Intrahepatic portosystemic venous shunt via right renal vein successfully treated with balloon-occluded retrograde transvenous obliteration

A 66-year-old woman with a history of alcoholic liver cirrhosis and hepatocellular carcinoma was repeatedly hospitalized for the treatment of encephalopathy. Computed tomography demonstrated a portosystemic venous shunt which continued from portal branch P7 to the right renal vein. Since pharmacotherapy for encephalopathy was ineffective, balloon-occluded retrograde transvenous obliteration (B-RTO) was performed to block the portal-renal flow. Hyperammonemia and encephalopathy improved remarkably after the procedure. Intrahepatic portosystemic venous shunt draining into the right renal vein is a rare condition. To the best of our knowledge, management of this type of shunt by B-RTO has not been previously described. We report this case accompanied by a short review of the related literature.     

Control of solitary gastric fundal varices and portosystemic encephalopathy accompanying liver cirrhosis by balloon-occluded retrograde transvenous obliteration (B-RTO): a case report.

In a patient with liver cirrhosis complicated by solitary gastric fundal varices and portosystemic encephalopathy, Balloon-occluded retrograde transvenous obliteration (B-RTO) of the varices was performed. The gastric varices were decreased in size 2 weeks after treatment and had not recurred after 1 year. B-RTO successfully occluded the portosystemic shunt (gastrorenal shunt). Accordingly, the patient's blood ammonia levels, total bile acid level, and 15 min retention rate of indocyanine green decreased, and his hepatic encephalopathy improved. However, since consecutive increase in blood flow through the portal collateral vessels except for gastrorenal shunt vessel at 6 months and 1 year after B-RTO was noted, further careful follow-up may be required.     

Surgical treatment for an extrahepatic portosystemic shunt: a case report

An extrahepatic portosystemic shunt that has neither liver cirrhosis nor portal hypertension is rare. A 60-year-old Japanese woman who had been suffering chronic liver disease and anemia with mild disorientation was admitted to investigate general fatigue with dizziness and disorientation. The laboratory data revealed mild pancytopenia and liver dysfunction including hyperammoniemia, an increased Indocyanine Green 15-min retention rate, and a decreased Fischer's ratio. Color Doppler ultrasonography, computed tomography, and arterial portography revealed an extrahepatic portosystemic shunt that extended tortuously from the superior mesenteric vein into the inferior vena cava, and decreased blood flow in the main portal vein. Judging from intraoperative measurement of portal pressure and intraoperative portography, shunt ligations were performed at both the efferent portion of shunt from the superior mesenteric vein and the afferent portion of the shunt into the inferior vena cava, and resection of the spleen was also performed. On the postoperative laboratory data, pancytopenia disappeared, and liver function improved. Postoperative abdominal imaging showed increased blood flow in the main portal vein and disappearance of the shunt vessel. Moreover, symptoms present before surgery also disappeared. In conclusion, surgical treatment of extrahepatic portosystemic shunts may result in better postoperative quality of life if it is performed in carefully selected patients.     

Spontaneous hemoperitoneum due to rupture of the paraumbilical vein successfully treated with balloon-occluded retrograde transvenous obliteration

Spontaneous hemoperitoneum is an uncommon condition, which may be critical even if treated appropriately. The paraumbilical vein is a portosystemic collateral vein that develops in patients with portal hypertension, and is rarely found to be a source of bleeding. Here we present a case report of spontaneous hemoperitoneum due to rupture of the paraumbilical vein successfully treated with balloon-occluded retrograde transvenous obliteration (B-RTO). A 69-year-old man with cirrhosis due to nonalcoholic steatohepatitis was admitted to our hospital with abdominal distention and pain. Computed tomography revealed hemoperitoneum with a dilated paraumbilical vein, and rupture of the paraumbilical vein was diagnosed to be the cause of anemia. B-RTO was performed via the left femoral vein with upstream embolization using microcoils, and thrombosis of the paraumbilical vein was confirmed after B-RTO. The patient was discharged without complications 20 days after B-RTO and he experienced no further episodes of bleeding during the subsequent 6-month period.     

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.     

Balloon-occluded retrograde transvenous obliteration improves liver function in patients with cirrhosis and portal hypertension

BACKGROUND AND AIM: Balloon-occluded retrograde transvenous obliteration (B-RTO) is a novel therapeutic method for the treatment of large gastric fundal varices with spontaneous splenorenal shunt (SRS). However, the effects of B-RTO on liver function remain unknown. METHODS: Fourteen patients with portal hypertension and gastric varices with SRS were studied, consisting of four patients with acute bleeding, five with high-risk varices, and five with refractory portosystemic encephalopathy. Hepatic venous catheterization was performed to evaluate hepatic blood flow and liver function using the continuous indocyanine green (ICG) infusion method. To assess the metabolic activity of the hepatocyte, the intrinsic clearance of ICG was calculated. In all patients, endoscopic study was performed before and 1 week and 1 month after the B-RTO, and followed every 6 months thereafter. After baseline measurements, B-RTO was performed. Four weeks after the B-RTO, the same catheter measurements were repeated. RESULTS: The B-RTO was successful in all patients. Contrast-enhanced computed tomography showed complete obliteration of the SRS prior to the follow-up measurements. Endoscopic eradication of the fundal varices was obtained 6 months after B-RTO in all patients and encephalopathy was improved within 1 week after B-RTO. Following the B-RTO, hepatic blood flow (441 +/- 214 vs 668 +/- 299 mL/min, P < 0.0001) and the intrinsic clearance of ICG (233 +/- 123 vs 285 +/- 148 mL/min, P < 0.05) were significantly increased. Furthermore, intrahepatic resistance decreased after the B-RTO (P < 0.005). CONCLUSION: From short-term assessment, B-RTO increases hepatic blood flow and improves the metabolic activity of the liver in patients with portal hypertension.     

Peripheral portosystemic shunt and its selectivity changes measured on duplex ultrasound

BACKGROUND/AIMS: Portosystemic shunts offer a symptomatic treatment for portal hypertension. Their main disadvantage is decreased perfusion of the liver with portal blood. Change of peripheral shunts into total shunts after a period of time is described. This study aims to evaluate long-term hemodynamic changes in peripheral portosystemic shunts. METHODOLOGY: The study was based on 12 patients in whom distal splenorenal shunts 8 patients) and mesocaval shunts (4 patients) were indicated respectively. Duplex sonography was used to measure the blood flow in the portal, splenic and mesenteric veins before shunt surgery and minimally 14 months postoperatively. RESULTS: It was found that the reduction of the portal blood flow was not critical and no centralization of the shunt was observed. CONCLUSIONS: Long-term blood flow in the portal vein was not severely reduced after peripheral portosystemic shunt creation, therefore the peripheral portosystemic shunt still has a role in the treatment of some patients with portal hypertension.     

Transhepatic catheter-directed thrombolysis for portal vein thrombosis after partial splenic embolization in combination with balloon-occluded retrograde transvenous obliteration of splenorenal shunt

A 66-year-old woman underwent partial splenic embolization (PSE) for hypersplenism with idiopathic portal hypertension (IPH). One week later, contrast-enhanced CT revealed extensive portal vein thrombosis (PVT) and dilated portosystemic shunts. The PVT was not dissolved by the intravenous administration of urokinase. The right portal vein was canulated via the percutaneous transhepatic route under ultrasonic guidance and a 4 Fr. straight catheter was advanced into the portal vein through the thrombus. Transhepatic catheter-directed thrombolysis was performed to dissolve the PVT and a splenorenal shunt was concurrently occluded to increase portal blood flow, using balloon-occluded retrograde transvenous obliteration (BRTO) technique. Subsequent contrast-enhanced CT showed good patency of the portal vein and thrombosed splenorenal shunt. Transhepatic catheter-directed thrombolysis combined with BRTO is feasible and effective for PVT with portosystemic shunts.     

An inferior mesenteric-caval shunt via the internal iliac vein with portosystemic encephalopathy

We report here a case of an unusual extrahepatic portosystemic venous shunt in a 37-year-old woman without liver cirrhosis or portal hypertension, who developed portal systemic encephalopathy. Angiography demonstrated an inferior mesenteric-caval shunt characterized by the presence of direct communication of the inferior mesenteric vein with the left internal iliac vein. After the treatment with percutaneous transcatheter embolization of the shunt via a femoral vein approach using coils, she had no episode of portal systemic encephalopathy.     

A case of successful management of portosystemic shunt with autosomal dominant polycystic kidney disease by balloon-occluded retrograde transvenous obliteration and partial splenic embolization

We describe a patient with autosomal dominant polycystic kidney disease who was successfully managed for severe abdominal distension, impaired liver function and a portosystemic shunt by interventional therapies. The patient's intra-hepatic portal vein was compressed and narrowed by multiple liver cysts, which resulted in a decrease of the portal blood flow and portal hypertension due to a huge gastro-renal shunt These haemodynamic changes were assumed to contribute to insufficient protein synthesis in the liver. Therefore, we first repeatedly performed minocycline hydrochloride instillations to treat the multiple liver cysts. Then, we conducted a partial splenic embolization to prevent elevation of the portal vein pressure prior to balloon-occluded retrograde transvenous obliteration which was performed to increase the portal blood flow. The portal blood flow markedly increased, and protein synthesis in the liver also recovered and the clinical symptoms improved. The patient has been monitored for more than two years up to the present and her liver function parameters have remained within the normal range. Renal insufficiency is known to be a major prognostic factor in autosomal dominant polycystic kidney disease. In some cases, however, liver involvement with multiple cysts may result in a fatal outcome. In such cases, interventional therapies, as provided to this patient, should be considered.     

Effective balloon-occluded retrograde transvenous obliteration of the superior mesenteric vein–inferior vena cava shunt in a patient with hepatic encephalopathy after living donor liver transplantation

Balloon-occluded retrograde transvenous obliteration (BRTO) has become a common and effective procedure for treating hepatic encephalopathy due to a portosystemic shunt related to cirrhosis of the liver. However, this method of treatment has rarely been reported in patients after liver transplantation. Here, we report the case of a 52-year-old patient who underwent living donor liver transplantation (LDLT) due to hepatitis C virus-infected hepatocellular carcinoma that was complicated with portal vein thrombosis and a large portosystemic shunt between the superior mesenteric vein (SMV) and inferior vena cava (IVC). The SMV–IVC shunt was not obliterated during LDLT because there was sufficient portal flow into the graft after reperfusion. However, the patient was postoperatively complicated with encephalopathy due to the portosystemic shunt. BRTO was performed and was demonstrated to have effectively managed the encephalopathy due to the SMV–IVC shunt, while preserving the hepatic function after LDLT.     

The incidence of portal vein thrombosis at liver transplantation.

The incidence of portal vein thrombosis was examined in 885 patients who received orthotopic liver transplantations for various end-stage liver diseases between 1989 and 1990. The thrombosis was classified into four grades. Grade 1 was thrombosis of intrahepatic portal vein branches, grade 2 was thrombosis of the right or left portal branch or at the bifurcation, grade 3 was partial obstruction of the portal vein trunk, and grade 4 was complete obstruction of the portal vein trunk. Among the 849 patients without previous portosystemic shunt, 14 patients (1.6%) had grade 1, 27 patients (3.2%) had grade 2, 27 patients (3.2%) had grade 3 and 49 patients (5.8%) had grade 4 portal vein thrombosis. The incidence of portal vein thrombosis was highest (34.8%) in the patients with hepatic malignancy in the cirrhotic liver, followed by those with Budd-Chiari syndrome (22.2%) and postnecrotic cirrhosis of various causes (15.7%). The patients with encephalopathy, ascites, variceal bleeding, previous splenectomy and small liver had significantly higher incidences of portal vein thrombosis than the others. The total incidence of portal vein thrombosis among the 36 patients with previous portosystemic shunt was 38.9%, which was significantly higher than that (13.8%) of those without shunt.     

Formation, hemodynamics and new management options for gastric varices

Abstract   Gastric varices develop in patients with portal hypertension, including liver cirrhosis, idiopathic portal hypertension as well as left sided-local portal hypertension such as splenic vein thrombosis or splenic AV malformation. The inflow vein is the left gastric vein, posterior vein, or short gastric vein, while the outflow vein is the gastro-renal shunt in most of the patients with gastric varices. The form of the gastric varices is classified into three types of venous dilatation; tortuous type, notched type and tumor type according to the shape and size of the varices. The location is classified into five sites; the posterior site, anterior site, greater curvature site and lesser curvature site of the cardiac area, and the fundic area. The risk of the rupture depends on the mucosal factor of the varices as well as the location and the form. The hemostasis rate has been improved to 94–97% with the usage of the endoscopic occlusive agent such as Histoacryl. It is absolutely necessary to eradicate the gastric varices within a few weeks after rupture of the gastric varices. There are new management options such as laparoscopic Hassab's operation or balloon-occluded retrograde transvenous obliteration of the varices (B-RTO). The 5-year cumulative rate of the non-variceal bleeding is more than 90% after the B-RTO as well as after surgery. Further prospective clinical trials are to be investigated for an evidence-based medicine.     

Embolization of splenorenal shunt associated to portal vein thrombosis and hepatic encephalopathy

Hepatic encephalopathy (HE) is a cognitive disturbance characterized by neuropsychiatric alterations. It occurs in acute and chronic hepatic disease and also in patients with portosystemic shunts. The presence of these portosystemic shunts allows the passage of nitrogenous substances from the intestines through systemic veins without liver depuration. Therefore, the embolization of these shunts has been performed to control HE manifestations, but the presence of portal vein thrombosis is considered a contraindication. In this presentation we show a cirrhotic patient with severe HE and portal vein thrombosis who was submitted to embolization of a large portosystemic shunt. Case report: a 57 years-old cirrhotic patient who had been hospitalized many times for persistent HE and hepatic coma, even without precipitant factors. She had a wide portosystemic shunt and also portal vein thrombosis. The abdominal angiography confirmed the splenorenal shunt and showed other shunts. The larger shunt was embolized through placement of microcoils, and the patient had no recurrence of overt HE. There was a little increase of esophageal and gastric varices, but no endoscopic treatment was needed. Since portosystemic shunts are frequent causes of recurrent HE in cirrhotic patients, portal vein thrombosis should be considered a relative contraindication to perform a shunt embolization. However, in particular cases with many shunts and severe HE, we found that one of these shunts can be safely embolized and this procedure can be sufficient to obtain a good HE recovery. In conclusion, we reported a case of persistent HE due to a wide portosystemic shunt associated with portal vein thrombosis. As the patient had other shunts, she was successfully treated by embolization of the larger shunt.     

Splenoadrenal shunt. An original portosystemic decompressive technique

Management of gastrointestinal hemorrhage from rupture of esophageal and gastric varices due to portal hypertension remains a debated question. In patients with sclerotherapy-resistant esophagogastric varices, and preserved hepatic function, a surgical shunt is considered the treatment of choice. A 63-year-old male was admitted in our Department with a diagnosis of idiopathic fibrosis of the liver, portal hypertension, esophageal and gastric varices and previous history of variceal bleeding. A distal splenorenal shunt was planned. During the isolation, a large diameter left adrenal vein was identified. An end-to-end anastomosis utilizing the distal splenic vein and the proximal adrenal stump was performed. The procedure was uneventful. An ultrasound color-Doppler on the 3rd postoperative day, showed normal intrasplenic resistance index, demonstrating the efficacy of the shunt. A splenic angiography carried out on the 8th postoperative day showed the complete patency of the splenoadrenal shunt. At the 15th postoperative day, the patient was discharged. In patients with portal hypertension, sclerotherapy-resistant esophagogastric varices and preserved hepatic function, a surgical portosystemic shunt is mandatory. Splenoadrenal shunt, utilizing a left adrenal vein represent an excellent option in selected cases.     

门静脉左支分流降低术后肝性脑病的临床研究

目的 检测和分析家兔门静脉及其分支的血氨浓度差异从而指导肝内门腔静脉分流术中对门静脉分支的选择,降低分流引起的肝性脑病的发病率。评价选择性门静脉左支作为经颈静脉肝内门腔静脉分流术分流道的临床意义,分析门静脉左、右支的血液动力学变化及重要液递物质浓度差异对术后预防肝性脑病及远期疗效的影响。方法 在家兔门静脉系统各分支分别取血测定血氨浓度并进行比较。341例有目的地选择肝内门静脉左支作为穿刺靶点,行经颈静脉肝内门静脉左支门腔分流术(transjugular intrahepatic leftbranch of portal vein portosystemicshunt,TILPS)建立门腔分流道,避开富含营养、毒素的门静脉右支血液。肝实质通道用8mm直径球囊扩张,限制分流口径。结果 所测得血氨浓度,肠系膜上静脉高于门静脉左、右主支,(19.3±19.3)μmol/L与(156.5±20.9)μmol/L、(176.3±22.5)μmol/L,t值为2.35、2.25,P<0.05;高于脾静脉与腔静脉;门静脉右支高于左支、所有患者术后3个月内无一例发生肝性脑病。随访期间(术后1年),TILPS术后341例患者仅5例 (1.47％)出现肝性脑病,19例(5.57％)出现支架内狭窄。结论 家兔门静脉系统各分支的血氨浓度存在差异,提示肝内门腔静脉分流术中门静脉左右支的选择可能会影响术后肝性脑病的发病率。选     

New type of asymptomatic congenital portosystemic shunt

Congenital extrahepatic portosystemic shunt (CEPS) is an extremely rare anomaly. In these malformations splanchnic blood bypasses the liver and drains into the systemic circulation through the inferior vena cava (IVC) or the left renal vein (LRV). Extrahepatic shunts may be divided into type 1 [end-to-side mesenterico-caval fistula with congenital absence of the portal vein (PV)] and type 2 (partial portocaval shunt caused by side-to-side mesenterico-caval fistula with normal or hypoplasic PV). Type 2 shunts typically are wholly extrahepatic between the PV or its right branch and the retrohepatic IVC. This report describes an asymptomatic case of CEPS not previously documented and not classifiable as type 1 or 2. CT revealed a normal PV with communication between the inferior mesenteric vein (IMV) and the LRV. The inferior mesenteric vein appeared tortuous, abnormally long and with a large calibre, and presented a connection with the LRV, in addition to a normal confluence into the splenic vein. Colour Doppler revealed hepatopetal normal flow in the PV and superior mesenteric vein with mild portal hypertension and an inversion of flow in the IMV directed to the LRV. Biochemical parameters showed a normal liver function without hyperammonaemia.     

经皮脾门静脉核素显像、B超及胃镜对肝硬化门脉高压诊断价值的对比研究

为探讨经脾门静脉核素显像、胃镜及B超检查在肝硬化门脉高压诊断中的价值及其相关性,采用99m锝-植酸钠（99mTc-Phytate）经脾细针穿刺给药法对58例肝病与非肝病患者进行了经脾门静脉单光子发射计算机断层（SPECT）显像,以观察及门脉循环情况,计算门体分流指数（PSSI）,并与胃镜、B超进行比较。结果显示,经脾门静脉核素显像可将肝硬化门脉高压分为四型,即无分流型、有分流型（包括肝内、外分流）、有侧支循环型、完全肝外分流型。PSSI值非肝病对照组为0.192±0.068,慢性肝病组为0.246±0.057,肝硬化门脉高压组为0.541±0.082。按肝硬化ChildA、B、C分级,三组分别为0.384±0.052、0.523±0.072、0.680±0.081。若以PSSI＞0.36为判别阈,肝硬化组总阳性率为91.7％,其诊断价值明显优于B超及胃镜。提示经脾门静脉核素显像既能显示脾门静脉形态及侧支循环,又能测定PSSI,有助于肝硬化的早期诊断、治疗选择及预后判断。     

Portal-systemic shunting in a patient with normal portal vein pressures and histological evidence of idiopathic portal hypertension

Although idiopathic portal hypertension (IPH) is clinically characterized by portal hypertension and marked splenomegaly, we have experienced a case of spontaneous portal-systemic shunt without splenomegaly in whom the liver histology resembled IPH but with normal portal pressure. We admitted a 64 year old man who had suffered from hepatic encephalopathy for 2 years. Laparoscopy revealed a dark grey liver with a sharp edge and a concave surface. Examination of a liver biopsy specimen revealed peri-portal fibrosis consistent with IPH. A single, large, portal-systemic shunt was identified by percutaneous transhepatic portography. The shunt arose from the left gastric vein and flowed through the left renal vein into the inferior vena cava. No varices were identified. There were no morphological changes in the hepatic or portal veins. Portal vein pressure was normal. There was a slight difference between the portal pressure and the wedged hepatic vein pressure, suggesting a presinusoidal block. This case raises important questions concerning the aetiology of IPH and the relationship between portal hypertension and the development of collateral venous circulation.     

Surgery for Portal Hypertension in Children

Management of children with portal hypertension has evolved considerably over the past decades. Development of physiologic shunts (meso-Rex bypass) and successful liver transplant has changed the paradigm of portal hypertension surgery. Children with pre-hepatic portal hypertension are investigated and, if suitable, candidates are offered the mesenteric-to-left portal vein bypass (meso-Rex) preemptively, before development of symptoms of portal hypertension. Aggressive medical management, endoscopic ligation of bleeding varices, and radiologically placed intrahepatic stents have greatly reduced the need for emergent surgical procedures. A larger number of surgical options offer a permanent solution for children with portal hypertension in the setting of well-compensated liver function. Portal hypertension in the setting of decompensated liver disease is managed medically (via endoscopy) or radiologically (via transjugular intrahepatic portosystemic shunt) with the aim to offer liver transplant as a permanent solution.