Cirrhosis and Portal Hypertension
Related narrative: Distal Splenorenal (Warren) Shunt
In the U.S., alcoholic (Laennec's) cirrhosis is the most common cause of portal hypertension. Bleeding from esophagogastric varices occurs in 40% of patients with cirrhosis and accounts for one third of all deaths related to this disease. The fatality from a significant variceal bleeding episode is at least 50%. The natural history dictates that of those surviving, two thirds will bleed again, with the same risk of death with each episode.
Because of the significant comorbidities these patients carry (encephalopathy, malnutrition, ascites, coagulopathy), emergent or urgent surgery carries high risks. Endoscopic treatment with banding or sclerotherapy has emerged as a safe and effective palliative treatment of the acute bleeding episode with a 85% success rate in experienced hands. In those with significant bleeding of gastric varices, efficacy is lower and secondary mesures may be required. These include vasopressin, octreotide or propranolol (which all serve to lower portal pressure) which are 50-80% effective overall. A less commonly used therapy is balloon tamponade which is 75% effective but is associated with a 50% rebleed rate when released after 24 hours.
While the endoscopic modalities are very effective for the acute bleed and give significant time for resuscitation and correction of coagulopathy, their long term efficacy is poor with rebleed rates of 30% even for patients followed closely with chronic sclerotherapy. The only effective long term treatment other than liver transplant remains portal decompression by a surgically created portal systemic shunt. These shunts are generally divided into nonselective (total shunt) or selective (diverting blood from the gastroesophageal area while still maintaining most portal flow through the liver). As a group, all the shunt operations reduce the incidence of rebleeding to10%. However, this modality is associated with a high mortality (5-20% depending on the Child's class). With the nonselective shunts (portal caval side to side, or end to side) 30-50% will develop progressive encephalopathy. This is compared to a 15-25% encephalopathy in those treated with either sclerotherapy or a selective shunt. The distal splenorenal (Warren) shunt involves anastamosing the distal splenic vein into the left renal vein. This along with ligation of the coronary and right gastroepiploic vein diverts flow from the gastroesophageal varices through the short gastric veins into the newly created low pressure splenic vein to renal vein outflow. As opposed to total shunts, the Warren shunt not only does not improve ascites but may exacerbate it, thus limiting its use. The overall 5-year patency is 90%.
A nonshunt operation which devascularizes the esophagogastric system by way of esophageal transection and reanastamosis may be more expedient but is followed by a recurrent long term hemorrhage rate of 35-55%. The most effective nonshunt operation is the Sugiura procedure which entails extensive esophagogastric devascularization combined with esophageal transection/reanastamosis and splenectomy. Although popular in Japan, this procedure is less successful in North America, possibly due to the different etiology of cirrhosis.
TIPS (transjugular intrahepatic portasystemic shunt) is relatively safe and effective as a minimally invasive procedure to both control acute bleeding and prevent rebleeding. It has a lower morbidity and mortality compared to surgery, especially in Childís class C patients. The rebleed rate is considered low at 20% at 2 years. Currently, however, TIPS is mainly used as either a bridge to transplant or in nonsurgical candidates because of its poor long term patency (60% at 1 year, and 40% at 2 years).
Overall, after presenting with variceal bleeding only 20-60% of patients will be alive in 5 years, including those with a shunting procedure (45% 5-year survival). Orthotopic liver transplantation should be considered as a treatment for end-stage liver disease, and provides resolution to all its complications, including portal hypertensive-related bleeding. The long term survival which depends on patient selection and timing of the transplant will generally be between 50-75% at 5 years.
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