Choledochal cyst
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Choledochal cyst is a rare congenital dilatation of the bile duct that occurs in 1 in 100,000 people. It is more common in Asian populations and women. The pathogenesis is typically due to an anomalous pancreaticobiliary junction, where the pancreatic and biliary ducts fuse before entering the duodenum. This allows pancreatic secretions to reflux into the bile duct and cause inflammation and cystic degeneration. Choledochal cysts are classified into 5 types based on their location and extent. Patients typically present with jaundice, right upper quadrant pain, or a palpable mass. Investigations include ultrasound, CT, MRCP and ERCP to establish the diagnosis and classification. Complications include
Choledochal cyst
- 2. EPIDEMIOLOGY Less than 1 in 1,00,000 Common in Asian descent Common in women 8:1
- 3. PATHOGENESIS Presence of anomalous pancreatic biliary junction(APBJ) it is seen in 90% choledochal cysts In APBJ pancreatic duct and biliary duct fuse to form common channel prior to opening in duodenal wall Pancreatic secretions reflux into bile duct which inflame and damage the biliary tree leading to cystic degeneration
- 4. CLASSIFICATION Originally classified by Alonso – Lej Latter modified by Todani
- 5. Type I – extra hepatic fusiform dilatation most common 60% Type II - sacular diverticulum of common bile duct seen in 5% Type III- cystic dilatation of intramural bile duct with in walls of duodenum Type IVa - multiple cystic dilatations in both intra hepatic and extra hepatic bile ducts Type IVb - multiple cysts limited to extra hepatic biliary tree seen in 30% Type V - multiple intra hepatic cysts only sometimes may be solitary also known as Caroli disease
- 6. PRESENTATION Jaundice Right upper quadrant pain Rarely palpable mass Other symptoms like nausea, pruritis, weight loss, cirrhosis, cholangitis pancreatitis, hepatic fibrosis, malignancy are reported during presentation Rarely rupture of cyst and bile peritonitis can also be presented
- 7. INVESTIGATIONS Usually diagnosed when imaging is done for non specific symptoms Ultrasound – dilated cystic lesion which communicates with the bile duct and is separate from the gall bladder CT scan – diagnosis is suspected MRCP – classification can also be done ERCP – distal bile duct can also be analysised which is difficult in MRCP Liver function tests – identify cholestatis jaundice secondary hepatic injury cirrhosis
- 8. COMPLICATIONS Pancreatitis Supparative cholangitis Gall stone and CBD stone formation Rupture of cysts Cholangiocarcinoma – seen in 10% to 30% cases malignant cystic degeneration is common due to chronic mucosal irritation from refluxed pancreatic enzymes
- 9. TREATMENT Resection of entire cyst and appropriate surgical reconstruction Type I – complete surgical excision Cholecystectomy and Roux-En-Y Hepaticojejunostomy entire dilated part is removed
- 10. Type II – completely excised if APBJ is involved enteric diversion with Roux-En-Y Hepaticojejunostomy is done Type III – transduodenal approach for transduodenal excision or sphincteroplasty in case of any duodenal or biliary obstruction Type IV a – involving one lobe partial hepatectomy Type IV b - extra hepatic cysts treatment as type I i.e complete surgical excision Cholecystectomy and Roux-En-Y Hepaticojejunostomy Type V or Caroli’s disease – range from resection of liver if disease is unilobular to liver transplantation if disease is diffuse