Download Mirizzi Syndrome

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THE GALLBLADDER
I. Introduction/General Information
A. Location:
1. Epigastric region
2. Right hypochondriac region
3. On inferior surface of liver
4. Between quadrate and right
lobes
B. Pear-shaped, hollow structure
Introduction/General Information, con’t.
C. Fundus slants inferiorly, to the right
D. Attached to liver by loose (areolar)
connective tissue
E. Peritoneum covers free surfaces
The Gall Bladder and Bile Ducts
Fundus
Introduction, continued …
F. Normal measurements:
7-10 cm long
~ 6 cm diameter
30 – 35 cc volume
G. Body and neck directed toward porta
hepatis
Introduction, continued …
H. Neck is continuous with cystic duct
I. Cystic duct:
1. joins common hepatic duct
2. superior and posterior to pylorus
of stomach
The Gallbladder and Biliary System with Pancreas
Introduction, continued …
J. Common Bile Duct
1. 10-15 cm long
2. Courses through lesser omentum
3. Deep to pyloric sphincter
4. Narrow tube, 1-2 mm diameter
5. Should be no more than 6 mm in
diameter
CBD, continued …
6. May be 8-10 mm in postcholecystectomy patients
7. Normally has smooth walls
8. Joins with pancreatic duct
9. convergence is seen
a. anterior to portal vein
b. posterior to head of pancreas
Introduction, continued …
K. Combined duct empties into duodenum
ampulla of Vater
@
L. Sphincter of Oddi guards duct, regulates
flow
bile
1. Closed: bile goes into gallbladder
2. Open: bile goes into duodenum
Ampulla of Vater with CBD and Pancreatic Duct
Ampulla of Vater
II. Detailed Anatomy
A. Fundus of GB:
1. may be palpated
2. in angle between lateral border of right
rectus abdominis and costal margin
3. At level of elbow
4. Most anterior visceral structure
Detailed Anatomy, con’t.
B. Body of Gallbladder
1. Visceral surface of liver
2. Deep to transverse colon or hepatic
flexure of colon
3. Descending portion of duodenum
is medial
Detailed anatomy, continued …
G. Cystic Duct
1. 3-4 cm long
2. Extends from neck of gallbladder to
common hepatic duct
3. Joins with common hepatic duct
inferior to porta hepatis
4. Spiral valve may extend into neck of
gallbladder
Biliary tract, continued …
L. Blood supply to gallbladder:
1. Cystic artery
a. arises (~ 60% of the time)
from right hepatic artery
b. passes posterior to hepatic
duct, then divides
Gallbladder Diseases
Cholelithiasis & Cholecystitis
1. Cholecystitis = inflammation of GB
2. Cholelithisis = Stone(s) in GB
Bile
• Bile
– Bile salts (primary: cholic, chenodeoxycholic acids;
secondary: deoxycholic, lithocholic acids)
– Phospholipids (90% lecithin)
– Cholesterol
• Cholesterol solubility depends on the relative concentration
of cholesterol, bile salts, and phospholipid
Types of Gallstones
• Mixed (80%)
• Pure cholesterol (10%)
• Pigmented (10%)
– Black stones (contain Ca bilirubinate, cirrhosis and
hemolysis)
– Brown stones ( biliary tract infection)
Gallstone Pathogenesis
• Pathogenesis of cholesterol gallstones involves: (1) cholesterol
supersaturation in bile, (2) crystal nucleation, (3) gallbladder
dysmotility, (4) gallbladder absorption
• Black pigment stones: contain Ca++ salts, hemolytic
conditions or cirrhosis, found in the gallbladder
• Brown pigment stones: Asians, contain Ca++ palmitate, found
in bile ducts, biliary dysmotility and bacterial infection
Gallstone Risk Factors
•
•
•
•
•
•
•
•
•
•
•
•
•
•
•
“Female, Fat, Forty, Fertile”
Oral contraceptives
Obesity
Rapid weight loss (gastric bypass pts)
Fatty diet
DM
Prolonged fasting
TPN
Ileal resection
Hemolytic states
Cirrhosis
Bile duct stasis (biliary stricture, congenital cysts, pancreatitis, sclerosing
cholangitis)
IBD
Vagotomy
Hyperlipidemia
Cholecystitis (Acute & Chronic)
ESSENTIAL FEATURES
• Cholesterol stones form in 20% of women and 10% of men by age 60
• Cholesterol stone risk factors include:
–Female gender
–Age
–Obesity
–Estrogen exposure
–Fatty diet
–Rapid weight loss
EPIDEMIOLOGY
• Symptoms develop in about 3% of asymptomatic patients each year (20–
30% over 20 years)
• Acalculous cholecystitis affecting patients with acute, severe systemic illness
CLINICAL FINDINGS
SYMPTOMS AND SIGNS
• Biliary colic but becoming unremitting and steady
in epigastrium or right upper quadrant
• Fever
• Nausea
• Vomiting
• Right upper quadrant pain to palpation with
peritoneal signs
• Murphy sign
• Anorexia
LABORATORY FINDINGS
• Leukocytosis
IMAGING FINDINGS
• Right upper quadrant US showing gallstones,
gallbladder wall thickening (> 4 mm), or pericholecystic
fluid (no stones if acalculous cholecystitis)
• HIDA scan showing failure of filling of gallbladder (>
95% sensitive)
• CT showing gallbladder wall thickening (> 4 mm),
pericholecystic fluid (for patients with suspected
acalculous cholecystitis) as sensitive as US
DIAGNOSTIC CONSIDERATIONS
• Other causes of acute abdominal pain
• Gallbladder: cholecystitis, choledocholithiasis, cholangitis
• Duodenal ulcer
• Hepatitis
• Appendicitis (atypical presentation)
• Pancreatitis
RULE OUT
• Choledocholithiasis
• Pancreatitis
WORK-UP
• History and physical exam
• CBC
• Amylase and lipase
• Liver function tests
• ALP, GGT
• Right upper quadrant US
• HIDA scan for difficult cases
• CT if abdominal US not technically possible
(patients with suspected acalculous cholecystitis,
large wounds etc)
• HIDA scan (Hepatobiliary Imino-Diacetic Acid scan) - radionuclide IV,
extracted from blood, excreted into bile
– Uptake by liver, GB, CBD, duodenum w/in 1hr = normal
– Slow uptake = hepatic parenchymal disease
– Filling of GB/CBD w/delayed or absent filling of intestine = obstruction
of ampulla
– Non-visualization of GB w/ filling of the CBD and duodenum = cystic
duct obstruction and acute cholecystitis (95% sensitivity & specificity)
TREATMENT AND MANAGEMENT
MEDICAL
• All patients require IV fluids and antibiotics
• Management then can include either early cholecystectomy
(generally preferred) or cholecystectomy after about 6 weeks
SURGERY
• Laparoscopic cholecystectomy
• Open cholecystectomy
• Cholecystostomy tube (if cholecystectomy too hazardous)
Indications
• Suspected acute cholecystitis
• Suspected acalculous cholecystitis
• Failure to resolve cholecystitis on antibiotics
Complications
• Bile duct injury or leak
• Empyema
–Suppurative cholecystitis occurs with frank pus in the gallbladder, high fever, chills and systemic toxicity
–Percutaneous drainage or cholecystectomy is necessary
• Pericholecystic abscess
–Localized perforation at the gallbladder can result in a pericholecystic abscess
–Treatment requires drainage with or without initial cholecystectomy
• Free perforation
–Rare but causes generalized peritonitis
–This occurs when a gangrenous portion of the wall necroses prior to local adhesion formation
–The diagnosis is rarely made before urgent laparotomy
–Treatment is cholecystectomy
• Cholecystoenteric fistula
–Perforation at the gallbladder into an adjacent viscous generally resolves the acute episode
–Symptomatic fistula and/or patients with continued gallstone symptoms should have cholecystectomy
and closure at the fistula
Gallbladder Diseases, continued …
Polyps of GB
a. Intraluminal echogenic projections
b. do not change position with patient
c. Must be differentiated from
septations, mucosal folds
1. septations extend across lumen
2. folds change configuration
upon inspiration
Cholelithiasis
ESSENTIAL FEATURES
• Divided into symptomatic and asymptomatic
• Caused by cholesterol (most common), black pigment, or brown pigment stones
• Cholesterol stones form in 20% of women and 10% of men by age 60
• Cholesterol stone risk factors include:
–Female gender
–Age
–Obesity
–Estrogen exposure
–Fatty diet
–Rapid weight loss
• Black pigment stone risk factors include:
–Hemolytic disorders
–Living in Asia
• Brown pigment stone risk factors include:
–Biliary stasis
–Biliary infections
EPIDEMIOLOGY
• 20 million affected in United States
• Symptoms develop in about 3% of asymptomatic patients each year (20–30% over 20 years)
• Each year, complicated gallstone disease affects 3–5% of patients who are symptomatic
CLINICAL FINDINGS
SYMPTOMS AND SIGNS
• Asymptomatic
• Biliary colic
–Right upper quadrant or epigastric
–Episodic, often after meals or at night, lasting as long as
2–4 hours
• Nausea
• Vomiting
• Diarrhea
• Mild right upper quadrant tenderness to palpation
LABORATORY FINDINGS
• Normal liver function tests, normal amylase
and lipase, normal WBC count
IMAGING FINDINGS
• Right upper quadrant US showing acoustically
dense stones in gallbladder with acoustic
shadowing without evidence of gallbladder
wall thickening or pericholecystic fluid (> 90%
sensitive for gallstones)
DIAGNOSTIC CONSIDERATIONS
• Other causes of abdominal pain
RULE OUT
• Cholecystitis
• Choledocholithiasis
• Pancreatitis
WORK-UP
• History and physical exam
• CBC
• Liver function tests
• Amylase and lipase
• Right upper quadrant US
TREATMENT AND MANAGEMENT
SURGERY
• Laparoscopic cholecystectomy
• Open cholecystectomy
Indications
• Symptomatic cholelithiasis
• Porcelain gallbladder (25% risk of carcinoma)
Contraindications
• First or third trimester of pregnancy (relative)
• Previous upper abdominal surgeries (laparoscopic)
Complications
• Bile duct injury or leak
Cholelithiasis, Rare Complications
ESSENTIAL FEATURES
Gallstone Ileus
• Small bowel obstruction secondary to 1 or more large
gallstones entering via cholecystoduodenal fistula
Mirizzi Syndrome
• Biliary stricture secondary to direct compression by
chronically impacted cystic duct gallstone or chronic
inflammation secondary to chronically inflamed gallbladder
EPIDEMIOLOGY
• Both gallstone ileus and Mirizzi syndrome are rare
complications mainly affecting patients older than 60 years
CLINICAL FINDINGS
SYMPTOMS AND SIGNS
Gallstone Ileus
• Signs and symptoms of small bowel
obstruction and possible antecedent history of
biliary colic
Mirizzi Syndrome
• Chronic or history of right upper quadrant pain
along with jaundice
LABORATORY FINDINGS
Gallstone Ileus
• Hypokalemia
• Prerenal azotemia
• Hypernatremia
• Leukocytosis
Mirizzi Syndrome
• Hyperbilirubinemia
• Elevated alkaline phosphatase
IMAGING FINDINGS
Gallstone Ileus
• Abdominal x-ray
–Air-fluid levels
–Dilated loops of small bowel
–Possible pneumobilia
• US
–Cholelithiasis and pneumobilia
• Hypaque swallow
–Fistula between duodenum and gallbladder
Mirizzi Syndrome
• US
–Biliary dilatation (> 6 mm)
–Cholelithiasis
–Possible thickened wall of gallbladder
• ERCP or percutaneous transhepatic cholangiogram (PTC)
–Stricture of common bile duct
DIAGNOSTIC CONSIDERATIONS
Gallstone Ileus
• Overall clinical status of patient
Mirizzi Syndrome
• Evaluate for malignant causes of stricture
WORK-UP
Gallstone Ileus
• History and physical exam
• CBC
• Electrolytes
• Blood urea nitrogen, creatinine
• Plain abdominal x-ray
• Right upper quadrant US
• Small bowel contrast study if partial small bowel obstruction
Mirizzi Syndrome
• History and physical exam
• CBC
• Liver function tests
• Right upper quadrant US
TREATMENT AND MANAGEMENT
SURGERY
Gallstone Ileus
• Removal of retained small bowel gallstone(s) via
enterostomy or partial resection if bowel damaged
• Cholecystectomy and resection of fistula and duodenal
closure at same operation or as staged procedure
Mirizzi Syndrome
• Cholecystectomy and resection and/or bypass of stricture
via hepaticojejunostomy
Contraindications
• Clinical status of patient during laparotomy for gallstone
ileus
Complications
Gallstone Ileus
• Missed enteral gallstone and recurrent
obstruction
• Duodenal leak
• Bile duct injury or leak
Mirizzi Syndrome
• Anastomotic leak or stricture
• Cholangitis
Choledocholithiasis & Gallstone
Pancreatitis
ESSENTIAL FEATURES
• Cholesterol stone risk factors include:
–Female gender
–Age
–Obesity
–Estrogen exposure
–Fatty diet
–Rapid weight loss
• Complicated gallstone disease affects < 0.5% annually of patients
who are asymptomatic
EPIDEMIOLOGY
• Average age generally 10 years older than those affected by
cholelithiasis (eg, 40–50 years of age)
CLINICAL FINDINGS
SYMPTOMS AND SIGNS
• Right upper quadrant pain
• Painless jaundice
• Both pain and jaundice
• Fever
• Asymptomatic
• Nausea
• Vomiting
• Anorexia
LABORATORY FINDINGS
• Conjugated hyperbilirubinemia (for choledocholithiasis)
• Elevated alkaline phosphatase (for choledocholithiasis)
• Leukocytosis (for pancreatitis or cholangitis)
IMAGING FINDINGS
• Right upper quadrant US showing presence of gallstones,
dilated common bile duct (CBD) (> 6 mm) and CBD stone in
only 20–30% of patients with choledocholithiasis
• ERCP showing dilated CBD and presence of single or
multiple CBD stones in patients with choledocholithiasis
• ERCP showing impacted ampullary gallstone in < 10 % of
patients with gallstone pancreatitis
DIAGNOSTIC CONSIDERATIONS
• Presence of signs or symptoms suggestive of
cholangitis
RULE OUT
• Biliary stricture
WORK-UP
• History and physical exam
• CBC
• Liver function tests
• Amylase and lipase
• Right upper quadrant US
• ERCP or laparoscopic cholangiogram
TREATMENT AND MANAGEMENT
SURGERY
• ERCP with sphincterotomy and stone extraction followed by laparoscopic cholecystectomy (preferred when
cholangitis present or if pancreatitis does not resolve)
• Laparoscopic cholecystectomy with CBD exploration
• Laparoscopic cholecystectomy and cholangiogram followed by ERCP and stone extraction (preferred stone extraction
technique is center specific for stones noted on screening cholangiogram following resolution of gallstone
pancreatitis)
• Percutaneous transhepatic cholangiography and stone extraction if ERCP unsuccessful and cholangitis present
Indications
• Choledocholithiasis noted to be symptomatic or asymptomatic
• Gallstone pancreatitis
MEDICATIONS
• Antibiotics to cover GI flora for cases of cholangitis
Complications
• Pancreatitis (for ERCP)
• Bile duct injury or leak
Prognosis
• Gallstone pancreatitis resolves in > 90% of cases
Prevention
• Treatment of symptomatic cholelithiasis
Case 1
• HPI: 46y F 4hr nausea and RUQ pain radiating to the R
scapula. Symptoms began 1 hr after a fatty meal. Pt
currently has no pain. No prior episodes.
• PE: RUQ minimally TTP, (-)Murphy’s
• Labs: WBC 8, LFT normal
• Studies: RUQ U/S : cholelithiasis without GB wall thickening
or pericholecystic fluid
• What is the diagnosis?
Case 1
• → denotes
gallstones
→
→
►
• ► denotes the
acoustic shadow
due to absence of
reflected sound
waves behind the
gallstone
Case 1: Continued
• Dx: symptomatic cholelithiasis
• Plan: NPO, IVF, cholecystectomy
Case 2
• 46y F 4hr nausea and RUQ pain radiating to the R scapula.
Symptoms began 1 hr after a fatty meal. Pt currently has no
pain. Has had multiple similar episodes.
• PE: RUQ minimally TTP, (-)Murphy’s
• Labs: WBC 6, LFT normal
• Studies: RUQ U/S :cholelithiasis without GB wall thickening
or pericholecystic fluid
• Diagnosis: ?
Case 2: Continued
• Dx: chronic calculous cholecystitis
• Recurrent inflammatory process due to recurrent
cystic duct obstruction leading to scarring/wall
thickening
• Treatment: cholecystectomy
Case 3
• 46yF >24hr of RUQ pain radiating to the R scapula, started
after fatty meal, nausea, vomiting, fever
• Exam: Febrile, RUQ TTP, (+)Murphy’s sign
• Labs: WBC 13, Mild ↑LFT
• U/S: gallstones, wall thickening, GB distension,
pericholecystic fluid, sonographic Murphy’s sign
• What is the diagnosis?
Case 3: Continued
• Curved arrow
– Two small stones at
GB neck
◄
• Straight arrow
– Thickened GB wall
• ◄
– pericholecystic fluid
= dark lining outside
the wall
Case 3: Continued
→
►
• → denotes the GB wall
thickening
• ► denotes the fluid
around the GB
• GB also appears
distended
Case 3: Continued
• Dx: acute calculous cholecystitis
• Persistent cystic duct obstruction leads to GB distension, wall
inflammation & edema
• Risk of: empyema, gangrene, rupture
• Treatment:
– NPO
– IVF
– ABX:
• Common organisms: E coli, Bacteroides fragilis, Klebsiella,
Enterococcus, and Pseudomonas
• Piperacillin/tazobactam (Zosyn), ampicillin/sulbactam
(Unasyn), or meropenem
– Cholecystectomy
Case 4
• 87y M critically ill, on long-term TPN RUQ pain
• PE: febrile, RUQ
• U/S: GB wall thickening, pericholecystic fluid,
no gallstones
• What is the diagnosis?
Case 4: Continued
• Dx: acute acalculous cholecystitis
• Caused by gallbladder stasis from lack of enteral
stimulation by cholecystokinin
• Risk of: gangrene, empyema, perforation due to
ischemia
• TX: cholecystectomy
• If pt is too sick, percutaneous cholecystostomy tube
followed by cholecystectomy
Case 5
• 46y F RUQ pain, jaundice, acholic stools, dark teacolored urine, w/o fever
• PMHx: cholelithiasis
• Exam: unremarkable
• WBC 8, T.Bili 8, AST/ALT NL, Hep B/C neg
• U/S: gallstones, CBD stone, dilated CBD > 1cm
• What is the diagnosis?
Case 5: Continued
• DX: choledocholithiasis
• Similar presentation as cholelithiasis, except with the
addition of jaundice
• DDx: cholelithiasis, hepatitis, cholangitis, CA, choledochal
cyst, bile duct stricture, UC, pancreatitis
• Plan:
– Endoscopic retrograde cholangiopancreatography
(ERCP) stone extraction and sphincterotomy
– Interval cholecystectomy after recovery from ERCP
Case 6
• 46y F p/w fever, RUQ pain, jaundice
• PE: tachycardic, hypotensive, RUQ pain
• Immediate management:
– ABC
– Resuscitate
– CBC, LFTs, blood cultures
– Abdominal U/S
• What is the diagnosis?
• What is the plan?
Case 6: Continued
• Dx: cholangitis
• Infection of the bile ducts due to CBD obstruction secondary to
stones/strictures
• Common organisms: E. coli, Klebsiella, Pseudomonas, Enterobacter,
Proteus, Serratia
• 70% Charcot’s
• May lead to life-threatening sepsis and septic shock (Raynaud’s
pentad)
• Common lab findings: leukocytosis, hyperbili, elevated alk phos
• Treatment:
– NPO, IVF, IV ABX
– Emergent decompression via ERCP or perc transhepatic
cholangiogram (PTC)
Case 7
•
•
•
•
•
46y F persistent epigastric & back pain
PMHx: symptomatic gallstones
PE: Tender epigastrum
Labs: Amylase 2000, ALT 150
U/S: gallstones
• What is the diagnosis?
• What is the plan?
Case 7: Continued
• Dx: gallstone pancreatitis
• 35% of acute pancreatitis secondary to stones
• Pathophysiology: reflux of bile into pancreatic duct and/or
obstruction of ampulla by stone
• ALT >150 (3-fold elevation) has 95% PPV for diagnosing
gallstone pancreatitis
• Treatment:
– ABC, resuscitate, NPO/IVF, pain medication
– ERCP once pancreatitis resolves
– Cholecystectomy
Choledochal Cyst
ESSENTIAL FEATURES
• Type I cysts (fusiform dilation of common bile duct [CBD])
account for 85–90%
• Type II (true diverticula of CBD) 1–2% of cases
• Type III (choledochocele–-dilation of distal/intramural
portion of CBD) < 2% of cases
• Type IV (multiple cysts involving intrahepatic and
extrahepatic ducts) as high as 15% of cases in some series
• Type V (cystic malformation of intrahepatic ducts) rare
• 3–5% incidence of carcinoma
EPIDEMIOLOGY
• Onset of symptoms usually in infancy or childhood
CLINICAL FINDINGS
SYMPTOMS AND SIGNS
• Jaundice
• Fever
• Pain
• Palpable right upper quadrant mass
• Hepatomegaly
• Bleeding varices
• Asymptomatic
LABORATORY FINDINGS
• Hyperbilirubinemia
• Elevated alkaline phosphatase
• Leukocytosis
• Elevated amylase and lipase
IMAGING FINDINGS
• US showing characteristic cystic dilation of biliary tree
corresponding to type as well as proximal dilation in
presence of obstruction
• ERCP or magnetic resonance cholangiopancreatography
(MRCP) showing cystic dilation corresponding to type and
proximal obstruction in presence of obstruction
WORK-UP
• History and physical exam
• Liver function tests
• CBC
• Amylase and lipase
• Abdominal US
• ERCP or MRCP (adults)
• HIDA scan or MRCP (children)
TREATMENT AND MANAGEMENT
SURGERY
• Cyst excision and biliary reconstruction (types I–III)
• Types IV and V individualized and may require partial
hepatectomy if unilobar involvement
TREATMENT MONITORING
• Surveillance for carcinoma since patients still at
increased risk for remainder of biliary tree
Complications
• Biliary stricture or leak
• Cholangitis
Cholangiocarcinoma
ESSENTIAL FEATURES
• Arises from biliary epithelium
• Risk factors
–Primary sclerosing cholangitis
–Choledochal cysts
–Clonorchis infection
EPIDEMIOLOGY
• < 4500 patients per year
• Average age, 50–70 years
• Evenly distributed among men and women
CLINICAL FINDINGS
SYMPTOMS AND SIGNS
• Painless jaundice
• Right upper quadrant pain
• Pruritus
• Anorexia
• Malaise
• Weight loss
• Cholangitis
• Asymptomatic
LABORATORY and IMAGING FINDINGS
LABORATORY FINDINGS
• Hyperbilirubinemia
• Elevated alkaline phosphatase
• Elevated CA 19-9
IMAGING FINDINGS
• US showing dilated extrahepatic and intrahepatic biliary ducts
(depending on level of tumor)
• CT or MRI with biliary dilatation and occasional visible hepatic tumor
• Percutaneous transhepatic cholangiography (PTC) or magnetic
resonance cholangiopancreatography (MRCP) visualizing proximal
and distal extent of tumor
–PTC provides opportunity for brushings for cytologic studies of tumor
• Mesenteric angiography for question of portal vasculature
involvement
DIAGNOSTIC CONSIDERATIONS
• History of pancreatitis (possible benign stricture)
• History of ulcerative colitis (possible primary
sclerosing cholangitis)
• Choledocholithiasis
RULE OUT
• Extrahepatic disease or bilobar involvement
• Choledocholithiasis
WORK-UP
• History and physical exam
• Liver function tests
• CA 19-9
• US to screen for anatomic causes of
hyperbilirubinemia
• Abdominal CT
• PTC or MRCP (PTC if brushings needed)
• Angiography if portal vessel involvement
suspected
TREATMENT AND MANAGEMENT
SURGERY
• Biliary resection followed by biliary-enteric resection
• Extended right or left lobectomy if proximal disease noted (isolated to 1 side) above
secondary radicals or if unilateral portal vein or hepatic artery involvement
• Pancreaticoduodenectomy (Whipple) for distal common bile duct (CBD) tumors
• Biliary-enteric bypass for PTC-placed wall stent for palliation
Indications
• Resectable cholangiocarcinoma or diagnosis of benign stricture can be difficult to
distinguish
• Presence of choledochal cyst
Contraindications
• Bilobar involvement or second order biliary radicals bilaterally
• Extrahepatic disease
• Main portal vein, bilateral portal vein, or bilateral hepatic artery involvement
Complications
• Anastomotic leak or stricture
• Cholangitis
• Recurrent disease
• Liver failure
• Hemorrhage
Prognosis
• 10–30% 5-year survival with curative resection of
proximal biliary tumor
• 30–50% 5-year survival with distal CBD tumor
Cholangitis, Primary Sclerosing
ESSENTIAL FEATURES
• Associated with ulcerative colitis 40–60%,
pancreatitis 12–25%, diabetes mellitus 5–10%,
and rarely other autoimmune disorders
• Onset during fourth or fifth decade of life
• Increased risk for cholangiocarcinoma
CLINICAL FINDINGS
SYMPTOMS AND SIGNS
• Intermittent jaundice
• Fever
• Right upper quadrant pain
• Pruritus
LABORATORY FINDINGS
• Elevated alkaline phosphatase
• Hyperbilirubinemia
• Leukocytosis
IMAGING FINDINGS
• Right upper quadrant US, ERCP, and magnetic resonance
cholangiopancreatography (MRCP) may show multiple dilatations
and strictures of extrahepatic biliary ducts
DIAGNOSTIC CONSIDERATIONS
• Cholangiocarcinoma
• Presence of cirrhosis
RULE OUT
• Cholangiocarcinoma
WORK-UP
• History and physical exam
• Liver function tests
• Abdominal US
• ERCP
• Liver biopsy if question of cirrhosis
• Brushings by percutaneous transhepatic
cholangiography (PTC) or ERCP if question of
malignancy
TREATMENT AND MANAGEMENT
SURGERY
• Balloon dilatation of multiple strictures
• Resection of dominant stricture followed by biliary reconstruction
• Liver transplantation (preferably before onset of cirrhosis)
Contraindications
• Cirrhosis
MEDICATIONS
• Ursodiol, also known as ursodeoxycholic acid and the abbreviation
UDCA, is one of the secondary bile acids, which are metabolic
byproducts of intestinal bacteria.
(improves liver function and histology but no difference in 5-year
clinical outcome)
TREATMENT MONITORING
• Alkaline phosphatase levels
Complications and Prognosis
Complications
• Cholangitis
• Recurrent strictures
• Primary nonfunction
• Allograft rejection
• Recurrent stricture post-transplant
Prognosis
• 85% survival rate 5 years post transplant
• 71% actuarial survival at 5 years for resection of dominant
stricture (only 20% if cirrhosis present)
• 43% long-term success with balloon therapy for multiple
strictures
Gallbladder Cancer
Epidemiology
•
•
•
•
5th most common GI malignancy
Women > men
High incidence in S America (Chile)
pt’s undergoing cholecystectomy for
symptomatic gallstones
Risk Factors
•
•
•
•
Gallstones
Gallbladder Polyps
Chronic Salmonella infection
Abnormal Pancreaticobiliary duct junction
* Porcelain gallbladder
* Age
Presentation/Diagnostic Imaging
• Presentation is non-specific
• Diagnositic Imaging
– Sono
– CT
– MR/MRCP
– EUS
Histology / Pathology
* Progression to Ca may take up to 15 yrs
•
•
•
•
Adenocarcinoma 80-90%
Anaplastic 7%
Squamus 6%
Lymphoma, Sarcoma
Staging
Surgical Management
• Only 10-30% resectable @ time of diagnosis
• Three Presentations:
– GB CA discovered during or after lap/open chole
for assumed benign dz
– GB CA suspected after diagnostic evaluation
– GB CA advanced stage at presentation
Surgical Options
•
•
•
•
Simple cholecystectomy
Radical cholecystectomy
Radical chole + anatomic liver resection
Radical chole + Whipple
What to do during elective lap chole if GB
Ca is suspected intraoperatively ?
• cases found to have GB CA in lap chole
• Convert to OPEN
• Resect PORTS
• No place for laparoscopic resection
Management of locally unresectable
(major vascular encasement)
• NO DEBULKING
• Chemoradiotherapy
– No identified impact on survival & remains
investigational
– Systemic chemotherapy – no optimal regimen
defined (5-FU based)