Top Banner
Massimo Primignani U.O. Gastroenterologia 1 Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico Milano 19th AISF Pre-Meeting Course “Vascular Diseases of the Liver” Roma, 22 Febbraio 2017
53

19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Feb 17, 2019

Download

Documents

LêAnh
Welcome message from author
This document is posted to help you gain knowledge. Please leave a comment to let me know what you think about it! Share it to your friends and learn new things together.
Transcript
Page 1: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Massimo Primignani

U.O. Gastroenterologia 1

Fondazione IRCCS Cà Granda

Ospedale Maggiore Policlinico

Milano

19th AISF Pre-Meeting Course

“Vascular Diseases of the Liver”

Roma, 22 Febbraio 2017

Page 2: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Massimo Primignani

U.O. Gastroenterologia 1

Fondazione IRCCS Cà Granda

Ospedale Maggiore Policlinico

Milano

Natural history and management of

complications of chronic extrahepatic

portal vein obstruction

Page 3: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Massimo PrimignaniU.O. Gastroenterologia 1

Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milano

Il sottoscritto dichiara di non aver avuto negli ultimi 12 mesi conflitto d’interesse in relazione a questa presentazione

e che la presentazione non contiene/contiene discussione

di farmaci in studio o ad uso off-label

Page 4: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Baveno VI

SESSION 6 – Vascular diseases of the liver in cirrhotic and non-cirrhotic portal

hypertension: coagulation, anti-coagulation, anti-platelet drugs

CONSENSUS STATEMENTS

EXTRA-HEPATIC PORTAL VEIN OBSTRUCTION (EHPVO) – DEFINITION

• EHPVO is the obstruction of the extra-hepatic portalvein, with or without involvement of the intra-hepaticportal veins or other segments of the splanchnicvenous axis. It does not include isolated thrombosisof splenic vein or superior mesenteric vein (SMV).

• EHPVO is characterized by features of recentthrombosis or of portal hypertension with portalcavernoma as a sequel of portal vein obstruction.

Page 5: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Baveno VI

SESSION 6 – Vascular diseases of the liver in cirrhotic and non-cirrhotic portal

hypertension: coagulation, anti-coagulation, anti-platelet drugs

CONSENSUS STATEMENTS

EXTRA-HEPATIC PORTAL VEIN OBSTRUCTION (EHPVO) – DEFINITION (II)

• Presence of cirrhosis, other underlying liver diseases(i.e. non-cirrhotic portal hypertension) and/ormalignancy should be ruled out.

• EHPVO in those situations should be considered asdifferent entities.

Page 6: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Portal Cavernoma

Porto-porto

collateral circulation

Dilated hepatic

artery

Bile Ducts

Venous Drainage

of CBD

Adapted from D. Lebrec

PV

Page 7: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

CEMRI image of a portal vein cavernoma

Page 8: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Chronic EHPVO-Clinical Features

Well tolerated variceal bleed

Splenomegaly

Hypersplenism

Growth retardation in children

Jaundice, ascites rare

Webb and Sherlock 1979, Mitra 1987, Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari 2003,

Page 9: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

EHPVO: Natural History

Esophageal variceal bleed, repeated, well tolerated

Gastric and anorectal varices - bleed

Slow liver dysfunction in some

Ascites – spontaneous (5-10%)

Portal biliopathy - jaundice

Growth retardation, delayed sexual characters

Webb and Sherlock 1979, Chawla 1990, Yachha, 1997, Mishra 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari 2003,

Page 10: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

EHPVO- Management of complications

I. Prophylaxis bleeding/rebleeding

II. Portal Biliopathy

III. Prevention of thrombosis extention and relapse: Anticoagulant therapy

IV. Chronic EHPVO in children

Page 11: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

EHPVO- Management of complications

I. Prophylaxis of bleeding/rebleeding

II. Portal Biliopathy

III. Prevention of thrombosis extentionand relapse: Anticoagulant therapy

IV. Chronic EHPVO in children

Page 12: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Primary prevention of bleeding

• No RCTs compared non-selective beta-blockers vs.endoscopic band ligation in EPVHO.

• In this scenario, as well as in the context of the acutebleeding and of secondary prophylaxis, Baveno VIrecommends to follow the guidelines regarding PH incirrhosis.

Page 13: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Baveno VI

SESSION 6 – Vascular diseases of the liver in cirrhotic and non-cirrhotic portal

hypertension: coagulation, anti-coagulation, anti-platelet drugs

CONSENSUS STATEMENTS

TREATMENT OF PORTAL HYPERTENSION IN EHPVO

•There is insufficient data on whether beta-blockersor endoscopic therapy should be preferred forprimary prophylaxis. Thus, guidelines for cirrhosisshould be applied (5;D).

Page 14: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Noronha Ferreira Hepatology 2016

• 178 patients with chronic PVT.

• Median follow-up : 49 (1-598) months.

• Variceal bleeding : initial manifestation in 15%

• Initial endoscopy in the remaining 151 :

no varices in 34%

small esophageal varices in 19%

large esophageal varices in 40%

gastric varices without LEVs in 7%

Page 15: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Actuarial probability of development of varices

probability of developing them was

2%, 22%, and 22% at 1, 3, and 5

years

2%, 22%, and 22% at 1, 3, and 5 years

Noronha Ferreira Hepatology 2016

Page 16: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Actuarial probability of enlargement of varices

13%, 40%, and 54% at 1, 3, and 5 years

Noronha Ferreira Hepatology 2016

Page 17: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Has the patient developed PVT-related complications?

• In chronic EHPVO more than two third of the subjects have gastro-esophageal varices at screening endoscopy.

• In patients without varices at baseline, varices developin 22% after 3 years

perform follow-up endoscopy

Noronha Ferreira Hepatology 2016, Condat B Gastroenterology. 2001

Page 18: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Actuarial probability of bleeding in patients on

primary prophylaxis

9%, 20%, and 32% at 1, 3, and 5 years

Noronha Ferreira Hepatology 2016

Median dose of NSBB: 100 mg (10-240)

Page 19: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Actuarial probability of rebleeding

9%, 20%, and 32% at 1, 3, and 5

Noronha Ferreira Hepatology 2016

Page 20: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Course of varices in chronic noncirrhotic, nontumoral PVT is similar to that in cirrhosis

Using the same therapeutic approach as for cirrhosis is associated with a low risk of bleeding and death

• baseline endoscopy at diagnosis of PV

• subsequent endoscopies at 2-year or 3-year intervals in patients with no EVs or small EVs at baseline

• beta-blockers or endoscopic band ligation (EBL) as a primary prophylaxis

• drug plus EBL to treat variceal bleeding and prevent rebleeding

Noronha Ferreira Hepatology 2016, Condat B Gastroenterology. 2001

Page 21: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Portal venogram following transjugular

puncture of the portal vein shows varices

and contrast accumulation within the

portal cavernoma

Following TIPS placement variceal filling is

reduced and contrast accumulation within the

cavernoma is no longer seen

Alimentary Pharmacology and Therapeutics, 2006

Page 22: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

EHPVO- Management of complications

I. Prophylaxis variceal bleeding

II. Portal Biliopathy

III. Prevention of thrombosis extention and relapse: Anticoagulant therapy

IV. Chronic EHPVO in children

Page 23: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Portal cholangiopathy

• Abnormalities of the biliary system and gallbladder due

to ischemia at the time of portal vein thrombosis and

local ischemia due to local wall compression by the

peribiliary collaterals.

• >80% of patients with portal cavernoma develop some

degree of portal cholangiopathy, but just a minority

develops symptoms during the follow up:

- right abdominal pain

- pruritus, jaundice

- gallbladder stones

- cholangitis

Dhiman RK et al. Gut. 2007

Page 24: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Portal cholangiopathy – clinical presentation

Asymptomatic: no biliary symptoms despite

the presence of biliary abnormalities on ERC or

MRC

With symptoms:

- Chronic cholestasis, likely to be caused by

biliary stricture

- Biliary pain or acute cholangitis, likely to be

caused by biliary stones (5-8%)

- Secondary Biliary Cirrhosis

Page 25: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

MRCP classification of biliary tree abnormality with clinical correlates

I. Minimal irregularities or angulation of the biliary tree

II. Indentations or strictures without dilatation of the biliary

tree;

III. Strictures with dilatation (intrahepatic duct >4 mm or

extrahepatic duct >7 mm)

Page 26: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

MRCP classification of biliary tree abnormality with clinical correlates

I. Minimal irregularities or angulation of the biliary tree

II. Indentations or strictures without dilatation of the biliary

tree;

III. Strictures with dilatation (intrahepatic duct >4 mm or

extrahepatic duct >7 mm)

Page 27: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

ERC in a patient with

EHPVO showing

irregular intrahepatic

ducts and indentations

of common hepatic and

bile duct

I. Minimal irregularities or angulation of the biliary tree

Page 28: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

MRCP classification of biliary tree abnormality with clinical correlates

I. Minimal irregularities or angulation of the biliary tree

II. Indentations or strictures without dilatation of the biliary

tree;

III. Strictures with dilatation (intrahepatic duct >4 mm or

extrahepatic duct >7 mm)

Page 29: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

ERC and MR cholangiography in EHPVO: strictures on common hepatic and

intrahepatic bile ducts with smooth narrowing of middle common bile duct

II. Indentations or strictures without dilatation of the biliary tree

Page 30: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

MRCP classification of biliary tree abnormality with clinical correlates

I. Minimal irregularities or angulation of the biliary tree

II. Indentations or strictures without dilatation of the biliary

tree;

III. Strictures with dilatation (intrahepatic duct >4 mm or

extrahepatic duct >7 mm)

Page 31: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

ERC: defects in the CBD suggestive

of stones, along with dilatation of

intrahepatic biliary ducts

MRC in the same patient : multiple filling

defects, due to choledochal varices in the

intrahepatic and extrahepatic biliary system.

III. Biliary strictures with dilatation

Page 32: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

MR cholangiography and portography

• MR cholangiography and portography is non-invasive and can demonstrate bile duct changes and portal collaterals simultaneously

• MR cholangiography with MR portography is superior to ERC in delineating changes in intrahepatic biliary ducts and in differentiating choledochal varices from CBD stones

• MR portography delineates the entire splenoportal axis and portal collaterals, which helps to assess the possibility of shunt surgery.

Page 33: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Treatment of Portal Colangiopathy

• Asymptomatic patients do not require any treatment

• Treatment of symptoms is focused on the management of portal hypertension and relief of obstructive jaundice

Page 34: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Endoscopic treatment

• endoscopic sphincterotomy

• stone extraction

• mechanical lithotripsy

• biliary stricture dilatation with/ without stent

or nasobiliary drain placement.

Page 35: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Adverse events of endoscopic treatment

• Bleeding from bile duct varices (haemobilia) during

biliary dilatation.

• Bleeding at sphincterotomy, due to venous collaterals in

ampullary and juxtapapillary region.

• Filling defects within the biliary system could be due to

stones or choledochal varices:

(varices do not move with the balloon catheter at ERC)

Prefer balloon catheter over Dormia basket for removing CBD stones after sphincterotomy to minimise

the risk of haemobilia.

Page 36: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Surgical treatment - 1

• High morbidity/mortality of bilioenteric anastomosis as the collateral vessels around the bile ducts make the surgical dissection difficult

• Patients should undergo portosystemic shunt surgery before any bilioenteric anastomosis is planned.

• Most patients improve with shunt surgery and may not require further surgery

Page 37: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Surgical treatment - 2

• Shunt surgery may be indicated in patients with

previous bleeding and hypersplenism resulting

in significant anaemia and/or thrombocytopenia.

• TIPS also reverses the biliary changes in these

patients: useful in selected patients.

• OLT is the only option in patients with

intractable disease, when both endoscopic and

surgical treatments have failed or are not

feasible.

Page 38: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Endoscopy or surgery?

• Surgical treatment is preferred for dominant biliary

strictures with a shuntable vein as it provides long-

lasting relief of symptoms.

• Endoscopic treatment is preferred for patients with CBD

stones, cholangitis or for patients with dominant biliary

stricture, but without a shuntable vein.

- risk of secondary biliary cirrhosis (recurrent stent blockage,

cholangitis)

- scheduled biliary stent exchange every 4–6 months

Page 39: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

EHPVO- Management of complications

I. Prophylaxis variceal bleeding

II. Portal Biliopathy

III. Prevention of thrombosis extention and relapse: Anticoagulant therapy

IV. Chronic EHPVO in children

Page 40: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Baveno VI

SESSION 6 – Vascular diseases of the liver in cirrhotic and non-cirrhotic portal

hypertension: coagulation, anti-coagulation, anti-platelet drugs

CONSENSUS STATEMENTS

ANTICOAGULATION IN CHRONIC EHPVO• In patients without underlying prothrombotic disease,

there is scarce information to recommend anticoagulant therapy (5;D).

• In patients with a persistent documented prothrombotic state, recurrent thrombosis or intestinal infarction long-term anticoagulant therapy is recommended (3b;B).

• Anticoagulation should be started after adequate portal hypertensive bleeding prophylaxis has been initiated (5;D).

Page 41: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Algorithm of the use of anticoagulation in the management of EHPVO

Page 42: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

EHPVO- Management of complications

I. Prophylaxis variceal bleeding

II. Portal Biliopathy

III. Prevention of thrombosis extention and relapse: Anticoagulant therapy

IV. Chronic EHPVO in children

Page 43: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Pediatric EHPVO

EHPVO is the commonest cause of varices and upper gastrointestinal bleeding in children

Umbilical vein catheterization or omphalitis are the most frequently reported causes of pediatric EHPVO

Meso-Rex bypass is a surgery option in children with complication of chronic EHPVO

Page 44: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Embryologic origins of the Rex Recessus

Meso-Rex Bypass

Page 45: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Classification of patency of the intrahepatic portal vein in children with portal vein cavernoma

Partial obstruction

of the left liver

thrombosis of the

left liver

thrombosis of

the right liver

extensive thrombosis

of the intrahepatic

portal veins

Complete patency

of the intrahepatic

portal veins

including the RR

Page 46: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Transjugular retrograde wedged

portography indicating complete

patency

Intraoperative portal venography

showing complete patency

Highest sensitivity and specificity

Page 47: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Meso-Rex Bypass, when feasible, is the primary approach in children with EHPVO with evidence of cavernoma

Criteria for ensuring a success rate of>90%.

• “A” type anatomy confirmed by wedge two dimensional portography

• Normal HVPG determined at the time of portography and/or liver

biopsy revealing no fibrosis or underlying liver disease that would

impact portal flow

• Patent superior mesenteric vein and splenic vein without thrombosis

• Bilateral internal jugular vein patency

• Hematological evaluation excluding an intrinsic prothrombotic

condition that would not be reversed by MRB

• Body weight >8 kg

• Echocardiography excluding significant cardiovascular abnormalities

and significant pulmonary hypertension

Page 48: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Resolution of biliopathy after reconstitution of

portal blood flow by MRB

Page 49: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Increase in liver mass and decrease in spleen

size after MRB

Page 50: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Resolution of hepatic nodules after

Meso-Rex Bypass

Page 51: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Baveno VI

SESSION 6 – Vascular diseases of the liver in cirrhotic and non-cirrhotic portal

hypertension: coagulation, anti-coagulation, anti-platelet drugs

CONSENSUS STATEMENTS

TREATMENT OF PORTAL HYPERTENSION IN EHPVO

Meso-Rex by-pass should be considered in all childrenwith complications of chronic EHPVO, who should bereferred to centers with experience in treating thiscondition (5;D).

Page 52: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari

Alternatives to Meso-Rex Bypass

• Endoscopic Variceal Ligation

• Endoscopic variceal sclerotherapy (dictated by patient size restrictions)

• Distal splenorenal shunt

Page 53: 19th AISF Pre-Meeting Course · 19th AISF Pre-Meeting Course ... Fondazione IRCCS Cà Granda Ospedale Maggiore ... Yachha 1997, Kato 2002, Sarin 2002, Condat and Valla, 2003, Rangari