Portosystemic Venous Shunt Research Articles

2452 Intrahepatic Portosystemic Venous Shunt: A Clinically Significant Rare Anomaly

INTRODUCTION: Intrahepatic Portosystemic Venous Shunts (IPSVS) are rare vascular malformations between the hepatic and portal veins. Most of the porto-venous shunts are asymptomatic and found incidentally on radiology imaging. These can be either congenital or acquired. Acquired shunts are typically less than 2 mm in size. Occasionally, complications such as encephalopathy, heart failure, pulmonary hypertension and elevated ammonia levels can occur. We report a case of incidental IPSVS in a patient who presented with complaints of fatigue and failure to thrive. CASE DESCRIPTION/METHODS: 82-year-old African American male with hypertension, diabetes mellitus and prostate cancer (treated) presented with complaints of fatigue and loss of appetite for one-month. Laboratory work up showed a hemoglobin of 11.6 g/dl, normal platelet count, and elevated aspartate aminotransferase of 45 U/L and decreased albumin of 2.0 g/dl. Serum ammonia level was normal. He was immune to hepatitis A, non immune to hepatitis B and hepatitis C antibody was negative. A computed tomography scan of the abdomen showed a contrast enhanced tubular shaped vascular connection between middle hepatic vein and the anterior branch of the right portal vein. The size of the shunt was approximately 10 mm (Figure 1). Liver appeared normal except for small calcifications within the parenchyma. Ultrasound Doppler of hepatic and portal veins were performed, and the porto-hepatic shunt was visualized (Figure 2). Patient had a complicated hospital course secondary to sepsis; however, he did not develop hyperammonemia or hepatic encephalopathy during the hospital stay. DISCUSSION: IPSVS in our patient was likely a congenital anomaly given the presentation, size and lack of related symptoms. IPSVS is divided into 4 types; Type 1- Shunt between portal vein and IVC; Type 2- connections between peripheral branches of portal and hepatic veins in one hepatic segment; Type 3- Aneurysmal connection between portal and hepatic vein; Type 4- multiple communications of portal and hepatic veins in both major lobes of the liver. Doppler sonogram with pulse doppler is highly sensitive and specific to evaluate for IPSVS. In symptomatic IPSVS, shunt closure has shown improvement in symptoms and correction of hyperammonemia. Clinicians should consider this rare vascular malformation in patients presenting with hepatic encephalopathy or hyperammonemia in the absence of cirrhosis where shunt closure can be curative.

Analysis of influencing factors on hepatic encephalopathy in patients with non-cirrhotic portal hypertension

Objective: To investigate the incidence of hepatic encephalopathy (HE) in patients with non-cirrhotic portal hypertension (NCPH) and to explore its risk factors. Methods: The incidence rate of HE in 150 cases with NCPH was evaluated in two hospitals, and 188 cases of compensated cirrhosis patients were taken as control. Logistic regression was used to screen for independent risk factors for HE in patients with NCPH. Results: The incidence of overt hepatic encephalopathy (OHE) in patients with NCPH was not statistically significantly different from that in patients with cirrhosis (4.7% vs. 6.9%, P = 0.682). The incidence of mild hepatic encephalopathy (MHE) was significantly lower than that of cirrhosis patients (32.7% vs. 46.3%, P < 0.05). The presence of upper gastrointestinal bleeding, infection and portosystemic venous shunt were the main independent factors for HE in NCPH patients (OR > 1, P < 0.05). Conclusion: HE is one of the important complications of NCP, and may be influenced by factors such as upper gastrointestinal bleeding, infection and portosystemic venous shunt.

Hepatic encephalopathy - current considerations

Hepatic encephalopathy (EH) is a syndrome involving a series of neuropsychiatric abnormalities in patients with hepatocellular damage and/or portosystemic venous shunts in the absence of other cerebral diseases. It is the late consequence of portal hypertension with a high degree of spontaneous or surgically created portosystemic shunts. The pathogenic substrate is the diffuse disturbance of cerebral metabolism due to insufficient processing or shunting of the liver by intestinal toxic nitric products. The clinical evaluation by psychometric tests may reveal minimal EH for an adequate early management and preservation of the integrity of cerebral functions in patients with cirrhosis.

Intrahepatic Portosystemic Venous Shunt in a Non-Cirrhotic Adult Female with Encephalopathy

Introduction: Intrahepatic portosystemic venous shunts (IPSVS) are rare anomalous vascular connections within the liver that are often asymptomatic. However, significant shunting of blood may present as encephalopathy. Cross sectional imaging and Doppler studies are essential to characterize the shunt and determine portosystemic shunt ratios. High flow shunts may be treated using coil embolization or surgery. Result: We present the case of a 57-year-old previously asymptomatic lady who was admitted for confusion and behavioral change. Work-up revealed deranged liver function and hyperammonemia. Triphasic CT scan showed an aneurysmal connection between the right anterior portal vein and the middle hepatic vein measuring 1.9 cm in widest diameter. The left portal vein was small. The liver had homogenous parenchyma and smooth borders. Aside from small hepatic cysts, there were no other lesions detected. Doppler ultrasound showed turbulent flow across the connection with a portovenous shunt ratio of 73%. The size of the shunt precluded safe coil embolization. Surgical treatment was then opted for and she underwent a hepatotomy from the superior border of the liver until the aneurysmal shunt connection was identified and isolated. The connection was then divided between clamps and the stumps sutured close. Blood flow in the left portal vein then increased immediately. Her postoperative course was unremarkable and she was discharged improved. She remains well 8 months after surgical correction of the shunt. Conclusion: A high index of suspicion should prompt the search for IPSVS in patients presenting with encephalopathy in the absence of liver cirrhosis. Imaging studies are essential in assessing the type and severity of the portovenous shunting and in guiding the management. Symptomatic patients with high shunt ratios should be treated promptly using interventional radiologic procedures or surgery.

Asymptomatic Intrahepatic Portosystemic Venous Shunt: Case Report and Review of Literature

A spontaneous portosystemic shunt is a rare malformation of the liver vessels. The etiology of these shunts is controversial. They can be divided into acquired (most commonly associated with liver cirrhosis) and congenital. Vascular shunts are asymptomatic in the majority of the patients, and when symptomatic were presented by severe complications. The specific way of management can be selected depended on whether the patient was symptomatic or not, and also on the size of the shunt, shunt ratio and whether it was associated with cancer. We will present a clinical case and discuss the importance of the radiological imaging in the screening, diagnosis and follow up of these anomalies.

Abernethy malformation: Our experience from a tertiary cardiac care center and review of literature.

Abernethy malformation, also called as congenital extrahepatic portosystemic venous shunt, is a rare anomaly involving the portal venous system. Although rare, it is increasingly being reported and is important to diagnose given the adverse clinical consequences in untreated patients. It has myriad of clinical presentations, from being completely asymptomatic to causing hepatic carcinoma, hepatic encephalopathy, severe pulmonary hypertension, and diffuse pulmonary arteriovenous malformation. We describe our experience with five cases in a tertiary pediatric cardiac care center with Abernethy malformation, with review of literature and also discuss possible therapeutic implications.

Ultrasound with Doppler evaluation of congenital hepatic vascular shunts.

Congenital aberrant hepatic vascular communications result from intrahepatic or extrahepatic errors in vascular development or involution during the transition from fetal to newborn hepatic circulation. These hepatic vascular shunts can be fortuitously discovered and asymptomatic, or can cause symptoms of varying severity, often presenting diagnostic dilemmas. Some hepatic vascular shunts resolve spontaneously while others require interventional radiologic or surgical closure. Affected patients are often first studied with real-time and Doppler ultrasound, so radiologists should familiarize themselves with the expected ultrasound findings of these vascular shunts for effective diagnosis, triage and management. In this review, the authors focus on ultrasound and Doppler findings of hepatic vascular shunts with underlying embryology, clinical features and management strategies. Broadly, these aberrant hepatic vascular communications include portosystemic venous shunts (which can be intra- or extrahepatic and include persistent patent ductus venosus), arterioportal, arteriovenous or mixed shunts.

Effective shunt closure for pulmonary hypertension and liver dysfunction in congenital portosystemic venous shunt.

Congenital portosystemic venous shunt (CPSVS) is a rare vascular malformation with a high risk of mortality from pulmonary arterial hypertension (PAH), but the treatment outcome of CPSVS closure remains elusive. Our aim was to investigate the clinical features and establish the optimal management of CPSVS with or without PAH. Twenty-four patients with CPSVS treated in Kyushu University Hospital between 1990 and 2015 were enrolled in this study. The patients were divided into a PAH group (n = 9) and a non-PAH group (n = 15). Clinical characteristics and outcomes were evaluated. The first manifestation of CPSVS at diagnosis (28.5 [1-216] months) was hypergalactosemia in 13 (54%) or PAH in six (25%) patients. PAH was the cause of all three deaths. The PAH group had higher levels of serum total bile acid, manganese, and total bilirubin, along with higher pulmonary vascular resistance index (PVRI) than the non-PAH group (7.2 [5.1-38.1] vs 1.2 [0.5-3.3] unit/m2 , P < 0.001). Five of nine PAH patients underwent CPSVS closure at a median of 38 months (range 21-118) after PAH diagnosis. Pulmonary artery pressure improved after CPSVS closure with PAH-specific therapy, but normal range was not achieved. CPSVS closure improved the hepatic synthetic function of four PAH patients. Eigh-t of 15 non-PAH patients who received CPSVS closure did not develop PAH for a median of 34.5 months (range 6-164) after the procedure. CPSVS closure with PAH-specific therapy successfully controlled PAH. Early CPSVS closure may prevent the occurrence and progression of PAH with CPSVS.

J-Pouch Ectopic Varices: A Rare Cause of Lower GI Bleed

Variceal bleeding is a life-threatening complication of portal hypertension in patients with cirrhosis. Varices are portosystemic venous shunts that arise as a result of portal hypertension, most commonly located in the gastroesophageal (GE) area. Ectopic varices (ECV) are enlarged portosystemic venous collaterals located anywhere outside of the GE region. ECV bleeds are uncommon but can lead to significant morbidity and mortality. We present a rare case of lower GI bleeding from a small bowel varix in a J-pouch. A 34-year-old female with a history of ulcerative colitis status post total proctocolectomy with ileal pouch-anal anastomosis (IPAA or J-pouch) and primary sclerosing cholangitis with compensated cirrhosis was transferred to our hospital for an overt, obscure GI bleed. She initially presented to an outside hospital (OSH) with hematochezia and syncope. EGD, push enteroscopy, pouchoscopy, and capsule endoscopy at the OSH all failed to find the source of bleeding. Due to continued overt bleeding requiring 14 U of packed RBCs over 10 days, she was transferred to our medical center. Repeat EGD found no blood and no gastroesophageal varices. Pouchoscopy revealed red blood and clots in the J-pouch, but normal underlying mucosa and no obvious source of bleeding. A tagged RBC scan was positive for bleeding localized to a loop of small bowel in the right lower quadrant. CT-angiogram demonstrated a large ectopic venous collateral adjacent to the IPAA (Fig. 1) which was supplied by the superior mesenteric vein and drained by the right gonadal vein (Fig. 2), consistent with a small bowel varix. This ECV was the most likely cause of her recurrent bleeding. She underwent TIPS with reduction of portosystemic gradient from 25 to 11 mmHg and significant decompression of the ECV, rendering selective embolization unnecessary. She was discharged 5 days later and had no further bleeding at a 2 month follow-up visit. ECV account for 1-5% of all variceal bleeding episodes. Small bowel varices are most commonly seen in patients with portal hypertension and prior abdominal surgery. A triad of portal hypertension, hematochezia without hematemesis, and previous abdominal surgery should prompt evaluation for small bowel varices via CT scan with both arterial and venous phases. This case illustrates that, although rare, small bowel varices can present as massive lower GI bleeding and require a multidisciplinary approach with therapy tailored based on institutional expertise.Figure: CTA venous phase showing large venous collateral adjacent to J-pouch consistent with small bowel varix.Figure: Venogram illustrating enlarged superior mesenteric vein (SMV) filling varix with outflow tract through the right gonadal vein (R Gonadal V.).

Complications in transcatheter closure of congenital portosystemic venous shunt using Amplatzer Vascular Plug

Abstract Amplatzer Vascular Plug (AVP) has been utilized to occlude a congenital portosystemic venous shunt (CPSVS) that is a rare splanchnic venous anomaly. We present a case of a 12-year-old boy in whom device migration and portal vein thrombosis occurred after transcatheter closure of CPSVS using AVP type2. We reviewed the published literatures regarding with complications of CPSVS closure using AVP. Among 23 patients, we identified 5 complications including hypovolemic shock, hypoxia, liver dysfunction, device migration, and thrombosis. It is recommended to choose a device more than twice the diameter of the shunt, and to treat with an anticoagulant after closure.

Disappearance of globus pallidum lesions in T1-weighted magnetic resonance images after ligation of congenital portosystemic venous shunt

Disappearance of globus pallidum lesions in T1-weighted magnetic resonance images after ligation of congenital portosystemic venous shunt

Congenital Intrahepatic Portosystemic Venous Shunt With an Associated Aneurysm: Sonographic Diagnosis

Intrahepatic portosystemic venous shunts with associated aneurysms are extremely rare anomalous communications between intrahepatic portal veins and systemic veins through intrahepatic venous channels. Intrahepatic portosystemic venous shunts are usually asymptomatic but can be the cause of hepatic encephalopathy and hypoglycemia, especially when a high degree of shunting exists. The sonographic incidental finding of an aneurysmal vascular connection between the left portal vein and left hepatic vein as diagnosed by sonography is presented and discussed. When evaluating patients with suspected symptoms of hepatobiliary disease, sonographers and radiologists should carefully scan and meticulously assess the liver for signs of abnormal venous communications.

Hepatic Encephalopathy; Prevalence, Precipitating Factors and Challenges of Management in a Resource-Limited Setting

Background and Aims: Hepatic encephalopathy, a neuropsychiatric syndrome caused by portosystemic venous shunting, clinical presentation ranges from minimal to overt H.E. It is a common complication of advanced liver disease with significant morbidity and mortality The aim of this study was to assess the prevalence, common precipitating factors, and outcomes of hepatic encephalopathy in patients with preexisting liver disease. Methods: A hospital based, retrospective chart review study was conducted at Bugando Medical Centre a tertiary hospital in Mwanza region. The target population included all patients aged > 18 years admitted with Hepatic encephalopathy from January 2009 to June 2015. Patients were enrolled using a detailed checklist, personal identifications were removed and analysis was done using the SPSS version 17.0. Results: A population of 88 patients with Hepatic encephalopathy were enrolled with a mean of 47 years (SD +/-17 years). Among patients admitted to the medical ward, the prevalence was 0.4% (88/23942). Most common liver disease and complications including alcoholic cirrhosis, hepatitis B infection, and hepatocellular carcinoma were present in 47.7% of (42/88), 22.7% (20/88), and 23.9% (21/88) patients, respectively. Majority had West Haven grade 3, 36.4% (32/88), and grade 4, 18.2% (16/88). Precipitating factors included diuretic therapy on patients with ascites 27.2% (44/162), infections 21.6% (35/162), blood transfusions, 16.7% (27/162), and upper gastrointestinal bleeding 17.3% (28/162). Most died during their hospitalization, 75% (66/88). The remaining 25% (22/88) were discharged. Majority of the cohort, 72.7% (64/88), had less than 3 months survival after diagnosis, while 27.3% (24/88) survived more than 1 year. Conclusions: We found severe Hepatic encephalopathy presenting in patients with preexisting liver disease associated with poor outcomes, posing challenges in management and survival. The use of newer and superior agents like polyethylene glycol, identification of subclinical Hepatic encephalopathy and targeting early removal of precipitating factors is imperative. Screening those at risk of developing Hepatic encephalopathy would likely improve outcomes.

Catheter interventional treatment of congenital portosystemic venous shunts in childhood.

The study aimed on the catheter interventional treatment of congenital portosystemic venous shunt (CPSVS) in childhood and the impact on vascular growth of hypoplastic portal veins. Clinical course and follow up of partial or complete closure of CPSVS are described. CPSVS is a very rare vascular malformation of the portal venous drainage resulting in potentially life threatening abdominal, pulmonary, cerebral, and cardiac complications. The anatomic type, clinical course, and diagnosis must be determined for optimal management. Single centre case series. Eight (6 female) children with extrahepatic (n = 5) and intrahepatic (n = 3) CPSVS were diagnosed invasively by catheterization, including test balloon occlusion of the shunt and simultaneous retrograde angiography, and treated by catheter interventions (n = 5) with partial (n = 2) and complete (n = 3) occlusion of CPSVS at a median age of 3.9 years (range 0.7-21). Congenital heart disease (CHD) was the most frequent associated organ manifestation (n = 5) followed by mild to severe pulmonary arterial hypertension (n = 4), hepatopulmonary syndrome (n = 2), and portosystemic encephalopathy (n = 1). CHD was simple (n = 3) or complex type (n = 2). Three patients were untreated so far, because they were in excellent clinical condition at an age <1 year, refused treatment, or planned for later treatment. Accurate invasive diagnosis of CPSVS with test balloon occlusion of the shunt is mandatory to depict the anatomic situation. Catheter interventional treatment of CPSVS offers a feasible and safe approach with complete or partial closure of the vascular malformation inducing potentially significant vascular growth of a former hypoplastic portal venous system. © 2015 Wiley Periodicals, Inc.

Transhepatic Embolization of Congenital Intrahepatic Portosystemic Venous Shunts with Associated Aneurysms.

Intrahepatic shunts between the portal and systemic venous systems with associated aneurysms are extremely rare. A middle aged woman presented with hepatic encephalopathy and was found to have two intrahepatic portosystemic venous shunts with associated aneurysms. Diagnosis was made by duplex ultrasound and was confirmed with contrast enhanced MRI. Treatment was performed percutaneously with an Amplatzer vascular plug.

Endovascular Treatment of Symptomatic Intrahepatic Portosystemic Venous Shunt Using Amplatzer Vascular Plug II

Endovascular Treatment of Symptomatic Intrahepatic Portosystemic Venous Shunt Using Amplatzer Vascular Plug II Spontaneous intrahepatic portosystemic venous shunt (IPSVS) is a rare condition that implies abnormal communication between portal and systemic venous system within the liver parenchyma. It can be asymptomatic or present with symptoms of hepatic encephalopathy. We show a case of 74-year-old female patient with symptomatic IPSVS and successful endovascular treatment using Amplatzer Vascular Plug (AVP) II.

Ultrasound diagnosis of congenital intrahepatic portosystemic shunt

To investigate the ultrasonographic features of congenital intrahepatic portosystemic venous shunt (CIPSVS) and to assess the clinical value of ultrasonography in the diagnosis of CIPSVS. Six cases of CIPSVS diagnosed in our hospital between March 2010 and March 2012 and confirmed by enhanced computed tomography (CT) were retrospectively reviewed. Five of the six cases had follow-up data that was included in the analysis. Among the six CIPSVS cases, only one was classified as Park's type II and the rest were classified as Park's type III. Five cases involved the right lobe of the liver and only one case involved the left lobe. The lesion shapes included cystic, tubular, and irregular with clear contour and appeared to be anechoic on CT scan. The lesions ranged in size from 1.1*0.6 cm to 2.0*1.7 cm. For all cases, the color Doppler ultrasound images showed blood flowing from the portal vein to the hepatic vein, and single-phase spectrum was detected in the diversion channel. The differences observed in level of lesion size and blood flow velocity at the shunt from the time of examinations at diagnosis and subsequent follow-up did not reach statistical significance (P = 0.223 more than 0.05 and P = 0.930 more than 0.05 respectively). Although cases of CIPSVS are rare, they share some specific sonographic features that may help in diagnosis. Color Doppler ultrasound findings have high diagnostic accuracy and may represent a preferred modality for follow-up monitoring.

Infusion of 50 % glucose solution to occlude an intrahepatic portosystemic venous shunt before percutaneous transhepatic portal embolization: report of a case

A 68-year-old man with cholangiocarcinoma underwent percutaneous transhepatic portal embolization to expand the indication for hepatic resection. Selective right posterior portography revealed an intrahepatic portosystemic venous shunt (IPSVS) connecting the segment VII branch to the right hepatic venous branch. An infusion of 50% glucose solution was given to occlude the shunt. This is novel management for IPSVSs when they are numerous, small, or torturous, and makes the subsequent procedures simpler, shorter, and less expensive.

Adult-onset seizures in a patient with Down syndrome and portosystemic shunt

Objective: The prevalence of epilepsy in patients with Down syndrome (DS) is 5–13%, which is higher than the prevalence in the general population. Transient hyperammonemia is often observed following seizure, but it typically resolves within a day. Here, we describe the case a 37-year-old woman who had DS and a history of adult-onset epilepsy and was admitted to our hospital with recurrent seizures. After admission, her ammonia levels fluctuated without any apparent cause, and dynamic computed tomography revealed a portosystemic shunt. The findings suggest that her seizures possibly precipitated from hyperammonemia secondary to a portosystemic shunt, and we reviewed the relevant literature. Methods: We conducted PubMed, Web of Science, and EMBASE searches without language restrictions for articles published between 1970 and February 2013. Results: In addition to the present case, 7 cases were ultimately included in this review. Four patients were newborns, 2 patients were 1month old, and 1 patient was 3years old. No adult cases were described until now. Conclusion: Adult patients with DS diagnosed with epilepsy are not routinely assessed for portosystemic venous shunts. Measuring ammonia levels in patients with DS the day after admission would help detect portosystemic shunts, even if the patients have been previously diagnosed with epilepsy. Practice Implications: If ammonia levels fluctuate without any apparent cause after seizure, dynamic computed tomography should be performed, especially for patients with DS, whether or not they have been previously diagnosed with epilepsy.

An unusual case of intrahepatic portosystemic venous shunt

Intrahepatic portosystemic venous shunts are rare vascular anomalies that may be detected in asymptomatic patients, given the recent advances in radiological imaging techniques. Accurate shunt evaluation and classification can be performed with ultrasound and multi-detector computed tomography. We report an unusual case of an intrahepatic portosystemic venous shunt with an incidental finding of neurofibroma.