CASES

1. CASES Dr Payal Damor R2 Radiodiagnosis S.S.G.H BARODA 24-12-08

2. CASE1

3. HISTORY • 32 years old male patient presented with • c/o breathlessness • c/o right upper quadrant pain with jaundice • c/o steatorrhoea

4. Severe cystic bronchiectasis bilaterally.

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

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

8. 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.

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

10. SYSTEMIC MENIFESTATIONS OF CYSTIC FIBROSIS

11. PLAIN X-RAY • Early, may be completely normal. • The earliest CXR findings are airway thickening & hyperinflation. • With progressive disease, the bronchial wall becomes thickened & nodular or tubular densities appear in lungs, owing to mucoid impaction. • The abnormalities most marked in upper lobes. • Advanced CF is characterized by segmental & lobar collapse & lung cysts. • Hilar enlargement may be seen due either Hilar lymphadenopathy or due to pulmonary arterial hypertension.

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

13. CT • Bronchiectasis • Peribronchial thickening • Bronchiectatic sacculations (bronchus directly leading into sacculation) • Bullae • Emphysema • Tubular & branching mucus plugs • Subsegmental atelectasis & consolidation

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

15. 19 yr woman with CF. Axial CECT image - diffuse low attenuation of the liver s/o marked fatty infiltration of the liver (arrow).

16. Biliary abnormalities range from cholelithiasis & sludge to ductal strictures & sclerosing cholangitis. • A common abnormality is the microgallbladder. • It is likely related to inspissated mucus &/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, & nutritional deficiencies.

17. Intra- & extrahepatic bile duct abnormalities include strictures, beading, dilatation, and ductal calculi. • Abscesses can occur. • Thick secretions &/or an inflammatory response within intrahepatic bile ducts resulting in periductal thickening & fibrosis. • The classic sonographic appearance is hyperechoic periportal thickening due to fibrosis or focal fat, with diffuse increased hepatic echogenicity.

18. 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).

19. 20 yr man with CF Axial CECT image - a focal, round, hypoattenuating lesion with an enhancing rim (arrow) in the anterior segment of the right hepatic lobe, an appearance consistent with an abscess. Lowerdown scan shows dilatation of an intrahepatic bile duct (arrow) in the left lobe.

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

21. Cirrhosis is estimated to occur in 5%–15% of CF pts, & progression to portal hypertension occurs in 1%–8%.

22. 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).

23. Pancreatic Manifestations • Exocrine gland insufficiency affects 85%–90% of all CF pts & is a result of inspissated secretions leading to proximal duct obstruction with subsequent acinar disruption & replacement by fibrous tissue & fat. • Complete fatty replacement is most common pancreatic finding at imaging in adult CF pts. • Sonographic findings include hyperechoic & atrophic pancreatic parenchyma. • At MR imaging, the degree of fatty replacement will vary, with increased signal intensity on T1-weighted images reflecting fatty change and intermixed low signal intensity on T1-weighted images representing fibrosis.

24. Complete fatty replacement of pancreas. (a, b) Axial CECT images, at levels of pancreatic tail & pancreatic head in a 32-year-old man with CF, show replacement of pancreatic parenchyma by fat (arrows). Splenomegaly is also present. (c) Axial T1-WMR image obtained in a 21-year-old man with CF shows similar findings (arrows).

25. Pancreatic cysts are relatively common in CF pts, are usually small, measuring 1–3 mm. • Occasionally, aggregates of true epithelium-lined cysts completely replace pancreas, a condition referred to as pancreatic cystosis. • It is thought to occur when functional secretory capacity of gland is maintained in presence of ductal obstruction

26. 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.

27. Abnormalities of the pancreatic duct include - Strictures, - Beading, - Dilatation, and - Frank obstruction. • Since most adult CF patients have an atrophic gland with fatty replacement, duct abnormalities can be difficult to demonstrate at imaging.

28. Gastrointestinal Manifestations • Nearly entire digestive tract is affected in pts with CF. • Gastroesophageal reflux is seen in up to 27% of CF pts younger than 5 years. • It is thought to be due to elevated abdominal pressures from chronic cough, hyperinflation, & diaphragm depression & to long-term use of medications that tend to reduce lower esophageal sphincter pressures. • Complications related to chronic reflux disease, namely, esophagitis, strictures, & development of Barrett metaplasia, can be seen in adult. • An increased frequency of peptic ulceration of gastric & duodenal mucosa has been reported.

29. 26 yr man with CF and a history of chronic esophageal reflux and Barrett esophagus. Barium esophagogram shows a distal malignant stricture (arrowhead).

30. Distal intestinal obstruction syndrome • Most common gastrointestinal complication in CF pts. • It is thought to be caused by pancreatic insufficiency, thickened intestinal secretions, undigested food remnants, poor motility, & fecal stasis with resultant impaction of muco-feculent material in the distal ileum & right colon. • Pt presents with colicky abdominal pain, distension, constipation, nausea & vomiting. • A palpable mass noted in RIF due to impaction of muco-feculent material in terminal ileum & right colon. • Radiographs of abdomen will show fecal loaded colon or a bubbly appearance or mass in rt side of abdomen & dilated small bowel loops.

31. Distal intestinal obstruction syndrome in a 37 yr man with CF. Axial CECT images- dilated proximal small bowel loops (arrows in b) with bubbly intraluminal contents (arrowhead in c). A small amount of ascites and mild edema of the small bowel mesentery are present.

32. Intussusception • Another cause of right lower quadrant pain. • It affects approximately 1% of pts but occurs most commonly in older pts. • It is most frequently ileocolic & is related to an inspissated fecal mass that acts as a lead point. • It can present in a similar way to DIOS & delay the diagnosis. • The passage of bloody stool is less common. • Clinical examination can be normal. • Acute appendicitis is somewhat unusual & is reported to occur in only 1%–4% of pts.

33. Ileo-ileal intussusception in a 29 yr man with CF. Axial CECT image - the intussusceptum (arrow) outlined by oral contrast material within the intussuscipiens (arrowhead).

34. Appendicitis in a 25 yr man with CF. Axial CECT image - an enlarged, enhancing tubular structure (arrow) in the right lower quadrant.

35. Fibrosing colonopathy • Colonic stricture is a recognized complication in children &can lead to intestinal obstruction. • It is due to irreversible & sometimes progressive narrowing of bowel lumen. • Right side of colon is most frequently affected. • Pt presents with s/s of distal intestinal obstruction. • US – assess the thickening of colonic wall, • Barium enema – loss of haustration, shortening of colon, narrowing of colonic lumen and nodular thickening of colonic wall.

36. Pneumatosis intestinalis is usually confined to the colon in pts with CF and often coincides with development of obstructive lung disease. • It is thought to result from dissection of air from pulmonary interstitium to perivascular connective tissue planes below diaphragm. • Gastrointestinal malignancies are now more commonly reported with CF.

37. Renal Manifestations • Renal disease is not a common complication of CF. • The most prevalent entity is nephrolithiasis. • Stones are usually composed of calcium oxalate, & pts are often found to have hyperoxaluria, decreased levels of urinary citrate, & depressed urinary volumes.

38. Nephrolithiasis in a 23 yr woman with CF. Axial CECT image - a calcification (arrow) in the right renal collecting system.

39. PULMONARY MANIFESTATIONS • Bronciectasis • Peribronchial cuffing • Atelectasis (RUL predomineance) • Superimposed pneumonia • Hilar lymphadenopathy • Pulmonary arterial hypertension

40. HEPATOBILIARY MANIFESTATIONS • Fatty liver • Hepatic abscesses • Biliary abnormalities range from cholelithiasis and sludge to ductal strictures and sclerosing cholangitis • Microgallbladder. • Strictures, beading, dilatation, and calculi in intra & extra hepatic biliary radicals • Focal biliary fibrosis • Cirrhosis

41. GASTROINTESTINAL MANIFESTATIONS • Distal intestinal obstruction syndrome (DIOS) • Fibrosing colonopathy • Intussusceptions • Appendicitis • Pneumatosis intestinalis • Gastro-oesophageal reflux disease • GI malignancies

42. PANCREATIC MANIFESTATIONS • Fatty replacement of pancreatic parenchyma • Small atrophic pancreas • Calcified pancreas • Pancreatic cystosis • Strictures, Beading, Dilatation, Frank obstruction of pancreatic duct

43. RENAL MANIFESTATION • Nephrolithiasis

44. CASE 2

45. HISTORY • Patient presented with a short history of a left abducens nerve palsy & a left-sided headache.

46. (a) Post-gadolinium axial T1 W & (b) T2 W axial images. There is extensive enhancement within left petrous apex, which is confluent with meningeal enhancement & that in middle ear/mastoid. The T2w image demonstrates an abscess extending superiorly into parasellar region.

47. DIAGNOSIS PETROUS APICITIS

48. So, now let’s discuss lesion of petrous apex.

49. Petrous apicitis: is a rare infection of petrous apex of temporal bone that occurs as an extension of a middle ear or mastoid infection. The petrous portion of temporal bone lies in a complicated anatomic position & has critical relationships to important neural & vascular structures. Consequently, infections arising within or spreading to petrous apex can result in severe clinical sequelae.

50. The petrous apex is the portion of petrous temporal bone: anteromedial to inner ear & lateral to petro-occipital fissure. The internal auditory meatus divides: it into larger anterior (principally consisting of bone marrow or air cells) & a smaller posterior (derived from the dense bone of the otic capsule) compartments.