Portal hypertension

An elevation in portal pressure is most commonly found accompanying liver cirrhosis, although it may be present in patients with extra hepatic portal vein occlusion, intrahepatic veno-occlusive disease or occlusion of the main hepatic veins [Budd—Chiari syndrome (BCS)]. As portal hypertension produces no symptoms it is usually diagnosed following presentation with decompensated chronic liver disease and encephalopathy, ascites or variceal bleeding.

Management of bleeding varices

General resuscitation

Varices usually present with the acute onset of a large volume haematemesis, the lower oesophagus being the most common site for variceal bleeding. The diagnosis may be suspected if the patient is known to have liver cirrhosis but needs to be confirmed following initial resuscitation of the patient. This involves obtaining peripheral and subsequently central venous access whilst adequate blood is obtained (initially 10 units). Liver function tests will reveal underlying liver disease and a coagulation profile will reveal any underlying coagulopathy. Vitamin K is administered (10 mg intravenously), but correction of a coagulopathy will require the administration of fresh frozen plasma. An associated thrombocytopenia is usually secondary to hypersplenism due to cirrhosis and is treated if the platelet count falls below 50x109/litre. Variceal bleeding is often associated with hepatic encephalopathy, and endoscopic evaluation under these circumstances may require sedation and mechanical ventilation. Bronchial aspiration is a frequent complication of variceal bleeding.

If the rate of blood loss prohibits endoscopic evaluation a Sengstaken—Blakemore tube may be inserted to provide temporary haemostasis. This is shown diagrammatically in (Fig. 52.11.) Once inserted, the gastric balloon is inflated with 250 ml of air and retracted to the gastric fundus where the varices at the oesophagogastric junction are tamponaded by the subsequent inflation of the oesophageal balloon to a pressure of 40 mmHg. The two remaining channels allow gastric and oesophageal aspiration. An X-ray is used to confirm the position of the tube. The balloons should be temporarily deflated after 12 hours to prevent pressure necrosis of the oesophagus.

Drug treatment for variceal bleeding

Vasopressin has been the most extensively used drug for the initial control of variceal haemorrhage (20 units in 10 ml of 5 per cent dextrose intravenously over 10 minutes). Nitro­glycerin (40 micro gram/min) may be as effective. Octreotide, the long-acting somatostatin analogue, has - recently been evaluated and may have an important role.

Endoscopic treatment of varices

Initial treatment of oesophageal varices in most centres would be endoscopic sclerotherapy using 5 per cent ethanolamine oleate. Banding has recently produced encouraging results and is associated with a lower incidence of oesophageal ulceration. The majority of variceal bleeds will respond to a single course of sclerotherapy. An early re-bleed is less likely to be controlled by further sclerotherapy and a third bleed only rarely.

Transjugular intrahepatic portosystemic stent shunts (TIPSS)

The emergency management of variceal haemorrhage has been revolutionised by the introduction of TIPSS in the early 1990s. Over a short period it has become the main treatment of variceal haemorrhage which has not responded to drug treatment and sclerotherapy. The shunts are inserted under local anesthetic, analgesia and sedation using fluoroscopic guidance and Ultrasonography. Via the internal jugular vein and SVC a guide wire is inserted into a hepatic vein and through the hepatic parenchyma into a branch of the portal vein. The track through the parenchyma is then dilated with a balloon catheter to allow insertion of a metallic stent which is expanded once a satisfactory position is achieved (Fig. 52.12). A satisfactory drop in portal venous pressure is usually associated with good control of the variceal haemorrhage. The main early complication of this technique is perforation of the liver capsule which can be associated with fatal intraperitoneal haemorrhage. TIPSS occlusion may result in further variceal haemorrhage and occurs more commonly in patients with well-compensated liver disease and good synthetic function. Post shunt encephalopathy is the confusional state caused by the portal blood bypassing the detoxification of the liver. It occurs in about 40 per cent of patients, a similar incidence to that found after surgical shunts. If severe, the lumen of the TIPSS can be reduced by insertion of a smaller stent. The main contraindication to TIPSS is portal vein occlusion. Care must be exercised in the positioning of the TIPS S in patients who may be subsequently considered for liver transplantation. Proximal placement may interfere with the caval anastomosis and too distal a placement may result in difficulty in reconstructing portal inflow to the graft. The main long-term complication to TIPSS is stenosis of the shunt, which is common (approximately 50 per cent at 1 year) and may present as further variceal haemorrhage.

Surgical shunts for variceal haemorrhage

The increasing availability of liver transplantation and TIPSS has greatly reduced the indications for surgical shunts. It is rarely considered for the acute management of variceal haemorrhage as the morbidity and mortality under these circumstances are high. The main current indication for a surgical shunt is a patient with Child’s grade A cirrhosis in whom the initial bleed has been controlled by sclerotherapy. Long-term beta-blocker therapy and chronic sclerotherapy or banding are the main alternatives.

Surgical shunts are an effective method of preventing re-bleeding from oesophageal or gastric varices as they reduce the pressure in the portal circulation by diverting the blood into the low-pressure systemic circulation. Shunts may be divided into selective (e.g. splenorenal) and nonselective (e.g. porto­caval), the former attempting to preserve blood flow to the liver whilst decompressing the left side of the portal circulation responsible for giving rise to the oesophageal and gastric varices. Selective shunts may be associated with a lower incidence of portal systemic encephalopathy (PSE), a confu­sional state commonly found in patients with chronic liver disease who have undergone radiological or surgical porto­systemic shunts. The different types of surgical shunts are shown in Fig. 52.13. There is no evidence that prophylactic shunting is beneficial in patients with varices who have not bled.

Oesophageal stapled transection

This technique for the management of bleeding oesophageal varices utilised the circular stapling device initially employed for anastomosis of the rectum for stapling and resecting a ring of the lower oesophagus. As with surgical shunts in the acute situation, it was associated with a high perioperative mortality and has been largely abandoned in centres where TIPSS is available.

Management of varices secondary to splenic or portal vein thrombosis

Accurate angiography is an important aspect of the assess­ment of patients with suspected extrahepatic portal vein thrombosis. Therapeutic options are limited. Patients with oesophageal and/or gastric varices secondary to splenic or portal vein thrombosis can be effectively treated by splenectomy and gastro-oesophageal devascularisation, in which the blood supply to the greater and lesser curve of the stomach and lower oesophagus is divided. Splenic vein thrombosis may be seen secondary to chronic pancreatitis and portal vein thrombosis is a common late complication of liver cirrhosis.

Variceal bleeding and liver transplantation

The management of variceal bleeding should always take into account the possibility of liver transplantation where this is available. The patient’s age or associated medical condition may be a contraindication. TIPSS would be the preferred management for bleeds resistant to sclerotherapy as long as placement is optimal. Previous surgical shunts greatly increase the morbidity associated with OLT and probably the mortality.

Ascites

The accumulation of free peritoneal fluid is a common feature of advanced liver disease independent of the aetiology. The fluid accumulation is usually associated with abdominal discomfort and a dragging sensation. Development is usually insidious. The aetiology of the ascites must be established. Imaging by CT will confirm the ascites and demonstrate the irregular and shrunken nature of a cirrhotic liver and asso­ciated splenomegaly. Intravenous contrast enhancement will allow abdominal varices to be demonstrated and patency of the portal vein, portal vein thrombosis being a common pre­disposing factor to ascites in chronic liver disease. In patients without evidence of liver disease malignancy is a common cause and a possible primary site may also be established on CT. Aspiration of the peritoneal fluid allows analysis for cytology, an amylase estimation to exclude pancreatic ascites, and both microscopy and culture to exclude primary bacterial peritonitis and tuberculous peritonitis. The urinary 24-hour sodium excretion is used as a guide to treatment.

Treatment of ascites in chronic liver disease

The initial treatment is to restrict additional salt intake and commence diuretics using either spironolactone or frusemide. This should be combined with advice on avoiding any precipitating factors for impaired liver function, such as alcohol intake in patients with alcoholic cirrhosis. Patients on diuretics should be monitored for the development of hyponatraemia and hypokalaemia.

Abdominal paracentesis

Patients failing to respond to diuretic treatment may require repeated percutaneous aspiration of the ascites (abdominal paracentesis) combined with volume replacement using salt-poor or standard human albumin solution dependent on the serum sodium level. This is an unsatisfactory treatment but may provide some short-term symptomatic relief.

Liver transplantation for ascites

Diuretic-resistant ascites is an indication for liver transplantation if associated with a deterioration in liver function (rising bilirubin, dropping albumin, prolonged PT). The patient’s age, underlying aetiology of liver disease and associated medical problems will be the major factors determining suitability for liver transplantation. In those considered inappropriate for liver transplantation, management is aimed at symptomatic control of ascites.

Peritoneovenous shunting

The Le Veen shunt is designed for the relief of ascites due to chronic liver disease. One end of the silastic tube is inserted into the ascites within the peritoneal cavity and it is then tunnelled subcutaneously to the neck, where it is inserted under direct vision into the internal jugular vein and fed into the SVC. Owing to a one-way valve within the tubing, peritoneal fluid is drawn from the abdomen and drained to the cir­culation due to the lower pressure in the SVC in comparison to the abdomen during the respiratory cycle. Although often effective when combined with continued diuretic therapy they are prone to occlude and become displaced or infected. In an attempt to prevent the high occlusion rate a further development was the insertion of a chamber placed over the costal margin to allow digital pressure and evacuation of any debris within the peritoneo-venous shunt (Denver shunt).

TIPSS for ascites

The procedure and its limitations are as outlined above for the emergency treatment of bleeding varices secondary to portal hypertension. The use of TIPS S for ascites is for symptomatic relief, and the procedure is associated with considerable risks including death from haemorrhage, renal failure or heart failure. Post stent encephalopathy is common (about 40 per cent) and the majority of stents will stenos on long-term follow-up (approximately 50 per cent by- 1 year). Although a useful treatment modality, it has not become widely utilised because ascites is not life threatening. More encouraging results have been obtained in those with persistent chylothorax.