Mid- to long-term radiological and respiratory outcomes following surgery for older early-onset scoliosis patients with Marfan syndrome

Abstract

Abstract This study aimed to elucidate mid- to long-term radiological and respiratory outcomes in patients aged 7–11 years at index surgery with Marfan syndrome and early-onset scoliosis (EOS) in a retrospective multicenter study. Primary outcomes were final thoracic height and final percentage of predicted vital capacity (%VC) at or after 16 years of age. We identified 21 (6 male and 15 female) patients with a mean age of 9.9 years and mean follow-up period of 149.3 months. Fifteen patients underwent primary fusion, whereas six underwent growth-friendly surgery (GFS). The mean preoperative and final T1–T12 heights were 204.0 mm and 248.0 mm, respectively. Final pulmonary function tests were available for 16 patients, and the mean final %VC was 54.0% with 10 patients exhibiting a final %VC < 60%. A significant moderate association was observed between the final T1–T12 height and final %VC. The predicted final T1–T12 height required for a final %VC of 60% was approximately 260 mm. Although most older patients with Marfan syndrome and EOS acquired a considerably large final T1–T12 height, a larger thoracic height was required for satisfactory respiratory function in many cases; hence, GFS may be indicated even in this population.

Keywords

• Marfan syndrome
• Early-onset scoliosis
• Thoracic height
• Respiratory function
• T1–T12 height