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040. Incidence of Tethered Spinal Cord in Neurofibromatosis Type I and II Pediatric Patients: A Population-Level Analysis

Authors: Debarati Bhanja

Introduction:
Tethered spinal cord (TC) is characterized by cutaneous attachments on the filum terminale that stretches the spinal cord, resulting in painful musculoskeletal sequelae. While neurocutaneous associations with TC have not been defined, high incidence of TC among a pediatric neurofibromatosis (NF) cohort has recently been reported. The objective is to utilize a national healthcare database to estimate the incidence of TC among NF type 1 and 2 (NF1, NF2) pediatric patients at the population-level as compared to the general pediatric population.Methods: TriNetX Research Network was queried to identify patients diagnosed with NF and/or TC before age 21. Symptomatic TCs requiring surgical intervention were identified with a corresponding CPT code within 12 months following TC diagnosis. Odds ratios were calculated to measure associations of NF1 and NF2 to TC.
Results:
12,417 pediatric NF patients were evaluated, including 11,769 (94.7%) with NF1 and 648 (5.3%) with NF2. Average ages of TC diagnosis among NF1, NF2 and non-NF patients were 11.4, 14.2, and 8.9 years, respectively. 2.17% (255) of patients with NF1 and 10.49% (68) of patients with NF2 developed TC before age 21. Odds ratios of TC incidence were significantly increased in NF1 (OR 13.15; 95% CI,?11.61-14.90) and NF2 (OR 69.63; 95% CI, 54.14-89.54) patients as compared to the general population. Symptomatic TCs requiring surgical intervention were significantly associated with NF2 patients compared to the general population (OR 3.3502; 95% CI, 2.01-5.58), but not with NF1.
Conclusion:
The analysis demonstrates a high incidence but a delayed intervention of TC in pediatric NF patients. Though the related pathophysiology is currently unclear, TC counseling, spinal MRI, and earlier intervention may be warranted for pediatric NF patients experiencing musculoskeletal symptomatology.