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home - Liver - Liver Masses - Choledochal cysts Written by Dr Sebastian Zeki

Knows the epidemiology pathology clinical presentation and natural
history of benign tumours of the liver

Can define a programme of investigation and characterisation of
benign liver lesions including haemangioma focal modular
hyperplasia and adenoma

Demonstrates ability to make an appropriate differential diagnosis
Formulates appropriate plan of management
Recognises importance of the role of multidisciplinary team in
diagnosis and management



Understands the epidemiology risk factors pathology prevalence
and range of presentations of HCC

Knows the appropriate investigation and staging of the disease
Aware of treatment options including trans-arterial
chemoembolisation (TACE) radiofrequency ablation (RFA) local
ethanol injection

Appreciates the indications and contraindications of each and how
the most appropriate is selected Aware of surgical treatment options

Aware of role of surveillance and referral for specialist multidisciplinary management including liaison with oncology

Appreciates the indications and contraindications of each modality of
treatment and how the most appropriate is selected

Understands the process of selection of patients for liver resection or

Appreciates Involvement of multi-disciplinary team in management
decisions close liaison with surgical radiology oncology and
pathology colleagues

Choledochal cysts

Pathology Features: A range from normal bile duct mucosa to carcinoma exists.In children -a fibrotic cyst wall + chronic and acute inflammation.In adults- erosions, sparse mucin glands, metaplasia.Malignancy -usually presents as a posterior cyst wall. Pathogenesis CT/ Cholangiography/ EUS -for extrahepatic biliary cysts / cyst wall and pancreaticobiliary junction/ Intraductal ultrasound (IDUS) Classification types:Type I- cystic dilatation of CBD- this is the most common -75 % of all biliary cysts.Type IA which is associated with APBJ.Type IB which does not have an APBJ.Type IC -assoc. with APBJ and continuous dilatation of the common duct into the intrahepatic ducts. Biliary Cysts (Choledochal cysts) Type II (rarest) is a true diverticulum of the extrahe-patic bile duct- proximal to the duodenum. Type III- cystic dilatation of intraduodenal portion of the distal CBD (= choledochocele).Lined by duodenal(usually)/biliary epithelium- may arise embryologically as duodenal duplications involving the ampulla.Type IIIA (in which the bile duct and pancreatic duct enter the choledochocele, which then drains into the duode-num at a separate orifice) Type IIIB (a diverticulum of the intraduodenal bile duct or common channel) Type IV includes cases of multiple cystsType IVA- both intrahepatic and extrahepatic cystic dilations- 2nd most common type of biliary cyst.Can be differentiated from Type IC cysts on the basis of a distinct duct caliber change or stricture at the hilum.Type IVB-multiple extrahepatic cysts +no intrahe-patic involvement. Type V includes isolated or multiple cystic dilatations of the intrahepatic ducts without extrahepatic duct disease, giving an appearance similar to Caroli's disease. (=pancreaticobiliary maljunction (PBM))Prevalence of 0.03 %In 70 % of biliary cysts pts -risk factor for malignancy in the biliary cyst.APBJ with no biliary cyst are at increased risk for gallbladder cancer.Complications:Pancreatic juice reflux, (ductal junction is outside of the duodenal wall)- can cause biliary epithelial damage, inflammation, ductal distension, and cyst formation.Proteinaceous plugs/ stones within dilated common channel may cause ductal obstruction and pancreatitis, and cyst resection alone may not resolve the patient's symptoms- may need sphincterotomy (a) Normal anatomy with a short common channel (arrow) and biliopancreatic junction within the duodenal wall. (b) Abnormal pancreaticobiliary junction type I, with a long common channel and dominant common bile. (c) Abnormal pancreaticobiliary junction type II, with a long common channel and dominant pancreatic duct EpidemiologyIt has an incidence of 1:100,000.It is more prevalent in Asians.M:F incidence is 3:1. Clinical ManifestationsThe triad of pain, jaundice, and abdominal mass presents in 50% of infants.Chronic intermittent abdo. pain occurs in 80% if >2yrs.Recurrent jaundice/cholangitisoccurs in 40%.Abdominal mass occurs in 15%.Pancreatitis occurs in 20%.Biliary lithiasis occurs in 8 %.Pancreatitis and ductal stones are more commonly encountered with choledochoceles. ManagementSurgical resection may be needed in Types I, II, and IV as there is a cancer risk.If extrahepatic cysts present,may need hepaticojejunostomy after resection for reconstruction.A partial hepatectomy may be indicated if intrahepatic cysts are present and resectable.Surgical resection effectively treats symptoms and decreases cancer risk. Hepaticojejunostomy: stenosis of the biliary-enteric anastomosis (in 25%) leading to cholangitis, jaundice, or cirrhosis.Monitor with yearly LFT’s- treat if further big ALP elevations even if no SxIntrapancreatic remnant of a Type I cyst can develop stones and malignancyConsider cholecystectomy in APBJ as inc gallbladder cancer risk.Type IIIA cysts (choledochoceles) are often amenable to endoscopic sphincterotomy.Type IIIB cysts (diverticular choledochoceles) may be resected surgically or endoscopically.No effective surveillance for dysplasia or intra-mucosal cancer. Surgical complications: No one mechanism is responsible. Theories include:-Unequal proliferation of embryologic biliary epithelial cells-Fetal viral infection may also have a role-Cyst formation may be the result of ductal obstruction or distension during the prenatal or neonatal period. Diagnosis: Abnormal pancreaticobiliary junction APBJ Written by Dr Sebastian Zeki

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