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Approach to cystic lung disease on hrct
- 1. الرحيم الرحمن هللا بسم
- 2. Practical approach to cystic lung disease on HRCT Ahmad Abu-Naglah Prof of chest diseases Al Azhar university
- 4. Definition of cyst • Lung cyst is an air filled structure with perceptible wall typically 1 mm in thickness but can be up to 3 mm. The diameter of a lung cyst is usually less than 1 cm. • Usually containing air, but occasionally fluid or solid material
- 5. Definition of cavity • A gas-filled space which develops in an area of pulmonary consolidation, mass or nodule . • In comparison with a lung cyst, the wall of a cavity is usually relatively thick (>4 mm).
- 6. Imaging Clues to Help Differentiate Pulmonary Cysts and Their Mimics Entity Imaging Characteristics Lung cyst Well-circumscribed, rounded, thin-walled air-filled structure within the lung parenchyma. Wall thickness of ≤3mm. Cavity Air-filled space within the pulmonary parenchyma with thicker walls (>4 mm). Emphysema Polygonal-shaped lucent area without definable walls. Bronchiectasi s Air-filled space within the lung parenchyma that branches and connects with the airway. Associated airway abnormalities including air trapping, bronchial wall thickening, and bronchiolar impactions. Honeycombi ng Clustered subpleural airspaces with variable size and wall thickness. Other signs of pulmonary fibrosis: architectural distortion, traction bronchiectasis, and reticular opacities.
- 9. • With the exception of centrilobular emphysema, pulmonary diseases characterised by cystic air spaces are uncommon or rare conditions
- 10. • Unlike in other solid organs, the lungs do not develop so-called simple cysts.
- 11. Diseases that may cause pulmonary cysts Centrilobular emphysema Lymphangioleiomyomatosis Langerhans cell histiocytosis Lymphoid interstitial pneumonia sjogren syndrome Pulmonary metastases (squamous/adenocarcinoma) Cystic fibrohistiocytic tumour of the lung Subacute (± chronic) hypersensitivity pneumonitis Barotrauma/ARDS Pulmonary infection—pneumatocoeles Desquamative interstitial pneumonia Necrobiotic nodules (end stage) neurofibromatosis Birt-Hogg-Dubé syndrome (rare)
- 12. Primary pulmonary disease with diffuse cysts • pulmonary Langerhan’s cell histiocytosis • lymphocytic interstitial pneumonitis (LIP) • sjogren syndrome • sporadic lymphangiomyomatosis • tuberous sclerosis complex (TSC) with lymphangiomyomatosis • neurofibromatosis • Birt-Hogg-Dubé syndrome (rare) • pulmonary mesenchymal cystic hamartoma (rare)
- 13. Acquired cystic lung disease or secondary feature of a primary disease • honeycombing in UIP pattern • cystic bronchiectasis • pulmonary laceration in trauma • pneumocystis pneumonia • sarcoidosis
- 14. A-Multiple diffuse pulmonary cysts B-Scattered pulmonary cysts with ancillary CT signs C-isolated or scattered cysts with no ancillary CT features
- 15. A-Multiple diffuse pulmonary cysts Langerhans cell histiocytosis (LCH) Lymphangioleiomyomatosis (LAM) Centrilobular emphysema (CLE)
- 16. Langerhans cell histiocytosis (LCH)
- 18. Pulmonary LCH • In the early stages, nodules (which correspond with Langerhans cell granulomas) are the predominant features, while cysts tend to develop later
- 19. • The combination of nodules, cavitating nodules and cysts in a smoker should allow a confident and accurate diagnosis to be made on CT alone
- 20. HRCT Findings • Small peribronchiolar nodules (1-5mm) • Thin-walled cysts (< 1cm), Bizarre and confluent • Ground glass opacities • Late signs: irreversible / parenchymal fibrosis Honey comb lung, septal thickening, bronchiectasis
- 21. Pattern o f cysts • The cysts are typically diffusely distributed, with a predominance in the lung apices and relative sparing of the lung bases . • Sparing of the medial tips of the middle lobe and lingula . • The cysts can have bizarre shapes and unequal sizes
- 22. LCH
- 23. LCH
- 24. HRCT demonstrates sparing of the medial segment of the middle lobe and lingula in a patient with LCH )
- 27. Cavitating nodules and cysts-1 year later
- 28. LCH ( Key Features) • SMOKING • Upper lobe predominance • Combination of cysts and nodules • Characteristic stages • Increased Lung volume • Sparing of costophrenic angle
- 30. Differential Diagnosis • Only small nodules Sarcoidosis Silicosis • Only cysts idiopathic Fibrosis LAM Destructive emphysema
- 32. Lymphangioleiomyomatosis (LAM) • Thin-walled cysts (2mm - 5cm) • Uniform in size / rarely confluent • Homogeneous distribution • Chylous pleural effusion • Lymphadenopathy • in young women
- 34. LAM
- 42. Tuberous sclerosis complex with lymphangiomyomatosis
- 43. Tuberous Sclerosis (young man)
- 45. Centrilobular emphysema • Most common type • Irreversible destruction of alveolar walls in the centrilobular portion of the lobule • Usually upper lobe predominance and uneven distribution • Strongly associated with smoking.
- 46. CLE • CLE is characterised by the presence of multiple rounded areas of low attenuation, which have diameters of several millimetres. • The presence of a central core vessel (centrilobular artery) in the low attenuation cystic air space is classical of CLE
- 47. • Manifests as multiple small areas of low attenuation without a perceptible wall, producing a punched-out appearance
- 51. CLE
- 53. B-scattered pulmonary cysts with ancillary CT signs • lymphocytic interstitial pneumonia (LIP) • hypersensitivity pneumonitis (HP) • desquamative interstitial pneumonia (DIP)
- 54. Lymphocytic Interstitial Pneumonia • Benign lymphoproliferative disorder • Diffuse interstitial infiltration of mononuclear cells • Not limited to the air ways as in follicular Bronchiolitis
- 56. LIP • Rarely idiopathic • In association with: Sjögren‟s syndrome Immune deficiency syndromes, AIDS Primary biliary cirrhosis Multicentric Castlemean‟s disease
- 57. LIP • Ground-glass opacities and nodules are almost universal features in LIP, with cysts seen in about two-thirds of patients . • Interlobular sepal thickening and reticular opacities have also been reported . • The lung cysts are usually small (less than 3 cm), thin walled and distributed in a scattered, random distribution
- 60. Sjoegren disease • Dry eye and dry mouth • Fibrosis, bronchitis and bronchiolitis • LIP • Up to 40 time increased risk for lymphoma (mediastinal adenopathy) • 2 times increased risk for neoplasm
- 61. Sjögren: LIP
- 62. Sub-acute HP • Cysts on HRCT are seen in approximately 10% of patients with subacute HP and are usually few in number and random in distribution.
- 63. • Despite their low frequency, the presence of cysts can be a helpful clue in making a radiological diagnosis of HP, when identified in conjunction with the more classical signs of the disease: these are centrilobular ground glass nodules and a mosaic attenuation pattern. • Furthermore, all of these signs of sub-acute HP can be seen in the more chronic fibrotic form of hypersensitivity pneumonitis
- 65. C-isolated or scattered cysts with no ancillary CT features • Birt-Hogg-Dube syndrome [Folliculin gene- associated syndrome ] • Malignancy
- 66. Birt-Hogg-Dube syndrome • This is a very rare condition that is associated with pneumothoraces, renal cell carcinomas and skin fibrofolliculomas • Family history of recurrent pneumothoraces • Reports suggest that there is a lower zone preponderance for cysts in Birt-Hogg-Dube
- 70. Amyloidosis
- 72. THANK YOU