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Spinal Deformity in Marfan Syndrome

Spinal Deformity Correction in Marfan Syndrome Versus Adolescent Idiopathic Scoliosis: Learning From the Differences

Gjolaj, Joseph P. MD*; Sponseller, Paul D. MD*; Shah, Suken A. MD; Newton, Peter O. MD; Flynn, John M. MD§; Neubauer, Philip R. MD*; Marks, Michelle C. MA, PT; Bastrom, Tracey P. MA‡,¶

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.

 

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Spine: 15 August 2012 – Volume 37 – Issue 18 – p 1558–1565