypeJournal Article
Year of Publication2005
AuthorsSivaci R, Kahveci OK, Celik M, Altuntas A, Solak M
JournalSaudi medical journal
Volume26
Issue12
Pagination1980-2
Date Published2005 Dec
Abstract

Kabuki make-up syndrome KMS is a rare condition with a number of characteristic congenitali abnormalities. The syndrome is characterized by peculiar facial appearance resembling the make-up of actors in Kabuki, the traditional Japanese theater, skeletal anomalies, dermatoglyphic abnormalities, postnatal growth deficiency, and mental retardation. These are rare reports of central nervous system dysfunctions, other than mental retardation, and no previously described congenital talipes calcaneo-valgusi in this syndrome. We report the case of a 22-month-old girl having Kabuki make-up. At presentation, she had an adenoid hypertrophyi and a history of recurrent otitisi media. She had also delay in motor development, and a postnatal growth deficiency. The variable phenotypic expression is a well-known characteristic of the syndrome. For that reason, we should perform careful morphologic examination in every patient and their parents, and use flexotype laryngoscope Heine, Germany to visualize vocal cord in case of difficult intubation. At preoperative examination, as clinicians, we must be careful regarding patient morphology. Congenital heart defects and epilepsy are important for anesthesia management in KMS.

Alternate JournalSaudi Med J
Citation Key16380786