Intrahepatic Shunt Research Articles

Detection of intrahepatic veno-venous shunts by three-dimensional venography using multidetector-row computed tomography during angiography

The hepatic vein (HV) can be removed during hepatectomy if there is an effective intrahepatic veno-venous shunt (vv-shunt). We evaluated the efficacy of vv-shunt detection by three-dimensional (3D) venography reconstructed from multidetector-row computed tomography (MDCT) during angiography. 3D venography was reconstructed using computer software in 88 patients with intrahepatic tumors. We found that 12 patients had one shunt [4 right hepatic vein (RHV)-middle hepatic vein (MHV) and 12 RHV- inferior right hepatic vein (IRHV)] and 1 patient had 2 shunts (RHV-MHV and -IRHV), confirming a clinically efficient vv-shunt in 14.8% of the patients. In one patient with an RHV-IRHV shunt, the preserved RHV-IRHV shunt worked well and prevented congestion of the postero-caudal subsegment after central bisegmentectomy with partial resection of the RHV ventral trunk for huge hepatocellular carcinoma (HCC). Although the vv-shunt detection rate by 3D venography is low, a visualized vv-shunt proved to be efficient. Thus, invasive occlusion venography is avoidable if a vv-shunt is seen on 3D venography.

Congenital Portosystemic Shunt

Congenital portosystemic shunts (CPSSs) are rare but increasingly recognized as a cause of important multisystem morbidity. We present new cases and a systematic literature review and propose an algorithm for the identification and care of affected patients. We reviewed the charts of consecutive patients seen in our pediatric liver clinic between 2003 and 2010 and systematically reviewed the literature of cases with CPSS. We identified 316 published cases and 12 patients in our own clinic. Of the published cases (177 male), 185 had an extrahepatic and 131 an intrahepatic portosystemic shunt. Diagnosis was made at any age, from prenatal to late adulthood. Cardiac anomalies were found in 22% of patients. The main complications were hyperammonemia/neurological abnormalities (35%), liver tumors (26%), and pulmonary hypertension or hepatopulmonary syndrome (18%). The spectrum of neurological involvement ranged from changes in brain imaging, subtle abnormalities on neuropsychological testing, through learning disabilities to overt encephalopathy. Spontaneous shunt closure occurred mainly in infants with intrahepatic shunts. Therapeutic interventions included shunt closure by surgery or interventional radiology techniques (35%) and liver transplantation (10%) leading to an improvement of symptoms in the majority. These findings mirror the observations in our own patients. In this largest review of the reported clinical experience, we identify that children with CPSS may present with otherwise unexplained developmental delay, encephalopathy, pulmonary hypertension, hypoxemia, or liver tumors. When CPSS is diagnosed, children should be screened for all of these complications. Spontaneous closure of intrahepatic shunts may occur in infancy. Closure of the shunt is indicated in symptomatic patients and is associated with a favorable outcome.

Educational Exhibit Abstract No. 368 - Portal vein interventions

Learning Objectives To discuss various portal venous interventions offered by interventional radiology including their indications, contraindications, clinical utility, expected outcome, and common complications. Background Different portal vein interventions include portal vein stent (PVS), portal vein embolizaion (PVE), transjugular intrahepatic portosystemic shunt (TIPS), and direct intrahepatic portocaval shunt (DIPS). Clinical Findings/Procedure Details Portal Vein Stent (PVS ): Performed in the setting of portal venous stenosis or occlusion that commonly occurs in neoplastic conditions or post liver transplantation. Liver abscess has been reported as a post procedural complication. Portal Vein Embolization (PVE) : Performed in preoperative treatment of major hepatic resection in patient when the future liver remnant is too small. PVE complications are rare but include transient hemobilia, infection, need for reembolization, portal vein thrombosis, and portal hypertension. Portal Vein Thrombolysis (PVT) : PVT is the preferred treatment for acute intravascular thrombosis. Percutaneous portal vein clot lysis can be initiated in a few minutes providing almost immediate clot dissolution without the need for surgical intervention. Transjugular Intrahepatic Portosystemic Shunt (TIPS) : Indications for TIPS procedure include recurrent or refractory variceal bleeding, Budd Chiari syndrome, hepatic hydrothorax, hepatorenal syndrome, and intractable ascites. Complications include but are not limited to hepatic encephalopathy, heart failure. There is improved survival of patients with refractory ascites when treated with TIPS. Direct Intrahepatic Portocaval Shunt (DIPS) : Indications are similar to TIPS procedure for decompression of portal venous system. DIPS procedure includes direct puncture from the IVC through the caudate lobe to the main portal vein. The safety and effectiveness is increased by eliminating blind PV puncture and hepatic vein stenosis which is the most common cause of chronic TIPS failure. Conclusion and/or Teaching Points Knowledge of portal vein interventions including their indication, clinical utility and expected outcome is critical for clinicians to improve patient care.

Percutaneous transhepatic variceal embolization with cyanoacrylate vs. transjugular intrahepatic portal systematic shunt for esophageal variceal bleeding.

To compare the long-term results of modified percutaneous transhepatic variceal embolization with cyanoacrylate (PTVE) and the transjugular intrahepatic portal systemic shunt (TIPS) for treating esophageal variceal bleeding. Patients with cirrhosis and variceal bleeding who underwent TIPS and PTVE with cyanoacrylate between January 2006 and December 2010 were selected. We performed chart reviews to determine the rebleeding rate, survival and the rate of encephalopathy. This retrospective study included 96 PTVE patients and 43 TIPS patients, with a median follow-up of 30.4 and 31.6months in the two groups, respectively. Rebleeding occurred in 13 patients (30.2%) in the TIPS group and in 20 patients (20.8%) in the PTVE group (p=0.229). For patients with model for end-stage liver disease (MELD) scores>18 at 1, 3 and 5years, the survival rates were 84.2, 39.9 and 16.0%, respectively, in the TIPS group, and they were 96.7, 72.0 and 36.0%, respectively, in the PTVE group (p=0.037). Sixteen (16.7%) PTVE patients and 25 (58.1%) TIPS patients developed encephalopathy (p=0.000). Mean MELD and Child-Pugh scores improved significantly in modified PTVE patients. However, no such changes were observed in TIPS patients. PTVE and TIPS were comparable in terms of variceal rebleeding prevention. However, in>18-MELD-score patients, PTVE offered better survival than TIPS. In addition, PTVE offered lower incidence of encephalopathy than TIPS.

Congenital Portosystemic Shunts in Children: Recognition, Evaluation, and Management

Congenital portosystemic shunts are present in one in 30,000 children. Among the associated risks of severe complications are neonatal cholestasis, benign and malignant liver tumors, hepatopulmonary syndrome, portopulmonary hypertension, and encephalopathy. They can be detected on prenatal ultrasonograms, during the investigation of a positive galactosemia screening test in neonates or of a complication, or be found fortuitously on an abdominal ultrasound. Small intrahepatic shunts may resolve spontaneously within one year of age, but other shunts such as extrahepatic, persistent ductus venosus or persisting intrahepatic shunts, must be closed in one or two steps, by interventional radiology techniques or surgically. The plasticity of the intrahepatic portal system allows revascularization of the liver after shunt closure, even when no intrahepatic portal structures can be detected on imaging studies. This leaves little or no place for liver transplantation in the management of these children.

Update on ultrasound imaging of liver fibrosis

Update on ultrasound imaging of liver fibrosis

Successful transjugular intrahepatic portal-systemic shunt in an ineligible liver transplant patient with primary biliary cirrhosis with refractory ascites and aplastic anemia

A transjugular intrahepatic portal-systemic shunt (TIPS) is a standard way to decompress the portal system in cirrhotic patients as a bridge to orthotopic liver transplantation (OLT). Traditionally, TIPS has been indicated for certain portal hypertensive sequelae such as refractory ascites, varices treatment and even hepato-hydrothorax. Herein is a case report on the efficacy of TIPS in an OLT ineligible patient with primary biliary cirrhosis and aplastic anemia who had developed refractive ascites requiring serial paracentesis and esophageal varices. He survived 2.5 years post-TIPS placement and died from complications related to severe leucopenia and the development of sepsis.

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

To Drain or Not Drain: PleurX Indwelling Drainage Catheters for Non-Malignant Refractory Ascites or Hepatic Hydrothorax

Purpose: The insertion of chronic indwelling drainage catheters for treatment of refractory ascites or hepatic hydrothorax in cirrhotic patients is controversial due to concerns related to infectious complications. PleurX drainage catheters are reported to have low infection rates due to their design and polyester cuff that promotes tissue ingrowth. The aim of the current study was to determine the 30 and 90-day mortality in cirrhotic patients with refractory non-malignant ascites or hepatic hydrothorax after placement of PleurX catheter. Methods: An interventional radiology database was queried for patients who underwent a PleurX catheter for indication of non-malignant refractory ascites or hepatic hydrothorax between the dates of January 1st, 2005 to January 1st, 2011 at a single transplant center. Medical records including the transplant database were reviewed by two of the study investigators. Demographics, laboratories, catheter related infectious morbidity, and mortality were collected. Results: A total of 26 patients with non-malignant ascites (n=16) and hepatic hydrothorax (n=10) underwent PleurX catheter insertion during the study period. The mean age was 57.1 ± 7.5 years and 61.5% were male. The mean MELD score was 21.7 ± 7.7 with majority of patients belonging to Child's - Pugh class C (69%). Follow up data was available for all the patients. The 30 and 90-day mortality in the study cohort was 30.8% and 61.5%, respectively. Two catheter related infections (8%) occurred within 30 days of insertion (Candida glabrata peritonitis and E. Coli sepsis). 69% (n=18) of the patients in the study cohort were transplant eligible and were being followed in the pretransplant clinic. The 30 and 90-day mortality in the study cohort was 27.8% and 66.7%, respectively. 11.1% of the patients were transplanted after insertion with an time to transplant of 107 ± 79.2 days. The two above-mentioned infections were within this group. In the remaining transplant non-eligible patients (n=8), PleurX was performed for palliation. The 30 and 90-day mortality was 37.5% and 50.0%, respectively. No catheter related infections were found in this subgroup. 4 patients survived to the end of the study period with average length of follow-up of 607 ± 128 days. Conclusion: Patients undergoing PleurX catheter drainage placement for refractory ascites and hepatic hydrothorax have high 30 day and 90-day mortality presumably from underlying liver disease. Due to low incidence of infectious complications, PleurX catheter could be an option when serial large volume paracentesis or transjugular intrahepatic shunts are not an option.

A Case Report of Congenital Multiple Intrahepatic Arterio-Portal Shunts with Esophago-Gastric Varices

A Case Report of Congenital Multiple Intrahepatic Arterio-Portal Shunts with Esophago-Gastric Varices

Letter: non‐cirrhotic portal hypertension and end‐stage liver disease

SIRS, The report by Schouten et al. on mortality in idiopathic non-cirrhotic portal hypertension (INCPH) was interesting. It adds to the growing evidence that INCPH can lead to features of end-stage liver disease in the absence of cirrhosis. Indeed some of the patients in this report had also suffered hepatic encephalopathy after transjugular intrahepatic port-systemic shunt (TIPSS) which is unusual given the fact that hepatic synthetic dysfunction is believed to be unusual. All these features indicate that INCPH indeed results in end-stage liver dysfunction in a subset of patients. It would be interesting if the authors could clarify the exact histologic findings in these patients who did need transplantation and also those who developed hepatic encephalopathy after TIPSS. Others have previously suggested that nodular transformation may represent a more severe end of the disease spectrum. Also it would be of interest to know any reasons which resulted in encephalopathy in TIPSS group but not in the surgical group.

Iron Deficiency Anaemia over the Last Decades - Osler's Disease Diagnosed by Doppler and Contrast-Enhanced Ultrasound

A72-year-old woman with a 30-year history of iron deficiency anaemia was admitted for fatigue and increasing weakness. On physical examination, the patient appeared dyspneic and pallor. The tongue showed angiodysplasias. Laboratory analysis showed a microcytary anaemia with an iron deficiency. Firstly a routine ultrasound examination (iU22, Philips Medical Systems) was performed. A left accessory artery, a dilated common hepatic artery and ectatic tortuous intrahepatic liver arteries were found. A contrast-enhanced ultrasound (CEUS) detected two intrahepatic arteriosystemic shunts in the left liver lobe. Endoscopy revealed multiple angiodysplasias of the stomach and the duodenum, 4 isolated angiodysplasias in the colon and telangiectasias in the oropharyngeal region. The angiodysplasias were treated with argon plasma coagulation. Osler's disease was diagnosed based on the Curacao criteria. Transabdominal B-mode sonography in combination with colour Doppler, pulsed wave Doppler and contrast-enhanced ultrasound is a very important tool to detect hepatic vascular malformations. It is an excellent procedure for the screening of patients with an iron deficiency anaemia. For the first time, we have demonstrated CEUS as an additional approach in the diagnosis of liver involvement in patients with Osler's disease.

Angiogenesis: a phenomenon which aggravates chronic liver disease progression.

It has become increasingly clear that angiogenesis occurring during chronic wound healing and fibrogenesis provides a key contribution to disease progression and complications. The association of fibrogenesis and angiogenesis should be regarded as crucial in the modern evaluation of liver disease progression and in the search for therapeutic targets. Physiological hepatic angiogenesis occurs during liver regeneration, contributing to the formation of new functional sinusoids. Pathological angiogenesis in liver is characterized by intrahepatic vascular remodeling with capillarization of the sinusoids and development of intrahepatic shunts, which lead to increased hepatic resistance and decreased effective hepatocyte perfusion. The problem of angiogenesis in chronic hepatitis C and nonalcoholic fatty liver disease has not been fully resolved. This manuscript briefly describes pathogenesis of new blood vessel formation in chronic hepatitis and potential role of angiogenesis in disease progression.

Perforation der A. carotis communis als Ursache eines Halsgefäßscheidenhämatoms

Perforation of the carotid artery is a rare, life-threatening emergency. This entity is usually caused by failed puncture of jugular veins, external trauma, or infection of the vascular wall. The existence of spontaneous rupture as a cause of vessel rupture is discussed in the literature. The case of a 57-year-old woman who suffered painful cervical swelling on the left side for 2 days is described. Six weeks prior to this, she had received transjugular intrahepatic shunt implantation (TIPS) via the jugular vein because of liver cirrhosis. Further signs were vocal cord dysfunction and Horners' syndrome on the left side. Computed tomography (CT) with contrast agent revealed a huge mass surrounding the common carotid artery. Differentiation between a solid tumor and carotid dissection was primarily not possible. Radiological considerations also comprised an abscess or even a paraganglioma. Only color duplex sonography revealed a pendular blood flow slightly caudal of the carotid bifurcation. In agreement with the CT findings, a calcified plaque appeared directly downstream of the presumed vessel injury. Operative revision was performed in collaboration with the vascular surgeon. Transluminal endarteriectomy and vessel reconstruction with patch plasty was performed. Cervical hematoma caused by carotid injury of unknown origin is a rare differential diagnosis of sudden cervical swelling. In this case, failed venous puncture in conjunction with pre-existing arterial plaque and therewith inflammation of the vessel wall could have caused the injury and delayed carotid rupture.

Ultrasound-guided transhepatic puncture of the hepatic veins for TIPS placement

This retrospective analysis was carried out to assess the feasibility and results of transjugular intrahepatic portal systemic shunt (TIPS) performed with ultrasound (US)-guided percutaneous puncture of the hepatic veins. Over a period of 3 years, 153 patients were treated with TIPS at our centre. In eight cases, a percutaneous puncture of the middle (n=7) or right (n=1) hepatic vein was required because the hepatic vein ostium was not accessible. Indications for TIPS were bleeding (n=1), Budd-Chiari syndrome (n=1), ascites (n=2), reduced portal flow (n=1) and incomplete portal thrombosis (n=3). A 0.018-in. guidewire was anterogradely introduced into the hepatic vein to the inferior vena cava (IVC) through a 21-gauge needle. In the meantime, a 25-mm snare-loop catheter was introduced through the jugular access to retrieve the guidewire, achieving through-andthrough access. Then, a Rosch-Uchida set was used to place the TIPS with the traditional technique. Technical success was achieved in all patients. There was one case of stent thrombosis. One patient died of pulmonary oedema. Three patients were eligible for liver transplantation, whereas the others were excluded due to shunt thrombosis (n=1) and previous nonhepatic neoplasms (n=3). The percutaneous approach to hepatic veins is rapid and safe and may be useful for avoiding traumatic liver injuries.

Focus

Focus

Large Spontaneous Intrahepatic Portal-Systemic Venous Shunt Treated With Coil and Amplatzer Vascular Plug Embolization

Spontaneous intrahepatic portal-systemic shunts are rare and can lead to significant encephalopathy. If intervention is recommended, transcatheter embolization is preferred. Various embolization techniques have been described using a wide range of embolization materials. In this report, the authors describe a useful embolization technique for managing a complex, large type 3 spontaneous intrahepatic portal-systemic shunt using Nester coils and Amplatzer vascular plugs through both percutaneous systemic and transhepatic approaches.

Portal Hypertension and Ascites in Extramedullary Hematopoiesis

Myeloproliferative diseases (MPD) are clonal stem cell disorders which mainly include polycythemia vera (PV), essential thrombocythemia (ET), and idiopathic myelofibrosis (IMF). They are characterized by leucocytosis, thrombocytosis, erythrocytosis, splenomegaly, and bone marrow hypercellularity. This might also result in extramedullary hematopoiesis. Abdominal manifestation has been recognized as a feature of these disorders. Splenomegaly and hepatomegaly are fairly common as opposed to ascites which is rare. The MPDs mainly affect the hepatic circulatory systems. The common hepatic manifestations are Budd-Chiari syndrome (BCS), portal vein thrombosis (PVT), and nodular regenerative hyperplasia. A few other features seen in MPDs are caused by extramedullary hematopoiesis, increased hepatic blood flow, and secondary hemosiderosis from multiple blood transfusions. Portal hypertension is found in up to 7% of patients. We report a case of portal hypertension with ascites in a patient with extramedullary hematopoiesis treated with transjugular intrahepatic portocaval shunt (TIPS).

Prosthetic H-Graft Portacaval Shunts vs Transjugular Intrahepatic Portasystemic Stent Shunts: 18-Year Follow-Up of a Randomized Trial

Widespread application of transjugular intrahepatic portasystemic shunt (TIPS) continues despite the lack of trials documenting efficacy superior to surgical shunting. Here we present an 18-year follow-up of a prospective randomized trial comparing TIPS with small-diameter prosthetic H-graft portacaval shunt (HGPCS) for portal decompression. Beginning in 1993, patients were prospectively randomized to undergo either TIPS or HGPCS as definitive therapy for portal hypertension due to cirrhosis. Complications of shunting and long-term outcomes were noted. Failure of shunting was prospectively defined as the inability to place shunt, irreversible shunt occlusion, major variceal rehemorrhage, unanticipated liver transplantation, or death. Survival and shunt failure were compared using Kaplan-Meier curve analysis. Median data are reported. Patient presentation, circumstances of shunting, causes of cirrhosis, severity of hepatic dysfunction (eg, Child's class, Model for End-Stage Liver Disease score), and predicted survival after shunting did not differ between patients undergoing TIPS (n = 66) or HGPCS (n = 66). Survival was significantly longer after HGPCS for patients of Child's class A (91 vs 19 months; p = 0.009) or class B (63 vs 21 months; p = 0.02). Shunt failure occurred later after HGPCS than TIPS (45 vs 22 months; p = 0.04). Compared with TIPS, survival after HGPCS was superior for patients with better liver function (eg, Child's class A or B). Shunt failure after HGPCS occurred later than after TIPS. Rather than TIPS, application of HGPCS is preferred for patients with complicated cirrhosis and better hepatic function.

Prospective evaluation of anticoagulation and transjugular intrahepatic portosistemic shunt for the management of portal vein thrombosis in cirrhosis

There is no established management algorithm for portal vein thrombosis (PVT) in cirrhotic patients. The aim of our study was to prospectively evaluate anticoagulation and transjugular intrahepatic portosystemic shunt (TIPS) to treat PVT. Cirrhotics with non-malignant PVT were included. Low weight molecular heparin anticoagulation was considered in all; TIPS was indicated if thrombosis progressed or anticoagulation was contraindicated. Patients who were not anticoagulated nor received TIPS served as controls. Fifty-six patients (of whom 21 controls) were included. PVT was occlusive in 11/35, with extension to the superior mesenteric or splenic vein in 13/35. In the study group 33 patients were anticoagulated, with a recanalization rate of 36% (12/33) compared with 1/21 among controls. A time interval between appearance of thrombosis and anticoagulation < 6 months predicted chance of repermeation. Thrombus progression occurred in 15/21 non anticoagulated patients and in 5/33 anticoagulated patients (P < 0.001). TIPS was placed in six patients. There were five variceal bleedings and two intestinal venous ischaemia episodes in the control group, compared with one variceal bleeding episode in the study group. In cirrhotics with PVT, a treatment algorithm using anticoagulation and TIPS achieves a good chance of complete repermeation, reduces portal hypertensive complications, and decreases the rate of thrombosis progression.