Portosystemic Venous Shunt Research Articles

Manganese elevations in blood of children with congenital portosystemic shunts.

Magnetic response imaging has demonstrated increased signal intensities within the basal ganglia in patients with hepatic encephalopathy; the densities are considered to represent manganese deposition. We measured whole blood manganese concentrations in nine children with congenital portosystemic venous shunts detected by screening tests for galactosaemia. Beyond 1 year of age, these patients showed significantly higher manganese concentrations than controls (2.40 +/- 0.43 versus 1.48 +/- 0.38 micrograms/dl; P = 0.0001). Four of the nine patients were studied by magnetic response imaging. T1-weighted images showed increased signal intensities in the basal ganglia of those four patients, suggesting manganese accumulation. Children with congenital portosystemic venous shunts showed manganese elevations in blood and magnetic response imaging changes in the basal ganglia. These children should avoid excessive manganese intake.

Congenital porto-left renal venous shunt as a cause of galactosaemia.

Congenital porto left renal venous (PRV) shunt was found to be the cause of galactosaemia in four galactosaemic neonates detected by mass screening (Paigen method). The patients did not have hereditary galactosaemias and were diagnosed as having galactosaemia of unknown cause, because porto-systemic venous (PS) shunts had not been recognized. At the time of diagnosis, hypergalactosaemia was not severe (0.44-0.55 mmol/L; 8-10 mg/dl) and plasma concentration of total bile acids (TBA) did not suggest a PS shunt (46-50 micromol/L). However, slightly but consistently increased concentrations of galactose and TBA strongly suggested the presence of a PS shunt, and careful ultrasonographic investigation revealed PRV shunt. We conclude that PRV shunt should be suspected in patients with hypergalactosaemia of unknown cause.

先天性門脈‐大静脈シャントの開腹根治術を行った乳児の麻酔経験

先天性門脈‐大静脈シャントの開腹根治術を行った乳児の麻酔経験

Increased hepatic arterial blood flow after decreased portal supply to the liver parenchyma owing to intrahepatic portosystemic venous shunt: angiographic demonstration using helical CT.

This study was conducted to investigate the haemodynamics of the liver parenchyma in the presence of intrahepatic portosystemic venous shunt. 3 patients with intrahepatic portosystemic venous shunts and 24 patients with normal intrahepatic haemodynamics underwent both CT arterial portography and CT during hepatic arteriography. Angiographic findings with helical CT were compared, and CT attenuated values were measured in both groups. The liver parenchyma on CT arterial portography had lower attenuation than on CT during hepatic arteriography in all patients with intrahepatic portosystemic venous shunts. Overall average CT attenuation was 92.2 +/- 7.7 Hounsfield units (HU) on CT arterial portography and 149.9 +/- 8.5 HU after CT during hepatic arteriography, with the opposite findings in all patients without intrahepatic portosystemic venous shunt: CT attenuation 142.0 +/- 25.7 HU on CT arterial portography and 100.7 +/- 16.4 HU after CT during hepatic arteriography. In conclusion, the portal venous supply to the liver parenchyma decreased due to intrahepatic portosystemic venous shunts, with a compensatory increase in hepatic arterial blood supply.

Intrahepatic spontaneous portosystemic venous shunt: Value of color and power Doppler sonography

Intrahepatic spontaneous portosystemic venous shunt: Value of color and power Doppler sonography

A Case of Stent Migration into Right Ventricle after Percutaneous Transluminal Angioplasty for Budd-Chiari Syndrome

Budd-Chiari syndrome (BCS caused by hepatic venous outflow obstruction due to occlusion of hepatic vein and/or the inferior vena cava (IVC consists of a variety of clinical manifestations such as abdominal pain, hepatomegaly, ascite associated with acute or chronic liver dysfunction and portal hypertension. A large number of therapies have been introduced for BCS, both medical (including diuretics, thrombolytics, and anticoagulants and surgical (such as peritoneovenous shunt, direct correction of the venous obstruction, decompressive portosystemic venous shunt, and in end-stage liver disease, orthotopic liver transplantation. More recently percutaneous transluminal angioplasty (PTA has been used to relieve the obstruction. PTA differs from surgery in that it can be used repeatedly because it is less invasive and safer. Possible complicati- ons of PTA include stent migration, occlusion due to thrombus formation or neointimal hyperplasia, pulmonary embolism, hemorrage and infection. We experienced one case with complication of stent migration into right ventricle after PTA for BCS. (Korean Circulation J 2000;30(7 :885-889

Aneurysmal Portosystemic Venous Shunt: A Case Report

A case of an aneurysmal portosystemic venous shunt detected by colour Doppler ultrasound (CDUS) is presented. A young female patient complained of postprandial fatigue and had paroxysmal tachycardia. A direct vascular communication between right portal vein and right hepatic vein was found at CDUS and confirmed by direct portal angiogram. Using detachable coils a complete occlusion of the intrahepatic shunt was obtained. Reports from the literature regarding portovenous aneurysms are reviewed.

Hepatosplenic Schistosomiasis ULTRASOUND EVALUATION UPDATE

Summary Ultrasound represents an important contribution to the diagnosis of Mansoni's hepatosplenic schistosomiasis. Abnormalities that may be recognized are hyperechoic fibrotic bands along the portal vessels, volumetric reduction of the right hepatic lobe, enlargement of the left lobe, perivesicular fibrotic thickening, and splenomegally. Moreover, the hemodynamic status of the portal venous system may also be evaluated by Doppler analysis, including identification of collateral veins and portosystemic venous shunts. In endemic areas, ultrasound has a highlighted role in the identification of hepatic lesions and splenomegally and allows the follow-up of patients undergoing clinical treatment. This article presents the ultrasonographic and Doppler features of hepatosplenic schistosomiasis. The relationship between these findings and the clinicopathological aspects of this disease are also discussed.

The long-term prognosis of congenital portosystemic venous shunt

Congenital portosystemic venous shunt (PSVS), considered to be a rare disease, can lead to hepatic encephalopathy (HE). With improvements in diagnostic imaging techniques, the number of infants and children with documented PSVS has increased. The natural course of the disease and indications for surgical closure of the shunt vessel have not been well defined. We reviewed 51 cases of congenital PSVS in Japan; 34 patients had an intrahepatic PSVS, and 17 had an extrahepatic PSVS. There were 12 patients with HE at the time of diagnosis. The frequency of HE increased in subjects over 60 years of age. Children with HE had a shunt ratio exceeding 60%. When the shunt ratio was less than 30%, HE did not occur. Twenty of 28 patients under the age of 15 years had hypergalactosemia at the time of neonatal screening. Part of the congenital intrahepatic PSVS spontaneously closed. Surgical closure of a PSVS may be an approach expected to prevent HE when the shunt ratio exceeds 60%. ( J Pediatr 1999;135:254-6)

Surgical correction of patent ductus venosus in three brothers.

We report the presence of a patent ductus venosus in three brothers who underwent surgical correction. Patent ductus venosus was demonstrated by ultrasonography. Portosystemic venous shunt ratios as evaluated by [123I]iodoamphetamine per rectal portal scintigraphy were 67%, 50%, and 77%, respectively. Histologic examination of liver biopsy specimens revealed fatty degeneration in all cases. Portal vein pressure before and after temporarily occluding the patent ductus venosus was estimated by an Anthron P-U catheter introduced into the portal vein via the ligament teres hepatis. Portal venous pressure increased from 10 to 17 cm H2O, 16 to 23 cm H2O, and 14 to 27 cm H2O, respectively. Therefore, banding of the ductus venosus with Teflon tape was attempted to prevent portal hypertension following complete ligation. As a result, portal venous pressures after stricture of the ductus venosus were 12, 21, and 20 cm H2), respectively. Bile acid and liver enzymes decreased and returned to normal within 14 days after surgery. Interestingly, serum concentrations of hepatocyte growth factor (HGF) increased significantly after restoration of the portal blood flow and then gradually decreased, but remained persistently elevated for at least two weeks in two cases measured after surgical correction. One month after correction, liver function returned to normal as assessed by serological and histological parameters in all cases. These results suggest that it is important to determine whether stricture or complete ligation is indicated for a patent ductus venosus during surgical correction, based on the portal venous pressure after temporal test occlusion of the duct. In addition, HGF may be a useful marker for normalization of hepatic microcirculation after surgery.

Treatment of Hepatic Encephalopathy

Hepatic encephalopathy is a complex neuropsychiatric syndrome that may occur in such diverse clinical situations as inherited errors of the urea cycle, acute or chronic liver disease, and spontaneous or iatrogenic portosystemic venous shunting, including that following procedures to establish a transjugular intrahepatic portosystemic shunt. The clinical manifestations of this syndrome range from subtle abnormalities detectable only by psychometric testing to deep coma. Several grading systems have been proposed; one based on clinical and electroencephalographic abnormalities is shown in Table 1.1 Hepatic encephalopathy may be present in 50 to 70 percent of all patients with cirrhosis, including those with abnormalities demonstrable . . .

A rare intrahepatic portacaval tubular shunt in a patient with spastic paraparesis

Although there are numerous communications between the portal and systemic venous systems, intrahepatic portosystemic venous shunts are not frequently encountered in clinical practice. Here we report a patient who presented with spastic paraparesis, who was found to have chronic liver disease with tubular intrahepatic portacaval shunting.

Imaging of congenital abnormalities of the portal venous system.

Although congenital abnormalities of the portal venous system are uncommon, they are sometimes encountered incidentally because of the widespread use of cross-sectional imaging techniques. These abnormalities include the preduodenal portal vein. duplication of the portal vein, portal vein aneurysm, rotation of the superior mesenteric vein. variations in portal venous tributaries, intrahepatic portosystemic venous shunts, and mesenteric vascular malformations. This pictorial essay illustrates the normal anatomy and the congenital abnormalities of the portal venous system as shown by radiologic imaging. focusing on the value of CT and MR imaging in diagnosing these abnormalities. Familiarity with the varied features of congenital abnormalities of the portal venous system will permit more accurate diagnoses and will help prevent false pathologic diagnoses.

Three brothers with progressive hepatic dysfunction and severe hepatic steatosis due to a patent ductus venosus

Three Japanese brothers with progressive deterioration of hepatic function and fatty degeneration of the liver eventually leading to hepatic encephalopathy were recently encountered. Each of them had a congenital intrahepatic portosystemic venous shunt due to a patent ductus venosus. Liver dysfunction and hepatic steatosis reverted to normal with surgical correction of the ductus venosus. Our observations suggest that impairment of liver function occurs first in the presence of malnutrition related to a reduction of blood in the portal vein. Hepatic steatosis is a consequence of a depleted metabolism of hepatocytes. Congenital portosystemic venous shunt due to a patent ductus venosus in this family has a genetic background. (Gastroenterology 1996 Jun;110(6):1964-8)

Intrahepatic portosystemic venous shunt in liver cirrhosis: is it congenital or acquired?

Intrahepatic portosystemic venous shunt in liver cirrhosis: is it congenital or acquired?M KudoAudio Available | Share

Ultrasound diagnosis of congenital intrahepatic portosystemic venous shunt

There are few radiologic descriptions of intrahepatic portosystemic venous shunts and all in adults. A case is reported of a newborn where the diagnosis of shunt was made with color Doppler and where the shunt resolved spontaneously at one year of age. Proposed etiologies for intrahepatic portovenous shunts are discussed.

Intrahepatic portosystemic venous shunt.

Among numerous variations of communications between the portal and systemic venous circulations, intrahepatic shunts between portal and systemic veins have seldom been described [1 , 2]. Recently we encountered an intrahepatic portosystemic venous shunt directly connecting the right portal vein to the inferior portion of the vena cava. We describe our case and discuss 14 other previously published cases [110].

Intrahepatic portosystemic venous shunt: occurrence in patients with and without liver cirrhosis

Portosystemic venous shunt within the hepatic parenchyma is rare, and its cause is disputed. Only 12 cases have been reported in the literature. Four new patients are presented here, all of whom had cerebral manifestations due to elevated blood-ammonia levels. One patient, initially misdiagnosed as having a psychiatric disorder, had multiple small portohepatic venous shunts in the peripheral hepatic parenchyma that were believed to be congenital in origin. The other three patients with clinical evidence of cirrhosis and portal hypertension had large tubular shunts between the posterior branch of the portal vein and the inferior vena cava. Shunts of this type were considered to be the collateral pathways developed in the hepatic parenchyma as a result of portal hypertension. The diagnosis of intrahepatic portosystemic venous shunts was established by angiography in all four patients. Sonography and CT failed to show the multiple small shunts, but did provide diagnostic information concerning the large tubular shunts. Intrahepatic portosystemic venous shunt can be the cause of hepatic encephalopathy. One should be familiar with the typical radiographic manifestations of this condition to prevent misdiagnosis as a psychiatric or neurologic disorder.

Prostaglandins in Diagnostic and Therapeutic Superior Mesenteric Artery Pharmacoangiography

The hemodynamic effects of prostaglandins PGA1, PGE1, PGE2, and PGF2 alpha on the splanchnic circulation were evaluated in five chronic dogs with portal hypertension, periportal fibrosis, and portosystemic venous shunting. The maximum effect was achieved with dosages of 2 micrograms/kg/min after bolus injection into the superior mesenteric artery. With this dosage a monophasic increase of portal blood flow and pressure was found with PGA1, PGE1, and PGE2, whereas PGF2 alpha caused a biphasic response: an initial decrease in portal blood flow and pressure was followed by an increase in these parameters. The average peak increase in portal blood flow and pressure was similar for PGE1 and PGF2 alpha, and significantly smaller for PGA1 and PGE2. Average peak iodine concentrations in the portal blood after superior mesenteric angiography were highest with PGF2 alpha, similar for PGE1 and tolazoline, and lowest in controls. The vasoconstrictor effect of PGF2 alpha is, overall, reduced and less reproducible when compared with vasopressin. This investigation suggests that both PGE1 and PGF2 alpha are effective for improved arterial portography, the latter agent appearing superior. On the other hand, PGF2 alpha cannot be recommended as a therapeutic agent for the treatment for gastrointestinal and particularly variceal bleeding.

Nonsurgical production of intrahepatic portosystemic venous shunts in portal hypertension with the double lumen balloon catheter

A nonsurgical production of intrahepatic shungs between the left portal and left hepatic vein was evaluated in twelve dogs with acute portal hypertension. Dilatation of the intrahepatic tract was achieved by means of a double lumen balloon catheter introduced into the portal system with the transjugular approach. The shunts proved to be effective in immediate reduction of increased portal pressure and no significant complications were encountered.