Portal HypertensionPortal Hypertension

Portal vein anatomyPortal vein anatomy

The portal vein is formed in front of IVC and The portal vein is formed in front of IVC and behind the neck of the pancreas behind the neck of the pancreas ( ( at the level at the level of 2of 2ndnd lumber vertebra lumber vertebra )) by union of the by union of the splenic & SMV.splenic & SMV.

It is 7-8 cm in length & contains no valves.It is 7-8 cm in length & contains no valves.

It courses in the lesser omentum posterior to It courses in the lesser omentum posterior to both the common hepatic artery & common both the common hepatic artery & common bile duct.bile duct.

Portal vein anatomyPortal vein anatomy

Causes of portal hypertensionCauses of portal hypertension

Increased resistance to flowIncreased resistance to flow A)A) Pre-hepatic (portal vein obstruction):Pre-hepatic (portal vein obstruction):

1- 1- congenital atresia or stenosis.congenital atresia or stenosis.

2-2- thrombosis of portal vein. thrombosis of portal vein.

3-3- thrombosis of splenic vein. thrombosis of splenic vein.

4-4- Extrinsic compression (e.g , tumor). Extrinsic compression (e.g , tumor).

Causes of portal hypertensionCauses of portal hypertension

B)B) Intra-hepatic:Intra-hepatic:

1-1- liver cirrhosis liver cirrhosis obstruction is obstruction is sinusoidal & post-sinusoidal.sinusoidal & post-sinusoidal.

2-2- bilharzial periportal fibrosis bilharzial periportal fibrosis obstruction is pre-sinusoidal.obstruction is pre-sinusoidal.

Causes of portal hypertensionCauses of portal hypertension

The causes of portal hypertension in cirrhotic The causes of portal hypertension in cirrhotic patients are:patients are:Diminution of the total vascular bed by Diminution of the total vascular bed by obliteration, distortion, & compression of obliteration, distortion, & compression of sinusoids.sinusoids.Compression of the tiny radicals of portal & Compression of the tiny radicals of portal & hepatic veins by excessive fibrosis.hepatic veins by excessive fibrosis.Development of multiple arteriovenous Development of multiple arteriovenous shunts between the branches of the hepatic shunts between the branches of the hepatic artery & portal vein. artery & portal vein.

Causes of portal hypertensionCauses of portal hypertension

C)C) Post-hepatic: Post-hepatic:1- 1- Budd-Chiari syndrome Budd-Chiari syndrome (( hepatic vein hepatic vein thrombosis thrombosis ) ) prominent prominent ascites ,hepatomegaly & abdominal pain.ascites ,hepatomegaly & abdominal pain.

2-2- Veno-occlusive disease. Veno-occlusive disease.

3- 3- Cardiac disease:Cardiac disease:

a-a- constrictive pericarditis. constrictive pericarditis.

b- b- valvular heart disease.valvular heart disease.

c- c- right heart failure.right heart failure.

Causes of portal hypertensionCauses of portal hypertension

Increased portal blood flowIncreased portal blood flow A)A) Arterial-portal venous fistula.Arterial-portal venous fistula.

B)B) Increased splenic flow:Increased splenic flow:

1-1- Banti’s syndrome Banti’s syndrome

( liver disease secondary to splenic disease, ( liver disease secondary to splenic disease, result from cirrhosis & other hepatic result from cirrhosis & other hepatic disorders)disorders)

2- 2- Splenomegaly (e.g tropical Splenomegaly (e.g tropical splenomegaly ,hematologic diseases). splenomegaly ,hematologic diseases).

Sequelae & Clinical picture Sequelae & Clinical picture

1-1- Porto-systemic collaterals:Porto-systemic collaterals:

In normal conditinos In normal conditinos collapsed.collapsed.

In portal hypertension In portal hypertension engorged engorged divert blood away from the portal divert blood away from the portal circulation.circulation.

Sequelae & Clinical pictureSequelae & Clinical picture

The important sites of these collaterals The important sites of these collaterals are:are:

a)a) At the lower end of oesophagusAt the lower end of oesophagus Oesophageal tributaries of Lt gastric vein (portal)Oesophageal tributaries of Lt gastric vein (portal)

Oesophageal tributaries of hemiazygous vein Oesophageal tributaries of hemiazygous vein (systemic).(systemic).

b)b) Around the umbilicusAround the umbilicus

Para umbilical vein (portal)Para umbilical vein (portal)

Superior & inferior epigastric veins (systemic)Superior & inferior epigastric veins (systemic)

Sequelae & Clinical pictureSequelae & Clinical picture

c)c) Lower rectum & analcanalLower rectum & analcanal Superior rectal vein (portal)Superior rectal vein (portal)

Middle & Inferior rectal veins (systemic)Middle & Inferior rectal veins (systemic)

d)d) At the back of the colonAt the back of the colon Rt & Lt colic veins (portal)Rt & Lt colic veins (portal)

Rt & Lt renal veins (systemic)Rt & Lt renal veins (systemic)

e)e) RetroperitoneumRetroperitoneum Tributaries of superior & inferior mesentric veins { Retzius } Tributaries of superior & inferior mesentric veins { Retzius }

(portal)(portal)

Posterior abdominal & subdiaphragmatic veins (systemic)Posterior abdominal & subdiaphragmatic veins (systemic)

Portal vein collateralsPortal vein collaterals

Sequelae & Clinical pictureSequelae & Clinical picture

2- 2- SplenomegalySplenomegalyThe most constant physical finding.The most constant physical finding.

In 80% of patients regardless the cause.In 80% of patients regardless the cause.

** In Bilharzial cases : In Bilharzial cases :

at 1at 1stst due to reticuloendothelial hyperplasia due to reticuloendothelial hyperplasia due to absorption of bilharsial toxins.due to absorption of bilharsial toxins.

With progress of portal hypertension With progress of portal hypertension due due to congestion.to congestion.

Sequelae & Clinical pictureSequelae & Clinical picture

3-3- Congestion of the whole GITCongestion of the whole GIT Leads to anorexia, dyspepsia, indigestion, and Leads to anorexia, dyspepsia, indigestion, and malabsorption.malabsorption.

4-4- Bleeding varices.Bleeding varices. 5-5-Ascites Ascites (multifactorial)(multifactorial)

Portal hypertension alone cannot cause ascites.Portal hypertension alone cannot cause ascites.Hypoalbuminaemia Hypoalbuminaemia below 3 gm below 3 gm//100 ml.100 ml.Salt &water retentionSalt &water retention high level of aldosterone, high level of aldosterone, oestrogens & anti-duretic hormone.oestrogens & anti-duretic hormone.Increased lymphatic transudation from liver surface. Increased lymphatic transudation from liver surface.

InvestigationsInvestigations

1.1. Assessment of liver function testsAssessment of liver function tests

(a)(a) Hypoalbuminaemia.Hypoalbuminaemia.

The liver is the only site of albumin synthesis.The liver is the only site of albumin synthesis.

(b)(b) ALT & AST are moderately raised.ALT & AST are moderately raised.

(c)(c) Prothrombin time and concentration Prothrombin time and concentration are disturbed. are disturbed.

This testThis test is the most sensitive liver function.is the most sensitive liver function.

InvestigationsInvestigations

2.2. Detection of oesophageal varices by:Detection of oesophageal varices by:

(a)(a) Fibreoptic upper endoscopyFibreoptic upper endoscopy

(b)(b) Barium swallow can visualize varices in Barium swallow can visualize varices in 90% of cases.90% of cases.

They appear as multiple, smooth, rounded filling They appear as multiple, smooth, rounded filling defects (honey-comb appearance)defects (honey-comb appearance)

(c)(c) Duplex scan can show dilated portal vein Duplex scan can show dilated portal vein and collateralsand collaterals..

Detection of oesophageal varicesDetection of oesophageal varices

InvestigationsInvestigations

3.3. Detection of splenic sequestration Detection of splenic sequestration and hypersplenism.and hypersplenism.(a)(a) Blood picture Blood picture anaemia, leucopenia, anaemia, leucopenia, thrombocytopenia or pancytopenia.thrombocytopenia or pancytopenia.(b)(b) Bone marrow examination Bone marrow examination hypercellularity. hypercellularity.

(c)(c) Radioactive isotope studies Radioactive isotope studies : : using the patients own RBCs tagged with using the patients own RBCs tagged with 5151Cr Cr

diminished half life of RBCs & increased diminished half life of RBCs & increased radioactivity over the spleen. radioactivity over the spleen.

InvestigationsInvestigations

4.4. Diagnosis of the aetiology of liver Diagnosis of the aetiology of liver disease is performed by:disease is performed by:

(a)(a) Immunological tests for hepatitis Immunological tests for hepatitis markers.markers.

(b)(b) Liver biopsy after assessment of Liver biopsy after assessment of prothrombin time and concentration.prothrombin time and concentration.

Child Pugh classificationChild Pugh classificationPointsPoints

11 22 33

Bilirubin (mg/dL)Bilirubin (mg/dL) < 2< 2 2 – 32 – 3 > 3> 3

Albumin (g/dL)Albumin (g/dL) > 3.5> 3.5 2.8 – 3.52.8 – 3.5 < 2.8< 2.8

Prothrombin time (seconds)Prothrombin time (seconds) 1 – 31 – 3 4 – 64 – 6 > 6> 6

AscitesAscites NoneNone SlightSlight ModerateModerate

EncephalopathyEncephalopathy NoneNone MinimalMinimal AdvancedAdvanced

Grade A, 5-6 points; Grade B, 7-9 points; Grade C, 10-15 points

TreatmentTreatment

Management of patients with actively bleeding Management of patients with actively bleeding oesophageal varicesoesophageal varices

1.1. Admission.Admission. The patient should be admitted to hospital. The patient should be admitted to hospital.

2.2. Resuscitation.Resuscitation. A wide bore cannula is inserted.A wide bore cannula is inserted. A blood sample is taken.A blood sample is taken. Restoration of blood volume should be rapid.Restoration of blood volume should be rapid.Overexpansion of the circulation should be avoided .Overexpansion of the circulation should be avoided .Morphine and Pethidine are contraindicated.Morphine and Pethidine are contraindicated.

TreatmentTreatment

3.3. Correct coagulopathy.Correct coagulopathy. Vitamin K is administered intravenously.Vitamin K is administered intravenously.

4.4. Prevent encephalopathy.Prevent encephalopathy. Blood in the intestine will be fermented to ammonia and other Blood in the intestine will be fermented to ammonia and other

nitrogenous products.nitrogenous products. Repeated enemas.Repeated enemas.

Oral lactulose. Oral lactulose. This is a disaccharide sugar, fermented by the intestinal flora This is a disaccharide sugar, fermented by the intestinal flora

lactic acid lactic acid combines with ammonia. combines with ammonia.

Neomycin 0.5 gm every 4 hours can reduce the bacterial flora.Neomycin 0.5 gm every 4 hours can reduce the bacterial flora.

TreatmentTreatment

SclerotherapySclerotherapy

Intra- or Para- Variceal.Intra- or Para- Variceal.

1-3 ml sclerosant (1-3 ml sclerosant (ethanolamine oleate).ethanolamine oleate).

Occludes venous channels.Occludes venous channels.

Multiple sessions (2 weekly).Multiple sessions (2 weekly).

Control bleeding in 80-95 %.Control bleeding in 80-95 %.

About 50% rebleed.About 50% rebleed.

30% complication rate.30% complication rate.

Endoscopic SclerotherapyEndoscopic Sclerotherapy

Intra-variceal Para-variceal

Complications of SclerotherapyComplications of Sclerotherapy

LOCALLOCAL

Ulceration.Ulceration.

Stricture.Stricture.

Perforation.Perforation.

Retrosternal discomfort Retrosternal discomfort

for few days.for few days.

SYSTEMICSYSTEMIC

FeverFever

PneumonitisPneumonitis

CNSCNS

TreatmentTreatment

Endoscopic BandingEndoscopic Banding

Occludes venous channelsOccludes venous channels

Sessions < sclerotherapySessions < sclerotherapy

Same results as sclerotherapySame results as sclerotherapy

complications vs sclerotherapycomplications vs sclerotherapy

Endoscopic treatment of choiceEndoscopic treatment of choice

Endoscopic BandingEndoscopic Banding

TreatmentTreatment

DrugsDrugs VasopressinVasopressin vasoconstriction of the splanchnic circulation. vasoconstriction of the splanchnic circulation.

DisadvantagesDisadvantages

colicky abdominal pains, & diarrhoea .colicky abdominal pains, & diarrhoea .anginal pains, so it is contraindicated in the elderly.anginal pains, so it is contraindicated in the elderly.

Produce temporary control of bleeding in about 80% of cases.Produce temporary control of bleeding in about 80% of cases.

To prolong its action it is combined with glycine To prolong its action it is combined with glycine ((GlypressinGlypressin))..

TreatmentTreatment

Somatostatin Somatostatin

lower the intravariceal pressure without significant lower the intravariceal pressure without significant side effects.side effects.

Initial bolus 100 microgramInitial bolus 100 microgram continuous infusion continuous infusion of 25 microgramof 25 microgram/ / h for 24 hs. h for 24 hs.

Beta blockareBeta blockare

bleeding by bleeding by cardiac output. cardiac output.

Does 20-60 mg bid Does 20-60 mg bid 25% 25% in HR. in HR.

Reduces 40% of bleeding episodesReduces 40% of bleeding episodes

Does Does notnot reduce mortality reduce mortality

TreatmentTreatment

Balloon tamponade by Sengestaken or Balloon tamponade by Sengestaken or Linton tube.Linton tube. The gastric balloon is inflated first by 200 ml The gastric balloon is inflated first by 200 ml of air, and pulled upwards to press the of air, and pulled upwards to press the gastric fundus.gastric fundus. If bleeding continues, the oesophageal If bleeding continues, the oesophageal balloon is inflated.balloon is inflated. The pressure in the oesophageal balloon The pressure in the oesophageal balloon should not exceed 40 mm Hg.should not exceed 40 mm Hg. This therapy is effective in controlling This therapy is effective in controlling bleeding in 80-90% of cases.bleeding in 80-90% of cases.

TreatmentTreatment

Disadvantages :Disadvantages : Discomfort to the patient.Discomfort to the patient.

The patient cannot swallow his salivaThe patient cannot swallow his saliva

Liability to cause oesophageal ulceration or Liability to cause oesophageal ulceration or stricture.stricture.

Once the tube is deflated, there is liability to Once the tube is deflated, there is liability to rebleeding in 60-80% of patients.rebleeding in 60-80% of patients.

Balloon tamponade is only used as a temporary Balloon tamponade is only used as a temporary measure before sclerotherapy or surgery.measure before sclerotherapy or surgery.

Balloon tamponadeBalloon tamponade

TreatmentTreatment

Emergency surgery.Emergency surgery.

If all the previous measures fail to stop bleeding, If all the previous measures fail to stop bleeding, surgery is recommended.surgery is recommended.

If the general condition of the patient is If the general condition of the patient is satisfactory satisfactory splenectomy, portoazygos splenectomy, portoazygos disconnection and stapling of the disconnection and stapling of the oesophagus.oesophagus.

If the patient is not very fit If the patient is not very fit stapling alone stapling alone can be performed.can be performed.

TreatmentTreatment

TTrans-juguJar rans-juguJar IIntra-hepatic ntra-hepatic PPorto-orto-SSystemic ystemic SShunt hunt ( ( TIPSS TIPSS ))

TreatmentTreatment

Indications for TIPSS:Indications for TIPSS:Refractory bleedingRefractory bleedingPrior to transplantPrior to transplantChild CChild CRefractory ascitesRefractory ascites

Main early complication:Main early complication:Perforation of liver capsule Perforation of liver capsule massive massive haemorrhage.haemorrhage.

TreatmentTreatment

Treatment of patients with history of Treatment of patients with history of bleeding oesophageal varices:bleeding oesophageal varices:

1.1. Repeated sclerotherapy until the varices Repeated sclerotherapy until the varices are obliterated is the first choice.are obliterated is the first choice.

2.2. Elective surgery is mainly indicated if Elective surgery is mainly indicated if sclerotherapy failed to stop recurrent sclerotherapy failed to stop recurrent attacks of bleeding provided that they are attacks of bleeding provided that they are fit.fit.

TreatmentTreatment

Operations for portal hypertensionOperations for portal hypertension

Shunt operations.Shunt operations.

The idea of these operations is to The idea of these operations is to lower the portal pressure by shunting lower the portal pressure by shunting the portal blood away from the liverthe portal blood away from the liver

Total shunt operationsTotal shunt operations

1-1- Porta-caval operationPorta-caval operation

End to side Side to sideEnd to side Side to side

Porta-caval operationPorta-caval operation

very efficient in lowering the portal very efficient in lowering the portal pressure pressure no bleeding occurs from the no bleeding occurs from the varices. varices.

disadvantages:disadvantages:

deprives the liver of portal blood flow deprives the liver of portal blood flow accelerates the onset of liver failure. accelerates the onset of liver failure.

Recurrent hepatic encephalopathy in 30-Recurrent hepatic encephalopathy in 30-50% of patients.50% of patients.

Proximal spleno-renal shuntProximal spleno-renal shunt

indicated if the portal vein indicated if the portal vein is thrombosed or if is thrombosed or if splenectormy is indicated splenectormy is indicated due to hypersplenism .due to hypersplenism .The incidence of The incidence of encephalopathy is less encephalopathy is less than after porta caval than after porta caval shunt. shunt. it is less effective In it is less effective In preventing further preventing further bleeding.bleeding. If the splenic vein is less If the splenic vein is less than 1 cm the anastmosis than 1 cm the anastmosis is liable to thrombosis.is liable to thrombosis.

Mesocaval Mesocaval ((DrapanasDrapanas)) shunt shunt

insertion of a a insertion of a a synthetic graft as synthetic graft as dacron, or autogenic dacron, or autogenic vein between the vein between the superior mesenteric superior mesenteric vein and inferior vein and inferior vena cava.vena cava.

The incidence of The incidence of thrombosis is highthrombosis is high

Selective shunt Selective shunt ((Warren shuntWarren shunt))

The Rt and Lt gastric The Rt and Lt gastric vessels are ligated.vessels are ligated. The proximal end of splenic The proximal end of splenic vein is ligated while the vein is ligated while the distal end is anastomosed to distal end is anastomosed to the left renal vein.the left renal vein. The short gastric veins are The short gastric veins are preserved and will preserved and will selectively decompress the selectively decompress the lower end of the lower end of the oesophagus.oesophagus. The incidence of The incidence of encephalopathy is low, and encephalopathy is low, and the liver functions remain the liver functions remain normal. normal.

Porta azygos disconnection Porta azygos disconnection operationsoperations

There are many techniques for There are many techniques for performing devascularization.performing devascularization.

Hassab Khairy operationHassab Khairy operation

splenectomy & ligation of the Rt and Lt splenectomy & ligation of the Rt and Lt gastric vessels, the short gastric gastric vessels, the short gastric vessels and the vascular arcade along vessels and the vascular arcade along the greater curvature of the stomach the greater curvature of the stomach leaving only the right gastroepiploic leaving only the right gastroepiploic vessels.vessels.

All vessels surrounding the lower 5-10 All vessels surrounding the lower 5-10 cm of the oesophagus are ligated.cm of the oesophagus are ligated.

There is no encephalopathy following There is no encephalopathy following this operation and the portal blood flow this operation and the portal blood flow is intact.is intact.

There is a low incidence of rebleeding There is a low incidence of rebleeding following the operation, but it can following the operation, but it can usually be controlled by sclerotherapy.usually be controlled by sclerotherapy.

Liver TransplantLiver Transplant

Indicated for liver failureIndicated for liver failure

NotNot for variceal bleeding. for variceal bleeding.

24% in USA die on waiting list24% in USA die on waiting list

Control of AscitesControl of Ascites

Sodium / Water Restriction.Sodium / Water Restriction.

Spironolactone.Spironolactone.

Loop Diuretic.Loop Diuretic.

Large Volume Large Volume Paracentesis. Paracentesis.

Peritoneal-Venous Shunt .Peritoneal-Venous Shunt .

TIPSSTIPSS

Special Cases of Special Cases of Portal HypertensionPortal Hypertension

Splenic Vein ThrombosisSplenic Vein Thrombosis

Etiology:Etiology: Pancreatitis - Acute or ChronicPancreatitis - Acute or Chronic

Pancreatic CarcinomaPancreatic Carcinoma

Hallmark: Hallmark:

Isolated Gastric VaricesIsolated Gastric Varices

Treatment:Treatment:

Splenectomy (Splenectomy (ifif bleeding) bleeding)

Portal Vein ThrombosisPortal Vein Thrombosis

Etiology:Etiology:

Congenital - Congenital - “Cavernous Transformation”“Cavernous Transformation”

Hallmark:Hallmark:

Normal Liver Function W/ VaricesNormal Liver Function W/ Varices

Treatment:Treatment:

Endo Tx OR DSRSEndo Tx OR DSRS

Budd-Chiari SyndromeBudd-Chiari SyndromeEtiologyEtiology

Hypercoagulable: Hypercoagulable: Estrogens, XRT, Myeloprolif, PNHEstrogens, XRT, Myeloprolif, PNH

IVC Occlusion: IVC Occlusion: RA Myxoma, Pericarditis,RA Myxoma, Pericarditis,

Liver MassLiver Mass

High Dose ChemoTxHigh Dose ChemoTx

Presentation: Classic TriadPresentation: Classic Triad

Abdominal PainAbdominal Pain

Ascites Ascites

HepatomegalyHepatomegaly

Budd-Chiari SyndromeBudd-Chiari Syndrome

DiagnosisDiagnosis

– U/S, CT, AngioU/S, CT, Angio

TreatmentTreatment

– NOT a static diseaseNOT a static disease

– If NO necrosis If NO necrosis Symptomatic Tx Symptomatic Tx

– If necrosis If necrosis Shunt (PCS or MAS) Shunt (PCS or MAS) oror Transplant Transplant