CASE DISCUSSION

1. CASE DISCUSSION Aarti Sardana Second yr Resident Dept of Radiodiagnosis, SSG Hospital Date: 28-02-08

2. CASE 1

3. HISTORY • A 50 yr old patient presented with a 6-month history of pain and swelling of the right knee.

4. Moderate joint effusion, and osteophytes

5. Long-axis ultrasound (US) image shows distention of the suprapatellar bursa by a mass with characteristic frond like projections (arrows) surrounded by a joint effusion (*).

6. Coronal T1w Sag T1

7. Axial T2w fat-suppressed T2 w

8. FINDINGS • Coronal T1 w image of the suprapatellar pouch of the knee shows the villous lipomatous proliferation of the synovium resembling a real branched tree. • Axial T2w image shows diffuse proliferation of synovium from suprapatellar pouch on both sides with the same signal intensity as the subcutaneous fat. • Corresponding fat-suppressed T2 w image shows the low signal intensity of the lesion similar to fat. • Sagittal T1w image shows diffuse synovial villous proliferation, similar to fat, with a small bony erosion and a synovial cyst.

9. DIFFERENTIAL DIAGNOSIS • Lipoma arborescens • Pigmented villonodular synovitis • Synovial lipoma • Synovial chondromatosis • Synovial haemangioma

10. Pigmented villonodular synovitis (PVNS) • Benign proliferative disorder of uncertain etiology that affects synovial lined joints, bursae, and tendon sheaths. • Two primary forms :- • diffuse form affects the entire synovial lining of a joint, bursa, or tendon sheath ,typically involves the large joints. • localized form typically occurs around the small joints of the hands and feet (most commonly in the fingers, in particular, the volar aspect of the first 3 fingers). Rarely, around large joints. • The joint most affected is the knee (80%), with the hip, ankle, and shoulder affected less commonly. The disease usually is monoarticular.

11. Plain radiographs • Signs similar to joint effusion or soft tissue swelling. Calcifications are not a usual feature of PVNS. Rarely, a focus of dystrophic calcification may be seen in an area of PVNS. CT • Secondary to the presence of intracellular and extracellular hemosiderin, lesions have high attenuation and appear hyperdense on CT scans. • CT is valuable in delineating bone cysts and erosions because of improved tissue contrast

12. Nodular intraarticular masses that demonstrate low SI on T1-w, T2-w & PDW sequences.Low signal intensity is due to hemosiderin deposits within the affected tissue.

13. Coronal T2w - low signal mass above the medial femoral condyle posteriorly.Sagittal T1w- inhomogeneous foci of low signal in the suprapatellar pouch and posteriorly just above the femoral condyle.

14. Presence of fat signal also is apparent secondary to the presence of lipid-laden macrophages, resulting in focal regions of high signal intensity on T1-weighted images and intermediate signal on T2-weighted images. • Hyperplastic and hypervascular synovium enhances following intravascular administration of gadolinium chelates.

15. Axial T1W and T2W MRI at level above the knee joint - mixed signal pattern in the suprapatellar pouch. Nodular low-signal foci are interspersed within inhomogeneous low and high signal areas . The foci correspond to areas of hemosiderin-laden hyperplastic synovium of pigmented villonodular synovitis.

16. Synovial Chondromatosis PLAIN RADIOGRAPHS– • Multiple small rounded calcified bodies seen around the joint in the suprapatellar portion with small amount of joint effusion. MRI - • These bodies follow the marrow signal . Synovial chondromatosis shows an intermediate-low signal on T1- and T2-weighted images, related to the cartilaginous nature of the lesion.

18. T1w

19. T2w

20. Synovial hemangiomas • Intermediate signal intensity on T1- and T2-weighted images with areas of low signal intensity due to phleboliths or fluid void within a linear punctate lesion of high signal intensity corresponding to fibrous fatty septa between the vascular channels.

21. Synovial lipomas • Rare and occur almost exclusively in the knee joint, producing nonspecific symptoms. • They are solitary round to oval masses of mature fat located intra-articularly, with a covering synovium having a round or oval contour. • On plain films, synovial lipomas appear as a swelling about the knee, and fat may be seen. • Arthrographydemonstrates a smooth, lobular synovial mass. • CT or MR imaging confirms the fatty nature of the tumor. synovial lipoma are mainly found in the infrapatellar fat pad.

22. PVNS- a low on Tl & T2w and on CT shows enhancement of the mass on CECT. • Synovial chondromatosis - Low to intermediate signal intensities on T1- and T2-weighted images These bodies follow the marrow signal • Synovial haemangiomas - Intermediate signal intensity on T1- and T2-weighted images with areas of low signal intensity due to phleboliths or fluid void • Synovial lipomas - fatty nature of the tumor having a round or oval contour. • Lipoma arborescens usually arises in the suprapatellar pouch, whereas synovial haemangioma and synovial lipoma are mainly found in the infrapatellar fat pad.

23. DIAGNOSIS • Lipoma arborescens

24. DISCUSSION • Lipoma arborescens is a rare benign intrarticular lesion characterized by the replacement of the subsynovial tissue by mature fat cells giving rise to a villous proliferation. • The term arborescens, from the Latin word arbor meaning tree, defines the characteristic morphology of this lipomatous villous synovial proliferation, i.e. resembling a tree in appearance. • Clinically the most common finding is a slow increase in painless swelling, accompanied by intermittent effusion of the joint. • Most affected patients are in the fifth to seventh decades of life.

25. It is monoarticular in distribution and is most commonly seen at the knee joint. • The condition is very rare in other joints (glenohumeral joint, sub deltoid bursa, hip and elbow). It is virtually unknown outside the joint capsule. • The underlying bone is usually normal. • The precise etiology is unknown, but it is most likely a non-specific synovial reaction to trauma and inflammation. It can be associated with degenerative joint disease and chronic rheumatoid arthritis. • Bilateral involvement of the knees, wrists, ankles and hips and involvement of multiple joints has also been observed .

26. On plain radiographs it appears as soft tissue swelling which may or may not be radiolucent. • Arthrography reveals multiple filling defects. • Ultrasound shows a hyperechoic, frond like mass in the suprapatellar bursa with a large associated effusion. The frondlike mass shows bending and waving in real time. • MR findings include a large frond like mass arising from the synovium with a signal intensity similar to that of fat on all sequences, and an associated effusion. There is no evidence of haemosiderin deposition.

27. CASE 2

28. HISTORY • 20 years old female patient presented with • c/o breathlessness • c/o right upper quadrant pain with jaundice • c/o steatorrhoea

29. Severe cystic bronchiectasis bilaterally.

31. Oblique US image of the right upper quadrant shows multiple shadowing calculi (arrows) in the intrahepatic bile ducts.

32. Endoscopic retrograde cholangiopancreatogram shows strictures (white arrowhead) and dilatation (black arrowhead) of the bile ducts. Multiple filling defects (arrows) are also noted.

33. Axial CECT images, obtained at the levels of the pancreatic tail and pancreatic head in a 32 yr man with CF, show replacement of the pancreatic parenchyma by fat. Splenomegaly is also present.

34. DIAGNOSIS • Thus a patient with severe bronchiectatic changes in chest, multiple stricures & dilatation of IHBR with multiple calculi within, with fatty replacement of pancreatic parenchyma-Cystic fibrosis

35. SYSTEMIC MANIFESTATIONS OF CYSTIC FIBROSIS

36. PLAIN X-RAY • Early: • May be completely normal. • Airway thickenig and hyperinflation: earliest findings • Later: • Bronchial wall thickening and nodular or tubular densities owing to mucoid impaction. • most marked in upper lobes. • Advanced: segmental and lobar collapse and lung cysts, barrel shaped chest. • Hilar enlargement may be seen due to either hilar lymphadenopathy or due to pulmonary arterial hypertension. • Increased risk of ABPA

37. Bilateral bronchiectasis with hilar prominence & Diffuse coarse interstitial opacities

38. CT • Bronchiectasis • Peribronchial thickening • Bronchiectatic sacculations (bronchus directly leading into sacculation) • Bullae • Emphysema • Tubular and branching mucus plugs • Subsegmental atelectasis and consolidation • “Target lobe” may be present where bronchiectasis & cyst formation is maximal, infections recurrent.

39. Hepatobiliary manifestations • Liver disease is the second leading cause of death in patients with CF . • Overall 40% of CF patients develop liver disease, but only 1%–5% of these cases progress to portal hypertension and end-stage liver disease. • Pathophysiology : multifactorial. It has classically been attributed to abnormally thickened secretions that accumulate within bile ducts, slowing biliary flow and concentrating caustic bile components in the hepatic tissues. • Fatty infiltration of the liver has been reported in 30% of all CF patients.

40. 19 yr woman with CF. Axial CECT image - diffuse low attenuation of the liver s/o marked fatty infiltration of the liver (arrow).

41. Biliary abnormalities range from cholelithiasis and sludge to ductal strictures and sclerosing cholangitis. • A common abnormality is the microgallbladder. • It is likely related to inspissated mucus and/or an inflammatory response resulting in atresia or stenosis of the cystic duct. • Gallbladder wall thickening is nonspecific, especially in the setting of cirrhosis, ascites, hypoalbuminemia, and nutritional deficiencies.

42. Intra- and extrahepatic bile duct abnormalities include strictures, beading, dilatation, and ductal calculi • Abscesses can occur. • Focal biliary fibrosis is considered a characteristic histopathologic lesion in CF . • Related to thick secretions and/or an inflammatory response within the intrahepatic bile ducts. • The classic sonographic appearance is hyperechoic periportal thickening due to fibrosis or focal fat, with diffuse increased hepatic echogenicity.

43. 29 yr man with CF. Oblique US image of the right upper quadrant -multiple shadowing calculi (arrow) and mild diffuse thickening of the G.B. wall (arrowhead).

44. 20 yr man with CF. Transverse US image of the right upper quadrant shows diffusely increased hepatic echogenicity with periportal increased echogenicity (arrows).

45. Cirrhosis is estimated to occur in 5%–15% of CF patients, and progression to portal hypertension occurs in 1%–8%

46. Axial CECT image- multiple small varices in the omentum (arrows) and surrounding the stomach (white arrowhead). Note the fatty replacement of the pancreas (black arrowhead).

47. Pancreatic Manifestations • Exocrine gland insufficiency affects 85%–90% of all CF patients , leads to malabsorption, glucose intolerance • Result of inspissated secretions leading to proximal duct obstruction with subsequent acinar disruption and replacement by fibrous tissue and fat. • Complete fatty replacement is the most common pancreatic finding at imaging in adult CF patients. • Sonographic findings include hyperechoic and atrophic pancreatic parenchyma. • MR : increased signal intensity on T1-weighted images reflecting fatty change and intermixed low signal intensity on T1-weighted images representing fibrosis

48. Pancreatic calcifications occur in roughly 7% of patients. • They are usually found along the course of pancreatic ducts, are variable in both size and shape. • Often difficult to detect with MR imaging

49. Pancreatic cysts : relatively common in CF patients, usually small, measuring 1–3 mm • Aggregates of true epithelium-lined cysts may completely replace the pancreas c/as pancreatic cystosis. • It is thought to occur when the functional secretory capacity of the gland is maintained in the presence of ductal obstruction.

50. 16 yr girl with CF. Axial T2w single-shot FSE MR image - complete replacement of the pancreatic parenchyma by innumerable cysts (arrows) s/o pancreatic cystosis.