All issues > Volume 37(8); 1994

Case Report

J Korean Pediatr Soc. 1994;37(8):1149-1155. Published online August 15, 1994.

Three Cases of Apert Syndrome (Acrocephalosyndactyly)

Young Sil YS Ahn¹, Jong Won JW Lee¹, Jin Keon JK Bang¹, Doo Bong DB Lee¹

¹Department of Pediatrics, St. Francisco General Hospital, Seoul ,Korea

Abstract

Apert syndrome is an uncommon, congenital disorder characterised by malformation of the skull, most often acrocephaly or oxycephaly, in association with symmetrical syndactyly of both hands and feet. It is due to disturbance in the growth of bone and soft tissue affecting principally the head, the hands and the feet. The original description was presented by Troquart in 1886, and acrocephalosyndactyly was named by Apert in 1906. Since then, more than 200 cases have been reported in the world upto 1970. Recently, we have experienced three cases for typical Apert syndrome and made a brief related literature review.

Keywords :apert syndrome, acrocephaly, syndactyly