Management of pneumothorax in children with chronic pulmonary disease.

Cystic fibrosis is the most common form of chronic pulmonary disease in 
the pediatric age group. As these children survive longer with improved 
therapy, the incidence of pneumothorax has increased. In our patients who 
were more than 10 years of age, the incidence of pneumothorax 
was 12.5 percent. We have reviewed the records of 22 patients 
who have had 44 episodes of pneumothorax complicating cystic fibrosis. In 
addition, our experience includes one child with histiocytosis in whom pneumothorax 
was a serious complication. One other pneumothorax occurred in a teen-age 
girl with apical bullous disease. A child who presents with even 
a minimal pneumothorax complicating cystic fibrosis is at considerable risk of 
developing an increasing lung collapse. Even if the pneumothorax resolves, there 
is a high risk of recurrence. In children treated with only 
a chest tube there was a 50 percent recurrence. Consequently, we 
recommend a chest tube for any episode of pneumothorax in a 
child with cystic fibrosis, with installation of a sclerosing agent when 
the lung expands. Three of our patients so treated had no 
recurrence. Children who have a persistent air leak are confined to 
bed, retain secretions, and pursue a rapid downhill course. A more 
aggressive approach to these children, consisting of thoracotomy, suture of the 
air leak, pteural symphysis, and pulmonary lavage is feasible and the 
mortality rate is low. A pneumothorax in a child with no 
evidence of cystic fibrosis is a rare event. A persistent air 
leak is an indication for thoracotomy, suture of the air leak, 
and lung biopsy. Histiocytosis X is one rare cause for this 
complication in children. 