000 Lymphangioleiomyomatosis LAM

  • Nutshell
    • Cystic lung disease
    • If there is an effusion with Cystic disease
      • most likely LAM
      • most likely chylous
        • LAM is the only cystic lung disease that has an
        • effusion
    • PTX 40%
  • Growth abnormality of smooth muscle
    • occurring in women of childbearing age.
    • and tuberous sclerosis
      • growing throughout the lungs,
        • airways – causing obstructive symptoms
        • lymph vessels (chylous effusions)
        • blood vessels,
      • resulting in a clinical entity similar to emphysema
          • cough
          • dyspnea
          • PTX
        • structurally resulting in the formation of cysts
          • two forms,
            • sporadically,
            • associated with tuberous sclerosis complex
        • CT
          • more than 10 thin walled cysts
          • scattered diffusely throughout the lungs.
          • upper
          • lower
          • mid
          • no nodules
          • associated pleural effusion (chylous)
          • usually in the thousands
        • Associated
          • lymphangionas
          • renal angiomyolipomas
    • Lymphangioleiomyomatosis
      LAM Diffuse Cysts Upper and Lower Lungs
      Imaging of Cystic Lung Disease Thomas Hartman MD Mayo Clinic Video on Imaging Cystic Lung Disease Society of Thoracic Radiology
      Lymphangioleiomyomatosis
      LAM
      Imaging of Cystic Lung Disease Thomas Hartman MD Mayo Clinic Video on Imaging Cystic Lung Disease Society of Thoracic Radiology
      Pneumothorax

      Effusion – likely chylous

      Lymphangioleiomyomatosis
      LAM Diffuse Cysts Chylous Effusion
      Imaging of Cystic Lung Disease Thomas Hartman MD Mayo Clinic Video on Imaging Cystic Lung Disease Society of Thoracic Radiology
      Lymphangioleiomyomatosis
      LAM Diffuse Cysts Chylous Effusion
      Imaging of Cystic Lung Disease Thomas Hartman MD Mayo Clinic Video on Imaging Cystic Lung Disease Society of Thoracic Radiology

      Pneumothorax

      Lymphangioleiomyomatosis
      LAM
      Imaging of Cystic Lung Disease Thomas Hartman MD Mayo Clinic Video on Imaging Cystic Lung Disease Society of Thoracic Radiology
      Pneumothorax

Wiki
Radiopaedia

Abbott, G et al  Lymphangioleiomyomatosis: Radiologic-Pathologic Correlation RadioGraphics Vol. 25, No. 3

Pallisa E eta al Lymphangioleiomyomatosis: Pulmonary and Abdominal Findings with Pathologic Correlation RadioGraphics Vol. 22, No. suppl_1