benign biliary diseases

1. BENIGN BILIARY DISEASES
PRESENTED BY: DR. ANJALY MOHAN
MODERATED BY: DR. PUNEET K AGARWAL

2. CONTENTS
• GALL STONES (CHOLELITHIASIS)
• EMPYEMA OF GALL BLADDER
• ACALCULOUS CHOLELITHIASIS
• CHOLECYTOSES
• CHOLEDOCHOLITHIASIS
• BILE DUCT STRICTURES
• BILIARY DYSKINESIA

3. CONTENTS
• SPHINCTER OF ODDI DYSFUNCTION
• PRIMARY SCLEROSING CHOLANGITIS
• CHOLEDOCHAL CYSTS
• PARASITIC INFESTATIONS OF BILIARY TRACT

4. CHOLELITHIASIS

5. CHOLELITHIASIS
• Most common disease state of GB and biliary tract
• Possible mechanisms:
• Increase in solute concentration
• Stasis in the GB
• Types of gall stones:
• Cholesterol stones
• Pigment stones: black (hemolysis) and brown (infection)

7. • Factors that predispose to gallstones formation:
• Supersaturation of secreted bile
• Concentration of bile in GB
• Crystal nucleation
• Gallbladder dysmotility (stasis)
Natural history:
• Most GB stones are asymptomatic
• Biliary colic : temporary obstruction of cystic duct, occurs after meal
as release of CCK causes GB contraction

8. • Prophylactic cholecystectomy is not warranted in patients with
asymptomatic cholelithiasis.
Indications of prophylactic cholecystectomy:
• Patients with hemolytic anemia (sickle cell disease)
• Calcified (porcelain) GB
• Large (>2.5cm) stones
• Common bilio-pancreatic duct channel
• Patients undergoing bariatric surgery
• Immunocompromised patient before transplant

9. Medical management of gall stones:
• Generally unsuccessful and rarely used
1. Oral bile salt therapy
2. Contact dissolution
• Cannulation of GB and infusion of organic solvents – 50% recurrence
• Extracorporeal shock wave lithotripsy- (single stone upto 2cm size)

10. CHRONIC CHOLECYSTITIS

11. • Recurrent attacks of biliary colic – inflammation and scarring of neck
of GB and cystic duct – fibrosis
• Repeated self limited episodes of inflammation
Clinical presentation:
• Pain after ingestion of a fatty meal
• Located in epigastrium or RUQ
• Radiate around the scapula
• Lasts a few hours
• Bloating, nausea and vomiting

12. Diagnosis:
• Clinical history + clinical manifestations
• Transabdominal USG: may show
• Presence of cholelithiasis
• CBD dilatation
• GB polyps
• Porcelain GB
• Evidence of hepatic parenchymal process
• Cholesterolosis
• GB sludge

13. • Treatment:
• Elective cholecystectomy in severe/recurrent symptoms
• Conservative management in mild cases

14. ACUTE CALCULOUS
CHOLECYSTITIS

15. Mechanism:
• Stone causing blockage of cystic duct – non resolving type leading to
inflammation – edema and subserosal haemorrhage – ischemia –
necrosis – gangrene
• Infection of the stagnant pool of bile – secondary phenomenon
• When complicated by gas forming organisms: acute emphysematous
cholecystitis

16. Presentation:
• Fever
• RUQ pain
• Tenderness to palpation & Guarding in RUQ
• Positive Murphy’s sign – arrest of inspiration with gentle pressure
under right costal margin
• Mild elevation of alkaline phosphatases, bilirubin and transaminases
level with leucocytosis support the diagnosis

17. • Diagnosis:
• Transabdominal USG: may show
• Gallstones
• Pericholecystic fluid collection
• Gallbladder wall thickening
• Sonographic Murphy’s sign
• HIDA Scan: filling of gallbladder rules out acute cholecystitis
• CT Scan: pericholecystic fluid collection, GB wall thickening &
emphysematous changes. Though CT is less sensitive than USG

18. Treatment:
• Most cases are infected with superinfection of the inflamed GB
• Patients are started with NBM
• Parenteral fluids and antibiotics (broad spectrum)
• Parenteral narcotics to control pain
• Cholecystectomy - treatment of choice for acute cholecystitis
• Early vs delayed cholecystectomy – still debatable
• In patients unfit for surgery, cholecystostomy should be considered

19. CHOLEDOCHOLITHIASIS

20. • CBD Stones are classified according to point of origin:
• Primary: arises de novo in the bile duct, brown stones (bile pigments
and cholesterol), more common in Asians and due to infection
• Secondary: pass from GB into bile duct
• Retained stones: if they are found within 2 year of cholecystectomy

21. Clinical presentation:
• Clinically silent and usually identified on cholangiography, if
performed routinely during cholecystectomy
• When not silent, symptoms may range from biliary colic to
obstructive jaundice
• Jaundice with choledocholithiasis is usually painful – acute setting –
dilatation of CBD – activation of pain fibres
• Charcot’s triad: fever, RUQ pain, jaundice – suggests acute cholangitis
– may progress to septic shock
• Reynold’s pentad: triad + shock with altered mental status

22. Diagnosis:
• Altered LFT: neither sensitive nor specific
• Leukocytosis: in cases of superinfection
• USG abdomen: bile duct stones or dilatation
• Patients with jaundice, gallstones and biliary pain, a CBD diameter
>8mm : highly suggestive of CBD stones
• ERCP: highly sensitive and specific , diagnostic + therapeutic
• MRCP: sensitivity (>90%), specificity (100%), non invasive, accurate
imaging of the biliary tree

23. • Treatment:
• ERCP: endoscopic sphincterotomy + stone extraction
• Laparoscopic CBD exploration: cholecystectomy + intraoperative
cholangiogram + CBD exploration (single setting)
• Transcystic approach: via the cystic duct using a cholangioscope
• Open CBD exploration: transduodenal sphincteroplasty,
choledochoenterostomy (choledochoduodenostomy/-jejunostomy)

26. EMPYEMA OF GALL BLADDER
• Can be a sequelae of acute cholecystitis or the result of an infected
mucocele
• GB is distended with pus
• Optimal treatment is DRAINAGE (cholecystostomy followed by
cholecystectomy)

27. ACALCULOUS CHOLECYSTITIS
• Inflammation of gall bladder can occur even in the absence of gall
stones
• In non specific inflammation of GB or in one of the conditions of
cholecytoses
• Particularly seen in critically ill patients and in patients recovering
from shock, burns, trauma
• Often missed diagnosis
• High mortality

28. CHOLECYTOSES

29. CHOLESTEROSIS
• aka “strawberry gallbladder”
• Mucosa of the gall bladder appears
like a strawberry
• Where the yellow specks corresponds to
seeds of strawberry (submucous aggregation
of cholesterol crystals and esters)
• May be associated with cholesterol stones

30. CHOLESTEROL POLYPOSIS OF GB
• USG may show a non mobile defect with no acoustic shadow
• Differential can be an adenomatous polyp
• Interval followup is indicated
• Surgery indiacted if diagnostic dilemma

31. CHOLECYSTITIS GLANDULARIS PROLIFERANS
• Polyp of mucus membrane is fleshy and granulomatous
• All layers of GB may be thickened
• Sometimes there may be an internal septa which separates
hyperplastic from normal
• Intraparietal mixed calculi may be present
• Can be complicatd by intramural/ extramural abscess and fistula
formation
• Symptomatic patients treated with cholecystectomy

33. DIVERTICULOSIS OF GB
• usually manifest as black pigment stones impacted in the
outpouchings of the lacunae of Luschka
• Demonstrated by cholecystography, especially when the gallbladder
contracts after a fatty meal
• small dots of contrast medium just within and outside the gallbladder
• septum may also be present, to be distinguished from the Phrygian
cap
• The treatment is cholecystectomy

35. TYPHOID INFECTION OF GB
• Salmonella typhi or typhimurium
• Acute cholecystitis can occur
• More frequently, chronic cholecystitis occurs
• Patients become typhoid carrier secreting bacteria in the bile
• Gall stones may be present
• Salmonella can be cultured from these stones
• Treatment with Ampicillin and cholecystectomy
• In penicillin allergy, give flouroquinolones

36. BILE DUCT STRICTURES

42. • ERCP with sphincterotomy, balloon dilation, and stent placement is
generally regarded as primary treatment for benign bile duct
strictures
• Endoscopic and percutaneous therapy can provide long-term success
in more than 50% of patients
• When this is unsuccessful, surgical management with anastomosis of
the biliary tree to a Roux-en-Y jejunal limb has success rates of up to
90%

44. BILIARY DYSKINESIA

45. • may present with classic symptoms of calculous biliary disease
• but have no ultrasonographic evidence of stones or sludge
• dysfunction of the gallbladder creates pain, even in the absence of
stones
• These patients will have other diagnoses excluded by CT and upper
endoscopy and should undergo a CCK-stimulated HIDA scan, in which
the radiolabeled iminodiacetic acid will collect in the gallbladder

46. • patient is given an IV dose of CCK
• An ejection fraction less than one third at 20 minutes after CCK
administration in patients without stones is considered diagnostic of
dyskinesia,
• which should be managed with cholecystectomy
• In nonresponders, ERCP with sphincterotomy may prove useful

47. SPHINCTER OF ODDI
DYSFUCTION

48. • manifested as biliary tract pain, with normal liver function test results
and recurrent pancreatitis
• May be caused by a structurally abnormal sphincter or a histologically
normal but functionally abnormal one
• injury to the sphincter from trauma, due to pancreatitis, gallstone
passage, or congenital anomalies – inflammation – fibrosis – elevated
sphincter pressure
• diagnosis of sphincter of Oddi dysfunction should be suspected in
patients with biliary pain and a common duct diameter of more than
12 mm

49. • Manometry has also been used to make the diagnosis, with sphincter
pressure higher than 40 mm Hg predicting good response to therapy
• Therapy consists of endoscopic sphincterotomy or transduodenal
sphincteroplasty, with approximately equivalent results from the two
approaches
• In patients with objective evidence of sphincter of Oddi dysfunction,
division of the sphincter will improve or resolve the pain in 60% to
80% of patients

50. PRIMARY SCLEROSING
CHOLANGITIS

51. • idiopathic, likely autoimmune process affecting the intrahepatic and
extrahepatic biliary trees
• associated with other autoimmune diseases, such as ulcerative colitis
and Riedel thyroiditis
• the disease causes inflammation and scarring in the biliary tree
• characterized by progressive chronic cholestasis and advances at an
unpredictable rate to biliary cirrhosis and eventually death from liver
failure
• The microscopic picture is one of inflammation, fibrosis, and
cholestasis

52. • Clinical Presentation:
• fatigue, pruritus, and jaundice
• Asymptomatic elevations of alkaline phosphatase levels can also
occur and may be associated with evidence of hepatocellular injury
and hyperbilirubinemia before clinical manifestations of symptoms
• Abnormal liver function tests in a patient observed for inflammatory
bowel disease should suggest PSC
• Elevation of perinuclear antineutrophil cytoplasmic antibodies can be
seen in 80% of patients

53. • ERCP is the preferred route for cholangiography and can demonstrate
the characteristic multifocal, diffusely distributed dilations and
strictures of the intrahepatic and extrahepatic biliary trees
• The sequential stricturing, proximal dilation, and more proximal
stricturing create a pattern described as beading or chain of lakes
• Other cholangiographic findings include multiple diverticulum-like
outpouchings of the bile ducts and multiple short-segment strictures
• Liver biopsy tends to show an onion skin concentric periductal fibrosis
• With disease progression, periportal fibrosis occurs, progressing to
bridging necrosis and eventually biliary cirrhosis

56. • Treatment:
• No specific effective medical therapy exists for PSC
• Early in the disease, with mild symptoms, observation is a reasonable
approach
• Medical therapies are generally targeted to the underlying
hepatobiliary disease process;
• these include choleretic agents such as ursodeoxycholic acid,
immunosuppressive agents, and antifibrogenic agents such as
colchicine
• However, none of these agents has shown a consistent benefit

57. • Surgical options include biliary reconstructive procedures and liver
transplantation
• Biliary reconstruction is an option for patients with a dominant
stricture at the hepatic bifurcation, for which resection of this region
with long-term Silastic stenting can be performed
• Orthotopic liver transplantation appears to be the only lifesaving
option for patients with progressive hepatic dysfunction from PSC
• 5-year survival rates ranging from 75% to 85%

58. Immunoglobulin (IgG4) related cholangitis
• presents with diffuse or segmental narrowing of the intra- or
extrahepatic bile ducts
• patients often have elevated serum IgG4 levels and concomitant
autoimmune pancreatitis, IgG4- related sialadenitis or retroperitoneal
fibrosis
• Biliary biopsies show lymphoplasmacytic sclerosing cholangitis
• Treatment is with systemic steroids

59. CHOLEDOCHAL CYSTS

60. • rare, occurring in fewer than 1/100,000 patients
• more common in Asian descent
• three to eight times more common in women than in men
• Considered premalignant and thus requires surgery
• Commonly diagnosed in infancy, but many present in adulthood
• Pathogenesis relies on the presence of an anomalous
pancreaticobiliary junction (APBJ)

61. • With APBJ, the pancreatic duct and biliary tree fuse to form a
common channel before passage through the duodenal wall
• APBJ is seen in up to 90% of patients with choledochal cysts
• Pancreatic duct has higher secretory pressures than the biliary tree,
exocrine pancreatic secretions reflux up into the bile duct and can
inflame and damage the biliary tree, resulting in cystic degeneration

63. • Presentation:
• classic presentation of jaundice, right upper quadrant pain, and a
palpable mass occurs rarely
• Other symptoms include nausea, pruritus, and weight loss
• Long standing disease can induce a chronic injury to the liver with
cirrhosis
• Cholangitis, pancreatitis, hepatic fibrosis, and malignant disease have
all been reported at the time of presentation
• An unusual presentation is that of acute rupture of the cyst, with
subsequent bile peritonitis

64. • Most cystic biliary lesions are originally identified and subsequently
diagnosed by imaging
• With the current liberal use of CT, the diagnosis of a choledochal cyst
is suspected, but it is further classified by MRCP
• ERCP is more useful for defining the distal biliary tree and pancreatic
duct–bile duct junction
• Laboratory studies may identify cholestasis and jaundice
• In late stages of disease, secondary hepatic injury and evidence of
cirrhosis may be seen

65. • incidence of malignant disease in patients with biliary cysts ranges from
10% to 30%
• Malignant cyst degeneration is common and is thought to relate to chronic
mucosal irritation from the refluxed pancreatic enzymes
• Surgical management of choledochal cysts consists of resection of the
entire cyst and appropriate surgical reconstruction
• Type I cysts: complete surgical excision, cholecystectomy, and Roux-en-Y
hepaticojejunostomy
• Type II cysts should be excised entirely, and in the presence of an APBJ,
biliary-enteric diversion by Roux en- Y hepaticojejunostomy is appropriate

66. • Type III cysts are uncommon and may be approached transduodenally
• In the setting of duodenal or biliary obstruction, transduodenal
excision or sphincteroplasty can be performed
• Type IV cysts affecting only the extrahepatic bile ducts are managed
similarly to type I cysts, with excision and hepaticojejunostomy
• Those with intrahepatic extension involving only one lobe can be
treated with partial hepatectomy and reconstruction
• Surgical treatment of Caroli disease ranges from resection if the
disease is unilobar to liver transplantation when diffuse disease is
detected

67. POLYPOID LESIONS OF GALL
BLADDER

68. Benign masses of the gallbladder are common and consist of:
• Pseudotumors
• Adenomas
• Pseudotumors are further divided into cholesterol polyps and
adenomyomatosis
• Cholesterol polyps appear as pedunculated echogenic lesions of the
gallbladder, are usually smaller than 1 cm, and are frequently multiple

69. • Adenomyomatosis is seen as a sessile lesion, commonly in the fundus,
with characteristic microcysts within the lesion, and is frequently
larger than 1 cm
• Polyps with size larger than 10 mm is a risk factor for
adenocarcinoma, along with growth, presence of gallstones, and age
of the patient older than 60 years
• Management of all symptomatic polypoid lesions of the gallbladder is
laparoscopic cholecystectomy

70. • Patients with a polypoid lesion and risk factors for adenocarcinoma or
those suspected of having in situ or invasive cancer should undergo
open cholecystectomy
• because perforation during laparoscopy may spread tumor cells
throughout the peritoneal cavity
• Asymptomatic lesions smaller than 10 mm with no other risk factors
and no ultrasonographic features suggesting malignant disease can be
observed with serial ultrasonography

71. PARASITIC INFESTATION OF THE
BILIARY TRACT

72. BILIARY ASCARIASIS
• The roundworm Ascaris lumbricoides commonly infests the intestines
of inhabitants of Asia, Africa and Central America
• may enter the biliary tree through the ampulla of Vater and cause
biliary pain
• Complications include strictures, suppurative cholangitis, liver
abscesses and empyema of the gallbladder
• antispasmodics can be given to relax the sphincter of Oddi and the
worms will return to the small intestine to be dealt with by anti
helminthic drugs

73. • Operation may be necessary to remove the worms or deal with
complications
• Worms can be extracted via the ampulla of Vater by ERCP

74. CLONORCHIASIS (ASIATIC
CHOLANGIOHEPATIS)
• endemic in the Far East
• The fluke, up to 25 mm long and 5 mm wide, inhabits the bile ducts,
including the intrahepatic ducts
• Fibrous thickening of the duct walls occurs
• Many cases are asymptomatic
• Complications include biliary pain, stones, cholangitis, cirrhosis and
bile duct carcinoma

75. • Choledochotomy and T-tube drainage and, in some cases,
choledochoduodenostomy are required
• Because a process of recurrent stone formation is set up, a
choledochojejunostomy with a Roux loop fixed to the adjacent
abdominal wall is performed in some centres to allow easy
subsequent access to the duct system

76. Hydatid disease of biliary system
• A large hydatid cyst may obstruct the hepatic ducts
• Sometimes, a cyst will rupture into the biliary tree and its contents
cause obstructive jaundice or cholangitis, requiring appropriate
surgery

77. REFERENCES
• LOVE AND BAILEY, 27TH EDITION
• SABISTON TEXTBOOK OF SURGERY, 20TH EDITION
• MAINGOT’S ABDOMINAL OPERATIONS, 12TH EDITION
• INTERNET

78. THANK YOU ALL