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THE BILIARY SYSTEM AnatomyBile Duct Intrahepatic bile ducts Glissons system Extrahepatic bile ducts Left and right hepatic ducts common hepatic ducts common bile ducts GallbladderAnatomy The shape of the gallbladder is approximately that of a pear.Its about 8cm long and holds 40 to 60ml of bile.The gallbladder is divided into four sections:fundus,body,neck and cystic duct.The corpus nestles into the substance of the liver.The neck of the gallbladder is lax,because it is not bound to the liver by peritoneum.The distal portion of the gallbladder has the appearance of a diverticulum,which is called Hartmanns pouch.Anatomy The cystic duct is 2 to 4 cm long and contains the spiral valves of Heister,which allow easy entry of bile into gallbladder but offer resistance to its outflow.The neck tapers and connects to the biliary tree via the cystic duct,which then joins the common hepatic duct to become the common bile duct.The extrahepatic bile ducts lie within the hepatoduodenal ligament.Anatomy Common bile duct CBD:length 4-8cm;diameter:0.6-0.8;Divided into four segments.The CBD courses through the pancreas and the wall of the duodenum to form the papilla of Vater on the medial wall of the duodenum.Its distal end is enveloped by the sphincter of Oddi,which regulates the flow of bile from the liver into the duodenum.Anatomy The triangle of Calot,a surgical landmark used to identify important structures during cholecystectomy,is bounded by the cystic duct,the common hepatic duct,and the inferior border of the liver.The right hepatic and cystic arteries are located within it,and anomalous structures often pass through it.Functions of Gallbladder Concentration and preservation of the bile.Secretion Contraction and emptyMethods of Diagnosis X-ray Ultrasonography,EUS CT MRI,MRCP(Magnetic resonance cholangiopancreatography)PTC,PTBD ERCP Scintigraphy Choledochoscopy Cholangiography Congenital Cystic Dilatation of Bile Duct They were classified into 5 types by Todani in 1977.Classification was based on site of the cyst or dilatation.Type I:Most common variety(80-90%)involving saccular or fusiform dilatation of a portion or entire common bile duct(CBD)with normal intrahepatic duct.Type II:Isolated diverticulum protruding from the CBD.Type III or Choledochocele:Arise from dilatation of duodenal portion of CBD or where pancreatic duct meets.Type IVa:Characterized by multiple dilatations of the intrahepatic and extrahepatic biliary tree.Type IVb:Multiple dilatations involving only the extrahepatic bile ducts.Type V or Carolis disease:Cystic dilatation of intra hepatic biliary ducts Congenital Cystic Dilatation of Bile Duct Clinical manifestation Diagnosis Treatment Ascariasis of biliary tractCholelithiasis Pathogenesis of Gallstones Gallstones are classified as cholesterol,pigment types.But most stones do not fit into this rigid classification system,pure cholesterol stones have the appearance of white pearls,are rare.Locations:gallstone;intrahepatic bile duct stone and extrahepatic duct stone.Pathogenesis of Gallstones Predominantly cholesterol stones The primary constituents of bile:Water Electrolytes Pigments cholesterol Phospholipids Bile salts Cholesterol,insoluble in water,is soluble in bile because it is associated with phospholipids and bile salts through the formation of mixed micelles and vesicles.The total amount of any one of these substances relative to the total amount of the other two determines the maximum amount of cholesterol that can be solubilized.When the relative amount of the three substances are insufficient to package all the cholesterol in micelles and vesicles,cholesterol crystals form on the surfaces of vesicles,making possible the formation of a cholesterol stone.Pathogenesis of Gallstones The precise cause of predominantly cholesterol gallstones is not yet known,and current evidence suggest that it is multifactorial.Cholesterol saturation of bile,stasis of bile within the gallbladder,and nucleating factors appear to be important.The incidence of predominantly cholesterol stones increases with age and is higher in females.Pathogenesis of GallstonesPigment stones Compared with the bile of patients with cholesterol stones or no stones,the bile of patients with pigment stones contains an excess of unconjugated bilirubin.The unconjugated bilirubin then form insoluble calcium bilirubinate,and a decrease in the amount of bile salts present could cause precipitation of calcium bilirubinate.Patients on long-term total parenteral nutrition therapy have an increased incidence of pigment gallstones.Diagnosis-Gallstone Recurrent attacks of right upper quadrant or epigastric pain or discomfort,nausea and vomiting Gallbladder colic,results from the temporary obstruction of the gallbladder outlet by a stone in the cystic duct or the infundibulum Physical findings:right upper quadrant or epigastric tenderness to palpation and voluntary muscle guarding.Jaundice is not a feature of cholelithiasis unless common duct obstruction.Diagnosis Imaging studies:Ultrasonography demonstration that the stones move to the dependent portion of the gallbladder when the position of the patient is changed and the stone produces acoustic shadowing.Oral cholecystography:iopanoic acidTreatment The definitive treatment of symptomatic gallstones is laparoscopic cholecystectomy.The mortality of laparoscopic cholecystectomy is as low as that previously reported for open cholecystectomy and ranges from 0%to 0.3%.The major advantages of the laparoscopic procedure are that patients have less pain and a shorter hospitalisation and are able to return to their activities sooner.Open cholecystectomy is indicated only in patients in whom the laparoscopic is impossible or unsafe.Impossible to establish safe access to the peritoneal cavity Adhesions Anatomic abnormalitiesTreatment Other treatments Oral dissolution therapy:(UDCA)Contact dissolution therapy:Methyl tert-butyl Extracorporeal shock wave lithotripsyCholedocholithiasis Clinical Manifestations and Diagnosis Common duct calculi may be asymptomatic or cause biliary colic,bile duct obstruction,cholangitis or pancreatitis.Jandice will be intermittent if the obstruction is partial and intermittent,or it maybe progressive if a stone becomes impacted in the distal duct.Chills and fever are usually associated with slight abdominal discomfort and a mild elevation of serum bilirubin,but any of these signs of cholangitis may be absent.Clinical Manifestations and Diagnosis Physical examination may be normal.Jaundice and mild tenderness in the epigastrium and right upper quadrant may be present.Ultrasonography is not reliable in the detection of common duct stones.Endoscopic retrograde cholangiopancreatography(ERCP)is indicated for most patient who have bile duct obstruction.Percutaneous transhepatic cholangiography(PTC)is an alternative,but ERCP permits visualization of other portions of the gastrointestinal tract and allows for the performance of pancreatography and endoscopic sphincterotomy with stone extraction,when indicated.Treatment Should be treated with antibiotic.Defervescence usually occurs rapidly.If it does not,AOSC(Acute Obstructive Suppurative Cholangitis)may be present,and decompression of the duct system must be carried out immediately.This can be done by establishing percutaneous transhepatic biliary drainage or by endoscopic sphincterotomy,but immediate laparotomy and insertion of a T-tube should be done if these simpler procedures fail or are nor available.Treatment Patients thought to have choledocholithiasis preoperatively undergo ERCP.when stones are identified,endoscopic sphincterotomy and stone extraction is performed Open choledocholithotomy and cholecystectomy are performed if the duct system cannot be cleared of stones.Open Choledocholithotomy Patients who are not candidates for laparoscopic procedures and those in whom endoscopic cholangiography and stone extraction are not possible may require open choledocholithotomy After the stones have been removed,the duct should be closed with a T-tube,which has a large side arm,allowing percutaneous stones removal later,if necessary.Intrahepatic Bile Duct Stone Causes and Pathophysiology Signs and Symptoms Diagnosis Treatment Acute Calculous Cholecystitis Acute cholecystitis is a chemical or bacterial inflammation of the gallbladder that may cause severe peritonitis and death unless proper treatment is instituted.In about 95%of cases,gallstones are present in the gallbladder,and in about 5%they are not.The incidence of Acute calculous cholecystitis is higher in females,with a female-to-male ratio of 3:1 Pathogenesis Obstruction:Obstruction of the cystic duct or the junction of the gallbladder and the cystic duct by a stone or by edema formed as the result of local mucosal erosion and inflammation caused by a stone Bacteria:Positive cultures of bile or gallbladder wall are found in 50%to 75%of cases.Deaths and complications from untreated cholecystitis are almost always related to septic complications of the disease.Other factors:In animal experiments,the presence of pancreatic juice,gastric juice,or concentrated bile in the lumen of the obstructed gallbladder causes acute cholecustitis.Pathology The inflamed gallbladder is enlarged The serosal surface is congested May have areas of gangrene or necrosis The wall is edematous and thickened.Manifestations Most patients have symptoms referable to the gallbladder prior to the development of acute cholecystitis but 20%to 40%are asymptomatic.The development of acute cholecystitis progresses through the sequence of distention,and later by inflammation of the gallbladder and adjacent peritoneal surfaces.Radiation of the pain around the right side toward the tip of the scapula.Nausea and vomiting occur in 60%to 70%of patients,are the only other significant symptoms.Physical Findings Tenderness in the right upper quadrant,the epigastrium,or both.Most common and reliable About half of all patients have muscle rigidity in the right upper quadrant,and about one fourth have rebound tenderness.Murphys sign.consisting of inspiratory arrest during deep palpation of the right upper quadrant,is not a consistent finding but is almost pathognomonic when present.Jaundice occurs in approximately 10%of patients.Bowel sounds are absent in only about 10%of patients Fever maybe absentLaboratory Finding White blood cell count is elevated in 85%of cases One half have elevation of the serum bilirubin Serum amylase is increased in one thirdImaging Studies Ultrasonography:Not specific,a thickened gallbladder wall and pericholecystic fluid are sometimes present.Complications Perforation:One third of these complications.Occurs when a gangrenous area becomes necrotic and bile leaks into the peritoneal cavity Pericholecystic abscess:Result from a perforation of the gallbladder that is walled off by omentum or adjacent organs such as the colon,stomach,or duodenum.Fistula:15%.occurs when the gallbladder becomes attached to a portion of the gastrointestinal tract and perforates into it.Treatment Preoperative management should include administration of an antibiotic that is effective against the enteric organisms found in the bile of approximately 80%of patients with gallstones and acute cholecystitis.These organisms include both gram-positive and negative aerobes and anaerobes.The definitive treatment of acute cholecystitis is cholecystectomy.The timing of operation was debated.Treatment Conversion to open cholecystectomy is indicated when the laparoscopic procedure cannot be completed safely or when bleeding or a bile leak cannot be stopped without risking injury to important structures.Cholecystectomy for acute cholecystitis is performed with a mortality rate of less than 0.2%and a major morbidity rate of less than 5%.The incidence of bile duct injury is approximately 0.4%.Chronic Cholecystitis The term chronic cholecystitis with cholelithiasis is often used to connote symptomatic gallbladder disease.Chronic inflammatory changes are found in the gallbladders.Approximately 98%of patients with symptomatic gallbladder disease have gallstones.Pathology The pathologic findings in chronic cholecystitis are best interpreted in light of the clinical manifestations of the disease.two types of chronic cholecystitis exist:Secondary chronic cholecystitis:Follows an episode of acute cholecystitis.Acute cholecystitis is caused by gallbladder outlet obstruction,always by a stone.In cases that do not progress to perforation,these abnormalities gradually resolve over 3 to 4 weeks.Simultaneously,granuloma formation begins.The mucosa itself becomes thin and loses its villous appearance.Pathology Primary chronic cholecystitis:Occurs primarily without antecedent acute cholecystitis Is characterized by a thin-walled gallbladder,with an intact mucosa that retains its villous configurationStones are almost present in both forms of chronic cholecystitis.Diagnosis Recurrent attacks of right upper quadrant or epigastric pain or discomfort,usually following meals.Nausea and vomiting may occur during the attack.Intervals between attacks are variable,maybe continuous or separated by several years.No fever or other signs of inflammation are present.Treatment The definitive treatment for symptomatic gallstones is laparoscopic cholecystectomy.Chronic Acalculous Cholecystitis Acute inflammation of the gallbladder without stones is a recognized entity that requires cholecystectomy.Occasionally,patients have signs and symptoms of gallbladder disease,but stones cannot be demonstrated by repeated ultrasonography or oral cholecystography.The criteria for cholecystectomy in this situation are not clearly defined.Cholangitis Cholangitis,originally described by Charcot in 1877,is a bacterial,parasitic,or chemical inflammation of the bile duct system.How bacteria enter the bile duct system:Small numbers of bacteria pass into the portal venous system from the intestine.Ascend from the duodenum Can be introduced into a normal or abnormal bile duct system by the tubes,catheters,scopes,guide wires,and other instruments used for diagnosis and treatment.Associated Pathology Choledocholithiasis(most common)Malignant strictures Cholangiocarcinoma Pancteatic cancer Ampullary cancer Gallbladder cancer Benign strictures(second common)Anastomotic stenosis Impacted stone Ampullary stenosis Indwelling tubes or stentsAssociated Pathology Cholangiography T-tube Percutaneous transhepatic Endoscopic retrograde Parasitic infestations Clonorchis sinensis Ascaris lumbricoides Ischemia Chemical irritation Carbamazepine ClinorilBacteriology The organisms found in the bile of patients with gallstones and other disease of the biliary tract are those that are cultured from the blood and the biliary tract during episodes of acute cholangitis or acute toxic cholangitis.Most are aerobic bacteria,including the gram-negative organisms,and gram-positive organisms.Clinical Manifestations The original description of cholangitis by Charcot consisted of intermittent chills and fever,jaundice,and abdominal pain.Charcots triad remains the hallmark of acute cholangitis by definition.Reynolds and Dargan described patients who had shock and central nervous system(CNS)depression in addition to Charcots triad and noted that this lethal combination of symptoms,now known as Reynolds pentad.Reynolds pentad is that this condition is rapidly lethal without emergency intervention,whereas Charcots triad is an acute but less toxic condition for which immediate intervention is usually not necessary.Clinical Manifestations The complete symptom triad of chills and fever,abdominal pain,and jaundice occurs in only 50%to 70%of patients who have cholangitis.Other than elevated temperature and jaundice,the positive physical findings are limited to the abdomen.60%to 80%of patients have abdominal tenderness,which is almost always in the right upper quadrant or epigastrium.Occasionally,a mass may be present in the right upper quadrant due to an enlarged gallbladder,a tumor,or an abscess.Clinical Manifestations The organisms most frequently cultured from the blood of patients with acute cholangitis are,in decreasing order,E.coli,Klebsiella pneumoniae,and S.faecalisClinical Manifestations AOSC(acute obstructive suppurative cholangitis)Reynolds Pentad The symptoms of AOSC are more severe,but the characteristic features are the persistent and progressive nature of the symptoms and the patients failure to respond rapidly to conventional therapy for sepsis.In acute toxic cholangitis,this means emergency decompression of the bile duct system.Diagnosis of the Underlying Condition Cholangiography:Is the definitive test and is necessary for planning definitive therapy,but it should not be done until the acute process is under control.Ultrasound:With special emphasis on the presence or absence of cholelithiasis,bule duct dilatation,masses in the head of the pancreas or within the hepatic portal,and choledocholithiasis Computerized tomography:Delineate the extent,as well as to assess the liver for hepatic metastases.Diagnosis of the Underlying Condition Cholescintigraphy:Differentiate between acute cholecystitis and acute cholangitis.Cholangiography:Can be obtained by the percutaneous transhepatic or endoscopic retrograde technique.Treatment Principles:Achieve complete control of the septic process Correct the underlying causeTreatment Antibiotic choice should be based on the organisms most often cultured from the blood In patients with mild cholangitis and no evidence of continuing,severe sepsis,antibiotic therap with a second-or third-generation cephalosporin is adequate.Monitoring of hemodynamic parameters,urine output,and blood gases.Most cholangitis patients repond rapidly to therapy.after they have been afebrile for 48 hours,cholangiography and other indicated studies should be done under continuing antibiotic coverage.Treatment Patients with acute cholangitis who do not respond completely to antibiotic therapy and supportive care or who deteriorate should have emergency decompression of the bile duct system.When transhepatic or endoscopic decompression of the biliary system is not available or possible,immediate laprotomy and insertion of a T-tube into the common bile duct should be carried out.Results and Complications The results of treatment of mild acute cholangitis are excellent.Deaths are almost always related to complications of the operation performed for the underlying condition and are unrelated to the original episode of cholangitisPrimary Sclerosing Cholangitis(PSC)Primary Sclerosing Cholangitis(PSC)PathogenesisPathogenesis PathologyPathology Clinical ManifestationsClinical Manifestations DiagnosisDiagnosis TreatmentTreatmentPolypoid lesions of Gallbladder Gallbladder polyps are growths or lesions resembling growths(polypoid lesions)in the wall of the gallbladder.The main types of polypoid growths of the gallbladder include cholesterol polyp/cholesterosis,cholesterosis with fibrous dysplasia of gallbladder,adenomyomatosis,hyperplastic cholecystosis,and adenoma.Symptoms and Diagnosis Most polyps do not cause noticeable symptoms.Gallbladder polyps are usually found incidentally when examining the abdomen by ultrasound for other conditions.Therapy Most polyps are benign and do not need to be removed.Polyps with symptoms;larger than 1 cm;locating the neck of gallbladder;co-occurring gallstones;occurring in people over the age of 50 may have the gallbladder removed(cholecystectomy).Carcinoma of the Gallbladder Carcinoma of the gallbladder is the most common malignant lesion of the biliary tract and accounts for 5%of all cancers found at autopsy.91%of patients who develop this malignancy are 50 years of age or older The incidence of the cancer in females is three to four times that in males.Type Adenocarcinoma 82%Undifferentiated carcinoma 7%Squamous 3%Mixed carcinoma 1%Routes of Metastasis The spread of carcinoma of the gallbla
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