Portosystemic Venous Shunt Research Articles

Congenital Intrahepatic Portosystemic Venous Shunt and Liver Mass in a Child Patient: Successful Endovascular Treatment with an Amplatzer Vascular Plug (AVP)

A congenital intrahepatic portosystemic shunt is a rare anomaly; but, the number of diagnosed cases has increased with advanced imaging tools. Symptomatic portosystemic shunts, especially those that include hyperammonemia, should be treated; and various endovascular treatment methods other than surgery have been reported. Hepatic masses with either an intra- or extrahepatic shunt also have been reported, and the mass is another reason for treatment. Authors report a case of a congenital intrahepatic portosystemic shunt with a hepatic mass that was successfully treated using a percutaneous endovascular approach with vascular plugs. By the time the first short-term follow-up was conducted, the hepatic mass had disappeared.

Embolization of congenital intrahepatic porto-systemic shunt by n-butyl cyanoacrylate

Congenital intrahepatic portosystemic venous shunt (IHPSVS) is rare vascular anomaly. We present one case of a 14-month male child who presented with global developmental delay. Child had high ammonia levels with low glutamine and high bile salts on the previous investigations and had history of neonatal seizures since day 13 of life. On admission, serum ammonia levels were elevated to 112micromol/L. Other laboratory investigations including liver and renal function test, and electrolytes were normal. He was, diagnosed to have IHPSVS on the basis of Doppler and CT, and treated by embolization with n-butyl cyanoacrylate (glue). A brief review of diagnostic modalities and endovascular management for the IHPSVS is presented including the present case.

Altered metabolisms of mediators controlling vascular function and enhanced oxidative stress in asymptomatic children with congenital portosystemic venous shunt

Children with congenital portosystemic venous shunt (PSVS) are at risk for developing pulmonary hypertension, irrespective of the severity of portal hypertension or liver damage. Altered metabolisms of nitric oxide (NO) and endothelin-1 (ET-1), which are linked with oxidative stress and control vascular tone, might contribute to the vascular disturbance. This study examined 14 children (aged 1-5 years) with congenital PSVS lacking major liver damage and portal hypertension. Serum levels of nitrite/nitrate (NOx) as stable metabolites of NO, and of asymmetric dimethylarginine (ADMA) as an endogenous NO synthase inhibitor were determined, along with the plasma level of ET-1. Oxidative stress, which might affect the production of such mediators, was also examined using specific urinary and blood markers. The NOx levels were significantly lower in affected children than in the age-matched control group, although ET-1 levels were significantly higher than the control levels. In the affected children, the ADMA levels and ADMA/NOx ratios were higher, respectively, by 30% and 130% and showed significant positive correlations with the shunt ratios. Oxidative stress markers, including plasma thiobarbiturate reactive substances and urinary acrolein-lysine and 8-hydroxy-2′-deoxyguanosine, were significantly higher in affected children than in the control group, consistent with them being subjected to enhanced oxidative stress. These results suggest the presence of altered metabolisms of vascular mediators and enhanced oxidative stress in asymptomatic preschool children with congenital PSVS.

Visceral Venous Aneurysms: Clinical Presentation, Natural History and Their Management: A Systematic Review

Aneurysms of the visceral veins are considered rare clinical entities. The aim is to assess their clinical presentation, natural history and management. An electronic search of the pertinent English and French literature was undertaken. All studies reporting on aneurysms of visceral veins were considered. Cases describing patients with arterial-venous fistulae and extrahepatic or intra-hepatic portosystemic venous shunts were excluded. Ninety-three reports were identified, including 176 patients with 198 visceral venous aneurysms. Patients' age ranges from 0 to 87 years, and there is no apparent male/female preponderance. The commonest location of visceral venous aneurysms is the portal venous system (87 of 93 reports, 170 of 176 patients, 191 of 198 aneurysms). Aneurysms of the renal veins and inferior mesenteric vein are also described. Portal system venous aneurysms were present with abdominal pain in 44.7% of the patients, gastrointestinal bleeding in 7.3%, and are asymptomatic in 38.2%. Portal hypertension is reported in 30.8% and liver cirrhosis in 28.3%. Thrombosis occurred in 13.6% and rupture in 2.2% of the patients. Adjacent organ compression is reported in 2.2% (organs compressed: common bile duct, duodenum, inferior vena cava). The management ranged from watchful waiting to intervention. In 94% of the cases, aneurysm diameter remained stable and no complications occurred during follow-up. In most of the cases, indications for operation were symptoms and complications. Six cases of renal vein aneurysm are reported; three of them were asymptomatic. Three of these patients were treated surgically. The most frequent location of visceral venous aneurysms is the portal venous system. They are often associated with cirrhosis and portal hypertension. They may be asymptomatic or present with abdominal pain and other symptoms. Watchful waiting is an appropriate treatment, except when complications occur. Most common complications are aneurysm thrombosis and rupture. Other visceral venous aneurysms are extremely rare.

Reversible hepatic decerebration: a case report and review of the literature

Hepatic encephalopathy is a syndrome of neuropsychiatric dysfunction caused by portosystemic venous shunting with or without the presence of intrinsic liver disease. Clinical presentations are variable ranging from an abnormal sleep pattern to somnolence and deep coma. Decerebrate and decorticate posturing, have been rarely reported with hepatic encephalopathy. We report a case of a 59-year-old-man with a history of Child-Pugh B liver disease secondary to chronic alcoholism who was admitted because of coma. He had a transjugular intrahepatic portosystemic shunt 3 months prior to his presentation. He was found to have decerebrating posture. He was treated for hepatic encephalopathy with complete recovery and resolution of the neurologic findings. The physician should be aware that decerebration and decortication posture can occur with hepatic encephalopathy and can be reversible.

Large Persistent Ductus Venosus: Retrograde Transvenous Embolization with a Double-catheter Technique

Previous investigators have reported on cases with patent ductus venosus (PDV) successfully treated with transcatheter embolization (1–3). There can be some cases, however, in which it is difficult to place the embolic materials because of the size and shape of the PVD. Herein, we report on a rare case of large persistent ductus venosus that was successfully treated with coil embolization. We attempted the double-catheter method for the case with a large PDV. A 2-year-old girl with hyperammonemia and hypergalactosemia detected with newborn screening tests was diagnosed with a congenital intrahepatic portosystemic venous shunt at abdominal ultrasonography (US).

Abstract No. 251: Coil Embolization for Portosystemic Venous Shunts Without Liver Cirrhosis - Outcomes and Effects to Liver Function

Abstract No. 251: Coil Embolization for Portosystemic Venous Shunts Without Liver Cirrhosis - Outcomes and Effects to Liver Function

A Case of Combined Intrahepatic Portosystemic Shunt, Midgut Malrotation, and Renal Rotational Anomaly in an Adult Patient

An intrahepatic portosystemic venous shunt is a rare condition that was initially reported by Doehner et al. in 1956. In addition, the occurrence of midgut malrotation is rare in adulthood. The association of a portosystemic shunt along with a midgut malrotation and a renal rotational anomaly has not yet been described. We report a case of an intrahepatic portosystemic shunt, midgut malrotation, and renal rotation anomaly.

Spontaneous Intrahepatic Portosystemic Venous Shunt

Purpose: Intrahepatic portosystemic venous shunts are rare and not well recognized. We report the case of a patient with a spontaneous intrahepatic portosystemic venous shunt. Methods: A 48 year old Hispanic woman was referred for a 1.6 cm enhancing lesion of unclear etiology in the right lobe of the liver identified on a CT scan that was performed by her primary care physician for evaluation of her abdominal pain. At her initial office visit she reported mild abdominal discomfort. Physical exam was revealed tenderness in her right upper quadrant. Laboratory evaluation was unremarkable, including normal LFTs, a normal Alpha-fetoprotein, and negative viral hepatitis serologies. MRI of the liver was performed and demonstrated a vascular malformation in the right lobe of the liver being fed by a branch of the portal vein and drained by a tributary of the hepatic vein. This lesion is compatible with a rare spontaneous intrahepatic portosystemic venous shunt. Results: Spontaneous intrahepatic portosystemic venous shunts are rare, described mostly in small series or case reports. More commonly these shunts are acquired in the setting of trauma or developed in chronic liver disease, and are generally due to an abnormal communication between the portal circulation and an extrahepatic systemic vein. The clinical significance and treatment of these shunts remains controversial. Instances of intrahepatic portosystemic venous shunts causing encephalopathy and larger fistulas adversely effecting systemic hemodynamics as well as causing liver fibrosis from longstanding diversion of portal flow have been reported. Treatment options include conservative management, surgery, or transcatheter embolization in symptomatic patients. IPSVS found in asymptomatic patients without liver disease are not thought to require treatment. Conclusion: We report a case of an asymptomatic spontaneous intrahepatic portosystemic venous shunt. The long term sequelae of this anatomic abnormality are not well described.Figure: T1-weighted MRI of the liver demonstrating a vascular malformation in the inferior aspect of the right lobe of the liver being fed by a branch of the portal vein and drained by a tributary of the hepatic vein.

Color Doppler sonography of intrahepatic vascular shunts

To illustrate the sonographic and Doppler features of the different types of intrahepatic vascular shunts. Patients presenting in our department with intrahepatic vascular shunts with healthy livers or with a variety of liver pathologies underwent color Doppler interrogation and spectral analysis. Intrahepatic vascular shunts may involve all liver vessels. Arterioportal and arteriosystemic venous connections can be recognized in normal and cirrhotic livers, following trauma, within tumors, and within nontumoral arteriovenous malformations. Portosystemic intrahepatic venous shunts are categorized into 4 morphologic types. Systemic-to-systemic shunts are more often recognized in patients with chronic hepatic venous congestion or with Budd-Chiari syndrome. Portal-to-portal intrahepatic venous shunts are rare, with few cases described. In general, all the liver vessels can be associated with formation of arterioportal, arteriosystemic, and portosystemic venous shunts and, rarely, systemic venous shunts and portal-to-portal communications. Sonography allows characterization of the different intrahepatic shunts.

Pulmonary Hypertension in Patients With Congenital Portosystemic Venous Shunt: A Previously Unrecognized Association

Pulmonary arterial hypertension has been reported to be observed in association with acquired portal hypertension. However, the contribution of congenital anomalies occurring in the portal system to the development of pulmonary arterial hypertension remains to be elucidated. Nine patients with congenital portosystemic venous shunt were studied from January 1990 through September 2005. Patent ductus venosus was detected in 5 patients, including 3 patients with an absence of the portal vein. The presence of either a gastrorenal or splenorenal shunt was evident in another 4 patients. Six patients had a history of hypergalactosemia with normal enzyme activities, as seen during neonatal screening. Six (66.7%) of the 9 patients were identified to have clinically significant pulmonary arterial hypertension (mean pulmonary artery pressure: 34-79 mm Hg; pulmonary vascular resistances: 5.12-38.07 U). The median age at the onset of pulmonary arterial hypertension was 12 years and 3 months. Histologic studies of lung specimens, which were available in 4 of the 9 patients with congenital portosystemic venous shunt, showed small arterial microthrombotic lesions in 3 patients. This characteristic finding was recognized even in the congenital portosystemic venous shunt patients without PAH. This study demonstrated thromboembolic pulmonary arterial hypertension to be a crucial complication in congenital portosystemic venous shunt, and this pathologic state may be latently present in patients with pulmonary arterial hypertension of unknown etiology.

Role of Magnetic Resonance in Understanding the Pathogenesis of Hepatic Encephalopathy.

A spectrum of neuropsychiatric abnormalities caused by portosystemic venous shunting occurs in hepatic encephalopathy (HE) patients with or without liver dysfunction. It is not completely clear how the astrocyte swelling leads to glial-neuronal dysfunction, and how the symptoms are manifested in HE. A major goal of this work is to review the current status of information available from the existing magnetic resonance (MR) modalities including MR imaging (MRI) and MR Spectroscopy (MRS) as well as other modalities in the understanding the pathogenesis of HE. First, we discuss briefly neuron-histopathology, neurotoxins, neuropsychological and neurophysiological tests. A short review on the progress with single-photon emission computed tomography (SPECT) and positron emission tomography (PET) is then presented. In the remaining part of the manuscript, the following topics pertinent to understanding the pathogenesis of HE are discussed: MRI, diffusion tensor imaging (DTI), one-dimensional MRS based single- and multi-voxel based spectroscopic imaging techniques and two-dimensional MRS.

The clinical anatomy of congenital portosystemic venous shunts

Congenital portosystemic venous shunts are rare. Their gross anatomy has not been well defined. Four different varieties of congenital portosystemic venous shunts are described in six children seen during a 10-year period, focusing on the anatomy of the shunt as determined by imaging studies and surgery. A detailed review of the literature indicates that congenital portosystemic venous shunts are best classified as: extrahepatic or intrahepatic. Extrahepatic shunts may be further subdivided into portocaval shunts (type 1 end-to-side and type 2 side-to-side) and others. Intrahepatic shunts are due to an abnormal intrahepatic connection between the portal vein and hepatic vein/inferior vena cava or a persistent patent ductus venosus. Additional congenital anomalies, particularly cardiac malformations, may be associated with any type. Some congenital intrahepatic portosystemic venous shunts close spontaneously in infancy; all other congenital portosystemic venous shunts tend to remain patent. To a variable extent, depending largely on the volume and duration of the shunt, affected individuals are at risk of developing hepatic encephalopathy and/or an intrahepatic tumor. The key to understanding the pathogenesis of these shunts lies in the normal developmental mechanisms underlying the formation of the portal vein and inferior vena cava in the embryo.

Traumatic Intrahepatic Portosystemic Venous Shunt: A Rare Complication of Grade V Liver Laceration

Oppermann, Timothy E. MD; Corcos, Alain C. MD; Jones, Larry M. MD; Barrette, Roger R. MD; Varcelotti, Jorge R. MD Author Information

Successful embolization using interlocking detachable coils for a congenital extrahepatic portosystemic venous shunt in a child

The authors report the case of a 6-year-old boy with a congenital extrahepatic portosystemic venous shunt. He had hyperammonemia. The shunt was 18 mm in diameter and located between the inferior mesenteric vein and the left internal iliac vein. The flow in the shunt was very rapid. After decreasing blood flow by inflating a balloon catheter inserted into the left internal iliac vein from the femoral vein, a microcatheter was coaxially advanced to the shunt to embolize the shunt. Embolization was successfully performed with interlocking detachable coils and microcoils without any complication. This patient's hyperammonemia resolved soon after the procedure.

A Preliminary Study Revealing a New Association in Patients Undergoing Maintenance Hemodialysis: Manganism Symptoms and T1 Hyperintense Changes in the Basal Ganglia

Patients undergoing parenteral nutrition and those with portosystemic encephalopathy secondary to chronic liver disease and acquired and congenital portosystemic venous shunts frequently present manganese deposition in the basal ganglia, detected by MR imaging as hyperintense areas on T1-weighted sequences. We also observed similar abnormalities in the basal ganglia of patients with chronic renal failure undergoing maintenance hemodialysis. Our aim was to evaluate the pallidal signal intensity on T1-weighted images in a series of patients undergoing hemodialysis, with further evaluation of serum manganese levels and neurologic correlation, comparing them with patients with chronic renal failure without dialytic treatment. We performed MR imaging examinations in 9 patients with chronic renal failure, 5 of whom were undergoing hemodialysis. An experienced neuroradiologist scrutinized the presence of symmetric hyperintensities in the basal ganglia on T1-weighted sequences. We also determined the serum manganese levels and performed the neurologic evaluations in all patients. All patients undergoing hemodialysis presented elevated serum manganese levels and symmetric hyperintensities within the globus pallidus. In this group, 4 patients presented with parkinsonian symptoms, myoclonus, and syndromes with vestibular and vestibular-auditory symptoms. The patients without dialytic treatment presented with neither bilaterally increased T1 MR imaging signal intensity within the globus pallidus nor symptoms of manganism. Our preliminary results demonstrated the occurrence of bilateral pallidal hyperintensity on T1-weighted images in all patients undergoing hemodialysis associated with high serum manganese levels, revealing a new association.

Intrahepatic portosystemic venous shunts: Color Doppler sonography

The aim of the study was to evaluate intrahepatic portosystemic venous shunts (IPSVS) patterns and to determine the role of Color Doppler sonography in the diagnosis and evaluation of related hemodynamic changes in portal perfusion. Sonography and Color Doppler imaging were performed in nine patients with IPSVS. Type and Doppler waveform of the shunt were determined; velocity measurements in the portal trunk and portal branches were studied to evaluate the effects of the shunt on intrahepatic circulation. Computed tomography was performed in six patients, magnetic resonance imaging in three patients, and angiography in two patients. The shunt between the portal and hepatic veins was aneurismal in six patients, while localized peripheral shunt with multiple tortuous vessels in one hepatic segment was observed in three patients. The shunts showed continuous low velocity spectral tracings and in the aneurismal shunts a low velocity bi-directional or helicoidal flow was detected. The feeding portal branches and the draining hepatic veins showed anomalous Doppler tracings and alterations of intrahepatic portal perfusion were observed in three cases. Color Doppler is essential for proper diagnosis of IPSVS and for evaluation of related hemodynamic changes in portal perfusion.

Successful embolisation of intrahepatic portosystemic venous shunt using coils and n-butyl cyanoacrylate through two approach routes

Interventional radiological treatment by transcatheter embolisation has been used to treat patients with symptomatic intrahepatic portosystemic venous shunt (IPSVS). We present a case of symptomatic IPSVS treated by both retrograde and antegrade transcatheter embolisation using coils and n-butyl cyanoacrylate.

Simultaneous resection of liver cell adenomas and an intrahepatic portosystemic venous shunt with elevation of serum PIVKA-II level

A 27-year-old woman with no history of liver disease or oral contraceptive use presented with sudden abdominal pain. Laboratory data showed mild liver dysfunction with jaundice. Computed tomography and angiography revealed centrally located large liver cell adenomas (LCAs) and an intrahepatic portosystemic venous shunt (IHPSS) in the left lobe. The serum des-gamma-carboxy prothrombin (known as "protein induced by a lack of vitamin K or antagonist II," PIVKA-II) level was extremely high (6,647 mAU/ml), indicating malignant transformation of the tumors. Under the diagnosis of LCAs and IHPSS, the patient underwent simultaneous resection of the four liver tumors and portovenous shunt, and the hepatic vascular abnormality was resolved. The pathological diagnosis was LCAs without hepatocellular carcinoma. Immunohistochemical analysis with an anti-PIVKA-II monoclonal antibody showed positive staining of the adenoma cells. This case shows that LCA without malignant transformation can produce PIVKA-II, leading to high serum levels of PIVKA-II. Simultaneous resection of multiple tumors and closure of the portosystemic shunt are strongly recommended in a patient with LCA associated with IHPSS.

Hepatobiliary and pancreatic: Spontaneous intrahepatic portosystemic venous shunt

Hepatobiliary and pancreatic: Spontaneous intrahepatic portosystemic venous shunt