One is not like the other: health quality of life scores vary in neuromuscular EOS by diagnosis.

Abstract

Purpose: The etiology of early-onset scoliosis (EOS) has been shown to significantly influence baseline parent-reported health-related quality of life (HrQOL). In combining these etiology groups, we obligatorily lump together many disparate diagnoses, particularly true in the neuromuscular (NM) cohort. We sought to evaluate the influence of underlying neuromuscular diagnosis on the HrQOL at 5 years following surgery for EOS.

Methods: A retrospective review of a multi-center EOS database was performed. Children treated with primary distraction-based, growth-friendly instrumentation (GFI) for EOS with complete baseline, 2-year, and 5-year post-surgical EOSQs were included. Neuromuscular scoliosis patients, as classified by the C-EOS system, were isolated and subdivided by underlying diagnosis into 5 groups. EOSQ domains and composite HrQOL score at presentation, 2-year, and 5-year follow-up were compared across underlying diagnosis.

Results: A total of 65 neuromuscular EOS patients were identified (mean 7.6 ± 1.99 years of age, 50% female). Cerebral palsy was the most common underlying diagnosis (30%, N = 18), followed by spinal muscular atrophy (SMA, N = 16). There were differences in EOSQ domains with CP, SMA, and MD having significantly lower scores than Chiari/Syrinx patients at 2-year follow-up. Chiari/Syrinx patients demonstrated EOSQ scores statistically similar to idiopathic EOS patients at all time points (P > 0.05). CP patients were most likely to experience improvement in HrQOL at 5-year follow-up.

Conclusion: Underlying NM diagnosis has direct implications on treatment response following GFI for EOS. Cerebral palsy patients demonstrate the best improvement in HrQOL at 5 years following surgery while others actually deteriorate over time. Children with EOS related to Chiari and Syringomyelia had similar HrQOL scores to idiopathic EOS and may not be best suited for inclusion in NM cohorts when assessing HrQOL scores following treatment. Increasing population-based HrQOL data may allow further refinement and prognostication of neuromuscular diagnoses over time.