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.     

Effect of portasystemic shunt operations on hepatic portal perfusion

We recently developed a radiocolloid technique for quantifying the fraction of superior mesenteric venous blood that bypasses liver sinusoids through extra- and intrahepatic collateral vessels. In the present investigation we applied this method, which is performed in conjunction with visceral angiography, to the assessment of patients with portal hypertension before and after surgical construction of portasystemic shunts. The mean corrected shunt index was 0.89 in 27 preoperative patients, and 48 percent of the patients had no evidence of sinusoidal perfusion by superior mesenteric venous blood (shunt index greater than 0.95). Sinusoidal perfusion was absent in five patients with residual hepatic portal flow by angiography, indicating that they had a high degree of intrahepatic shunting. Hepatic portal perfusion was preserved in 80 percent of patients after distal splenorenal shunt, and the corrected shunt index was significantly smaller after this procedure than after portacaval and interposition shunts. Three patients with no sinusoidal perfusion by superior mesenteric blood preoperatively had restoration of portal flow after distal splenorenal shunt. Five patients undergoing portacaval and interposition shunts had no evidence of portal sinusoidal perfusion by the radiocolloid technique either before or after the operative procedure.     

H-type shunt with an autologous venous graft for treatment of portal hypertension in children

From 1981 to 1987, 86 children aged 16 months to 16 years underwent a portosystemic shunt procedure using an autologous venous graft (internal jugular vein in 80 cases). Fifty-five mesocaval, 20 splenorenal, 4 portacaval, and 7 makeshift shunts were constructed. The indication for shunting was an extrahepatic portal obstruction in 59 cases, intrahepatic portal obstruction in 23 cases (including 6 cases of congenital hepatic fibrosis), and Budd-Chiari syndrome in 4 cases. One patient of the latter group died early from intractable ascites with a nonfunctioning shunt, and a second child died 2 months after operation from unknown reasons with a patent shunt. With a follow-up over 1 year for 58 of the 84 survivors, 78 successes and 6 failures were recorded according to the clinical outcome and the findings of ultrasonic and endoscopic examinations. Three of the six children with a failed shunt have been submitted to a second successful H-type shunt operation. No case of encephalopathy was recorded in this series. Thus, with an approximate success rate of 95%, the H-type shunt with a venous graft should be recommended for treatment of portal hypertension of extrahepatic origin, especially in young children.     

Transjugular intrahepatic portosystemic shunt vs. small-diameter prosthetic H-graft portacaval shunt: Extended follow-up of an expanded randomized prospective trial

We report herein the results of extended follow-up of an expanded randomized clinical trial comparing transjugular intrahepatic portosystemic shunt (TIPS) to 8 mm prosthetic H-graft portacaval shunt as definitive treatment for variceal bleeding due to portal hypertension. Beginning in 1993, through this trial, both shunts were undertaken as definitive therapy, never as a “bridge to transplantation.” All patients had bleeding esophageal/gastric varices and failed or could not undergo sclerotherapy/banding. Patients were excluded from randomization if the portal vein was occluded or if survival was hopeless. Failure of shunting was defined as inability to shunt, irreversible shunt occlusion, major variceal rehemorrhage, hepatic transplantation, or death. Median follow-up after each shunt was 4 years; minimum follow-up was 1 year. Patients undergoing placement of either shunt were very similar in terms of age, sex, cause of cirrhosis, Child’s class, and circumstances of shunting. Both shunts provided partial portal decompression, although the portal vein-inferior vena cava pressure gradient was lower after H-graft portacaval shunt (P<0.01). TIPS could not be placed in two patients. Shunt stenosis/occlusion was more frequent after TIPS. After TIPS, 42 patients failed (64%), whereas after H-graft portacaval shunt 23 failed (35%) (P <0.01). Major variceal rehemorrhage, hepatic transplantation, and late death were significantly more frequent after TIPS (P <0.01). Both TIPS and H-graft portacaval shunt achieve partial portal decompression. TIPS requires more interventions and leads to more major rehemorrhage, irreversible occlusion, transplantation, and death. Despite vigilance in monitoring shunt patency, TIPS provides less optimal outcomes than H-graft portacaval shunt for patients with portal hypertension and variceal bleeding. Presented at the Forty-First Annual Meeting of The Society for Surgery of the Alimentary Tract, San Diego, Calif., May 21–24, 2000.     

Hepatofugal portal blood flow in hepatic cirrhosis.

A variety of indirect techniques has been claimed to provide evidence of spontaneous reversal of portal blood flow in hepatic cirrhosis but the existence of the phenomenon has been doubted by some who do not accept the validity of the indirect evidence. There are few reports of the demonstration of hepatofugal portal flow by selective hepatic arteriography, which is the only acceptable technique. We report three patients with histologically confirmed cirrhosis in whom hepatofugal portal blood flow was unequivocally demonstrated by arteriography, in whom no surgical portosystemic shunt had been performed and in whom there was no evidence of the Budd-Chiari Syndrome or hepatoma, situations accepted as associated with reversed portal blood flow. Theoretical considerations suggest that shunt surgery for bleeding esophageal varices should not be ruled out on the grounds of hepatofugal portal flow. However, end-to-side portacaval anastomosis and distal splenorenal shunt might predispose to the early redevelopment of esophageal varices when reversed portal flow is present. Side-to-side portacaval and conventional splenorenal shunts might be preferable in having less effect on hepatic parenchyma perfusion than when orthograde portal flow in the case.     

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.     

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.     

Results of portal systemic shunts in Budd-Chiari syndrome.

Nine patients with Budd-Chiari syndrome (BCS) were treated by a portal systemic shunt. One had thrombosis of the superior mesenteric vein (SMV) and another had complete obstruction of the retrohepatic inferior vena cava (IVC). All other patients had a marked stenosis of the retrohepatic IVC with caval pressure ranging from 12 to 24 mmHg (mean: 17 mmHg). Seven patients had an interposition mesocaval shunt using an autologous jugular vein. The patient with a thrombosed SMV had a portoatrial shunt. The patient with an obstructed IVC had a cavoatrial shunt after an erroneous portacaval shunt had failed to relieve ascites. There were no operative deaths and no major postoperative complications. One patient died 19 months after operation of acute leukemia complicating polycythemia rubra vera. All other patients were alive and well 8 months to 6 years after operation. None of them had encephalopathy. These results suggest several comments: Portal systemic shunts are a good treatment for BCS and have a low operative risk. The mesocaval shunt is an efficient procedure, even when there is stenosis of the IVC with high caval pressure; shunts to the right atrium should be performed only in the case of complete obstruction or inaccessibility of the IVC. The long-term prognosis is excellent, except in patients with potential malignancies. Therefore, portal systemic shunts should be indicated early in patients with symptomatic BCS.     

Myeloproliferative disorders.

Forty-three operative procedures were performed on a population of 250 patients with myeloproliferative disorders, including polycythemia vera, myeloid metaplasia (MM) and chronic myelogenous leukemia (CML). The overall operative mortality was approximately 7% and the incidence of excessive bleeding which could be related to coagulopathy was 5%. Twenty-one patients with MM or CML underwent splenectomy for palliation of symptoms related to the enlarged spleen or hematologic problems. Eighty-four percent of the latter group were improved. Adverse hematologic effects which could be attributed to splenectomy in these patients were confined to two patients who developed marked thrombocytosis. Among the 23 patients with MM, 9 had portal hypertension. Three underwent portacaval shunt and one a splenorenal shunt for bleeding varices. One of the patients died of hepatic necrosis. Estimated hepatic blood flow determinations (EHBF) in 4 patients with portal hypertension demonstrated a marked absolute increase and an increase in the ratio of EHBF/Cardiac Index. Absence of any evidence of intrahepatic or extrahepatic obstruction in these patients and the demonstration that splenectomy relieved portal hypertension defined at surgery in 4 patients, suggests that augmented adhepatic flow contributes to portal hypertension in some cases. The review leads to the conclusions that: 1) Operative procedures in prepared patients with myeloproliferative disorders are not associated with prohibitive mortality and morbidity rates. 2) Splenectomy is indicated for patients with increasing transfusion requirements and symptomatic splenomegaly or hypersplenism and should be performed early in the course of disease. 3) When associated portal hypertension and bleeding varices are present, hemodynamic studies should be carried out to define if splenectomy alone, or a portal systemic decompressive procedure is indicated.     

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.     

Transjugular intrahepatic portasystemic shunt vs surgical shunt in good-risk cirrhotic patients: a case-control comparison

HYPOTHESIS: In good-risk patients with variceal bleeding undergoing portal decompression, surgical shunt is more effective, more durable, and less costly than angiographic shunt (transjugular intrahepatic portasystemic shunt [TIPS]). DESIGN: Retrospective case-control study. SETTING: Academic referral center for liver disease. PATIENTS: Patients with Child-Pugh class A or B cirrhosis with at least 1 prior episode of bleeding from portal hypertension (gastroesophageal varices, portal hypertensive gastropathy). INTERVENTION: Portal decompression by angiographic (TIPS) or surgical (portacaval, distal splenorenal) shunt. MAIN OUTCOME MEASURES: Thirty-day and long-term mortality, postintervention diagnostic procedures (endoscopic, ultrasonographic, and angiographic studies), hospital readmissions, variceal rebleeding episodes, blood transfusions, shunt revisions, and hospital and professional charges. RESULTS: Patients with Child-Pugh class A or B cirrhosis undergoing TIPS (n = 20) or surgical shunt (n = 20) were followed up for 385 and 456 patient-months, respectively. Thirty-day mortality was greater following TIPS compared with surgical shunt (20% vs 0%; P =.20); long-term mortality did not differ. Significantly more rebleeding episodes (P<.001); rehospitalizations (P<.05); diagnostic studies of all types (P<.001); shunt revisions (P<.001); and hospital (P<.005), professional (P<.05), and total (P<. 005) charges occurred following TIPS compared with surgical shunt. CONCLUSIONS: Operative portal decompression is more effective, more durable, and less costly than TIPS in Child-Pugh class A and B cirrhotic patients with variceal bleeding. Good-risk patients with portal hypertensive bleeding should be referred for surgical shunt.     

Liver transplantation for variceal hemorrhage

At the present time, liver transplantation must be considered among the treatment options for patients with variceal hemorrhage. For a significant percentage of variceal bleeders throughout the world, however, transplantation is not a viable option either because the patient is not an appropriate transplant candidate or because of the etiology of the patient's portal hypertension. Sclerotherapy and portosystemic shunts remain the mainstay of therapy for these patients. The survival rates with liver transplantation are superior to those reported for other therapies for variceal hemorrhage in patients who have moderate or severe liver disease in addition to variceal hemorrhage. Child's C patients whose variceal hemorrhage is controlled medically should be evaluated for transplantation and receive chronic sclerotherapy while they wait on the transplant list. If the variceal hemorrhage cannot be controlled medically in a transplant candidate, then the patient should undergo an emergency shunt procedure. The shunt of choice is a large-bore H-graft mesocaval or mesorenal shunt. This shunt effectively controls the acute hemorrhage, is relatively simple to perform, does not adversely impact on the subsequent liver transplant, and can simply be ligated after the transplant is completed. Patients who experience variceal hemorrhage as the only manifestation of their liver disease should be treated initially with endoscopic sclerotherapy. For that small group of patients who are either not candidates for sclerotherapy or who rebleed despite sclerotherapy, the choice of shunt or transplantation is presently a difficult one, because both therapies provide excellent results in this group of patients. The choice of therapy should be made on an individual basis and only after consultation with both transplant and shunt surgeons. If a shunt is chosen, we prefer the DSRS because it maintains hepatic portal perfusion in many patients and does not require dissection of the porta hepatis. The management of patients with a prior portosystemic shunt at the time of transplantation depends on the type of shunt and the duration of time between the shunt and the transplant. Shunts not involving the hepatic hilum have little adverse impact on the performance of the transplant. There are insufficient data to assess accurately the effect of a prior portacaval shunt on the transplant. However, our clinical experience and that of other transplant groups indicate that the transplantation of these patients is technically more difficult than that of patients with shunts not involving the hilum. With the availability of other shunting procedures that do not involve extensive dissection of the hepatic hilum, there is little role for either end-to-side or side-to-side portacaval shunts in patients who are potential liver transplant candidates.(ABSTRACT TRUNCATED AT 400 WORDS)     

The use of sclerotherapy for the management of oesophageal varices in portal hypertension

Summary Although sclerotherapy is currently the most widely used treatment for the management of both acute variceal bleeding and the long-term management of patients with varices, its definitive role in the treatment of these patients has yet to be finally proven. Sclerotherapy appears to be the most effective treatment for the majority of patients with acute variceal bleeding. Failures require either a shunt or a transection and/or devascularisation procedure. Current evidence favours simple staple gun transection or a shunt (either a portacaval shunt or a side-to-side narrow diameter polytetrafluoroethylene graft between the portal vein and vena cava). In long-term management of patients after a variceal bleed the currently favoured treatment is repeated sclerotherapy. However, failures should be identified early. We define failures as patients who present with varices that are either difficult to eradicate by sclerotherapy or who have repeated life-threatening variceal bleeds during the course of repeated injection sclerotherapy. Such patients should have either a portal-to-systemic shunt or a transection and devascularisation operation. Further controlled trials are required to define the specific indications for the individual forms of therapy. Prophylactic treatment for varices that have not yet bled is unjustified at present. Based on a presentation to the International Congress on Surgical Endoscopy, Ultrasound, and Interventional Techniques, Berlin 1988     

Graft interposition splenocaval shunt for total or selective decompression of portal hypertension

A new operation for selective or total decompression of the portal venous system in cases of intrahepatic portal hypertension is described. It involves interposition of a large-caliber Dacron graft between the splenic vein and the inferior vena cava. The graft-interposition splenocaval shunt is performed readily and quickly, satisfying the variable hemodynamic needs of patients with portal hypertension. It can be either selective (S-SCS) or total (T-SCS) from the beginning, or a T-SCS may be converted subsequently to a S-SCS should surgically induced hepatic decompensation supervene. It is less demanding technically than distal splenorenal shunt (D-SRS). The S-SCS conserves portal venous perfusion of the liver, preserves hepatocellular function and architecture at the preoperative levels, avoids precipitation of postshunt portal-systemic encephalopathy, and decompresses gastric-esophageal varices with prevention of further variceal bleeding even better than D-SRS. One hundred percent graft patency has been obtained, and the surgical results have been superior to those following portacaval shunt in patients with large liver blood flow and relative benignity of the liver disease, be it cirrhosis or noncirrhotic portal fibrosis. In patients with advanced cirrhosis, variceal bleeding, and small liver blood flows, T-SCS would be indicated. Patients of this category obtained inferior surgical results and had operative deaths (16.7%) following S-SCS. The concept of the operation has merits and deserves further evaluation.     

Sugiura procedure in portal hypertensive children

Bleeding from esophageal varices is an important cause of morbidity and mortality in children with portal hypertension. The treatment protocol is planned according to the etiologic factors underlying the portal hypertension, which may be either intrahepatic or extrahepatic. Although portasystemic venous shunt operations were common previously, they are now regarded as nonphysiologic and are rarely used because of their unexpected results and complications. Today, in many centers, endoscopic procedures have become the first-step treatment modality in bleeding esophageal varices. More complicated surgical procedures, such as devascularization procedures in extrahepatic portal hypertension, and liver transplantation in patients with failing liver, should be performed when conservative measures fail. We followed up 69 patients with portal hypertension with endoscopic sclerotherapy in our department. Here we present a retrospective evaluation of the effect of the Sugiura operation on the prognosis of 12 children (6 with extrahepatic and 6 with intrahepatic portal hypertension) who were not responsive to the sclerotherapy program. No rebleeding was seen in 9 of the 12 (75%) patients after the procedure, and the mortality rate in this series was 1 of 12 (8.3%); this patient died of hepatic failure. Received: November 7, 2000 / Accepted: January 25, 2001     

Role of surgical portosystemic shunts in the era of interventional radiology and liver transplantation

BACKGROUND: In the present era of liver transplantation and transjugular intrahepatic portosystemic shunts, the role and choice of shunt surgery for portal hypertension was reviewed. METHODS: This retrospective study analysed the management of patients with portal hypertension in a tertiary liver transplant unit between June 1993 and May 2002. During this 9-year interval, 394 patients underwent endoscopic control of varices, 235 transjugular intrahepatic portosystemic shunts were inserted, 1142 liver transplants were performed, while only 29 patients needed a surgical portosystemic shunt. RESULTS: Twenty-nine shunt operations were performed in nine patients with cirrhosis, one patient with congenital hepatic fibrosis and 19 without parenchymal liver disease. There were 12 side-to-side lienorenal, nine mesocaval, three proximal lienorenal, two distal lienorenal, two portacaval and one mesoportal shunts. Encephalopathy was seen in five of 11 patients with a non-selective shunt, but did not occur after side-to-side or selective lienorenal shunt procedures. At a median follow-up of 42.5 months, one mesocaval shunt had thrombosed and one portacaval shunt had stenosed; both were successfully managed by percutaneous intervention. To date, six patients have died; two succumbed to postoperative complications, one of which was related to the shunt. CONCLUSION: Patients with Budd-Chiari syndrome and cirrhosis can nearly always be managed by a combination of endoscopy, interventional radiology and liver transplantation. In the rare instances when these therapies fail in patients with cirrhosis, a side-to-side lienorenal shunt is a good option.     

Budd-Chiari syndrome: portacaval shunt and subsequent liver transplantation

The Budd-Chiari syndrome (BCS) is caused by hepatic venous outflow obstruction, which often leads to death as a result of portal hypertension and liver failure. Therapeutic approaches vary widely from conventional medical therapy to liver transplantation. If and when a patient suffering with BCS needs surgery remains a matter of contention. However, it is well accepted that portacaval shunt surgery and orthotopic liver transplantation represent efficient surgical treatments of this condition. We report on a patient with an eventful course after BCS was diagnosed. After portacaval shunt surgery the patient had acute liver failure and had a successful orthotopic liver transplantation.     

Flow- and pressure-adapted portal arterialization in dogs

The effects of portal arterialization after portacaval shunt were studied in dogs. Flow- and pressure-adapted portal arterialization was performed by mounting a Teflon cuff on an autogenous vein bypass graft between the hepatic stump of the portal vein and the right renal artery. Immediately following operation, the total hepatic blood flow and intrahepatic portal venous pressure were within normal range. Eight weeks after operation, the intrahepatic portal venous pressure remained within the preoperative range, while total hepatic blood flow had increased double or triple. However, structual change due to increased flow was absent in the liver, even sixteen months after operation. Body weight, liver enzyme chemistry, ICG clearance rate, and amino acid metabolism were well maintained for the entire period of investigation. These findings suggest that sequelae such as hepatic encephalopathy and impaired hepatic metabolism after portacaval shunt can be avoided by portal arterialization, in the presence of an appropriate flow and pressure.     

Flow- and pressure-adapted portal arterialization in dogs

The effects of portal arterialization after portacaval shunt were studied in dogs. Flow- and pressure-adapted portal arterialization was performed by mounting a Teflon cuff on an autogenous vein bypass graft between the hepatic stump of the portal vein and the right renal artery. Immediately following operation, the total hepatic blood flow and intrahepatic portal venous pressure were within normal range. Eight weeks after operation, the intrahepatic portal venous pressure remained within the preoperative range, while total hepatic blood flow had increased double or triple. However, structural change due to increased flow was absent in the liver, even sixteen months after operation. Body weight, liver enzyme chemistry, ICG clearance rate, and amino acid metabolism were well maintained for the entire period of investigation. These findings suggest that sequelae such as hepatic encephalopathy and impaired hepatic metabolism after portacaval shunt can be avoided by portal arterialization, in the presence of an appropriate flow and pressure.     

Use of splenic artery embolization to relieve tense ascites following liver transplantation in a patient with paroxysmal nocturnal hemoglobinuria.

Recurrent venous thrombosis following liver transplantation for Budd-Chiari syndrome is common, particularly in the setting of an underlying myeloproliferative disorder. We describe a patient who developed refractory ascites due to portal vein thrombosis following liver transplantation for Budd-Chiari syndrome in the setting of paroxysmal nocturnal hemoglobinuria. Extensive portal vein thrombosis, dense abdominal adhesions, and portosystemic collaterals precluded the use of a transjugular intrahepatic portosystemic shunt or surgical portosystemic shunt to manage the patient's ascites. Splenic artery embolization to decrease portal hypertension was performed, and this resulted in complete resolution of ascites. This case demonstrates the successful use of splenic artery embolization to manage ascites due to portal vein thrombosis following liver transplantation. Splenic artery embolization may be considered as an alternative option for the management of refractory ascites due to portal hypertension in patients who are unable to undergo safe transjugular intrahepatic portosystemic shunt or surgical shunt placement.