1. Portal hypertension
Usually caused by increased resistance to portal venous blood flow
The obstruction is prehepatic, hepatic or posthepatic
The normal pressure in the portal vein varies from 5 to 15 cmH2O.
When the portal venous pressure is consistently raised above 25 cmH2O may → serious clinical consequences
Portal hypertension

2. Portal hypertension

3. Anatomy of the portal venous system

4. Causes of portal hypertension
Obstruction to portal flow
Increased blood flow (rare)
Prehepatic
Congenital atresia of the portal vein
Portal vein thrombosis
Neonatal sepsis
Pyelophlebitis
Trauma, Tumour
Extrinsic compression of the portal vein
Pancreatic disease
Lymphadenopathy
Biliary tract tumours
Intrahepatic
Cirrhosis
Schistosomiasis
Posthepatic
Budd-Chiari syndrome
Constrictive pericarditis
Arteriovenous fistula
Increased splenic blood flow in hypersplenism
Table 19.1 Causes of portal hypertension

5. Portal hypertension Prehepatic
Portal vein thrombosis
Rare cause
Commonly due to neonatal umbilical sepsis, though the effects may manifest after many years
Portal hypertension

6. Portal hypertension Hepatic - Liver cirrhosis
Cirrhosis of the liver
The commonest cause
Results from chronic liver disease
Characterized by liver cell damage, fibrosis & nodular regeneration
Micronodular cirrhosis
even distribution of nodules a few millimeters in diameter
Macronodular cirrhosis
the nodules vary in size
usual in end-stage cirrhosis
The fibrosis obstructs portal venous return & → portal hypertension
Portal hypertension

7. Portal hypertension Hepatic - Liver cirrhosis
Alcohol
The commonest etiological factor in western countries
Abnormal resistance is predominantly postsinusoidal (increase in wedge hepatic venous pressure)
The hepatic veins become distorted by regenerative nodules, there is narrowing of the central veins by centrilobular collagen deposition, and swelling of the hepatocytes encroaches on the sinusoidal lumen
Schistosomiasis
Due to Bilharzia mansonii is a common cause in North Africa, Middle East
Granulomas from parasitic involvement are seen in the portal triads
Hypertension is presinusoidal
Portal hypertension

8. Portal hypertension Hepatic - Liver cirrhosis
Other causes
Chronic active hepatitis
Primary & secondary biliary cirrhosis
Cryptogenic cirrhosis (obscure cause)
Portal hypertension

9. Portal hypertension Posthepatic
Rare
Due to spontaneous thrombosis of the hepatic veins
Associated with
Neoplasia
Oral contraceptive agents
Polycythaemia
Presence of abnormal coagulants in the blood
The resulting Budd-Chiari syndrome is characterized by portal hypertension, liver failure & gross ascites
Portal hypertension

10. Abdominal radiograph demonstrating calcified & thrombosed portal & splenic veins

11. Effects of portal hypertension
Gradual chronic occlusion of the portal venous system →
Collateral develop between the portal & systemic venous circulations
The most important consequence of shunting is the development of varices in the submucosal plexus of veins in lower esophagus & gastric fundus.
The esophageal varices may then rupture → acute massive gastrointestinal bleeding (occurs in 40% of patients with cirrhosis).
Initial episode of variceal hemorrhage is fatal in ⅓ of patients.
Bleeding from retroperitoneal & periumbilical collaterals.
Collaterals may develop & → bleeding at site of stomas.
Anorectal varices are found at proctoscopy but rarely cause bleeding.
Progressive enlargement of the spleen due to vascular engorgement & associated hypertrophy
Portal hypertension

12. Sites of portosystemic shunting
Fig The portal venous system. Sites of portosystemic shunting are marked Retroperitoneal communications also exist.

13. Effects of portal hypertension
Hematological consequences
Anemia, thrombocytopenia & leukopenia (hypersplenism)
Ascites
Increased formation of hepatic & splanchnic lymph
Hypoalbuminaemia
Retention of salt & water
Increased aldosterone & antidiuretic hormone
Portosystemic encephalopathy due to
Increased level of toxins (ammonia) in systemic circulation
Develop where there are large spontaneous or surgically created portosystemic shunts
Gastrointestinal hemorrhage increases absorption of nitrogenous products → encephalopathy
Portal hypertension

14. Clinical presentation
Patients with cirrhosis
Anorexia
Generalized malaise
Weight loss
Clinical manifestations of liver disease
Hepatosplenomegaly
Ascites
Jaundice
Spider naevi
Serum bilirubin may ↑
Serum albumin ↓
Anaemia
Leukocyte count ↑ or ↓ (hypersplenism)
Prothrombin time & other indices of clotting may be abnormal
Portal hypertension

15. Gross splenomegaly secondary to portal hypertension
Abdominal radiograph demonstrating gross splenomegaly secondary to portal hypertension.

16. Modified Child's grading system (Clinical & biochemical parameters)
Points scored
Criterion 1 2 3
Encephalopathy
Ascites
Bilirubin (µmol/L)
Albumin (g/L)
Prothrombin ratio
None
< 35
> 35
< 1.4
Minimal
Slight
35-50
28-35
Marked
Moderate
> 50
<28
> 2.0
Table 19.2 Assessment of patients with portal hypertension by a modification of Child's grading system
Grade A = 5-6 points; grade 6 = 7-8 points; grade C =10-15 points.

17. Portal hypertension Acute variceal bleeding
Patients presenting with acute upper gastrointestinal bleeding are carefully examined for evidence of chronic liver disease.
Distended collateral veins around the umbilicus ('caput medusae‘).
Slurring of speech, a flapping tremor or dysarthria may point to encephalopathy
may be precipitated or intensified by accumulation of blood in gastrointestinal tract
The key investigation during an episode of active bleeding is endoscopy
Allows detection of varices & defines whether they are or have been site of bleeding.
Peptic ulcer & gastritis are common complaints (in 20% of patients with varices).
Portal hypertension

18. Management of bleeding esophageal varices
Active resuscitation
Group & cross-match blood
Assessment of coagulation status
Prothrombin time
Platelet count
Establish i.v. infusion line(s)
Monitor
pulse , blood pressure
hourly urine output , central venous pressure
Urgent endoscopy

19. Management of bleeding esophageal varices
Control of bleeding
Tamponade (Minnesota tube) or injection sclerotherapy
Pharmacological measures (e.g. vasopressin/somatostatin)
Treatment of hepatocellular decompensation
Treatment/prevention of portosystemic encephalopathy
Portal hypertension

20. Management of bleeding esophageal varices
Prevention of further bleeding from varices
Injection sclerotherapy
Stapled esophagogastric junction
Portosystemic shunting/TIPSS
Liver transplantation
Portal hypertension

21. Active resuscitation
Large volumes of blood may be lost rapidly and the aim is to replace blood loss quickly.
Fresh blood is preferred for transfusion purposes
Fresh-frozen plasma (FFP)
Platelet transfusion
Portal hypertension

22. Rarely, bleeding occurs from varices in gastric fundus.
Endoscopy
Performed at the earliest opportunity, and in patients threatened by massive bleeding, active resuscitation is instituted and continued in the endoscopy suite.
The tortuous varices are usually in three columns & most prominent in the lower third of the esophagus.
If varices are the source of blood loss, this usually occurs from the lowest few centimeters of esophagus.
Rarely, bleeding occurs from varices in gastric fundus.
Portal hypertension

23. Medical agents to lower portal venous pressure & arrest bleeding
Control of bleeding
Medical agents to lower portal venous pressure & arrest bleeding
synthetic form of somatostatin, octreotide
If variceal hemorrhage is apparent at the endoscopy
injection of a sclerosant (ethanolamine)
application of bands
If hemorrhage is torrential & prevents direct injection, balloon tamponade may be used to stop the bleeding.
Minnesota tube (four-lumen)
Sengstaken-Blakemore tube (three-lumen)
Portal hypertension

24. Control of bleeding Minnesota tube (four lumen)
Aspiration of gastric contents
Inflation of a gastric balloon with 150 ml of water with radio-opaque dye (Hypaque) to be checked radiologically
compresses the gastric fundus & EGJ → reducing flow of blood into esophageal varices
Inflation of esophageal balloon with air to a pressure of 40 mmHg (direct pressure to the esophageal varices)
Aspiration of esophagus & pharynx above esophageal balloon, reducing the risk of aspiration pneumonitis
Traction is applied to the Minnesota tube by pulling the gastric balloon up against EGJ & taping the tube as it emerges from the angle of the mouth.
Pharynx & stomach are aspirated every minute.
Arrests bleeding from varices in over 90% of patients
The tube is not left in place for more than hours for fear of causing esophageal necrosis.

25. Esophageal tamponade using a Minnesota tube

26. Tamponade should be regarded as a holding measure
Control of bleeding
Tamponade should be regarded as a holding measure
Further resuscitation & treatment of hepatic decompensation.
More definitive measures are used to prevent further variceal bleeding
two-thirds rebleed while still in hospital
90% rebleed within a year
Portal hypertension

27. Resuscitation & treatment of hepatocellular decompensation
Control of variceal bleeding allows blood loss to be made good & permits a full assessment of coagulopathy.
Blood may be evacuated from the gut by a bowel washout to reduce the risk of portosystemic encephalopathy.
Lactulose (15-30 ml tid) to reduce bacterial degradation of blood in the gut lumen & further reduce risk of encephalopathy.
Esophageal varices due to liver disease frequently have major defects in intrinsic & extrinsic clotting systems.
Vitamin K to aid restoration of the extrinsic system
FFP, factor concentrates & platelet transfusion may all be required to cover specific procedures such as sclerotherapy.
Transfusion measures have transient effects on blood coagulation
Ultimate coagulation status depends upon restoration of hepatic function.
Portal hypertension

28. Prevention of further bleeding Injection sclerotherapy
Carried out by fibreoptic endoscopy.
Injection is repeated at weekly or fortnightly intervals until the varices are completely sclerosed.
Following complete ablation, fibreoptic examination is repeated periodically & any recurrent varices are injected.
Excessive or too frequent injection may be complicated by ulceration & necrosis, sometimes with a fatal result.
Sclerosant can be injected directly into the varix or into the surrounding mucosa.
Reduced the number of patients undergoing surgery
Most successful in patients with well-preserved liver function.
Improves long-term survival?.
Portal hypertension

29. Prevention of further bleeding Endoscopic banding
Used to occlude varices at esophagogastric junction.
The reduced risk of esophageal ulceration & perforation has resulted in this technique being favoured in many centres.
Portal hypertension

30. Prevention of further bleeding Surgical disconnection
Rarely used in management of variceal hemorrhage.
The gastric veins & short gastric veins are ligated and the distal esophagus is transected & reanastomosed just above the cardia using a stapling gun.
Stapled esophageal transection occludes flow into varices.
Technically difficult in patients who have been submitted to repeated injection sclerotherapy.
Considerable morbidity & mortality when employed as a last resort in the emergency situation.

31. Esophageal stapling
Esophageal stapling The gun is inserted through an anterior gastrostomy. A ligature is tied just above the cardia, invaginating a flange of esophageal wall between the two parts of the gun. Inset: the gun has been fired, simultaneously resecting a full-thickness ring of esophageal wall and anastomosing the cut ends with staples.

32. Prevention of further bleeding Emergency portosystemic shunting
High mortality.
Elective portosystemic shunting is still used to decompress portal system & reduce risk of further variceal hemorrhage, but portosystemic encephalopathy can be troublesome.
Operation is rarely considered in patients whose condition is complicated by jaundice, ascites or encephalopathy, and where there is a clear indication for liver transplantation.
Portal hypertension

33. Types of shunt procedure The distal splenorenal (Warren) shunt
Selectively decompresses lower esophagus & upper stomach
Maintains liver blood flow
The incidence of encephalopathy is lower than after other shunt procedures
Portal hypertension

34. Relatively safe (no GA & no laparotomy).
Types of shunt procedure Transjugular intra hepatic portosystemic stent shunting (TIPSS)
In this procedure a metal stent is inserted via the transjugular route using a guide-wire passed through the hepatic vein to the intrahepatic branches of the portal vein.
Relatively safe (no GA & no laparotomy).
The risk of encephalopathy is similar to that of a surgical portosystemic shunt.
Now considered routinely before surgical intervention in both the acute and the elective setting.
Portal hypertension

35. Types of portosystemic shunt
Portal vein
IVC
Superior mesenteric vein
Normal
End-to-side portocaval shunt
Mesocaval shunt (Dacron graft interposition)
Distal splenorenal shunt
Fig Types of portosystemic shunt.

36. Ascites
Ascites can be controlled by bed rest, salt & water restriction & a diuretic such as the aldosterone-inhibitor spironolactone.
If refractory, ascites can be treated by inserting a peritoneojugular (LeVeen) shunt, which allows one-way flow between the peritoneum & jugular vein.
Portal hypertension

37. Peritoneovenous (Le Veen) shunt to relieve ascites