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

Abstract

Background: To evaluate the prevalence of Klippel-Feil syndrome (KFS) in pediatric patients obtaining cervical CT imaging in the emergency room (ER). Methods: 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). Results: 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. Conclusions: 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.