Learning Radiology xray montage

Lymphangiomyomatosis and Tuberous Sclerosis

  • Similar in pathology and x-ray appearance
  • Widespread proliferation of smooth muscle in pleura, alveolar septa, bronchi, pulmonary vessels and lymphatics as well as lymph nodes, especially in posterior mediastinum and retroperitoneum
  • Focal emphysema develops as result of narrowing of airways
  • Thoracic duct may be obliterated
  • Produce multiple small cysts with a hamartomatous proliferation of smooth muscle in their walls


Characteristic imaging triad of:


  • Progressive, diffuse interstitial disease
  • Recurrent chylous effusions and sometimes chylous ascites
  • Recurrent pneumothorax


  • Tuberous sclerosis is inherited as a dominant with variable penetrance:

o       Mental defects

o       Epilepsy

o       Retinal phacoma

o       Angiomyolipomas of the kidneys

o       Rhabdomyomas of the heart

o       Intracranial calcifications

o       Sclerotic skull lesions

o       Adenoma sebaceum

o       Subungual fibromas

o       Pulmonary lymphangiomyomatosis (syn:pulmonary myomatosis)

§         Exclusively in females ages 17-47 years

§         Rare


Imaging findings

  • Identical in both tuberous sclerosis and lymphangiomyomatosis and indistinguishable from pulmonary fibrosis except for decreased lung volume in fibrosis and increased lung volume in the others
  • CT
    • Coarse, reticular interstitial pattern
    • Normal/increased lung volume
    • Numerous thin-walled pulmonary cysts and honeycombing
    • Various sizes/surrounded by normal lung parenchyma

·         Unilateral or bilateral pleural effusions which are usually large and recurrent

·         Spontaneous pneumothorax is common




  • Progressive exertional dyspnea and cough
  • Hemoptysis