Pneumothorax due to lymphangioleiomyomatosis

Case contributed by Craig Hacking
Diagnosis almost certain


2 weeks history of shortness of breath on exertion. Reduced air entry on the right.

Patient Data

Age: 40 years
Gender: Female

Large right pneumothorax with complete collapse of the right lung. Allowing for rotation, there is some mild mediastinal shift to the left.

Large volume right pneumothorax with near complete collapse of the right lung. Mild left mediastinal shift. Multiple variably sized, thin walled cystic lesions are diffusely located throughout the lung parenchyma and along the fissural and pleural surfaces. Ruptured cyst is the likely cause for the pneumothorax. Similar, though less extensive, appearance within the left lung. No parenchymal nodules or consolidation identified. Tracheobronchial tree is patent and there is no bronchial thickening. No pleural fluid.

Heart, pericardium and remnant thymic tissue are unremarkable. Normal appearance of the thoracic vessels. No mediastinal or hilar lymphadenopathy

No osseous lesions. Normal appearance of the upper abdominal viscera. No renal lesions.


  • Large volume right pneumothorax with mild left mediastinal shift likely secondary to a ruptured subpleural bleb.
  • Diffuse thin-walled cystic lung disease. Given the patient demographic this may represent lymphangioleiomyomatosis (LAM).

Post ICC


Right pigtail pleural catheter is well positioned. Significant reduction in the volume of the right pneumothorax. No mediastinal shift.

Case Discussion

Despite the absence of other supportive clinical and diagnostic features of LAM such as renal AMLs, neurocutaneous features of tuberous sclerosis, and chylous pleural effusions, a provisional diagnosis of LAM was made based on the patient demographics and chest CT findings. A VAT pleurodesis and lung biopsy was performed but histology was inconclusive for LAM. The case was discussed at a tertiary hospital interstitial lung disease multidisciplinary team meeting and the diagnosis of sporadic LAM was supported. The patient was referred to a lung transplant center.

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