[Long-Term Pulmonary Function after Combined Anteroposterior Spinal Fusion for Scoliosis in Neurofibromatosis Type 1 and Marfan Syndrome at a Mean Follow-Up of 13 Years].

Objective: To compare the effect of combined spinal fusion (anteroposterior) on pulmonary function in patients with scoliosis secondary to Marfan syndrome versus neurofibromatosis type 1 (NF1) at long-term follow-up (> 10 years).

Methods: Retrospective comparative study with nine patients, operated from March 1997 to December 2009, groups: Marfan syndrome versus NF1. Outcome measures were sex; age (at diagnosis and surgery); corrected height by wingspan; body mass index (BMI); duration of surgery (minutes); estimated blood loss (mL); last follow-up (years); pulmonary and implants related complications; pre- and postoperative Cobb angle of main thoracic curve and of thoracic kyphosis (T5 to T12); number of instrumented levels; absolute and percentage predicted values of forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1). The data was processed in the IBM SPSS Statistics for Windows software (IBM Corp.), version 20.0, and the comparisons of means used the Student's -test and analysis of variance (ANOVA), or the Mann-Whitney and Kruskal-Wallis/Dunn tests, with a -value of 0.05.

Results: There was no difference in the absolute and predicted percentage values of pulmonary function, FVC and FEV1, and Cobb of the main thoracic curve between the groups, pre- and postoperatively (  > 0.05). However, there was a significant reduction in the Cobb of the main thoracic curve in the Marfan syndrome group (74→46°,  < 0.05).

Conclusion: There was no worsening of pulmonary function in patients who underwent the combined approach after a follow-up of more than 10 years, and there were no significant differences in the postoperative values of the pulmonary function test between the groups.Level of evidence IV; case series.