1538. Demographics, Comorbidities, and Common Procedures in Children with Klippel-Feil Syndrome: Analysis of the Pediatric National Surgical Quality Improvement Program
Authors: Hardy Evans; Kishan Patel, BS; Abhijith Matur, BS; Rafael De La Garza Ramos, MD; Jonathan Nakhla, MD; Reza Yassari, MD, MS; Rani Nasser, MD; Charles Stevenson, MD; Francesco Mangano, DO; Aria Nouri, MD, MSc; Joseph Cheng, MD, MS (Cincinnati, OH)
Klippel-Feil Syndrome (KFS) is a congenital anomaly that manifests with one or more levels of cervical vertebrae fusion. No large scale studies have been conducted on KFS. To address this, we investigated the Pediatric NSQIP database to report on the demographics, comorbidities, and common procedures associated with KFS.
Pediatric NSQIP data from 2012-2016 were analyzed. Pearson Chi-Squared statistics were calculated to determine significant differences in demographics, comorbidities, and outcomes in patients with KFS. Independent Samples t Tests were used to determine differences in procedure lengths and time to discharge. All statistical analyses were performed with significance at p<0.05.
152 patients (0.04%) in Pediatric NSQIP had KFS. There was no statistically significant difference in race, ethnicity, or gender between patients with and without KFS. When compared to the non-KFS population, KFS patients had higher rates of pulmonary and airway abnormalities, including structural anomalies (14.5% p<0.001) and asthma (13.2%, p<0.001). Additionally, 25.6% of KFS patients had one or more cardiac comorbidities (p <0.001), including ostium-secundum type ASD (8.6%, p=0.027), VSD (7.9%, p<0.001), and tetralogy of fallot (4.6%, p<0.001). Other common comorbidities included neuromuscular disorders (19.1%, p<0.001) and renal agenesis/dysgenesis (10.5%, p<0.001. The most commonly performed procedures were suboccipital craniectomy with laminectomy (n=15), and laminectomy with release of tethered spinal cord (n=12). There were no significant differences in post-procedure outcomes between the KFS and control populations.
Patients with KFS have a higher rate of comorbidities than the general population, particularly those of the cardiovascular, pulmonary, and urogenital systems. Neurosurgical procedures commonly performed on pediatric patients with KFS include suboccipital craniectomy and release of tethered spinal cord. These procedures resulted in similar outcomes to patients without KFS. Further research should include characterization of the adult KFS population, and comparisons of surgical approaches to the spine in patients with KFS.