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Biliary biliary or gallstone colic

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Biliary biliary or gallstone colic Powered By Docstoc
					                                                    Biliary

Embryology .................................................................................................... 2
Anatomy ........................................................................................................ 2
Physiology...................................................................................................... 6
Extrahepatic biliary atresia ................................................................................... 8
Choledochal cysts ........................................................................................... 10
Bile duct strictures........................................................................................... 12
Primary sclerosing cholangitis (PSC)..................................................................... 14
Primary biliary cirrhosis (PBC)............................................................................ 16
Acute acalculous cholecystitis ............................................................................. 18
Chronic acalculous cholecystitis........................................................................... 18
Cholesterolosis............................................................................................... 19
Gallstones..................................................................................................... 19
IOC ............................................................................................................ 27
Complications of Gallstones ............................................................................... 28
Choledocholithiasis.......................................................................................... 35
Bile duct injury............................................................................................... 41
Mx malignant jaundice...................................................................................... 43
Benign Bile duct tumours .................................................................................. 46
Cholangiocarcinoma ........................................................................................ 47
GB cancer..................................................................................................... 55




Biliary                                                                                                          1
                                     Embryology
•   Epithelial origin
•   Bud from foregut
    — Ventral pancreas
    — GB
    — Bile ducts
    — Liver

                                       Anatomy

Gallbladder
• Lies in GB fossa
• 10cm long
• 30-300mls
• Fundus @ transpyloric plane     tip 9th CC
• 4 parts
   — fundus
   — body
   — infundibulum
   — neck
• Hartmanns pouch
   — Develops as pathological feature
   — in infundibulum & neck
   — associated with gallstones

Structure
• 3 layers
   — serosa
   — fibromuscular
           longitudinal
           spiral
   — mucosa with cuboidal epithelium

Blood supply
Cystic artery
• 65%         Arises from RHA posterior to CHD → behind RHD → GB
• 20%         Arises from RHA anterior to CHD
• 10%         Arises proximally from RHA → anterior to RHD
              NB RHA still goes posterior
• 5%          Arises apex of RHA loop / hump
v Can arise from coeliac, aberrant CHA, SMA & can be multiple (up to 20%)
• Usually anterior & posterior branches
• 20% have accessory artery
• Fundus has poorest supply




Biliary                                                                     2
Venous
• Venous drainage variable
  — → R portal vein

Nerves
• Pain
   — visceral sympathetics
   — coeliacs
   — T7-T9
   — With artery
• Motor
   — Vagal
   — via coelaic ganglion
   — Hepatic branch anterior vagus

Anomalies
v ‘Normal’ biliary anatomy in 50%
Gall bladder
• Absence (agenesis)
   — 1/40000
• Multiple
   — Double most common
• Ectopic
• Bilobed
• Intrahepatic
   — Most common anomaly
• Long mesentery
   — ‘floating’
   — Predisposes to torsion
• Phrygian cap
   — Folded fundus
• Diverticulum of GB
• Lieing to L of falciform

Cystic duct
• 2cm long 2mm wide
• Mucosa forms semicircular folds
  — Spiral valve of Heister
• Passes down, posterior to L→ hepaticodoudenal ligament
  — Joins CBD ≈ 3cm below confluence 80%              (A)




Biliary                                                     3
Variations
• Length
   — Long & short
• Course
   — Parallel for long distance             25%              (B)
   — Inseperable from CBD                                    (C)
   — Behind CBD                             10%              (E)
   — Anterior → L side CBD                  10%              (H)
• Origin/join
   — L side CBD                             20%              (E,H)
   — Behind duodenum                        15-20%           (F)
   — @ confluence/R hepatic                 2%               (D)
• Number
   — Absent                                                  (G)
   — Double
   — Accessory
          90% in Calot’s ∆



Calot’s triangle

Boundaries
• CHD
• Cystic duct
• Inferior surface of liver
v NB original description cystic artery as superior border

Contents
• Cystic duct
• Cystic artery
• Aberrant RHA 16% in groove between CHD & portal v
  — Can be seen in 90% of people with abnormality
• Aberrant R posterior or R anterior sectoral ducts

Ducts

Intrahepatic

Extrahepatic
R & L hepatic
• R 1cm
• L 2cm
• Confluence anterior to portal vein near artery
• Accesory R hepatic        ≈ 5%


Biliary                                                              4
•   Duct of Lushke          1%
    — Accesory duct
            liver ↔ body GB
            CHD ↔ GB
            RHD ↔GB
CHD
• 4cm long, 4mm wide
Cystic
• See above
CBD
• 8cm long, 8mm wide
• Supraduodenal
  — Free edge of lesser omentum
• Retroduodenal
  — Behind D1
  — slopes down to R
          IVC posterior
          PV & gastroduodenal artery to L
• Paraduodenal
  — slopes down to R
  — Behind or through pancreas
  — Anterior to R renal vein prior to entering ampulla
  — Distal 2cm within duodenal wall
          Spincter of Oddi
• Ampulla
  — Opens in posteromedial aspect D2
          10cm from pylorus
  — Termination with pancreatic duct
          70%        Y
          20%        V
          10%        U
• Structure
  — Similar to gb
  — Fibrous tissue in outer fibromuscular layer
  — Mucosa is single layer columnar epithelium with microvilli
• Blood supply
  — Arteries from
          RH
          Gastroduodenal
          Superior posterior pancreaticoduodenal
          Pancreaticoduodenal
  — Run @
          3 o’clock
          9 o’clock




Biliary                                                          5
                                        Physiology

Function
• Primary role is the storage and concentration of bile
  — 2-10x conc
  — reabsorbs Na+ and H2 O
• Can reduce the volume of bile by 80-90%
• Na+ is actively transported (Na+ - K+ ATPase) and H2 O follows


Motility
• Basal secretion
• Stimulation
  — CCK
           fat , aminoacids
  — Motilin, secretin, prostaglandins & histamine
• Inhibition
  — Somatostain
  — VIP
• Maximal emptying occurs 90-120min after a meal
• Truncal vagotomy
  — loss of vagal supply from hepatic branch of the anterior vagus
  — reduces gallbladder emptying
  — increased incidence of gallstones
• Gallbladder filling
  — ? passive
  — more complex with mediation by various peptides (VIP, PP, PYY)
• Bile duct has minimal motor activity and acts as a conduit
• Sphincter of Odi has phasic contractions which allow intermttent passage of bile at rest

Bile formation
• Isotonic
• Neutral pH
   — or alkaline
• Predominate organic solutes
   — bile salts
   — cholesterol
   — phosopholipids
• Active transport of bile acids into the cannaliculi by the hepatocytes with water following
• Active transport of Na+
• Secretin stimulates bile secretion

Composition of Bile
• 3 major constituents
  — bile salts
  — cholesterol
  — phospholipid


Biliary                                                                                         6
v 80% dry weight of bile

Bile salts
1˚ bile acids
• chenodeoxycholic acid
• cholic acid
    — synthesized in the liver from cholesterol and conjugated there
    — negative feedback from bile salts in the portal circulation
2˚ bile acids
• Deoxycholic acid
• Linocholic acid
Enterohepatic Circulation
• congugated chenodeoxycholic acid and cholic acid
   — secreted into the biliary tract
   — stored and concentrated in the GB
   — emptied into the duodenum in response to meal-stimulated CCK release
• 80%        1o bile salts absorbed in terminal ileum → liver for reuse
• ≈20%       1˚ bile salts decongugated by bacteria in terminal ileum or colon
             absorbed→ liver for reuse
• Small percentage of 1o bile salts converted by colonic bacteria to the 2o bile salts
   — deoxycholic
   — lithocolic acid
   — some of these 2o bile salts are also reabsorbed
• Ultimately some 95% of secreted bile salts become available for reuse

Lecithin
• Phosolipid
   — accounting for 90% of such in the bile
• Synthesized by the liver
• Inhibited by bile acids

Cholesterol
• Most of the cholesterol in bile is synthesized by the liver
• Dietry intake contributes minimally to the bile cholesterol pool
• Rate limiting step→HMG-CoA reductase
• -ve feedback by total body cholesterol and bile salts

Solubility of Cholesterol
• Insoluble in aqueous media
• Depends on
   — micelle formation by bile salts with incorporation of cholesterol & lecithin
   — vesicles consisting of cholesterol and phospholipid
• Crystal formation is thought to be due to a disruption in the equilibrium between these
   components



Biliary                                                                                     7
Solubility of cholesterol in bile
v   Admirand and Small 1968
•   Phase diagram of the solubility of cholesterol in bile
•   Shows the physical state of all the possible combinations of bile salts in aqueous solutions.
•   The area below the line in the bottom left represents the maximum amount of cholesterol
    solubilised by any mixture of lecithin and bile salt.
•   Combinations above and to the right of this area means that cholesterol is present in bile in
    crystal or liquid crystal form


                               Extrahepatic biliary atresia

Epidemiology
• Racial difference
• Caucasians
   — 1:10000 live births
   — M:F 1:1
• Asians
   — Incidence ↑er
   — M:F 1:2

Aetiology
• Unknown
• Not found in stilborn or prem
   — ? perinatal injury
• Patent intrahepatic ducts suggest against developmental absence
• 1st stool & meconium bile stained
   — ? post natal sclerosis

Clinical
• Jaundice 2-3/52 post partum
• (Cholestatic) jaundice
• Liver failure
• Portalhypertension
• 2-6/12 failure to thrive

Ix
• LFT
• USS
   — Excludes choledochal cysts
• Liver Bx excludes intrahaptic cause in 90%
• Exploratory lap if ∆ not made in 60/7
   — Liver bx
   — IOC
• ? role MRCP



Biliary                                                                                             8
∆∆
• Neonatal hepatitis
• Bile duct paucity
• Choledochal cyst
• Intrinsic duct obstruction

Rx
Kasai procedure
• Portoenterostomy
• @ 6-10/52
• If > 4/12 bile drainage does not occur
   → progressive cirrhosis
• Succesful           35-50% 5yrs
• Unsuccesful         ≈ 12/12 survival
Tx
• Rx of choice for failed drainage or irreversible cirrhhosis
• 60-80% 5yrs




Biliary                                                         9
                                 Choledochal cysts

Epidemiology
• 1:13000 hospital admissions
• 1:50000 live births
• M:F        1:9
• 25%        <1yr
• 60%        <10yrs

Aetiology
• Unknown
• ↑ common in Asia

Pathology
• Size
   — Vary
   — 2cm – giant cysts
• Micro
   — Wall of fibrous tissue
         Up to 1cm thick
   — Lined with cuboidal biliary epi
   — Often ulcerated in adults

Classification
Todani 1977
• I       Fusiform dilatation of CBD                    82%, 65% involve cystic
• II      Supraduodenal diverticulum                    3%
• III     Intraduodenal diverticulum / choledochocele   5%
• IV      Multiple small fusiform, with strictures      9%
          A Intra & extrahepatic
          B Extrahepatic only
• V       Intrahepatic                                  1%
          A Type I
          B Type II
          C Type IV         (Caroli’s)



Caroli’s disease
• Aurtosomal recessive
• Recurrent cholangitis
• Risk choalngiocarcinoma 8%




Biliary                                                                           10
Clinical
v    Classical triad in 15%
•    Cholestatic jaundice
•    Abdominal mass
•    Abdominal pain

Complications
• Cholangitis             15%
  — Recurrent
• Pancreatitis
• Hepatic abscess
• Calculous disease
• Biliary cirrhosis       30%
• Rupture
  — Biliary peritonitis
• Portal veain thrombosis
• Cholangiocarcinoma
  — ↑ with age
           <10yrs         1%
           10-20yrs       10%
           >20yrs         20%
  — No ↑ incidence if cyst excised as child

Ix
•    USS
•    CT
•    ERCP
•    MRCP

Rx
• Type I, II, IVb
   — Extrahepatic duct excision
   — Hepaticojejunostomy
   — R-en-Y
• Type III
   — ERCP & sphincteroromy with close observation
   — Whipples
• Type IVa, V
   — Hepatic resection
   — Tx




Biliary                                             11
                                     Bile duct strictures

Aetiology

Congenital
• Biliary atresia
• Cholecochal cysts (IV)

Aquired

Inflammatory
• Uncommon
• 2˚ to:
    — Chronic pancreatitis
    — DU
    — Cholangitis
    — Mirizzi
• Papillary stenosis
    — Narrowing of the papilla following recurrent papillitis
    — ∆∆ spasm, papillitis
    — Rx      sphincterotomy
• PSC
    — See below
• 2˚ SC
    — ?
Infective
• Chlonorcis
Iatrogenic
• Thermal
• Ischaemia
• Trauma
Idiopathic
• Idiopathic fibrosing cholangitis
Mechanical
• Retained stones
  — Cystic / Hartmans with stone → Mirizzi’s
  — CBD stone
• Compression 2˚ lymphadenopathy
  — 2˚ malignancy
  — lymphoma
  — Inflammatory / infective
         TB
         Sarcoid



Biliary                                                         12
Tumour
• Benign
  — Rare
  — Usually pathological ∆
  — If recur
          Stent
          ‘core out’ JC!
• Malignant
  — Cholangiocarcinoma
• Other tumours
  — 1˚ liver
          HCC
          Angiosarcoma
  — 2˚ liver
          CRC
  — Lymphoma
  — Sarcoma
Vascular

Classification

Benign
Bismuth
• I           > 2cm from confluence          20%
• II          < 2cm from confluence          35%
• III         @ confluence                   30%
• IV          Involving confluence           15%
              Involving accesory duct (V)    5%

Rx
• Reconstruction with hepatico jejunostomy

Malignant
Peripheral
Hilar (aka Klatskins)
v   Bismuth
•   I         Below confluence
•   II        @ confluence
•   III a     involving RHD
        b     involving LHD
•   IV        involving L & R
              multifocal
Distal


Biliary                                            13
                      Primary sclerosing cholangitis (PSC)

Definition
• Inflammatory condition of the bile ducts
   — unknown cause
   — progressive obliteration

Epidemiology
• Rare
• M:F       2:1
• 25-45yrs
   — average 40yrs

Aetiology
• Unknown
• ?Autoimune

Associations
• HLAD8
• UC
  — UC present in 50% PSC
  — PSC present in 4% UC
  — Removing colon does not alter course
• Crohns
  — Lesser degree than UC
• Retroperitoneal fibrosis
• Mediastinal fibrosis
• Riedel’s thyroiditis
• Lymphoma

Pathology

Classification
• I distal CBD
• II diffuse following gallstone cholangitis
• III diffuse & progressive
• IV diffuse & progressive assoc with UC

Micro
• Fibrosis of bile ducts
   — Also includes GB
• Patchy involvementn of portal tracts
• Piecemeal necrosis & cholestasis




Biliary                                                      14
Natural history
• Patchy progressive obliteration of bile ducts
• Precursor for cholangiocarcinoma

Clinical
• Progressive jaundice
• Hepatic failure
   — varices
• Cholangiocarcinoma

Ix
• Hx & Ex
   — UC, Crohns, Lymphoma, thyroiditis
• Bloods
   — LFT
   — CRP, ESR
   — Autoantibodies
• USS
• MRCP
   — Beaded ducts
• ERCP
   — Beaded ducts
   — Advantages cf MRCP:
          brushings
          stenting
• ± Liver Bx
   — can miss lesions


Rx
• Steroids
• Immunosuppression
• ERPC & stent
• Surgery
   — Biliary enteric bypass
• Liver Tx

Prognosis
• Variable, but inevtiable progression
• ∆-death
   — mean 7yrs




Biliary                                           15
                           Primary biliary cirrhosis (PBC)

Definition
• Inflammatory condition of the bile duct
   — Unknown cause
   — chronic cholestasis
   — progressive destruction

Epidemiology
• M:F      1:9
• 35-70yrs

Aetiology
• Unknown

Associations
• RA
• Scleroderma
• Sicca complex
• Autoimmune thyroiditis

Pathology

Stages

•   Stage I
    v Destruction
    — Patchy inflammation
    — Destruction septal & interlobular ducts
    — Granulomas present
•   Stage II
    v Proliferation
    — Ductal proliferation
    — Distortion portal tracts
    — Parenchymal inflammation
    — Periportal fibrosis
•   Stage III
    v Fibrosis
    — ↓ inflammation
    — ↓ proliferation
    — ↑ scarring
•   Stage IV
    v Cirrhosis
    — Firm, irregular, bile stained regenerating nodules



Biliary                                                      16
Clinical
• Insidious presentation
• Asymptomatic              50%
• Pruritus, fatigue         >50%
• Hepatomegaly              50%
• Jaundice                  20%
• Splenomegaly              25%
• Xanthelasma               15%
• Hyperpigmentation         10%
• Others:
   — Clubbing
   — Osteoporisis
   — Peripheral neuropathy
   — Renal tubular acidosis
   — Steatorrhea

Ix
• Bloods
   — ↑↑ALP & GGT
   — ↑↑ IgM
   — ↑ Bili & AST/ALT
   — ↑ cholesterol and lipoproteins
   — Antimitochondrial Abs +ve 95%
• USS
• ERCP / MRCP
• ± Liver Bx

∆∆
• Extrahepatic biliary obstruction
• Intrahepatic cholestasis
   — drugs
• PSC

Rx
• ˚specific
• Tx
   — Excellent results

Prognosis
• Progressive, variable
• Symptoms devedlop over 2-7yrs

Poor prognostic features
• Rapid onset of symptoms
• Rising bilirubin
• Assoc AI disorders
• Advanced histo changes


Biliary                               17
                           Acute acalculous cholecystitis

Incidence
• 10% of all cases cholecystitis

Aetiology
• Transfusion        →       ↑ bile load
• Dehydration        →       ↑ bile viscosity
• TPN, ventialtion   →       ↑ bile stasis
• Shock              →       ischaemia

Associations
• DM
• Burns
• Arteritis
• Trauma
• ICU

Pathology
• Cholecystits in absence of cholelithiasis
• Often necrosis, gangrene & peerforation

Clinical
• Beware of sudden change in LFT in @ risk patient
• Rapid course

Rx
• Antibiotics
• Urgent cholecystectomy
   — Open vs laparoscopic
          Ventialtaion pressures

Prognosis
• MR 40%

                         Chronic acalculous cholecystitis

Incidence
• Fairly rare

Aetiology
• Adenomyosis
• Cholesterolosis
• Biliary sludge
   — ∴ not true aclaculous


Biliary                                                     18
Ix
• HIDA scan with CCK stimulation
   — ↓ ejection fraction

Rx
• Once ∆ as for symptomatic choleloithiasis

                                        Cholesterolosis
v Aka          strawberry gallbladder

Incidence
• 10% autopsies

Pathogenesis
• XS absorption of bile in GB wall
   — Choesterol stones frequently present
• 2˚ ↑ levels of cholesterol in bile
• Macro
   — GB has multiple yellow flecks
• Micro
   — Foamy macrophages 2˚ XS lipid

Clinical
• Often none

                                          Gallstones

Epidemiology
• Common
• ↑ with age
   — F       20% @ 60yrs
   — M       5% @ 60yrs
• M:F        1:3
• Geographical variation
   — Cholesterol predominate stones
          75% in West
   — Pigment stones
          20% in West
          60% Japan
          v Commonest world wide




Biliary                                                   19
Aetiology
• Age
   — ↑ biliary cholesterol secretion with ↑ age
• Environmental
   — Obesity
   — ↑ calorie diet
   — Fasting
   — TON
   — Ileal resection
   — gastrectomy
• Familial
• Predisposing conditions
   — Hypercholesterolaemia
   — Haemolysis
   — Cirrhosis
   — Biliary infection
   — Crohns
          Reduced bile salt pool

Pathogenesis

Classification
• Cholesterol
• Pigment
• Mixed
• Others
   — Calcium salts
   — Silicon / aluminium oxides

Cholesterol stones
v Cholesterol saturation → ppt → crystals → stones
Cholesterol saturation
  — See above
Nucleation
• Formation of cholesterol monohydrate crystals
  — Nucleation ↑ in pts with stones
  — Glycoprotein ↓ nucleation time
  — Mucous gel ↓ nucleation time
• Agglomeration requires nidus
  — Bacteria, epithelial cells, Ca++ crystals, GB mucin
Stone growth
• Ca++
   — part of central matrix of most cholesterol stones
   — ↑ in bile late & early in biliary disease

Biliary                                                   20
•   Gallbladder stasis
    — 2˚ to:
            OCP
            Pregnancy
            TPN
            Fasting
    — Factors
            ? sequestration of bile acids
            ? lengthen time cholesterol saturated bile in contact with mucosa
            ? alterations in GB absorption

Pigment stones
Black
• 20% of common stones
• XS insoluble unconjugated bilirubin
   — Ppt of calcium bilirubinate
• Soft tarry
• Exclusively in GB
• Associated with haemolysis or cirrhosis
• Not infected
Brown
v Accounts for majority of Asian stones & majority of stones post-chole
• Associated with:
   — Infection
         Biliary bacteria adhere to pigment stones
   — ß-glucoronidase
         Deconjugates bile salts, produced by E.coli
   — Liver flukes
         Deconjugate bile salts, ? mechanism
   — Cholestasis
         Choledochal cysts
         Biliary dyskinesia
• Found in intrahepatic & extrahepatic ducts

Biliary sludge
• Calcium bilirubinate crystals
• ↑ phospholipids

Morphology
• Pure cholesterol
  — Large
  — Round
  — Pale
  — Smooth




Biliary                                                                         21
•    Pigment (black)
     — Small
     — Multiple
     — Mulberry like
•    Pigment (brown)
     — Soft
     — Large
     — Crumble easily
•    Mixed
     — Multiple
     — Faceted
     — Laminated on X-section

Clinical
• Variable

Ix
• Full Hx & Ex
• FBC , LFT, Amylase, Coags
• AXR
   — 50% stones radio-opaque
   — gas in billiary tree
   — gas in lumen / wall
   — Porcelain GB
• USS
   — Sens > 90% for GB stones
• Biliary Scintigraphy
   v HIDA scan
   — 99Tc labelled iminodiacectic acid
          Conc of radioisotope by hepatocytes
   — Acute
          Non-filling
   — Elective
          + CCK
          Funtional study, ejection fraction
   — ERCP / MRCP
          For complications

Rx

Medical
Bile salt dissolution
• Cheno / ursodeoxycholic acid
    — Only suitable for cholesterol stones
    — Inhibits HMG CoA reductase



Biliary                                         22
•   Marginal efficiency
    — Prolonged Rx
    — 40% stone free @ 2yrs
•   Recurrence 50% @ 5yrs
•   Alternative to surgery in infirm, with ↓ no calculi & functioning GB
Lithotripsy
• Need functioning GB
• Fragments can cause pancreatitis
• Problem of remaining nidus
• Recurrence 50% @ 5yrs
Direct contact disssolution
• Methyl-tert-butyl ether (MTBE)
• 90% can be dissolved
• Requires perc catheter in GB
• Rx for 3/7
• Not well tolerated
• Recurrence 50% @ 5yrs
ERCP
• For complications
• If sphincterotomy performed evidence that cholecystectomy not required in elderly

Surgery

Indication
• Syptomatic cholelithiasis in patient fit for surgery
• Proven symptomatic acalculous cholecystitis
• Complications of gallstone disease
   — Acute cholecystitis
• Acute acalculous cholecystitis
v No data to support routine chole for asymptomatic stones
   — May be appropriate for:
           Individual with poor access to hospital care / prolonged travel overseas
           High risk elderly undergoing laparotomy for other reason
Procedures
• Laparoscopic ± open cholecystectomy ± IOC
   — Safe
   — Low M&M
   — 95% pts suitable
   — Few CI
         Suspected GB Ca
         Portal hypertension
         Severe respiratory co-morbidity
         Coagulopathy
         Cholangitis with septic shock
         Unfit for GA
         Preg (relative)

Biliary                                                                               23
    — Day case
            Can be performed safely & effectively
            Considerations: Age, Distance from hospital, Carer / help @ home, 1st on list
                             Analgesia, including ‘rescue’ package
       v Hollington ANZJS 99
            18% admission for nausea
            Return to work 1.8 weeks
•   Percutaneous cholecystotomy
    — Infirm patients with complications
Lap chole
• “In appropriately investigated, prepared & consented patient with DVT & antibiotic
   prophylaxis, I would…”
• GA, II table, supine, 30˚ scope, suction & irrigation, assistant
• LA, Sub umbilical incision, stay suture 1vicryl UR6, blunt port
• Insufflation 12mmHg, Table positioned reverse Trendelenburg and R side up
• Ports introduced under direct vision after infiltration with LA in R flank, RUQ, epigastrium
• Gall bladder grasped @ fundus, retracted cranially, Hartmans grasped & retracted laterally
• Peritoneum overlying hepatocystic ∆ divided anteriorly & posteriorly
• Blunt dissection Lateral to medial exposing contents of hepatocystic ∆
• Develop windows duct → artery & artery → liver, unequivocal identification
• Triple clip artery then duct (± IOC @ this stage), divide
• Diathermy dissection of GB bed, inspect bed
• Level table, lavage & aspirate
• Remove GB via umbilical port
   — Bag if rupture of GB
• Desufflate
• Closure umbilical port with 1 vicryl, sub cut 3-0 monocryl PS1 skin

•   Issues:
    — Consent
            Conversion to open
            Possibility of IOC
            Risk of bile duct injury
    — Aware of anatomical variants
    — Diathermy injury
            Use only when in contact with tissue
            Full view at all time
            Minimal tissue
            Use of ‘back’ of hook
    — Types of graspers, grasper trauma
    — Decompression of acute GB
    — Conversion to open if:
            Cannot identify anatomy
            Failure to progress      (in 30mins)
            Uncontrolled bleeding
            Bile leak
            Thickend (calcified) immobile GB

Biliary                                                                                          24
   — Drain
         Difficult dissection
         Ooze
Open Chole
• II table, patient supine, assistant, Book-Walter available
• Stand on pt L
• Subcostal incision
• Pack RUQ contents inferiorly
• ± pack liver down
• Asst with deaver retractor to liver
• Traditional vs Fundus first dissection
• Use of Leahey swabs, Leaheys
• Ligation of artery then duct
  — In continuity
  — Tie then divide
• ± IOC
• Haemostasis of GB bed
• ±drain
• Closure in layers 1PDS CTXB & s/c 3-0 monocryl PS1 to skin

Complications

Spilled stones
• Incidence
   — ≈10%
• Mechanism
   — Instrument injury
   — XS traction
   — Diathermy injury to GB
   — Dislodgement of clip
   — During removal
• Complications
   — 8-70% incidence of gallstones yielding +ve culture
   — Case reports of dropped stones causing abscess / extruding
• Recommendations
   — Pre-op abx
   — Attempt to remove allstones
   — Washout if spillage




Biliary                                                           25
Early
• Haematoma/collection             0.5%
   — Percutaneous drainage
• Bile leak
   — ERCP & stent                  0.5%
• CBD injury
   — Incidence                     0.5% LC
                                   0.2% open
•   Wound infection                0.5%
•   Respiratory
    — Atelectasis, infection, PE   <0.5%
•   DVT                            <1%
•   Hernia @ trochar site          0.4%
•   GIT injury                     0.2%

Late
• CBD injury / stricture
• Retained CBD stones
• Post cholecystectomy syndrome
   — ? original ∆

Mortality
• 1.7% for open chole
• Age related
  — <50yrs          0.3%
  — 50-65yrs        1.6%
  — >65yrs          5%
• <1% less for lapchole
  — 0.15% for elective LC




Biliary                                        26
                                           IOC
v Indications
  — Define anatomy
  — Detect CBD stones
• Sensitivity 98%
• Minimal morbidity
• False +ve 2%
• False –ve 2%
• Routine IOC
  — Will detect stones in ≈ 10%
  — 5% asymptomatic

Routine vs Selective

                              Routine                  Selective
Preop predictors CBD stones   Imprecise, PPV ≤ 75%     NPV ≥95%
Asymptomatic CBD stones       5% pts                   < 1% become symptomatic
                              Rx @ same time           ˚ evidence for Rx of asymp stones
IOC technique                 Practice                 Unneccesary in 95%
                                                       3-10% failure rate
Bile duct injury              Prevention               ˚ evidence of prevention overall,
                              Identify established     but will pevent more serious
                              injury                   injuries ( Type E)

                                                       False sense security
                                                       No substitute for adequate
                                                       dissection
                                                       Convert if concern
Medicolegal                   Support
ERCP service                  Poor
Operative time                                         Takes up to 30 minutes
Cost                          $700US/procedure
Rhetoric                      Being able to do is no   Selective is invitation to avoid
                              excuse to do


Suggested strategy

Selective IOC

Low risk
• No ERCP, No IOC
   — Asymptomatic
   — N LFT’s & USS




Biliary                                                                                    27
Medium risk
• IOC
  — Mild biliary pancreatitis
  — mildly deranged LFT’s
  — abnormal USS
  — prev choledocholithiasis
High risk
• Pre-op ERCP yield ≈ 50%
   — Cholangitis
   — Persistent jaundice
   — abnormal LFT’s
   — dilated CBD
   — visualistaion of stone on USS

Future
• possibilty of MRC for Medium ± high risk groups

                           Complications of Gallstones

Incidence
• 20% of pts with asymptomatic stones
• Serious complications
   — Asymptomatic                 1-2%/yr
   — Mild symptomatic     3%/yr

Classification
Complication                    % Hospital admissions
Biliary colic                     30%
Cholecystitis                     40%
Pancreatitis                      15%
Jaundice                          10%
Cholangitis                       5%
Biliary Fistulae
   Gallstone Ileus                 <1%
   Mirizzi syndrome
   Bouverets syndrome
Perforation                        <1%
Carcinoma of the gallbladder       <1%




Biliary                                                  28
Biliary colic
• Commonest presentation
• Due to impaction of stone in neck of GB.
Clinical
• Symptoms
    — Severe pain
    — sudden onset,
    — lasts several hours
    — Usually continuous
    — associated with
           restlessness
           vomiting
           sweating
    — May radiate to back (not to scapula).
• Signs
    — mild tachycardia
    — normal temperature
    — mild/non-tender abdomen.
Ix
•    USS
•    FBC
•    LFT
•    Amylase
•    ± OGD
•    ± HIDA
Rx
• Analgesia
• elective cholecystectomy

Cholecystitis
v    Chemical or bacterial inflammation of the gallbladder
•    95% of cases associated with gallstones
•    <10% of pts overall with cholelithiasis
•    20% of symptomatic patients

Pathogenesis
• Obstruction of the cystic duct by stone or oedema
   — distension
   — subserosal oedema
   — lymphatic and venous congestion
• Chemical irritants → inflammation
   — ?lecithin
• Role of bacteria not clear



Biliary                                                      29
Macro
• Thick walled, oedematous, hyperaemic ulcerated mucosa ±gangrene
• Empysematous cholecystits
  — Gas within wall or lumen
  — Primarily male DM
• Mucocele
  — Epithelium gone
  — Lined by CT
• Chronic
  — GB wall thickened by fibrosis
  — Mucosa intact
          Some inflammatory change
  — Rokistansky-Aschoff sinuses
          Herniations of mucosa through muscle coat

Clinical
• Symptoms
   — persistent pain in area of gallbladder
          epigastrium / RUQ
   — radiation of pain towards scapula
   — Frequently worse after eating
Acute
• Signs
   — Fever                   80%
   — RUQ tenderness
          ± peritonism
   — palpable mass           40%
   — ileus                   10%
   — jaundice                10%
• Complications
   — Perforation
   — Abscess
   — fistulae
Chronic
• Recurrent attacks of RUQ/epigastric pain
• often @ night
• Associated with
   — Nausea
   — vomiting
   — ± distension

Ix
•    USS
•    FBC
•    LFT
•    Amylase


Biliary                                                             30
Rx
• Analgesia
• NBM
• Antibiotics
• Cholecystectomy
   — Elective
   — Acute

Pancreatitis
v (One of) commonest causes of pancreatitis
• 15% of symptomatic gallstone disease
• % chance CBD stone after acute gallstone pancreatitis
  — Day 0         66%
  — Day4          20%
  — Day7          10%

Aetiology
• gallstone migration through ampulla of Vater
• → diversion of bile into pancreatic duct
• subsequent bile induced parenchymal damage
• Data from clinical studies suggests that it is the migration not the impaction that is
   responsible for the initiation of pancreatic injury.
• Further evidence from cholangiographic studies
   — up to 90% of patients with a history of gallstone pancreatitis have a common channel
       between CBD and pancreatic duct
   — cf. 20-30% incidence in pts with no history of pancreatitis.
• Recent experimental evidence suggests that pancreatic duct obstruction alone can initiate
   pancreatitis
• Associated with multiple small stones.

Ix
• Early USS
• LFT’s

Rx
• if evidence of stones in CBD early ERCP and sphincterotomy is indicated.
• Cholecystectomy

Cholangitis
• Choledocholithiasis accounts for 60% of cases
• CBD stones usually associated with cholelithiasis, but can occur de novo in a small
  percentage of patients
• The signs and symptoms of cholangitis are the most frequent manifestations of common duct
  stones.
• Biliary enteric fistulae lead to an increased incidence of cholangitis due to direct
  communication with GI tract.


Biliary                                                                                       31
Clinical

Symptoms
v Charcot’s triad
• 50-70%
  — Fever
  — Pain
  — Jaundice
v Reynolds pentad
• Above + shock and CNS depression 15%
• associated with
  — complete obstruction of CBD with pus under pressure
  — rapidly lethal unless emergency drainage.

Aetiology
• Causative organisms
   — E.coli
   — K..pneumoniae
   — S.faecalis
• 2/3 of patients have multiple organisms
• anaerobic organisms eg. B.fragilis, C.perfringens are found rarely, but do occur.

Ix
• Cholangiography
   — definitive test
   — only done when acute situation under control
   — Injection of contrast may provoke cholangio-venous reflux and exacerbate sepsis.
• Blood gases
• Coagulation parameters

Mx
• Monitor
   — Urine o/p
   — ± CVP etc
Rx
• Resuscitate
   — Volume replacement
   — Inotropic support as necessary
• Antibiotic therapy
   — need to cover Gram-ve and also anaerobic if severe
          triple therapy
          imipenem
• Decompression of biliary system
   — Operative               insertion of T-tube
   — Endoscopic
          Stent / nasobiliary drain
   — Percuataneous

Biliary                                                                                 32
Biliary Fistulae
v         Established and abnormal connection between biliary tree and some other area.

Internal biliary fistula

Aetiology
• Gallstones
   — 90% of internal fistulae caused by gallstones
• neoplasia
Complications
• Ascending cholangitis
• Mirizzi syndrome

External biliary fistula
Aetiology
• Gallstones
• peptic ulcer
• trauma
• neoplasia
Complications
• Gallstone ileus
• Bouverets syndrome

Mirizzi syndrome
v Unusual and specific cause of common hepatic duct obstruction by a stone impacted in the
  cystic duct

Classification
• I Stone compressing Duct
• II Sroine eroding into the duct causing a cholecysto-choledochal fistula.

Clinical
• obstructive jaundice

Ix
• Cholangiography
   — narrow bile duct
   — may be mistaken for cholangiocarcinoma.

Rx
• Cholecystectomy
• Repair defect
   — over T-tube
   — biliary enteric reconstruction.


Biliary                                                                                      33
Gallstone Ileus
v Gall stone or common duct stones enter intestinal lumen through an internal biliary fistula.

Incidence
• Most gallstones that enter the GI tract are either passed or vomited
• 10-15% may lead to gallstone ileus
• 1% of all cases of intestinal obstruction
• 25% of simple obstruction > 70yrs

Pathology
• 3/4 of fistulae occur between gall bladder and duodenum
   v cholecystoenteric fistulae

Clinical
• Hx of frequent previous episodes of partial SBO
   v tumbling obstruction
   — exacerbates as the stone travels distally into the narrower parts of SB
• Finding of a large mass lesion at site of obstruction

Ix
• Xray
   — easily seen if opaque
   — if non-opaque, can get outlined with air.
   — Air in biliary tree.

Rx
• Relief of obstruction
   — enterotomy
• Concomitant repair of fistula if:
   — patient in good condition
   — no prolonged pre-op losses
   — no intra-op complications
   — no significant inflammatory reaction at fistula site

Prognosis
• Enterotomy alone
   — 10% recurrence
   — higher incidence of
          cholangitis
          recurrent cholecystitis
          gallbladder ca.




Biliary                                                                                          34
Bouverets syndrome
v   Gastric outlet obstruction by a gallstone
•   first reported by Bouveret in 1896
•   very uncommon
•   large stone occludes duodenum → gastric outlet obstruction
•   stones ≥ 2.5 cm
•   symptoms
    — those of bowel obstruction
             abdominal pain, nausea, and vomiting
    — Less commonly melaena
    — Rarely haematemesis

Carcinoma of the gallbladder
• 5% of all cancers found a autopsy
• Association between gallstones and gallbladder carcinoma well established
• present in at least 70% of patients
• Parallel exists between incidence and ethnic variation of gall bladder carcinoma and gall
   stones
• No predilection between multiple or single stones
• Possible relationship between size of stone and incidence
   — reported that stones >3cm have 10x incidence than stones <1cm
• 15% incidence in patients with cholecystoenteric fistulae

                                  Choledocholithiasis

•   ≈10% of patients with cholelithiasis
•   ≈30% with deranged LFT, dilated CBD
•   5% if all tests normal
•   Incidence increases with age
•   Site of impaction usually immediately above the ampulla

Aetiology

Primary
• Can form de novo
   — Primary common duct stones usually composed of calcium bilirubinate
   — ovoid, brown and crumble easily
   — always associated with bile duct obstruction
   — freq. Assoc with infection
   — In Asia associated with parasites in biliary tract.
• Or 2 years post chole with no symptoms / signs




Biliary                                                                                       35
Secondary
• From GB

Natural Hx
• 1/3 become symptomatic /10yrs
• up to 20% incidence of pancreatitis
• MR unRx stones ≈5%

Clinical

Asymptomatic
Complications
• Obstructive jaundice     70%
  — Hepatorenal syndrome
           See Liver
           Ocurs in 10% with obs jaundice
• Cholangitis
  — Charcot’s triad
           RUQ pain
           Fevers / rigors
           jaundice
• Pancreatitis             12%
• Liver abscess
• 2˚ biliary cirrhosis

Ix
• LFT
   — ↑ bili & ALP assoc with ≈ 30% chance choledocholithiasis
• US
   — Overall ≈30% sensitivity
   — Diameter ↑ 1mm/decade from 3mm @ 30yrs
   — Diameter vs incidence
          <6mm              2%
          >10mm             45%
     v NB 45% of CBD stones diameter <6mm
• ERCP
   — 95% success in visualising biliary tree
• MRC
   — As accurate as ERCP
          Recent studies suggest 100% sens, 96% spec
   — ∆ 95% of stones >5mm
   — No complicatins
• PTC
• EUS
   — ˚ ∆ studies of note
• IVC

Biliary                                                         36
     — Sensitive, large radiation dose
•    Helical CT
     — Not very sensitive
•    IOC
     — See above

Rx

Pre Op ∆

ERCP
• Overall 90% success @ extracting CBD stones
  — Lower if :
          >5 stones
          Any stone > 1cm
• Mortality                 0.1-0.2%
  — + sphincterotomy        1%
• Morbidity                 2-5%
  — + sphincterotomy        ≈10%
  — Worse for:
          Smaller ducts
          Young people       incidence of papilary stenosis
  — Might be less for dilatation cf sphincterotomy
• Indications
  — Cholangitis
      v Poon et al HK
          95% pts with cholangitis Rx sucesfully with ERCP & LC
          Mortality 1.6%
          Recurrent biliary probs with no LC 25%
  — Persistent jaundice
  — Bile duct dilatation
  — Abnormal LFT’s
• C/I
  — ≈ Recent pancreatitis
  — Billroth II
  — oesophageal, gastric or duodenal obstruction.
  — ≈ duodenal diverticulum
  — Clotting disorders
• Complications
  — Pancreatitis            5%
  — Bleeding
  — Perforation
  — Impacted basket
Others
• Oral dissolution
• MTBE
• ECSWL
• Radiological via T-tube
Biliary                                                           37
Intra op ∆

Approach
• <6mm stone, Duct <10mm, non obstructed
      1. Leave
      2. Post op MRC
      3. ERCP if symptomatic
• <6mm stone, Duct >10mm ± obstructed
      1. 1mg Glucagon IV & Flush ≈200ml saline
      2. Transcystic exploration
      3. Open exploration if obstructed
      4. Consider leaving & postop ERCP if unobstructed
• >6mm stone
      1. Transcystic exploration
      2. Open exploration
Laparoscopic choledocholithotomy
   Trans-cystic exploration
   • Technique
      — Flexible choledochoscopy
             Extra 5mm port
             Stay sutures in cystic duct
             ± dilatation of cystic duct
             3mm choledochoscope
             Basket under direct vision
             ± crush stone
      — Direct basketing is possible without choledochoscopy, but more difficult
   • Limitations:
      — max size of stone ≈ 8mm
      — Difficult to access proximal stones
   Direct choledochal exploration
   • Technique
      — dilated bile duct
      — additional 10mm port
      — longitudinal incision in CBD
      — duct exploration ± choledochoscope and basketing or ballooning
      — Duct closed:
             Primarily
             Over biliary stent
             Over T-Tube
   • Advantages: access all duct
   • Limitations: Technically difficult, time consuming.
   v Rhodes Lancet 1998
      Randomised trial demonstrates parity with post op ERCP and shorter hospital stay
      But ERCP results poor



Biliary                                                                                  38
Open Choledochotomy

   Supra duodenal exploration
   • Technique
     — Anterior surface CBD exposed above duodenum
     — Duodenum Kocherised
     — CBD confirned by needle aspiration
     — Stay sutures inserted in CBD
     — CBD opened longitudinally
         v Site of choledochotomy should be distal (to cystic duct if N anatomy)
     — Duct exploration with flexible choledochoscope & fogartys,
             stone forceps (Desjardins) historical interest
     — Duct closed over T-tube with fine absorbable suture 4-0
     — T-tube brought out laterally
     — T-tube cholongiogram performed.
   • Post op
     — Free drainage,
     — T-tube cholangiogram and then clamping of tube
     — T-tube removed 4/52
   Trans duodenal exploration
   • Technique
      — Bile duct approached across duodenum
      — Duodenum mobilised
      — longitudinal incision made in right lateral wall opposite ampulla
      — Ampulla cannulated and divided with scissors / needle point diathermy
          11 o’clock to duct
      — Mucosa of bile duct apposed to duodenum and stones extracted
      — Incision closed transversely
Drainage procedures:
• Indications
   — Recurrent stones
   — primary duct stones
• Procedures
   — side to side or end to side choledochoduodenostomy
   — choledochojejunostomy
Post op ERCP
• Indications
   — intra-op detection
   — retained or recurrent stones
• Morbidity
Radiological removal
• aka         Burhenne technique
• Less common now
• T-tube track needs to mature ≈ 6 weeks
• Technique
  — flexible catheter passed down T-tube tract and stones basketed
Biliary                                                                            39
Prognosis
• MR 1.7% all open chole
• CBD explorn MR 3% elective, 8% acute
   — ↑ with age, cholangitis, pancreatitis
• incidence of retained stones post exploration 1-5%
• Small ducts @ risk of stricture

Refs:
Oxford 1994
Sabiston 1997
Perissat J. et al., Management of bile duct stones in the era of lap chole. BJS 1994, 81 799-810.
*Rhodes M et al., Randomised trial of Laparoscopic exploration of common bile duct versus
postop ERCP for CBD stones. Lancet 1998; 351: 159-61




Biliary                                                                                             40
                                   Bile duct injury

Epidemiology
• Incidence
   — Open 0.1-0.5%
   — Lap 0.2-1%
v I.e. 2x risk

Aetiology

Dangerous anatomy
• Variant anatomy

Dangerous Pathology
• Acute cholecystitis
• Fibrosis in hepatocystic ∆
• Mirizzi
• Obesity

Dangerous Surgery
• Failure to recognise anatomy
• Local haemmorrhage
• Technical
  — Inadequate dissection
  — Thermal injury
  — Failure to secure cystic duct stump
  — Too deep on liver bed
• IOC
  — Will help prevent transection
      injuries, but does not ↓ overall
      incidence

Classification
• Strasberg 1995

Prevention
• Dissection
   — Blunt
   — Minimal cautery
   — Retrograde GB→ CBD
• Exposure
   — 30˚ scope
   — Open hepatocystic ∆
   — windows



Biliary                                               41
•    Identification
     — Prior to ligation
     — See tips of stapler
•    Conversion
     — Low threshold

Clinical
• Immediate recognition
   — Bile leak
   — IOC findings
• Early
   — Persistent pain or nausea   
   — Low grade fever              = bile leak
   — Mildly deranged LFT’s       
         Bilirubin
         Transaminases
         Cholestatic enzymes
   — ± Peritonitis
   — ± Jaundice
• Late
   — Jaundice
   — Cholagitis
v NB may be accompanied by vascular injury
   — Most common is occlusion / division RHA

Ix
• US
   — Free fluid in pelvis
• ERCP

Rx

Intrap recognition
• Convert
• Transection
    — 50% stricture rate if 1˚ repair 2˚ to thermal injury
    — ∴ Hepaticojejunostomy
• Incision
    — Repair over T-Tube

Postop
Early
• ERCP
   — ∆
   — Rx if minor injury by stent
• Percuataneous drainage for collection
• Primary repair

Biliary                                                      42
    — Close over T tube if <50% circumference
    — Hepaticojejunostomy if transection
Late
• Resuscitate
• Control sepsis
   — Intrahepatic
          PTC & drain
   — Extrahepatic
          Percutaneous drain
• Biliary drainage
• Operative Rx




                                Mx malignant jaundice
•   ∆ cause
•   ∆ resectability
•   Selection of best Rx

Aetiology
v   Post hepatic malignant jaundice
•   Pancreatic Ca             60%
•   Cholangiocarcinoma        35%
•   GB Ca
•   Ampullary Ca
•   CRC mets @ hilum
•   Periportal LNM

Clinical
• Jaundice
• Cholangitis
• Those of 1˚

Ix
• MRI / CT
• ERCP




Biliary                                                 43
Rx

Selection
• Resectability
   — Intraop vs preop ∆ of non-resectability
• Requirement of GI bypass
• Fitness for anaesthesia
• Expected survival
   — >6/12 → surgery
• Endoscopic CI / difficulty

Resectable lesions
• Immediate resection
  — Less sepsis
  — ↑ risk hepatorenal syndrome
• Stent & operate
  — ↑ sepsis
  — ↑ periop morbidity

Non resectable

Indications for surgical bypass
• If ∆ in OT
• Expected survival > 6/12
• Irresectable hilar stricture
• C/I ERCP / stenting
• Pt preference
Surgical options
• Cholecystojejunostomy
   — Laparoscopic / Open
   — No dissection ectrapancreatic ducts
   — GB can be used if tumour 2-3cm from cystic / BCD confluence
   — R-enY or simple loop
   — Cholangitis / recurrent jaundice 20%
• Choledochojejunostomy
• Segment III chalngiojejunostomy
   — Good palliation for hilar strictures
   — ↓ cholangitis recurrent jaundice cf stenting
   — Drainage of segment III sufficient to relieve jaundice & pruritus
   — Median survival better than stenting
ERC Stenting
• Plastic stents
  — 10-12Fr
  — block easily
  — are interchangeable
  — Lifespan 3-6/12
Biliary                                                                  44
•   Metal stents
    — Large lumen
    — ↓ cholangitis
    — last longer
•   > 1 attempt required in 1/3 those sucesfully stented
•   Complications
    — Early            Bleeding, pancreatitis, cholangitis, stent migration
    — Late             Cholangitis, stent migration, T overgrowth, blockage
PCT stenting
• High strictures
• Plastic or metal stents
• External stent / drain
  — External loss of bile salts
  — Daily care
  — 25% incidence of cholangitis
Results
• Good palliation in > 90%
                               Stent                            Surgical bypass
Recurrent jaundice             35%                              5%
Cholangitis                    ↑                                ↓
Morbidity (early)              10%                              30%
Mortality                      3%                               10%
Prognosis                      21/52                            26/52
• Prognosis
   — Overall survival same in both groups
            1 study showing marginal advantage with surgical bypass




Biliary                                                                           45
                              Benign Bile duct tumours

Epidemiology
• Incidence
   — 6% extrahepatic biliary tumours
   — 3/ 2500 @ autopsy
• M:F       1:1

Aetiology
• No association with choledocholithiasis

Pathology
• Papilloma
   — Pedunculated
   — Papillary with well differentiated epitheium
• Adenoma
   — Sessile
   — Can be multiple
   — Glands in epithelium
   — 10% risk of Ca in situ
          Risk of Ca ↑ with size
• Adenomyoma
   — Localised collection of gland like structures in GB wall
   — Surrounded by hyperplastic SM
   — Gross
          Nodule
   — Micro
          Microcysts
   — ?hamartoma
   — Cholecystectomy on basis of symptoms
   — When affecting large part of GB → adenomyomatosis
   — ? association with GB Ca
• Fibroma
• Leiomyoma
• Granular cell angioblastoma
• Neural tumour




Biliary                                                         46
                                  Cholangiocarcinoma

Definition
• Malignant hepatic tumours of biliary epithelium
• Arise anywhere from small biliary radicles → extrahepatic ducts

Epidemiology
• Incidence
   — 0.3-0.4% autposy
   — 1.2 / 100000
• 50-70yrs
• M:F        3:2

Aetiology
• Acute or chronic biliary epithelial injury

Risk factors
• Hepatolithiasis
   — 10% will develop Ca
   — Common SE Asia
• PSC
   — Ca in 40% @ autopsy
   — 10% @ liver Tx
• UC
   — Incidence 0.1-1%
          10x more common
       th
   — 5 decade
• Liver flukes
   v Most commonly intrahepatic
   — Clonorchis senensis
   — Ascaris lumbrocoides
• Choledochal cyst
   — ↑ with age
          <10yrs              1%
          10-20yrs            10%
          >20yrs              20%
   — ? due to pancreatic refluxate
• Transduodenal sphincteroplasty
   — 7% incidence
   — 2˚ to chronic irritation
   v ˚ evidence for ↑ risk with ERCP




Biliary                                                             47
•   Other
    — Infective
          HCV
          Chronic typhoid
    — Chemicals
          Asbestos
          Dioxins
          Nitrosamines
          Biphenyls
    — Drugs
          Isoniazid
          Methyldopa
          OCP
    — Radionuclides
          Thorium dioxide

Pathology

Types
• Sclerosing / Nodular sclerosing
   — 90%
   — Obliterates duct
   — Can be eccentric
   — Desmoplastic reaction surrounding
   — Spreads within wall of duct
   — Mucosa often intact
• Papillary
   — 10%
   — Intraductal variant
   — 10% cholangiocarincomas
   — Expansive
   — Distal bile duct
   — Often contained, with narrow stalk
   — More favourable prognosis

Classification
• Distal
   — Middle 1/3             20%
   — Lower 1/3              20%
• Diffuse                   5%
Peripheral
• <10% cholangio Ca in western series
• Scirrhous, never encapsulated
• Infiltrative growth
• Usually poorly differentiated adenocarcinoma
   — Small number papillary
   — SCC, mucoepidermoid & spindle cell variants

Biliary                                            48
•   Can combine with HCC
    — Cholangiohepatocellular Ca
•   Stain for CEA
    — Many will stain for CA50 & CA 19-9
Hilar
• Aka Klatskins
• 50% cholangioCa
• Type
    — Nodular sclerosing 80%
    — Papillary            10%
    — Benign pathology     10%
• ↑ Ca 19-9
• 30% 5yrs after resection
Distal
• Adenocarcinoma
• Papillary more common

Spread
• 30% peripheral have peritoneal or hepatic mets @ presentation
• Less tendency to invade
• Metastasis to varierty of sites

Staging
• Peripheral
   — as for HCC
• Hilar
   — Bismuth
          I          Below confluence
          II         Confined to confluence
          IIIa       RHD
          IIIb       LHD
          IV         RHD & LHD
   — TNM
• Distal
   — TNM
TNM
• T
  — T1
         a Subepithelial CT
         b Fibromuscular layer
    — T2   Perifibromuscular TC
    — T3   Adjacent structures
•   N
    — N1   local LNM
    — N2   regional LNM


Biliary                                                           49
TNM Stage
• I           T1
• II          T2
• III         T1-2, N1-2
• IV
  — a         T3
  — b         M1

Clinical

Peripheral
• Malaise
• Abdominal pain
• ± fever
• Weight loss
• Jaundice
   — Peripheral      30%
   — Papillary       episodic
   — Hilar           Frequent, but late
   — Extrahepatic    90%

Ix

Bloods
• LFT, FBC, U&E, Clotting
• Ca19-9, CEA
USS
• Duplex for defining vascular anatomy
  — Especially @ porta
  — Predicts portal v involvement in 90%
MRI
• Peripheral / Hilar
  — Hypo/isodense         T1
  — Hyperdense            T2
  — Defines disease extent & vascular anatomy
• Distal
  — Stricture
  — Usually patent pancreatic duct
CT
• Peripheral
   — Avascular soft tissue mass
• Hilar
   — Mass lesion
   — Extrahepatic disease
   — Lobar atrophy
          Implies portal vein obstruction

Biliary                                         50
•    Distal
     — Often no mass lesion on CT
ERCP
• Hilar
  — Can undrestimate extent
  — Associated with increased incidence of bactobilia (60%)
         ↑ incidence of cholangitis
• Distal
  — Stricture
  — Usually patent pancreatic duct
• Brushings
  — Sens /spec ≈50%

∆∆

Peripheral
• GIT mets
• HCC

Hilar
• Benign strictures 5-10%
• Mirizzi syndrome
• GB Ca

Distal
• GB Ca
• Cystic duct tumours
• Benign strictures
• Pancreatic Ca




Biliary                                                       51
Rx

Pre-op
Stenting
• Endoscopic
   — 100% incidence of bactobilia
• Percutaneous
   — 65% incidence bactobilia
• Indications
   — Cholangitis
   — Elderly
   — Comorbidities eg DM
   — Bili
          500-600 if otherwise well
          >200 if DM
   — Long time to surgery
   — Instrumentation CBD
• For
   — Improve coagulation
   — ↓ endotoxaemia
   — Improve renal function
• Against
   — ↑ post op infection
          ≈ 30%
   — ↑ blood loss
Laparoscopy ± US
• to exclude peritoneal disease 10%

Peripheral
• Exclude upper / lower GI malignancy
• Exclude poor diff HCC
   — αFP, HBV,HCV serology
• Consider for resection
   v 20% have resectable lesions at presentation
   — Medically fit
   — Childs A
   — Frozen section of ducts mandatory to ensure full clearance
• Tx
   — Option for localised tumours




Biliary                                                           52
Hilar

Nutritional support
• Consider oral supplements pre-op
• Feeding jejunostomy intra-op
Operation
• Hilar resection ± hepatic resection
  — + hepatic resection appears to have better results
  — Begin with division of CBD @ upper border of D1
  — Allows
          skeletoisation of PV & HA
          assessment of local infiltration
  — Frozen section of ducts mandatory to ensure full clearance
  — Reconstruct with Roux loop

Distal
• Duct excision
   — Frozen section of ducts mandatory to ensure full clearance
• Pancreaticoduodenectomy

Complications

Liver resection
• Liver failure
   — Non cirrhotic <50% resection         1%
                    >50%                  10%
   — Cirrhosis      <50%                  50%
                    >50%                  100%
• Bile leak
• Coagulopathy
• Hypophosphataemia
   — Begin to fall 12 hrs post op
   — Lowest level @ 48hrs

Hilar resection
• Biliary fistula
   — ERCP
   — Drainage
• Portal v thrombosis
   — Rare
   — Operative intervention & thrombectomy

Prognosis
• ≈1/3 unresectable after staging




Biliary                                                           53
Peripheral

Prognostic factors
• Margins
• Vascular invasion
• Satellite lesions
Survival
• Resection
   — 40% 5yrs
• Tx
   — 64% 3yrs for small node –ve

Hilar

Periop mortality
• 5-10%
Survival
• 30% 5yrs
Prognostic factors
• Margins

Distal
Prognostic factor
• Margin
• LNM
Survival
• 30% 5yrs




Biliary                            54
                                       GB cancer
•   Most common malignancy of biliary tract
•   5th most commmon GI
    — CRC
    — Gastro-oesoph
    — Liver
    — Pancreas
    — GB

Epidemiology
• Incidence
   — 2.5/100000
   — ? increasing
• Geographical variation
• Racial variation
   — Native Americans
   — Israelis
• M:F         1:3
• 50-70yrs

Aetiology
• Genetic
   — P53
   — C-erb-B2
   — K-Ras
• Congential
   — Anomolous biliary/pancreatic duct union
          Pancreaticobiliary reflux
   — Choledochal cyst
          Overall ↑ biliary malignancy include GB Ca
          Does not appear to ↓ after eection
• Cholelithiasis
   — 75%
   — Mirizzi syndrome
   — ? Related to size
• Bld group A
   — Common with somach, colon, pancreas, bladder
• Porcelain GB
   — Diffuse calcification
          <0.1% cholecystectomies
          95% assoc with gall stones
          M:F        1:5
          10-25% assoc with Ca
• Chronic typhoid
   — Uganda, early GB Ca
• Occupation
   — Rubber industry

Biliary                                                55
•   IBD
    — 13% of IBD assoc Ca in GB
    — Risk ↑dt with longstanding pancolitis
•   Benign neoplasms
    — Adenomas
           Particularly non-papillary
           >10mm
           Risk 30% @ 30mm
    — ? Adenomyomatosis
           probably not premalignant
           common coexisting condition
    — no reports of Ca in Rokistansky-Aschoff sinuses

Pathology

Type

1˚
• 98% of GB malignant umours are carcinomas
   — Adenocarcinoma       80%
   — SCC
   — Adenosquamous
   — Oat cell
• 2% Others
   — Carcinoid
   — Melanoma
   — Lymphoma
   — Rhabdomyosarcoma
   — Spindle cell tumours
2˚
• Breast
• Lung
• Melanoma

Spread
• Local invasion
   — Commonest
          Up to 70% @ presentation
   — Segments IV & V
   — Adjacent structures
• Haematogenous spread
• Lymphatic
   — To cystic and portal nodes
          Up to 45% @ presentation




Biliary                                                 56
Staging

Nevin
• I       Intramucosal
• II      Muscularis
• III     Through wall
• IV      Through wall, cystic LNM
• V       Involving liver ± distant mets
TNM
• T
  — T1
          a LP
          b Invades muscle
    — T2    Perimuscular CT, not through serosa
    — T3    Through serosa
            Directly invades 1 adjacent organ
            Directly invades liver <2cm
    — T4    >2cm into liver
            ≥ 2 adjacent organs
•   N
    — N1    Local LNM
    — N2    Regional LNM
•   Stage
    — I     T1
    — II    T2
    — III   T3
            T1-3, N1
    — IV
          a T4 ± N1
          b N2
            M1

Clinical
• Mimic GB disease
   — acute cholecystitis         20%
   — chronic cholecystitis       40%
   v ≈1% of choles have evidence of Ca
• ∆ often made post chole
• Malignant biliary disease      30%




Biliary                                           57
Ix
• Bloods
   — CEA
   — CA 19-9
• USS
   — Irregular thickening of wall
   — Intraluminal mass
   — Poorly defined boundaryt with liver
   v ∆ 70%
• CT
   — ∆ ≈80%
   — Assess liver involvement
• MRI/MRCP
   — More accurate than ERCP to differentiate hilar vs GB
   — Detects hepatic invasion
   — Hilar LNM
   — Portal involvement
• ERCP
   — Obstruction / distrotion of CHD
   — Non filling GB
   v ∆ 60- 80%

Rx
• ≈ 60%unsuitable for resection

Options
T1
• Simple cholecystectomy
T2-4
• Extended cholecystectomy
   — En-bloc removal of IV & V
   — Distal tumours (fundal)
• Extended hepatectomy ± extrahepatic duct excision
   — Proximal tumours
v All re-explorations should have port sites excised & portal lymphadenectomy
v Adherent structures removed en-bloc
v Use of frozen section for duct margins

Prognosis
• T1                90% 5yrs
• T2                60-80% 5yrs
   — Radical resection
• T3 & T4           20-30% 5yrs
v Major prognostic factor is clear margins

Biliary                                                                         58

				
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