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CHOLEDOCHAL CYST

CHOLEDOCHAL CYST. OCTOBER 2010. BACKGROUND. • Congenital anomalies of the bile ducts. • First found western literature 1723 (Vater and Ezler). • First systematic description of 96 cases in 1959. • Classification of 5 types (Todani 1977). PATHOPHYSIOLOGY. • Multifunctional

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CHOLEDOCHAL CYST

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  1. CHOLEDOCHAL CYST OCTOBER 2010

  2. BACKGROUND • Congenital anomalies of the bile ducts. • First found western literature 1723 (Vater and Ezler). • First systematic description of 96 cases in 1959. • Classification of 5 types (Todani 1977)

  3. PATHOPHYSIOLOGY •Multifunctional • Majority of choledochal cysts have anomalous junction of common bile duct with pancreatic duct (90%). • This allows reflux of pancreatic secretions and enzymes into common bile duct. • Results in inflammation and weakening of bile duct wall. • Formation of choledochal cyst.

  4. FREQUENCY • Rare in US and western countries. • Reported 1 case per 2 million live births. • Prevalent in Asia 33% cases Japan = 1:1000. • Females 3-4:1

  5. MORTALITY/MORBIDITY • Infants and children – pancreatitis, cholangitis and histological evidence of hepatocellular inflammation and damage. • cholangiocarcinoma 9-28%.

  6. CLASSIFICATION (TODANI 1977) • Type I 80-90% • Type II • Type III • Type IV • Type V

  7. TYPE I CHOLEDOCHAL CYST • Dilation of entire common hapatic and common bile duct or segments of each. • Sacular or fusiform.

  8. TYPE II CHOLEDOCHAL CYST • Relatively isolated protrusion that project from common bile duct wall. • Connected by narrow stalk.

  9. TYPE III CHOLEDOCHAL CYST • Found in intraduodenal portion of the common bile duct (choledochocade).

  10. TYPE IV CHOLEDOCHAL CYST • Multiple dilations of the intrahepatic and extrahepatic biliary tree. • Large extrahepatic cyst with multiple intrahepatic cysts.

  11. TYPE V CHOLEDOCHAL CYSTS • Dilation of intraphepatic biliary radicles. • Numerous cysts with structures causing intraphepatic stone formation, obstruction and cholangitis.

  12. SYMPTOMS • Jaundice • Pale stools (putty colour) • Palpable mass right upper abdominal quadrant • Hepatomegaly • Acute pancreatitis

  13. INVESTIGATIONS • USS abdomen • CT (93%) or MRI (100%) + cholangiogram • HIDA scan • Plain AXR do not identify choledochal cysts well.

  14. TREATMENT TYPE I • Surgery • Complete excision with Rovx-en-y biliary end to side anastromosis to restore biliary continuity to gastrointestinal track.

  15. TREATMENT TYPE II • Total excision

  16. TREATMENT TYPE III • 3 cm or less endoscopic removal. • >3 cm surgical excised.

  17. TREATMENT TYPE IV • Dilated extraphepatic duct removed.

  18. TREATMENT TYPE V • Liver Transplantation

  19. REFERENCES • Btaiche I and Khalidi N (2002). Parenteral Nutrition - Associated Liver Complications in Children. Pharmacotherapy. 22(2): 188- 211 • Howard E, Stringer M and Colombani P (2002) (2ND Ed). Surgery of the Liver, bile ducts and Pancreas in Children. Arnold. London • Piwko J, Caty M, Ryan R (2003). Neonatal considerations for the Paediatric Surgeon. www.emedicine.com/ped/topic2982.htm • Puri P (2003) Newborn Surgery (2nd Ed). Arnold. London • Sadiq J, Nondi B, Lakhoo K (2009). An unusual variant of choledochal cyst : a case report. Journal of Medical Case Reports. 3 : 54

  20. REFERENCES CONT. • Sawyer M, Varma M, Murphy T (2009). Choledochal Cyst. http://emedicine.medscape.com/article/366004-overview • Strodtbeck F(2003) The pathophysiology of prolonged periods of No Enteral Nutrition or Nothing by Mouth. Newborn and Infant Nursing Reviews. www.medscape.com/viewarticle/458473 • Subramanian K, Yoon H and Toral J (2002). Extremely Low Birth Weight Infant. www.emedicine.com/ped/topic2784.htm • Tortora G and Grabowski S (1993) (7TH Ed). Principles of Anatomy andPhysiology. Harper Collins College Publishers. New York Pp790

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