Portal

Biliopathy

Dr Niket M Shah

Moderator – Prof S Sankar

July 2020
  Biliary tract anatomy & Blood supply

 Portal Cavernoma formation

 Pathogenesis of PB

 Clinical profile

 Diagnosis

 Management
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 Anatomy

Arterial supply

Venous drainage

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 INTRA-HEPATIC BILIARY
TRACT

▸ Parallels portal venous & hepatic arterial

supply.

▸ RHD pattern – 57%

▸ LHD pattern – 67%

Length Diameter

RHD 0.9 cm 2.6 mm

LHD 1.7 cm 3.0 mm

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 EXTRAHEPATIC BILIARY
TRACT
▸ CBD
▹ Supra-duodenal – right border of lesser omentum
▹ Retro-duodenal – Right of GDA
▹ Intra-pancreatic – 85% > 15% - Retro-pancreatic
▹ Intra-duodenal

Length Diameter
CHD 1 – 7.5 cm 4 mm
CD 3 – 4 cm 4 mm
CBD 6 – 8 cm External 9 mm
Internal 8 mm
Ampulla 4 mm
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 ▸ Totally dependant on hepatic arterial supply - ???

▸ Slieker et al – 40% by portal vein to microvascular blood

flow through CBD in surgically simulalated condition

▸ Such contribution in normal physiologic conditions –

ARTERIAL SUPPLY Inconclusive

▸ 50% of hepatic arterial supply for biliary tract

▸ 4 parts
▹ Supra-duodenal CBD & CHD
▹ Retro-pancreatic CBD
▹ Hilar ducts
▹ Intra-hepatic bile ducts
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 ▸ Supraduodenal CBD including CHD
66%
33%
Descending vessels
Ascending
vessels

▹ Inferior border of cystic duct – Zone of overlap b/w both vessels

▹ Paracholedochal & Epicholedochal arteries

▹ 3 & 9 o’clock / Left & Right arch arteries / Left & Right marginal
artery
LMA = 3 o clock RMA = 9 o’clock

95% present 82.5%

< PSPDA / GDA < PSPDA

7 Joins RHA Joins CA

 ▸ Retroportal artery [ >90% cases ]
▹ SMA [60%] or CT [40%]
▹ SupraDuodenal & RetroDuodenal CBD
▹ Ascends on posterior surface of portal vein & head of pancreas
▹ Joins
■ PSPDA [ Type I ]
■ RHA [ Type II ]

▹ Larger caliber than 3 & 9 o’clock vessels

▹ Diameter 0.92 mm

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 Retro-duodenal CBD Cystic & Hilar hepatic duct Intra hepatic bile duct

PSPDA – Dominant Cytic artery Peri-biliary plexus

RHA & LHA
Retro-portal artery Hilar plexus

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 Intra hepatic biliary ducts
▸ Peri-biliary plexus
▹ Drain into venules
Arterio-Portal
▹ Joining intrahepatic portal system system
▹ Reaching sinusoids

▸ Ischemic changes – LEAST COMMON

▸ Peri-biliary plexus  Extra hepatic bile duct plexus

▸ [ Hepatic artery  GDA ]

▸ Occlusion of hepatic artery  ??

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 Wall of CBD

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 ▸ Plexus

▹ Epicholedochal p of SAINT – Surface of CBD

▹ Paracholedochal p of PETREN – Parallel to CBD

▸ 3 & 9 o’clock marginal veins

Venous Drainage
▸ Few – 6 o’clock marginal vein on posterior surface of
CBD

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 Cystic vein

ALWAYS joins
9 o’clock vein

NEVER joins
Right portal vein

3 o’clock vein

ALWAYS joins
Right gastric
vein

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 Portal Cavernoma

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 Portal Cavernoma formation
▸ Drainage of HOP & Duodenum ASPD
Anterior V
AIPDV
▹ 2 venous arcade
Posterio PSPDV
r PIPDV

▸ When PVT extends into SMV & SV

Hepatopetal collateral venous flow -

preferentially routed around HOP involving

PSPDV, GCT & bile duct venous plexus rather

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than via GV & GEJ.
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 Portal HTN

External compression
FIRST
Paracholedochal Protrusion of varicose PCD
veins dilatation veins into thin duct wall

Epicholedocha
Smooth intraluminal
l veins
surface  Irregular
dilatation

Subepithelial Subepithelial varices

plexus
enlargement Troublesome hemobilia

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 VARICOID FIBROTIC
APPEARANCE Types of abnormal morphology APPEARANCE

Dilatation of
Dilatation of large intramural
paracholedochal epicholedochal
veins veins

Compression & Compromises

Distortion of arterial supply of
bile duct wall
Ischemic changes
Varicoid Fibrosis
appearance Stricture

REVERSIBLE
with collateral decompression IRREVERSIBLE
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 ▸ Serpiginous tortuous & dilated
▹ 3 & 9 o’clock marginal veins
▹ Venous plexus of petren
▹ Venous plexus of saint
▹ Cholecystic veins surrounding & within GB wall

▸ Duration for formation – weeks to years

▸ Long term – “Solid tumor like cavernoma” – Fibrous hilar mass

with multiple collateral veins

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 Etiology of cavernoma formation
▸ Any cause of PVT

Adult
Children

RARE in cirrhosis
Hypercoagulable states with Portal HTN
Neonatal umbilical sepsis Myeloproliferative disorders
Bechet’s syndrome
Dehydration Pancreatitis
Pylephlebitis

Idiopathic in
30%
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 Studies ..
▸ Denys et al

▹ Cavernoma composed of tiny vessels in wall of CBD

▹ Choledochal & cystic veins – most frequent porto-portal bypass routes in PVT

▸ De Gaetano et al
▹ Formation of cavernoma in 6-20 days of acute PVT

▹ Majority – intrahepatic extension of cavernoma

▹ Both Porto-systemic [ LGV ] & Porto-portal [ Periportal & pericholecystic venous

channels ] seen

▹ ALL – HEPATO-PETAL FLOW

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 PORTAL
BILIOPATHY

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 ▸ CHOLANGIOGRAPHIC ABNORMALITIES DUE TO PORTAL
CAVERNOMA

▸ Similar abnormal cholangiography

▹ Choledocholithiasis
▹ PSC NOT DEFINED

▹ Biliary parasitosis AS

▹ AIDS cholangiopathy PORTAL

▹ Oriental cholangio hepatitis BILIOPATHY

▹ Cholangiocarcinoma

▸ In cirrhosis & idiopathic portal HTN

▹ Hepatic nodularity & fibrosis  Intra-hepatic biliary abnormalities
▹ NOT BY CAVERNOMA
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 ▸ Synonyms

▹ Pseudosclerosing cholangitis
▹ Pseudocholangiocarcinoma sign
▹ Portal hypertensive biliopathy
▹ Extra-hepatic portal biliopathy
▹ Vascular biliopathy
▹ Portal ductopathy
▹ Portal cholangiopathy
▹ Portal cavernoma cholangiopathy

▹ Sarin et al – PORTAL BILIOPATHY

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 PATHOGENESIS
Reversible Component Fixed Component

Shallow bile duct

impressions Rigid stricture

Indentation GB
stones
Wall irregularity Angulation

Smooth strictures with

upstream dilatation Ductal ectasia

Luminal filling defects

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 Clinical profile

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 ▸ Majority – ASYMPTOMATIC , NO BILIARY SYMPTOMS

ASYMPTOMATIC STAGE CLINICAL STAGE

Early cholangiographic Elevated bilirubin &/or icterus

abnormalities Advanced cholangiographic Biliary pain
Duct iregularity abnormalities Cholangitis
Serration Ectasia of ducts
Undulation Angulation
Scalloping of wall Displacement Older population
Extrinsic nodular, spiral or stenotic Stricture [ long & multifocal ] Extensive PVT & advanced Liver
impressions Aneurysmal dilatation of IHBR disease
Filling defects in CBD & CHD

Elevated ALP

Last for years

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 DIAGNOSIS

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USG with color doppler CT or MRI EUS ERC

► Gold standard
▸ Portovenography ▸ Visualization of
▸ Atretic/Recanalized PV ► Being replaced by
▸ Etiology of PVT choledochal &
▸ Serpentine collaterals in USG & MRCP
▸ MRI >>> CT subepithelial venous
porta hepatis
▸ MRI – IxOC plexus important as
▸ Increased hepatic artery flow
▹ Epi – signal void biliary endotherapy
▸ GB wall varices
defects may increase risk of
▸ Collaterals in thickened BD
▹ Para – low signal hemobilia
wall
channels
▸ Stones
▸ Liver & spleen parenchyma

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 MANAGEMENT

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 THANK YOU

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