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

TL;DR

This study compares long-term lung function after spinal surgery for scoliosis in patients with Marfan syndrome and neurofibromatosis type 1.

Contribution

It provides long-term follow-up data on pulmonary outcomes after combined spinal fusion in rare genetic conditions.

Findings

No significant differences in pulmonary function were found between the two groups.

Marfan syndrome patients showed significant improvement in thoracic curve correction.

Pulmonary function remained stable over 10 years post-surgery.

Abstract

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).

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
t
-test and analysis of variance (ANOVA), or the Mann-Whitney and Kruskal-Wallis/Dunn tests, with a
p
-value of 0.05.

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 (
p
 > 0.05). However, there was a significant reduction in the Cobb of the main thoracic curve in the Marfan syndrome group (74→46°,
p
 < 0.05).

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.