Introduction. Pulmonary alveolar proteinosis (PAP) is a relatively rare lung disorder, probably under diagnosed, characterized by the accumulation of lipoproteinaceosus material in the lung alveoli. The primary (acquired or idiopathic) form occurs in more than 90% of all cases. Whole lung lavage is considered the golden standard of treatment. In this report, we describe a rare case of pulmonary alveolar proteinosis with severe and incapacitating respiratory, in which whole lung lavage followed the thoracotomy for spontaneous pneumothorax.

Case presentation. A 34-year-old white male patient presented at the respiratory intensive care with severe respiratory failure, aggravated in the last two months, cough, night sweats and important weight loss and left spontaneous pneumothorax. The initial diagnosis of diffuse interstitial pneumopathy was revised to Pulmonary Alveolar Proteinosis after bronchoalveolar lavage. Active pleural drainage of the pneumothorax was unsuccessful and after two months, surgical suture of the lung was performed under general anaesthesia. One month later a whole left lung lavage was performed. The same procedure was also performed on the right lung. Eight months later the patient had a good exercise tolerance, normal arterial blood gas (ABG) values, and persistent ground-glass opacities in some of the pulmonary segments on CT scan.

Conclusions. The most severe forms of pulmonary alveolar proteinosis, in which hypoxemia and cyanosis occur, have a high mortality risk during anaesthesia and whole lung lavage. When a rare complication like spontaneous pneumothorax occurs, the suturing of the pulmonary apical blebs seems to be the only viable solution, despite the high risk of dehiscence of the sutures due to the poor pulmonary tissue integrity at the time of the whole lung lavage and during postoperative care.