Journal of Pediatric Orthopaedics - 2026-09-18 - Journal Article
Spinal Fusion in Prader-Willi Syndrome: Early Radiographic Outcomes and Complications With a Subset Analysis of Two-year Follow-up in a Multicenter Cohort.
Lobato AG, Andras L, Vaughan M, Ramo BA, McQuerry JL, Huser AJ, Feldman DS, Hariharan AR, Pediatric Spine Study Group
Topics
Key Takeaway
Spinal fusion in Prader-Willi syndrome achieves 55.8% major Cobb angle correction (76.8° to 35.4°) with a 21.7% complication rate and 17.4% unplanned return to OR, with higher BMI independently associated with more complications (P=0.03).
Summary Depth
Choose how much analysis to show on this article page.
Summary
This multicenter registry study examined radiographic outcomes, complications, and UPROR after spinal fusion in 23 PWS patients (mean age 11.9 years). Major Cobb angle improved 55.8% (76.8° to 35.4°, P<0.001) and kyphosis improved 22.7% (69.5° to 48.4°, P=0.001), both maintained at final follow-up. Nine complications occurred in 5 patients (21.7%), 6 UPROR in 4 patients (17.4%) at median 41 days postoperatively, and higher BMI predicted greater complication burden on negative binomial regression (P=0.03).
Key Limitation
The sample size of 23 patients with mean follow-up of only 1.5 years is insufficient to characterize long-term implant durability, pseudarthrosis rates, or curve progression in this syndromic population.
Original Abstract
BACKGROUND
Prader-Willi syndrome (PWS) is a rare genetic disorder characterized by hypotonia, obesity, and endocrine dysfunction. Scoliosis affects over 70% of patients and frequently requires surgical treatment. Despite recognition of PWS as a high-risk syndromic population, contemporary multicenter data describing radiographic outcomes, complications, and unplanned return to the operating room (UPROR) after spinal fusion remain limited.
METHODS
Prospectively collected multicenter registry data were retrospectively reviewed for patients with PWS who underwent spinal fusion. Variables included demographics, body mass index (BMI), age at surgery, prior treatments, growth hormone therapy, radiographic parameters, EOSQ-24 outcomes, perioperative complications, and UPROR. Analyses were primarily descriptive, with paired statistical comparisons limited to complete data pairs. Associations between BMI and complications or reoperations were assessed with negative binomial regression.
RESULTS
Twenty-three patients were included, with a mean age at surgery of 11.9±2.5 years and a mean follow-up of 1.5±1.8 years. A subset of 10 patients with >2 years follow-up (mean 3.0±1.4 y) was analyzed for durability. Among 21 patients, 76.2% had prior treatment (bracing, halo, casting), and 61.9% used growth hormone therapy. Mean major Cobb angle improved from 76.8±21.1 degrees preoperatively to 35.4±16.3 degrees postoperatively (55.8% improvement, P<0.001), with maintenance at final follow-up. Mean kyphosis improved from 69.5±27.6 degrees to 48.4±17.9 degrees postoperatively (22.7% improvement, P=0.001) and remained stable. Nine complications occurred in 5 patients (21.7%), with 6 UPROR in 4 patients (17.4%), at a median of 41 days postoperatively (IQR 0 to 484). Higher BMI was associated with more complications (P=0.03). EOSQ-24 data (n=10) demonstrated the greatest improvements in satisfaction and pulmonary function.
CONCLUSION
Spinal fusion in patients with PWS provides durable deformity correction with early functional improvement, but carries a higher risk of perioperative complications and early UPROR. The combined impact of sagittal deformity and obesity highlights the need for risk-focused counseling and careful perioperative planning in this population.
LEVEL OF EVIDENCE
Level IV-case series.