Spinal deformity correction in Marfan syndrome versus adolescent idiopathic scoliosis: learning from the differences

Spine (Phila Pa 1976). 2012 Aug 15;37(18):1558-65. doi: 10.1097/BRS.0b013e3182541af3.


Study design: Retrospective case control study.

Objective: To compare patients with Marfan syndrome and matched patients with adolescent idiopathic scoliosis (AIS) to illustrate the differences and identify areas for potential surgical improvement.

Summary of background data: Patients with Marfan syndrome commonly require spinal deformity surgery, but practice guidelines and results are not as established as those for patients with AIS.

Methods: We matched 34 adolescents with Marfan syndrome with patients with AIS (ratio, 1:2) for age, sex, and degree of major deformity. Overall mean age was 14 ± 2 years and mean curves were 51° thoracic and 46° lumbar. Mean follow-up was 5.3 and 3.6 years, respectively.

Results: The Marfan syndrome group had significantly more thoracolumbar kyphosis correction (9.5° vs. 0.1°, P = 0.05), significantly more levels fused (12 ± 2 vs. 9 ± 3, P ≤ 0.01), significantly more fusions to the pelvis (7 vs. 0, P = 0.01), and significantly more correction of sagittal imbalance (2.4 vs. -0.6 cm, P = 0.035). The Marfan syndrome group also had more intraoperative cerebrospinal fluid leaks (3 vs. 0, P = 0.01), significantly more instrumentation complications (3 vs. 1, P = 0.007), more reoperations for indications [such as fixation failure, distal degeneration, and spine fracture (9 vs. 0, P = 0.01)], and lower SRS-22 total (3.9 vs. 4.5, P = 0.01) and partial (P < 0.015) subscores. There were no significant differences between the groups in progression of unfused proximal thoracic curves, blood loss, neurological deficit, hospital stay, percent correction, or infection rate.

Conclusion: Patients with Marfan syndrome differ in several ways from those with AIS: they require more levels of surgical correction, more distal fusion, greater correction of sagittal balance, and more reoperations, and they have more intraoperative cerebrospinal fluid leaks and instrumentation-related complications. Knowledge of these differences is important for planning surgery.

Publication types

  • Comparative Study
  • Multicenter Study

MeSH terms

  • Adolescent
  • Case-Control Studies
  • Child
  • Female
  • Follow-Up Studies
  • Humans
  • Kyphosis / surgery
  • Lumbar Vertebrae / surgery*
  • Male
  • Marfan Syndrome / surgery*
  • Reoperation
  • Retrospective Studies
  • Scoliosis / surgery*
  • Spinal Fusion / instrumentation
  • Spinal Fusion / methods*
  • Thoracic Vertebrae / surgery*
  • Treatment Outcome
  • Young Adult