대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Courtesy

Asan Medical Center

Discussion

Tuberous Sclerosis
- AD disorder of mesodermal development (Chr 9)
- Classical triad
- Epilepsy, mental retardation, adenoma sebaseum

Lung involvement
- Uncommon (1- 2.5%)
- Female predominent
- Similar to LAM
- Chest radiograph: Diffuse reticular pattern c/s cystic or bullous changes
- HRCT: Thin-walled cysts of various size through both lungs
- Pneumothorax is common (50%)
- Chylous pleural effusion
- pneumodediastinum

Micronodular pneumocyte hyperplasia
- Widespread, evenly distributed nodules
- 1-3 mm (up to 8 mm) on HRCT
- Represent hamartomatous proliferation of type 2 pneumocytes
- May affect male TS patients (unlike LAM)
(Br J Radiol. 2000 ;73 :91-8)

Keywords

Lung, Interstitial lung disease, Tuberous sclerosis,