To determine the frequency, recurrence rate, and optimal management of pneumothorax in pulmonary Langerhans’cell histiocytosis(PLCH).
Retrospective data review of 102 adults with confirmed PLCH, seen at our institution over 23 years.
Sixteen of 102 (16%) patients with PLCH, 12 men and 4 women, experienced 37 pneumothoraces, 10 patients (63%) had more than one, and in 11 (69%) was the initial presentation. Mean age at diagnosis was lower in the pneumothorax group (29.4 ±8.4 [mean±SD] vs. 42.3± 12.7; p<0.001). Recurrence was 58% (14/24 episodes) when pneumothorax was managed with observation or chest tube and 0% with surgery and pleurodesis. Survival was 13.8 years with pneumothorax and 18.3 without (p=0.715). Pulmonary function was not different in the pneumothorax group (p > 0.1).CONCLUSIONS: Recurrence was high when the pneumothorax was managed conservatively. No significant differences were observed in pulmonary function and survival in the pneumothorax group.
Our data supports the early use of surgical therapy with pleurodesis for pneumothorax in patients with PLCH.
Mode of Pneumothorax Management and Recurrences Rates in 16 Patients with PLCHMode of ManagementNumber of Episodes &Recurrence Rate,% (n)Observation617 (1)Chest tube alone1872 (13)Surgery #120 (0)&
Details regarding management were available in 34 of 37 episodes involving 36 hemithoraces (2 bilateral pneumothoraces)#
Surgery included thoracotomy with mechanical pleurodesis (10 episodes), pleurectomy (1 episode) or chemical pleurodesis (1 episode)
J.L. Mendez, None.