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Spinal fusion in pediatric patients with marfan syndrome: a nationwide assessment on short-term outcomes and readmission risk

European Spine Journal volume 30, pages 775–787 (2021)Cite this article

Abstract

Purpose

The purpose of this study was to utilize the National Readmission Database (NRD) to determine estimates for complication rates, 90-day readmission rates, and hospital costs associated with spinal fusion in pediatric patients with Marfan syndrome.

Methods

The 2012–2015 NRD databases were queried for all pediatric (< 19 years old) patients diagnosed with Marfan syndrome undergoing spinal fusion surgery. The primary outcome variables in this study were index admission complications and 90-day readmissions.

Results

A total of 249 patients with Marfan syndrome underwent spinal fusion surgery between 2012 and 2015 (mean age ± standard deviation at the time of surgery: 14 ± 2.0, 132 (53%) female). 25 (10.1%) were readmitted within 90 days of the index hospital discharge date. Overall, 59.7% of patients experienced at least one complication during the index admission. Unplanned 90-day readmission could be predicted by older age (odds ratio 2.3, 95% confidence interval 1.3–4.2, p = 0.006), Medicaid insurance status (56.0, 3.8–820.0, p = 0.003), and experiencing an inpatient medical complication (42.9, 4.6–398.7, p = 0.001). Patients were readmitted for wound dehiscence (8 patients, 3.2%), nervous system related complications (3 patients, 1.2%), and postoperative infectious related complications (4 patients, 1.6%).

Conclusion

This study is the first to demonstrate on a national level the complications and potential risk factors for 90-day hospital readmission for patients with Marfan syndrome undergoing spinal fusion. Patients with Marfan syndrome undergoing spinal fusion often present with multiple medical comorbidities that must be managed carefully perioperatively to reduce inpatient complications and early hospital readmissions.

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References

  1. Judge DP, Dietz HC (2005) Marfan’s syndrome. Lancet 12:1965–1976. https://doi.org/10.1016/S0140-6736(05)67789-6

    CAS  Article  Google Scholar 

  2. Pyeritz RE, McKusick VA (1979) The marfan syndrome: diagnosis and management. N Engl J Med 300:772–777. https://doi.org/10.1056/NEJM197904053001406

    CAS  Article  PubMed  Google Scholar 

  3. MagotteauxBulkFarhatSakalihasanDefraigneSeghaye SSNNJOMC (2016) Marfan syndrome in childhood, adolescence. Rev Med Liege 71(7–8):342–348

    Google Scholar 

  4. Sponseller PD, Hobbs W, Riley LH III, Pyeritz RE (1995) The thoracolumbar spine in marfan syndrome. J Bone Jt Surg Am. 77:867–76

    CAS  Article  Google Scholar 

  5. Keech MK, Wendt VE, Read RC, Bistue AR, Bianchi FA (1966) Family studies of the marfan syndrome. J Chronic Dis 19:57–58. https://doi.org/10.1016/0021-9681(66)90151-2

    CAS  Article  PubMed  Google Scholar 

  6. Birch JG, John AH (1987) Spinal deformity in Marfan syndrome. J Pediatr Orthop 7:546–552

    CAS  Article  Google Scholar 

  7. Robins PR, Moe JH, Winter RB (1975) Scoliosis in Marfan’s syndrome. Its characteristics and results of treatment in thirty-five patients. J Bone Jt Surg Am 57(3):358–368

    CAS  Article  Google Scholar 

  8. Jones KB, Erkula G, Sponseller PD, Dormans JP (2002a) Spine deformity correction in Marfan syndrome. Spine 27:2003–12

    Article  Google Scholar 

  9. Demetracopoulos CA, Sponseller PD (2007) Spinal deformities in Marfan syndrome. Orthop Clin North Am 38:563–572. https://doi.org/10.1016/j.ocl.2007.04.003

    Article  PubMed  Google Scholar 

  10. Zuckerman RB, Sheingold SH, Orav EJ, Ruhter J, Epstein AM (2016) Readmissions, observation, and the hospital readmissions reduction program. N Engl J Med 374:1543–1551. https://doi.org/10.1056/NEJMsa1513024

    CAS  Article  PubMed  Google Scholar 

  11. NRD Overview (n.d.) https://www.hcup-us.ahrq.gov/nrdoverview.jsp. Accessed 3 Feb 2020

  12. Passias PG, Poorman GW, Jalai CM, Neuman B, De La Garza-Ramos R, Miller E, Jain A, Sciubba D, McClelland S, Day L, Ramchandran S, Vira S, Diebo B, Isaacs E, Bono O, Bess S, Gerling M, Lafage V (2017) Morbidity of adult spinal deformity surgery in elderly has declined over time. Spine (Phila. Pa. 1976) 42:E978–E982. https://doi.org/10.1097/BRS.0000000000002009

    Article  Google Scholar 

  13. Lee NJ, Guzman JZ, Kim J, Skovrlj B, Martin CT, Pugely AJ, Gao Y, Caridi JM, Mendoza-Lattes S, Cho SK (2016) A Comparative Analysis Among the SRS M&M NIS, and KID Databases for the Adolescent Idiopathic Scoliosis. Spine Deform 4:420–424. https://doi.org/10.1016/j.jspd.2016.05.005

    CAS  Article  PubMed  Google Scholar 

  14. Roddy E, Diab M (2017a) Rates and risk factors associated with unplanned hospital readmission after fusion for pediatric spinal deformity. Spine J 17:369–379. https://doi.org/10.1016/j.spinee.2016.10.008

    Article  PubMed  Google Scholar 

  15. Jain A, Puvanesarajah V, Menga EN, Sponseller PD (2015) Unplanned Hospital Readmissions and Reoperations After Pediatric Spinal Fusion Surgery. Spine (Phila. Pa. 1976) 40:856–862. https://doi.org/10.1097/BRS.0000000000000857

    Article  Google Scholar 

  16. Gjolaj JP, Sponseller PD, Shah SA, Newton PO, Flynn JM, Neubauer PR, Marks MC, Bastrom TP (2012) Spinal deformity correction in Marfan syndrome versus adolescent idiopathic scoliosis: Learning from the differences. Spine (Phila. Pa. 1976) 37:1558–1565. https://doi.org/10.1097/BRS.0b013e3182541af3

    Article  Google Scholar 

  17. Li Z, Shen J, Qiu G, Yu H, Wang Y, Zhang J, Zhao H, Zhao Y, Li S, Weng X, Liang J, Zhao L (2014) Unplanned reoperation within 30 days of fusion surgery for spinal deformity. PLoS One 9:e87172. https://doi.org/10.1371/journal.pone.0087172

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  18. Golladay ES, Char FL, Mollitt DL (1985) Children with Marfan’s syndrome and pectus excavatum. South Med J 78(11):1319–23

    CAS  Article  Google Scholar 

  19. Arn PH, Scherer LR, Haller JAJ, Pyeritz RE (1989) Outcome of pectus excavatum in patients with Marfan syndrome and in the general population. J Pediatr 115:954–958

    CAS  Article  Google Scholar 

  20. Redlinger RE, Rushing GD, Moskowitz AD, Kelly RE, Nuss D, Kuhn A, Obermeyer RJ, Goretsky MJ (2010) Minimally invasive repair of pectus excavatum in patients with Marfan syndrome and marfanoid features. J Pediatr Surg 45:193–199. https://doi.org/10.1016/j.jpedsurg.2009.10.037

    Article  PubMed  Google Scholar 

  21. Schairer WW, Carrer A, Deviren V, Hu SS, Takemoto S, Mummaneni P, Chou D, Ames C, Burch S, Tay B, Sawyer A, Berven SH (2013) Hospital readmission after spine fusion for adultspinal deformity. Spine (Phila. Pa. 1976) 38:1681–1689. https://doi.org/10.1097/BRS.0b013e31829c08c9

    Article  Google Scholar 

  22. Roddy E, Diab M (2017b) Rates and risk factors associated with unplanned hospital readmission after fusion for pediatric spinal deformity. Spine J 17:369–379. https://doi.org/10.1016/j.spinee.2016.10.008

    Article  PubMed  Google Scholar 

  23. Jones KB, Erkula G, Sponseller PD, Dormans JP (2002b) Spine deformity correction in Marfan syndrome. Spine 27:2003

    Article  Google Scholar 

  24. Paul JC, Lonner BS, Vira S, Feldman D, Errico TJ (2018) Does reoperation risk vary for different types of pediatric scoliosis? J Pediatr Orthop 38:459–464. https://doi.org/10.1097/BPO.0000000000000850

    Article  PubMed  Google Scholar 

  25. Gott VL, Cameron DE, Alejo DE, Greene PS, Shake JG, Caparrelli DJ, Dietz HC (2002) Aortic root replacement in 271 Marfan patients: a 24-year experience. Ann Thorac Surg 73:438–443. https://doi.org/10.1016/S0003-4975(01)03336-7

    Article  PubMed  Google Scholar 

  26. Thaku NA, Czerwein JK, Butera JN, Palumbo MA (2010) Perioperative Management of Chronic Anticoagulation in Ortho. JAAOS J Am Acad Orthop Surg 18:729–738

    Article  Google Scholar 

  27. Biteker M, Tekkeşin AI, Can MM, Dayan A, Ilhan E, Türkmen FM (2012) Outcome of noncardiac and nonvascular surgery in patients with mechanical heart valves. Am J Cardiol 110:562–567. https://doi.org/10.1016/j.amjcard.2012.04.031

    Article  PubMed  Google Scholar 

  28. Streeten EA, Murphy EA, Pyeritz RE (1987) Pulmonary function in the Marfan syndrome. Chest 91:408–412. https://doi.org/10.1378/chest.91.3.408

    CAS  Article  PubMed  Google Scholar 

  29. Wood JR, Bellamy D (1984) Pulmonary disease in patients with Marfan syndrome. Thorax 39:780–784. https://doi.org/10.1136/thx.39.10.780

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  30. Quigley PM, Haller JA, Jelus KL, Laughlin GM, Marcus CL (1996) Cardiorespiratory function before and after corrective surgery in pectus excavatum. J Pediatr 128:638–643. https://doi.org/10.1016/S0022-3476(96)80128-4

    CAS  Article  PubMed  Google Scholar 

  31. Karpman C, Aughenbaugh GL, Ryu JH (2011) Pneumothorax and bullae in Marfan syndrome. Respiration 82:219–224. https://doi.org/10.1159/000322958

    Article  PubMed  Google Scholar 

  32. Lawrence VA, Cornell JE, Smetana GW (2006) Strategies to reduce postoperative pulmonary complications after noncardiothoracic surgery: systematic review for the American college of physicians. Ann Intern Med 144:596–608. https://doi.org/10.7326/0003-4819-144-8-200604180-00011

    Article  PubMed  Google Scholar 

  33. Giampietro PF, Peterson M, Schneider R, Davis JG, Raggio C, Myers E, Burke SW, Boachie-Adjei O, Mueller CM (2003) Assessment of bone mineral density in adults and children with Marfan syndrome. Osteoporos Int 14:559–563. https://doi.org/10.1007/s00198-003-1433-0

    Article  PubMed  Google Scholar 

  34. Kohlmeier L, Gasner C, Bachrach LK, Marcus R (2009) The bone mineral status of patients with marfan syndrome. J Bone Miner Res 10:1550–1555. https://doi.org/10.1002/jbmr.5650101017

    Article  Google Scholar 

  35. Vitale MA, Arons RR, Hyman JE, Skaggs DL, Roye DP, Vitale MG (2005) The contribution of hospital volume, payer status, and other factors on the surgical outcomes of scoliosis patients: a review of 3,606 cases in the State of California. J Pediatr Orthop 25(3):393–399

    Article  Google Scholar 

  36. Boylan MR, Riesgo AM, Chu A, Paulino CB, Feldman DS (2019) Costs and complications of increased length of stay following adolescent idiopathic scoliosis surgery. J Pediatr Orthop Part B 28:27–31. https://doi.org/10.1097/BPB.0000000000000543

    Article  Google Scholar 

  37. Imahiyerobo T, Minkara AA, Matsumoto H, Vitale MG (2018) Plastic multilayered closure in pediatric nonidiopathic scoliosis is associated with a lower than expected incidence of wound complications and surgical site infections. Spine Deform 6:454–459. https://doi.org/10.1016/j.jspd.2017.12.009

    Article  PubMed  Google Scholar 

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Funding

This work was conducted without the support of additional funding.

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Affiliations

  1. Department of Orthopaedics, Columbia University Medical Center, The Och Spine Hospital at New York-Presbyterian, 161 Fort Washington Avenue, New York, NY, USA

    Michael W. Fields, Nathan J. Lee, Jacob R. Ball, Venkat Boddapati, Justin Mathew, Daniel Hong, Josephine R. Coury, Zeeshan M. Sardar & Lawrence G. Lenke

  2. Department of Pediatric Orthopedics, Columbia University Medical Center, Morgan Stanley Children’s Hospital at New York-Presbyterian, New York, NY, USA

    Benjamin Roye & Michael Vitale

Authors
  1. Michael W. Fields
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  2. Nathan J. Lee
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Contributions

MWF, NJL, JRB, VB, JM, DH, JRC, ZMS, BR, MV: Substantial contributions to the conception or design of the work; or the acquisition, analysis, or interpretation of data for the work; Drafting the work or revising it critically for important intellectual content; Final approval of the version to be published.

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Correspondence to Michael W. Fields.

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Appendix

Appendix

See Table 6.

Table 6 ICD9 diagnosis and procedure description and codes
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Fields, M.W., Lee, N.J., Ball, J.R. et al. Spinal fusion in pediatric patients with marfan syndrome: a nationwide assessment on short-term outcomes and readmission risk. Eur Spine J 30, 775–787 (2021). https://doi.org/10.1007/s00586-020-06645-8

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  • DOI: https://doi.org/10.1007/s00586-020-06645-8

Keywords

  • Marfan syndrome
  • Scoliosis
  • Spinal fusion
  • Readmission
  • Complicaitons