Barbara Girard

A 27-year experience with surgical treatment of Budd-Chiari syndrome.

ObjectiveTo determine the effects of surgical portal decompression in Budd-Chiari syndrome (BCS) on survival, quality of life, shunt patency, liver function, portal hemodynamics, and hepatic morphology during periods ranging from 3.5 to 27 years. Summary Background DataExperiments in the authors’ laboratory showed that surgical portal decompression reversed the deleterious effects of BCS on the liver. This study was aimed at determining whether similar benefit could be obtained in patients with BCS. MethodsFrom 1972 to 1999, the authors conducted prospective studies of the treatment of 60 patients with BCS who were divided into three groups: the first had occlusion confined to the hepatic veins treated by direct side-to-side portacaval shunt (SSPCS); the second had occlusion involving the inferior vena cava (IVC) treated by a portal decompressive procedure that bypassed the obstructed IVC; and the third group, who had advanced cirrhosis and hepatic decompensation and were referred too late for treatment by portal decompression, required orthotopic liver transplantation. ResultsIn the 32 patients with BCS resulting from hepatic vein occlusion alone, SSPCS had a surgical death rate of 3%, and 94% of the patients were alive 3.5 to 27 years after surgery. All 31 survivors remained free of ascites and almost all had normal liver function. No patient with a patent shunt had encephalopathy. The SSPCS remained patent in all but one patient. Liver biopsies showed no evidence of congestion or necrosis, and 48% of the biopsies were diagnosed as normal. Mesoatrial shunt was performed in eight patients with BCS caused by IVC thrombosis. All patients survived surgery, but five subsequently developed thrombosis of the synthetic graft and died. Because of the poor results, mesoatrial shunt was abandoned. Instead, a high-flow combination shunt was introduced, consisting of SSPCS combined with a cavoatrial shunt (CAS) through a Gore-Tex graft. There were no surgical or long-term deaths among 10 patients who underwent combined SSPCS and CAS, and the shunts functioned effectively during 4 to 16 years of follow-up. Ten patients with advanced cirrhosis were referred too late to benefit from surgical portal decompression, and they were approved and listed for orthotopic liver transplantation. Three patients died of liver failure while awaiting a transplant, and four patients died after the transplant. The 1- and 5-year survival rates were 40% and 30%, respectively. ConclusionsSSPCS in BCS with hepatic vein occlusion alone results in reversal of liver damage, correction of hemodynamic disturbances, prolonged survival, and good quality of life when performed early in the course of BCS. Similarly good results are obtained with combined SSPCS and CAS in patients with BCS resulting from IVC occlusion. In contrast, mesoatrial shunt has been discontinued in the authors’ program because of an unacceptable incidence of graft thrombosis and death. In patients with advanced cirrhosis from long-standing, untreated BCS, orthotopic liver transplantation is the only hope of relief and results in the salvage of some patients. The key to long survival in BCS is prompt diagnosis and treatment by portal decompression.

Bleeding esophagogastric varices from extrahepatic portal hypertension: 40 Years' experience with portal-systemic shunt

BACKGROUND This article discusses the largest and longest experience reported to date of the use of portal-systemic shunt (PSS) to treat recurrent bleeding from esophagogastric varices caused by extrahepatic portal hypertension associated with portal vein thrombosis (PVT). STUDY DESIGN Two hundred consecutive children and adults with extrahepatic portal hypertension caused by PVT who were referred between 1958 and 1998 after recovering from at least two episodes of bleeding esophagogastric varices requiring blood transfusions were managed according to a well-defined and uniformly applied protocol. All but 14 of the 200 patients were eligible for and received 5 or more years of regular followup (93%); 166 were eligible for and received 10 or more years of regular followup (83%). RESULTS The etiology of PVT was unknown in 65% of patients. Identifiable causes of PVT were neonatal omphalitis in 30 patients (15%), umbilical vein catheterization in 14 patients (7%), and peritonitis in 14 patients (7%). The mean number of bleeding episodes before PSS was 5.4 (range 2 to 18). Liver biopsies showed normal morphology in all patients. The site of PVT was the portal vein alone in 134 patients (76%), the portal vein and adjacent superior mesenteric vein in 10 patients (5%), and the portal and splenic veins in 56 patients (28%). Postoperative survival to leave the hospital was 100%. Actuarial 5-year, 10-year, and 15-year survival rates were 99%, 97%, and 95%, respectively. Five patients (2.5%), all with central end-to-side splenorenal shunts, developed thrombosis of the PSS, and these were the only patients who had recurrent variceal bleeding. During 10 or more years of followup, 97% of the eligible patients were shown to have a patent shunt and were free of bleeding. No patient developed portal-systemic encephalopathy, liver function tests remained normal, liver biopsies in 100 patients showed normal architecture, hypersplenism was corrected. CONCLUSION PSS is the only consistently effective therapy for bleeding esophagogastric varices from PVT and extrahepatic portal hypertension, resulting in many years of survival, freedom from recurrent bleeding, normal liver function, and no encephalopathy.

Treatment of Budd-Chiari syndrome due to inferior vena cava occlusion by combined portal and vena caval decompression☆☆☆

This study concerns Budd-Chiari syndrome (BCS) caused by occlusion of the subdiaphragmatic inferior vena cava (IVC). It describes the experimental and clinical evaluation of the treatment of this disorder by one-stage combined portal and vena caval decompression with a direct side-to-side portacaval shunt (PCS) and a caval-atrial shunt (CAS) graft. BCS was produced in rats by gradual occlusion of the suprahepatic IVC with an ameroid constrictor. When ascites and portal hypertension were established, 12 control rats survived a sham thoracolaparotomy, 16 rats survived a mesoatrial shunt, and 16 rats survived combined PCS and CAS graft. All control rats re-formed ascites and died within 2 months. Nine of 16 rats with mesoatrial shunt developed graft thrombosis, re-formed ascites, and died within 2 months. In contrast, only 2 of 16 rats that underwent combined PCS and CAS developed graft thrombosis, re-formed ascites, and died. Liver biopsies showed reversal of severe pathologic changes in rats with patent grafts. Clinical evaluation of combined PCS and CAS using a 20-mm ring-reinforced Gore-Tex graft has been undertaken in five patients with BCS and ascites, hepatosplenomegaly, intense hepatic congestion on biopsy, and angiography showing occlusion of both the IVC and hepatic veins. All five patients are alive and well 6 months to 7.5 years postoperatively with patent grafts, no ascites or need for diuretics, no encephalopathy, normal liver function, and reversal of liver pathology. It is concluded that combined PCS and CAS create a high-flow shunt that decompresses both the portal system and IVC, has a low incidence of graft thrombosis, has been consistently effective in relieving BCS caused by IVC occlusion, and appears to be superior to mesoatrial shunt.

Comparison of the metabolic control of diabetes achieved by whole pancreas transplantation and pancreatic islet transplantation in rats.

To compare the long-term effectiveness of whole pancreas transplantation and pancreatic islet transplantation in controlling the metabolic disorders of alloxan diabetes, metabolic studies were performed monthly for 2 years in 4 groups of highly inbred rats: (1) NC-116 nondiabetic controls; (2) DC-273 untreated alloxan-diabetic controls; (3) PDT-182 rats that received syngeneic pancreaticoduodenal transplants shortly after induction of diabetes with alloxan; and (4) IT-92 rats that received an intraportal injection of at least 1500, but usually 2000, syngeneic pancreatic islets shortly after induction of diabetes with alloxan. Whole pancreas transplantation maintained strict metabolic control throughout the 2 years of study. In group PDT, hyperglycemia was abolished; plasma glucose concentration was maintained tightly within the normal range; markedly depressed plasma insulin levels were raised to above normal; glucose tolerance tests had insulin levels above normal and glucose levels that increased less and declined more rapidly than normal; and body weight gain and growth approached normal. In contrast, pancreatic islet transplantation failed to maintain precise metabolic control. In group IT, plasma glucose concentration initially fell to normal but then was elevated significantly above normal beginning with the 3rd post-transplant month; plasma insulin level declined progressively after the 6th posttransplant month; glucose tolerance tests had a diabetic glucose tolerance curve as a result of a markedly deficient plasma insulin response; and body weight gain and growth were significantly less than in group PDT. The results of these long-term metabolic studies may explain the effectiveness of whole pancreas transplantation and the ineffectiveness of pancreatic islet transplantation in preventing diabetic nephropathy.

Portal vein thrombosis in cirrhosis with variceal hemorrhage

Organized thrombus in the main trunk of the portal vein was encountered in 85 (6.5%) or 1300 patients with cirrhosis and variceal hemorrhage who underwent direct portacaval shunt (PCS). The thrombus was successfully removed with restoration of portal blood flow in all patients by phlebothrombectomy and balloon catheter extraction. Of the 85 patients, 65 were among 400 unselected patients who underwent emergency PCS (16%), and 20 were among 900 selected patients who underwent elective PCS (2%). All patients were closely followed for at least 5 years. Patients with portal vein thrombosis (PVT) had more advanced liver disease than those without PVT, reflected preoperatively in significantly higher (P<0.01) incidences of ascites (75%), severe muscle wasting (52%), varices of very large size (94%), the hyperdynamic state (94%), severe hypersplenism with a platelet count of less than 50,000/mm3 (92%), and placement in Child’s class C (52%). Side-to-side PCS reduced the portal vein-inferior vena cava pressure gradient to a mean of 23 mm saline solution in patients with PVT, similar to the marked pressure reduction obtained in patients without PVT.PCS promptly stopped variceal bleeding in all patients in the emergency PCS group. Permanent prevention of recurrent variceal bleeding was successful in 95% of patients with PVT and more than 99% of patients without PVT. Survival rates were similar in patients with and without PVT. In patients with PVT, survival rates at 30 days and 1,5, 10, and 15 years following emergency PCS were 69%, 66%, 65%, 55%, and 51%, respectively, and following elective PCS were 95%, 90%, 70%, 65%, and 60%, respectively. Quality of life was similar in patients with a without PVT, respectively, were the incidences of recurrent encephalopathy (9% vs. 8%), alcohol abstinence (61% vs. 64%), improved liver function (68% vs. 62% to 75%), and return to work (52% vs. 56% to 64%). It was concluded that in patients with cirrhosis and variceal hemorrhage it is almost always possible to remove portal vein thrombus by means of phlebothrombectomy and then perform a direct PCS with results similar to those achieved in the absence of PVT.

Long-term results of radical esophagogastrectomy for bleeding varices due to unshuntable extrahepatic portal hypertension☆

This report describes the long-term results of one-stage total gastrectomy and distal two-thirds esophagectomy, with reconstruction by esophagojejunostomy (16 Roux-en-Y; 2 interposition), in 18 adult patients with recurrent variceal hemorrhage due to unshuntable extrahepatic portal hypertension (EHPH) from occlusion of all major tributaries of the portal venous system. The etiology of portal venous occlusion was unknown in 11 patients, abdominal trauma in 3, peritonitis in 3, and thrombotic coagulopathy in 1. Almost half of the patients had their first episode of bleeding in childhood, and 83% experienced bleeding before 40 years of age. The severity of the problem was reflected by frequent previous bleeding episodes (mean: 12.8, range: 4 to 21), a large cumulative requirement for blood transfusions (mean: 129 units, range: 28 to 247 units), repeated, costly hospital admissions (mean: 15, range: 4 to 24), and numerous previous unsuccessful operations (mean: 4.4, range: 1 to 14). Blood transfusions transmitted serum hepatitis to three patients and AIDS to one, for an incidence of 22%. Bleeding recurred after repetitive endoscopic sclerotherapy in 10 patients and after various operations in 16 (failed portal-systemic shunts in 9, splenectomy in 16, devascularization procedures in 13). All patients had large esophageal and gastric varices on endoscopy, normal liver function, and widespread portal venous occlusion on visceral angiography. Radical esophagogastrectomy was usually a long and arduous operation because of dense adhesions, extensive collateral veins, and a scarred, contracted bowel mesentery due to previous operations. All patients survived the operation and are currently alive. No patient has had recurrent bleeding during 1 to 26 years of follow-up (mean: 13.9 years, 7 or more years in 14 patients). Quality of life has been good. It is concluded that radical esophagogastrectomy is the only effective treatment of unshuntable EHPH and that the operation should be performed promptly when this disease, which is associated with high mortality, high morbidity, and high costs, is diagnosed.

Lifelong reversal of the metabolic abnormalities of advanced diabetes in rats by whole-pancreas transplantation

Evidence suggests that metabolic abnormalities are responsible for the widespread microvascular complications of insulin-dependent diabetes mellitus (IDDM). Interest in endocrine pancreas replacement therapy, including pancreas transplantation, is based on the hope that such treatment will reverse the complications of IDDM by providing more precise metabolic control than conventional therapy. To determine if whole pancreas transplantation is capable of reversing well-established metabolic abnormalities of diabetes mellitus (DM) and maintaining strict metabolic control for life, we performed monthly metabolic studies for 2 years in 141 nondiabetic control rats, 273 diabetic control rats with alloxan-induced DM, and 267 diabetic rats that received syngeneic whole pancreaticoduodenal transplants 6, 9, 12, 15, 18, and 21 months after induction of DM with alloxan. Whole-pancreas transplantation in rats with long-standing DM permanently reversed the metabolic disorders. Elevated plasma glucose concentrations were permanently reduced to normal, depressed plasma insulin levels were permanently increased to normal, elevations of BUN and serum creatinine were permanently normalized, and there was a striking gain in body weight. Hyperglycemia during glucose tolerance tests was of lesser magnitude and shorter duration than normal, as a result of greater-than-normal plasma insulin levels. The only abnormality the persisted was hyperglycemia and is of unknown significance. These results indicate that whole-pancreas transplantation produces the most complete and sustained correction of the metabolic abnormalities of experimental DM of any available therapeutic modality.

Unshuntable extrahepatic portal hypertension revisited: 43 years' experience with radical esophagogastrectomy treatment of bleeding esophagogastric varices.

BACKGROUND In 1994, the authors reported their experience with radical esophagogastrectomy for bleeding esophagogastric varices due to unshuntable extra-hepatic portal hypertension. Since then, the series has expanded from 22 to 44 patients. The aim of this study was to assess the validity of the previous observations and conclusions in the largest series with the longest follow-up. METHODS From 1968 to 2005, 44 patients with unshuntable extra-hepatic portal hypertension were treated by total gastrectomy and resection of the distal two thirds of the esophagus. Before referral, the patients experienced 4 to 24 episodes of variceal bleeding requiring a mean 130 U of blood transfusion, 15 hospital admissions, and 6 previous unsuccessful operations. RESULTS Transient postoperative complications occurred in 50% of patients. The survival rate is 100%, with no recurrence of variceal bleeding during 7 to 43 years of follow-up. Liver function and biopsy results have been normal. Quality of life has been excellent or good in 91%. Eighty-six percent have resumed employment or full-time housekeeping. CONCLUSIONS In unshuntable extra-hepatic portal hypertension, radical esophagogastrectomy is the only consistently effective treatment of variceal hemorrhage. Prompt use of this lifesaving procedure is warranted.

Randomized trials of endoscopic therapy and transjugular intrahepatic portosystemic shunt versus portacaval shunt for emergency and elective treatment of bleeding gastric varices in cirrhosis

IMPORTANCE Bleeding esophageal varices has been studied extensively, but bleeding gastric varices (BGV) has received much less investigation. However, BGV has been reported in ≤ 30% of patients with acute variceal bleeding. In our studies of 1,836 bleeding cirrhotics, 12.7% were bleeding from gastric varices. BGV mortality rate of 45-55% has been reported. The BGV literature has mainly involved retrospective case reports, often with short-term follow-up. OBJECTIVE We sought to describe the results of a prospective, randomized, controlled trial (RCT) in unselected, consecutive patients with BGV comparing endoscopic therapy (ET) with portacaval shunt (PCS; n = 518), and later comparing emergency transjugular intrahepatic portosystemic shunt (TIPS) with emergency portacaval shunt (EPCS; n = 70). DESIGN, SETTING, AND PARTICIPANTS Initially, our RCT involved 518 patients with BGV comparing ET with direct PCS regarding control of bleeding, mortality rate, and disability. When entry of patients ended, the RCT was expanded to compare emergency TIPS with EPCS (n = 70). This RCT of BGV was separate from our other RCTs of bleeding esophageal varices. INTERVENTIONS Initially, ET was compared with PCS. In the second part of our RCT, emergency TIPS was compared with emergency PCS (EPCS). MAIN OUTCOME MEASURES Outcomes were survival, control of bleeding, portal-systemic encephalopathy (PSE), quality of life, and direct costs of care. In the RCT of ET versus PCS, 28 and 30%, respectively, were in Child class C. In the expanded RCT of TIPS versus EPCS, 40 and 41%, respectively, were in Child class C. Permanent control of BGV was achieved in 97-100% of patients treated by emergency or elective PCS, compared with 27-29% by ET. TIPS was even less effective, achieving long-term control of BGV in only 6%. Survival rates after PCS were greater at all time intervals and in all Child classes (P < .001). Repeated episodes of PSE occurred in 50% of TIPS patients, 16-17% treated by ET, and 8-11% treated by PCS. Shunt stenosis or occlusion occurred in 67% of TIPS patients, in contrast with 0-2% of PCS patients. CONCLUSION These results support the conclusion that PCS is uniformly effective, whereas ET and TIPS are not very effective.