Abstract
Purpose
Outcomes after spinal deformity surgery in patients with Marfan syndrome (MFS) are poorly characterized given the rarity of the condition. Updated analyses from nationally representative samples, and comparison to outcomes after more commonly performed procedures for conditions such as adolescent idiopathic scoliosis (AIS) could help define the relative risks.
Methods
Using the 2010–2020 PearlDiver administrative databases, patients who underwent posterior spinal fusion for > 7 segments were extracted. MFS patients were matched 1:4 to AIS patients based on age, sex, and Elixhauer comorbidity index (ECI). Ninety-day outcomes and 5-year reoperation rates were compared. Significance was set at p < 0.05.
Results
In total, 206 MFS patients were matched to 825 AIS patients. After adjusting for age, sex, and ECI, multivariate odds ratios (OR) for 90-day any, serious, and minor adverse events, as well as readmissions, were not significantly different for those with MFS compared to those with AIS (p > 0.05 for each). Five-year reoperation rates were also not significantly different (13.1% for the MFS cohort and 13.0% for the AIS cohort (no difference by log-rank, p = 0.9).
Conclusion
While deformity surgery is much less commonly performed for MFS than AIS, it is not uncommonly considered for patients with this condition. Despite some known technical challenges for MFS deformity surgery, the current study leveraged a large, national database to find that 90-day adverse events and 5-year reoperations were not different for matched MFS and AIS patients undergoing deformity surgery. For select patients, these findings should be useful for surgical planning and patient counseling.
Similar content being viewed by others
Availability of data and material
All data is available from the national insurance claims database, PearlDiver MSpine.
References
Vanem TT, Böker T, Sandvik GF et al (2020) Marfan syndrome: evolving organ manifestations—a 10-year follow-up study. Am J Med Genet A 182(2):397–408
Hayward C, Brock DJ (1997) Fibrillin-1 mutations in Marfan syndrome and other type-1 fibrillinopathies. Hum Mutat 10(6):415–423
Shirley ED, Sponseller PD (2009) Marfan syndrome. J Am Acad Orthop Surg 17(9):572–581
Sponseller PD, Bhimani M, Solacoff D, Dormans JP (2000) Results of brace treatment of scoliosis in Marfan syndrome. Spine (Phila Pa 1976) 25(18):2350–2354
Sponseller PD, Hobbs W, Riley LH 3rd, Pyeritz RE (1995) The thoracolumbar spine in Marfan syndrome. J Bone Jt Surg Am 77(6):867–876
Böker T, Vanem TT, Pripp AH et al (2019) Dural ectasia in Marfan syndrome and other hereditary connective tissue disorders: a 10-year follow-up study. Spine J 19(8):1412–1421
Lundby R, Rand-Hendriksen S, Hald JK et al (2009) Dural ectasia in Marfan syndrome: a case control study. Am J Neuroradiol 30(8):1534–1540
Fattori R, Nienaber CA, Descovich B et al (1999) Importance of dural ectasia in phenotypic assessment of Marfan’s syndrome. Lancet 354(9182):910–913
Demetracopoulos CA, Sponseller PD (2007) Spinal deformities in Marfan syndrome. Orthop Clin N Am 38(4):563–72, vii
Roman MJ, Rosen SE, Kramer-Fox R, Devereux RB (1993) Prognostic significance of the pattern of aortic root dilation in the Marfan syndrome. J Am Coll Cardiol 22(5):1470–1476
Liang W, Yu B, Wang Y et al (2015) Comparison of posterior correction results between Marfan syndrome scoliosis and adolescent idiopathic scoliosis-a retrospective case-series study. J Orthop Surg Res 10:73
Di Silvestre M, Greggi T, Giacomini S et al (2005) Surgical treatment for scoliosis in Marfan syndrome. Spine (Phila Pa 1976) 30(20):E597-604
Fields MW, Lee NJ, Ball JR et al (2021) Spinal fusion in pediatric patients with marfan syndrome: a nationwide assessment on short-term outcomes and readmission risk. Eur Spine J 30(3):775–787
Kurucan E, Bernstein DN, Ying M et al (2019) Trends in spinal deformity surgery in Marfan syndrome. Spine J 19(12):1934–1940
Linden A, Samuels SJ (2013) Using balance statistics to determine the optimal number of controls in matching studies. J Eval Clin Pract 19(5):968–975
Galivanche AR, Gala R, Bagi PS et al (2020) Perioperative outcomes in 17,947 patients undergoing 2-level anterior cervical discectomy and fusion versus 1-level anterior cervical corpectomy for treatment of cervical degenerative conditions: a propensity score matched national surgical quality improvement program analysis. Neurospine 17(4):871–878
Lonner BS, Ren Y, Yaszay B et al (2018) Evolution of surgery for adolescent idiopathic scoliosis over 20 years: have outcomes improved? Spine (Phila Pa 1976) 43(6):402–410
Lee NJ, Fields MW, Boddapati V et al (2020) The risks, reasons, and costs for 30- and 90-day readmissions after fusion surgery for adolescent idiopathic scoliosis. J Neurosurg Spine. https://doi.org/10.3171/2020.6.SPINE20197
Sabatino MJ, Burroughs PJ, Moore HG, Grauer JN (2020) Spine coding transition from ICD-9 to ICD-10: not taking advantage of the specificity of a more granular system. NASSJ. 4:100035
Funding
No funds, grants, or other support were received for this study.
Author information
Authors and Affiliations
Contributions
All authors meet the required criteria for authorship. PYJ: Material preparation, data analysis, writing-original draft preparation, edited manuscript drafts, approval of final version of manuscript, agree to be accountable for the work. DLC: Conceptualization, methodology, writing-original draft preparation, edited manuscript draft preparation, approval of final version of manuscript, agree to be accountable for the work. MJG: Conceptualization, methodology, writing-original draft preparation, edited manuscript draft preparation, approval of final version of manuscript, agree to be accountable for the work. HGM: Conceptualization, methodology, writing-original draft preparation, edited manuscript draft preparation, approval of final version of manuscript, agree to be accountable for the work. JRZ: Conceptualization, methodology, writing-original draft preparation, edited manuscript draft preparation, approval of final version of manuscript, agree to be accountable for the work. BA: Edited manuscript draft preparation, approval of final version of manuscript, agree to be accountable for the work. JNG: Conceptualization, methodology, edited manuscript draft preparation, approval of final version of manuscript, agree to be accountable for the work, supervision.
Corresponding author
Ethics declarations
Conflicts of interest
The authors declare they have no financial interests. Author JG is on the board of directors of NASS and LSRS and receives no compensation as member of the board of directors.
Code availability
All codes used were either from pre-existing codes from the PearlDiver software or custom-made directly in the software. Codes are available on request.
Ethics approval
This study was deemed exempt from Institutional Review Board review.
Informed consent
Informed consent was not required as the data analyzed was from a deidentified database.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Joo, P.Y., Caruana, D.L., Gouzoulis, M.J. et al. Marfan syndrome and adolescent idiopathic scoliosis patients have similar 90-day postoperative outcomes and 5-year reoperation rates after spinal deformity surgery. Spine Deform 10, 1169–1174 (2022). https://doi.org/10.1007/s43390-022-00501-z
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s43390-022-00501-z