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Extrahepatic portal vein thrombosis (EHPVT) may occur in children or adults and usually comes to clinical attention due to complications of portal hypertension such as variceal hemorrhage. A variety of standard surgical techniques exist to manage these patients, but when these fail surgical options are limited. We describe two novel portosystemic shunts that utilize the gonadal vein as an autologous conduit. Four patients were evaluated for EHPVT with variceal bleeding. None of the patients were candidates for a standard splenorenal shunt due to prior surgical procedures. The first patient underwent a left mesogonadal shunt and the remaining 3 patients underwent a right mesogonadal shunt. Postoperative ultrasound or computed tomography (CT) scan confirmed early patency of the shunt in each patient. There have been no further episodes of variceal hemorrhage with follow-up of 3.5 years in the child who underwent the left mesogonadal shunt, and 17, 19, and 20 months in the patients who underwent the right mesogonadal shunt. Three of the 4 shunts remain patent. One shunt thrombosis occurred in a patient homozygous for the Factor V Leiden mutation despite anticoagulation with coumadin. This is the first report of the successful use of the gonadal vein as an in situ conduit for constructing a portosystemic shunt. In conclusion, the right and left mesogonadal shunts may be useful as salvage operations for patients with EHPVT who have failed standard surgical shunt procedures.

In the period from 1985 to 1992 sixty-three patients aged from 7 months to 15 years were treated for the portal hypertension syndrome, 60 had the extrahepatie form. Forty-five various operations for portosystemic shunting were carried out: formation of proximal splenorenal anastomosis in 31, distal splenorenal anastomosis in 4, mesentericocaval anastomosis in 6, gastrocaval anastomisis in 2, and an atypical vascular shunt in 2 cases. Nonshunting operations were performed on 8 patients. Since 1986 39 sessions of endoscopic sclerotherapy were conducted, 16 of them were carried out in cases with esophagogastric bleeding.

Surgical shunts in the management of portal hypertension are one component of overall management. The main complications of portal hypertension is variceal bleeding. The very high mortality rate of this complication mandates careful assessment and appropriate treatment. Despite the introduction of new treatment methods such as pharmacologic reduction of portal pressure and endoscopic sclerotherapy, taken in conjunction with the coming of age of liver transplantation, surgical decompressive shunts still have a role in the overall treatment of these patients. The choice of therapy is based on full evaluation. The emphasis of that evaluation is on hepatic function. The patients who can live with their liver for an acceptable period of time and do not have the bleeding controlled with lesser medical managements require decompressive shunts. Surgical decompression of portal hypertension may also be required in the patient who is bleeding to death in the acute setting and, finally, is required for the treatment of acute Budd-Chiari syndrome. The choice of shunt procedures should take into account the potential for liver transplantation at some point in the future.

No single shunt operation is ideal for all patients or all circumstances. The selective distal splenorenal shunt is preferred for the majority of patients, because it has the potential to preserve hepatic portal perfusion. Most evidence suggests that the distal splenorenal shunt is followed by a lower frequency of encephalopathy than is any type of nonselective shunt. Although theoretically attractive, the small-diameter interposition portacaval shunt has not yet been subjected to the scrutiny of controlled trials.

Distal splenorenal shunt was performed in 17 patients with bleeding esophageal varices and/or hypersplenism. As an elective operation the results were very good, whereas the shunt should not be used as an emergency operation. During an observation period of 6-30 months none of the 13 surviving patients developed a recurrent hemorrhage or portosystemic encephalopathy. An existing hypersplenism was improved in all cases. In our opinion the distal splenorenal shunt is the optimal elective surgical management for bleeding esophageal varices, since it offers effective control of recurrent hemorrhage without the risk of encephalopathy.

Fifteen patients with a patent shunt after distal splenorenal shunt with gastrosplenic disconnection were prospectively studied by angiography three to 36 months after operation. In all patients a collateral circulation from the portomesenteric to the gastrosplenic system was developed through enlarged venous channels. In all patients the portal flow decreased, as suggested by the angiographically assessed degradation of the portal perfusion of the liver and/or the diminution of the diameter of the portal vein. In two patients the portal vein was thrombotic. We conclude that three months after operation distal splenorenal shunt with gastrosplenic disconnection is not hemodynamically different than portacaval laterolateral shunt.

Three cases of pseudocysts of the pancreas complicating the operative procedure of distal splenorenal shunt are reported. In all of these patients, gross changes in the pancreas, consistent with chronic pancreatitis were found. Attention is called to the possibility of this complication occurring in association with this particular operation, specifically, when it is carried out in patients who may prove to have a considerable degree of pancreatic and retroperitoneal fibrosis secondary to alcoholic pancreatitis. All three pseudocysts were satisfactorily managed by percutaneous drainage under ultrasonic control, which provided a very satisfactory alternative to surgical decompression.

Thirty-five patients of bleeding oesophageal varices were treated by modified Warren's distal splenorenal procedure. In this procedure the spleen was removed. The value of the technique in maintaining liver perfusion, and relieving hypersplenism is discussed.

Two types of modified distal splenorenal shunt with expanded polytetrafluoroethylene (Gore-Tex; WL Gore & Associates Inc., Elkton, Md.) interposition were performed in 18 consecutive patients with esophageal or esophagogastric varices. There were 12 men and six women ranging in age from 32 to 76 years. The causes of portal hypertension were cirrhosis of the liver in 15 patients, chronic hepatitis in two, and idiopathic portal hypertension in one. In five patients the left gastric vein branched off from the splenic vein; bilateral gastric venous decompression was achieved by preserving the splenic vein. Porta-azygos disconnection was routinely performed by confirming repeated intraoperative direct splenoportography. The operations were elective in seven and were emergencies in five patients. Six patients underwent a prophylactic shunt; all patients had "red color signs" endoscopically, and three of them had concomitant hepatocellular carcinoma. Postoperative morbidity was minimal and there was no mortality. Shunt patency was confirmed angiographically in all patients 14 to 56 days after surgery. The varices disappeared or significantly improved in all patients. No patients had variceal bleeding postoperatively. Hepatic encephalopathy was transiently seen in one (the oldest) patient.

Twenty-three patients with portal hypertension due to alcoholic cirrhosis were treated by distal splenorenal shunt and gastrosplenic mesenteric disconnection. In the post-operative period, two patients died; in one patient gastro-intestinal bleeding recurred 7 days after surgery and was due to ruptured varices with thrombosis of the splenorenal shunt; ascites developed in 14 patients. Twenty patients were followed up 6 months or more after surgery. Intestinal bleeding occurred in 3 patients with patent shunt. Ascites always disappeared within 3 months after operation. Four patients developed encephalopathy. Seventeen patients were investigated by angiography 3 months or more after surgery. The shunt was patent in 15 and occluded in 2 patients. In all patients with patent shunt a collateral circulation between the portomesenteric to gastrosplenic systems developed. In all patients, the portal flow decreased as suggested by diminution of the diameter of the portal vein. The authors conclude that 3 months after operation, results of distal splenorenal shunt with gastrosplenic disconnection are not hemodynamically different from those of side-to-side portocaval shunt.

The management of portal hypertension focuses on control of its complications, the most important of which is bleeding esophageal varices. Other complications, such as ascites, bleeding intestinal stomas, and hypersplenism, rarely require surgical intervention. Other than medical management, the three basic procedures now available for the treatment of bleeding esophageal varices include decompression of varices with a portosystemic shunt, nonshunting operations that attack directly the esophageal variceal-bearing area, and liver transplantation as the procedure of choice in selected patients. Patients who present with episodes of acute bleeding are usually treated initially with medical therapy including acute sclerotherapy or balloon tamponade techniques when necessary. If the patient fails to respond or if episodes of bleeding recur, further therapy is required. Although selection of therapy remains controversial, it is based on multiple factors. These include the basic pathogenic mechanism of portal hypertension in the individual patient, status of the patient as defined by Child's classification, elective or urgent nature of the operation, hemodynamic stability of the patient at the time of the procedure, site of the block in the portal system, and caliber and anatomic relationship of the vessels available for anastomosis in the portal system. Additional factors include the presence and severity of ascites or encephalopathy, age of the patient, site of bleeding (esophageal or gastric), severity of associated hypersplenism, and techniques and expertise available at a given institution. Shunting procedures achieve the best long-term control of bleeding, but they can precipitate the development of encephalopathy. Nonshunting procedures do not induce encephalopathy, but they are usually associated with a high rate of rebleeding. Also, with the possible exception of sclerotherapy, they are still associated with a high operative mortality rate in alcoholic patients classified as Child's C. Although sclerotherapy controls acute variceal bleeding more successfully than conventional methods, it is not readily applicable in patients with bleeding gastric varices. Also, it has not yet clearly been proved to be an effective method of permanent control of gastroesophageal bleeding and has not been demonstrated to increase survival. The new methods of extensive esophagogastric devascularization (for example, porta-azygos disconnection using the Sugiura procedure) are attractive because of the low late recurrence rate for bleeding without the induction of encephalopathy.(ABSTRACT TRUNCATED AT 400 WORDS)

This paper reports the preliminary results of a prospective randomized trial comparing endoscopic variceal sclerosis and distal splenorenal shunt (DSRS) in the management of patients with cirrhosis and variceal bleeding. Seventy-one patients have been entered; 36 have received sclerosis and 35 DSRS. Randomization of the study population was stratified on Child's A/B (56%) and Child's C (44%). Sixty-one per cent had alcoholic and 39% non-alcoholic cirrhosis. No patients have been lost to follow-up, which currently stands at a median of 26 months. Rebleeding occurred significantly (p less than 0.05) more frequently in patients in the sclerosis group (19 of 36: 53%) compared to DSRS (1 of 35: 3%), but only 11 of 36 (31%) were not controlled by further sclerosis and failed that therapy. Patients in whom sclerosis failed underwent surgery. Survival was significantly (p less than 0.01) improved in the sclerosis group (+ surgery in 31%), with an 84% 2-year survival compared to a 59% 2-year survival in the DSRS group. Portal perfusion was significantly (p less than 0.05) better maintained in the sclerosis (95%) compared to the DSRS (53%) group. Galactose elimination capacity improved significantly (p less than 0.05) in 21 patients successfully managed by sclerosis at 1 year and was significantly (p less than 0.01) better maintained in the sclerosis compared to DSRS group. The authors conclude that endoscopic sclerosis: has a higher rebleeding rate than DSRS, with one third of patients failing therapy from rebleeding; allows significant improvement in liver function when successful; and gives significantly improved survival in the management of variceal bleeding when backed up by surgical therapy for patients with uncontrolled rebleeding.

Our initial use of the distal splenorenal shunt (DSRS) in 1973 was fostered by disappointment with the results of so-called total shunts. This selective shunt was, when anatomically feasible, our preferred therapy until 1980, when surgical referral was affected by enthusiasm for sclerotherapy. Our study of 71 DSRSs is uncontrolled because we could not recruit patients for a prospective randomized trial that involved either no treatment of operations that had proven faults. Our experience shows that operative risk (4%) and incidence of postshunt encephalopathy (6%) are low, that the rate of shunt occlusion is acceptable (10%), and that bleeding is as well controlled as with other shunts. Survival rates correlate with the cause of portal hypertension and with hepatic functional reserve. Analysis of the causes of death shows that the natural history of cirrhosis and coexistent disease are major determinants of prognosis.

A technique for temporary occlusion of the splenic artery during construction of the distal splenorenal shunt is described. This maneuver is proposed to reduce intraoperative blood loss associated with mobilization of the splenic vein in the cirrhotic patient.

The selective distal splenorenal shunt is the preferred portal decompression procedure for patients with refractory bleeding esophageal varices. An autogenous jugular vein interposition graft in the distal splenorenal position obviates the tedious struggle associated with mobilizing the splenic vein from the pancreatic substance, thereby lessening blood loss, avoiding postoperative pancreatitis and shortening operative time. An autogenous jugular vein interposition distal splenorenal shunt can, therefore, be performed with less morbidity while affording the same physiologic benefits as the standard distal splenorenal shunt.

Patients who have previously bled from esophageal varices should have their hepatic function, neurologic status and nutritional status evaluated and should be examined for ascites before elective variceal decompression is done to prevent recurrent variceal hemorrhage. The two most important procedures used for this evaluation are liver biopsy and visceral angiography. If the liver biopsy reveals little or no necroinflammatory activity, shunt surgery is indicated. For patients with moderate or severe necroinflammatory activity, surgery is delayed until the inflammation subsides. The central aim of visceral angiography is to determine whether the patient has hepatopetal or hepatofugal portal venous flow. Patients with hepatopetal flow are treated with a selective distal splenorenal shunt. Those with hepatofugal flow are managed with a total shunt, such as an interposition H-graft portacaval or mesocaval shunt.

This article reports on a retrospective clinical comparison between the original Warren shunt and one that we modified with Gore-Tex (expanded polytetrafluoroethylene) interposition. The former operation was performed on 35 patients between June 1969 and November 1983 and the latter on 29 patients between October 1983 and January 1986. There were no significant differences in the patients' backgrounds between the two study groups. Blood loss during surgery was significantly greater and operation time was longer in the original shunt group than in the modified shunt group. The incidence of postoperative morbidity and mortality was also significantly higher in the former group than in the latter (major complication rate: 20.0% versus 3.4%; operative death within 1 month: 5.7% versus 0%; in-hospital death: 11.4% versus 3.4%). The modified shunts had a 100% patency rate, and no variceal bleeding was evident, whereas shunt occlusion was observed in two patients and portal thrombosis in one patient of the original shunt group. The incidence of hepatic encephalopathy was 14.3% in the original shunt group and 6.9% in the modified shunt group, and the follow-up time was shorter in the latter group. A significantly greater rate of survival was achieved with the modified Warren shunts. Thus the current study seems to indicate that our modifications could be alternatives to the original distal splenorenal shunt in terms of postoperative morbidity, mortality, and survival.

One hundred consecutive Child's A or B patients with portal hypertension who survived the index episode of variceal bleeding were electively treated by a distal splenorenal shunt modified by a retroperitoneal approach. The operative mortality of the whole series was 11 per cent, but fell from 16 per cent in the first 50 patients to 6 per cent in the second half of the series. The median survival probability (68 months) and the 5 year survival rate (52 per cent) of Child's A patients differed significantly from those of Child's B patients (8 months and 15 per cent respectively; P less than 0.001). The probabilities of freedom from rebleeding and hepatic encephalopathy at 5 years were also significantly greater in Child's A patients (70 per cent and 70 per cent respectively) than Child's B patients (25 per cent and 30 per cent respectively). These results suggest that the modified distal splenorenal shunt is an effective and relatively safe procedure for the elective treatment of variceal bleeding in Child-Campbell 'A' patients but that patients in group B should be considered for other forms of therapy.

Controversy still surrounds the place of portalsystemic shunting in the therapy of bleeding esophageal varices. Recently, a selective shunt, the distal splenorenal shunt, has achieved some degree of popularity and, apparently, is associated with less chronic encephalopathy. Because of this, a trial was initiated at the Massachusetts General Hospital and continued at the University of Cincinnati Medical Center, prospectively randomizing central and distal splenorenal shunts in consecutive elective cases of patients with established variceal bleeding. Preoperative evaluation included endoscopic examination at the time of hemorrhage, angiography and upper gastrointestinal series, emphasis on mental function including EEG, amino acids, neurologic examination, as well as standard liver chemistries. Nineteen patients underwent central splenorenal shunts and 23 distal splenorenal shunt. There was one operative death from hemorrhagic pancreatitis in a Child's Class A patient with distal splenorenal shunt. Four late deaths, from gunshot wound, auto accident, overwhelming pneumonitis similar to postsplenectomy syndrome, and metastatic carcinoma (2.5 years after operation), have been recorded in the distal splenorenal shunt group, and none in the central splenorenal shunt group. On follow-up angiographic examination, six shunts have clotted, with three patients requiring reoperation, generally mesocaval shunt. There has been no chronic encephalopathy, three individual episodes of encephalopathy, two in the central splenorenal shunt group and one in the distal splenorenal shunt group, two associated with gastrointestinal bleeding and one with intercurrent infection and overdiuresis. Follow-up liver chemistries and amino acids which may be useful as an indicator of hepatic function suggest that although the distal shunt group had a better amino acid pattern before operation, branched-chain amino acids tend to become lower in the distal group while remaining the same in the central group. Aromatic amino acids increase post shunt, equally in the two groups. The results do not support the contention that distal splenorenal shunt is associated either with greater survival or freedom from encephalopathy than central splenorenal shunt, a small side-to-side shunt. Ascites seems better controlled by the central splenorenal shunt.

We have found that the use of intraoperative Doppler ultrasound greatly facilitates the performance of the selective distal splenorenal shunt by precisely identifying the retroperitoneal location of the renal vein. The amount of dissection and tissue injury required for performing this operation was, thereby, minimized. This may theoretically result in a lowered incidence of postoperative ascites and complications after the Warren shunt.

Repeated direct portography was performed during distal splenorenal shunt in 13 consecutive patients with esophageal varices due to cirrhosis of the liver. The roentgenogram taken at the completion of all operative procedures demonstrated incomplete disconnection between the mesentericoportal and gastrosplenic compartments in four patients. After further disconnection of the veins, the third portogram showed a successful isolation of both systems. The flow state through and the size of the shunt were appropriate in all instances on the roentgenograms. The shunt was patent in all patients, which was confirmed before discharge from the hospital. Hepatic encephalopathy was encountered one year after operation in one patient with the poorest hepatic functions. Esophageal varices disappeared or nearly disappeared at the time of discharge in ten patients. The remaining varices at discharge in another three patients completely disappeared within six months after operation. None of the patients experienced variceal bleeding during the follow-up period of three to 25 months. The results may indicate that repeated intraoperative direct portography is useful in achieving a selective distal splenorenal shunt.

A new modification of the Warren shunt by which the coronary and short gastric venous systems can be simultaneously decompressed was carried out on two patients with oesophagogastric varices due to liver cirrhosis. One was an elective and the other an emergency operation. The left gastric vein entering the splenic vein was also drained through a Gore-Tex graft between the splenic and left renal veins. The proximal end of the splenic vein was ligated at its junction to the superior mesenteric vein. Porta-azygos disconnection was achieved by ligating the right gastric and gastro-epiploic veins. The shunt was patent and its effect on the varices was immediate with good decompression in both patients. The patients have had no recurrent variceal haemorrhage or postshunt encephalopathy. This modification may be indicated for selected patients with portal hypertension for both elective and emergency operations.

A preliminary report is presented of a posterior exposure of the splenic vein allowing H-graft splenorenal shunt to be carried out without removing the spleen. The operation was successfully performed in 14 patients with portal hypertension due to schistosomal hepatic fibrosis, and this was associated with post-hepatitic cirrhosis in 5 of them. Dacron grafts were inserted in 8 patients and autologous internal jugular vein grafts in 6 patients. The long term follow-up of the patients (maximum 45 months) showed that recurrence of bleeding developed in 2 patients due to shunt thrombosis and one died. Complications occurred in 3 patients, including one patient who developed first grade encephalopathy. Shunt patency was demonstrated in 12 patients, including all the vein grafts.

Warren's distal splenorenal shunt has been effective in treatment of recurrent variceal hemorrhage. To simplify the operative procedure, two alterations in the technique were used in this study. The first was to approach the splenic and renal veins simultaneously through a single inframesocolonic retroperitoneal incision in the plane of the pancreatic fusion fossa. The second was abandonment of the portoazygos disconnection. Since 1974, twenty-nine patients have undergone this modified operation. The patients ranged in age from 21 to 76 years. Fourteen patients had alcoholic cirrhosis, 13 had nonalcoholic cirrhosis, and the etiology was unknown in two. There were 14 Child's class A patients, 12 class B, and three class C. Significant ascites was present at operation in seven patients. Hepatofugal flow was demonstrated angiographically in four patients. The follow-up period ranged from 6 months to 6 years; 19 patients were followed up for 1 year or longer. There were three early postoperative deaths (10.3%), and one patient died 2 months postoperatively. Recurrent variceal bleeding was seen early in one patient and late (2 months) in another. Only one of the surviving patients developed encephalopathy. Technical modification to simplify performance of the distal splenorenal shunt did not compromise the results of the operation. However, four of seven patients with ascites died either immediately or early after the procedure, and two of these four patients also had hepatofugal flow. Ascites and hepatofugal flow may adversely influence the results of the operation.

This report describes 140 cases in which Linton splenorenal shunts were performed for the management of the complications of portal hypertension by a large number of surgeons in a single hospital. There was a history of variceal bleeding in 130. Using the Childs designation to reflect hepatic functional reserve, the overall operative mortality was 12% (3 for A; 6 for B; 26 for C). Five-year survival was 41% (57 for A; 35 for B; 26 for C). Subsequent variceal bleeding was noted in 10% of survivors; hepatic encephalopathy in 19%; and terminal liver failure in 18%. Classification and results are reported in a form that should facilitate comparison with other methods of management.

This report presents results of surgery for bleeding esophageal varices in 80 patients. A prospective randomized study addresses the efficacy of the distal splenorenal shunt (DS) versus the mesocaval shunt (MS) in 50 patients undergoing elective surgery. An additional 30 patients underwent emergency MS for uncontrollable hemorrhage. In the elective series, patients averaged "B" according to Child's Classification. Operative mortality rates were similar (5%). Incidences of encephalopathy were also similar (10%). Those patients undergoing MS experienced an overall operative mortality of 9 per cent, which included emergency shunts (operative mortality 13%). This latter figure is the lowest in the world's literature. Our technique of mesocaval shunting emphasizes short (mean, 3.8-cm) and wide (mean, 21.5-mm) cloth prostheses. Utilizing this approach, we have been able to reduce operative portal venous pressure from a mean (x +/- SE) of 40.1 +/- 1.9 to 13.1 +/- 0.6 cm H2O. The latter value correlated inferior vena caval pressure, 11.8 +/- 0.6 and central venous pressure (recorded by the anesthesiologist) 11.4 +/- 0.5 cm H2O. This is the highest reduction (67%) in portal pressure thus far recorded and reflects our emphasis upon meticulous and extensive dissection of the involved structures. The former minimizes blood loss, which in our hands has been minimal (0.45 +/- 0.18 units per case), reducing the threat of further liver damage; the latter facilitates the "optimal shunt," one which returns portal venous pressure to normal.

Bleeding esophageal varices are treated in the first line as emergency by sclerosing procedures. Later on shunt surgery is still to be considered as an important definite procedure. Criteria to be met before surgery are discussed. In our experience the shunting procedure as described by Warren yields good results, low incidence of thrombosis and low lethality. In special cases subtotal esophagectomy can be performed, lethality being as low as after the Warren shunt. Repeating sclerosing over years in contrast carries a high risk of complications and death and does not prevent further bleeding safely enough.