Imaging Case of the Month July, 2010, Page 3

Answer: All of the above. 

Findings

Diffuse bilateral thin walled cysts are seen with areas of normal intervening lung. These air filled cysts are located in all lobes of the lungs. There is no evidence of pneumothorax or pleural effusion.  Findings are most consistent with lymphangioleiomyomatosis.

Lymphangioleiomyomatosis: 

  • Due to proliferation of atypical smooth muscle cells in lungs and thoracic/retroperitoneal lymph nodes, felt to be caused by a gentic mutation
  • Generally presents in women of childbearing age with progressive dyspnea
  • Top differential considerations include lymphocytic interstitial pneumonia and tracheobronchial papillomatosis
  • Diffuse bilateral uniform thin walled air filled cysts are characteristic HRCT findings
  • Clinical complications include pneumothorax and chylous pleural effusion
  • 50-70% 10 year survival
  • Lung transplant is the recommended treatment in severe cases

References

  1. McCormack F: Lymphangioleiomyomatosis: a clinical update. Chest. 133(2):507-16.2008

  2. JohnsonS: Lymphangioleiomyomatosis. Eur Respir J. 27(5): 1056-65. 2006

  3. Abbott G et al: From the archives of the AFIP: Lymphangioleiomyomatosis: radiologic-pathologic correlation. Radiographics. 25(3)803-28.2005

 

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