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1、Biliary-tract-disease-Biliary-tract-disease-Emmet-Andrews-Emmet-AndrewsOverviewGallstonesBiliary tract tumoursOther conditionsAcute acalculous cholecystitisMirizzis syndromePrimary Biliary CirrhosisPrimary Sclerosing CholangitisBiliary tract cystsBiliary stricturesBiliary TractPart of the digestive
2、system.Made up of:Intra hepatic ductsExta hepatic ductsGallbladderCommon Bile DuctThe GallbladderThe gallbladder concentrates and stores bile.Bile:Secreted by the liver Contains cholesterol, bile pigments and phospholipidsFlows from the liver, through the hepatic ducts, into the gallbladderExits the
3、 gallbladder via the cystic ductFlows from the cystic duct into the common bile duct, into the small intestineIn the small intestine, aids digestion by breaking down fatty foods and fat-soluble vitaminsGallstones PathophysiologyCholesterol, ordinarily insoluble in water, comes into solution by formi
4、ng vesicles with phospholipids If ratio of cholesterol, phospholipids, and bile salts altered, cholesterol crystals may form Gallstone formation involves a variety of factors:Cholesterol supersaturationMucin hypersecretion by the gallbladder mucosa creates a viscoelastic gel that fosters nucleation.
5、 Bile stasisOccurs in diabetes, pregnancy, oral contraceptive use, and prolonged fasting in critically ill patients on total parenteral nutrition.Gallstones FrequencyGallstone disease is one of the most common and costly of all digestive diseases9% of those 60 yearsIn USA, 6.3 million men and 14.2 m
6、illion women aged 20-74 years have gallbladder diseaseIncidence of gallstones is 1 million new cases per yearPrevalence is 20 million cases in USAGallstonesSexHigher among females than males (lifetime risk of 35% vs 20%, respectively)Due to endogenous sex hormones (enhance cholesterol secretion and
7、increase bile cholesterol saturation)Progesterone may contribute by relaxing smooth muscle and impairing gallbladder emptying.AgeIncreased age is associated with lithogenic bile and increased rate of gallstonesGallstones Types Two main types:Cholesterol stones (85%): 2 subtypespure (90-100% choleste
8、rol) or mixed (50-90% cholesterol). Pure stones often are solitary, whitish, and larger than 2.5 cm in diameter. Mixed stones usually are smaller, multiple in number, and occur in various shapes and colors. Pigment stones (15%) occur in 2 subtypesbrown and black. Brown stones are made up of calcium
9、bilirubinate and calcium-soaps. Bacteria involved in formation via secretion of beta glucuronidase and phospholipase Black stones result when excess bilirubin enters the bile and polymerizes into calcium bilirubinate (patients with chronic hemolysis)Gallstones Natural History80% of patients, gallsto
10、nes are clinically silent20% of patients develop symptoms over 15-20 yearsAbout 1% per yearAlmost all become symptomatic before complications developBiliary-type pain due to obstruction of the bile duct lumenPredictive value of other complaints (eg, intolerance to fatty food, indigestion) too low to
11、 be clinically helpfulGallstones Diverse symptomsAbdominal painAching or tightness, typically severe and located in the epigastriumMay develop suddenly, last for 15 minutes to several hours, and then resolve suddenlyReferred pain posterior scapula or right shoulder areaNausea and vomitingJaundice Pr
12、uritus: Itching, typically worse at night. FatigueWeight lossMiscellaneous: Fatty food intoleranceGasBloatingDyspepsiaComplications of GallstonesIn the gallbladderBiliary colicAcute and chronic cholecystitisEmpyemaMucocoeleCarcinomaIn the bile ductsObstructive jaundicePancreatitisCholangitisIn the G
13、utGallstone ileusBiliary ColicSymptomsRight upper quadrant painSignsUsually noneInvestigationsBloods U&E, FBC, LFT, Amylase, CRPUltrasound of abdomenOGD (Oesophagogastroduodenoscopy)Treatment AnalgesiaCholecystectomyAcute Calculous Cholecystitis Inflammation of the gallbladder that develops in the s
14、etting of an obstructed cystic or bile ductMost patients have complete remission within 1-4 days.25-30% of patients either require surgery or develop some complicationPerforation occurs in 10-15% of cases.Acute Calculous CholecystitisSymptomsRight upper quadrant pain continuous, longer durationSigns
15、Fever, Local peritonism. Murphys sign2 fingers on RUQ, ask patient to breathe in. Positive if pain and arrest of inspirationInvestigationsBloods U&E, FBC, LFT, Amylase, CRPUltrasound of abdomenThickened gallbladder wall, pericholecystic fluid and stonesOGD (Oesophagogastroduodenoscopy)Treatment Nil
16、by mouthAnalgesiaIntravenous antibioticsCholecystectomyEmpyema / Mucocoele Empyema refers to a gallbladder filled with pus due to acute cholecystitisMucocele refers to an overdistended gallbladder filled with mucoid or clear and watery content. Empyema / Mucocoele SymptomsRight upper quadrant pain c
17、ontinuous, longer durationSignsFever, Local peritonism. Murphys sign2 fingers on RUQ, ask patient to breathe in. Positive if pain and arrest of inspirationInvestigationsBloods U&E, FBC, LFT, Amylase, CRPUltrasound of abdomenThickened gallbladder wall, distended gallbladder, pericholecystic fluid, st
18、onesTreatment Nil by mouthAnalgesiaIntravenous antibioticsCholecystectomyObstructive JaundiceBlockage of the biliary tree by gallstonesSymptomsPain, Jaundice, dark urine, pale stoolsSignsJaundice. InvestigationsBloods U&E, FBC, LFT, Amylase, CRP, Hepatitis screen, Coagulation screenUltrasound of abd
19、omenTreatment Endoscopic Retrograde CholangioPancreatogramAscending CholangitisObstruction of biliary tree with bile duct infectionSymptomsUnwell, pain, jaundice, dark urine, pale stoolsCharcot triad (ie, fever, right upper quadrant pain, jaundice) occurs in only 20-70% of casesSignsSepsis (Fever, t
20、achycardia, low BP), Jaundice. InvestigationsBloods U&E, FBC, LFT, Amylase, CRP, Coagulation screenUltrasound of abdomenTreatment Intravenous antibioticsEndoscopic Retrograde CholangioPancreatogramAcute PancreatitisAcute inflammation of pancreas and other retroperitoneal tissues.SymptomsSevere centr
21、al abdominal pain radiating to back, vomitingSignsVariable None to Sepsis (Fever, tachycardia, low BP), Jaundice, acute abdomenInvestigationsBloods U&E, FBC, LFT, Amylase, CRPUltrasound of abdomenMRCPCT PancreasTreatment Supportive Endoscopic Retrograde CholangioPancreatogramGallstone ileusObstructi
22、on of the small bowel by a large gallstoneA stone ulcerates through the gallbladder into the duodenum and causes obstruction at the terminal ileumSymptomsSmall bowel obstruction (vomiting, abdominal pain, distension, nil pr)SignsAbdominal distension, obstructive bowel sounds. InvestigationsBloods U&
23、E, FBC, LFT, Amylase, CRP, Hepatitis screen, Coagulation screenPlain film of abdomen Air in CBD, small bowel fluid levels and stoneTreatment Laparotomy and removal of stone from small bowel.CholecystectomyLaparoscopic cholecystectomy standard of careTimingEarly vs interval operationPatient consentCo
24、nversion to open procedure 10%BleedingBile duct injuryDamage to other organsMirizzi Syndrome Refers to common hepatic duct obstruction caused by an extrinsic compression from an impacted stone in the cystic ductEstimated to occur in 0.7-1.4% of all cholecystectomiesOften not recognized preoperativel
25、y, which can lead to significant morbidity and biliary injury, particularly with laparoscopic surgery.Acute Acalculous Cholecystitis Presence of an inflamed gallbladder in the absence of an obstructed cystic or common bile ductTypically occurs in the setting of a critically ill patient (eg, severe b
26、urns, multiple traumas, lengthy postoperative care, prolonged intensive care) Accounts for 5% of cholecystectomiesAetiology is thought to have ischemic basis, and gangrenous gallbladder may resultIncreased rate of complications and mortalityAn uncommon subtype known as acute emphysematous cholecysti
27、tis generally is caused by infection with clostridial organisms and occlusion of the cystic artery associated with atherosclerotic vascular disease and, often, diabetes.Primary Sclerosing CholangitisChronic cholestatic biliary disease characterized by non-suppurative inflammation and fibrosis of the
28、 biliary ductal systemCause is unknown but is associated with autoimmune inflammatory diseases, such as chronic ulcerative colitis and Crohn colitis, and rare conditions, such as Riedel thyroiditis and retroperitoneal fibrosisMost patients present with fatigue and pruritus and, occasionally, jaundic
29、eNatural history is variable but involves progressive destruction of the bile ducts, leading to cirrhosis and liver failureClinical features of cholangitis (ie, fever, right upper quadrant pain, jaundice) are uncommon unless the biliary system has been instrumented.Primary Sclerosing CholangitisMedi
30、cal CareChronic progressive disease with no curative medical therapyGoals of medical management are to treat the symptoms and to prevent or treat the known complicationsLiver transplantation is the only effective therapy and is indicated in end-stage liver disease.Surgical CareIndications for liver
31、transplantation include variceal bleed or portal gastropathy, intractable ascites, recurrent cholangitis, progressive muscle wasting, and hepatic encephalopathy. Recurs in 15-20% of patients after transplantation.Primary Biliary Cirrhosis Progressive cholestatic biliary disease that presents with fa
32、tigue and itching or asymptomatic elevation of the alkaline phosphatase. Jaundice develops with progressive destruction of bile ductules that eventually leads to liver cirrhosis and hepatic failure. Autoimmune illness has a familial predispositionAntimitochondrial antibodies (AMA) are present in 95%
33、 of patientsGoals of treatment are to slow the progression rate of the disease and to alleviate the symptoms (eg, pruritus, osteoporosis, sicca syndrome)Liver transplantation appears to be the only life-saving procedure.Biliary Tract CystsCholedochal cystsConsist of cystic dilatations of the extra-h
34、epatic biliary treeUncommon abnormality50% present with combination of jaundice, abdominal pain, and an abdominal mass. ? Due to anomalous union of the pancreatic and biliary ductal system. Classified into 5 typesTreatment for choledochal cysts is surgical (excision of the cyst with construction).Bi
35、liary Tract TumoursCholangiocarcinomaCancer of the Gall BladderBiliary Tree NeoplasmsClinical symptoms:Weight loss (77%)Nausea (60%)Anorexia (56%)Abdominal pain (56%)Fatigue (63%)Pruritus (51%)Symptomatic patients usually have advanced disease, with spread to hilar lymph nodes before obstructive jau
36、ndice occursAssociated with a poor prognosis. Fever (21%)Malaise (19%)Diarrheoa (19%)Constipation (16%)Abdominal fullness (16%). Cholangiocarcinoma Adenocarcinoma of the bile ductsMay occur without associated risk factorsAssociated with chronic cholestatic liver disease such as:Primary Sclerosing Ch
37、olangitisCholedochal cystsAsbestos.Accounts for 25% of biliary tract cancersPresentation:JaundiceVague upper or right upper quadrant abdominal pain Anorexia, weight lossPruritus.CholangiocarcinomaSlow growing malignancy of biliary tract which tend to infiltrate locally and metastasize late.Gall Blad
38、der cancer = 6,900/yrBile duct cancer = 3,000/yrHepatocellular Ca = 15,000/yrCholangiocarcinomaDiagnosis and Initial WorkupJaundiceWeight loss, anorexia, abdominal pain, feverUS bile duct dilatationQuadruple phase CTMRCP/MRIERCP with Stent and Brush BiopsyPercutaneous Cholangiogram with Internal Ste
39、nt and Brush BiopsyMRCP: Cholangiocarcinoma at the BifurcationKlatskin tumour = Cholangiocarcinoma of junction of right & left hepatic ducts ERCP: Distal CBD CancerSurgical RemovalNode Dissection in Bile Duct ExcisionRoux-en-Y HepaticojejunostomyCholangiocarcinomaIf positive Margins or Unresectable:
40、StentChemotherapy +/- Radiation TherapySurvival with surgery and chemo/radiation is 24 to 36 monthsWith chemotherapy / radiation alone survival is 12 to 18 monthsGallbladder Cancer6th decade1:3, Male:FemaleHighest prevalence in Israel, Mexico, Chile, Japan, and Native American women.Risk Factors: Ga
41、llstones, porcelain gallbladder, polyps, chronic typhoid and some drugsGallbladder CancerUncommon malignancy 2.5 per 100,000 populationRepresents 54% of biliary tract cancers.Gall Bladder CancerPresentation (1)Discovered on pathology after a routine cholecystectomy. (T-1a/b - invades muscularis)CT/C
42、hest and Abdomen, 4 phase CT of liverIf negative for metastasis: Radical cholecystectomy with nodal dissection, central hepatectomy, w or w/o bile duct excisionExcise port sitesFollowed by Chemo/Radiation5 year survival = 60%Gall Bladder CancerPresentation 2RUQ pain, jaundice, weight loss: CTBiopsy
43、yields adenocarcinoma consistent with GB primaryBiliary DecompressionChemo/Radiation Median survival with chemoradiotherapy is 9 months.Biliary StrictureBiliary stricture is an abnormal narrowing of the bile duct.Among biliary strictures:90% are malignantPancreatic cancer is the most common malignan
44、t cause, followed by cancers of the gallbladder, bile duct, liver, and large intestine. Biliary Stricture Non Cancerous CausesNoncancerous causes of bile duct stricture include:Injury to the bile ducts during surgery for gallbladder removal (accounting for 80% of nonmalignant strictures)Pancreatitis
45、 (inflammation of the pancreas)Primary sclerosing cholangitis (an inflammation of the bile ducts that may cause pain, jaundice, itching, or other symptoms)GallstonesRadiation therapyBlunt trauma to the abdomenBiliary Stricture Patient SymptomsPatients with biliary strictures may present with:Jaundic
46、e (yellow skin color)Abdominal painFever VomitingBiliary Stricture Diagnostic TestsCommon diagnostics for biliary stricture are:UltrasoundCTMRIBiopsy Cholangiography A cholangiogram is an X-ray of the bile ductsCan be performed:Endoscopically Percutaneously Cholangiogram- Endoscopic ApproachEndoscop
47、ic retrogradecholangiopancreatography (ERCP) Endoscopic tube is placed into the patients mouth, through the stomach, and into the duodenal portion of the small intestine. Contrast is introduced into the biliary tract through the endoscope, in a retrograde manner. X-rays taken.Indications For Biliary
48、 StentingIndications for stent insertion include:Ampullary StenosisManagement of patients with bile duct injuryManagement of benign or malignant biliary obstructionPrevention of obstruction where stone extraction is not possible at that timeManagement of selected pancreatic duct strictures, stones a
49、nd sphincter of Oddi dysfunctionStent Placement - Endoscopic ApproachThe Endoscope is positioned in the duodenum at the opening of the bile duct.Stent Placement -Endoscopic ApproachA catheter is inserted through the endoscope into the ostium of the common bile duct. While maintaining the endoscope p
50、osition in the duodenum, a wire is inserted through the catheter into the bile duct. The stent delivery system is then inserted over the wire to the site of obstruction, where the stent is deployed. Stent Placement Endoscopic ApproachSuccess rate of ERCP 90-95%Complication rate of approximately 3-5%
51、. Complications:PancreatitisBleedingPerforationInfectionCardiopulmonary depression from conscious sedation. Biliary Stent - Percutaneous Approach For biliary stent placement using a percutaneous approach:A fine needle is inserted between the 4th and 5th rib on the patients right sideThe puncture is
52、through the liverThe needle is inserted into an intrahepatic duct under image guidance.Transhepatic ApproachPhoto on file at MedtronicBiliary Stent - Percutaneous ApproachSuccess rate of percutaneous transhepatic cholangiography approaches 95% when ducts are dilated. Percutaneous approach associated
53、 with a 5-10% rate of major complications which include:SepsisBile leakIntraperitoneal haemorrhageHaemobiliaHepatic and perihepatic abscessPneumothoraxSkin infection and granuloma at the catheter entry site. Percutaneous transhepatic cholangiography is contraindicated in patients with bleeding diath
54、eses and significant ascites.SummaryGallstonesIn the gallbladderBiliary colicAcute and chronic cholecystitisEmpyemaMucocoeleBiliary tract tumoursOther conditionsAcute acalculous cholecystitisMirizzis syndromePrimary Biliary CirrhosisPrimary Sclerosing CholangitisBiliary tract cystsBiliary stricturesIn the bile ductsObstructive jaundicePancreatitisCholangitisIn the GutGallstone ileus结束！结束！
