Portal hypertension management

Summary Injection sclerotherapy is the mainstay of treatment for acute variceal bleeding and for long-term management after a variceal bleed. In those few patients in whom sclerotherapy fails to control acute bleeding, either a surgical shunt or a simple esophageal transection is recommended. A surgical shunt or a more extensive esophagogastric evascularization and transection operation is advocated for the failures of long-term sclerotherapy management. The role of pharmacological agents in acute variceal bleed management remains in question, and the use of propranolol in long-term management, either as an alternative to sclerotherapy or in combination with sclerotherapy, is controversial. The definitive roles of the newly described variceal banding and transjugular intrahepatic portosystemic shunts (TIPS) procedures have yet to be established. All patients presenting with end-stage liver disease and esophageal variceal bleeding should be evaluated for a liver transplant, although few will qualify. A possible future transplant should be kept in mind when emergency treatment is planned. Any form of prophylactic therapy for patients with esophageal varices that have not yet bled will remain unjustified until those patients at high risk of a first variceal bleed can be identified. The gastric mucosal lesion, portal hypertensive gastropathy, has been underdiagnosed in the past. Although bleeding does occur, it is seldom a major clinical problem. When necessary, bleeding can be controlled by propranolol or a surgical shunt.     

The surgeon''s role in the management of portal hypertension.

Patients with portal hypertension are referred to surgeons for several reasons. These include the management of continued active variceal bleeding; therapy after a variceal bleed to prevent further recurrent bleeds; consideration for prophylactic surgical therapy to prevent the first variceal bleed; or, rarely, an unusual cause of portal hypertension which may require some specific surgical therapy. Injection sclerotherapy is the most widely used treatment for both acute variceal bleeding and long-term management after a variceal bleed. Unfortunately it has probably been overused in the past. The need to identify the failures of sclerotherapy early and to treat them by other forms of major surgery is emphasized. The selective distal splenorenal shunt is the most widely used portosystemic shunt today, particularly in nonalcoholic cirrhotic patients. The standard portacaval shunt is still used for the management of acute variceal bleeding as well as for long-term management, particularly in alcoholic cirrhotic patients. For acute variceal bleeding the surgical alternative to sclerotherapy or shunting is simple staple-gun esophageal transection, whereas in long-term management the main alternative is an extensive devascularization and transection operation. Liver transplantation is the only therapy that cures both the portal hypertension and the underlying liver disease. All patients with cirrhosis and portal hypertension should be assessed as potential liver transplant recipients. If they are candidates for transplantation, sclerotherapy should be used to treat bleeding varices whenever possible, as this will interfere least with a subsequent liver transplant.     

Endoscopic sclerotherapy

Various sclerotherapy techniques have proved successful in the management of acute variceal bleeding and in long-term control of patients after a variceal bleed. We prefer either an intravariceal or a combined intravariceal and paravariceal technique using ethanolamine oleate, but we advocate that individual units utilize the technique with which they have the most experience. The use of an unmodified flexible endoscope has been almost universally accepted. Once active variceal bleeding is diagnosed on emergency endoscopy, immediate emergency sclerotherapy should be performed. When this is not possible, bleeding should be controlled by balloon-tube tamponade with subsequent delayed emergency sclerotherapy after resuscitation. Patients with variceal bleeding that has stopped at the time of the diagnostic endoscopy can either be treated by immediate sclerotherapy or be observed initially and subsequently treated using the long-term management policy of the unit concerned. Over 90% of actively bleeding patients should be controlled using emergency sclerotherapy. Failures are defined as patients who have more than two acute variceal bleeds during a single hospital admission. Such patients should be identified early and treated either by simple staple-gun transection or by an emergency portosystemic shunt. Repeated injection sclerotherapy using a flexible endoscope and the technique with which the group concerned has the most experience is recommended as the primary form of treatment for the majority of patients after a proven esophageal variceal bleed. Repeat injection treatments should probably be performed at weekly intervals until the esophageal varices are eradicated, with follow-up at 6-month or yearly intervals thereafter. Recurrent varices should be treated similarly. Failures of sclerotherapy are defined as patients who have either recurrent bleeds or in whom varices are difficult to eradicate. They require either a portosystemic shunt or a devascularization and transection operation. All patients presenting with cirrhosis and variceal bleeding should be evaluated for liver transplantation; unfortunately, however, few variceal bleeders are candidates for transplantation. Prophylactic sclerotherapy in patients with esophageal varices that have not bled remains unjustified outside of controlled trials. Available trials have produced conflicting data.     

Portal hypertension: a surgical hepatologist's view of current management

Current strategies for management of acute esophageal variceal bleeding and for long-term treatment after an episode of variceal bleeding are outlined. Acute variceal bleeding is best managed by means of endoscopic therapy (sclerotherapy, band ligation, or “superglue”), whereas the role of pharmacologic agents remains controversial. In cases of failure of endoscopic therapy, a transjugular intrahepatic portosystemic shunt (TIPS) procedure, an emergency shunt, or a transection operation should be performed. Patients who experience an acute variceal bleeding episode require long-term management to prevent recurrent bleeding. Endoscopic treatment is preferred using either sclerotherapy or banding. The principal alternative is long-term pharmacologic therapy with beta-adrenergic receptor blocking agents. Major surgical procedures should be reserved for failures of endoscopic or pharmacologic therapy. The distal splenorenal shunt or the new narrow-diameter polytetrafluoroethylene portacaval shunt is preferred. All patients who are first seen with acute variceal bleeding should be considered for a liver transplant, although few will ultimately become transplant candidates. Patients with end-stage liver disease who are not transplant candidates should be identified and major high-cost therapy discontinued. Prophylactic therapy prior to variceal bleeding should be considered in selected patients. At present, only pharmacologic therapy is justified. The major problem remains identification of those patients at high risk for a first episode of variceal bleeding.     

A hepatologist's view of variceal bleeding

Patients with cirrhosis and esophagogastric varices have a 25% to 33% risk of initial variceal bleeding, a risk of up to 70% for recurrent variceal bleeding, and an associated mortality of up to 50%. Based on a review of prospective randomized trials, control of acute variceal bleeding should involve vasopressin plus nitroglycerin as indicated for minor bleeding episodes, sclerotherapy for more severe bleeding episodes, and staple transection of the esophagus for patients who do not respond to these initial measures. Emergency portasystemic shunt surgery cannot be recommended at this time. For prevention of recurrent variceal hemorrhage, the data support the use of nonselective beta-adrenergic blockers (propranolol or nadolol) for patients with good liver function (Child's class A and B) and the use of chronic sclerotherapy to obliterate esophageal varices for patients with decompensated cirrhosis (Child's class C). Surgical procedures should be reserved for failures of medical management. The use of beta-adrenergic blockers offers the most promise for prevention of initial variceal bleeding.     

Has sclerotherapy altered the management of patients with variceal bleeding?

Sclerotherapy is currently the primary treatment of choice for the majority of patients who present with esophageal variceal bleeding. Although it has altered the management of these patients, unanswered questions and controversies remain. Patients with acute variceal bleeding should preferably be treated in a specialized center. The primary treatment should be immediate sclerotherapy, when possible. Portosystemic shunts and esophageal transection should be reserved for the 5% to 10% of patients in whom sclerotherapy fails to control acute bleeding. There are several treatment options for long-term management after a variceal bleeding episode. Sclerotherapy is one option and has become the primary treatment in most major centers. All patients with end-stage liver disease must be considered for liver transplantation, and sclerotherapy should be the primary method of treatment in those who are selected. Pharmacologic therapy remains controversial. I propose that portosystemic shunts and devascularization and transection operations be reserved for those few patients in whom sclerotherapy fails to eradicate the varices and to prevent recurrent variceal bleeding. Patients in whom sclerotherapy is unsuccessful should be identified and treated early.     

Results of a Modified Sugiura's Devascularisation in the Management of “Unshuntable” Portal Hypertension

The results of a modified Sugiura devascularisation procedure were assessed in 14 patients with thrombosis of the portal and splenic vein requiring surgery for variceal hemorrhage, with no vein suitable for orthodox shunt surgery. The venous anatomy was determined by ultrasonography with Doppler studies and portovenography. Liver biochemistry as well as liver architecture on histopathology was normal in all. The surgery was elective in 9 cases for documented bleed from diffuse fundal gastric varices (FGV) and emergency in 5 cases, 3 having bleeding FGV and 2 for failure of emergency esophageal variceal sclerotherapy. All were subjected to a transabdominal extensive devascularisation of the upper two third of the stomach and lower 7–10cm of the esophagus. Stapled esophageal transection (n=11) or esophageal variceal under-running (n=1) was performed in all with esophageal varices. FGV were underrun. Follow up endoscopies were done six monthly. There were 9 males and 5 females with a mean age of 17.2 years (SD 12.8). There was no operative mortality. Acute variceal bleeding was controlled in all patients. Over a mean follow up of 38 months, all but one remain free of recurrent bleeding. We conclude that a modified Sugiura devascularisation procedure is effective in the immediate and medium term control of variceal bleeding in patients with “unshuntable” portal hypertension.     

The Evolving Role of Endoscopic Treatment for Bleeding Esophageal Varices

The treatment of acute and recurrent variceal bleeding is best accomplished by a skilled, knowledgeable, and well-equipped team using a multidisciplinary integrated approach. Optimal management should provide the full spectrum of treatment options including pharmacologic therapy, endoscopic treatment, interventional radiologic procedures, surgical shunts, and liver transplantation. Endoscopic therapy with either band ligation or injection sclerotherapy is an integral component of the management of acute variceal bleeding and of the long-term treatment of patients after a variceal bleed. Variceal eradication with endoscopic ligation requires fewer endoscopic treatment sessions and causes substantially less esophageal complications than does injection sclerotherapy. Although the incidence of early gastrointestinal rebleeding is reduced by endoscopic ligation in most studies, there is no overall survival benefit relative to injection sclerotherapy. Simultaneous combined ligation and sclerotherapy confers no advantage over ligation alone. A sequential staged approach with initial endoscopic ligation followed by sclerotherapy when varices are small may prove to be the optimal method of reducing variceal recurrence. Overall, current data demonstrate clear advantages for using ligation in preference to sclerotherapy. Ligation should therefore be considered the endoscopic treatment of choice in the treatment of esophageal varices.     

Duodenal varices as a cause of massive upper gastrointestinal bleeding

Duodenal varices are an uncommon but serious manifestation of portal hypertension. Our management of three patients with massive bleeding due to duodenal varices stimulated a review of this subject. Thirteen cases of this condition were previously reported. Endoscopic examination of the entire duodenal mucosa is essential to document bleeding from duodenal varices. Medical therapies, including vasopressin and endoscopic sclerotherapy, have had limited success in controlling active duodenal variceal bleeding. Duodenal varix suture ligation or resection also resulted in a high rate of rebleeding. End-to-side portocaval shunt was the most effective procedure in stopping acute and subsequent bleeding in patients with duodenal varices. Despite therapy with or without portosystemic shunt, mortality risk is high in Child's class C patients and in patients with emergency duodenal variceal bleeding.     

Elective-selective Warren splenorenal shunt operation. Prognosis of liver function and esophageal varices hemorrhage after repeat sclerotherapy in liver cirrhosis]

From March 1st, 1982 to March 1st 1990 399 patients were admitted to the Heinz-Kalk-Hospital with recurrent bleeding from esophageal varices. Therapy of first choice was acute or elective endoscopic sclerotherapy. Early recurrences and uncontrollable hemorrhage were treated by Linton-Nachlas tube or if unsuccessful by devascularisation procedure. Two early or late bleeding recurrences were defined as sclerotherapy failures and choosen after passing a selection analysis (liver volume 1000 to 2500 ml, portal perfusion more than 30%, liver biopsy without activity or progression, exclusion of stenosis in the arterial supply of the liver and Child-Pugh classification A and B) for a selective-elective splenorenal Warren shunt (SRS). In 10 of 44 selected patients (11%) with an underlying disease of intrahepatic block in 95%, mostly alcoholic origin (65%) intraoperatively the performance of an SRS was technically problematic or impossible. Therefore, a mesocaval interposition shunt was carried out. Early mortality of 34 SRS was 5.9% (2 patients) and late mortality 17.6% (6 patients). No encephalopathy and shunt thrombosis were recorded. Postoperative angio- and sequential scintigraphies proved that portal perfusion was preserved during the first two years, but diminished. Liver function remained stable, too. One case of early rebleeding could be successfully managed by emergency endoscopic sclerotherapy. Five- and eight-years survival rate, according to the method of Kaplan-Meier is about 70%. We conclude that the SRS is the treatment of choice for elective management of recurrent bleeding of esophageal varices refractory to sclerotherapy. Its performance should be not enforced; in case of technical difficulties narrow-lumen mesocaval interposition shunt is an excellent alternative.     

Devascularization following endoscopic sclerotherapy of oesophageal varices: dangers and difficulties

Twenty-three patients underwent devascularization operations for acute variceal bleeding. All had received endoscopic sclerotherapy, either long-term or just before surgery. The oesophagus and peri-oesophageal tissues showed either oedematous or fibrotic reaction depending on the number and duration of sessions of sclerotherapy. These changes in the oesophagus and surrounding tissues were responsible for intraoperative oesophageal perforation and postoperative anastomotic leaks. To obviate these problems, stapling of the anterior and posterior walls of the stomach was tried and found to be safer than stapled transection of the oesophagus. During follow-up, varices reappeared in over 75 per cent of patients and were managed by further sclerotherapy. Patients who did not receive sclerotherapy had a higher incidence of rebleeding.     

A review of injection sclerotherapy--the Cape Town experience

Sclerotherapy is currently used to treat acute variceal bleeds and also in the long-term management after a variceal bleed. The technical variants and results of sclerotherapy in both settings are reviewed and compared with alternative surgical treatment options. Sclerotherapy has become an accepted therapy for acute variceal bleeding. In Cape Town it is used in combination with the Sengstaken tube. A preliminary analysis of an ongoing trial comparing a rigid scope technique with a fibreoptic scope technique provides support for the use of the rigid scope in acute variceal bleeding. The place of repeated sclerotherapy in long-term management has become controversial. Varices can be eradicated and repeated variceal bleeds markedly reduced, but its role in improving survival requires further clarification.     

Portal hypertension and bleeding esophageal varices

Summary Bleeding from esophageal varices exacts a high mortality and extraordinary societal costs. Prophylaxis—medication, sclerotherapy, or shunt surgery to prevent an initial bleeding episode—is ineffective. In patients who have bled from varices, endoscopic injection sclerotherapy can control acute bleeding in more than 90% of patients. Because recurrent bleeding frequently occurs and survival without definitive therapy is dismal, selection of a permanently effective treatment is mandatory once variceal bleeding has been controlled.Long-term injection sclerotherapy can be performed in compliant patients; it is relatively safe but is associated with a 30–50% rebleeding rate. Betablockers significantly reduce portal pressure and recurrent bleeding but have not been shown to diminish mortality from BEV. Portal decompressive surgery permanently halts bleeding in more than 90% of patients; the risk of operative mortality is high in decompensated cirrhotics, and long-term complications of encephalopathy and accelerated liver failure may limit indications for shunt surgery to good-risk cirrhotics who are not liver transplant candidates. Devascularization procedures have a low operative mortality and encephalopathy rate but unacceptably high rates of recurrent bleeding.Liver transplantation is curative therapy for bleeding esophageal varices and the associated underlying hepatic dysfunction; cost and availability of donor organs generally limit its use in this setting to variceal bleeders with end-stagè liver disease not associated with active alcoholism.     

Management of Variceal Hemorrhage in Children with Extrahepatic Portal Venous Obstruction-Shunt Surgery Versus Endoscopic Sclerotherapy

Extrahepatic portal venous obstruction (EHPVO) is a common cause of portal hypertention in children. Esophageal variceal hemorrhage is a major cause of morbidity and mortality in these patients. For many decades, portal systemic shunts were considered as the most effective treatment of variceal hemorrhage. Endoscopic injection sclerotherapy (EIS) was first introduced for emergency management of bleeding varices and subsequently as definitive treatment to prevent recurrent hemorrhage. The purpose of the study was to compare the safety and efficacy of shunt surgery and endoscopic sclerotherapy for patients with proven esophageal variceal bleeding due to EHPVO. The study was a prospective randomized study of 61 children with bleeding esophageal varices due to EHPVO carried out jointly by the department of General Surgery and Gastroenterology at Sher-i-Kashmir Institute of Medical Sciences, Srinagar, between March 2001 and September 2003. Thirty patients received surgery and other 31 patients received EIS. Overall incidence of rebleeding was 22.6% in sclerotherapy group and 3.3% in shunt surgery group. Treatment failure occurred in 19.4% patients in sclerotherapy group and 6.7% in shunt surgery group. The rebleeding rate of sclerotherapy is significantly higher than that of shunt surgery. However, the therapy failure rate of sclerotherapy is not significantly different from that of shunt surgery.     

A review of injection sclerotherapy —The cape town experience—

Sclerotherapy is currently used to treat acute variceal bleeds and also in the long-term management after a variceal bleed. The technical variants and results of sclerotherapy in both settings are reviewed and compared with alternative surgical treatment options. Sclerotherapy has become an accepted therapy for acute variceal bleeding. In Cape Town it is used in combination with the Sengstaken tube. A preliminary analysis of an ongoing trial comparing a rigid scope technique with a fibreoptic scope technique provides support for the use of the rigid scope in acute variceal bleeding. The place of repeated sclerotherapy in long-term management has become controversial. Varices can be eradicated and repeated variceal bleeds markedly reduced, but its role in improving survival requires further clarification. Based on a paper presented to: Japanese Research Society for Portal Hypertension, Tokyo, July 1984     

Combined sclerotherapy and operation for the treatment of bleeding oesophageal varices.

OBJECTIVE--To identify prognostic factors in a consecutive series of patients with bleeding oesophageal varices and develop an optimum regimen of treatment. DESIGN--Retrospective review. SETTING--I Department of Surgery, University Hospital, Vienna, Austria. PATIENTS--301 consecutive patients with bleeding oesophageal varices. OUTCOME MEASURES--Median survival and survival at one year after sclerotherapy alone (n = 213), or sclerotherapy with portosystemic shunt (n = 54), Hassab's devascularisation (n = 29), or liver transplantation (n = 5). RESULTS--Prognosis was dependent on the severity of liver damage at the start of treatment. Median survival for Child's class A was 47 months, for Child's class B 54 months, and for Child's class C 2 months. The overall one year survival for patients in Child's class C was 33%, for sclerotherapy alone 28%, and for sclerotherapy and portosystemic shunt 42%, Hassab's devascularisation 50%, and liver transplantation 80%. CONCLUSION--Despite the small number of patients who underwent liver transplantation and their poor initial prognosis (Child's class C, n = 4; class B, n = 1) our results suggest that liver transplantation should be considered for the treatment of patients with end stage cirrhosis and bleeding varices.     

Endoscopic sclerotherapy in the management of esophageal varices in 61 children with biliary atresia

Sixty-one children who have survived 2.5 years or more after corrective surgery for biliary atresia were prospectively followed by endoscopy. Esophageal varices were detected in 41 patients (67%), 17 of whom (28%) had experienced episodes of variceal hemorrhage. Control of variceal bleeding was achieved by endoscopic injection sclerotherapy in all but one child who died from hemorrhage before the completion of treatment. Complications of the technique comprised episodes of bleeding before variceal obliteration (7), esophageal ulceration (5), and stricture (3). These resolved with conservative management and without long-term sequelae. During a mean follow-up period of 2.8 years after variceal obliteration, rebleeding from recurrent esophageal varices developed in only one child and responded to further sclerotherapy. These results are better than those following surgical procedures for portal hypertension in biliary atresia, and therefore endoscopic sclerotherapy is recommended as the treatment of choice.     

Mesobiileal shunt in an infant for control of bleeding from gastroesophageal varices

There are various means to control bleeding gastroesophageal varices in children: sclerotherapy by injection, esophageal transection and variceal ligation, and different portosystemic shunts. Because of a high incidence of shunt thrombosis in children, many clinicians prefer other forms of therapy. However, only a successful shunt will lower the elevated portal venous pressure and eliminate the varices. This report describes a type of portosystemic shunt that will remain patent, even in an infant.     

Options for elective treatment of portal hypertension in cirrhotic patients in the transplantation era

We review herein our experience in the management of bleeding esophageal varices in cirrhotic patients and consider our findings in light of the dramatic changes in the treatment of cirrhosis resulting from the more widespread use of orthotopic liver transplantation (OLT). It does not seem realistic, at present, to propose OLT as the only effective treatment of variceal bleeding for a variety of reasons, and there remains a large group of patients who are noncompliant or unsuitable for liver transplantation. We propose that initial bleeding be controlled by endoscopic sclerotherapy, thereby allowing careful evaluation to be made electively. Grade A patients appear to be managed best by a reduced-size portacaval shunt (RPS) with prospects of good survival and few complications. Grade B patients can be managed by either sclerotherapy, RPS, or OLT, depending upon individual circumstances. Grade C patients are best managed by liver transplantation, again with excellent survival. In those grade C patients not deemed suitable for OLT (especially alcoholic patients), long-term endoscopic sclerotherapy is the best option. Changes in patient status may sometimes require revision of the treatment decision.     

Urgent transjugular retrograde obliteration for prophylaxis of rebleeding from gastric varices in patients with a spontaneous portosplenorenal shunt

BACKGROUND: Bleeding isolated gastric varices with a spontaneous portosplenorenal shunt are difficult to control. The urgent use of transjugular retrograde obliteration (TJO) to prevent early rebleeding and to improve early mortality has not yet been demonstrated. We report our experience with this technique in patients with isolated gastric varices after treatment of acute bleeding. METHODS: We reviewed our experience of 6 patients with isolated gastric varices with a spontaneous portosplenoral shunt treated with TJO after treatment of acute bleeding. We basically applied endoscopic glue embolization using cyanoacrylate monomer for treatment of acute bleeding. TJO was a method using an occlusive balloon catheter to control a spontaneous portosplenorenal shunt flow while injecting sclerosant retrograde into the gastric varices. RESULTS: Treatment of acute bleeding was achieved immediately by endoscopic glue embolization, endoscopic variceal ligation, and ligating the varices with sutures following anterior gastrotomy in 4, 1 and 1 patients, respectively, and then TJO was performed. Permanent hemostasis and variceal eradication was achieved in these 6, and they all survived. They were alive for 6-66 months without gastric variceal recurrence. CONCLUSIONS: We conclude that urgent TJO is effective in the prophylaxis of early and late rebleeding from isolated gastric varices in patients with a spontaneous portosplenorenal shunt.