Uploaded byDr Ritesh Dhanbhar
Choledochal cyst
Choledochal cysts are congenital dilations of the bile duct that can be extrahepatic or intrahepatic. There are several proposed causes including abnormalities in the common bile duct that allow pancreatic juices to damage the duct wall. Histologically they show chronic inflammation and metaplasia. Todani classification divides choledochal cysts into 5 types based on location and extent of dilation. Clinical features include jaundice, abdominal pain, and palpable mass. Diagnosis involves imaging like ultrasound, CT, MRCP and biopsy. Treatment involves surgical excision and reconstruction, with more extensive procedures like liver transplantation needed for severe cases.
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Presentation by Dr. Ritesh Dhanbhar.
Defined as congenital dilatation of the biliary tree, either isolated or combined.
Discusses theories explaining cyst formation, including the Babbitt theory and embryogenesis.
Features include chronic inflammation and metaplasia, with two histological types identified.
Outlines Todani classification: Types I to V, describing different forms of biliary tree dilatation.
Common symptoms, particularly in females and Japanese populations; highlights obstructive jaundice.
Various diagnostic methods including US, CT, ERCP, and MRCP to assess biliary anatomy.
Details treatment for each type, emphasizing surgical interventions and necessity of cholecystectomy.
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Choledochal cyst
- 1.
- 2. Definetion Defined as isolated/focalor combined/diffuse congenital dilatation of extra or intrahepatic biliary tree.
- 3. Etiology 1.Babbitt theory : Thereis long common channel more than 2 cm. It causes reflux of pancreatic juice into the bile duct, enzymatic destruction of the bile duct wall, ductal wall weakening and dilatation. 2. During embryogenesis, there is abnormal early canalisation of bile duct with distal obstruction causing increased proximal pressure, weakening and ductal dilatation. 3. Reduced postganglionic autonomous neurons in the distal portion of the cyst causing its poor function distally.
- 4. Histology 1. Histological featuresare Chronic inflammation, sparse mucin glands and metaplasia. 2. Histological types A. Glandular with normal cuboidal epithelium with cavities in the mucosa. B. Fibrotic type with fibrous cyst wall with thickened bile duct.
- 5. Types (Todani Modificationof Alonso-Lej Classification) Type I: Dilatation of extrahepatic biliary tree (60%). • Type Ia—cystic. • Type Ib—focal segmental (saccular). • Type Ic—fusiform.
- 6. Type II: Diverticulumof extrahepatic biliary tree. Type III: Choledochocele— cystic dilatation of intraduodenal part of CBD .
- 7. Type IV: Dilatationof extra- and intrahepatic or multiple parts of extrahepatic biliary tree (30%). • Type IVa: Dilatation of extrahepatic and intrahepatic biliary tree. It is 2nd most common. • Type IVb: Dilatation of multiple sections of the extrahepatic bile duct.
- 8. • Type V:Dilatation of the only intrahepatic biliary tree(Caroli’s disease).
- 10. Clinical feature • Commonin female • Common in Japan • Obstructive jaundice (80%), pain in right hypochondrium,cholangitis. • Mass per abdomen—mass is to the right and above umbilicus, smooth, soft, not moving with respiration, not mobile and resonant. • Chield Failure to thrive.
- 11. Triad of choledochalcyst 1. Right upper quadrant pain 2. Jaundice 3. Palpable abdominal mass
- 12. Diagnosis • US abdomen—unilocularcyst mainly in infants. • CT scan—mainly to see intrahepatic biliary system. • ERCP, Cholangiography—to see ductal anatomy. • MRCP—to see status of pancreatic and biliary system and pancreaticobiliary maljunction–ideal. • Liver function tests. • PTC to see intrahepatic biliary tree.
- 14. Treatment • In typeI—excision of cyst with its mucosa , cholecystectomy and reconstruction by Roux-en-Y hepaticojejunostomy. • In type II—excision of the diverticulum, cholecystectomy and either suturing of the CBD wall or Roux-en-Y hepaticojejunostomy can be done. • In type III—endoscopic sphincterotomy is done. Excision is also often needed. Intrahepatic dilatation is difficult to treat. If it is localised, hepatectomy is sufficient, but if it is diffuse, liver transplantation may be required.
- 16. • In typeIV if cyst is adherent to portal vein posteriorly, that part of the cyst wall over the portal vein is left behind. But mucosa of the part should be removed (Lily’s operation). • Type IVa-- Cholecystectomy and Roux-en-Y hepaticojejunostomy , liver transplantation. • Type IVb-- Resection of extrahepatic biliary tree with removal of choledochal cyst along with cholecystectomy and Roux- en-Y hepaticojejunostomy. • Type V: Cholecystectomy , liver transplantation
- 18. • Cholecystectomy isa must in all types. • If cyst has already turned into malignancy— adenocarcinoma, then radical surgery and chemotherapy is given. • Liver transplantation is the choice in type IV and V cases.