The turricephaly index: A validated method for recording turricephaly and its natural history in Apert syndrome

Introduction We present the CT scan-derived turricephaly index (TI) as a quotient of the maximal occipito-frontal length of the skull to the distance from the centre of the sella to the highest point on the vertex as a validated tool for assessing turricephaly and evaluating surgical techniques aimed at reducing it. Materials and methods Measurements taken from CTs of non-operated children with Apert syndrome and age-matched controls were analysed using Centricity PACS system (from the lateral scout image) and the thick-sliced Osirix tool. CTs from non-operated children with Apert syndrome were used to investigate the natural history of their turricephaly both as a group and individually. Results There was statistically significant agreement between measurements taken from the CT scout and Osirix for 42 control children (R2 = 0.97) and 42 children with Apert syndrome (R2 = 0.98) and between two separate observers. There was a statistically significant difference (p < 0.001) between CT scout-derived TI value between controls (1.73 ± 0.12, range 1.46–1.99) and Apert children (1.42 ± 0.15, range 1.13–1.73). Analysis of 113 CTs of 65 non-operated children with Apert syndrome showed a decrease in turricephaly with age (positive spearman correlation: r = 0.50, p < 0.001). Analysis of 37 CTs of those with multiple (>2) CT's showed a similar decrease in turricephaly in the individual child (p < 0.001). Conclusions TI derived from the CT scout view provides a simple, objective and validated method for assessing turricephaly. We recommend it for monitoring and for the prospective evaluation of reconstructive techniques in children with complex/syndromic craniosynostosis.