Diseases of the Gallbladder

and Bile Ducts

Chapter 339
Norton J. Greenberger, Gustav Paumgartner
Harrison’s Principles of Internal Medicine 20 th edition
Reporter:
PGI Mc Ivan A. Alberto
 Bile

 Formed in the hepatic lobules

 Stored and concentrated in gallbladder
 Daily basal secretion: 500-600 ml
 Bile components:
 Bile acids (80%)
 Lecithin and other phospholipids (16%)
 Cholesterol (4%)
 Mechanism for regulation of bile flow

Active transport of bile

Active transport of other organic ions
Cholangiocellular secretion
 Bile Acids
 detergent like molecules
 Primary bile acids: cholic acid, chenodeoxycholic acid

 Secondary Bile Acids: deoxycholate, lithocholate, ursodeoxycholic

Function
Facilitate biliary excretion of cholesterol
 Facilitate normal intestinal absorption of dietary fats (cholesterol, fat soluble vitamins
 Serves as major physiologic driving force hepatic bile flow
 Aid in water and electrolyte transport in the small bowel and colon
Enterohepatic circulation

 Absorbed by passive diffusion along the entire gut( unconjugated, conjugated less)
 Active transport for conjugated bile acids in the distal ileum

Normal bile acid pool : 2 to 4 g

 Bile acid pool circulates 5-10 times daily
 Intestinal reabsorption is about 95%
 Fecal loss: 0.2-0.4g/day
 Maximum rate of synthesis: 5g/day
Gallbladder and sphincteric functions
 Bile is concentrated in gallbladder by energy dependent transmucosal absorption of water and
electrolytes
 Normal capacity of gallbladder: 30 ml

Sphincter of Oddi
 Offers high pressure zone of resistance to bile flow from CBD
into the duodenum tonic contraction serves to:
 Prevent reflux of duodenal contents into the pancreatic and
bile ducts
 Promote filling of the gallbladder
Major factor gallbladder evacuation:
Cholecystokinin
 Powerful contraction of the gallbladder
 Decrease resistance of SOD
 Enhances flow of biliary contents into the duodenum
Diseases of the gallbladder
 Congenital anomalies
 Phrygian cap—having seperation of fundus from the body with partial or
complete septum
 Agenesis of the bladder
 Duplications
 Rudimentary or oversized gallbladders
 Diverticula
 Left side gallbladder
 Intrahepatic gallbladder
 Floating gallbladder
Gallstones
 Very Common
 Prevalence increases after age 50
 Women 16.6% > men 7.9%
 Formed due to abnormal bile composition
1. Cholesterol stones 2. Pigment stones
Cholesterol stones
Account for >90% of all gallstones
 usually contain >50% cholesterol monohydrate + admixture of calcium salts + bile
pigments + proteins + fatty acids

 Mostly formed due to increased biliary secretion of cholesterol

 Risk factors: obesity, metabolic syndrome, high caloric and cholesterol rich diets,
drugs (clofibrate), genetics

 May result from increased activity of HMG CoA reductase – rate limiting enzyme
Cholesterol stones
 Important pre- requisite: supersaturation of bile with cholesterol
 Mechanism: Nucleation of cholesterol monohydrate crystals

 Accelerated nucleation may be due to

 excess of pronucleating factors : mucin , nonmucin glycoproteins
( immunoglobulins)
 deficiency of antinucleating factors : apolipoproteins A –1 and A2, other
glycoproteins

 Another mechanism: gallbladder hypomotility

 Biliary Sludge
 A thick, mucous material, microscopic examination : lecithin-cholesterol liquid
crystals, cholesterol monohydrate crystals, calcium bilirubinate and mucin gels
 Typically forms a crescent like layer in the most dependent portion of the
gallbladder echoes on ultrasonography

 Implies two abnormalities:

1. derangement of the normal balance between gallbladder
mucin secretion and elimination
2. occurrence of nucleation of biliary solutes.
 Two other conditions are associated with cholesterol-stone or biliary-
sludge formation:
 Pregnancy and rapid weight reduction through a very-low-calorie diet
There appear to be two key changes during
pregnancy that contribute to a “cholelithogenic state”:

(1) a marked increase in cholesterol saturation of bile during the third

trimester
(2) sluggish gallbladder contraction in response to a standard meal,
resulting in impaired gallbladder emptying
 Special conditions :

Pregnancy:
 Prevalence of gallbladder Sludge: 20-30 % ; usually asymptomatic ;
resolves spontaneously after delivery

 Prevalenceof gallstones : 5-12% ; frequently associated with biliary colic ;

may disappear after delivery
Pigment stones
 Primarily composed of calcium bilirubinate
 Contain < 20% cholesterol
 Classified into Black and Brown types.
Black pigment stones
 composed of either pure calcium bilirubinate or polymer like complexes with calcium and mucin
glycoproteins
 more common in patients with chronic hemolytic states and ileal related conditions
Brown pigment stones
 Composed of calcium salts of unconjugated bilirubin with varying amount of cholesterol and protein.
 formation may be mediated by endogenous beta- glucuronidase or spontaneous hydrolysis
 also associated with Chronic Biliary Infection; frequent in Asia
Diagnosis
 Ultrasonography :accurate in identification Stones as small as 1.5 mm can be identified
 False negative/ positive rate : 2-4%
 can also assess the emptying function of the gallbladder

Biliary sludge vs Gallstones

 acoustic “shadowing” of opacities that are within the gallbladder lumen and that change with
the patient’s position [by gravity])
 biliary sludge is material of low echogenic activity that typically forms a layer in the most
dependent position of the gallbladder. This layer shifts with postural changes but fails to
produce acoustic shadowing
Plain abdominal film:
 May detect gallstones containing sufficient calcium to be
radiopaque (10-15% cholesterol and ~50% of pigment
stones)
 Can be used in emphysematous cholecystitis, porcelain
gallbladder, limey bile and gallstone ileus
 Diagnosis
Oral Cholecystography (OCG)
 Historically has been a useful procedure but has been replaced by ultrasound
 Regarded as Obsolete
 May be used to assess the patency of the cystic duct and gallbladder emptying function
 Can also delineate the size and number of gallstones and determine presence of calcification
 Based on radiopharmaceuticals which are rapidly extracted from blood and excreted into the
biliary tree in high concentration
 Failure to image the gallbladder in the presence of biliary duct visualisation may indicate
cystic duct obstruction, acute or chronic cholecystitis or surgical absence of the organ
Symptoms of Gallstone Disease

Symptoms are produced by inflammation or obstruction due to migration of stones

into the cystic duct or CBD
 Most specific and characteristic symptom: BILIARY COLIC

• constant and often long lasting pain

• sudden onset persisting with severe intensity for 30 min to 5 hours

• located in the epigastrium or right upper quadrant

Symptoms of Gallstone Disease

• frequently radiated to the interscapular area, right scapula or shoulder

• an episode persisting beyond 5 hours suspect acute cholecystitis

• caused by increased intraluminal pressure and distension of the viscus due to

obstruction of the cystic duct or CBD can not be relieved by repetitive biliary
contractions
 Treatment
 Recommendation for cholecystectomy is based on :

1. the presence of symptoms that are frequent / severe enough to interfere with the
patients general routine

2. the presence of prior complication of gallstone disease

3. the underlying condition predisposing the patient to increased risk of gallstone

complications
 Prophylactic cholecystectomy

• patient with very large gallstones (>3cm in diameter)

• patients harbouring gallstones in a congenitally anomalous gallbladder

 Laparoscopic cholecystectomy --- gold standard treatment for symptomatic

cholelithiasis
 Medical Therapy
Can be instituted in selected patients with

a functioning gallbladder and with radiolucent stones < 10 mm in

diameter

 complete dissolution can be achieved in ~ 50% of patients within 6

months to 2 years

 For good results , limited to radiolucent stones < 5mm in diameter

 Dose of UDCA : 10-15 mg/kg per day

 Pigment stones are not responsive to UDCA therapy

 High recurrence rate after treatment ( 30-50% over 3-5 years follow up)

 Recurrent choledocholithiasis after cholecystectomy--- long term

treatment with UDCA.
 Acute cholecystitis
 Defined as Acute inflammation of the gallbladder wall usually follows obstruction of the cystic duct by
a stone

Factors for inflammatory response:

1. mechanical inflammation

2. chemical inflammation

3. bacterial inflammation
Acute cholecystitis
Begins as an attack of biliary pain with progressive worsening

60-70% may have history of prior attack with spontaneous resolution

Pain is more generalized in the right upper quadrant

may radiate to the interscapular area, right scapula or shoulder

peritoneal signs of inflammation may present- -increased pain with

jarring or on deep respiration
 Acute cholecystitis

 Vomiting, nausea and anorexia

 Low grade fever may be with shaking chills or rigors

 RUQ invariably tender to palpation

 Enlarged, tense gallbladder – palpable in 25-50% of patients

 Acute cholecystitis

 Murphy’s Sign : deep inspiration or cough during subcostal palpation of the RUQ
produces increased pain and inspiratory arrest

 Localised rebound tenderness in RUQ, abdominal distension, hypoactive bowel sounds

( paralytic ileus)

 Generalized peritoneal signs and abdominal rigidity – in the presence of perforation

Diagnosis
 Usually made on the basis of a characteristic history and physical examination
 Presence of Triad : Sudden onset of RUQ tenderness ; fever ; leukocytosis
 Leukocytosis: 10,000 – 15,000 /microlitre
 Serum bilirubin : mildly elevated ( 5 mg/dL) in less than half of the patients
 Serum aminotransferase : moderately elevated ( less than 5 fold) 1/4 the of the
patients
 Ultrasound may demonstrate calculi in 90-95% of cases
Diagnosis

Ultrasound
useful for detection of signs of gallbladder inflammation
• thickening of the wall
• pericholecystic fluid
• dilatation of the bile duct
HIDA
• radionuclide biliary scan
• may be confirmatory if bile duct imaging is seen without visualization of the
gallbladder
•

•
Treatment

Medical treatment
 High remission rate (75%) of acute symptoms within 2-7 days
 High complications rate (25%) despite therapy

Surgical treatment intervention is required

✓early surgery whenever possible

Mirizzi’s syndrome – preoperative diagnosis is important

- to avoid CBD injury
Acalculous Cholecystitis

 Present in 5-10% of patients with acute cholecystitis

 Calculi obstructing the cystic duct not found at surgery
 Risk factors for development : prolonged fasting, serious trauma or burns
postpartum period following prolonged labor, orthopaedic and other non biliary
major surgical operations in postoperative period
 Precipitating factors: vasculitis, obstructing adenocarcinoma of the gallbladder,
DM, torsion of the gallbladder, bacterial Infections / parasitic infestation of
gallbladder,
 Management : early diagnosis and surgical intervention with meticulous attention
to postoperative care
Acalculous Cholecystopathy
 Disordered motility of the gallbladder causes recurrent biliary pain in
patients without gallstones.
 Infusion of an octapeptide of CCK can be used to measure the gallbladder
ejection fraction during Cholecystography
 Criteria used to identify patients:
1. recurrent episodes of typical RUQ pain characteristic of billiary tract pain
2. abnormal CCK cholescintigraphy demonstrating a gallbladder ejection
fraction of < 40%
3. infusion of CCK reproducing the patients pain

Additional clue: identification of a large gallbladder on ultrasound examination

Emphysematous cholecystitis
 Begin with acute cholecystitis followed by ischemia or gangrene of the gallbladder wall and
infection by gas producing organisms

Most frequent bacteria : Clostridium welchii , C. Perfringens, E. Coli

 Occurs most frequently in elderly men and DM patients

 Diagnosis is made on plain abdominal film by finding gas within the gallbladder lumen ;
dissecting within the gallbladder wall to form a gaseous ring or in the pericholecystic tissues

 Management: prompt surgical intervention + appropriate antibiotics

 High morbidity and mortality

Chronic Cholecystitis

 Chronic inflammation of the gallbladder wall

 Almost always associated with the presence of gallstones
 Results from repeated bouts of subacute/acute cholecystitis OR from persistent
mechanical irrigation of the gallbladder wall by gallstones
 Presence of bacteria in bile in > 25% of the patients
 Maybe asymptomatic for years
Complications of Cholecystitis
Empyema and Hydrops

• usually results from progression of acute cholecystitis with persistent cystic duct
obstruction
• superinfection of the stagnant bile with a pus forming bacterial organism
• clinical picture : high fever , severe RUQ pain, marked leukocytosis , prostration
• carries a high risk of gram negative sepsis and/ or perforation
• Management: emergency surgical intervention+ proper antibiotics
Hydrops or Mucocele of the gallbladder
• results from prolonged obstruction of the cystic duct – large solitary calculus
• cholecystectomy is indicated, because empyema, perforation and gangrene may
complicate the condition

Physical examination: a visible, easily palpable, non tender mass sometimes extending
from the RUQ into the right illiac fossa

Management : Cholecystectomy to avoid complications ( empyema, perforation,

gangrene)
Gangrene and perforation

results from ischemia of the wall and patchy/ complete tissue necrosis
• gangrene usually predisposes to perforation of the gallbladder
• Bacterial superinfection - Abscess formation
• localised perforation usually contained by omentum/ adhesions
• Most patients are best treated with cholecystectomy
• sudden transient relief of RUQ pain as the distended gallbladder decompresses;
this is followed by signs of generalized peritonitis.
Fistula formation

• fistula formation may be occur into an adjacent organ adherent to the gallbladder
wall
• may result from inflammation and adhesion formation
• most commonly into the duodenum, hepatic flexure of the colon, stomach or
jejunum, abdominal wall, and renal pelvis.
• clinically “silent” biliary-enteric fistulas occurring as a complication of acute
cholecystitis have been found in up to 5% of patients

Treatment: cholecystectomy , CBD exploration and closure of the fistulous tract

Gallstone ileus

mechanical intestinal obstruction resulting from the passage of a large gallstone into
the bowel lumen
stone enters the duodenum through a cholecystoenteric fistula
usual site of obstruction – ileocecal valve
• large stones > 2.5 cm causes gradual erosion through the gallbladder fundus
forming fistula

Treatment/ Procedure of choice : laparotomy with stone extraction/ propulsion into

the colon - procedure of choice
evacuation of large stones within the gallbladder
Limey ( milk of calcium) Bile
calcium precipitation and diffuse , hazy opacification of bile or a layering effect
on plain abdominal roentgenography
 Cholecystectomy is recommended ( especially if with hydropic gallbladder)

Porcelain gallbladder
 calcium salts deposition within the wall of a chronically inflammed
gallbladder
 detected on the plain abdominal film
 highly associated with carcinoma of gallbladder
 Cholecystectomy is advised
 Treatment: Acute Cholecystitis
Medical Therapy
• a period of in-hospital stabilization may be required before cholecystectomy.

• Oral intake is eliminated, nasogastric suction, and extracellular volume depletion and
electrolyte abnormalities are repaired.

• Meperidine or nonsteroidal anti-inflammatory drugs (NSAIDs) such as ketoralac or

opioids, employed for analgesia.

• Intravenous antibiotic therapy is usually indicated in patients with severe acute

cholecystitis
Effective antibiotics include
 piperacillin plus tazobactam
 ceftriaxone plus metronidazole
 levofloxacin plus metronidazole

• Imipenem and meropenem should be reserved for the most severe, life-
threatening infections when other regimens have failed

• Postoperative complications of wound infection, abscess formation, and sepsis

are reduced in antibiotic-treated patients
Surgical Therapy

• The optimal timing of surgical intervention in patients with acute cholecystitis depends on
stabilization of the patient.

• Urgent (emergency) cholecystectomy or cholecystostomy is probably appropriate in most patients

in whom a complication of acute cholecystitis such as empyema, emphysematous cholecystitis, or
perforation is suspected or confirmed

• Patients with uncomplicated acute cholecystitis should undergo early elective laparoscopic
cholecystectomy, ideally within 48–72 h after diagnosis.
Delayed surgical intervention
(1) patients in whom the overall medical condition imposes an unacceptable risk for early
surgery
(2) patients in whom the diagnosis of acute cholecystitis is in doubt.

Thus, early cholecystectomy (within 72 h) is the treatment of choice for most patients with
acute cholecystitis.

 Mortality rate 1 to 3%
 mortality risk for early elective cholecystectomy is ~0.5% in patients under age 60.
 Seriously ill or debilitated patients with cholecystitis may be managed with
cholecystostomy and tube drainage of the gallbladder.
 Elective cholecystectomy may then be done at a later date.
Postcholecystectomy Complications

 Early complications : atelectasis , abscess formation, external/ internal hemorrhage, biliary enteric
fistula, bile leaks (jaundice)

 Postcholecystectomy syndrome
 Biliary strictures
 retained biliary caliculi
 cystic duct stump syndrome
1. stenosis dyskinesia of the SOD
2. bile salt induced diarrhoea or gastritis
Cystic duct stump syndrome
• symptoms resembling biliary pain or Cholecystitis in the postcholecystectomy
patient in the ABSENCE of cholangiographically demonstrable retained
stones
• attributed to disease in a long (> 1 cm) cystic duct remnant
SOD Stenosis
• results from Acute or chronic inflammation of the papilla of Vater or from glandular hyperplasia of the
papillary segment

Definition Criteria
1. Upper abdominal pain ( RUQ/ epigastrium)
2. Abnormal liver tests
3. dilatation of the CBD upon MRCP /ERCP examination
4. Delayed (> 45 min ) drainage of contrast material from the duct
5. increased basal pressure of the SOD
SOD stenosis

Treatment : endoscopic or surgical sphincteroplasty AFTER exclusion of Acalculous

Cholecystopathy

Indications for sphincterotomy

1. prolonged duration of symptoms

2. Lack of response to symptomatic treatment
3. Presence of severe disability
4. Patients choice of sphincterotomy over surgery
Biliary SOD disorders ( SOD dyskinesia, biliary dyskinesia)
Criteria :
Biliary pain
1. Absence of bile duct stones or other abnormalities
2. Elevated liver enzymes or a dilated CBD but not both

Proposed mechanisms to account SOD dysfunction

3. Spasm of the sphincter
4. Denervation sensitivity resulting in hypertonicity
5. Abnormalities in the sequencing or frequency rates of the sphincteric contraction
waves
Treatment:
Medical Therapy : nitrites or anticholinergics ( relaxation of SOD)

Surgical:
1. when fail to respond to a 2- 3 months trial of medical therapy
2. when SOD pressures are elevated

endoscopic biliary sphincterotomy / surgical sphincterotomy

• long term pain relief in 45 % of the patients
Bile Salt induced diarrhoea and gastritis
After cholecystectomy :
• Dyspepsia --- attributed to duodenogastric reflux of bile

• persistent change in gut transit ; modification of bowel habits

• shortens gut transit time ; shift in more diarrhoeagenic secondary Bile Acids
Deoxycholic Acid

• Diarrhea occurs in 5-10 % patients

Treatment: bile acid sequestering agents---- Cholestyramine or Colestipol

Hyperplastic Cholecystoses
Group of disorders of the gallbladder characterized by excessive proliferation of normal tissue
components

Adenomyomatosis
 benign proliferation of gallbladder surface epithelium with gland like formations, extramural
sinuses, transverse strictures ,fundal nodules formation
Hyperplastic Cholecystoses

Cholesterolosis
 abnormal deposition of lipid, especially cholesterol esters within macrophages in the lamina
propria of the gallbladder wall

• Localised form : solitary or multiple “ cholesterol polyps” studding the gallbladder wall
• Diffuse form : brick red gallbladder mucosa and speckled with bright yellow flecks of lipid ; also
called strawberry gallbladder

TREATMENT : Cholecystectomy
Gallbladder polyps

• 1-4% in adults with male predominance

Types: cholesterol polyps , adenomyomas polyps, inflammatory polyps, adenomas (rare)
• No significant changes occur in asymptomatic patients with polyps < 6mm, few
changes in polyps of size 7-9 mm

Cholecystectomy is recommended in symptomatic patients as well as in

asymptomatic patients > 50 years whose polyps > 10 mm or associated with gallstones or
polyp growth on serial ultrasonography
 Diseases of the bile ducts
Congenital anomalies
1. Biliary Atresia and hypoplasia

• most common biliary anomalies of infancy

• causes severe obstructive jaundice during the first month of life
• present with pale stools
• diagnosis confirmation : surgical exploration and operative cholangiography
• 10 % of cases treatable with Roux-en- Y choledochojejunostomy
•

Most patients eventually develop chronic cholangitis , extensive hepatic fibrosis, portal
hypertension
Choledochal Cysts
Cystic dilatation may involve the free portion of the CBD
• may present as diverticulum formation in the intraduodenal segment
• gradual process: ~ 50 % of patients present with onset of symptoms after age 10 years
Diagnosis : ultrasound, abdominal CT, MRC , cholangiography
✓ Classic triad: 1/3 of patients
• abdominal pain • jaundice • abdominal mass

Surgical treatment : excision of the cyst and biliary- enteric anastomosis

Patients are at increased risk for development of cholangiocarcinoma
Congenital biliary Ectasia

Dilatation of intrahepatic bile ducts

1. Involving major intrahepatic radicles – Caroli’s disease

• include recurrent cholangitis • abscess formation in and around the affected ducts • brown pigment
gallstones formation within portions of ectatic intrahepatic biliary radicles.
2. Interlobular and intralobular ducts – congenital hepatic fibrosis
3. Both
Diagnosis: Ultrasound, MRC and CT
Treatment : antibiotic therapy
: to limit the frequency and severity of recurrent bouts of cholangitis
Progression to secondary biliary cirrhosis is common
 Choledocholithiasis

• passage of gallstones into the CBD in 10- 15% of patients with cholelithiasis
• 25 % of elderly patients may have calculi in the common duct at the time of
cholecystectomy
• Majority are cholesterol stones migrated from the gallbladder
• Primary calculi arising de novo in the ducts are usually brown pigment stone
• most often present with biliary colic or a complication
Complications of Choledocholithiasis
Cholangitis
• may be acute or chronic; symptoms results from inflammation
• caused by at least partial obstruction to the flow of bile
• presence of bacteria in bile culture in ~ 75% of patients with acute cholangitis in the symptomatic
course
• Characteristic presentation : Charcot’s Triad
• biliary pain • jaundice • spiking fevers with chills

Leukocytosis is typical; frequently positive blood cultures

1. Non suppurative cholangitis

2. • most common • respond relatively rapidly to supportive measures and to antibiotics
2. Suppurative acute cholangitis
• presence of pus under pressure in completely obstructed ductal system
• present with symptoms of severe toxicity
• mental confusion • bacteremia • septic shock
• Response to antibiotics alone is relatively poor

Prompt endoscopic or surgical relief of the obstruction and drainage of infected bile
should be carried outotherwise mortality rate approaches 100%
 ERCP with endoscopic sphincterotomy
Safe, preferred initial procedure for establishing a definitive diagnosis and providing
effective therapy
Obstructive jaundice
• caused by gradual obstruction of the CBD over a period of weeks or months
• pruritus and painless jaundice
• may be present without associated symptoms of biliary colic or cholangitis

Courvoisier’ s law
“ presence of a palpably enlarged gallbladder suggest that the biliary obstruction is secondary to an underlying
malignancy rather than calculous disease”

• Malignancy of the head of the pancreas, bile ducts, or ampulla of Vater

• Hepatic bile flow is suppressed, and reabsorption and regurgitation of conjugated bilirubin into the bloodstream lead to
jaundice
• accompanied by dark urine (bilirubinuria) and light-colored (acholic) stools.
Obstructive jaundice

CBD stones suspected in any patients with Cholecystitis and bilirubin level > 5mg / dL
• Maximum bilirubin level is seldom > 15 mg/ dL with choledocholithiasis
• unless concomitant hepatic or renal disease or another factors
• Serum bilirubin > 20 mg /dL suggest possibility of neoplastic obstruction
• Serum alkaline phosphatase level almost always elevated
• precedes clinical jaundice • may be the only abnormality in routine liver function tests
• may be 2 to 10 fold elevation of serum aminotransferases
• Following relief of obstructing process
• serum aminotransferases return rapidly to normal
• serum bilirubin level may take 1 to 2 weeks to return to normal.
 Pancreatitis

• Non alcoholic pancreatitis is most commonly associated with biliary tract disease
• Complications of acute cholecystitis in 15% and choledocholithiasis in > 30 % occur in relation
to pancreatic inflammation
• Coexisting pancreatitis should be suspected in patients with symptoms of Cholecystitis who
develop
1. back pain or pain to the left of the abdominal midline
2. Prolonged vomiting with paralytic ileus
3. Pleural effusion , especially on the left side

Surgical treatment of gallstone disease usually resolves the pancreatitis

Secondary biliary cirrhosis

• may complicate prolonged or intermittent duct obstruction with/without

recurrent cholangitis
• more common in cases of prolonged obstruction from stricture or
neoplasm
• may be progressive even after correction of obstructing process
• may lead to severe hepatic cirrhosis, portal hypertension , hepatic failure
and death
• prolonged billiary obstruction - deficiencies of the fat soluble vitamins.
Diagnosis of Choledocholithiasis

• Cholangiography
• preoperatively by endoscopic retrograde cholangiogram (ERC)
• MRCP
• intraoperatively at the time of cholecystectomy

About 15% patients undergoing cholecystectomy shows CBD stones

CBD stones should be suspected in gall stones patients with any of the risk factors
1. a history of jaundice or pancreatitis
2. Abnormal tests of liver function
3. Ultrasonographic or MRCP evidence of a dilated CBD or stones in the duct

Treatment of choice
EBS followed by spontaneous passage or stone extraction
Trauma , Stricture and Hemobilia

 Most benign strictures of the extra hepatic bile ducts result from surgical
trauma
 occur in 1 in 500 cholecystectomies
 May present with bile leak or abscess formation in the immediate
postoperative period
 May present with biliary obstruction or cholangitis as long as 2 years or more
following the trauma
 Diagnosed by percutaneous or endoscopic cholangiography
 Endoscopic brushing of the strictures helps in establishing the nature of the
lesion / more accurate than bile cytology alone


Positive exfoliative cytology – diagnosis of neoplastic stricture

important in patients with primary sclerosing cholangitis - cholangiocarcinoma

 Hemobilia
May follow

1. traumatic or operative injury to the liver or bile ducts

2. Intraductal rupture of a hepatic abcess or aneurysm of the hepatic artery
3. Biliary or hepatic tumor hemorrhage
4. Mechanical complications of Choledocholithiasis or hepatobiliary parasitism
Patients present with classic triad:
• biliary pain
• obstructive jaundice
• melena or occult blood in the stools

✓ Diagnosis can be made cholangiographic evidence of blood clots in the

biliary tree but selective angiographic verification is required
Treatment
• Minor episodes : resolve without operative intervention
• Major episodes : surgical ligation of the bleeding vessel
Extrinsic compression of the bile ducts

 partial or complete biliary obstruction

 most common cause is carcinoma of the head of the pancreas
 may occur as complications of acute/ chronic pancreatitis
 may occur in involvement of lymph nodes in the porta hepatis by lymphoma or
metastatic carcinoma
 Hepatobiliary parasitism

infestation of the biliary tract by adult helminths or their ova

• may produce a chronic, recurrent pyogenic cholangitis with/without multiple hepatic
abcesses , ductal stones or biliary obstruction

Most common organisms : trematodes or flukes

• Clonorchis sinensis • Opisthorchis viverrini/ felineus
• Fasciola hepatica • Ascaris lumbricoides • Echinococcus app
 Hepatobiliary parasitism

Diagnosis : cholangiography and presence of characteristic ova on stool examination

Treatment of choice - laparotomy under antibiotic coverage with common duct exploration
and a biliary drainage procedure
Sclerosing cholangitis

Primary or idiopathic sclerosing cholangitis

progressive, inflammatory, sclerosing and obliterative process affecting the

extrahepatic and /or the intrahepatic bile ducts
• occurs up to 90% in association with inflammatory bowel disease , especially
ulcerative colitis
Primary or Idiopathic sclerosing cholangitis

 present with sign and symptoms of chronic/ intermittent biliary obstruction

• RUQ abdominal pain • jaundice
• pruritus • acute cholangitis
Late in the course
• complete biliary obstruction • secondary biliary cirrhosis
• hepatic failure • portal hypertension with bleeding varices
Diagnosis : cholangiography ( MRCP and ERCP)
Multifocal, diffusely distributed strictures with intervening segments of normal or
dilated ducts , producing a beaded appearance
Primary Sclerosing cholangitis
Autoimmune Hepatitis Overlap Syndrome
• characterized by clinical, biochemical and histological features of AIH and
cholangiographic features of PSC.

Small Duct PSC

• defined by the presence of chronic cholestasis and hepatic histology
consistent with PSC but with normal findings on Cholangiography
• found in ~ 5% of patients with PSC
• But may progress to classic PSC and / or End stage liver disease requiring
liver transplantation
Primary Sclerosing cholangitis
 In patients with AIDS, cholangiopancreatography may demon strate a broad range of biliary
tract changes as well as pancreatic duct obstruction and occasionally pancreatitis
 Further, biliary tract lesions in AIDS include infection and cholangiopancreatographic
changes similar to those of PSC

Cholangiographic changes
1. Diffuse involvement of intra hepatic bile ducts alone
2. Involvement of both intra and extra hepatic bile ducts
3. Ampullary stenosis
4. Stricture of the intrapancreatic portion of the CBD
5. Pancreatic duct involvement
 Associated infectious organisms include

Cryptosporidium,Mycobacterium avium intracellulare, Cytomegalovirus,

Microsporidia, Isospora

ERCP sphincterotomy can provide significant pain reduction in patientswith AIDS-

associated papillary stenosis
Sclerosing cholangitis Treatment

• Cholestyramine therapy for pruritus

• Antibiotics for cholangitis
• Vitamin D and Calcium supplement to prevent loss of bone mass
• Balloon dilatation or stenting for high grade biliary obstruction
• Surgical intervention is rarely indicated
 Sclerosing cholangitis Treatment

Prognosis is unfavorable with median survival of 9 – 12 years following the diagnosis,

regardless of therapy

Risk scoring for prediction of survival : based on 4 variables

• age • serum bilirubin level • histologic stage • splenomegaly

PSC is one of the most common indications for liver transplantation

FIN