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

Joseph P. Gjolaj, Paul D. Sponseller, Suken A. Shah, Peter O. Newton, John M. Flynn, Philip R. Neubauer, Michelle C. Marks, Tracey P. Bastrom

Research output: Contribution to journalArticle

26 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1558-1565
Number of pages8
JournalSPINE
Volume37
Issue number18
DOIs
StatePublished - Aug 15 2012
Externally publishedYes

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Keywords

  • Marfan syndrome
  • complications
  • idiopathic scoliosis
  • spinal instrumentation

ASJC Scopus subject areas

  • Orthopedics and Sports Medicine
  • Clinical Neurology

Cite this

Gjolaj, J. P., Sponseller, P. D., Shah, S. A., Newton, P. O., Flynn, J. M., Neubauer, P. R., Marks, M. C., & Bastrom, T. P. (2012). Spinal deformity correction in Marfan syndrome versus adolescent idiopathic scoliosis: Learning from the differences. SPINE, 37(18), 1558-1565. https://doi.org/10.1097/BRS.0b013e3182541af3