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Growth-friendly Spinal Instrumentation in Marfan Syndrome Achieves Sustained Gains in Thoracic Height Amidst High Rates of Implant Failure.

Publication ,  Journal Article
Bellaire, LL; Zhang, C; Smith, JT; Heflin, JA; Klatt, J; Roye, D; Sponseller, P; Samdani, A; Fedorak, GT; Pediatric Spine Study Group,
Published in: J Pediatr Orthop
March 1, 2021

BACKGROUND: There are few reports on the surgical management of early-onset scoliosis (EOS) associated with Marfan syndrome (MFS). Affected patients tend to have more rapid curve progression than those with idiopathic EOS, and their course is further complicated by medical comorbidities. As surgical techniques and implants for growing spines become more widely applied, this study seeks to better delineate the safety and efficacy of growth-friendly spinal instrumentation in treating this population. METHODS: A prospective registry of children treated for EOS was queried for MFS patients treated between 1996 and 2016. Forty-two patients underwent rib-based or spine-based growing instrumentation and were assessed on preoperative, surgical, and postoperative clinical and radiographic parameters including complications and reoperations. Subgroup analysis was performed based on spine-based versus rib-based fixation. RESULTS: Patients underwent their index surgery at a mean age of 5.5 years, when the major coronal curve and kyphosis measured 77 and 50 degrees, respectively. Over half were treated with traditional growing rods. Patients underwent 7.2 total surgical procedures-4.7 lengthening and 1.9 revision surgeries not including conversion to fusion-over a follow-up of 6.5 (±4.1) years. Radiographic correction was greatest at index surgery but maintained over time, with a final thoracic height measuring 23.8 cm. Patients experienced a mean of 2.6 complications over the course of the study period; however, a small group of 6 patients experienced ≥6 complications while over half of patients experienced 0 or 1. Implant failures represented 42% of all complications with infection and pulmonary complications following. CONCLUSIONS: This is the largest report on patients with EOS and MFS. All subtypes of growth-friendly constructs reduced curve progression in this cohort, but complications and reoperations were nearly universal; patients were particularly plagued by implant failure and migration. Further collaborations are needed to enhance understanding of optimal timing and fixation constructs for those with MFS and other connective tissue diseases.

Duke Scholars

Published In

J Pediatr Orthop

DOI

EISSN

1539-2570

Publication Date

March 1, 2021

Volume

41

Issue

3

Start / End Page

e204 / e210

Location

United States

Related Subject Headings

  • Treatment Outcome
  • Thoracic Vertebrae
  • Spinal Fusion
  • Scoliosis
  • Retrospective Studies
  • Reoperation
  • Prostheses and Implants
  • Orthopedics
  • Orthopedic Procedures
  • Marfan Syndrome
 

Citation

APA
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ICMJE
MLA
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Bellaire, L. L., Zhang, C., Smith, J. T., Heflin, J. A., Klatt, J., Roye, D., … Pediatric Spine Study Group, . (2021). Growth-friendly Spinal Instrumentation in Marfan Syndrome Achieves Sustained Gains in Thoracic Height Amidst High Rates of Implant Failure. J Pediatr Orthop, 41(3), e204–e210. https://doi.org/10.1097/BPO.0000000000001730
Bellaire, Laura L., Chong Zhang, John T. Smith, John A. Heflin, Joshua Klatt, David Roye, Paul Sponseller, Amer Samdani, Graham T. Fedorak, and Graham T. Pediatric Spine Study Group. “Growth-friendly Spinal Instrumentation in Marfan Syndrome Achieves Sustained Gains in Thoracic Height Amidst High Rates of Implant Failure.J Pediatr Orthop 41, no. 3 (March 1, 2021): e204–10. https://doi.org/10.1097/BPO.0000000000001730.
Bellaire LL, Zhang C, Smith JT, Heflin JA, Klatt J, Roye D, et al. Growth-friendly Spinal Instrumentation in Marfan Syndrome Achieves Sustained Gains in Thoracic Height Amidst High Rates of Implant Failure. J Pediatr Orthop. 2021 Mar 1;41(3):e204–10.
Bellaire, Laura L., et al. “Growth-friendly Spinal Instrumentation in Marfan Syndrome Achieves Sustained Gains in Thoracic Height Amidst High Rates of Implant Failure.J Pediatr Orthop, vol. 41, no. 3, Mar. 2021, pp. e204–10. Pubmed, doi:10.1097/BPO.0000000000001730.
Bellaire LL, Zhang C, Smith JT, Heflin JA, Klatt J, Roye D, Sponseller P, Samdani A, Fedorak GT, Pediatric Spine Study Group. Growth-friendly Spinal Instrumentation in Marfan Syndrome Achieves Sustained Gains in Thoracic Height Amidst High Rates of Implant Failure. J Pediatr Orthop. 2021 Mar 1;41(3):e204–e210.

Published In

J Pediatr Orthop

DOI

EISSN

1539-2570

Publication Date

March 1, 2021

Volume

41

Issue

3

Start / End Page

e204 / e210

Location

United States

Related Subject Headings

  • Treatment Outcome
  • Thoracic Vertebrae
  • Spinal Fusion
  • Scoliosis
  • Retrospective Studies
  • Reoperation
  • Prostheses and Implants
  • Orthopedics
  • Orthopedic Procedures
  • Marfan Syndrome