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.     

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.     

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.     

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.     

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.     

RECURRING ENCEPHALOPATHY ABOLISHED BY GASTRORENAL SHUNT LIGATION IN A DIABETIC HEMODIALYSIS PATIENT

A 57-year-old man was admitted to our hospital for hepatic encephalopathy. He previously had undergone a partial gastrectomy for gastric ulcer, and also had been on maintenance hemodialysis because of diabetic nephropathy. Despite treatment with branched-chain amino acids and lactulose, encephalopathy occurred repeatedly. The findings of his laboratory examinations, computed tomography, and liver biopsy were not suggestive of chronic liver damage. Angiography revealed a portal-systemic shunt from the superior mesenteric vein via the left gastric vein to the left renal vein. A ligation of the gastrorenal shunt was performed. After the shunt ligation, hepatic encephalopathy no longer recurred, and no medication was required to prevent it. The insulin requirements also decreased, the plasma ammonia concentration then decreased, and serum concentration of several amino acids related to the ammonia metabolism also decreased. The molar ratio of branched-chain amino acids to aromatic amino acids increased. The ligation of the portal-systemic shunt was thus considered to be the key to the successful treatment of hepatic encephalopathy in this unusual case.     

Novel therapy for non-cirrhotic hyperammonemia due to a spontaneous splenorenal shunt

Spontaneous splenorenal shunts in the absence of cirrhosis have rarely been reported as a cause hyperammonemia with encephalopathy. Several closure techniques of such lesions have been described. Here we report a case of a patient with no history of liver disease who developed significant confusion. After an extensive workup, he was found to have hyperammonemia and encephalopathy due to formation of a spontaneous splenorenal shunt. There was no evidence of cirrhosis on biopsy or imaging and no portal hypertension when directly measured. The shunt was 18 mm and too large for embolization so the segment of the splenic vein between the portal vein and the shunt was occluded using an Amplatzer plug. Thus, the superior mesenteric flow was directed entirely to the liver. After interventional radiology closure of the shunt using this technique there was complete resolution of symptoms. The case represents the first report of a successful closure of splenorenal shunt via percutaneous embolization of the splenic vein with an amplatzer plug using a common femoral vein approach.     

Clinical and Portal Hemodynamic Features in Cirrhotic Patients Having a Large Spontaneous Splenorenal and/or Gastrorenal Shunt

Clinical and portal hemodynamic features in 28 cirrhotic subjects with a large spontaneous spleno- and/or gastrorenal shunt were studied in comparison with 30 control cirrhotic cases without such collaterals. Forty-six percent of the former had chronic hepatic encephalopathy, but none of the latter was encephalopathic. These patients with large renal shunts were divided into those with and those without encephalopathy. Large esophageal varices were significantly less common in patients with a large shunt and encephalopathy compared with those who had a large shunt but no encephalopathy, and the control. But there was no significant difference of past variceal bleeding among these three groups. In all those with encephalopathy, part of superior mesenteric venous blood was shunting through these collaterals into the left renal vein or inferior vena cava, but the same was not demonstrable in patients with a large shunt and no encephalopathy and control cirrhotics. In the chronic encephalopathic, portal venous flow was estimated to be less than one-half of that in control cirrhotics, and the portion of superior mesenteric venous blood that was flowing hepatofugally through a large shunt into the left renal vein seemed about the same or greater than the portal venous flow. Thus, a large spontaneous spleno- and/or gastrorenal shunt might prevent development of large esophageal varices but not variceal hemorrhage and it increased a risk of chronic hepatic encephalopathy.     

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.     

Hepatic encephalopathy caused by congenital extrahepatic portosystemic venous shunt

Congenital portosystemic venous shunt is a relatively rare disease. Recently, a 60-year-old woman was admitted to our hospital for hepatic encephalopathy caused by congenital extrahepatic portocaval shunt. She had been in good health until the onset of this event, with no liver damage and no experience of abdominal surgery or history of abdominal trauma. In May 1993, hepatic encephalopathy manifested suddenly, with the chief complaint of orthostatic disturbance. Although conservative treatment was administered during the subsequent 5 years, on admission, liver damage and slight splenomegaly were shown, for which complete resection of the shunt vessel and splenectomy were performed. Postoperatively, the patient's symptoms have been alleviated. Hepatic encephalopathy caused by congenital portosystemic venous shunt requires long-term conservative treatment, and the patient's quality of life is reduced. For this reason, surgical intervention or embolization with interventional radiology should be considered, and the maintenance of hepatic blood flow should also be considered.     

Fulminant liver failure induced by hepatosplenic alphabeta T-cell lymphoma

Since survival rates of fulminant liver failure are low, early consideration of liver transplantation in patients developing hepatic encephalopathy due to progressive liver failure is mandatory. Rapid diagnostic work-up is necessary to identify the underlying disease and to rule out contraindications to liver transplantation. We report the case of a 35-year-old patient presenting with fulminant hepatitis and a four-week history of biopsy-proven autoimmune hepatitis. Despite high-dose steroid-treatment liver function progressively worsened and hepatic encephalopathy rapidly developed. Histopathologic evaluation of a liver biopsy specimen revealed necrotizing hepatitis and rare atypical lymphocytes. Surgical biopsy specimens confirmed the suspicion of an aggressive hepatosplenic alphabeta T-cell lymphoma which represents a contraindication to liver transplantation.     

Patent ductus venosus

BACKGROUND: Patent ductus venosus is extremely rare with only 14 cases reported in the world literature. We present a case of patent ductus venosus. METHODS AND RESULTS: A 29-year-old male was admitted with melaena stool caused by gastric haemorrhagic ulcers. Laboratory data disclosed severe anaemia; however, liver function tests were normal. Serum ammonia was also within the normal range. Serological viral markers for hepatitis B or C were all negative. The abdominal ultrasonography and computed tomography indicated a 12 mm diameter shunt located in the left lobe of the liver, which connected the portal vein with the left hepatic vein. After treatment for gastric ulcers, percutaneous transhepatic portography was performed and an enormous shunt connecting the umbilical portion of the portal vein with the left hepatic vein was revealed. CONCLUSIONS: Histological findings of the liver biopsy showed that portal venules could not be observed in the portal areas and that no fibrosis or inflammatory cell infiltration were shown. Because of the anatomical position of the shunt, the case was diagnosed as patent ductus venosus.     

Surgical treatment of a patient with idiopathic portal hypertension and hepatic encephalopathy.

Idiopathic portal hypertension is clinically characterized by splenomegaly and portal hypertension. Hepatic encephalopathy is rare in cases with idiopathic portal hypertension. In a 59-year-old man with recurrent hepatic encephalopathy for one year, a large splenorenal shunt was detected in the computed tomography and angiography, and liver biopsy revealed a portal fibrosis consistent with idiopathic portal hypertension. Devascularization of the upper stomach, splenectomy and closure of the splenorenal shunt were done. The patient has had no experience of encephalopathy since the operation even without drug treatment. Surgery should be considered for treatment of chronic hepatic encephalopathy in patients with idiopathic portal hypertension and portosystemic shunts.     

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.     

Liver transplantation in the setting of a spontaneous shunt between superior mesenteric vein and right renal vein

Shunts between the superior mesenteric vein (SMV) and the right renal vein (RRV) are very rare. Here, we describe and depict the rare case of a liver transplant (LT) in the setting of shunt between SMV and RRV. A 67-year-old white man presenting with Child C cirrhosis secondary to hemochromatosis and persistent encephalopathy was listed for LT. Preoperative abdominal angiotomography revealed the presence of a large spontaneous shunt between the SMV and the RRV. The patient underwent LT by receiving a liver from a 17-year-old brain-dead deceased donor victim of trauma. A large shunt between the SMV and the RRV was confirmed intraoperatively. Although there was no portal vein (PV) thrombosis, the PV was atrophic and had a reduced flow. PV pressure was 22 mmHg (an arterial line was inserted inside the PV stump, and this line was connected to a common pressure transducer, the pressure readings was expressed in the anesthesia monitor). After shunt ligation PV pressure increased to 32 mmHg. There were no post-transplant vascular complications, and the patient was discharged home in good health. Preoperative study of all LT candidates with angio CT scan is mandatory. Whenever there is PV thrombosis, an attempt to remove the entire thrombus is warranted. After thrombectomy or whenever there is not PV thrombosis, all large shunts should be ligated. PV pressure and flow should be measured before and after shunt ligation. In the absence of PV thrombosis, ligation of the shunt should enable an increase in PV flow and pressure, as reported herein.     

Chronic portal-systemic encephalopathy with normal portal vein pressure possibly due to noncirrhotic portal fibrosis

Summary This is the report of a 50-year-old man with a more than 20-year history of chronic progressive portal-systemic encephalopathy. Liver tests were normal except for increased serum ammonia and indocyanine green plasma retention. The liver pathology was compatible with idiopathic portal hypertension or noncirrhotic portal fibrosis, demonstrating localized surface nodularity and portal fibrosis. Percutaneous transhepatic catheterization of the portal vein revealed near top normal portal vein pressure and a large shunt connecting the left gastric or superior mesenteric vein and the left renal vein. Presumably, the patient had portal hypertension in the past and formation of a short, largecaliber shunt between the portal system and the renal vein effectively decompressed the portal circulation.     

Development of portal vein thrombosis complicating idiopathic portal hypertension. A case report

A 58-yr-old woman with biopsy-proven idiopathic portal hypertension presented with ascites and pretibial pitting edema. On admission, ultrasonic Doppler flowmetry demonstrated hepatopetal flow of a markedly reduced velocity in the portal vein, hepatofugal flow in the splenic vein, and a large spontaneous splenorenal shunt. The patient spontaneously developed hepatic encephalopathy 1 mo later. Percutaneous transhepatic portography demonstrated mural thrombi at the porta hepatis after the catheter had penetrated the mural thrombi without resistance; there was also a long retention of contrast medium in the portal vein. 99mTc-Macroaggregated albumin instilled into the superior mesenteric vein was caught in the lungs, and no activity entered the liver. Measurements of ammonia and immunoreactive insulin clearly indicated that superior mesenteric venous blood was shunted through the splenic vein and the splenorenal shunt. Subsequent ultrasonic examination with Doppler flowmetry suggested further growth of the thrombi and lack of blood flow in the portal vein. Although the procedure of percutaneous transhepatic catheterization could have contributed to the growth of thrombi, it is more likely that the thrombosis in the portal vein was a sequela to idiopathic portal hypertension, and was growing at the time of catheterization. This case may be of significance in the understanding of the relationship between idiopathic portal hypertension and extrahepatic portal obstruction.     

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.     

Combination treatment of transjugular retrograde obliteration and endoscopic embolization for portosystemic encephalopathy with esophageal varices

The treatment of chronic portosystemic encephalopathy with esophageal varices has not yet been established. We were able to control a case of chronic portosystemic encephalopathy with esophageal varices using a combination treatment of transjugular retrograde obliteration and endoscopic embolization. A 57-year-old man came to our hospital in a confused, apathetic and tremulous state. The grade of encephalopathy was II. The plasma ammonia level was abnormally elevated to 119 microg/dL, and the ICGR15 was 59%. Endoscopic examination revealed nodular esophageal varices with cherry-red spots. There were no gastric varices. Ultrasonography and CT revealed liver cirrhosis with a splenorenal shunt. We first applied endoscopic embolization for the esophageal varices before transjugular retrograde obliteration. We injected 5% ethanolamine oleate with iopamidol retrogradely into the esophageal varices and their associated blood routes under fluoroscopy and obliterated the palisade vein, the cardiac venous plexus and left gastric vein. Transjugular retrograde obliteration was performed 14 days after endoscopic embolization. Retrograde shunt venography visualized the splenorenal shunt and communicating route to the retroperitoneal vein. There was no communicating route to the azygos vein. After obliteration of the communicating route to the retroperitoneal vein with absolute ethanol, 5% ethanolamine oleate with iopamidol was injected into the splenorenal shunt as far as the root of the posterior gastric vein. After transjugular retrograde obliteration, the encephalopathy improved to grade 0 even without the administration of lactulose and branched-chain amino acid. The plasma ammonia level and ICGR15 were reduced to 62 microg/dL and 26%. We conclude that combination treatment of transjugular retrograde obliteration and endoscopic embolization is a rational, effective and safe treatment for chronic portosystemic encephalopathy complicated with esophageal varices.