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Choledochal cyst

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Choledochal cyst

  1. 1. Choledochal cyst Dr Manmohan Bir Shrestha For RADIOLOGY
  2. 2. Objectives Introduction Biliary tree anatomy Classification Etiology Clinical presentation Laboratory investigations Imaging modalities Prenatal diagnosis Caroli’s disease Differentials Treatment Complications
  3. 3. Choledochal cyst • Congenital anomaly involving cystic dilation of various ducts of the biliary tract.
  4. 4. Epidemiology • Incidence • Western population- 1 in 100,000 to 150000 live births • More common in Asia • 1 in 1000 live births in Japan • Sex prevalence Female dominance- • 3-4:1
  5. 5. Biliary tree anatomy Intrahepatic Ductular and canalicular network of the acini Interlobular ducts Septal bile ducts RHD & LHD Extrahepatic RHD & LHD CHD Cystic duct & GB CBD
  6. 6. Normal size of bile ducts • CBD • Adolescents & adults • Upto 8mm • Post cholecystectomy • Upto 12 mm • >60 years of age • +1mm per decade • Cystic duct • Upto 1.8 mm CHD at porta hepatis- 5 mm
  7. 7. Todani’s classification of choledochal cyst
  8. 8. Types • I a- dilation of extrahepatic bile duct (entire) • Ib- dilation of of extrahepatic bile duct (focal) • Ic- dilation of CBD portion of extrahepatic bile duct • II- diverticulum of the CBD • III- cystic dilation of the distal intraduodenal CBD • IVa-multiple cysts intrahepatically & extrahepatically • IVb- multiple cysts extrahepatically • V- single or multiple intrahepatic cysts without extrahepatic duct dilation.
  9. 9. 1st Qtr 2nd Qtr 3rd Qtr 4th Qtr Type IV Type V Occurrence Type I – 61 % Type II – less common Type III – less common Type IV - 28.5 % Type V – 4.6%. Type I Type II & III
  10. 10. Types of union between CBD+MPD • Normal junction (union inside duodenal wall) 1) 2-10 mm short common channel with a diameter of 3-5 mm(55-85%) 2) Separate entrances into duodenum(42%) 3) 8-15 mm long common channel • Abnormal junction (union outside duodenal wall) 1) MPD inserting into CBD >15 MM from entrance into duodenum 2) CBD entering into MPD
  11. 11. Etiology • Weakness of the duct wall • Pancreaticobiliary maljunction / Long common channel o Proximal insertion of MPD into CBD or vice versa outside duodenal wall producing a long common channel (>15 mm) o Reflux of pancreatic enzymes into CBD that causes damage to the ductal wall and leads to cyst formation
  12. 12. Clinical presentation • Triad of – • Abdominal pain • Jaundice • Right upper quadrant palpable mass Infantile form • Present before 12 months of age • Present with obstructive jaundice, acholic stools & hepatomegaly • Signs of hepatic fibrosis may be present Adult form • Anytime after 12 months of age • May present with fever, nausea, vomiting & jaundice. • Undiagnosed cases may present with cholelithiasis.
  13. 13. Laboratory investigations • Increased bilirubin • Increased alkaline phosphatase
  14. 14. Histopathology • Ducts are thickened (connective tissue+smooth muscle strands) • Inflammatory reaction increases with age, may lead to ulceration in mucosa & submucosa • May lack a typical biliary mucosal lining • Repeated destruction and regrowth causes epithelial metaplasia • High risk of adenocarcinoma
  15. 15. Imaging modalities • USG • CT Scan • CT cholangiography • MRI • MRCP • ERCP • PTC • HIDA Scan
  16. 16. Objectives of imaging • To classify • Mention extension • To visualize pancreaticobiliary junction • To rule out association with other diseases • To search for associated complications • To rule out differentials
  17. 17. USG • Ballooned/fusiform cyst beneath porta hepatis separate from GB • *Communication with bile duct needs to be demonstrated* • Abrupt change of caliber at junction of dilated segment to normal ducts • Intrahepatic bile duct dilatation secondary to stenosis.
  18. 18. CT Scan • Useful in identifying type IV & type V disease • CT Cholangiography • More sensitive for visualizing biliary tree • More sensitive in detecting choledochal cysts • Less sensitive to characterize pancreatic duct
  19. 19. MRI • T1WI : hypointense dilatation of bile duct • T2WI : hyperintense
  20. 20. T1WI
  21. 21. T2WI
  22. 22. Type III choledochal cyst in coronal T2WI
  23. 23. MRCP • Gold standard for imaging choledochal cyst • Administration of secretin increases pancreatic secretion and dilates the pancreatic duct
  24. 24. Type II ( diverticulum) choledochal cyst arising from lateral wall of CBD
  25. 25. ERCP • More useful in type III choledochal cyst to exclude periampullary mass Fusiform choledochal cyst with a long common channel & stricture at pancreatico biliary junction
  26. 26. Percutaneous transhepatic cholangiogram(PTC) Cholangiogram – Caroli’s disease with characteristic strictures and segmental intrahepatic duct dilations.
  27. 27. HIDA Scan • The normal scan provides functional & morphological information about the hepatic parenchyma in the first 10min ( after inj. of radionuclide), extrahepatic biliary tree by 20 min and excretion into the bowel by 1 hour. • Useful in demonstrating biliary peritonitis after choledochal cyst rupture
  28. 28. Prenatal diagnosis Ultrasound Earliest diagnosis at 25 weeks MRI
  29. 29. Ultrasound D/D 1) Duodenal atresia 2) Cyst of -ovary -mesentery -omentum -pancreas -liver
  30. 30. Caroli’s disease / Type V Choledochal cyst • Rare congenital disease • Autosomal recessive disorder • Characterized by multifocal segmental saccular cystic dilatation of the large intrahepatic bile ducts, which retain their communication with the biliary tree • May be diffuse / segmental / lobar
  31. 31. • Types 1)Simple form 2)Caroli’s syndrome • associated with periportal hepatic fibrosis
  32. 32. Etiology- Ductal plate malformation ?perinatal hepatic artery occlusion ?hypoplasia/aplasia of fibromuscular wall components Association- Benign renal tubular ectasia Medullary sponge kidney(80%) Infantile polycystic kidney disease Congenital hepatic fibrosis
  33. 33. saccular dilatation of the intrahepatic biliary ducts
  34. 34. Central dot sign -enhancing fibrovascular bundle of portal vein radicles completely surrounded by dilated bile ducts
  35. 35. Multiple dilated intrahepatic ducts with tiny dots of contrast representing portal radicles
  36. 36. D/D of Caroli’s disease Polycystic liver disease No associated biliary duct dilatation Rarely communicate with biliary ducts Biliary hamartomas Cyst size < 5mm (generally) Do not communicate with biliary tree Primary sclerosing cholangitis Dilatation more fusiform & isolated Associated inflammatory bowel disease Recurrent pyogenic cholangitis Saccular dilatation favors Caroli’s disease Obstructive biliary dilatation
  37. 37. Type I Type II Type III Type IV Type V
  38. 38. D/D of Choledochal cyst • Duodenal diverticulum • Pancreatic pseudocyst / cystic tumors • Other causes of biliary tree dilatation
  39. 39. Causes of Biliary Obstruction Benign miscellaneous Choledocholithiasis Haemobilia Congenital biliary disease  Choledochal Cyst  Biliary Atresia Infectious cholangitis Sclerosing cholangitis Neoplasms Cholangiocarcinoma GB carcinoma Locally invasive tumors Ampullary tumors Metastases Extrinsic compression Mirizzi syndrome Pancreatitis Adenopathy
  40. 40. Treatment • Type I & IV • Cyst excision & hepaticojejunostomy to a Roux-en-Y limb • Type II • Simple cyst resection • Type III • Marsupialization of the cyst , usually through transduodenal approach • Type V • Segmental resection of liver if disease is localized • Liver transplantation
  41. 41. Complications • Stones in GB, CBD, within cyst, in MPD, in intrahepatic biliary tree • Malignant transformation • Recurrent pancreatitis • Cholangitis/Cholecystitis • Cyst rupture with bile peritonitis • Bleeding • Biliary cirrhosis + portal hypertension • Portal vein thrombosis • Hepatic abscess
  42. 42. References • Textbook of Radiology & Imaging - David Sutton 7th Edition • Diagnostic Ultrasound - Carol M. Rumack 4th Edition • Radiology Review Manual - Wolfgang Dahnert 7th Edition • Bailey & Love’s Short Practice of Surgery - 7th Edition • Paediatric Surgery - Arnold G. Coran - 7th Edition

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