The Biliary TractSviatoslav Balaka

(by Raymond W Pryor III M.D.)

History

History Circa 200 AD – Galen – the gallbladder

as a subsidiary organ for the liver & responsible for yellow bile

Renaissance period – Gallbladder seat of many emotions (gall)

1652 - Thomas Bartholin – gallbladder part of bile tract from liver to intestine

1654 - Thomas Glisson – formed more detailed anatomy of liver & biliary tract

More relevant history 1420 - Antonio Benevieni – 1st account of

gallstones 1687 - Stal Pert Von Der Wiel – 1st operation

on gallstones 1733 – Jean-Louis Petit -1st successful

removal of gallstones with fistula formation 1859 – J.L.W. Thudichum – Two stage

cholecystostomy July 15, 1867 – Dr John Stough Bobbs –

Single stage cholecystostomy

Still More relevant History 1630 & 1667 – Zambecarri & Teckoff – proved

the gall bladder not essential to life 1878 - Theodor Kocher – refined

cholecystostomy procedure July 15, 1882 – Dr Langenbuch – 1st open

cholecystectomy 1886 – cholecystotomy 27% mortality vs 12%

mortality for Langenbuch’s cholecystectomy – became gold standard

1940’s – Mirizzi introduced cholangiography for CBD stones

Anatomy

Anatomy

Variations in Bile Ducts

Gallbladder physiology Gallbladder wall has

no muscularis mucosa or submucosa

Predominantly columnar epithelial cells

Rokitansky-Aschoff sinuses

Ducts of Luschka

Biliary Physiology

1.Storage and concentration of hepatic bile

2.Secretion of water and electrolytes

3.Empting bile into the common bile duct

controled by secretin, cholecystokinin (CCK) and gastrin.

Storage and concentration Normal capacity of 40-50 mL Liver secretes >600 mL of bile

daily Greatest absorptive capacity Concentrates bile 5-10 fold NaCl transport by epithelium is

driving force and water passively absorbed

Composition of BileCharacteristic (mEa/L) Hepatic GallbladderNa 160 270K 5 10Cl 62-112 1-10HCO3 45 10Ca 4 25Water 95-98% 85-90%Bilirubin 1.5 15Protein (mg/dL) 250 700Bile Acids 3-50 290-340Phospholipids 8 40Cholesterol (mg/dL) 60-70 350-930Total Solids -- 125pH 7.0 - 7.8 6.0 - 7.2

Biliary Motility Contraction of ampullary sphincter

(Sphincter of Oddi) After meal, sphincter of Oddi relaxes &

CCK released — contraction of gallbladder

When stimulated, 50-70% of contents ejected over 30-40 minutes

Refills over next 60-90 minutes

Gallstones

Cholelithiasis 10-20% of Europeans and

Americans carry gallbladder stones

Majority are asymptomatic Symptoms and severe

complications - 25% 1-2% of asymptomatic

individuals develop symptoms per year

Each year, an estimated 700,000 cholecystectomies (US)

$6.5 billion (US)

Classification of gallstones

Cholesterol Gallstones

Pigment gallstones

Is Everyone Still Awake?

Diagnosis of cholelithiasis Abdominal plain film Ultrasound

› 95-98% sensitive, 98% specific Radionuclide scan – hepatobiliary iminodiacetic

acid (HIDA)› Acute cholecystitis: 94% sensitive, 65-85% specific› Chronic cholecystitis: 65% sensitive, 6-10% specific

CT scan› 50-70% sensitive› More expensive

MRCP› Cost prohibitive

Ultrasound

More images

CT Scan

MRCP

MRCP with morphine

Chronic Calculous Cholecystitis Recurrent cystic duct obstruction & inflammation Presents with biliary colic Association with meals present in only 50% Symptoms

› Pain duration 1-5 hours (rare >24 hrs or <1 hr)› Nausea & vomiting present 60-70% of time› Bloating & belching in 50%› Fever & jaundice rare

Exam may be normal unless during attack Laboratory values usually normal Differentials include: GERD, PUD, IBS, pancreatitis Treatment is elective laparoscopic cholecystectomy

Chronic Calculous Cholecystitis

Xanthogranulomatous cholecystitis

Variant of chronic cholecystitis lipid-laden inflammatory process Marked wall thickening with intramural

nodules visible on CT and US Cannot distinguish radiographically from

gallbladder carcinoma

Porcelain gallbladder Rare disorder where chronic cholecystitis

causes mural calcification of gallbladder wall Cholecystectomy is warranted as there is a

30-65% risk of underlying gallbladder carcinoma

Gallstone Ileus Large gallstone the obstructs small bowel (distal ileum

at ileocecal valve) Fistula between gallbladder and duodenum (can be

colon or stomach) Most commonly in elderly (>70) females History of gallstone-related symptoms present in only

50% Only account for <1% of SBO cases <0.1% of those with gallstones will develop Up to 25% of SBO in elderly patients who have not had

previous abdominal surgery or have a hernia Treat with Ex-lap and enterotomy with removal of stone.

Take back for takedown of biliary-enteric fistula & cholecystectomy when more stable

Gallstone Ileus

Intrahepatic Stones Most common in East

Asian population Most associated with

biliary strictures, primary sclerosing cholangitis, choledochal cysts & biliary tract tumors

Spontaneous in <10% of cases

Treatment depends on location & underlying condition(s)

Choledocholithiasis 7-15% of patients

undergoing cholecystectomy have CBD stones

Findings of jaundice, lightening of stool & dark urine

Fevers & elevated WBC indicate cholangitis

Serum Bilirubin has PPV of 28-50%

Biliary obstruction may be partial or transient, so labs may be normal in up to 30%

Choledocholithiasis (cont’d) May have overlying pancreatitis (up to 45% of all cases

of pancreatitis) Dilated CBD in 58% of patients Ultrasound only 60-70% sensitive for CBD stones MRCP 95% sensitive & 89% specific ERCP Gold Standard

› Can both diagnose and treat 90% of time› Pancreatitis occurs in up to 5% of Patients

If unable to remove stone with ERCP (or none available) need to do laparoscopic or open CBD exploration

Stone found in CBD within 2 years of cholecystectomy is termed retained, >2 years is recurrent

Mirizzi Syndrome Rare cause of biliary

duct obstruction Large stone

contained within gallbladder compresses the CBD

Local spread of inflammation from gallbladder to CBD may also result in duct narrowing

Classification

Type I - no fistula present: Type IA - presence of

the cystic duct. Type IB - jbliteration of

the cystic duct. Types II-IV - fistula

present: Type II - defect smaller

than 33% of the CBD diameter.

Type III - Defect 33-66% of the CBD diameter. Type IV - Defect larger than

66% of the CBD diameter

Wake up!

Cholangitis Acute bacterial infection of biliary tract Most common cause of biliary obstruction as often associated with

choledocholithiasis Occurs in 4-7% of ERCP and PTC (Percutaneous transhepatic

cholangiography) Other causes of obstruction associated with cholangitis:

› Strictures, neoplasms (rare), chronic pancreatitis, congenital cysts, duodenal diverticula

Presentation of fever, RUQ pain, jaundice › Charcot’s triad

May also have Hypotension & mental status change› Reynold’s pentad

Lab findings: Leukocytosis, elevated alk phos, AST, ALT, Bilirubin (direct)

Blood cultures positive 40-50% CT scan or Ultrasound can help make diagnosis

Treatment of Cholangitis Immediate IV antibiotics and fluid

resuscitation Biliary decompression necessary

› Endoscopic or percutaneous› May need open CBD exploration & T-tube

placement (higher mortality) Overall mortality 2% (5% with toxic

cholangitis)

Primary Sclerosing Cholangitis

Choleststic liver disease characterized by fibrotic strictures in the intrahepatic and extrahepatic biliary tree in ABSENCE of any known cause.

Associated with HLA B8/DR3, IDDM, Graves’ Disease, Sjögren’s syndrome, & Myasthenia gravis

Clinical presentation highly variable› Jaundice, pruritus, fatigue, abnormal LFT’s

Mean age 30-50 years Male : female 3:1 Diagnosis by ERCP Median survival after diagnosis 10-12 years Primary treatment is liver transplantation

Sclerosing cholangitis Sclerosing

cholangitis caused by stones, cholangitis or operative trauma termed Secondary sclerosing cholangitis

Choledochal cysts Rare congenital

dilation of biliary tract

1 : 150,000 incidence in Western countries

Much more common in Japan

Female : male 8:1 Most often diagnosed

in infancy Treatment is

resection

Biliary Strictures & Bile duct injury 80-90% caused by iatrogenic

injuries Laparoscopic cholecystectomy

most common Present days to weeks after

surgery May be years after surgery as

scar tissue obstructs duct Treatment is decompression,

drainage, possible surgical resection.

Stenting being used with increasing frequency

With ligation of CBD & strictures, need Roux-en-Y hepaticojejunostomy

Gallbladder Cancer 5th most common GI tract malignancy 2-3 times more common in females 75% over age 65 5,000 new cases in US annualy Found incidentally in 1% to 3% of

cholecystectomy specimens Majority of the time, diagnosed in late stages

with distant mets Cholelithiasis present in 75-90% of cases

› Only 0.4% of those with gallstones develop cancer

Gallbladder Cancer Over 90% are adenocarcinoma

› 60% scirrhous, 25% papillary, 15% mucoid Squamous cell, oat cell, undifferentiated,

adenosquamous & carcinoid tumors less common Only 10% are correctly diagnosed preoperatively 1-3 out of every 100 cholecystectomy

specimens will show carcinoma at pathology

At diagnosis:› 25% contained to gallbladder wall› 35% metastases to regional lymph nodes› 40% have metastasized to distant sites

Average survival is 6 months after diagnosis

Lymphatic drainage & spread Initial drainage to

cystic duct node Descents along CBD

nodes Nodes at posterior

head of pancreas Interaortocaval

nodes Can also spread by

direct invasion into liver

Gallbladder Cancer Most commonly presents with RUQ pain Weight loss, jaundice, palpable mass very late findings Many report change in quality or frequency of biliary colic episodes US sensitivity 70-99% CT approx 75% sensitive MRI 90-99% sensitive

Management & Prognosis Tumor confined to mucosa or

submucosa (T1a) or to muscularis (T1b) have overall 5-year survival of 100% & 85%

Spillage of bile during cholecystectomy can seed abdomen

Invasion beyond muscularis (T2 & T3) need extended cholecystectomy with lymph node dissection

Stage III has ~15% 5-year survival Stage IV has median survival of 1-

3 months from diagnosis Majority of cases, therapy is

palliative Chemo & radiation hot been

shown to increase survival

Survival following radical resection of T2 gallbladder

cancer vs simple cholecystectomy

Cholangiocarcinoma Uncommon tumor anywhere along intrahepatic or

extrahepatic biliary tree 60-80% occur at bifurcation Most present with obstructive jaundice, hepatomegaly,

palpable gallbladder (Courvoisier’s sign) or cirrhosis (advanced disease)

2,500-3,000 new cases in US annualy Mean age in 50’s, men & women equal Increased risk with choledochal cysts, intrahepatic

stones, Liver flukes, dietary nitrosamines & exposure to dioxin

Following biliary-enteric anastomosis, 5% will develop Tend to spread by direct extension

Staging & classification Intrahepatic tumors

(Klatskin tumors)treated like hepatocellular carcinoma (hepatectomy)

Perihilar treated with resection with local hepatic resection

Distal treated like periampullary tumors with pancreatoduodenectomy (Whipple)

TNM staging

Diagnosis Intrahepatic easily

visualized on CT scan Perihilar & distal

tumors difficult to visualize on US and CT

ERCP & MRCP have near equal sensitivity (85-95%)

Most patients have serum Bilirubin >10, elevated Alk Phos & CA 19-9

Treatment

Preoperative placement of stents in hepatic & hilar tumor

Resected Left lobe & hilum with reconstruction

Prognosis Long-term survival highly

dependant on stage & treatment

For resectable intrahepatic tumors, overall 5-year 30-40%

Resectable peri-hilar tumors 10-20%

Resectable distal tumors 28-45%

Median survival for all unresectable tumors is 6-7 months

Thanks God it’s the weekend!