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.     

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.     

Transjugular intrahepatic portosystemic shunt in combination with oral anticoagulant for Budd-Chiari syndrome.

The antiphospholipid antibody syndrome is characterized by arterial and venous thrombosis including hepatic veins. Although transjugular intrahepatic portosystemic shunt or liver transplantation have been considered for Budd-Chiari syndrome, treatment options for patients with complete obstruction of three hepatic veins including the junction with the inferior vena cava are limited. We describe a 27-year-old female, who suffered thrombotic obliteration of hepatic veins including the portion confluent with the inferior vena cava (Budd-Chiari syndrome) associated with marked ascites and liver dysfunction. Transjugular intrahepatic portosystemic shunt using a Wall-stent (10 mm in diameter) between inferior vena cava and intrahepatic portal vein was performed. Intrastent coagulation and recurrence of thrombosis were prevented by combination therapy with warfarin potassium and ticlopidine hydrochloride. These treatments induced loss of ascites and improvement of liver function, and she has been able to resume daily life. The portosystemic shunt described above in addition to combination therapy with warfarin potassium and ticlopidine hydrochloride appeared to be one of the options for treating Budd-Chiari syndrome associated with antiphospholipid antibody syndrome.     

Disappearance of hepatic encephalopathy and improvement of liver function after surgical treatment of portal-systemic shunt in a patient with liver cirrhosis

Large portal-systemic shunts in cirrhotic patients often cause recurrent hepatic encephalopathy and might promote liver dysfunction because of the reduced portal blood flow. We report a case of liver cirrhosis in which hepatic encephalopathy disappeared and liver function improved together with an increase of hepatopetal portal blood flow and liver volume after shunt resection. A 70-year-old woman with liver cirrhosis was admitted because of recurrent disorientation. Serum ammonia levels ranged from 174 to 321 micrograms/dL. Computed tomography demonstrated an atrophic liver and a large shunt. Portography disclosed that this shunt originated from the superior mesenteric vein and flowed into the inferior vena cava, common iliac vein and ovarian vein. Portal blood flow was poor because of the deviation into this shunt. After the surgical resection of the shunt, ammonia levels were normalized and hepatic encephalopathy no longer occurred. Portography and computed tomography after surgery demonstrated that hepatopetal portal blood flow evidently improved and the liver volume increased (before 369; after 574 cm3). Two years after surgery, hepaplastin test and serum albumin level improved from 41 to 76% and from 2.7 to 3.4 g/dL, respectively. This case supports the effectiveness of shunt resection for hepatic encephalopathy and the deteriorated liver function in cirrhotic patients with large portal-systemic shunt.     

A case of liver cirrhosis with an unusual portosystemic shunt via the right testicular vein.

We report a case of liver cirrhosis with a rare portosystemic shunt via the right testicular vein. Angiographic examination demonstrated two major hepatofugal collateral pathways. First, the enlarged and tortous left gastric and short gastric veins which drain into the left renal vein after forming varices in the stomach. Second, the superior mesenteric vein forms the large mesenteric varices which drain into the inferior vena cava via the enlarged right testicular vein, which is a very rare shunt.     

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.     

Clinical classification of congenital extrahepatic portosystemic shunts

Aim: Congenital extrahepatic portosystemic shunt (CEPS) is a rare anomaly in which the enteric blood bypasses the liver and drains into the systemic veins through various venous shunts. Patients with CEPS often have liver tumors and complications such as cardiac or other anomalies, but portosystemic encephalopathy and gastrointestinal bleeding occur only occasionally. The clinical problems differ for each individual with CEPS, and establishing a prognosis can be very difficult. Methods: We reviewed the clinical features of 136 reported cases of CEPS and classified these cases according to their portosystemic shunts. Results: We classified portal blood flow directly into the inferior vena cava (IVC) as type A (88 cases), portal blood flow into the renal vein as type B (36 cases), and portal blood flow into the iliac vein via an inferior mesenteric vein as type C (12 cases). Type A patients were complicated with cardiac anomalies at a higher rate than other types. Type C patients had lower prevalences of cardiac anomalies and portosystemic encephalopathy than the other types, but the prevalence of gastrointestinal bleeding was significantly higher (P < 0.0001). The prognosis of CEPS has improved, and only six deaths have been previously reported, all of which occurred in type A patients. Conclusions: We reviewed the previously reported cases of CEPS. Classification according to the portosystemic shunt system might be useful for investigating the clinical features of CEPS.     

A Case of Liver Cirrhosis with Large Portacaval Shunt from the Superior Mesenteric Vein: Normalization of Reversed Portal Blood Flow with Medical Treatment

A case of liver cirrhosis with large portacaval shunt from the superior mesenteric vein is reported. The portal blood flow was diagnosed as reversed on admission, but normalized with treatment. Blood flow was measured noninvasively and repeatedly using an ultrasonic Doppler duplex system. The reversed portal flow led to the superior mesenteric vein, right (and some left) internal inguinal vein, and inferior vena cava. This rare collateral pathway was clearly demonstrated by percutaneous transhepatic portalgraphy. A link between shunt and an appendectomy carried out 22 yr ago is considered, and the possibility ofthe temporarily reversed portal blood flow on admission being due to deteriorated hepatichemodynamic conditions is discussed.     

Reversal of Portosystemic Encephalopathy after Endoscopic Embolization of Recurrent Gastroesophageal Varices

Chronic portosystemic encephalopathy (CPSE) due to a shunt via gastroesophageal varices is uncommon. We were able to control a case of CPSE due to recurrent gastroesophageal varices using endoscopic embolization (EE). A 68-year-old man presented at our hospital in a confused, apathetic and tremulous state. He had undergone devascularization, proximal gastric transection and splenectomy for esopha-geal varices due to liver cirrhosis 16 years previously. The patient had hyperammone-mia (228 µg/dl), and endoscopic examination revealed nodular cardiac varices and large recurrent esophageal varices. Superior mesenteric arterial portography revealed that a large volume of superior mesenteric venous blood drained into the cardiac and esophageal varices through the remnant left gastric vein. The plasma ammonia level in the esophageal varices taken at the time of EE was 419 pg/dl, which was a much higher level than that of peripheral vessels, the superior vena cava and azygos vein, and suggested that the encephalopathy was due to the portosystemic shunt via the gastroesophageal varices. EE was performed using 5% ethanolamine oleate with iopamidol under fluoroscopy. The esophageal and cardiac varices were successfully embolized, the CPSE disappeared, and the plasma ammonia level decreased to 27 pg/dl. No complications were observed, and the patient was discharged on day 37 after EE. We conclude that EE is an effective and safe treatment for CPSE due to gastroesophageal varices.     

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     

Portal-systemic encephalopathy in non-cirrhotic patients: classification of clinical types, diagnosis and treatment

Hepatic encephalopathy is suspected in non-cirrhotic cases of encephalopathy because the symptoms are accompanied by hyperammonaemia. However, the cause of the large portal-systemic shunt formation observed in these cases is not clear, as cirrhosis and portal hypertension are absent. The frequency of such cases reported in the literature is increasing with progress and spread of abdominal imaging diagnostic techniques. Some cases have been misdiagnosed as psychiatric diseases (dementia, depression and others) and consequently patients have been hospitalized in psychiatric institutions or geriatric facilities. Some paediatric cases have also been misdiagnosed. Therefore, the importance of accurate diagnosis of this disease should be strongly emphasized. Some paediatric cases have also been misdiagnosed. When psychoneurological symptoms are suggestive of hepatic encephalopathy but objective and subjective symptoms or abnormal values of liver function tests are not sufficiently indicative of liver cirrhosis, portal-systemic encephalopathy should be suspected. Abnormal angiograms of the portal vein, superior mesenteric vein or splenic vein are conclusive evidence of portal-systemic encephalopathy. Transrectal portal scintigraphy also provides information useful for detection of shunts and a quantitative estimation of shunt index. We classified the disease into five types based on whether the shunt is formed inside or outside the liver. Type I (intrahepatic type) designates cases in which shunts are located between the portal and systemic veins. Type II designates a type of intra/extrahepatic shunt that originates from the umbilical part of the portal vein and serpentines in the liver, then leaves the liver. Type III (extrahepatic type) occurs most frequently. Type IV (extrahepatic) is accompanied by shunts similar to those in type III, but hepatic pathology presents as idiopathic portal hypertension. Type V (extrahepatic) represents the congenital absence of the portal vein, where the superior mesenteric vein joins the intrahepatic inferior vena cava or the left renal vein. The prevalence of each type in our country was examined by a nationwide investigation. In addition to the conventional diet or drug treatments, obliteration by less invasive interventional radiology using a metallic coil and ethanol has recently been used more frequently than surgical occlusion of shunts. Shunt-preserving disconnection of portal and systemic circulation and partial splenic artery embolization are also performed. International investigation of the disease status and establishment of diagnostic and therapeutic methods for the disease are awaited and investigation of long-term prognosis after therapy is also necessary.     

Could there be light at the end of the tunnel? Mesocaval shunting for refractory esophageal varices in patients with contraindications to transjugular intrahepatic portosystemic shunt

Cirrhotic patients with recurrent variceal bleeds who have failed prior medical and endoscopic therapies and are not transjugular intrahepatic portosystemic shunt candidates face a grim prognosis with limited options. We propose that mesocaval shunting be offered to this group of patients as it has the potential to decrease portal pressures and thus decrease the risk of recurrent variceal bleeding. Mesocaval shunts are stent grafts placed by interventional radiologists between the mesenteric system, most often the superior mesenteric vein, and the inferior vena cava. This allows flow to bypass the congested hepatic system, reducing portal pressures. This technique avoids the general anesthesia and morbidity associated with surgical shunt placement and has been successful in several case reports. In this paper we review the technique, candidate selection, potential pitfalls and benefits of mesocaval shunt placement.     

Inferior mesocaval shunt for bleeding anorectal varices and portal vein thrombosis

Intractable bleeding from anorectal varices is a serious and often misdiagnosed complication of portal hypertension and no agreement has been reached on which could be the optimal diagnostic and therapeutic strategy. Indeed, fatal outcome has been often reported resulting from delayed diagnosis and improper treatment. The case of a 67-year-old gentleman with life-threatening bleeding from anorectal varices who successfully underwent inferior mesocaval shunt is reported, and surgical technique for establishing a shunt between the inferior mesenteric vein and inferior vena cava is described. A review of other therapeutic options is presented and results are discussed and compared to those obtained with this novel form of treatment. In our experience, immediate control of recurrent bleeding from anorectal varices was obtained with inferior mesocaval shunt. Technical ease, promptness of action and effectiveness, low procedure-related morbidity are the main features of the shunt. With the introduction of new promising second-line treatment modalities to primary and metastatic liver tumors, like percutaneous radiofrequency thermal ablation, and improvement in outcome of portal vein thrombosis, the inferior mesocaval shunt may represent a sound alternative for patients who are ineligible for transjugular intrahepatic portosystemic shunt or presenting with clotted shunt.     

Postsurgical chylous ascites in sarcoidosis with portal hypertension

A patient with extensive thromboses of portal and mesenteric veins and sarcoid of the liver developed recurrent pulmonary emboli, which necessitated the insertion of an umbrella into the inferior vena cava. Chylous ascites appeared shortly thereafter; the ascitic fluid fat content was strikingly reduced after reduction of dietary long chain triglycerides, but the ascitic fluid volume was reduced only after insertion of a peritoneovenous shunt (LeVeen). The shunt was found to be nonfunctioning 4 months later, but ascites did not recur. Seven years later, while eating a normal diet and still having a nonfunctioning shunt, he remains free of ascites. We postulate that iatrogenic obstruction of the inferior vena cava in the presence of mesenteric and portal vein thromboses, as well as obstruction of mesenteric lymphatics by sarcoid lymphadenopathy, led to the formation of chylous ascites and that establishment of adequate mesenteric and portal venous and/or lymphatic collateral circulation was responsible for his sustained improvement.     

Left trisegmentectomy and combined resection of the inferior vena cava, without reconstruction, for giant cystadenocarcinoma of the liver

A 54-year-old woman with giant liver cystadenocarcinoma underwent left trisegmentectomy with combined resection of the inferior vena cava (IVC) and the right hepatic vein. As a result, only the right inferior hepatic vein was preserved as a drainage vein. Because the perivertebral plexus and the azygos vein were both well developed, neither veno-venous bypass nor IVC reconstruction was performed. The developed collateral veins acted as the venous drainage pathway to maintain a stable systemic circulation. On the seventh postoperative day, portal vein flow dramatically decreased and the patient tended to liver failure. Prostaglandin E₁ (PGE₁) was administrated via the superior mesenteric artery. The portal flow then gradually increased and liver failure was avoided. Six months after the operation, she was re-admitted due to obstructive jaundice and presented with complete stenosis of the common bile duct (CBD). The jaundice persisted and liver dysfunction progressed. The patient died seven months after the operation. The confluence of the right inferior vein and the IVC could have been deformed, causing outflow blockade. The intrinsic shunt was not good enough to act as the drainage pathway, and IVC reconstruction may have been needed.     

Relationship between encephalopathy and portal vein-vena cava shunt： Value of computed tomography during arterial portography

AIM: TO assess the value of computed tomography during arterial portography (CTAP) in portal vein-vena cava shunt,and analysis of the episode risk in encephalopathy.METHODS: Twenty-nine patients with portal-systemic encephalopathy due to portal hypertension were classified by West Haven method into grade Ⅰ(29 cases), gradeⅡ(16 cases), grade Ⅲ(10 cases), grade Ⅳ( 4 cases). All the patients were scanned by spiraI-CT. Plane scans, artery phase and portal vein phase enhancement scans were performed, and the source images were thinly reconstructed to 1.25 mm. We reconstructed the celiac trunk, portal vein,inferior vena cava and their branches and subjected them to three-dimensional vessel analysis by volume rendering(VR) technique and multiplanar volume reconstruction (MPVR) technique. The blood vessel reconstruction technique was used to evaluate the scope and extent of portal vein-vena cava shunt, portal vein emboli and the fistula of hepatic artery- portal vein. The relationship between the episode risk of portal-systemic encephalopathy and the scope and extent of portal vein-vena cava shunt,portal vein emboli and fistula of hepatic artery- portal vein was studied.RESULTS: The three-dimensional vessel reconstruction technique of spiraI-CT could display celiac trunk, portal vein,inferior vena cava and their branches at any planes and angles and the scope and extent of portal vein-vena cavashunt, portal vein emboli and the fistula of hepatic artery- portal vein. In twenty-nine patients with portal-systemicencephalopathy, grade Ⅰ accounted for 89.7% esophageal varices, 86.2% paragastric varices; grade Ⅱ accounted for 68.75% cirsomphalos, 56.25% paraesophageal varices,62.5% retroperitoneal varices and 81.25% dilated azygos vein; grade Ⅲ accounted for 80% cirsomphalos, 60%paraesophageal varices, 70% retroperitoneal varices, 90% dilated azygos vein, and part of the patients in grades Ⅱand Ⅲ had portal vein emboli and fistula of hepatic arteryportal vein; grade Ⅳ accounted for 75% dilated left renal vein, 50% paragallbladder varices, all the patients had fistula of hepatic artery- portal vein.CONCLUSION: The three-dimensional vessel reconstruction technique of spiraI-CT can clearly display celiac trunk, portal vein, inferior vena cava and their branches at any planes and angles and the scope and extent of portal vein-vena cava shunt. The technique is valuable for evaluating the episode risk in portal-systemic encephalopathy.     

A large inferior mesenteric-caval shunt via the internal iliac vein

A large portosystemic shunt between the inferior mesenteric vein and the right internal iliac vein in a 28-yr-old non-cirrhotic man is presented. This collateral was discovered by ultrasound done as a screening examination for gastrointestinal bleeding. The direct communication of the inferior mesenteric vein with the internal iliac vein was demonstrated by computed tomography and percutaneous transhepatic portography. Surgical ligation of the collateral, performed to prevent future portosystemic encephalopathy, resulted in reduction of serum ammonia level and cessation of long-standing hemorrhoidal bleeding.     

A large inferior mesenteric-caval shunt via the internal iliac vein

A large portosystemic shunt between the inferior mesenteric vein and the right internal iliac vein in a 28-yr-old non-cirrhotic man is presented. This collateral was discovered by ultrasound done as a screening examination for gastrointestinal bleeding. The direct communication of the inferior mesenteric vein with the internal iliac vein was demonstrated by computed tomography and percutaneous transhepatic portography. Surgical ligation of the collateral, performed to prevent future portosystemic encephalopathy, resulted in reduction of serum ammonia level and cessation of long-standing hemorrhoidal bleeding.     

Stent-graft to treat intrahepatic portosystemic venous shunt causing encephalopathy

Spontaneous intrahepatic portosystemic venous shunts (IPSVS) are rare, and angiographic intervention to treat IPSVS has been limited. To our knowledge, we present the first case of an IPSVS between the right portal vein and inferior vena cava accompanied by intractable encephalopathy that was successfully obliterated using a stent-graft deployed in the inferior vena cava to disconnect the portal and systemic venous circulation. Our patient, a 58-year-old woman, has shown no evidence of recurrent portosystemic venous shunt or encephalopathy for 30 months. We believe this intervention to be a useful alternative therapy for this type of shunt.     

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.