cystic lung disease
Lymphangioleiomyomatose
der Lunge in der Computertomographie: Multiple, dünnwandige zystoide Veränderungen mit gleichmäßiger Verteilung. Aktuell kein Pneumothorax.
Imaging of
congenital lung diseases presenting in the adulthood: a pictorial review. A 32-year-old man with neurofibromatosis type 1. Coronal (a) reconstruction CT image in the lung window shows lung cysts (white arrows). Cutaneous neurofibroma (blue arrow) and neurofibroma arising from the intercostal nerve (red arrow) are seen on the coronal reconstruction image in the mediastinal window (b)
Retroperitoneal
hemorrhage • Hemorrhagic renal angiomyolipoma - Ganzer Fall bei Radiopaedia
Cystic
bronchiectasis • Cystic bronchiectasis - Ganzer Fall bei Radiopaedia
Lymphocytic
interstitial pneumonitis • Lymphocytic interstitial pneumonitis - Ganzer Fall bei Radiopaedia
Lymphocytic
interstitial pneumonitis • Lymphocytic interstitial pneumonitis with infective exacerbation of bronchiectasis - Ganzer Fall bei Radiopaedia
Cystic lung
disease • Pulmonary light chain deposition disease - Ganzer Fall bei Radiopaedia
Birt-Hogg-Dubé
syndrome • Birt-Hogg-Dubé syndrome - Ganzer Fall bei Radiopaedia
Langerhans
cell histiocytosis • Pulmonary Langerhans cell histiocytosis - Ganzer Fall bei Radiopaedia
Lymphangioleiomyomatosis
• Tuberous sclerosis complex with lymphangioleiomyomatosis - Ganzer Fall bei Radiopaedia
Lymphangioleiomyomatosis
• Cystic lung diseases (illustrations) - Ganzer Fall bei Radiopaedia
A practical
approach to cystic lung disease on HRCT. Multiple cystic lesions are present in the left lung (arrow) with a small pneumothorax (arrowhead). The patient had a known history a previous oropharyngeal squamous cell carcinoma and the lesions were confirmed as cystic squamous cell carcinoma metastases
Imaging of
congenital lung diseases presenting in the adulthood: a pictorial review. A 53-year-old man with Birt–Hogg–Dubé Syndrome. Coronal (a) and sagittal (b) reconstruction images in the lung window show large cysts with irregular shapes. Contrast-enhanced coronal reconstruction image in the mediastinal window (c) demonstrates an enhancing heterogeneous solid mass in the right kidney
Imaging of
congenital lung diseases presenting in the adulthood: a pictorial review. A 32-year-old woman with lymphangioleiomyomatosis. Bilateral multiple thin-walled cysts without zonal distribution are seen on contrast-enhanced coronal (a) and sagittal (b) reconstruction images in the lung window. Coronal image in the mediastinal window depicts fat containing liver lesions compatible with angiomyolipomas (arrows)
Imaging of
congenital lung diseases presenting in the adulthood: a pictorial review. Bronchogenic cysts showing air-fluid and fluid–fluid levels. Axial CT image in lung window in a patient shows air-fluid level within the bronchogenic cyst (a). In another patient, air-fluid level and also milk of calcium can be seen within the bronchogenic cyst on axial CT images in lung (b, arrow) and mediastinal windows (c, arrow). Axial CT image in mediastinal window in another patient shows a bronchogenic cyst (d, white arrow). Fat suppressed T2 weighted (e, white arrow) and post-contrast T1 weighted (f, white arrow) MR images demonstrate the fluid-fluid level within the cyst. The red arrows show the ectopic thyroid nodule (d–f)
A practical
approach to cystic lung disease on HRCT. HRCT of a patient with LCH. Multiple cysts are present of varying sizes and shapes (arrow)
Metabolic and
storage lung diseases: spectrum of imaging appearances. A man with amyloidosis presenting as cystic lung disease. HRCT image shows multiple cysts (arrows) and scattered irregular calcified nodules (arrowheads)
Birt-Hogg-Dubé
syndrome detected incidentally by asymptomatic bilateral pneumothorax in health screening: a case of a young Japanese woman. Chest CT showed right-sided pneumothorax and multiple cystic lesions in both of the patient"s lungs. Adhesion and thickened visceral pleura were found.
A practical
approach to cystic lung disease on HRCT. HRCT shows a thick-walled cavity in the left lung adjacent to the oblique fissure (arrow) in a patient with a pneumonia. Note the thick wall and ragged edges to the cavity
A practical
approach to cystic lung disease on HRCT. HRCT image showing thin-walled cysts in the right lung (arrow) in a patient with LAM
Pneumatozele
mit Flüssigkeitsansammlung als Hinweis auf mögliche Superinfektion bei einem COPD-Patienten. Computertomographie axial und coronar.
A practical
approach to cystic lung disease on HRCT. HRCT demonstrates sparing of the medial segment of the middle lobe and lingula in a patient with LCH (arrows)
A practical
approach to cystic lung disease on HRCT. HRCT of a patient with LAM and right pneumothorax. Coronal reformat demonstrates cystic involvement of the juxtraphrenic recesses, but sparing of the extreme apices
A practical
approach to cystic lung disease on HRCT. HRCT shows three cysts in the right lung of a patient with LAM. Note the regular conformity of the cysts (arrow) with a thin wall
A practical
approach to cystic lung disease on HRCT. HRCT shows thin walled cysts (arrow) and ground-glass opacities (*) in a patient with LIP. The cysts have a random distribution
A practical
approach to cystic lung disease on HRCT. HRCT from a patient with subacute HP. Small cysts are present (arrow) on a background of widespread ground glass opacity and mosaic attenuation in the anterior segment of the left upper lobe (*)
A practical
approach to cystic lung disease on HRCT. HRCT showing widespread ground glass opacities and several small cysts in a patient with a strong smoking history, who was subsequently diagnosed with DIP
A practical
approach to cystic lung disease on HRCT. HRCT of a patient presenting with a pneumothorax (arrowhead). The only parenchymal abnormality was the presence of a few small cysts, such as the one in the posterior segment of the right upper lobe (arrow). The patient was later diagnosed with LAM
A practical
approach to cystic lung disease on HRCT. Coronal reformat CT thorax shows multiple thin-walled cysts with a lower lobe predominance in a patient with Birt-Hogg-Dube. The cysts have irregular shapes (arrow) and a right apical pneumothorax is present (arrowhead). The patient had a family history of pneumothoraces
Lymphangioleiomyomatosis
• Sporadic lymphangioleiomyomatosis - Ganzer Fall bei Radiopaedia
A practical
approach to cystic lung disease on HRCT. Coronal reformat CT thorax in a patient with CLE demonstrates a cyst with a wall in the left lower lobe (arrow). More classical CLE with a core centrilobular artery and a much less perceptible wall is seen more superiorly (arrowhead)
Cystic lung disease is an umbrella term used to group the conditions coursing with multiple lung cysts.
Clinical presentation
The clinical presentation is an important clue to the differential diagnosis of cystic lung diseases .
Diseases that present with insidious dyspnea or spontaneous pneumothorax:
- lymphangioleiomyomatosis
- Birt-Hogg-Dubé syndrome
- pulmonary Langerhans cell histiocytosis
- desquamative interstitial pneumonia
- lymphocytic interstitial pneumonitis
Congenital cystic lung diseases that present with recurrent pneumonia or are asymptomatic:
Diseases that present with signs/symptoms of infection:
Multisystemic diseases that primarily present with extrapulmonary signs/symptoms:
- neurofibromatosis type 1
- amyloidosis
- light chain deposition disease
- lymphangioleiomyomatosis associated with tuberous sclerosis
Pathology
A lung cyst is a gas-filled structure with a thin perceptible wall, typically <2 mm in thickness but can be up to 4 mm. The diameter of a lung cyst is usually <1 cm. By conventional definition in the literature, a lung cyst can be distinguished from a cavity for which the wall thickness is greater than 4 mm. However, in practice, clear separation of the two entities can sometimes be difficult .
Etiology
Primary pulmonary disease where diffuse cysts are the predominant feature:
- pulmonary Langerhans cell histiocytosis
- lymphocytic interstitial pneumonitis
- lymphangioleiomyomatosis with or without tuberous sclerosis
- tracheobronchial papillomatosis
- desquamative interstitial pneumonia
- Sjogren syndrome
- neurofibromatosis type 1
- Birt-Hogg-Dubé syndrome (rare)
- pulmonary mesenchymal cystic hamartoma (rare)
Acquired cystic lung disease or cysts as a secondary feature of a primary disease:
- honeycombing in usual interstitial pneumonia pattern
- pulmonary laceration in trauma
- pneumocystis pneumonia
- sarcoidosis
- light chain deposition disease
- amyloidosis
Radiographic appearance
CT
The approach to cystic lung disease centers around the appearance on high resolution CT and history .
Once the lucencies are found to be true cysts (thin walled), the location should be determined. Subpleural or intrapleural cysts include:
- bullae/blebs, including in paraseptal emphysema: upper lobe predominance
- honeycombing due to pulmonary fibrosis: lower lobe predominance, stacked (multiple rows)
In contrast, intraparenchymal cysts are surrounded by lung parenchyma and can be further evaluated by their associated features:
Intraparenchymal cysts with associated ground glass opacities:
- pneumocystis pneumonia: diffuse ground glass opacities with few cysts, septal thickening, in patients with immunodeficiency (e.g. HIV/AIDS)
- desquamative interstitial pneumonia: lower lung predominant ground glass opacities with few cysts, in smokers
- lymphocytic interstitial pneumonitis: lower lung predominant perilymphatic cysts, interlobular septal thickening, few centrilobular nodules, possible ground glass opacities, in patients with autoimmune disease (e.g. Sjögren syndrome) or immunodeficiency (e.g. HIV/AIDS)
Intraparenchymal cysts with associated pulmonary nodules:
- pulmonary Langerhans cell histiocytosis: upper lung predominant, bizarre shaped cysts with variable wall thickness, stellate nodules, in young smokers
- lymphocytic interstitial pneumonitis: as above
- light chain deposition disease: diffuse cysts, diffuse irregular nodules, lymphadenopathy, in patients with plasma cell dyscrasias
- amyloidosis: peribronchovascular or subpleural cysts, often calcified nodules, in patients with Sjögren syndrome (with or without lymphocytic interstitial pneumonitis)
Most other causes of intraparenchymal cysts do not have associated findings and may be instead distinguished by their distribution:
Unifocal pulmonary cysts:
- incidental pulmonary cyst
- pneumatocele: usually transient, related to recent pneumonia or trauma
- hydatid disease (echinococcosis)
- bronchogenic cyst
- congenital pulmonary airway malformation
- pulmonary sequestration (intralobar)
Multifocal pulmonary cysts :
- lymphangioleiomyomatosis: diffuse, uniformly round cysts, with spontaneous pneumothorax or chylothorax, in young women or patients with tuberous sclerosis
- Birt-Hogg-Dubé syndrome: lower lobe predominant, disproportionately paramediastinal, elongated (floppy) cysts, with spontaneous pneumothorax and skin lesions or renal cancer
- lymphocytic interstitial pneumonitis: as above
- neurofibromatosis type 1: upper lobe predominant
- tracheobronchial papillomatosis
- cystic lung metastases
Differential diagnosis
If the pulmonary lucencies in question are not true cysts (not thin walled airspaces), there are several considerations other than cystic lung disease:
- cystic bronchiectasis: connected to tubular airways
- centrilobular emphysema: without distinct walls
- cavitating lung lesions: wall thickness >4 mm
Siehe auch:
- Bronchiektasen
- Pneumatozele
- Langerhanszell-Histiozytose der Lunge
- Sarkoidose
- Neurofibromatose Typ 1
- Tuberöse Sklerose
- Honigwabenmuster
- Lymphangioleiomyomatose
- Lungenzysten
- Emphysembullae
- Sjögren-Syndrom
- Lungenmetastasen
- cystic bronchiectasis
- kongenitale pulmonale Atemwegsmalformation (CPAM)
- Kavernöse Lungenläsionen
- gewöhnliche interstitielle Pneumonie (UIP)
- lymphozytisch interstitielle Pneumonie
- Neurofibromatose
- Pneumocystis jiroveci Pneumonie
- zystische Lungenläsionen bei Kindern
- tracheobronchiale Papillomatose
- Emphysem
- Birt-Hogg-Dubé-Syndrom
- multiple Lungenherde
- Lungenlazeration
und weiter:
Assoziationen und Differentialdiagnosen zu multiple zystische Lungenherde:
kongenitale
pulmonale Atemwegsmalformation (CPAM)
gewöhnliche
interstitielle Pneumonie (UIP)
