Prachi P. Agarwal MD, Presenter: Nothing to Disclose

Ezra Haggerty MD, Abstract Co-Author: Nothing to Disclose

Barry Howard Gross MD, Abstract Co-Author: Nothing to Disclose

Victoria Raymond, Abstract Co-Author: Nothing to Disclose

Ella A. Kazerooni MD, Abstract Co-Author: Nothing to Disclose

William J. Weadock MD, Abstract Co-Author: Owner, Weadock Software, LLC

1. To illustrate the clinical manifestations of Birt Hogg Dube (BHD) syndrome 2. To demonstrate the spectrum of imaging findings in BHD syndrome pertaining to kidneys and lungs 3. To compare & contrast cyst characteristics in BHD syndrome and other cystic lung diseases  

1. Introduction 2. Skin manifestations: Classic triad 3. Renal manifestations a. Solid renal tumors (oncocytomas & renal cell cancers) b. D/D for bilateral hereditary renal masses 4. Lung manifestations a. Cyst characteristics in BHD syndrome i. Cysts of variable shapes & sizes ii. Lower lung predominance b. Differentiating features from other cystic diseases i. Lymphangioleiomyomatosis (LAM): Similarities between LAM & BHD include skin, renal & lung involvement. Cysts involve costophrenic (CP) angles in both. Differentiating features of LAM are uniform round cysts, diffuse distribution and the type of renal (angiomyolipomas) & skin lesions (facial angiofibromas etc). ii. Langerhans cell histiocytosis (LCH): Similarities include variably shaped lung cysts. Differentiating features of LCH are sparing of CP angles, upper lung predominance & association with nodules iii. Lymphocytic interstitial pneumonitis: Typically perivascular cysts in a specific clinical setting (e.g. Sjogren syndrome) often associated with nodules 5. Conclusion  

http://abstract.rsna.org/uploads/2014/14004352/14004352_x7hv.pdf

Agarwal, P, Haggerty, E, Gross, B, Raymond, V, Kazerooni, E, Weadock, W, Birt Hogg Dube Syndrome: Not Your Usual Cystic Lung Disease.  Radiological Society of North America 2014 Scientific Assembly and Annual Meeting, - ,Chicago IL. http://archive.rsna.org/2014/14004352.html