7/24/2012
Biliary Tract Disease
John J. Vargo, MD, MPH
Talk Outline
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Demographics
Types / pathogenesis
Complications of cholelithiasis
Complications of choledocholithiasis
Other gallbladder disorders
Sphincter of Oddi dysfunction
Other bile duct disorders
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Gallstones
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Affects 20 million Americans
Gender and Ethnic Predisposition
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Over 65% are female
Hispanic and Native American
Lower socioeconomic status
600,000 annual cholecystectomies in U.S.
$6.5 billion annual cost
Gallstone Types
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Black Pigment Stones (20%)
Cirrhosis (hemolysis d/t hypersplenism)
Chronic hemolysis
Older age
Female gender
No relationship to obesity
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Cholesterol Gallstone Pathogenesis
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75% of cholelithiasis
Cholesterol super saturation
Nucleation
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Calcium nidus
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Defective acidification
Nucleation vs. Anti-nucleation factors
Growth
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Gallbladder dysmotility (TPN, pregnancy)
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Gallbladder mucus
Gallstone Types
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Cholesterol
80% of GB stones
Female gender
Obesity
Rapid weight loss
Terminal ileal resection or disease
Pregnancy
Drugs: estrogen, OCPs, octroetide
Hypertriglyceridemia, DM, ?vagotomy
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Cholesterol Gallstone Formation
Micellar
Liquid
-----------------Moles % Bile Salts---------------
Pigmented Gallstones
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10-25% of gallstones
Bilirubin deconjugation and precipitation
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Bacterial β glucuronidase
Chronic hemolytic disorders
Decreased CaCO
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Gallbladder stasis
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Brown Pigment Stones
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Stasis and Infection s/p cholecystectomy > 2 yrs
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Gallbladder Sludge
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Mixture of crystals and mucus
Can cause the same symptoms as stones
Can be transient
Diagnosed on ultrasound
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Natural History of Gallstones
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70-80%: remain asymptomatic
When symptoms occur, high likelihood that they will recur
Unknown why symptoms occur
Minority may have only one attack
Gracie and Ransohoff
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5 yrs: 10% symptomatic
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10 yrs: 15% symptomatic
15 yrs: 18% symptomatic
No sig complications
Complications of Cholelithiasis
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Biliary colic
Cholecystitis
Fistulization (GB to duodenum)
Mirizzi syndrome
Cholangitis
Pancreatitis
GB cancer
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Biliary Colic
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RUQ, midepigastric
Lasts hours not weeks
Postprandial but may have nocturnal awakening
Crescendo, plateau, relief
Nausea and vomiting
Radiation to shoulder and back
Acute Cholecystitis
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Duration: hours to days
Continued cystic duct obstruction
Cholescintigraphy: Sens/Spec > 90%
Ultrasonography: Sens/Spec > 90%
Alk phos, transaminases rarely twice normal
Mortality: 5-10% advanced age, comorbidities, complications
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Acute Cholecystitis
Complications
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Emphysemaotus cholecystitis
Perforation
Mirizzi Syndrome
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Cystic duct stone leading to biliary obstruction
Cholecystoenteric fistula
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Pneumobilia
Duodenum, Colon, Jejunum, Stomach
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Gallstone ileus
Cholescintigraphy False
Positives
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Prolonged fasting
Liver disease
Chronic cholecystitis
Total parenteral nutrition
Critical illness
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Acalculous Cholecystitis
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Older and more likely male
Triad
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Hemodynamic instability
Prolonged fasting
Immobility
Fever, RUQ pain, leukocytosis, tenderness may be absent
More fulminant course
Acalculous Cholecystitis
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CT scan
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GB wall > 4 mm
Sonographic Murphy’s sign
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Subserosal edema
Intramural gas
Pericholecystic fluid collection
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Sloughed mucosa
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Sens / Spec >95%
Sensitivity: 67-92%
Specificity: > 90%
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Scintigraphy
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False (+)#
* In the absence of hypoalbuminemia / ascites
# narcotics, anticholinergics, diabetes mellitus, celiac sprue, obesity
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Cholecystectomy
Open or laparoscopic: 0.1% mortality
Laparoscopic: reduction in postoperative
LOS and pain
Complicated presentation, coagulopathy or adhesions->open
IOC: allows for ID and potential treatment of choledocholithiasis
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False (+) rate of 1-5.6%
Ford JA et al. Br J Surg 2012;99:160-7.
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Other Treatment Modalities
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Ursodeoxycholic acid
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Small floating stones on oral cholecystogram
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Non-calcified on ultrasound
Much less effective
High recurrence rate (50%)
Risk of pancreatitis and cholangitis
ESWL not approved in U.S.
Cholecystostomy tube
Choledocholithiasis
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Can present with biliary colic
Elevated liver profile
With chronicity, dilated common bile duct
Jaundice
Cholangitis
Pancreatitis
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Choledocholithiasis
RUQ Pain
Fever
Charcot’s
Triad
Jaundice
Choledocholithiasis:
Acute Suppurative Cholangitis
RUQ Pain
Hypotension
Raynaud’s
Pentad
Altered
Level of
Consciousness
Fever
Jaundice
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Choledocholithiasis
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Recurrent biliary colic in the postcholecystectomy patient
ERCP for patients with high probability
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95 success rate
5% complication rate
For low or intermediate probability
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MRCP
EUS
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Intraoperative cholangiogram
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Choledocholithiasis
Biliary sphincterotomy
Balloon /basket extraction
Balloon sphincteroplasty
Cholangioscopy
Lithotripsy
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Mechanical (basket)
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Laser
Electrohydrualic
Extracorporeal shockwave lithotripsy
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Recurrent Pyogenic Cholangitis
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Prototypic syndrome seen in Asia and
Asian immigrants
Parasitic infestation &/or malnutrition
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Polymicrobial cholangitis
Brown and black stones
CBD,CHD; L > R hepatic duct
Complications: Cholangitis, PV thromobosis, Abscess, Cholangiocarinoma
Treatment :Endoscopic, IR, Surgical
Gallbladder Dyskinesia
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Decreased GB ejection (via CCK-HIDA) fraction may predispose to gallstone formation
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GB ejection fraction > 38%
Controversial whether cholecystectomy leads to symptomatic improvement
More common in subjects with functional bowel disorders
DiBaise et al., Clin Nuc Med 2012;37:63-70.
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Gallbladder Polyps
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Cholesterolosis
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Cholesterol / TG within epithelial macrophages
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Local or diffuse
Can be combined with cholesterol polyps
10-15% autopsy series
Rarely detectable via imaging
Rarely thought to be asymptomatic
Gallbladder Polyps
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Adenomyomatosis
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Hypertrophy of the muscle layer
Usually focal (fundus) although can be diffuse
Incidental and asymptomatic
Surgery when wall > 10 mm, associated mass or in rare instances with diffuse disease
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Gallbladder Polyps
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1-5% of population
> 95% non-neoplastic (cholesterol)
Follow with ultrasound for 6 month-1 yr intervals
If growth > 10 mm, cholecystectomy
If no growth after 2 yrs, can dispense surveillance
GB polyp (any size) + PSC = surgery
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GB Cancer
Rarely diagnosed early
Surgery only curative option
5 yr survival: <10%
Associations:
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Female gender
Porcelain gallbladder
IBD
PSC
Anomalous pancreaticobiliary junction
Cholelithiasis
Chronic Salmonella typhi
Adenomatous GB polyps
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Sphincter of Oddi Dysfunction
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Stenosis or spasm of the Sphincter of Oddi
Persistent / recurrent biliary pain following cholecystectomy without structural abnormalities
Idiopathic recurrent pancreatitis
Biliary type pain with intact gallbladder and no stones
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No role for Sphincter of Oddi manometry with gallbladder in place
Corraziara ES, Cotton PB. Am J Gastroenterol 2010;105: 764-9.
Biliary Sphincter of Oddi
Dysfunction
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Type I: biliary type pain, elevated liver enzymes (> 2x nl on two occasions), bile duct diameter > 8 mm; delayed contrast drainage > 45 minute
Type II: biliary type pain and 1 or 2 of the aforementioned criteria
Type III: biliary type pain only
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Type I
Type II
Type III
Biliary Sphincter of Oddi
Dysfunction
SOD Type Prevalence of
Manometric
Abnormalities (%)
65-95*
50-63
12-59
Biliary Sphincter of Oddi
Dysfunction
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Type I: empiric biliary sphincterotomy
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85-100% response
Type II: sphincter of Oddi manometry
(SOM)
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Pain relief up to 40-60% in this subset with biliary sphincterotomy with SOD
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Normal SOM and sphincterotomy: controversial
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Biliary Sphincter of Oddi
Dysfunction
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Type III:
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10-58% response to sphincterotomy if SOD
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Current subject of an NIH trial
Consider other treatments for functional bowel disorders
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Sphincter of Oddi Manometry
Basal pressure > 40 mm Hg
Correlates best with presence of symptoms and relief following sphincterotomy
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Abnormal Biliary and Pancreatic
Sphincters in Pts with Suspected
SOD
Patient
#
360
Biliary
Alone (%)
41 (11.4)
Pancreatic
Alone (%)
68 (18.9)
Both
(%)
113
(31.4)
Total (%)
219 (60.1)
Eversman D, et al. Gastrointest Endosc 1999;50:637-41.
Pancreatic SOD
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Consider in idiopathic acute recurrent pancreatitis
Type I: Acute pancreatitic pain, dilated
MPD (6 mm head, 5 mm body) Amylase or lipase > 1.5 x normal at least one occasion
Type II: Pain + one other criterion
Type III: Pain only
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Choledochal Cysts
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Congenital anomalies of the biliary tree
Incidence: 1/15,000
15x’s more common in Japan
Females more commonly affected
2/3 cases manifest by age 10
Choledochal Cysts
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Type I: (most common) 80-90% of cases.
Fusiform dilation of CBD (segmental or diffuse)
Type II: Choledochal diverticulum
Type III: Cholodochocoele (intraduodenal dilation
Type IV: Multiple intra- extrahepatic cysts
Type V: Multiple intra- extrahepatic dilation (Caroli’s disease)
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Choledochal Cysts
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Association with anomalous union of pancreatic and biliary ducts
Jaundice, abdominal pain, intraductal stones
At risk for cholangiocarcinoma
Surgical excision
Caroli's disease associated with congenital hepatic fibrosis, renal tubular ectasia and polycystic kidney disease
IgG4 Cholangiopathy
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PSC or hilar cholangiocarcinoma “look alike”
>90% associated with autoimmune pancreatitis
Elevated IgG4 levels
Tissue diagnosis required (ampullary bx, liver bx, bile duct biopsy)
Dramatic response to steroids
Zen Y, Nakanuma Y. Int J Hepatol ;doi:10.1155/2012/472376.
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Summary
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Demographics
Types / pathogenesis
Complications of cholelithiasis
Complications of choledocholithiasis
Other gallbladder disorders
Sphincter of Oddi dysfunction
Other bile duct disorders
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