Postoperative pneumothorax occurrence in patients with Marfan syndrome

Marfan syndrome is a congenital connective tissue disorder frequently complicated by pneumothorax. However, the long-term efficacy of surgical intervention in these cases remains underreported. This study aimed to assess the surgical outcomes of pneumothorax associated with Marfan syndrome.

A retrospective review was conducted at a single center, including patients diagnosed with secondary pneumothorax associated with Marfan syndrome who underwent surgery between 2004 and 2019. Postoperative pneumothorax recurrence rates and potential predictors of postoperative recurrence were assessed. The incidence of ipsilateral pneumothorax after surgery was analyzed and compared with that before surgery.

Overall, 20 patients (60% male, 40% female) with a median age of 18.5 years (range 13-40 years) were included in the analysis. Seventeen patients underwent bullectomy, while the remainder received pulmorrhaphy. The cumulative incidence of postoperative pneumothorax at 5 and 10 years was 25% and 44%, respectively. Notably, patients with pectus excavatum had a 5-year cumulative recurrence rate of 25%, and those with a flat chest had a rate of 60%, compared with 9.1% for patients without chest deformities (P = 0.017). Chest deformity emerged as a significant predictor of postoperative pneumothorax recurrence (hazard ratio 7.63; 95% confidence interval 1.29-45.1; P = 0.025). The frequency of ipsilateral pneumothorax significantly decreased postoperatively, from 1.09 ± 0.66 episodes/year (mean ± SD) pre-surgery to 0.04 ± 0.10 episodes/year post-surgery (P < 0.001).

Surgical intervention is an effective treatment for pneumothorax in patients with Marfan syndrome. Chest deformity may serve as a predictor of postoperative pneumothorax recurrence in this patient population.