Spontane pneumothorax bij jonge vrouwen: mogelijk lymfangioleiomyomatose

– (Recurrent) pneumothorax developed spontaneously in three women aged 33, 35 and 36 years, two of whom were pregnant. Morbid-anatomical examination of lung biopsy samples in two of them revealed proliferation of smooth muscle cells which through infiltration of pleura, septa and alveoli had led to loss of pulmonary parenchyma and formation of cystic lesions; the cystic lesions were visible in a CT scan in all three patients. The diagnosis in 2 patients was ‘lymphangioleiomyomatosis’; the third patient had anomalies compatible with lymphangioleiomyomatosis, but these were interpreted as tuberous sclerosis because of the presence of renal angiomyolipomas.

This disorder occurs exclusively in women in the reproductive age. Treatment consisted in drainage of the pneumothorax, pleurodesis and pleurectomy, with administration of medroxyprogesterone. One year later, the pneumothorax had not recurred.