Post-natal size and morphology of the sella turcica in Williams syndrome.

摘要

Williams syndrome (WS) is a rare congenital disorder with distinctive craniofacial features, cardiovascular abnormalities, mental retardation, and behaviour characteristics.The purpose of this study was to investigate the size and morphology of the sella turcica on profile cephalograms in a group of individuals with WS. The material consisted of radiographic cephalograms of 62 Norwegian children, adolescents, and adults with an age range of 4.7-44.4 years. The length, depth, and diameter of the sella turcica were measured and the mean values were compared with normal reference material from the Oslo University Craniofacial Growth Archive. In total, the two-dimensional size of the sella turcica in the WS group was smaller in length, depth, and diameter compared with the control group, but only occasionally reached a significance level of 5 per cent (Student's t-test).The morphology of the sella turcica was assessed and five different morphological types were identified; oblique anterior wall, extremely low sella turcica, sella turcica bridging, irregularity (notching) in the posterior part of the dorsum sellae, and pyramidal shape of the dorsum sellae. The occurrence of these morphological types was more frequent in the WS subjects compared with the reference material, except for sella turcica bridging, which was equally frequent. The females with WS had more dysmorphic sella turcicas than males.This study has demonstrated morphological aberrations in the sella turcica in Norwegian individuals with WS, which should be further elucidated in future research and combined with neurological andendocrinological investigations.