Recurrent pneumothorax in course of cystic fibrosis – treatment challanges. Case report.
Cystic fibrosis (cystic fibrosis) is the most common genetic disease with autosomal recessive inheritance among the Caucasian population. The incidence varies from country to country. In Poland, it is about 1/2300 of live births. In its course, as a result of mutations on both copies of the CFTR (cystic fibrosis transmembrane conductance regulator) gene, which encodes a chloride channel, ion transport in all exocrine glands is disrupted, leading to dysfunction of key organs for survival, such as the pancreas, liver, lungs and intestines [1].Respiratory complications are the first cause of premature death in CF patients. Pulmonary manifestations of CF include hemoptysis, pneumothorax, chronic sinusitis and airway inflammation, bronchial dilatation, and respiratory failure, among others. Pneumothorax is the presence of air in the pleural cavity. Risk factors are associated with chronic infection with Burkholderia cepacia and Pseudomonas aeruginosa and the coexistence of other complications such as hemoptysis and pancreatic insufficiency [4]. Significant disease progression (FEV1 30%) [2] also favors the occurrence of this complication. Taking into account the criterion of time, we can distinguish the first case of emphysema, or recurrent emphysema, when the air leak reappears within 7 or more days after the previous episode. If the air leak persists for 5 or more days, we can recognize persistent emphysema (Shidlow 1993). Therapy can be divided into medical (oxygen therapy and observation) or surgical interventions. The latter group, which includes drainage, pleurodesis and pleurectomy depending on the course of treatment, is considered a more effective therapeutic option for patients with emphysema without significant concomitant lung disease. However, in CF-affected patients, it may complicate the lung transplant procedure in the future. There are no clinical trials available that reliably compare the efficacy and risk of complications of both types of interventions in CF patients [3-4]. An additional complication is that emphysema recurs in 50-90% of these patients, and in almost half of them it also occurs on the opposite side of the chest. Emphysema significantly worsens lung function, and the patient experiences shortness of breath and significant pain. If refractory to treatment, it is an independent indication for lung transplantation.[5]Here we present the case of a 16-year-old female patient with recurrent unilateral pneumothorax, in which, due to the severity of the disease, the diagnosis and therapeutic measures taken were fraught with numerous problems.




