- Cyst is a
- nonspecific term for a
- thin-walled (usually <3 mm),
- well-defined and circumscribed,
- air containing lesion
- 1 cm or more in diameter.
- heterogenous group of disorders characterised by
- multiple air-filled lucencies
- surrounded by discrete walls.
- heterogenous group of disorders characterised by
- Diseases
- Lymphangioleiomyomatosis (LAM),
- Langerhans cell histiocytosis (PLCH),
- Birt-Hogg-Dubé syndrome (BHD), and
- Lymphoid interstitial pneumonia (LIP),
- Thin walled cysts
- No central core structures
- Differential Diagnosis
-
- Lymphangioleiomyomatosis (LAM),
- Langerhans cell histiocytosis (PLCH),
-
- Langerhans Cell Histiocytosis
CT scan shows multiple small cysts sometimes irregular in shape predominantly in the upper lobes. The cysts are round and air filled large and are between 5mm-8mm
- Langerhans Cell Histiocytosis
- Birt-Hogg-Dubé syndrome (BHD), and
- Lymphoid interstitial pneumonia (LIP), although the differential diagnosis can at times expand to encompass other extremely rare etiologies
- Amyloidosis
- Light Chain Deposition Disease
- Neurofibromatosis
- Lymphangioleiomyomatosis (LAM),
- Less Common
- Sjögren syndrome
- light chain deposition disease
- Ehlers Danlos syndrome type IV
- fire-eater’s lung (pneumatoceles)
- lymphomatoid granulomatosis
- neurofibromatosis
- congenital pulmonary airway (cystic adenomatoid) malformation,
- smoking related small airways injury [
- Proteus syndrom
- Look Alikes
- Emphysema
- No wall
- Central soft tissue elements
- Blebs
- Bullae
- >1 cm in diameter, bounded by a
- thin wall (<1 mm)
- background emphysema
- Bronchiectasis
- continuity with airways
- Honeycombing
- enlarged airspaces
- .3 -1.0cms (up to 2.5cms)
- thick fibrous walls
- End stage fibrotic disease
- subpleural
- stacked
- enlarged airspaces
- Pneumatoceles
- thin-walled parenchymal cyst
- Infection
- bacterial pneumonia eg children
- PCP Pneumocystis jirovecii,
- coccidioidomycosis,
- hyperimmunoglobulinemia E syndrome,
- staphylococcal pneumonia
- Chest Trauma
- Emphysema
- Look Alikes
- LAM
Upper
- Infections
- PCP
-
Complication of PCP Lung Cysts
- PTX,
- incidence 35% in patients with cysts
- frequently bilateral
- often refractory to chest tube Rx,
- frequently need surgery eg pleurodesis
- associated higher mortality rate,
- especially in patients on ventilation.
- PTX,
- ILD
- DIP
-
LIP
- Lower Lobe predominance
-
- LIP Lymphoid Interstitial Pneumonia