HIRAL THAKKAR II ND YEAR RESIDENT BARODA MEDICAL COLLEGE SSGH 27-11-07

1. CASES HIRAL THAKKAR II ND YEAR RESIDENT BARODA MEDICAL COLLEGE SSGH 27-11-07 Baroda medical college, SSGH

2. CASE 1 Baroda medical college, SSGH

3. A 64 year old man presented with progressively worsening dyspnea and nonproductivecough . Baroda medical college, SSGH

4. High-resolution CT image obtained at presentation shows reticular opacities, honeycombing (arrowhead), and focal ground-glass opacity (thick arrow). Moderate traction bronchiectasis is present (thin arrow). Baroda medical college, SSGH

5. Follow-up CT image obtained 12 months later shows marked progression of the honeycombing (arrowheads) and traction bronchiectasis (arrows). Baroda medical college, SSGH

6. Coronal CT image shows an obvious apicobasal gradient of the lung alterations. Baroda medical college, SSGH

7. In a 64 yr old pt, with h/o progressive dyspnoea and coughing without any associated other diseases with HRCT showing typical – • Apico –basal gradient • Macrocystic honey combing • Sub pleural reticular opacities • Traction broncheictasis Baroda medical college, SSGH

8. MOSTLY LIKELY DIAGNOSIS IS… • IDIOPATHIC PULMONARY FIBROSIS • [ALSO K/A USUAL INTERSTITIAL PNEUMONIA] Baroda medical college, SSGH

9. American Thoracic Society (ATS) $ European RespiratorySociety (ERS) has dividedIDIOPATHIC INTERSTITIAL PNEUMONIAS into: Baroda medical college, SSGH

10. In their idiopathic form, IIPs are rare diseases. • However, morefrequent disorders such as sarcoidosis, vasculitis, and connectivetissue diseases can display identical morphologic patterns,and the IIPs are considered "prototypes" for these morphologicalterations. • Because imaging plays a crucial role in identifyingboth the idiopathic and the secondary interstitial pneumonias,radiologists need to be familiar with the morphologic and clinicalmanifestations and the diagnostic approach to these conditions. Baroda medical college, SSGH

11. IDIOPATHIC PULMONARY FIBROSIS [IPF]USUAL INTERSTITIAL PNEUMONIA [ UIP] • IPF is the most common entity of the IIPs. • IPF has a substantially poorer prognosis than other IIP’s. • AGE: 50 yrs or more SEX: M > F • Cigarette smoking: an ass. Risk factor for development of the dz, however doesn’t affect prognosis • Response to cortico steroids: none, infact CI • Histologic Features: The histologic hallmark is the presence of scatteredfibroblastic foci . Typically, the lung involvement isheterogeneous and areas of normal lung alternate with interstitialinflammation and honeycombing Baroda medical college, SSGH

12. Imaging FeaturesCHEST RADIOGRAPH: • normal in most patients with early disease. • In advanced disease, the chest radiograph shows decreased lungvolumes and subpleural reticular opacities that increase fromthe apex to the bases of the lungs. • HRCT: • A trio of signs that is highlysuggestive of UIP include: • Subpleural reticular opacities • Macrocystic honeycombing combined withTractionbronchiectasis, • The apicobasal gradient • Typically, imaging findings are heterogeneous,with areas of fibrosis alternating with areas of normal lung Baroda medical college, SSGH

13. Distribution (a), CT image (b), and CT pattern (c) of UIP. The distribution is subpleural with an apicobasal gradient (red area in a). CT shows honeycombing (green areas in c), reticular opacities (blue areas in c), traction bronchiectasis (red area in c), and focal ground-glass opacity (gray area in c). Baroda medical college, SSGH

14. NON-SPECIFIC INTERSTITIAL PNEUMONIA • NSIP is less common than IPF. • Owing to the clinical, radiologic, and pathologic variabilityof NSIP, the term should be considered a provisional diagnosisuntil further characterization of this entity has been established • AGE: 40 - 50 yrs SEX: M = F • Cigarette smoking: not an obvious risk factor • Response to cortico steroids: good • Histologic Features: characterized by temporallyand spatially homogeneous lung involvement . This homogeneityis a key feature in differentiating the NSIP pattern from theUIP pattern. NSIP is divided into cellular and fibrosing subtypes • In cellular NSIP, the thickening of alveolar septais primarily caused by inflammatory cells; (less c’n) • In fibrosing NSIP,interstitial fibrosis is seen in addition to mild inflammation. Baroda medical college, SSGH

15. Imaging Features • CHEST RADIOGRAPH: In patients with early NSIP, the chest radiograph is normal. • In advanced disease, bilateral pulmonary infiltrates are themost salient abnormality. The lower lung lobes are more frequentlyinvolved, but an obvious apicobasal gradient, as seen in UIP,is usually missing . • HRCT: • Typically reveals a subpleural and rathersymmetric distribution. • The mostcommon manifestation consists of patchy ground-glass opacitiescombined with irregular linear or reticular opacities and scatteredmicronodules. {related to the histologicfinding of homogeneous interstitial inflammation } • In advanced disease: tractionbronchiectasis and consolidation, subpleural cysts{compared to those of UIP, these are smaller & limitedin extent}therefore k/a microcystic honey combing. Baroda medical college, SSGH

16. Distribution (a), CT image (b), and CT pattern (c) of NSIP. The distribution is subpleural with no obvious gradient (red area in a). CT shows ground-glass opacity (gray areas in c), irregular linear and reticular opacities (blue areas in c), micronodules (red areas in c), and microcystic honeycombing (green areas in c). Baroda medical college, SSGH

17. Although it is primarily defined as an idiopathic disease, themorphologic pattern of NSIP is encountered in association withfrequent disorders, such as • connective tissue diseases, • hypersensitivitypneumonitis, or • drug exposure. • Once the morphologicpattern of NSIP has been determined in a patient, these secondaryforms of NSIP must be ruled out by the clinician. Baroda medical college, SSGH

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19. CRYPTOGENIC ORGANIZING PNEUMONIA • COP is an IIP with characteristic clinical and radiologic features[formerlyreferred to as bronchiolitis obliterans organizing pneumonia(BOOP)] • AGE: mean age of 55 yrs SEX: M = F • Cigarette smoking: most pts are non-smokers • Response to cortico steroids: good, relapses c’n within 3 months • Histologic Features:The histologic hallmark of organizing pneumonia is the presenceof granulation tissue polyps in the alveolar ducts and alveoli.Typically, thereis patchy lung involvement with preservation of lung architecture.The granulation tissue is all the same age and contains fewinflammatory cells. Baroda medical college, SSGH

20. Imaging Features • CHEST RADIOGRAPH: • Usually shows unilateralor bilateral patchy consolidations that resemble pneumonic infiltrates. However, the consolidations in COP do not represent anactive pneumonia but result from intraalveolar fibroblast proliferations,which may be associated with prior respiratory infection. • Lung volumes are preserved in most patients. • HRCT: • The lung abnormalitiesshow a characteristic peripheral or peribronchialdistribution,and the lower lung lobes are more frequently involved. In some cases, the outermost subpleural area is spared. • Typically, the appearance of the lung opacitiesvaries from ground glass to consolidation; in the latter, airbronchograms and mild cylindrical bronchial dilatation are acommon finding. • These opacities have a tendency to migrate,changing location and size, even without treatment. Theyare of variable size, ranging from a few centimeters to an entirelobe. Baroda medical college, SSGH

21. Distribution (a), CT image (b), and CT pattern (c) of COP. The distribution is peripheral or peribronchial with a basal predominance (red areas in a). CT shows consolidation with air bronchograms (dark gray areas in c), ground-glass opacities (light gray areas in c), linear opacities (blue areas in c), and mild bronchial dilatation (red areas in c). Baroda medical college, SSGH

22. The pattern of organizing pneumonia may occur in a wide varietyof entities, notably in collagen vascular diseases and in infectiousand drug-induced lung diseases. • In the appropriate clinical context, that is, consolidationthat increases over several weeks despite antibiotics, the CTfeatures of COP are often suggestive. • Other atypical imaging findingsinclude irregular linear opacities, solitary focal lesions thatresemble lung cancer, or multiple nodules that may cavitate. • In either case, the diagnosis shouldbe confirmed with surgical lung biopsy Baroda medical college, SSGH

23. RESPIRATORY BRONCHIOLITIS – ASSOCIATED INTERSTITIAL LUNG DISEASE • RB-ILD is a smoking-related interstitial lung disease and isthought to represent an exaggerated and symptomatic form ofthe histologically common and incidental finding of respiratorybronchiolitis. • AGE: 30 – 40 yrs SEX: M > F • Cigarette smoking: avg smoking history of 30 pack-yrs • Response to cortico steroids: good, with cessation of smoking • Histologic Features:The histopathologic hallmark of RB-ILD is the intraluminal accumulationof pigmented macrophages centered around the respiratory bronchioles Baroda medical college, SSGH

24. Imaging Features • CHEST RADIOGRAPH:The chest radiograph is insensitive for detection of RB-ILDand is often normal. Sometimes, bronchial wall thickening orreticular opacities can be seen • HRCT: • The distribution at high-resolution CT is mostly diffuse. • The key features are centrilobularnodules (caused by the peribronchial distribution of the intraluminalinfiltrates ) in combination with ground-glass opacities (correlating with macrophage accumulation in alveolarducts and alveolar spaces) and bronchialwall thickening. • Coexisting moderate centrilobular emphysemais common, given that most patients have a smoking history. Baroda medical college, SSGH

25. Distribution (a), CT image (b), and CT pattern (c) of RB-ILD. RB-ILD has an upper lung predominance (red area in a). CT shows ground-glass opacity (gray area in c) and centrilobular nodules (red areas in c). Baroda medical college, SSGH

26. DESQUAMATIVE INTERSTITIAL PNEUMONIA • DIP is strongly associated with cigarette smoking and is consideredto represent the end of a spectrum of RB-ILD. • However, DIP alsooccurs in nonsmokers and has been related to a variety of conditions,including lung infections and exposure to organic dust. • AGE: 30 – 40 yrs SEX: M > F • Cigarette smoking: avg smoking history of 18 pack-yrs • Response to cortico steroids: good, with cessation of smoking • Histologic Features:The major histopathologic feature of DIP is the accumulationof pigmented macrophages and a few desquamated alveolar epithelialcells in the alveoli . As opposed to the bronchiolocentricdistribution in RB-ILD, lung involvement in DIP is more diffuseand uniform Baroda medical college, SSGH

27. Imaging Features • CHEST RADIOGRAPH: • Chest radiographs of DIP are nonspecific and may reveal hazyopacities • HRCT: • DIP is characterized by diffuse ground-glassopacities, which correlate histologically with the spatiallyhomogeneous intraalveolar accumulation of macrophages and thickeningof alveolar septa • Usually, there is a peripheraland lower lung lobe predominance. • Despite differences in the CT appearance of RB-ILD and DIP,imaging findings may overlap and may be indistinguishable, thus lung biopsy is requiredin all cases of suspected RB-ILD or DIP . Baroda medical college, SSGH

28. Distribution (a) CT image (b), and CT pattern (c) of DIP. DIP has a peripheral predominance (red areas in a). CT shows ground-glass opacity (gray area in c), irregular linear opacities (blue areas in c) and cysts (green areas in c). Baroda medical college, SSGH

29. LYMPHOID INTERSTITIAL PNEUMONIA • As an idiopathic disease, LIP is exceedingly rare. • It is farmore common as a secondary disease in association with systemicdisorders, most notably Sjögren syndrome, human immunodeficiencyvirus infection, and variable immunodeficiency syndromes. • AGE: 50 yrs SEX: F > M • Cigarette smoking: none • Response to cortico steroids: unpredictable • Histologic Features: • The LIP pattern is characterized by diffuse infiltration ofthe interstitium by lymphocytes, plasma cells, and histiocytes. Reactive lymphoid follicles are often presentand distributed along the peribronchiolar regions Baroda medical college, SSGH

30. Imaging Features • CHEST RADIOGRAPH: • reveals nonspecificfindings, such as bilateral reticular, reticulonodular, or alveolaropacities. • HRCT: • shows bilateral abnormalities that are diffuse or have a lowerlung predominance. • The dominant feature is ground-glass attenuation (relatedto the histologic evidence of diffuse interstitial inflammation). • Another frequent finding is thin-walled perivascularcysts. • In contrast to the subpleural, lower lungcystic changes in UIP, the cysts of LIP are usually within thelung parenchyma throughout the mid lung zones (result from air trapping due to peribronchiolar cellular infiltration). In combination with ground-glass opacities, these cystsare highly suggestive of LIP. Baroda medical college, SSGH

31. Distribution (a), CT image (b), and CT pattern (c) of LIP. The distribution is diffuse (red area in a). CT shows ground-glass opacity (gray area in c) and perivascular cysts (green areas in c). Baroda medical college, SSGH

32. ACUTE INTERSTITIAL PNEUMONIA • AIP is the only entity among the IIPs with acute onset of symptoms. • AGE: mean age of 50 yrs SEX: M> F • Cigarette smoking: not related • Typically, a h/o viral illness exists • Response to cortico steroids: good, in early phase. However, prognosis is poor. • Histologic Features: The histologic pattern of AIP includes diffuse alveolar damage,which can be categorized into an early exudative phase and achronic organizing phase. As opposed to the heterogeneous appearance of UIP,fibrotic changes in AIP are uniform and characterized by numerousfibroblasts but relatively little collagen deposition Baroda medical college, SSGH

33. Imaging Features: • The radiographic and high-resolution CT features of AIP aresimilar to those of acute respiratory distress syndrome. • However, AIP has a symmetric, bilateraldistribution with a lower lobe predominance. • Thecostophrenic angles are often spared. • In the early phase ofAIP, ground-glass opacities are the dominant CT pattern andreflect the presence of alveolar septal edema and hyaline membranes. • Areas of consolidation are limited to the dependent area ofthe lung (resulting from intraalveolar edema and hemorrhage). • In the late phase, there is bronchial dilatation and architectural distortion Baroda medical college, SSGH

34. Distribution (a), CT image (b), and CT pattern (c) of AIP. AIP has a basal predominance (red area in a). CT shows airspace consolidation (dark gray areas in c), ground-glass opacities (light gray areas in c), and bronchial dilatation (red areas in c). Baroda medical college, SSGH

35. Summarizing their radiological appearances acc to their distribution: RB –ILD Sometimes can be diffuse involvement Baroda medical college, SSGH

36. Summarizing HRCT appearances: Baroda medical college, SSGH

37. CASE 2 Baroda medical college, SSGH

38. HISTORY • A 19-year-old woman presented with a 3-week history of headaches and blurred vision and with new onset of weakness on the right side. • Computed tomography (CT), magnetic resonance (MR) imaging, and digital subtraction arteriography were performed. Baroda medical college, SSGH

39. Axial NECT scan of the brain reveals a subtle area of low attenuation in the right frontal area and a second, more obvious region in the left parietal lobe (arrows). (b) Contrast-enhanced CT scan obtained at the same level demonstrates an "empty delta sign" (arrowhead) with additional enhancement that is gyriform and probably venous (arrows). Baroda medical college, SSGH

40. Sagittal MR image does not demonstrate the flow void normally seen in the superior sagittal sinus. Instead, there is an area of irregular intermediate signal intensity within the sinus (arrows). Baroda medical college, SSGH

41. (b) Axial intermediate weighted MR image demonstrates increased subcortical and cortical signal intensity in the areas that appeared abnormal at CT, a finding consistent with stroke. (c) Axial T2-weighted MR images help confirm stroke. Baroda medical college, SSGH

42. (d) Axial contrast-enhanced T1-weighted MR image demonstrates prominent venous enhancement as well as irregular enhancement in the areas of infarct and slow-flowing veins (arrows). The superior sagittal sinus does not demonstrate normal filling (arrowhead). (e) Coronal contrast-enhanced T1-weighted MR image reveals prominent enhancement of the left parietal lobe infarct and an empty delta sign in the superior sagittal sinus (arrow). Baroda medical college, SSGH

43. On a venous-phase digital substraction angiogram, the superior sagittal sinus demonstrates no filling (arrowheads). The vein of Galen demonstrates normal filling (arrow), whereas the straight sinus demonstrates only minimal filling, a finding consistent with thrombosis. Baroda medical college, SSGH

44. DIAGNOSIS: Superior sagittal sinus thrombosis and associated infarcts. Baroda medical college, SSGH

45. DISCUSSION • Sinus- vein thrombosis is a multistep process that begins when thrombus completely occludes a dural sinus, usually the superior sagittal sinus. • The thrombus progresses, obstructing the sinus and then extending to involve bridging veins anterior to the obstruction. • Once the tributary cortical veins are occluded, petechial perivascular heorrhages and cortical venous infarctions occur. • Clinical signs and symptoms are non- specific and imaging is critical to diagnosis Baroda medical college, SSGH

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47. Veno-occlusive disease of the brain most commonly affects the superior sagittal sinus, followed by the transverse, sigmoid, and straight sinuses. • Commonly occluded veins are the superficial cortical veins. • Less commonly internal cerebral vein thrombosis occurs. Baroda medical college, SSGH

48. Imaging findings in dural sinus thrombosis can be conveniently grouped into those related to (a) intraluminal thrombus (eg, empty delta sign, "cord sign"), (b) venous collateral flow, (eg, gyral and tentorial enhancement), (c) brain involvement (eg, hemorrhage, infarcts, edema), (d) changes in flow dynamics, which are most evident atMR imaging. Baroda medical college, SSGH

49. COMPUTED TOMOGRAPHY • Unenhanced CT may demonstrate hyperdense thrombus in the thrombosed dural sinus or veins (cord sign). • On CECT scans the engorged dural cavernous spaces, meningeal venous tributaries and colateral venous channels that surround the relatively hypodense occluded sinus may enhance around the thrombus, producing the so – called empty delta sign. • In chronic and sub-acute cases of dural sinus thrombosis, the tentorium and falx appear strikingly thickened, engorged, and somewhat ill –defined or shaggy. Baroda medical college, SSGH

50. Axial CECT scans in a patient with SSS thrombosis show thickened and a shaggy appearing tentorium. Baroda medical college, SSGH