Kabuki Syndrome and oral health

Abstract

Kabuki Syndrome (KS), also known as Niikawa-Kuroki Syndrome, is a rare congenital disorder first discovered in Japan in 1981. This disorder owes its name to the resemblance of patients’ facial features to the makeup of traditional theatre actors. The prevalence of KS in Japan is estimated at 1:32,000 live births. The live birth prevalence outside Japan presumably approximates that seen in the Japanese population. It has been calculated a minimum birth incidence of 1:86,000 in Australia and New Zealand. The presence of individuals with KS within the same family has suggested an autosomal dominant transmission. Although several chromosomal abnormalities have been associated with this syndrome, mutations in the KMT2D, MLL2 and KDM6A genes are considered to date, the main causes of Kabuki Syndrome. The current literature is poor in information regarding oral health findings in patients affected by KS. The aim of the present systematic review was to review the literature about oral health features associated with KS.

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