Huh Jung Kyu, Chung Moon Sang, Baek Goo Hyun, Oh Joo Han, Lee Young Ho, Gong Hyun Sik
Department of Orthopaedics, Hand and Upper Extremity Service, Seoul National University Bundang Hospital, Seongnam, Gyeonggi-do, Korea.
J Hand Surg Am. 2011 Apr;36(4):653-7. doi: 10.1016/j.jhsa.2010.12.033.
Kabuki make-up syndrome (KMS) is a multiple malformation/mental retardation syndrome that was first described in Japan but is now reported in many other ethnic groups. Kabuki make-up syndrome is characterized by multiple congenital abnormalities: craniofacial, skeletal, and dermatoglyphic abnormalities; mental retardation; and short stature. Common hand anomalies associated with KMS include persistent fingertip pad, brachydactyly, clinodactyly, and lax joints. We report a patient with KMS who presented with cleft hand, a feature that has not yet been described in KMS, and describe the potential genetic cause.