| 제목 |
Analysis of Pneumothorax in Non-invasive Ventilator Users with Duchenne Muscular Dystrophy |
| 소속 |
Gangnam Severance Hospital , Department of Rehabilitation Medicine1, Yonsei University College of Medicine, Rehabilitation Institute of Neuromuscular Disease2 |
| 저자 |
Han Eol Cho1,2*, Justin Byun1,2, Won Ah Choi1,2, Myungsang Kim1,2, Kyeong Yeol Kim1,2, Seong-Woong Kang1,2† |
Background
With the advancement of cardio-respiratory interventions, the survival rate among patients with Duchenne muscular dystrophy has increased. Subsequently, pneumothorax has become a significant problem in patients with prolonged ventilatory support. We aimed to investigate the frequency, recurrence rate, risk factors, and prognosis of pneumothorax in patients with Duchenne muscular dystrophy under non-invasive ventilation.
Method
This retrospective longitudinal cohort study included 176 patients between 2006 and 2019. We collected information regarding location, severity, treatment methods, recurrence frequency, risk factors in computed tomography, spirometric values at non-invasive ventilation application, and date of death. We compared the pneumothorax and non-pneumothorax groups and the recurrence and non-recurrence groups. We calculated the estimated survival probabilities from the age at non-invasive ventilation application according to pneumothorax occurrence.
Results
Sixteen of the 176 patients (9.0%) had pneumothorax (median age at diagnosis: 24.6 years [range: 20.7-33.7 years]). Among the 16 patients, 15 developed pneumothorax after non-invasive ventilation application (median time between diagnosis and initial non-invasive ventilation application: 5.6 years [range: 3 days to 9.6 years]). Sixteen patients had 31 episodes of pneumothoraces (range: 1-5); among them, 7 (22.6%) episodes were asymptomatic. The lungs were eventually destroyed in 8 (50.0%) patients. There were no significant between-group differences in demographic and spirometric characteristics. However, the pneumothorax group showed a significantly higher survival rate after non-invasive ventilation application.
Conclusion
Upon pneumothorax occurrence in patients with Duchenne muscular dystrophy, recurrences and severe lung damage are common; moreover, they have higher mortality rates than patients without pneumothorax. Chest computed tomography scans should be performed to identify risk factors and treatment should be initiated accordingly. Regular examination is required since pneumothorax is often asymptomatic. |
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Table 1. Epidemiologic and clinical features for 16 patients with Duchenne muscular dystrophy with comorbid pneumothorax
Figure 1. The survival curves from the application of non-invasive ventilation
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