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

Note multiple thin-walled cysts throughout both lungs

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

·         Spontaneous pneumothorax is common 

Clinically

• Progressive exertional dyspnea and cough
• Hemoptysis