Liver transplantation in a randomized controlled trial of emergency treatment of acutely bleeding esophageal varices in cirrhosis.

BACKGROUND: Bleeding esophageal varices (BEV) in cirrhosis has been considered an indication for liver transplantation (LT). This issue was examined in a randomized controlled trial (RCT) of unselected, consecutive patients with advanced cirrhosis and BEV that compared endoscopic sclerotherapy (EST; n = 106) to emergency direct portacaval shunt (EPCS; n = 105). METHODS: Diagnostic work-up and treatment were initiated within 8 hours. Patients were evaluated for LT on admission and repeatedly thereafter; 96% underwent over 10 years of regular follow-up. The analysis was supplemented by 1300 unrandomized cirrhotic patients who previously underwent portacaval shunt (PCS) with 100% follow-up. RESULTS: In the RCT long-term bleeding control was 100% following EPCS, only 20% following EST. Also, 3-, 5-, 10-, and 15-year survival rates were 75%, 73%, 46%, and 46%, respectively, following EPCS compared with 44%, 21%, 9%, and 9% following EST, respectively (P < .001). Only 13 RCT patients (6%) were ultimately referred for LT mainly because of progressive liver failure; only 7 (3%) were approved for LT and only 4 (2%) underwent LT. The 1- and 5-year LT survival rates were 0.68% and 0, respectively, compared with 81% and 73%, respectively, after EPCS. In the 1300 unrandomized PCS patients, 50 (3.8%) were referred and 19 (1.5%) underwent LT. The 5-year survival rate was 53% compared with 72% for all 1300 patients. CONCLUSIONS: If bleeding is permanently controlled, as occurred invariably following EPCS, cirrhotic patients with BEV seldom require LT. PCS is effective first-line and long-term treatment. Should LT be required in patients with PCS, although technically more demanding, numerous studies have shown that PCS does not increase mortality or complications. EST is not effective emergency or long-term therapy.

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

OBJECTIVE: To 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 DATA: Experiments 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. METHODS: From 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. RESULTS: In 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. CONCLUSIONS: SSPCS 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.

Adjunctive 3D US for achieving portal vein access during transjugular intrahepatic portosystemic shunt procedures.

PURPOSE: To evaluate the usefulness of information provided by three-dimensional ultrasound (3D US) and to determine whether 3D US decreased the number of passes required to obtain portal vein (PV) access during creation of transjugular intrahepatic portosystemic shunts (TIPS). MATERIALS AND METHODS: Intermittent 3D US volume acquisitions were obtained during creation of TIPS in 20 patients. Useful information provided by 3D US was tabulated. The number of passes required to achieve PV access was recorded and results were compared retrospectively to 25 patients who underwent TIPS without 3D US. RESULTS: 3D US documented that the operator's opinion of which hepatic vein had been selected was incorrect in nine patients (45%), detected unfavorable PV anatomy that required modification of equipment or technique in seven patients (35%), permitted estimation of the trajectory required to access the targeted PV in all patients (100%), assisted in selecting the optimal point along the hepatic vein for origination of the needle pass in 11 patients (55%), allowed avoidance of a large hepatocellular carcinoma in one patient (5%), and confirmed that access into the main PV was intrahepatic in four patients (20%). The mean number of needle passes decreased from 10.4 in the historic control group to 4.6 in the 3D US group (P = .0001). CONCLUSION: 3D US provided imaging information that detected technical errors and altered anatomy, and provided positional and directional information to significantly improve needle pass efficiency.

Adjunctive 3D US for achieving portal vein access during transjugular intrahepatic portosystemic shunt procedures.

PURPOSE: To evaluate the usefulness of information provided by three-dimensional ultrasound (3D US) and to determine whether 3D US decreased the number of passes required to obtain portal vein (PV) access during creation of transjugular intrahepatic portosystemic shunts (TIPS). MATERIALS AND METHODS: Intermittent 3D US volume acquisitions were obtained during creation of TIPS in 20 patients. Useful information provided by 3D US was tabulated. The number of passes required to achieve PV access was recorded and results were compared retrospectively to 25 patients who underwent TIPS without 3D US. RESULTS: 3D US documented that the operator's opinion of which hepatic vein had been selected was incorrect in nine patients (45%), detected unfavorable PV anatomy that required modification of equipment or technique in seven patients (35%), permitted estimation of the trajectory required to access the targeted PV in all patients (100%), assisted in selecting the optimal point along the hepatic vein for origination of the needle pass in 11 patients (55%), allowed avoidance of a large hepatocellular carcinoma in one patient (5%), and confirmed that access into the main PV was intrahepatic in four patients (20%). The mean number of needle passes decreased from 10.4 in the historic control group to 4.6 in the 3D US group (P = .0001). CONCLUSION: 3D US provided imaging information that detected technical errors and altered anatomy, and provided positional and directional information to significantly improve needle pass efficiency.

Lifelong prevention of mesangial enlargement by whole pancreas transplantation in rats with diabetes mellitus.

BACKGROUND: Mesangial enlargement (ME) is one of the hallmark lesions of diabetic nephropathy and plays a major role in diabetic renal failure. Conventional treatment of type 1 diabetes mellitus with insulin injections, diet, and medications has often failed to prevent development and progression of ME, presumably because of difficulty in achieving tight metabolic control. Although many pancreas transplantations have been done in patients with type 1 diabetes mellitus, there is insufficient information about their influence on ME or other diabetic lesions that are responsible for its morbidity and mortality. HYPOTHESIS: Whole pancreas transplantation will prevent diabetic ME throughout the life of the rat with alloxan-induced diabetes mellitus. DESIGN: Mesangial enlargement was studied for 28 months by a highly reproducible quantitative morphologic method in 55 nondiabetic control rats, 57 control rats with alloxan-induced diabetes mellitus, 97 diabetic rats that received a pancreaticoduodenal isograft shortly after the induction of diabetes mellitus, and 126 diabetic rats that received a duct-ligated pancreas isograft shortly after the induction of diabetes mellitus. Mesangial enlargement was determined by measuring the area occupied by camera lucida tracings of the mesangium using an electronic planimeter connected to a computer. RESULTS: Monthly metabolic studies showed that whole pancreas transplantation maintained very tight metabolic control of diabetes mellitus. Alloxan-induced diabetes mellitus produced progressive accumulation of mesangial matrix and progressive enlargement of all elements of the mesangium during the study. The 2 types of whole pancreas transplants provided lifelong protection against abnormal ME (P = .006). CONCLUSIONS: These results, combined with our previous finding of lifelong prevention of abnormal glomerular capillary basement membrane thickening, demonstrate that whole pancreas transplantation performed early in the course of alloxan-induced diabetes mellitus is capable of preventing diabetic kidney lesions. Moreover, these results suggest that whole pancreas transplants might be useful preventive therapy in patients with diabetes mellitus who undergo kidney transplantation for renal failure, in whom recurrence of nephropathy often develops in the transplanted kidney.

Budd-Chiari syndrome caused by Behçet's disease: treatment by side-to-side portacaval shunt.

BACKGROUND: Behçet's disease is a chronic multisystem vasculitis of unknown etiology that involves skin, mucous membranes, eyes, blood vessels, joints, central nervous system, digestive system, and occasionally other organs. Budd-Chiari syndrome from occlusion of the major hepatic veins is a rare and serious complication of Behçet's disease. Although the mortality rate of Behçet's disease is only 3% to 4%, development of Budd-Chiari syndrome in patients with Behçet's disease has been associated with a mortality rate of 61%. This report presents the largest reported experience of Behçet's disease-related Budd-Chiari syndrome confined to the hepatic veins, and results of treatment by side-to-side portacaval shunt (SSPCS). These results are compared with those we have obtained in Budd-Chiari syndrome confined to the hepatic veins without Behçet's disease, and with results of treatment of Budd-Chiari syndrome in Behçet's disease reported in the literature. STUDY DESIGN: SSPCS was performed in 5 patients with Behcet's disease who had developed acute Budd-Chiari syndrome, and 27 patients with Budd-Chiari syndrome from other causes. In all patients, Budd-Chiari syndrome was confined to the hepatic veins without involvement of the inferior vena cava (IVC). Patients were studied prospectively and were followed up at regular intervals for from 1.5 to 26 years (mean 10.6 years, 81% more than 5 years). Followup was 100%. Patients were mainly young adults; mean age was 24.6 years in the patients with Behçet's disease and 30.0 years in those without Behçet's disease. All patients had massive ascites, abdominal pain, hepatosplenomegaly, and abnormal liver function. Diagnosis was based on angiographic demonstration of occlusion of the major hepatic veins, and liver biopsy findings of intense hepatic congestion and necrosis. SSPCS was performed within 4 months of the onset of Budd-Chiari syndrome in all but 3 patients. Every year or two in followup, patients underwent liver biopsy and evaluation of SSPCS by Doppler duplex ultrasonography and angiography with pressure measurements. Outcomes criteria included mortality rate, SSPCS patency, maintenance of portal decompression, liver function, presence of ascites, presence of portal-systemic encephalopathy (PSE), need for diuretics, quality of life, and return to work. Our results were compared with those reported in the literature in 42 patients who had Budd-Chiari syndrome with Behçet's disease. RESULTS: SSPCS permanently reduced the mean portal vein-IVC pressure gradient (mm saline) from 205 to 7 in the 5 patients with Behçet's disease, and from 250 to 4 in the 27 without Behçet's disease. There was only one operative death, a patient without Behcet's disease. One patient with Behçet's disease died 2 years postoperatively from diffuse vasculitis, a complication of Behçet's disease, and the other 4 (80%) remain alive. All 26 operative survivors in the group without Behçet's disease (96%) are alive. Only one patient developed occlusion of the SSPCS, a man without Behçet's disease, and he required liver transplantation as a result of hepatic decompensation, PSE, and recurrent ascites. All other patients with or without Behçet's disease remained free of ascites, required no diuretics, were free of PSE, and had reversal of hepatic dysfunction. Serial liver biopsies showed normal architecture in 60% of patients with Behçet's disease and 46% of those without Behçet's disease. Return to fulltime work or housekeeping occurred in 80% of patients with Behçet's disease and 96% without Behçet's disease. Comparison of outcomes of our patients with 42 cases of Behçet's disease with Budd-Chiari syndrome reported in the literature, 79% of whom were treated medically, showed striking differences with an overall mortality rate of 61% in generally shortterm followup. (ABSTRACT TRUNCATED)

Experimental, clinical, and metabolic results of side-to-side portacaval shunt for intractable cirrhotic ascites.

BACKGROUND: Intractable ascites, refractory to medical therapy, occurs in approximately 10 percent of patients with ascites from cirrhosis and is almost always fatal. Sinusoidal hypertension resulting from hepatic venous outflow obstruction plays a primary role in the pathogenesis of cirrhotic ascites and provides the rationale for decompression of the liver by side-to-side portacaval shunt in treatment of intractable ascites. This report presents the experimental basis for the use of side-to-side shunt and long-term results of a prospective study in 34 selected patients with intractable cirrhotic ascites. STUDY DESIGN: In the experimental studies, hepatic venous outflow obstruction and massive ascites were produced in dogs by ligation of the hepatic veins, and the effect of portacaval shunts on ascites, thoracic duct lymph flow, and aldosterone secretion were measured. In the clinical study, 34 carefully selected patients with cirrhosis (91 percent alcoholic) and truly intractable ascites (failure of medical therapy for 5 to 24 months) underwent side-to-side portacaval shunt. The effects on ascites, survival, metabolic abnormalities, and quality of life were studied prospectively during follow-up that was longer than 5 years in all but two patients. Quantitative Child's risk classes in percent of patients were A in 0, B in 68, and C in 32. RESULTS: In the experimental studies, side-to-side portacaval shunt permanently relieved severe ascites, reduced the 13-fold increase in thoracic duct lymph flow rate to almost normal, and abolished the aldosterone hypersecretory response to minimal hepatic venous outflow obstruction. End-to-side portacaval shunt was much less effective. In the clinical study, side-to-side portacaval shunt reduced mean portal vein-inferior vena cava pressure gradient from 282 mm saline to 4 mm and permanently relieved all patients of ascites without subsequent requirement of diuretic therapy. Two patients who died of hepatoma, and one who died of heart failure developed terminal ascites. Thirty-day mortality rate was 6 percent, and long-term survival rates at 5, 10, and 15 years were 75 percent, 74 percent, and 73 percent. In metabolic studies, side-to-side shunt produced marked diuresis and natriuresis and abolished hypersecretion of aldosterone. Quality of life was generally improved as a result of a low incidence of recurrent portal-systemic encephalopathy (6 percent), abstinence from alcohol in 91 percent, improvement in liver function in 81 percent, and improvement in Child's risk class. The portacaval anastomosis remained permanently patent in every patient. CONCLUSIONS: Side-to-side portacaval shunt is very effective treatment of intractable ascites from cirrhosis. Our results are attributable to careful selection of patients, an organized system of care, and a program of rigorous, lifelong follow-up that emphasizes abstinence from alcohol and dietary protein restriction.

Portal vein thrombosis in cirrhosis with variceal hemorrhage.

Organized thrombus in the main trunk of the portal vein was encountered in 85 (6.5%) of 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 and without PVT. Long-term PCS patency was demonstrated yearly in 93% of patients in the group with PVT and in 99.7% of patients without PVT. Other similarities after 5 years between patients with and 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.

Treatment of bleeding from portal hypertensive gastropathy by portacaval shunt.

Portal hypertensive gastropathy is a vascular disorder of the gastric mucosa distinguished by ectasia of the mucosal capillaries and submucosal veins without inflammation. During 1988 to 1993, 12 patients with biopsy-proven cirrhosis (10 alcoholic, 2 posthepatitic) were evaluated and treated prospectively by portacaval shunt for active bleeding from severe portal hypertensive gastropathy. Eleven patients had been hospitalized for bleeding three to nine times previously, and one was bleeding uncontrollably for the first time. Requirement for blood transfusions ranged from 11 to 39 units cumulatively, of which 8 to 30 units were required specifically to replace blood lost from portal hypertensive gastropathy. Admission findings were ascites in 9 patients, jaundice in 8, severe muscle wasting in 10, hyperdynamic state in 9. Child's risk class was C in 7, B in 4, A in 1. Ten of the 12 patients had previously received repetitive endoscopic sclerotherapy for esophageal varices, which has been reported to precipitate portal hypertensive gastropathy. Eight patients had failed propranolol therapy for bleeding. Portacaval shunt was performed emergently in 11 patients and electively in 1, and permanently stopped bleeding in all by reducing the mean portal vein-inferior vena cava pressure gradient from 251 to 16 mm saline. There were no operative deaths, and two unrelated late deaths after 13 and 24 months. During 1 to 6.75 years of follow-up, all shunts remained patent by ultrasonography, the gastric mucosa reverted to normal on serial endoscopy, and there was no gastrointestinal bleeding. Recurrent portal-systemic encephalopathy developed in only 8% of patients. Quality of life was generally good.(ABSTRACT TRUNCATED AT 250 WORDS)

Three decades of experience with emergency portacaval shunt for acutely bleeding esophageal varices in 400 unselected patients with cirrhosis of the liver.

BACKGROUND: Emergency treatment of acute bleeding is of singular and paramount importance in the therapy of portal hypertension and esophagogastric varices. Accordingly, for more than three decades we have conducted prospective studies of emergency therapy, and particularly of emergency portacaval shunt (EPCS). STUDY DESIGN: Emergency portacaval shunt was performed upon 400 patients with cirrhosis of the liver and acutely bleeding esophagogastric varices according to three principles: operation within eight hours of initial contact; unselected patients, meaning that no patient with variceal bleeding caused by hepatic disease was excluded from EPCS, and prospective study, meaning that a well-defined protocol was consistently used and data were collected on-line. Patients were divided into an early group of 180 treated from 1963 to 1978 and a recent group of 220 treated from 1978 to July, 1990, with similar characteristics, but strikingly different outcome. Follow-up rates at one, five, and ten years were 100, 98, and 97 percent, respectively; 96 percent of patients underwent EPCS five or more years ago. Proof of acute variceal bleeding and of cirrhosis of the liver (alcoholic in 95 percent) was obtained in every patient. Child's risk classes determined quantitatively were A in 11 percent of the patients, B in 65 percent, and C in 24 percent. All patients had a direct portacaval shunt, side-to-side in 85 percent, which reduced the mean portal vein to inferior vena cava pressure gradient from 271 to 21 mm saline solution. RESULTS: All but four patients (99 percent) had immediate and permanent control of variceal bleeding. Thrombosis of the shunt occurred in only two patients (0.5 percent). Survival rates at 30 days, five years, ten years, and 15 years in the early group were 58, 40, 30, and 30 percent, respectively, while in the recent group they were 85, 78, 71, and 57 percent, respectively (p < 0.0001). Other striking gains in the recent group were abstention from alcohol, improvement in liver function and improvement in Child's class, all in 70 percent of patients. Recurrent portal-systemic encephalopathy occurred in 9 percent of the early group and 8 percent of the recent group. CONCLUSIONS: Emergency portacaval shunt substantially improved survival and quality of life of patients with cirrhosis of the liver and bleeding varices. Our results are attributable to rapid and simplified diagnosis, prompt operation, an organized system of care, and rigorous, lifelong follow-up evaluation that emphasized abstinence from alcohol and dietary protein control. Transplantation of the liver is infrequently required in patients whose bleeding is permanently controlled.

Prospective randomized trial of emergency portacaval shunt and emergency medical therapy in unselected cirrhotic patients with bleeding varices.

A prospective randomized trial was conducted in unselected, consecutive patients with bleeding esophageal varices resulting from cirrhosis comparing (1) emergency portacaval shunt performed within 8 hr of initial contact (21 patients) with (2) emergency medical therapy (intravenous vasopressin and esophageal balloon tamponade) followed in 9 to 30 days by elective portacaval shunt in survivors (22 patients). All patients underwent the same diagnostic workup within 3 to 6 hr of initial contact, and received identical supportive therapy initially. All patients were followed up for at least 10 yr. The protocol contained no escape or cross-over provisions. There were no statistically significant differences between the two treatment groups in the incidence of any of the clinical variables, results of laboratory tests or degree of portal hypertension. Child's risk classes in the shunt group were A-2 patients, B-8 patients and C-11 patients, whereas in the medical group they were A-10 patients, B-5 patients, and C-7 patients, a significant difference (p < 0.01) that might have favored emergency medical treatment. Bleeding was controlled initially and permanently by emergency shunt in every patient, but by medical therapy in only 45% (p < 0.001). Mean requirement for blood transfusion was 7.1 +/- 2.6 units in the shunt group and 21.4 +/- 2.6 units in the medical group (p < 0.001). Eighty-one percent of the patients in the shunt group were discharged alive compared with 45% in the medical group (p = 0.027). Five- and 10-yr observed survival rates were 67% and 57%, respectively, after emergency shunt compared with 18% and 18%, respectively, after the combination of emergency medical therapy and elective shunt (p < 0.01). These survival rates produced by emergency shunt performed within 8 hr of initial contact confirm the effectiveness of this procedure observed in our previous unrandomized studies.

Free jejunal autograft combined with extensive esophagogastrectomy for unshuntable extrahepatic portal hypertension.

This is the first report of the use of a free jejunal autograft vascularized by the internal thoracic (internal mammary) artery and vein to restore continuity of the digestive tract after total gastrectomy and distal 65% esophagectomy for recurrent bleeding esophagogastric varices caused by unshuntable extrahepatic portal hypertension. The procedure was used in two young adults who, because of numerous previous abdominal operations, had a severely scarred and contracted intestinal mesentery that precluded conventional use of the small or large intestine with an intact blood supply to bridge the gap between the upper thoracic esophagus and the abdominal jejunum. Before referral, the two patients had 21 and eight bouts of variceal hemorrhage, respectively, that necessitated a cumulative total of 108 and 74 units of blood transfusion, necessitated 17 and 12 admissions to the hospital, and failed to respond to four and five operations and 14 and 18 sessions of endoscopic sclerotherapy. After extensive esophagogastrectomy combined with a free jejunal autograft, both patients have done well during follow-up of 9 and 3 years, respectively. Both have been in good to excellent health with stable weight, freedom from digestive tract bleeding, normal liver function, and no encephalopathy. These results confirm our recently reported conclusions regarding the uniform long-term effectiveness of extensive esophagogastrectomy in the treatment of unshuntable extrahepatic portal hypertension and suggest that thoracic and general surgeons familiar with microvascular techniques may find the free jejunal autograft to be useful in various circumstances in which it is necessary to replace all or a substantial part of the thoracic esophagus.

Treatment of bleeding esophagogastric varices due to extrahepatic portal hypertension: results of portal-systemic shunts during 35 years.

From 1958 to 1990, elective therapeutic portal-systemic shunt (PSS) procedures were performed for recurrent bleeding esophagogastric varices in 162 children and adults with extrahepatic portal hypertension (EHPH) resulting from portal vein thrombosis (PVT). The onset of EHPH was in childhood for at least 74% of patients. Of the 162 patients, 147 were eligible for and received 5 years of follow-up (100%), and 117 were eligible for and received 10 years of follow-up (100%). The longest follow-up was 35 years. The cause of PVT was unknown in 68%, neonatal omphalitis in 12%, umbilical vein catheterization in 8%, peritonitis in 6%, trauma in 4%, and thrombotic coagulopathy in 2%. The number of variceal bleeding episodes ranged from 2 to 18 (mean, 5.6). None of the patients had clinical, biochemical, or liver biopsy evidence of liver disease. Esophageal varices were demonstrated by endoscopy, and/or contrast x-rays, and/or angiography in all patients. Visceral angiography was always used to demonstrate the extent of portal obstruction and the veins available for shunting. Before referral, the following procedures had failed: endoscopic sclerotherapy (68 patients), splenectomy alone (32 patients), central splenorenal shunt with splenectomy (10 patients), transesophageal varix ligation (12 patients). Three types of PSS were used: (1) central side-to-side splenorenal without splenectomy (75 patients, 46%); (2) central end-to-side splenorenal with splenectomy (34 patients, 21%); and (3) mesocaval (end-to-side cavomesenteric) (53 patients, 33%). PSS reduced the mean corrected portal pressure from 292 to 28 mm saline. All patients survived the procedure and left the hospital (100%). The actuarial survival rate for 5 years is 99%, and for 10 years is 96%. Three of the 6 deaths were unrelated to EHPH or PSS. Shunt patency for up to 35 years was demonstrated in 98% of patients by angiography and/or ultrasonography. In four patients (2%), all of whom had end-to-side splenorenal shunts, shunt thrombosis and rebleeding developed 3, 4, 4, and 6 years (respectively) after PSS. There were the only patients who experienced rebleeding. A diligent and repeated effort was made to detect portal-systemic encephalopathy (PSE), and no instance of PSE was found during 3 to 35 years of follow-up. Liver function and morphology remained normal, and hypersplenism was corrected in all patients. Quality of life was good in 98% of patients, and 5 years after PSS 96% were gainfully employed, engaged in full-time homemaking, or attending school.(ABSTRACT TRUNCATED AT 400 WORDS)

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.