Intrahepatic Portosystemic Venous Shunt Research Articles

Successful Treatment Using Coil Embolization of a Symptomatic Intrahepatic Portosystemic Venous Shunt Developing through a Patent Ductus Venosus in a Noncirrhotic Adult

The patient was a 60-year-old man with encephalopathy without liver cirrhosis. CT angiography revealed a patent ductus venosus between the anterior segmental branch of the portal vein and the middle hepatic vein. Coils were framed in the patent ductus venosus and then used to fill in the frame. After treatment, transarterial portography showed that the shunt flow of the ductus venosus had decreased significantly. After one day, the patient's disturbance of consciousness disappeared. Our case involved the adult-onset of a patent ductus venosus, which is extremely rare. This case is the first in which coil embolization was successfully achieved in a noncirrhotic elderly patient with a patent ductus venosus.

Percutaneous Embolization of Congenital Portosystemic Venous Fistula in an Infant with Down Syndrome

Congenital intrahepatic portosystemic venous shunts are rare vascular malformations often associated with severe complications. We describe a term male infant with Down syndrome with high output heart failure secondary to a congenital arterial to portal venous fistula that was diagnosed by Doppler ultrasound. Percutaneous embolizations of the left hepatic vein, portal vein, and communicating fistulas were performed without complications, resulting in clinical improvement. A subsequent hepatic ultrasound demonstrated resolution of the pathologic fistulous communication and shunting effects.

Color Doppler ultrasonography and contrast-enhanced ultrasound as a diagnostic tool for congenital intrahepatic portosystemic shunts

Objective To evaluate the color Doppler ultrasonography and contrast enhanced ultrasound (CEUS) findings of congenital intrahepatic portosystemic venous shunts (IPSVS).Methods Nineteen patients of congenital IPSVS were examined with color Doppler ultrasonography and CEUS.All patients were confirmed by CT angiography.The hepatic artery arrival time (HAAT),portal vein arrival time (PVAT),and hepatic vein arrival time (HVAT) on CEUS were recorded.The interval time between hepatic artery arrival time and hepatic vein arrival time (HA-HVTT) and the interval time between portal vein arrival time and hepatic vein arrival time (PV-HVTT) were calculated.Results The types of IPSVS between portal and systemic veins were based on Park's classification.Color Doppler ultrasonography showed abnormal communication between the portal vein branch and the hepatic veins,duplex Doppler ultrasonography showed abnormal spectral pattern from the portal vein such as undulating,triphasic waveform mimicked that of the hepatic vein.CEUS demonstrated abnormal communication between portal vein branch and hepatic vein.HVAT,HA-HVTT,and PV-HVTT were shorter statistically in congenital IPSVS group than those in cirrhosis and normal groups.Conclusions Congenital IPSVS is a rare vascular abnormality that is usually asymptomatic.Color Doppler ultrasonography is a useful tool for diagnosis of congenital IPSVS.CEUS provides helpful data for the diagnosis and differential diagnosis of congenital IPSVS. Key words: Ultrasonography; Microbubbles ; Congenital intrahepatic portosystemic shunts

Hepatic encephalopathy due to intrahepatic portosystemic venous shunt successfully treated by balloon occluded retrograde transvenous embolization with GDCs

We report a 65-year-old man with hepatic encephalopathy due to an intrahepatic portosystemic venous shunt that was successfully occluded by balloon occluded retrograde transvenous embolization with Guglielmi and interlocking detachable coils as performed percutaneously.

Intrahepatic Portosystemic Venous Shunt: Successful Embolization Using the Amplatzer Vascular Plug II

A 67-year-old woman presented with memory impairment and behavioral changes. Brain MRI indicated hepatic encephalopathy. Abdominal CT scans revealed an intrahepatic portosystemic venous shunt that consisted of two shunt tracts to the aneurysmal sac that communicated directly with the right hepatic vein. The large tract was successfully occluded by embolization using the newly available AMPLATZERTM Vascular Plug II and the small tract was occluded by using coils. The patient's symptoms disappeared after shunt closure and she remained free of recurrence at the 3-month follow-up evaluation.

RE: Endovascular Treatment of Congenital Intrahepatic Portosystemic Shunts with Amplatzer Plugs

Received August 2, 2011; accepted after revision August 3, 2011. Corresponding author: Sergio Sierre, MD, Department of Interventional Radiology, Hospital Nacional de Pediatria “JP Garrahan”, Combate de los Pozos 1881 (1245), Buenos AiresArgentina. • Tel: (5411) 4308-4330 (x1283) • Fax: (5411) 4308-5325 • E-mail: sergio.sierre@usa.net This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. error related to a claimed statement of primacy, specifically about the use of this kind of device for this indication as mentioned in the fifth paragraph of the discussion section. In our paper entitled “Endovascular treatment of congenital portal vein fistulas with the Amplatzer occlusion device” published in the Journal of Vascular and Interventional Radiology in 2004, we already reported the use of the AVP in the treatment of an intrahepatic portosystemic venous shunt (2). This situation does not undervalue the quality of the reported case, but for didactic purposes, we believe it is important to state that the work of Dr. Lee confirms, as was previously reported, that these devices are useful and safe for these rare situations. Sincerely yours,

An asymptomatic case with multiple intrahepatic portosystemic venous shunts

A 35-year-old male patient was admitted for mild abdominal pain. He had no history of operation, medication, or any injury. Physical examination and laboratory results were normal. Portal vein (PV) Doppler ultrasound showed a shunt between middle hepatic vein (HV) and left branch of PV. Angiography of magnetic resonance imaging of the liver revealed aneurysms and shunts between left PV and middle HV (Fig. 1). Three shunts were seen between segment 2 and segment 3 branches of the left PV and the left HV (Fig.

Multidetector CT findings of right intrahepatic portosystemic venous shunt

Purpose To evaluate multidetector CT features of right intrahepatic portosystemic venous shunt (IPSVS). Materials and Methods Pathways of right IPSVS were evaluated from 20 patients. Diameters of right portal veins were measured in IPSVS patients, 30 cirrhotic and 30 healthy patients. Result Among 22 IPSVSs, shunt between posterior branch and inferior phrenic vein was most common. Diameters of the posterior branch were larger in IPSVS patients than in other groups. Conclusion Most right IPSVSs drain to inferior phrenic vein through dilated posterior branch.

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.

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.

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).

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.

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

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