Case report: intrahepatic portal-hepatic venous shunts associated with a huge pelvic leiomyoma

We present a case of portal-systemic encephalopathy due to intrahepatic multiple portal-hepatic venous shunts. A 71-year-old woman was admitted to our hospital because of recurrent episodes of disturbed consciousness. She showed no clinical signs of portal hypertension. Liver function was normal, except for an indocyanine green retention rate of 34% at 15 min and blood ammonia level of 282 microg/dL. Portal venography revealed dilatation of the portal vein and multiple portal-hepatic venous shunts, and a liver biopsy specimen revealed almost normal liver. Further clinical examination revealed a huge pelvic tumour. At laparotomy, two dilated veins were seen to arise from the pelvic tumour with blood flow into the mesentery. The tumour was resected successfully and a histological diagnosis of leiomyoma was made. The blood ammonia concentration decreased to the normal range postoperatively. A follow-up portal venogram demonstrated decreased portal vein dilatation and minor portal-hepatic venous shunts, considered to be congenital in origin. It is concluded that hepatic encephalopathy was produced in this patient due to an excess portal blood flow from the huge pelvic leiomyoma via the mesentery, with portosystemic shunting through pre-existent (probably congenital) intrahepatic anastomoses.     

Portographic Opacification of Hepatic Veins and (Anomalous) Anastomoses between the Portal and Hepatic Veins in Cirrhosis—Indication of Extensive Intrahepatic Shunts

On the premise that extensive intrahepatic portal-venous anastomoses known to occur in cirrhosis would be demonstrated by contrast medium directly placed in the portal vein, percutaneous transhepatic portograms were analyzed in 82 patients with liver cirrhosis in relation to the estimated degree of shunting. The degree of intrahepatic shunt was measured during transhepatic portography using 99mTc-macroaggregated albumin. Hepatic veins began to opacify at 4 to 10 s after the start of injection of contrast medium during portography in 20 patients with cirrhosis who had intrahepatic shunt indices of 58.5 +/- 18.5%, but it was not visualized in 62 patients with cirrhosis whose shunt indices were 19.9 +/- 14.1%. Anomalous large anastomoses of 1 or 2 mm in size between the right portal vein and the right hepatic vein were clearly visualized during portography in 18 of 20 patients in whom the hepatic vein was opacified. Frequency and time of beginning opacification of the hepatic vein were closely correlated with the degree of intrahepatic shunt. Thus, opacification of the hepatic vein and abnormal anastomoses between the portal and hepatic vein systems in an early phase of portography indicates extensive intrahepatic shunts.     

Arterioportal Fistula: A Role for Pre-TIPSS Arteriography and Hepatic Venous Pressure Measurements

A 70-yr-old male presented with massive upper gastrointestinal bleeding secondary to esophageal varices. Because the bleeding was not controlled by sclerotherapy or vasopressin and nitroglycerin, the patient was evaluated for a transjugular intrahepatic portosystemic shunt. Preprocedure arteriography was performed because the etiology of the portal hypertension was uncertain. The arteriogram revealed a hepatic artery to portal vein fistula. Hepatic venous pressure measurements documented an elevated hepatic venous pressure gradient, which diminished dramatically upon embolization of the fistula. Rebleeding from the varices was associated with reestablishment of the fistula via collaterals and elevation of the hepatic venous pressure gradient. The case is presented to establish a role for arteriography prior to transjugular intrahepatic portosystemic shunting, especially in patients with unexplained portal hypertension, and to establish the potential value of hepatic venous pressure measurements in the treatment of arterioportal fistulas.     

Hepatic encephalopathy due to a large intrahepatic communication between the portal vein and inferior vena cava

A 59 year old man with cirrhosis presented with encephalopathy and hyper-ammonaemia. T1 weighted magnetic resonance imaging demonstrated a large void tubular structure connecting the right posterior portal vein branch and the inferior vena cava through the right hepatic lobe inferiorly, and cine-mode imaging showed a flow within this channel. Clearly in this patient a significant portion of the portal venous blood was being shunted into the inferior vena cava, causing encephalopathy. The exact origin of this channel is not known, but several possibilities are discussed. It is also predicted that similar previously unknown large intrahepatic shunts will be discovered increasingly with the availability of modern imaging techniques.     

Left-Sided gallbladder with intrahepatic portal venous anomalies

The congenital anomaly in which the gallbladder is found on the left of the round and falciform ligaments (left-sided gallbladder) is rare. We report two patients with left-sided gallbladder in whom intrahepatic portal venous anomalies were identified. Computed tomography and intraoperative ultrasonography were used to define the portal venous anomaly. A long straight left main portal vein was demonstrated, which did not have the typical umbilical portion. The right anterior segmental portal branch (case 1), or the right main portal vein (case 2) were shown to course in a ventral direction and terminate as a cul de sac. The round ligament (right round ligament) was attached to this venous termination, forming the right umbilical portion. The left medial segmental portal venous branches originated from the right umbilical portion, and coursed to the left. In contrast, cholangiography disclosed that the left medial segmental bile duct coursed to the right after arising from the left hepatic duct (case 1), or the common hepatic duct (case 2). The essence of this anomalous condition is not a left-sided gallbladder, but a right round ligament, which is an embryologic abnormality of the umbilical vein. A review of the English language literature revealed no reports of left-sided gallbladder with intrahepatic portal venous anomalies.     

Intrahepatic Heterogeneity of Hepatic Venous Pressure Gradient in Human Cirrhosis

Background: The hepatic venous pressure gradient (HVPG) is used to evaluate portal hypertension. Methods: We measured HVPG in two separate liver veins in 169 liver vein catheterizations in 102 cirrhosis patients and in 27 patients with no liver disease (controls). Results: In the controls, the two measurements differed by 0.0 ± 1.8 mmHg (mean ± s , n = 27), upper 95% confidence limit 3.6 mmHg (mean + 2 s ). HVPG ranged from-0.1 to 8.3 mmHg, upper 95% confidence limit 6.7 mmHg. In cirrhosis, the two measurements agreed within ± 3.6 mmHg in 39%. In 61%, the measurements differed by 4-34 mmHg. In 35%, fluoroscopy demonstrated hepatic vein-to-hepatic-vein shunting in veins with low HVPG values. In some patients with HVPG measurements above 30 mmHg, Doppler ultrasound examination showed arterialization of the hepatic vasculature. Discussion: Our results demonstrate a hitherto unrecognized notable heterogeneity of the intrahepatic vasculature and HVPG measurements in cirrhosis. The presumption of interposition of non-flowing blood between the catheter tip and the portal system for the measurement of HVPG may thus be violated in about one-third of the cirrhosis cases because of abnormal outlet into hepatic venous shunts and in a minor fraction because of abnormal arterial inlet. In 26%, one measurement was below 12 mmHg, the other measurement above. If the HVPG had been measured in only one liver vein, 13% of the cases would have been classified in a lower risk group than appropriate according to the 12 mmHg concept of risk of bleeding from oesophageal varices.     

Portal hepatic venous shunt via an intrahepatic portal vein aneurysm.

A rare case of portal hepatic venous shunt (PHVS) via an intrahepatic portal vein aneurysm (PVA) is presented. A 66-year-old man was admitted for examination of a mass in the liver. Ultrasonography demonstrated a cystic lesion (15 mm in diameter) at the posterior superior segment of the right hepatic lobe which communicated with the right portal vein (RPV) and right hepatic vein (RHV). Superior mesenteric portography showed a biloculate aneurysm in RPV and PHVS. Color doppler ultrasonography indicated that flow in the tracts entered the aneurysmal cavity from RPV and drained into RHV.     

Hepatic encephalopathy due to intrahepatic portosystemic venous shunt successfully treated by interventional radiology

We treated a 66-year-old woman with hepatic encephalopathy secondarily induced by an intrahepatic portosystemic venous shunt (IPSVS). In serial observations, the volume of the liver became smaller and encephalopathy could not be controlled with conservative therapy. We occluded the IPSVS successfully using percutaneous transcatheter embolization with micro coils. Following embolization, encephalopathy disappeared and blood flow of all branches of portal vein improved. In cases with an IPSVS without liver cirrhosis, blood flow in the portal vein and liver volume must be followed carefully, and interventional radiology may be considered effective in those who do not show a satisfactory response to conservative therapy.     

Magnetic resonance imaging of macroscopic intrahepatic portal-hepatic venous shunts

Direct communication between portal branches and the hepatic vein [macroscopic intra-hepatic portal-hepatic venous shunt (IPHVS)] is a rare entity. We have recently studied five patients with this condition. Magnetic resonance imaging (MRI) clearly demonstrated in each case the portal-hepatic venous shunt due to "flow void." Multiple diffuse shunts were present in one case and a solitary shunt was demonstrated in the others. The solitary shunt was either tubular, focally dilated or racemose in configuration. The MRI findings and clinical significance of this rare entity are discussed.     

急性重症酒精性肝炎肝动脉缓冲效应研究进展*

目的 急性重症酒精性肝炎患者肝窦存在致密胶原沉积,阻力增加阻碍了血液流经肝窦,窦性压力增加,门静脉血流不畅,门静脉向肝窦的灌注显著减少,此时就启动了肝动脉缓冲效应,后者可以抵消肝脏灌注的两个主要血管肝动脉或门静脉中任何一个的流量减少,维持肝脏总血流量在一个生理范围内,使肝脏灌注(肝动脉和门静脉血流之和)恢复正常。双功能多普勒超声可以无创评估肝脏血流动力学和定量肝动脉缓冲效应。因此,肝动脉缓冲效应可能成为诊断急性重症酒精性肝炎的重要检测方法之一。     

Hepatic adenoma associated with portasystemic shunting in a young woman.

The development of a hepatic adenoma is described in a young female of reproductive age who had documented portasystemic shunting and hyperestrogenemia. The intrinsic hyperestrogenemia and the increased hepatic arterial blood flow, both of which occurred as a result of the portasystemic shunting secondary to portal venous obstruction, may have been important factors in the development of her hepatic tumor. We would postulate that prolonged endogenous hyperestrogenemia, like exogenous sex steroids, may predispose to hepatic adenoma formation.     

老年人原发性肝癌螺旋CT肝双期扫描的表现特点

目的 探讨老年人原发性肝癌螺旋CT肝双期扫描表现特点。方法 回顾性对比分析50例老年人(60～93岁)及27例青年人(15～35岁)原发性肝癌螺旋CT肝双期扫描表现。结果 老年人原发性肝癌多见于肝右叶(36例，占72．0％)，巨块型(41例，占80．0％)，边界清楚(35例，占70．0％)，肿瘤内多发斑点状CT“密度更低区”(12例，占24．0％)，门静脉癌栓(9例，占18．0％)，动-静脉瘘(7例，占14．0％)，肝总管内癌栓并肝内胆管扩张(7例，占14．0％)，侵犯邻近周围结构(11例，占22．0％)，肝内广泛转移(12例，占24．0％)；肝动脉期呈多血供型(13例，占26．0％)，少血供型(33例，占66．0％)，无强化(4例，占8．0％)，门静脉期强化(8例，占16．0％)。与青年人原发性肝癌比较，老年人原发性肝癌边界多清楚，胆管内癌栓多见，肝动脉期强化多为少血供；肝内转移、侵犯周围器官结构及门静脉期强化相对较少见；发病部位、形态类型、门静脉癌栓、动静脉瘘、肿瘤内CT“密度更低区”、腹水及肿瘤内出血等与青年人原发性肝癌差异性无显著性。结论 螺旋CT肝双期扫描是诊断与评价老年人原发性肝痛的有效方法。     

Spontaneous intermittent reversal of blood flow in intrahepatic portal vein branches in cirrhosis of the liver

The intrahepatic portal venous flow in cirrhosis of the liver was evaluated by percutaneous transhepatic portography and hepatic arteriography. Spontaneous reversal of flow in segmental portal vein branches was documented. Changes in hepatic arterial inflow and portal venous pressure may result in intermittent changes in the direction of flow in segmental portal venous branches within the cirrhotic liver. Segmental reversal of blood flow seems to be the precursor of total hepatofugal portal flow.     

Hepatic siderosis in extrahepatic portal vein obstruction

We report on the observation of a 26-year-old woman with portal vein obstruction, diagnosed at the age of 3, and liver iron overload. Celiac and superior mesenteric angiography showed large and multiple venous collaterals between the portal and caval systems. Liver biopsy demonstrated, on Perl's staining, an important hemosiderin deposition, confirmed by an increased hepatic iron concentration (15.6 mumol/100 mg dry weight). No other histologic abnormality was found. This report suggests that large spontaneous portosystemic shunting may stimulate hepatic iron deposition in an otherwise normal liver. This mechanism could, at least in part, explain the significant hepatic siderosis observed in some cirrhotic patients.     

A comparison of Doppler flowmetry with conventional assessment of acute changes in hepatic blood flow

The validity and clinical relevance of Doppler flowmetry in measuring changes in regional blood flow are uncertain. In the present study we compared changes induced by ketanserin in regional splanchnic blood flow as measured by Doppler flowmetry with changes in conventionally measured systemic and in hepatic haemodynamic indices estimated pharmacokinetically using indocyanine green. Fourteen patients with alcoholic cirrhosis and portal hypertension were evaluated. On multivariate analyses, significant associations were noted for only three indices: changes in estimated hepatic blood flow were predicted jointly by changes in flow in the main and right portal veins and hepatic artery (R²= 0.80); changes in intrahepatic shunting (indocyanine green extraction) were predicted by changes in flow in the main and right portal veins (R²= 0.55); and changes in sinusoidal perfusion (indocyanine green clearance) were significantly predicted by changes in main portal vein flow alone (R²= 0.76). These data support the validity of Doppler flowmetry in quantifying change in regional blood flow, but highlight the limitations in its clinical application and interpretation. The association of changes in main portal vein flow with changes in sinusoidal perfusion has clinical potential but requires confirmation using other modulating drugs.     

Ultrasound Visualization of Hepatic Peribiliary Cysts: A Comparison with Morphology

We report herein a hitherto unrecognized, interesting ultrasound finding ("hilar multicystic echo complex"), the result of peribiliary cysts in the liver. This ultrasound finding was discovered around intrahepatic large bile ducts and large portal vein branches near the hepatic hilum in an autopsy case with hepatocellular carcinoma, submassive hepatic necrosis superimposed on chronic active hepatitis, and portal hypertension. Antemortem ultrasound examination revealed the hilar multicystic echo complex around the portal venous branches near the hepatic hilum. Autopsy confirmed that the hilar multicystic echo complex was due to peribiliary cysts that were present around the bile ducts at the hilum. The peribiliary cysts were thought to have arisen from cystic dilatation of preexisting intrahepatic peribiliary glands. These peribiliary cysts reportedly occur in livers with portal hypertension (e.g., cirrhosis, hepatocellular carcinoma, idiopathic portal hypertension, extrahepatic portal obstruction, and portal thromboembolism), adult-type polycystic disease of the liver and kidneys, and systemic infection. Therefore, recognition of peribiliary cysts at sonography would have diagnostic value, and may indicate that presence of one of the above described liver diseases.     

Hepatic vascular anomalies in nonparasitic cysts of the liver.

Vascular abnormalities demonstrated by angiography in 3 patients with nonparasitic liver cyst are described. In two cases of polycystic liver, dilated and mutually anastomosing hepatic vein branches were seen around a cyst as opacified by hepatic venography, and in one case of solitary liver cyst, anastomoses between large portal branches and a right hepatic vein was demonstrated by celiac angiography. These vascular abnormalities most likely represent developmental malformation along with aberrant bile duct formation expressed in the form of cystic liver. To exclude the possibility of these changes being secondary to expansion of the cyst, an angiographic study of excised polycystic liver was carried out, and it was found that cyst enlargement produced displacement and thinning but no anastomosis of the intrahepatic blood vessels.     

Location and function of intrahepatic shunts in anaesthetised rats

BACKGROUND: In the present study we determined the proportion of shunt flow due to patent intrahepatic portal systemic shunts in the normal rat liver and its relationship with microsphere induced portal hypertension. METHODS: Systemic and hepatic haemodynamics were measured continuously before, during, and after intraportal injection of 15 micro m diameter microspheres in anaesthetised male Wistar rats. Functional hepatic blood flow and intrahepatic shunt flow were determined by the use of constant intraportal infusion of sorbitol and simultaneous measurements in the portal vein, hepatic vein, and carotid artery. The percentage of large shunts of diameter >15 micro m were estimated by intraportal injection of (51)Cr labelled 15 micro m diameter microspheres. RESULTS: Hepatic sorbitol uptake was 97.9 (0.5)% in normal control rats, with functional hepatic blood flow equalling total hepatic blood flow (2.52 (0.23) ml/min/100 g body weight). Microsphere injection decreased sorbitol uptake to 12.8 (4.3)% and further to 4.1 (0.7)% when followed by hepatic arterial ligation. In the latter two groups, intrahepatic shunt flow (1.46 (0.15) and 1.16 (0.19) ml/min/100 g body weight, respectively) was not significantly different from portal venous flow (1.36 (0.20) and 1.20 (0.20) ml/min/100 g body weight, respectively). Portal venous flow remained at 70% of basal values and portal venous pressure only increased by 50% from baseline. (51)Cr labelled microsphere shunt fraction through large shunts (>15 micro m) was less than 1.0%. CONCLUSION: The site of confluence between the hepatic artery and portal vein is in zone II. Intrahepatic shunts originate in presinusoidal regions in zone I in the normal liver and, when activated by intraportal injection of microspheres, divert 70% of the total portal blood flow away from zone III and thereby reduce acute increases in portal venous pressure.     

Intrahepatic Portosystemic Venous Shunt: Diagnosis by Color Doppler Imaging

Intrahepatic portosystemic venous shunt is a rare clinical entity; only 33 such cases have been reported. It may be congenital, or secondary to portal hypertension. Five patients with this disorder are presented, each of whom was diagnosed by color Doppler imaging, including waveform spectral analysis. One patient with clinical evidence of cirrhosis and portal hypertension had episodes of hepatic encephalopathy and elevated blood levels of ammonia. This patient had a large tubular shunt between the posterior branch of the portal vein and the inferior vena cava. Shunts of this type are considered to be collateral pathways which develop in the hepatic parenchyma as a result of portal hypertension. The other four patients had no evidence of liver disease, and all four evidenced an ancurysmal portohepatic venous shunt within the liver parenchyma. Shunts of this type are considered congenital. The diagnosis of intrahepatic portosystemic venous shunts was established by color Doppler imaging, which demonstrated a direct communication of color flow signals between the portal vein and hepatic vein, in addition to the characterization of the Doppler spectrum at each sampling point from a continuous waveform signal (portal vein) to a turbulent signal (aneurysmal cavity), and finally, to a biphasic waveform signal (hepatic vein). As demonstrated by the five patients, color Doppler imaging is useful in the diagnosis of an intrahepatic portosystemic hepatic venous shunt, and the measurement of shunt ratio may be useful in the follow-up and determining the therapeutic option.     

Effects of Cimetidine and Ranitidine on Splanchnic Hemodynamics in Patients with Chronic Liver Disease

Effects of two histamine H2 receptor antagonists, cimetidine and ranitidine, on systemic and splanchnic hemodynamics were studied in patients with chronic liver disease by simultaneous catheterization of the portal vein and the right hepatic vein and measurement of portal venous flow using the ultrasound doppler system or cineangiography. Neither infusion of 200 mg of cimetidine nor 50 mg of ranitidine reduced cardiac output, portal venous pressure, the gradient between wedged hepatic venous pressure and free hepatic venous pressure, hepatic blood flow, and portal venous flow. It is unlikely that histamine is an important modulator of flow via the H2 receptor.