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.     

Distal splenorenal shunt for decompression of portal hypertension in children with cystic fibrosis.

Apart from the sound physiologic basis for the distal splenorenal shunt as compared with the portacaval shunt and the conventional central splenorenal shunt, there are two important reasons why we think the use of this type of shunt is especially advantageous in children with portal hypertension secondary to cystic fibrosis. Firstly, the thick, fibrotic retroperitoneal area in the porta hepatis, where a portacaval shunt has to be constructed, can be avoided, which makes the distal splenorenal shunt the easier operation. Secondly, notwithstanding the relatively small-sized vessels, a wide anastomosis can be constructed with a high flow rate and, therefore, a minimal chance of shunt thrombosis.     

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.     

Suprarenal mesocaval shunt

A new technique for performing a mesocaval shunt above the level of the renal veins is described. It should be considered an option for those patients with perirenal vena caval occlusion or obstruction who require surgical decompression of the portal venous system.     

Maternal postpartum Group B Beta-hemolytic streptococcus ventriculoperitoneal shunt infection

BACKGROUND: Women with cerebrospinal fluid shunts require special management during the course of pregnancy. CASE REPORT: We describe a case of delayed postpartum ventriculoperitoneal shunt infection by Group B streptococcus in a 19-year-old who presented complaining of headache and a fever. The CSF culture from the shunt tap and the distal shunt tip both grew Group B beta-hemolytic streptococcus. CONCLUSION: Women who are colonized with Group B streptococcus and who have cerebrospinal fluid shunts should receive perinatal antibiotic prophylaxis, and may require more extended prophylactic antibiotics with cesarean section deliveries to prevent catheter tip colonization and subsequent shunt infection.     

Left sided segmental portal hypertension following mesocaval interposition shunt.

Following a mesocaval interposition shunt in three patients with cirrhosis of the liver, bleeding esophageal varices recurred in two and left sided portal hypertension partially persisted in one patient. Angiographic and pressure studies of the portal system demonstrated effective decompression of the greater splanchnic venous system but continued lesser splanchnic venous hypertension. Recurrent variceal hemorrhage ceased following splenectomy done as an emergency. In contrast to a standard portacaval shunt, it is suggested that after an interposition mesocaval shunt, altered jet streaming of mesenteric blood flow may divert gastrosplenic venous drainage away from the interposition shunt with persistence of lesser splanchnic venous hypertension. Recognition of this entity and of the need for splenectomy is advocated.     

Portacaval shunt as emergency procedure in unselected patients with alcoholic cirrhosis.

A prospective evaluation of emergency portacaval shunt has been conducted during a 12 year period in 138 unselected, consecutive patients with alcoholic cirrhosis and bleeding esophageal varies. An extensive diagnostic evaluation was completed within seven hours of hospital admission, and the shunt operation was undertaken within a mean of 8.5 hours. Follow-up study was conducted in a special clinic, and the current status of 97.1 per cent of the patients had jaundice, ascites or encephalopathy alone or in combination on admission. Systemic intravenous administration of posterior pituitary extract temporarily controlled the hemorrhage in 94 per cent of the patients, and the emergency portacaval shunt promptly and permanently controlled the varix bleeding in 96 per cent of the patients. Contrary to recent proposals, patients with the highest portal perfusion pressure and, presumably, the largest hepatopetal portal flow had the highest survival rate and those who were presumed from pressure measurements to sustain the smallest portal flow diversion from the shunt had the lowest survival rate. The operative survival rate was 51 per cent, the predicted seven year survival rate for those operated upon seven or more years ago was 42.5 per cent. Encephalopathy requiring dietary protein restriction developed at some time in 17 per cent of the survivors. Sixty per cent of the survivors abstained from alcohol, and 53 per cent resumed gainful employment or full time housekeeping. Preoperative factors that adversely influenced survial rate were ingestion of alcohol within one month of bleeding, ascites, severe muscle-wasting and a small liver. Postoperatively, the single most important factor that compromised long term survival was resumption of alcoholism. In comparisons with our previous prospective studies, emergency portacaval shunt resulted in a significantly greater long term survival rate than did either emergency medical therapy or emergency varix ligation, followed by elective shunt. It is concluded that emergency portacaval shunt is the most effective treatment of bleeding esophageal varices in patients with alcoholic cirrhosis. Criteria for exclusion of those patients who are unlikely to derive long term benefits from portacaval shunt remain to be defined by further studies.     

Portosystemic anastomosis during pregnancy]

There is discussed the surgical treatment of massive otherwise uncontrolable haemorrhage from oesophageal varices during advanced pregnancy. The authors have performed in the above situation a splenorenal shunt in a women suffering from the prehepatic portal hypertension during her sixth month of pregnancy. The pregnancy was maintained but an unviable child was born. The authors were able to find only 5 quotations from the references available to them dealing with 5 similar cases in women who delivered healthy babies thanks to a surgical intervention (5 times a portal systemic shunt, once a direct operation). The most important prevention of those exceptional surgical indications is the careful evaluation of all circumstances for a pregnancy in women with portal hypertension.     

23例脐静脉-门静脉-体静脉分流胎儿的产前诊断和产后随访分析

Objective?To investigate the current status of the prenatal diagnosis and postnatal follow-up of fetuses with umbilical-portal-system shunt (UPSVS). Methods?23 fetuses with UPSVS, diagnosed by Peking University People's Hospital from July 2013 to June 2020, were divided into three types according to the sonographic features: TypeⅠ, umbilical–systemic shunt (USS); TypeⅡ, ductus venosus–systemic shunt (DVSS); and TypeⅢ, portal–systemic shunt (PSS), which was divided into two subgroups: TypeⅢa, intrahepatic portal–systemic shunt (IHPSS); and TypeⅢb, extrahepatic portal–systemic shunt (EHPSS). Prenatal diagnosis and postnatal follow-up were analyzed retrospectively. Results?Compared with USS (2/23, 8.7%) and DVSS (4/23, 17.4%), PSS was the most common (17/23, 73.9%). The median follow-up time was 43 months(range 12-84). Three cases failed to be followed up. The rate of prenatal multi-disciplinary team (MDT) counselling was 15.0% (3/20). Fetal karyotyping was done at 35.0% (7/20). There were 11 cases opting to terminate the pregnancy, 9 cases with live born fetuses. Only 4 cases (4/9, 44.4%) had regular pediatric examination, compared with 2 cases with irregular examination and 3 cases without any examination. Conclusion?Prenatal MDT, genetic screening, and postnatal pediatric follow-up related to USPVS need to be further improved.     

Thrombosis of the portal vein following distal splenorenal shunt.

Portal vein thrombosis with a patent shunt is a distinct clinical entity which can follow selective distal splenorenal shunt and should be looked for in patients in whom ascites and abdominal pain develop postoperatively. Possible mechanisms include an increase in blood viscosity and a decrease in portal flow which may be aggravated by inadequate devascularization.     

Uterine perforation by a cystoperitoneal shunt, an unusual cause of recurrent vaginal discharge

BackgroundWe describe the first reported case of uterine perforation by a cystoperitoneal shunt. The mechanism of this unusual complication is unclear.CaseA 17-year-old patient had a cystoperitoneal shunt for a porencephalic cyst. She presented with recurrent watery vaginal discharge. A pelvic ultrasound examination showed that the uterus had been perforated by the distal tip of the shunt. The cystoperitoneal shunt was converted to a ventriculo-atrial shunt, and the vaginal discharge subsequently resolved.ConclusionThe appearance of light and clear vaginal discharge in a patient with a cystoperitoneal shunt raises the possibility of uterine perforation. This can be confirmed by ultrasound and analysis of the discharge. Removal of the shunt leads to spontaneous closure of the uterine defect.     

The jugular vein as an interposition graft in the distal splenorenal shunt

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.     

The beneficial and harmful effects of positive end expiratory pressure.

The effects of increasing levels of positive end expiratory pressure on gas exchange and pulmonary mechanics were determined utilizing an ex vivo ventilated perfused canine pulmonary lobe. When zero positive end expiratory pressure was used, shunting, weight gain and a decrease in compliance occurred over the four and one-half hour experiment. Shunting was eliminated when 5, 10 or 15 centimeters of water of positive end expiratory pressure were used. However, increasing extravascular fluid sequestration and decreasing pulmonary compliance occurred progressively with increasing levels of positive end expiratory pressure above 5 centimeters of water. Pulmonary artery pressure increased immediately along with end inspiratory pressure, an amount approximately equal to the increase in positive end expiratory pressure, and this is thought to be the primary cause of the increased rate of fluid sequestration. These experiments suggest that an optimal level of positive end expiratory pressure exists when the shunt can be reduced and oxygenation improved without increasing the rate of extravascular fluid accumulation to the point where long time deleterious effects could outweigh immediate benefits.     

The influence of portal blood upon lipid metabolism in normal and diabetic dogs and baboons

Complete diversion of portal blood in dogs caused sustained falls in serum cholesterol and phospholipid concentrations an declines in hepatic cholesterol and triglyceride synthesis. The hepatocytes in these canine livers were deglycogenated, and they atrophied to about half of their original size within two months. At the same time, there was evidence of increased mitoses. Ultrastructurally, the dominant change in the hepatocytes was in the rough endoplasmic reticulum which decreased in amount, underwent marked dilatation, and became depleted of ribosomes. There was also marked loss of glycogen granules, variable mitochondrial abnormalities, and widespread accumulation in the hepatocyte cytoplasm of lipid vacuoles. Bypass of intestinal venous return around the liver through a mesenteric caval shunt did not influence the serum lipid concentrations in dogs and baboons, although cholesterol synthesis was depressed in the canine livers and significant morphologic changes, including atrophy, were produced. In both species, the addition of a second stage central portacaval shunt which diverted venous return from the pancreaticogastroduosplenic area caused declines in serum cholesterol and phospholipid concentrations. After the second operation, hepatic cholesterol synthesis in the dogs was further reduced, and triglyceride synthesis was markedly depressed. The eventual ultrastructural changes were similar to those after one stage portal diversion. In other experiments on dogs, discrete regions of the liver were provided with portal perfusion from different splanchnic sources during a two month period. When the right lobes received pancreatiogastroduodenosplenic venous blood and the left lobes received intestinal venous effluent, in vivo cholesterol and triglyceride synthesis were higher in the hormone-enriched right lobes. This advantage was eliminated with pre-existing alloxan-induced diabetes or by the concomitant performance of total pancreatectomy in dogs that were treated during the ensuing two months with subcutaneously administered insulin. The nutrient-enriched left lobes had the higher lipid synthesis. In a final series of experiments, the right lobes of dogs were given the total splanchnic flow, and the left lobes were perfused with systemic venous blood by anastomosing the left portal vein to the suprarenal vena cava. The right lobar advantage in lipid synthesis could not be eliminated in this preparation with alloxan-induced diabetes or total pancreatectomy. These results indicate that a reduction of hepatic lipid synthesis is an important, although not necessarily the sole, factor in the antilipidemic influence of portacaval shunt. The effects upon synthesis and blood lipids apparently are due more to the diversion of endogenous hormones than to the bypass of intestinal nutrients. The substances in portal venous blood that subserve hepatic lipid metabolism are presumably largely the same as the hepatotropic factors which have been described before as profoundly affecting hepatic structure, function, and the capacity for regeneration. These portal blood factors are multiple and interrelated, but the single most important one seems to be insulin.     

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.     

Pregnancy in liver cirrhosis and/or portal hypertension.

Clinical features of pregnancy in women with liver cirrhosis and/or portal hypertension have been reviewed. Termination of pregnancy is seldom indicated in a woman with compensated cirrhosis or a young woman with extrahepatic venous obstruction. However, the risk of spontaneous abortion is increased in cirrhotic women without shunt even if there is no deterioration of liver function. The risk of bleeding from esophageal varices or deterioration of liver function is usually unpredictable. Shunt surgery can be done with relatively little effect on both the mother and the fetus if conservative measurements fail to control the hematemesis. Vaginal delivery can be anticipated in most women, and cesarean section should be preserved for obstetric indications. The risk of postpartum hemorrhage is greatly increased, particularly in patients with previous shunt surgery. Perinatal loss is high because of the increased rate of premature delivery and stillbirth. Maternal prognosis is grave in women with cirrhosis.     

Successful early in utero management of fetal hydrothorax in a twin pregnancy

We present a case of dichorionic diamniotic twin pregnancy in which one of the fetuses was found to have a major pleural effusion at 15 weeks of gestation. A single-needle pleural fluid aspiration was performed at 15 and 16 weeks, but the fluid reaccumulated quickly after each procedure and at 16 weeks, the fetus was found to become progressively hydropic. A shunt was then successfully inserted at 17 weeks, which is the earliest gestation reported so far in the literature for such a procedure to treat isolated hydrothorax. Because we felt that the fetus would be too small for a classical double-pigtail pleuroamniotic shunt, we used a multilength double-pigtail bladder stent (Harrison drain; Cook; Spencer; Indiana; USA) via a 13-gauge echo tip trocar. This shunt could be used for both singleton and twin pregnancies presenting with fetal pleural effusion from as early as 16 to 17 weeks to prevent the development of fetal hydrops and polyhydramnios and subsequent premature delivery. Treatment at this stage of gestation would also minimize the risk of lung hypoplasia, which is the main clinical issue when shunts are inserted after 24 weeks.     

Combined hepatic and pancreaticoduodenal procurement for transplantation

We have used a procurement method whereby both the liver and whole pancreas grafts are procured from the same donor and successfully transplanted. During the combined procurement, the hepatic artery is completely mobilized; the splenic artery is transected from the hepatic artery and the gastroduodenal artery is ligated from the hepatic artery. The portal vein is mobilized 2 centimeters from the head of the pancreas. The whole pancreas graft includes the splenic artery and the superior mesenteric artery, which are reconstructed. The hepatic graft includes the entire length of the hepatic artery with the celiac axis, and no further reconstruction is required. Using this technique, we have performed nine combined hepatic and whole pancreas procurements; only one liver was not transplanted because of technical complications. When a replaced right hepatic artery is identified from the superior mesenteric artery, we have abandoned the pancreatic retrieval. All combined retrievals have included successful renal retrieval, and the majority have been associated with cardiac retrieval also. Combined hepatic and whole pancreas procurement is feasible with minimal technical complications with the liver or the pancreatic graft and should be standard in most procurements.     

Portosystemic shunt versus orthotopic liver transplantation for the Budd-Chiari syndrome.

We have analyzed the indications and results of shunt operation versus orthotopic liver transplantation (OLT) in 22 patients with Budd-Chiari syndrome (BCS). The underlying cause of the syndrome was similar between the two groups and was related to myeloproliferative disorders or the use of birth control pills in 18 of 22 patients. The results of biopsies of the liver showed centrilobular congestion and necrosis in all candidates who underwent shunting and the presence of fibrosis and cirrhosis in the OLT candidates. The indications for shunts included symptoms related to portal hypertension only and well-preserved synthetic hepatic function. Ten patients were treated with 12 shunt procedures, including mesoatrial (eight patients) and side to side portacaval shunt (four patients). Significant complications after shunt procedure included fulminant (one of ten patients) and progressive (one of ten patients) hepatic failure requiring urgent OLT; one death occurred because of pulmonary sepsis. Indications for OLT were signs of end stage liver expressed by severe portal hypertension and variceal bleeding (four of 14 patients), progressive encephalopathy (seven of 14 patients) and poor synthetic function (bilirubin greater than 3 milligrams per deciliter in eight of 14 patients and albumin less than 3.0 grams per liter, or both, in ten of 14 patients). Fourteen patients were treated with 16 OLT, three patients had retransplantation for primary nonfunction graft (two of 14 patients) or chronic rejection (one of 14 patients). There were two early deaths in the group. With a follow-up period between two months to five years, 12 of 14 patients undergoing OLT are alive, fully functional and have normal liver function tests. Seven of ten patients who had shunts are alive, six are able to maintain normal activity and one has progressive end stage hepatic disease and is not a candidate for OLT. However, the hepatic function continues progressively to be abnormal. Various options are available for the treatment of the syndrome. Portosystemic decompression is effective and should be considered at the early stage of the disease, prior to the development of significant hepatic failure. However, few of the patients will continue to have slow, but progressive hepatic failure and may require OLT. The only effective treatment for end stage hepatic disease secondary to the BCS is OLT.     

Portal hypertension

The management of both acute and recurrent variceal bleeding continues to be a significant challenge to the clinician. The cause and pathogenesis of portal hypertension has been described. Alcoholic cirrhosis is the most common cause of intrahepatic sinusoidal and postsinusoidal obstruction in the United States. Long term survival depends on rapid institution of an established protocol of surgical management for variceal hemorrhage. A patient who presents with variceal bleeding must be rapidly stabilized with fluid resuscitation, and specific measures, such as the use of vasopressin and balloon tamponade, must be instituted to control hemorrhage so that endoscopy can be used to establish the diagnosis. Sclerotherapy achieves a high rate of success in the acute situation, but if hemorrhage cannot be controlled, percutaneous transhepatic embolization or emergent shunting must be performed, depending on the condition of the patient. Angiography, prior to surgical treatment, is necessary to define venous anatomy and determine portal hemodynamics, both of which provide information vital in choosing the type of shunt. If bleeding is massive and the patient is unstable, H-grafts are most appropriate, for they are technically easier and give excellent short term results. In a stable Child's A or B patient with minor ascites as well as suitable anatomy and hepatopedal flow, DSRS is the procedure of choice because it produces the smallest degree of HE postoperatively and increases the survival rate for nonalcoholics. If this is not feasible or if the surgeon lacks the technical expertise to perform DSRS, PCS is the logical alternative. In view of the data from the series observed in the United States, ablative procedures cannot be recommended at the present for the treatment of variceal bleeding. In the Child's C poor-risk patient, the operative mortality rate is prohibitive, and only nonsurgical means should be used to establish control of bleeding. In the elective situation, the surgical options change. The efficacy of ES as a definitive procedure to control recurrent variceal bleeding is unproved, and rebleeding can be significant; therefore, it cannot be recommended. H-grafts have a prohibitively high rate of long term thrombosis and are also not recommended, and the Linton or proximal splenorenal shunt offers no advantages over conventional portacaval shunting. Moreover, arterialization of the hepatic stumps of the portal vein does not prevent hepatic encephalopathy or alter the survival rate. Both PCS and DSRS prevent rebleeding, yet neither alters the survival rate for alcoholic patients.(ABSTRACT TRUNCATED AT 400 WORDS)