1 b iliary t ract. 2 bile system diseases textbook of medicine gallbladder primary disease biliary...
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BILIARY TRACT
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Bile System Diseases Textbook of Medicine
Gallbladder primary disease
Biliary tract obstruction
Drugs
Other gastro
Neural disorders
Metabolic disorders
Gallstones, Cholecystitis
Cystic duct, Common Duct, Pancreas Head, Oddi dysfunction, Stenosis
Narcotics, Anticholinergic Agents
Irritable Bowel Syndrome
Spinal Cord Injury, Truncal Vagotomy, Achalasia
Obesity, Diabetes, Sexual Hormones, Pregnancy, Somatostatin therapy, Sprue, Vipoma, Sickle Hemoglobinopathy
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Valves of Heister
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Variation is common
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Gallbladder and Bile Ducts Function
Bile storage (~30ml) Bile concentration (water & electrolytes absorption) & mucus secretionBile release
Fasting State:
High pressure Oddi
Prevents duodenal reflux
Promote gallbladder filling
AminoA
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Composition of Hepatic and Gallbladder BileMeq/l Hepatic Gallbladder
Na 160 270
K 5 10
Cl 90 15
HCO3 45 10
Ca 4 25
Bilirubin 1.4 15
Protein 150 -
Bile acids 50 150
Phospholipids 8 40
Cholesterol 4 18
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Bacteriology
Bile is normally sterile Bactibilia present in 11% to 30% in
chronic biliary disease, 50% in acute cholecystitis, 60% in common bile duct stones and 95% in cholangitis
Highest in elderly (decreased motility and clearance)
Mainly gram-neg aerobe single organism, polymicrobial infection in cholangitis
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Antibiotic Selection
Antibiotics should be used prophylactically in elective biliary tract surgery or manipulations
Not mandatory in low-risk patients as wound infection is low (1%) following laparoscopic cholecystectomy
Single dose of first generation cephalosporin provides good coverage in high risk patients
Bile infections are treated with second generation cephalosporins, aminoglycoside and fluoroquinolones
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Imaging
US UltrasonographyEUS Endoscopic ultrasonographyHIDA Hepatobiliary iminodiacetic acid scanERCP Endoscopic retrograde cholangio
pancreatog.PTC Percutaneous transhepatic
cholangiographyIOC Intraoperative cholangiography (open or
lap)CT MRCPCholedocoscopy (IO, ER, TH)
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Diseases of the Gallbladder
Congenital Anomalies Gallstones Acute and Chronic Cholecystitis Hyperplastic Cholecystoses
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Congenital Anomalies
Anomalies in number, size and shape Agenesis Duplication Rudimentary or giant gallbladder Diverticula
Anomalies of position or suspension Left-sided Intrahepatic Floating predisposing to torsion
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Gallbladder stasis, sludge & stone formation
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Cholelithiasis- type of gallstones
Type of Stone Cholesterol Black Pigment Brown Pigment
Location Gallbladder Gallbladder Bile Ducts
Pathogenesis 80% Infectious
Cholesterol monohydrate
>50% <20% ++
Calcium & Bilirubin
+ +++ calcium bilirubinate
++
Obesity, weight loss, estrogens
Chronic hemolysis, alcoholic cirrhosis, pernicious anemia, infections, ileal disease
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Natural History of Gallstone Disease Silent
over 20y period, 2/3 remain symptoms free Biliary pain by cystic duct obstruction
2/3 have further episodes within 1 yeardelaying treatment contribute to high prevalence of complications (44% of cholecystectomies performed for complications)
Complications include cholecystitischoledocolithiasis with or without cholangitisgallstone pancreatitisgallstone ileusgallbladder carcinoma
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Inflammation Obstruction
Severe steady ache or fullness in epigastrium or ruq with frequent radiation to right scapula
Migration Distension Biliary “colic” Nausea vomiting, precipitated by eating
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Acute Cholecystitis
Etiology: cystic duct obstruction with secondary bacterial infection (75%)
Mechanical inflammationincreased intraluminal pressure, distension, ischemia of gallbladder mucosa and wall
Chemical inflammationrelease of lysolecithin and other tissue factors
Bacterial inflammationescherichia coli, klebsiella, streptococcus, clostridium spp
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Acute Cholecystitis
Clinical presentation women 30-80y old, prior episodes of biliary colic
or previous cholecystitis (60% recurrent within 5y)fever , nausea, vomitingright upper quadrant pain following a fatty mealMurphy sign, palpable mass
Diagnosis: ultrasound, HIDA, CBC, LFT, Amylase , ECG , Chest x-ray
Treatment: Cholecystectomy – immediate or delayed
Perioperative antibioticsCholecystostomy in high risk patients (diabetes,
elderly) Emphysematous cholecystitis
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Acute
Cholecystitis
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Acute Cholecystitis
Complications occur mainly in not-treated patientsEmpyema and hydropsGangrenous and Perforated cholecystitis with localized / generalized peritonitisBilio-enteric and Cholecysto-choledocal fistulaGallstone ileusCalcium deposition: Limey, PorcelainCarcinoma of gallbladderSepsis
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Acalculous Cholecystitis 5-10%
Absence of gallstonesUsually complicate the outcome of
severeburns , sepsis , trauma or collagen
diseaseEtiology: thrombosis of cystic art
sphincter spasmprolonged fastingdehydration
systemic disease, sepsis
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Hyperplastic Cholecystoses
Adenomyomatosis Cholesterolosis
Strawberry gallbladder Cholesterol “polyps”
Cholecystectomy when symptomatic or gallstones
Gallbladder polyp Cholecystectomy when symptomatic or
over 50 y or polyps > 10mm or gallstones or polyp growth on serial US
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Diseases of the Bile Ducts
Congenital anomalies Choledocolithiasis Trauma, strictures, hemobilia (95%
iatrogenic) Extrinsic compression Hepatobiliary parasitism Sclerosing cholangitis
Increased risk for cholangiocarcinoma
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Obstructive Jaundice, diagnosis Increased production unconjugated
multiple transfusion, transfusion reaction, sepsis, burns, hemolysis, congenital hemoglobinopathies
Impaired uptake or conjugation unconjugated
Gilbert’s disease, neonatal jaundice, sepsis, viral hepatitis, drug inhibition, Crigler-Najjar syndrome
Impaired transport and excretion conjugated
cirrhosis, amyloidosis, cancer, pregnancy, hepatitis, Dubin-Johnson syndrome
Biliary obstruction conjugated
choledocolithiasis, periampullary cancer, chronic pancreatitis, primary sclerosing cholangitis, cholangiocarcinoma
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Obstructive Jaundice, goals of imaging
Confirmation of intrahepatic and/or extrahepatic bile duct dilation
Identification of site and cause of the obstruction
Selection of treatment modalitypreoperative biliary drainage?anticipate!
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Obstructive Jaundice: Additive Operative Risk Factors
Alterations in hepatic and pancreatic functiongastrointestinal barrierimmune functionhemostatic mechanismswound healing
Malnutrition (hypoalbuminemia), cholangitis and renal insufficiency are associated with increased morbidity and mortality
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Congenital anomalies = Risk!
Biliary Atresia and Hypoplasia 10% treatable with Kasai procedure
within 1m Chronic cholangitis, hepatic fibrosis,
liver tx Choledocal Cysts
>50% develop symptoms after 10y of age Increased risk for cholangiocarcinoma
Congenital Biliary Ectasia Caroli’s disease Congenital hepatic fibrosis
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Treatment is surgical by Complete Resection and biliary-enteric anastomosis
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Choledocolithiasis
Asymptomatic, found in about 10 % of patients undergoing Cholecystectomy
Associated with gallstones in general
Can cause obstructive jaundice, cholangitis, or pancreatitis
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Endoscopic view of sphincterotomy and basket extraction
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Choledocolithiasis Complications
Cholangitis Acute cholangitis (suppurative / non
suppurative) Chronic cholangitis, hepatic abcesses
Obstructive Jaundice In association of cholecystitis
Pancreatitis Secondary Biliary Cirrhosis
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Acute Cholangitis
- Infection of bile ducts by obstruction (choledocal stones, benign stricture, sclerosing cholangitis and tumors of CBD or periampullary neoplasm)
- Enteric flora most common (E coli)- Charcot’s triad of right upper quadrant
pain, fever and jaundice (70% of patients)
- Relapse if cause not treated, life threatening with generalized sepsis, MOF. Chronic cholangitis may lead to secondary biliary liver cirrhosis
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Cholangiopancreatography in acute cholangitis
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Indications for Cholecystectomy
Asymptomatic gallstones?18% cumulative risk at 15 y for development of symptoms or complications in male silent gallstones patients.-cumulative risk of death on expectant management = small-prophylactic cholecystectomynot warrantedexcept in young age, diabetics,large stones, anomalies
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Choledocolithiasisdiagnosed during Cholecystectomy
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Postcholecystectomy complications
Overlooked non-biliary disorder Biliary strictures Retained biliary calculi Cystic duct stump syndrome (?) “Papillary dysfunction, papillary
stenosis, sphincter of Oddi spasm, biliary dyskinesia”
Bile salt – induced diarrhea (5%, cholestyramine)
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Operative Complications of Laparoscopic Cholecystectomy
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Retained common bile duct stone after Cholecystectomy
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Gallbladder adenocarcinoma: risk factorsIsrael & Chile: highest incidence worldwideChronic Gallstones: 70-90% presence
Estrogens / Female sex / Obesity
75% older than 65y
Choledocal cyst
Salmonella typhi carriers
Porcelain gallbladder (neglected cholecystitis)
Carcinogens
Gallbladder polyps
Smoking, Alcohol consumption
PSC
Repetitive epithelial repair (dysplasia to invasive carcinoma, ~15y)
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Gallbladder AdenoCarcinoma: Spread & Staging
TNM* Tumor Location
Tis Carcinoma in situ
T1a GB wall: mucosa
T1b GB wall: muscle
T2 Perimuscular tissue
T3 Serosa,organ, liver <2 cm
T4 >2 organs, liver >2 cm
N1a Hepatoduodenal nodes
N1b Other regional nodes
M0 No distant metastases
M1 Distant metastases
Early lymphatic spread: hepatoduodenal, retroperitoneal, celiac. Direct invasion of the liver, extrahepatic biliary ducts. Intraperitoneal seeding.
Stage 0 Tis N0 M0
Stage I T1 N0 M0
Stage II T2 N0 M0
Stage IIIT1-2T3
N1N0-1
M0M0
Stage IVA T4 N0-1 M0
Stage IVBT1-4T1-4
N2N0-2
M0M1
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44intraluminal mass enhancing heterogeneously after IV contrast
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Porcelain gallbladder: 60y old woman
carcinoma in 25% of patients with "porcelain" GB
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Gallbladder adenocarcinoma: Presentation
Fifth most common GI malignancy Incidental finding in lap. excised
gallbladders Patients in early stages show signs and
symptoms that mimic cholelithiasis and/or cholecystitis.
Patients in later stages present with weight loss, hepatomegaly, and jaundice, which are considered poor prognostic signs.
Duodenal or colonic obstruction or cholecystenteric fistula may signal GB carcinoma
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Gallbladder adenocarcinoma: Presentation
Typically unresectable at presentation Prognostic is poor with the exception of
early-stage cases. Overall mean survival rate of 6 months, and the 5-year survival rate is 5%
In locally advanced gallbladder cancer, radical surgery with negative tumor margins is an accepted treatment; if negative margins were seen, patients achieved a 5-year survival rate Stage I, 95% Stage II, 75% Stage III, 25% Stage IV, 15% Stage V, 2%
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Chronic Cholecystitis Cholelithiasis Small Cystic Duct Papillary Tumor
Prevention of gallbladder carcinoma by cholecystectomy?
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Cholangiocarcinoma: risk factors
Primary sclerosing cholangitis: 30% of CC in patients with PSC and UC (lifetime risk 10-15%).
· Choledochal cysts: related to duration of disease. 15% per year after first two decades.
· Parasitic infections: Liver flukes (Clonorchis and Opisthorchis)
· Hepatolithiasis :Asian patients with gallstones.
· Toxin exposures: auto, rubber, chemical, wood-finishing occupations
· Genetics: “cancer family" syndromes (eg, Lynch, Li-Fraumeni), Caroli’s syndrome (congenital dilation of intrahepatic ducts), multiple biliary papillomatosis
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Cholangiocarcinoma: presentation2000-3000 US cases per year. 3% of GI
malignancies.
Symptoms: pruritus 60%, abdominal pain 30-50%, weight loss 30-50%, fever 20%, fatigue, clay stools , dark urine.
Signs: jaundice (often intermittent) 90%, hepatomegaly 25-40%
Lab test: increased bilirubin, increased alkaline phosphatase.
CEA elevated in some but not sensitive or specific.
CA 19-9 elevated in 80% and most helpful in pts with PSC.
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Cholangiocarcinoma: imaging
Ultrasound: segmental dilatation, nonunion of R and L ducts, polypoid intraluminal masses, nodular smooth masses with mural thickening.
Doppler: assess vascular invasion
CT: contracted gallbladder in Klatskin tumor, Courvoisier in CBD tumor.
Cholangiography (ERCP or PTC) with cytology and biopsy.
MRCP
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Perihilar cholangiocarcinoma: Klatskin
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Cholangiocarcinoma: treatment
Surveillance of congenital disease and PSC!
Prognosis: 5 year survival without surgery 5-10%. With surgery 10-30% or higher.
Surgery is best option in resectable tumor
Adjuvant therapy: post-operative radiation may provide benefit in incomplete resections.
Chemotherapy combined with radiation therapy may improve survival time by up to 10 months in pts with unresectable tumors.
Palliative therapy: Stenting of biliary tree
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Intra-pancreatic cholangiocarcinoma
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