Introduction: Isolated tethered cord syndrome (iTCS) may be diagnosed in a symptomatic patient with a low-lying conus caused by a tight and/or fatty filum. Its incidence, patient characteristics, and outcomes after treatment with untethering surgery have been described largely through single-center studies. Indications for and the proper timing of surgery remain a subject of debate.

Methods: Utilizing the Optum Insight dataset, we examined all pediatric patients in a large nationwide healthcare network with an International Classification of Disease (ICD) diagnosis code of tethered cord between 2001 and 2014 (n=3,218). Using a combination of ICD and Current Procedural Terminology (CPT) codes, we identified iTCS diagnoses and untethering procedures. We examined demographic and clinical predictors of surgical intervention (n=482) as well as postoperative complications.

Results: The incidence of iTCS in children over the entire study period was similar in females and males with incidence rates of diagnosis of 13 and 11 cases per 100,000 insured patient-years, respectively. Factors that increased the odds for surgical untethering included living in the West census region (unadjusted OR 2.335 when compared to New England census region, p-value<0.001) and the presence of a syrinx (unadjusted OR 2.189, p-value<0.001). Of note, the presence of a Chiari malformation and gender did not affect the odds of undergoing surgery. In the surgical group, 3-6 year olds demonstrated the lowest odds of sustaining post-surgical complications (unadjusted OR 0.297 when compared to patients younger than 1 year of age, p-value=0.0174).

Conclusions: Analysis of a large nationwide healthcare network showed higher odds of untethering procedures for pediatric iTCS patients with a syrinx as well as significant regional variation. The 3-6 year age range was associated with lower surgical morbidity compared to older or younger patients.

Patient Care: If the results regarding the ideal age for surgery hold up in potential prospective trials, waiting until the safe age of 3 years old to offer a tethered cord release will decrease the complication rate for children undergoing this procedure.

Learning Objectives: By the conclusion of this session, participants should be able to: 1) Understand the significance of large-database analysis in studying rare diseases, 2) Identify predictors of surgery in children with isolated tethered cord syndrome, 3) Describe the potential ideal age for surgical untethering in patients with isolated tethered cord syndrome.

References: Tethered cord syndrome in children: a review CUONG J. BUI, M.D.,1 R. SHANE TUBBS, M.S., P.A.-C., PH.D.,1,2 W. JERRY OAKES, M.D.1 Neurosurg Focus 23 (2):E2, 2007