Emergency portosystemic shunt in patients with variceal bleeding

Thirty-five patients for whom emergency sclerotherapy or conservative treatment, or both, failed to arrest variceal bleeding, or who had early rebleeding and required emergency portosystemic shunts (EPSS) were studied. EPSS permanently controlled the variceal bleeding in all but one patient. In this patient, the shunt was patent as demonstrated by angiography. Esophageal varices disappeared in 18 patients and were reduced in 14. Three patients died before the endoscopic examination could be performed. The causes of death were hepatic failure in two and bleeding ulcerations of the gastric fundus in the other patient. One patient was classified in Child's category B and two in Child's category C. Thirty-two patients submitted to EPSS and were discharged alive. Twelve of these patients subsequently died, at an average of 11.2 months after undergoing the shunt procedure. Four of 12 patients died of hepatic failure; two patients died of hepatomas; two, other neoplasia; three, hemorrhaging duodenal ulcers, and one patient, renal failure. Analysis of actuarial survival rates showed that the five year survival rate was 43 per cent. The long term survival rates were fewer for patients with Child's category C than for those with combined Child's categories A and B (five year survival rates were 21 versus 55 per cent; p less than 0.05). During the follow-up period, none of the patients had variceal bleeding. Chronic encephalopathy developed in six, which was mild in three, moderate in one instance and severe in two. It developed soon after EPSS, with onset in the first month after discharge in three. Thus, when conservative treatment fails to arrest variceal bleeding, EPSS should be performed to guarantee definitive control of hemorrhage and prolong the survival period.     

The successful management of portal vein thrombosis after hepatic transplantation with a splenorenal shunt

Portal vein thrombosis is an infrequent complication after hepatic transplantation, but is quite dramatic when it occurs. It is usually managed by retransplantation with a significant mortality rate. We present a patient in whom portal vein thrombosis after hepatic transplantation was ultimately managed by a splenorenal shunt. The portal vein thrombosis was manifested by bleeding esophageal varices and, yet, normal hepatic function obviated the need for a new graft (one was not readily available). To the best of our knowledge, this is the first presentation of a patient with a transplant of the liver with acute portal vein occlusion and maintained hepatic function who has been successfully managed by a portosystemic shunt.     

The Denver type for peritoneovenous shunting of malignant ascites

Peritoneovenous shunts of the Denver type were inserted into 36 patients to control malignant ascites. The Denver system features a compressible pump chamber bearing a pressure sensitive valve. Initially, all the shunts functioned well. The shunt remained open until death in 21 patients, and at the beginning of the analysis, another two patients were still alive with an open shunt. Blockage of the shunt occurred in 13 patients before death. The cumulative survival time for patients after shunt insertion was 129 months and the cumulative shunt functioning time was 92 months. The over-all median survival time after shunt installation was 13 weeks, and calculated actuarially, the median shunt functioning time for long term survivors was 14 weeks. The cytologic state of the ascitic fluid did not make a statistically significant difference to the blockage-free interval (p = 0.99), neither did the type of primary tumor (p = 0.37). Complications were of a minor type. There was no laboratory or clinical evidence of disseminated intravascular coagulation. Tumor spread through the tubing was seen in one of the three autopsies performed. Denver type peritoneovenous shunting appears to provide effective palliation in the majority of patients. It should, however, only be performed as a last resort.     

The Sugiura procedure for patients with hemorrhagic portal hypertension secondary to extrahepatic portal vein thrombosis

The Sugiura procedure (SP) was performed upon 27 patients with hemorrhagic portal hypertension secondary to extrahepatic portal vein thrombosis (EPVT) without associated hepatic disease. There were 14 female and 13 male patients. The mean age was 28 +/- 14 years. The causes of EPVT were protein C deficiency in two; antithrombin III deficiency in one patient, a history of omphalitis in two patients, a history of pancreatitis in one patient and idiopathy in 21 patients. The SP was completed in two surgical stages in 14 patients and in one stage in nine. There was one operative death. One patient had mild postoperative encephalopathy, and two patients rebled at long term follow-up study. Actuarial survival rate was 82 per cent at five and ten years. It is concluded that the SP is a good alternative for the management of hemorrhagic portal hypertension secondary to EPVT.     

Long term results of treatment of Budd-Chiari syndrome by side to side portacaval shunt

This is a report of a long term prospective study of 13 seriously ill patients with Budd-Chiari syndrome as a result of occlusion of the hepatic veins who were treated by side to side portacaval shunt from four to 78 weeks after the onset of symptoms and who were under observation for three to 16 years. The patient population was young, ranging in age from 19 to 45 years; seven were men and six were women. The presumed cause was the use of oral contraceptives in three, polycythemia rubra vera in two, Behcet disease in one patient and unknown in seven patients. All of the 13 patients had abdominal pain, marked ascites, hepatosplenomegaly, wasting and disturbed liver function. Diagnosis was based on the symptoms and signs: angiographic demonstration of hepatic vein occlusion and a patent inferior vena cava; pressure measurements that showed an inferior vena caval pressure that was normal or within the usual range for patients with massive ascites and an elevated wedged hepatic vein pressure that was much higher than the inferior vena caval pressure, and the results of biopsy of the liver showing centrilobular congestion and necrosis. Side to side portacaval shunt was very effective in decompressing the liver, reducing the mean corrected portal pressure from 240 millimeters of saline solution before to 7 millimeters of saline solution after the shunt. Operative survival rate was 92 per cent, and the long term survival rate for three to 16 years is 85 per cent. All of the survivors are free of ascites without requiring diuretic therapy.(ABSTRACT TRUNCATED AT 250 WORDS)     

妊娠合并布-加综合征

布-加综合征(BCS)以淤血性门静脉高压(portal hypertension,PHT)或门静脉和下腔静脉高压为特征。妊娠合并布-加综合征少见,但凶险。文章就BCS的诊断、与妊娠的相互影响及处理等方面进行讨论。     

Effect of portasystemic shunts on subsequent transplantation of the liver

Portasystemic (PS) shunts have been regarded as a relative contraindication to hepatic transplantation (HT) because of the potential for increased technical difficulties during the transplant operation. We compared operative blood loss, morbidity and mortality in 27 patients with PS shunts and 147 patients with no shunts (NS) who underwent HT. The PS shunt group included 12 portocaval (PC), eight mesocaval, four central splenorenal and four distal splenorenal shunts. The PS shunt and NS groups were similar with respect to age, preoperative medical status and ABO blood group matching between donors and recipients. There were no significant differences in the mean (plus or minus S.D.) intraoperative blood transfusion (9.1 +/- 7.6 versus 9.2 +/- 11.0 units), mean (plus or minus S.D.) duration of anesthesia (8.1 +/- 1.4 versus 7.8 +/- 1.5 hours) and operative mortality (7 versus 11 per cent) between the PS shunt and NS groups, respectively. Complications of the biliary tract were significantly higher in the PS shunt group (22.0 versus 5.4 per cent, p less than 0.01) but they did not increase the mortality rate. We conclude that a prior PS shunt should not influence the decision to accept patients for HT. PS shunts remain a reasonable surgical option for patients with cirrhosis and variceal hemorrhage (refractory to sclerotherapy) who, by virtue of good hepatic function, do not merit immediate HT.     

Cerebral venous thrombosis and hepatic venous thrombosis during pregnancy

Cerebral venous thrombosis (CVT) and Budd-Chiari syndrome (BCS) are rarely encountered thrombotic diseases of two different vascular systems. A 20-year-old woman in the 14th week of pregnancy was brought to the emergency department with complaints of sudden headache and loss of vision. Thrombosis in the left transverse and sigmoid sinus was demonstrated at MR angiography. She was diagnosed with BCS by using hepatic Doppler ultrasonography after an elevation of liver enzymes. Although CVT and BCS are rarely seen during pregnancy, they need emergency treatment because of high mortality.     

Surgical approaches for unresectable primary carcinoma of the hepatic hilus

Thirteen patients with proximal malignant obstruction of the biliary tract secondary to primary cholangiocarcinoma are presented. Seven of these patients had bilateral involvement of the major vessels of the porta hepatis, and 12 had bilateral involvement of the main hepatic ducts up to the level of segmental bifurcation. Resection of the tumor was carried out in all of the patients. Extensive resection of the liver with resection of the regional vascular structures was done in six. Another six underwent resection of the liver without resection of the regional vascular structures. The remaining patient underwent resection of the tumor and of the regional revascular structures alone. Vascular resection included resection and reconstruction of both the portal vein and hepatic artery in four patients or only of the portal vein in three. Biliary drainage was carried out by intrahepatic cholangiojejunostomies between segmental hepatic ducts and a Roux-en-Y loop. Two patients died and ten are alive. The quality of postoperative life is satisfactory and the patients are free of sequelae and symptoms secondary to operation or disease. This approach is recommended for the management of a number of patients with bilateral vascular or biliary involvement secondary to primary cholangiocarcinoma of the porta hepatis. For those patients, this approach offers a promising alternative.     

HELLP Syndrome as an indication for liver transplantation]

Liver rupture followed by multiorgan failure is perhaps the most catastrophic complication of the HELLP syndrome (hemolysis, elevated liver function, low platelets). Specific treatment options are currently limited and not routinely clarified. Here, we present a patient successfully managed by an innovative surgical approach consisting of combined total hepatectomy, portacaval shunt, and liver transplantation. A 26-year-old primipara (39th week of gestation) who suffered liver rupture as a complication of HELLP syndrome after delivery underwent a portacaval shunt after total hepatectomy. This combination was sufficient until the patient underwent orthotopic liver transplantation. The patient was discharged from the hospital after a dramatic recovery. Bridging portacaval shunt and consecutive orthotopic liver transplantation represented an effective therapy for this patient and should be considered early as a treatment option in patients with liver rupture complicating severe HELLP syndrome.     

Two-stage surgical management of Budd-Chiari syndrome with obstruction of the inferior vena cava

A two-stage approach to the surgical management of acute Budd-Chiari syndrome complicated by inferior vena caval obstruction was advocated by our group in 1984. This entailed initial hepatic decompression by suprahepatic, mesoatrial shunt, with subsequent takedown of the mesoatrial shunt combined with conversion to a short infrahepatic, portacaval shunt. We report herein the late follow-up results for two patients managed in this manner. While both patients are alive and doing well, both of the courses have been complicated by stenosis of the inferior vena cava. The cause is unclear but probably relates to fibrosis at the hepatic venous orifices. Management was by percutaneous balloon dilation, which relieved the recurrent hepatic congestion. This cautions others considering this approach to provide careful longitudinal follow-up study for such patients.     

Portosystemic shunts for extrahepatic portal hypertension in children

Twenty-three children with prehepatic portal hypertension and hemorrhage due to ruptured esophagogastric varices had portosystemic shunts. Their ages ranged from two years and seven months to 15 years. Eleven were less than eight years of age. Twenty patients had portal vein cavernomatosis and three patients had double portal veins. In 21 patients, a mesocaval type of shunt was done. A splenorenal shunt was performed in two. There was no surgical mortality. Two shunts occluded, both in rather young infants--two years and seven months and three years of age. In all the others, there was no further bleeding, and the shunts remained patent, as shown by abdominal angiograms. Neuropsychiatric disorders, probably due to hepatic encephalopathy, occurred in only one patient. On the basis of this favorable experience, we believe that an elective portosystemic shunt should, in general, be performed upon children with prehepatic portal hypertension after one major variceal hemorrhage. We favor a mesocaval type of shunt in these children because of the larger diameter of the vessels involved in the anastomosis and because it preserves the spleen, maintaining defense against subsequent infection.     

Prospective comparison of partial versus total portal decompression for bleeding esophageal varices.

Eighty-six patients underwent portacaval shunt (PCS) to treat bleeding esophagogastric varices during a period of four years. Twenty-eight patients (group 1) underwent emergency total portal decompression, while 58 patients (group 2) underwent elective partial PCS. Age, gender, preshunt and postshunt alcohol consumption and modified Child-Pugh classification at the time of operation, and at latest follow-up evaluation, did not differ significantly between the two groups. Early mortality was higher after emergency shunts than after elective operation (p < 0.01). However, partial portal decompression, when compared with total shunt, resulted in a significantly lower likelihood of late mortality (13 versus 39 percent) (p < 0.05), as well as portasystemic encephalopathy (8 versus 56 percent) (p < 0.0005). All shunts remained patent postoperatively and no patient had variceal rebleeding during follow-up evaluation averaging 2.2 years. Duplex sonography demonstrated hepatofugal portal flow in all patients in both groups. The results of the current study suggest that partial portal decompression is technically feasible, prevents further variceal hemorrhage and confers significant protection against late mortality and the development of postshunt neuropsychologic dysfunction.     

Effect of Corynebacterium parvum in prevention and reversal of atrophy of the liver following portacaval shunt.

Since it has been demonstrated that the administration of Corynebacterium parvum is associated with increased hepatocyte proliferation in both normal and regenerating livers, it seemed appropriate to determine whether the use of this agent would affect the hepatic atrophy which occurs following end-to-side portacaval shunts. When administered at shunting, liver weight and liver deoxyribonucleic acid failed to decrease to the same extent as that occurring in untreated, shunted rats. An increase in liver deoxyribonucleic acid synthesis was observed following portacaval shunt only in treated rats. Corynebacterium parvum administration to portacaval shunt rats with livers demonstrating marked atrophy 15 to 18 days following shunting resulted in the reversal of such atrophy. There was an increase in liver weight and deoxyribonuclei acid to the extent that those values were equal to, or greater than, those of livers from nonshunted rats. By increasing the number of cells within liver, Corynebacterium parvum administration prevents or reverses the decrease in liver size, that is, atrophy, which occurs after portacaval shunts were performed.     

Intraoperative direct portography to achieve selective distal splenorenal 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.     

Interposition mesocaval shunt for chronic primary occlusion of the hepatic veins.

Five patients with primitive chronic Budd-Chiari syndrome were treated by Dacron interposed mesocaval shunts for medically uncontrollable ascites. In two instances, hepatomegaly and ascites disappeared for four and four and one-half years. In one patient with severe stenosis of the inferior vena cava, moderate ascites required tapping once a month one year later, despite proved prosthesis patency. In two patients, death occurred ten and 30 days after shunting due to thrombosis of the graft. Inferior vena cava stenosis appears to be the major factor for decision of opportunity and type of portacaval shunt. From our material, we can describe three types of stenosis: type I, due to caudate lobe hypertrophy, and type II, due to right lobe hypertrophy, are suitable for side-by-side portacaval or mesocaval shunts. Type III, regular and extended narrowing of inferior vena cava, observed in long term evolutive forms, is presumably due to fibrosis and is not a good indication for conventional infrahepatic shunting procedures. Since this study was completed, another patient had a side-to-side portacaval anastomosis for chronic Budd-Chiari syndrome without caval stenosis. The patient has been observed for seven months, and ascites did not reappear. This underlines the importance of a complete radiologic and hemodynamic preoperative study of inferior vena cava outflow impairment.     

Surgical treatment for carcinoma at the confluence of the major hepatic ducts

During the period of November 1976 through October 1984, we have treated 46 patients with carcinoma at the confluence of the major hepatic ducts. Curative resection of the tumor (where no carcinoma cells at the margin of the resected portion were found macroscopically and microscopially), was performed upon ten patients, palliative resections were done in 11, palliative resection and intraoperative radiotherapy (IOR) in ten, bile duct drainage and IOR in three, and percutaneous transhepatic cholangiodrainage in 12. Among the ten patients who underwent curative operation, five are living six to 104 months after operation while the remaining five died four to 72 months postoperatively. Of the 11 patients who underwent palliative resection, five are alive two to 22 months after operation and six died between four and 20 months after operation. For the ten patients with carcinoma in the advanced stages, palliative resection with IOR was performed. Eight patients died 20 days to 16 months after operation while two patients are alive 13 and 14 months after the procedure. Of the three patients who underwent drainage of the bile duct and IOR, two died three months after operation and one patient survived 34 months. Twelve patients underwent drainage of the bile duct only and all died after 13 months. To perform a curative operation for carcinoma located at the confluence of major hepatic ducts, it is necessary to resect the right or left lobe, including the caudate lobe, in many instances. For the patients with carcinoma in the advanced stages, the possibility of long term survival period after operation exists through the use of the combined techniques of IOR and palliative resection of the tumor.     

Chemoinfusion of the hepatic artery for metastases to the liver

During a five year period, 69 patients were treated for carcinoma of the liver (seven primary tumors and 62 metastatic tumors) with 5-fluorodeoxyuridine (5-FUDR) administered through a hepatic artery (n = 62) or portal vein (n = 3) implantable infusion pump. Ten patients proved to have previously unsuspected extrahepatic nodal metastases at laparotomy for pump insertion. 5-FUDR was given in 14 day cycles for three months. At the end of that period and at three month intervals thereafter (mean follow-up time of 18 months, a range of one to 60 months), patients were evaluated with roentgenograms of the chest, liver function tests, carcinoembryonic antigen levels, radionuclide scans and computed tomography. Thirty-five patients had a partial response, nine had stabilization of the disease and 25 had progression of the disease (five during therapy, who were given mitomycin C). Median regression was 6.8 months (a range of three to 18 months). Six of the 35 partial responders, three of the nine patients with stabilization and ten of the 25 patients with progression had extrahepatic visceral disease. Survival time averaged 18.4 months (a range of five to 60 months) for the partial responders, 12.6 months (a range of two to 40 months) for patients with stabilization and seven months (a range of one to 17 months) for those with progression of the disease.     

Assessment of sexual dysfunction and sexually related personal distress in patients who have undergone orthotopic liver transplantation for end-stage liver disease

IntroductionSexual dysfunction (SD) status post‐orthotopic liver transplant (OLT) for end‐stage liver disease (ESLD) has long been recognized. To date, there are no studies examining how sexually related personal distress (SRPD) impacts sexual function in this population.AimsTo assess SD and SRPD in men and women who have undergone OLT for ESLD and to compare them with previously published reports on subjects without SD.Methods283 subjects (182 men and 101 women) who underwent OLT since 2005 were mailed a survey. Men received the International Index of Erectile Function (IIEF) and Female Sexual Distress Scale—Revised (FSDS‐R). Women received the Female Sexual Function Index (FSFI) and the FSDS‐R. All surveys asked about the presence of a current sexual partner.Main Outcome MeasuresTotal and subscale scores on the IIEF, the FSFI, and the FSDS‐R.ResultsNinety‐six patients (33.9%) completed and returned the surveys consisting of 34 women (33.7%) and 62 men (34.0%). Also, 83.9% of men and 88.2% of women reported having an available sexual partner. Two‐thirds of men and three‐quarters of women were sexually active. In all domains, IIEF demonstrates that men have mild to moderate SD. FSFI demonstrates that women also have SD. Both genders reported relatively mild SRPD based on FSDS‐R. Compared to previously published controls, all domain values were lower in both genders.ConclusionThe IIEF, FSFI, and SDS‐R results demonstrate that men and women who undergo OLT do exhibit mild to moderate SD. Their distress, though, is also mild to moderate, as evidenced by a high rate of continued sexual activity after OLT. Therefore, although SD may be widely prevalent in people who undergo OLT, aggressive intervention may not be warranted so long as the level of sexual distress remains low. Park ES, Villanueva CA, Viers BR, Siref AB, and Feloney MP. Assessment of sexual dysfunction and sexually related personal distress in patients who have undergone orthotopic liver transplantation for end‐stage liver disease. J Sex Med 2011;8:2292–2298.     

Two stage surgical management of the Budd-Chiari syndrome associated with obstruction of the inferior vena cava

A new, staged surgical approach to the treatment of Budd-Chiari syndrome complicated by inferior vena caval obstruction has been described. The first stage consists of a mesoatrial shunt. After an interval to allow hepatic decongestion, re-establishment of caval flow and overall improvement in the status of the patient, a portacaval shunt is established and the mesoatrial shunt removed. Such a staged procedure produced an excellent result in the patient reported upon in this study and represents a treatment option in similar patients with Budd-Chiari syndrome complicated by obstruction of the inferior vena cava.