The prevalence of Klippel-Feil syndrome in pediatric patients: analysis of 831 CT scans.

Abstract

To evaluate the prevalence of Klippel-Feil syndrome (KFS) in pediatric patients obtaining cervical CT imaging in the emergency room (ER). We evaluated CT scans of the cervical spine of pediatric patients treated in the ER of a Level I Trauma Center from January 2013 to December 2015. Along with analysis of the CT scans for KFS, the following demographics were collected: age, sex, race and ethnicity. Mechanism of injury was also established for all patients. If KFS was present, it was classified using Samartzis classification as type I (single level fusion), type II (multiple, noncontiguous fused segments) or type III (multiple, contiguous fused segments). Of the 848 cervical CTs taken for pediatric ER patients during the study period, 831 were included. Of these patients, 10 had KFS, a prevalence of 1.2%. According to Samartzis classification, 9 were type I and 1 type III. The average age of patients with KFS was 16.02 years (10-18 years), with 8 males (80%) and 2 females (20%). Three had congenital fusions at vertebral levels C2-C3, two at C3-C4, three at C5-C6, one at C6-C7, and one with multiple levels of cervical fusion. The prevalence of KFS amongst 831 pediatric patients, who underwent cervical CT imaging over a 3-year period, was 1.2% (approximately 1 in 83). The most commonly fused spinal levels were C2-C3 and C5-C6. The prevalence of KFS in our study was higher than previously described, and thus warrants monitoring.