Hepatic Vascular Malformations Research Articles

Hepatic vascular malformation with capillary proliferation: a clinicopathological analysis of four cases

Hepatic vascular malformation with capillary proliferation: a clinicopathological analysis of four cases

Hepatic Vascular Variants in Hereditary Haemorrhagic Telangiectasia

Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterised by vascular dysplasia. Hepatic vascular m alformations (VMs) range from small telangiectases to significant vascular shunting. Here we report two cases of HHT. Case 1 had diffuse ectasia of the hepatic artery along its intrahepatic and extrahepatic course with a hepatic arterial aneurysm. Case 2 presented with ileal and hepatic telangiectases. Knowledge of these vascular variants is indispensable for clinicians and radiologists in aiding diagnosis and surgical and interventional management. Keywords: Vascular Malformations; Hereditary Haemorrhagic Telangiectasia; Arteriovenous Malformation; Osler Weber Rendu.

Current guidelines for diagnosis and management of hepatic involvement in hereditary hemorrhagic teleangiectasia.

Hereditary hemorrhagic teleangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is the most common cause of hepatic vascular malformations in adults. Different vascular shunts (arteriovenous, arterioportal or portovenous) lead to different clinical manifestations. Even though no hepatic-related symptoms are reported in the majority of cases, the severity of liver disease could lead to refractory medical conditions, in some cases requiring liver transplantation. The aim of this manuscript is to provide an updated overview of the current evidence regarding the diagnosis and treatment of HHT liver involvement and liver-related complications.

Insights into contrast-enhanced ultrasound features of non-tumorous hepatic vascular malformations.

Insights into contrast-enhanced ultrasound features of non-tumorous hepatic vascular malformations.

Endovascular management of a congenital hepatic arterioportal malformation in a low resource setting

BackgroundArterioportal malformations, a rare type of vascular malformation, have significant associated morbidity and mortality. Management requires a carefully thought out approach by a multidisciplinary team. Low resource settings have an added challenge of limited treatment options and consumables.Case presentationWe report a case of a 14-month-old male with failure to thrive due to a congenital hepatic arterioportal fistula. He was successfully treated via an endovascular approach with metallic coil embolization.ConclusionHepatoportal fistula, a rare hepatic vascular malformation, has limited treatment options which can further be restricted by overall patient wellness. Minimally invasive endovascular treatment options can offer a high rate of success and reverse the morbidity associated with the disease as was seen with our case.

ALK1 controls hepatic vessel formation, angiodiversity, and angiocrine functions in hereditary hemorrhagic telangiectasia of the liver.

In hereditary hemorrhagic telangiectasia (HHT), severe liver vascular malformations are associated with mutations in the Activin A Receptor-Like Type 1 ( ACVRL1 ) gene encoding ALK1, the receptor for bone morphogenetic protein (BMP) 9/BMP10, which regulates blood vessel development. Here, we established an HHT mouse model with exclusive liver involvement and adequate life expectancy to investigate ALK1 signaling in liver vessel formation and metabolic function. Liver sinusoidal endothelial cell (LSEC)-selective Cre deleter line, Stab2-iCreF3 , was crossed with Acvrl1 -floxed mice to generate LSEC-specific Acvrl1 -deficient mice ( Alk1HEC-KO ). Alk1HEC-KO mice revealed hepatic vascular malformations and increased posthepatic flow, causing right ventricular volume overload. Transcriptomic analyses demonstrated induction of proangiogenic/tip cell gene sets and arterialization of hepatic vessels at the expense of LSEC and central venous identities. Loss of LSEC angiokines Wnt2 , Wnt9b , and R-spondin-3 ( Rspo3 ) led to disruption of metabolic liver zonation in Alk1HEC-KO mice and in liver specimens of patients with HHT. Furthermore, prion-like protein doppel ( Prnd ) and placental growth factor ( Pgf ) were upregulated in Alk1HEC-KO hepatic endothelial cells, representing candidates driving the organ-specific pathogenesis of HHT. In LSEC in vitro , stimulation or inhibition of ALK1 signaling counter-regulated Inhibitors of DNA binding (ID)1-3, known Alk1 transcriptional targets. Stimulation of ALK1 signaling and inhibition of ID1-3 function confirmed regulation of Wnt2 and Rspo3 by the BMP9/ALK1/ID axis. Hepatic endothelial ALK1 signaling protects from development of vascular malformations preserving organ-specific endothelial differentiation and angiocrine signaling. The long-term surviving Alk1HEC-KO HHT model offers opportunities to develop targeted therapies for this severe disease.

Blue Rubber Bleb Nevus Syndrome: A Rare Case of Gastrointestinal Hemorrhage Necessitating Bowel Resection.

Blue Rubber Bleb Nevus Syndrome is a congenital rarity that manifests as vascular malformations throughout the body, including the gastrointestinal tract. With fewer than 300 cases reported, the etiology and clinical course is poorly understood; however, the literature suggests TEK mutations on chromosome 9 result in unregulated angiogenesis. We present the case of a young female treated for anemia of unknown etiology who presented in hemorrhagic shock due to gastrointestinal hemorrhage necessitating small bowel resection, with cutaneous, intestinal, hepatic, and lingual vascular malformations associated with a single somatic pathologic TEK mutation. Although uncommon, this case suggests that Blue Rubber Bleb Nevus Syndrome should be considered in the differential of a patient with persistent anemia and cutaneous lesions, carrying the potential for multiple gastrointestinal vascular malformations progressing to hemorrhage necessitating operative management. Additionally, a severe phenotype can occur without a double-hit TEK mutation.

High‐output cardiac failure in a dog secondary to hepatic vascular malformation

AbstractA 2‐month‐old, 8.5 kg (18.7 lb), entire, female Chesapeake Bay Retriever presented for increased respiratory effort. Physical examination revealed a V/VI left basilar systolic heart murmur, mild dyspnoea and hepatomegaly. An echocardiogram revealed left‐sided volume overload, evidence of moderate pulmonary hypertension, markedly distended hepatic veins and caudal vena cava, and increased aortic and pulmonary flow velocities in the absence of overt structural heart disease, which was suspicious for high‐output cardiac failure. Thoracic radiographs revealed pulmonary oedema. An abdominal ultrasound revealed a hepatic vascular malformation. Medical management with furosemide was elected. A computed tomography was not pursued. The dog has done well over 9‐month follow‐up, with only one escalation in dose. Treatment of high‐output cardiac failure in a dog secondary to a hepatic vascular malformation has not been previously reported. Intervention to attenuate flow through the hepatic vascular malformation or portocaval shunt could be considered; however, short‐term treatment goals are controlling congestion.

Recurrent Epistaxis and Diffuse Hepatic and Splenic Vascular Malformations

Question: A 48-year-old man was admitted to emergency department because of left upper abdominal pain for 3 weeks and yellow urine for 1 week. The patient was diagnosed with splenic hemangioma for 3 years and recurrent epistaxis for 5 years, without further treatment. Physical examination showed tenderness and rebound pain in the left upper abdomen, no muscle tension, with shifting dullness. Laboratory studies showed a neutrophil count of 8990/mm3 (reference range, 1900–8000/mm3), a hemoglobin level of 66 g/L (reference range, 131–172 g/L), a total bilirubin level of 57.23 μmol/L (reference range, 5.1–20 μmol/L), a serum alanine aminotransferase level of 302.9 U/L (reference range, 9–50 U/L), a serum aspartate aminotransferase level of 117.5 U/L (reference range, 15–40 U/L), an activated partial prothrombin time of 34.6 (reference range, 25–34), and a quantitative D-dimer level of 361.23 (reference range, 0–0.55). Emergency abdominal computed tomography scan showed diffuse hepatic and splenic vascular malformations (Figure A), and a ruptured splenic tumor (Figure B, arrow). It is worth noting that the patient's father had the same disease during his lifetime after asking about the family history. What is the most likely diagnosis? Look on page 808 for the answer and see the Gastroenterology website (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and images in GI. After consultation with the patient, he underwent emergency splenectomy and liver biopsy. Histopathology demonstrated the dilatation of hepatic capillaries, showing hemangioma-like changes (Figure C). The ruptured splenic tumor was confirmed pathologically as hemangioendothelioma. After a multidisciplinary consultation, the patient was diagnosed with hereditary hemorrhagic telangiectasia (HHT) according to the Curaçao criteria.1Faughnan M.E. Palda V.A. Garcia-Tsao G. et al.International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia.J Med Genet. 2011; 48: 73-87Crossref PubMed Scopus (745) Google Scholar HHT, also called Osler–Weber–Rendu disease, is an autosomal-dominant disease. Up to one-third of patients have hepatic involvement and manifest as vascular malformations,2Chung D.Y. Federle M.P. Barrett J.P. et al.Hepatic hereditary hemorrhagic telangiectasia.Clin Gastroenterol Hepatol. 2006; 4 (xx)Abstract Full Text Full Text PDF Scopus (1) Google Scholar such as hepatic arteriovenous fistula (Figure D, rectangle). Martini3Martini G.A. The liver in hereditary haemorrhagic teleangiectasia: an inborn error of vascular structure with multiple manifestations: a reappraisal.Gut. 1978; 19: 531-537Crossref PubMed Scopus (95) Google Scholar classified patients with hepatic disease due to HHT into 3 subgroups: patients who had telangiectases with fibrosis or cirrhosis (as in our case), those who had cirrhosis without telangiectases, and those who had telangiectases without fibrosis or cirrhosis. Treatment is mostly supportive, but severe patients may require hepatic artery embolization or liver transplantation.2Chung D.Y. Federle M.P. Barrett J.P. et al.Hepatic hereditary hemorrhagic telangiectasia.Clin Gastroenterol Hepatol. 2006; 4 (xx)Abstract Full Text Full Text PDF Scopus (1) Google Scholar During the period of waiting for liver transplantation, the patient developed hepatic and renal insufficiency and respiratory failure. On day 11 of hospitalization, the patient's family asked to be transferred back to the local hospital for treatment. The patient died of multiple organ failure on the third day after transfer.

High output cardiac failure in 3 patients with hereditary hemorrhagic telangiectasia and hepatic vascular malformations, evaluation of treatment

BackgroundThis report addresses how patients with hereditary hemorrhagic telangiectasia (HHT) and high output cardiac failure (HOCF) due to hepatic vascular malformations, should be evaluated and could be treated. HHT is a genetic disorder, leading to vascular abnormalities with potentially serious clinical implications. In the liver, arteriovenous malformations occur in more than 70% of patients, but only about 8% present clinical symptoms such as HOCF with pulmonary hypertension and less commonly portal hypertension, biliary ischemia and hepatic encephalopathy.ResultsThree female patients with HHT type 2 and HOCF caused by severe arteriovenous malformations in the liver are presented in this case series. The patients were seen at the HHT-Centre at Odense University Hospital. Treatment with either orthotopic liver transplantation (one patient) or bevacizumab (two patients) was initiated. All patients experienced marked symptom relief and objective improvement. New York Heart Association—class were improved, ascites, peripheral edema and hence diuretic treatment was markedly reduced or discontinued in all three patients. Bevacizumab also resulted in notable effects on epistaxis and anemia.ConclusionOur findings substantiate the importance of identification of symptomatic arteriovenous malformations in the liver in patients with HHT. Bevacizumab may possibly, as suggested in this case series and supported by previous case studies, postpone the time to orthotopic liver transplantation or even make it unnecessary. Bevacizumab represents a promising new treatment option, which should be investigated further in clinical trials.

The Role of Liver Imaging in Hereditary Hemorrhagic Telangiectasia.

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder characterized by spontaneous epistaxis, telangiectasia, and visceral vascular malformations. Hepatic vascular malformations are common, though a minority are symptomatic. Symptoms are dependent on the severity and exact type of shunting caused by the hepatic malformation: Arteriosystemic shunting leads to manifestations of high output cardiac failure, and arterioportal shunting leads to portal hypertension. Radiologic imaging, including ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI), is an important tool for assessing liver involvement. Doppler ultrasonography is the first-line screening modality for HHT-related liver disease, and it has a standardized scale. Imaging can determine whether shunting is principally to the hepatic vein or the portal vein, which can be a key determinant of patients’ symptoms. Liver-related complications can be detected, including manifestations of portal hypertension, focal liver masses as well as ischemic cholangiopathy. Ultrasound and MRI also have the ability to quantify blood flow through the liver, which in the future may be used to determine prognosis and direct antiangiogenic therapy.

Sequence variations of ACVRL1 play a critical role in hepatic vascular malformations in hereditary hemorrhagic telangiectasia

BackgroundHereditary Hemorrhagic Telangiectasia (HHT) is an autosomal dominant disorder characterized by multiple telangiectases and caused by germline disease-causing variants in the ENG (HHT1), ACVRL1 (HHT2) and, to a lesser extent MADH4 and GDF2, which encode proteins involved in the TGF-β/BMP9 signaling pathway. Common visceral complications of HHT are caused by pulmonary, cerebral, or hepatic arteriovenous malformations (HAVMs). There is large intrafamilial variability in the severity of visceral involvement, suggesting a role for modifier genes. The objective of the present study was to investigate the potential role of ENG, ACVRL1, and of other candidate genes belonging to the same biological pathway in the development of HAVMs.MethodsWe selected 354 patients from the French HHT patient database who had one disease causing variant in either ENG or ACVRL1 and who underwent hepatic exploration. We first compared the distribution of the different types of variants with the occurrence of HAVMs. Then, we genotyped 51 Tag-SNPs from the Hap Map database located in 8 genes that encode proteins belonging to the TGF-β/BMP9 pathway (ACVRL1, ENG, GDF2, MADH4, SMAD1, SMAD5, TGFB1, TGFBR1), as well as in two additional candidate genes (PTPN14 and ADAM17). We addressed the question of a possible genetic association with the occurrence of HAVMs.ResultsThe proportion of patients with germline ACVRL1 variants and the proportion of women were significantly higher in HHT patients with HAVMs. In the HHT2 group, HAVMs were more frequent in patients with truncating variants. Six SNPs (3 in ACVRL1, 1 in ENG, 1 in SMAD5, and 1 in ADAM17) were significantly associated with HAVMs. After correction for multiple testing, only one remained significantly associated (rs2277383).ConclusionsIn this large association study, we confirmed the strong relationship between ACVRL1 and the development of HAVMs. Common polymorphisms of ACVRL1 may also play a role in the development of HAVMs, as a modifying factor, independently of the disease-causing variants.

IMPROVEMENT IN PULMONARY HYPERTENSION SECONDARY TO HEREDITARY HEMORRHAGIC TELANGIECTASIA FOLLOWING BEVACIZUMAB THERAPY

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease characterized by multiple vascular malformations, including telangiectasias and arteriovenous malformations (AVM) in the brain, lung and liver. When hepatic AVMs occur, high output cardiac failure can result, ultimately leading to acute cor pulmonale. We discuss a patient case with high output cardiac failure, due to hepatic AVMs. CASE PRESENTATION: This is a 57 year old female with confirmed HHT (ACVRL1 gene mutation) presented to pulmonary clinic for dyspnea. She was recently hospitalized due to dyspnea, lower extremity edema and recurrent epistaxis. Chest radiography with enlarged cardiac silhouette with pulmonary vascular congestion, chest angiography and V/Q were negative for acute pulmonary embolism. Echocardiography showed ejection fraction of 60-65% with severe tricuspid regurgitation, and severe pulmonary arterial hypertension with estimated right ventricular systolic pressures of 82mmHg. Outpatient pulmonary function test with FEV1 of 1.83 which was 64% of predicted, with FEV1/FVC 74% of predicted along with reduced six minute walk test of 356 meters, with elevated right heart pressures (Table 1). She was started on intravenous bevacizumab every three months for maintenance therapy. Repeat right heart catheterization after 6 months with significant improvement (Table 2). Repeat six minute walk test was 494 meters. Upon follow up, she reported significant improvement in epistaxis, dyspnea and lower extremity swelling. DISCUSSION: Due to this patient’s extensive hepatic AVM, she developed high output cardiac failure and severe pulmonary hypertension. In patients with HHT, hepatic AVMs are considered the most prevalent visceral AVM, although more than 90% can be clinically silent. Patients with hepatic AVMs can develop pulmonary hypertension along with high output cardiac failure. Interestingly, these patients are usually carriers of the ACVRL mutations [1], such as in our patient. Given the rarity of this disease, only few studies suggest treatments for HHT, including VEGF inhibitor, bevacizumab. In 2009, a French open label phase 2 single arm trial was conducted in 25 patients with severe HHT with symptomatic liver involvement. A significant decrease of high cardiac index had been demonstrated in 17 of 24 patients. Initially, mean CI was 5.01 L/min/m2, with a significant decrease after three months of treatment, with a mean CI at 4.2 L/min/m2 [2]. Due to significant improvement in our patient's respiratory symptoms and pulmonary hypertension, as noted on repeat right heart catheterization, intravenous bevacizumab should be considered as treatment for patients with HHT. CONCLUSIONS: In patients with hereditary hemorrhagic telangiectasias, it is imperative to consider intravenous bevacizumab, especially in patients with pulmonary hypertension. Reference #1: Sopeña, B., Pérez-Rodríguez, M. T., Portela, D., Rivera, A., Freire, M., & Martínez-Vázquez, C. (2013). High prevalence of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia. European journal of internal medicine, 24(3), e30-e34. Reference #2: Dupuis-Girod S, Ginon I, Saurin J-C, Marion D, Guillot E, Decullier E, et al. Bevacizumab in patients with hereditary hemorrhagic telangiectasia and severe hepatic vascular malformations and high cardiac output. JAMA. 2012; 307: 948–955. https://doi.org/10.1001/jama.2012.250 PMID: 22396517 DISCLOSURES: No relevant relationships by Bindu Akkanti, source=Web Response No relevant relationships by NEAL GEORGE, source=Web Response no disclosure on file for Juneja Harinder; no disclosure on file for Victor Machicao; No relevant relationships by Jennifer Sunny, source=Web Response

Pediatric Hepatobiliary Interventions in the Setting of Intrahepatic Vascular Malformations, Portal Hypertension, and Liver Transplant

Within the broad spectrum of pediatric hepatobiliary disorders, hepatic vascular malformations, portal hypertension, and hepatic transplant interventions pose numerous challenges. The role of interventional radiology within each of these conditions is discussed herein, beginning with endovascular management of high flow hepatic vascular malformations. Next, while becoming less common in adult populations, surgical portoportal and portosystemic shunts remain prevalent in many pediatric centers. Shunt anatomy is reviewed along with endovascular management techniques for shunt dysfunction. Next, the growing experience with pediatric transjugular intrahepatic portosystemic shunt placement is reviewed along with tips for success in pediatric patients. Finally, pediatric hepatic transplant interventions are discussed with technical notes pertinent to split liver anatomy.

Identification of two distinct hereditary hemorrhagic telangiectasia patient subsets with different hepatic perfusion properties by combination of contrast-enhanced ultrasound (CEUS) with perfusion imaging quantification.

BackgroundHereditary hemorrhagic telangiectasia (HHT) is marked by arteriovenous fusion comprising hepatic vascular malformations (HVaMs) with the chance of bleeding.AimsWe investigated HVaMs in HHT patients by combination of contrast-enhanced ultrasound (CEUS) with perfusion imaging quantification to be able to sub-classify a high risk cohort of asymptomatic HHT patients.MethodsThe imaging characteristics on CEUS in 34 patients (aged 21–84 years; mean 58.9) with HHT were retrospectively evaluated. Real-time contrast harmonic imaging, sulfur hexafluoride-filled microbubbles and motion adjustment were utilized. Cine loops of the liver were digital stored, perfusion was quantified using a software reading DICOM data`s.ResultsHVaMs were diagnosed in 31 out of 34 patients. Significant uppermost peak enhancement (PE), wash-in area under the curve (WiAUC) and wash-in perfusion index (WiPI) were identified in the shunt region (100%), next in the hilar region (PE 32.6%; WiAUC 33.9%; WiPI 34.1%), and the lowest in the hepatic parenchyma (PE 10.2%; WiAUC 12.0%; WiPI 9.5%). The perfusion parameters in the shunt region compared to the other regions were significantly increased in one subgroup of patients. Consistent with this, the intrahepatic portal vein diameter and Buscarini grading was significantly higher, while portal vein peak velocity was significantly lower in this patient subset. By statistical analysis, we could correlate PE and WiPI to these clinical parameters, while WiAUC showed no clinical association.ConclusionsFor the first time we combined CEUS findings with motion adjustment software to quantitative determine perfusion parameters of a cohort of HHT patients. Hereby, we could identify a subset of HHT patients with two markedly increased parameter values in the shunt region compared to the hilus/hepatic parenchyma. This could contribute to sub-classify a high-risk group of HHT patients with therapeutic indication.

Computerized registry of patients with hemorrhagic hereditary telangiectasia (RiHHTa registry) in Spain: Objectives, methods, and preliminary results

IntroductionHereditary hemorrhagic telangiectasia (HHT) is a rare disease with autosomal dominant inheritance that causes systemic vascular affectation. Material and methodAfter development a multicentric Spanish national registry, called RiHHTa, main clinical manifestations and diagnostic procedures of the first patients introduced are described. Results141 patients were included, of which 91 (64.5%) were women. The mean age at diagnosis was 42 years. Mutations in the ACVRL1 gene predominated over the ENG gene. The initial symptom was recurrent epistaxis in 130 (92.2%) patients and in three (2.1%), brain abscess. Pulmonary arteriovenous (AV) fistula were detected in 36 (45%) of the 79 patients who underwent thoracic CT angiography. The contrast echocardiography detected very few bubbles (grade I) or none, in 36 (45%) of these 79 affected patients. In 43 (67.2%) of the 64 patients with an abdominal CT angiography, hepatic vascular malformations were detected, mostly telangiectasias, AV and arterio-portal fistula, and extrahepatic in 14 (10%) subjects. More than half of the patients were screened for the presence of brain arteriovenous malformations which was found in 3.9% of them. The upper part of the intestinal tube was the most (95%) affected region. ConclusionThe RiHHTa registry allows improving the management of patients with HHT. An inadequate use of thoracic CT angiography and the usefulness of abdominal CT angiography has been detected in order to define subtypes of hepatic vascular involvement and detect extrahepatic vascular involvement.

Registro informatizado de la telangiectasia hemorrágica hereditaria (Registro RiHHTa) en España: objetivos, métodos y resultados preliminares

IntroductionHereditary hemorrhagic telangiectasia (HHT) is a rare disease with autosomal dominant inheritance that causes systemic vascular affectation. Material and methodAfter development a multicentric Spanish national registry, called RiHHTa, main clinical manifestations and diagnostic procedures of the first patients introduced are described. Results141 patients were included, of which 91 (64.5%) were women. The mean age at diagnosis was 42 years. Mutations in the ACVRL1 gene predominated over the ENG gene. The initial symptom was recurrent epistaxis in 130 (92.2%) patients and in three (2.1%), brain abscess. Pulmonary arteriovenous (AV) fistula were detected in 36 (45%) of the 79 patients who underwent thoracic CT angiography. The contrast echocardiography detected very few bubbles (grade I) or none, in 36 (45%) of these 79 affected patients. In 43 (67.2%) of the 64 patients with an abdominal CT angiography, hepatic vascular malformations were detected, mostly telangiectasias, AV and arterio-portal fistula, and extrahepatic in 14 (10%) subjects. More than half of the patients were screened for the presence of brain arteriovenous malformations which was found in 3.9% of them. The upper part of the intestinal tube was the most (95%) affected region. ConclusionThe RiHHTa Registry allows improving the management of patients with HHT. An inadequate use of thoracic CT angiography and the usefulness of abdominal CT angiography has been detected in order to define subtypes of hepatic vascular involvement and detect extrahepatic vascular involvement.

Ultrasonography in Liver Vascular Disease.

Vascular liver diseases include a heterogeneous group of disorders affecting the micro- and the macro-circulation of the liver. Thrombosis and obstruction of the inflow (portal vein) and/or outflow venous system (Budd-Chiari syndrome), spontaneous porto-systemic shunts, diseases affecting the sinusoids, and hepatic vascular malformations are the most important vascular liver diseases. Thrombosis of the portal venous system and of the hepatic venous system occur most commonly and are potentially life-threatening conditions, while congenital and acquired pro-thrombotic diseases are major causal factors, together with local factors triggering thrombotic events. Despite their overall low prevalence, vascular liver diseases represent the second cause of portal hypertension in the Western world. Imaging techniques are of paramount importance in the diagnostic process, as well as in the follow-up of patients affected by these conditions. In this review, we focus on the role of ultrasonography in the management of vascular liver diseases by highlighting advantages and drawbacks of this imaging technique. In addition, we provide a state of the art presentation of the possibilities offered by ultrasound in the evaluation of vascular and parenchymal features in vascular liver diseases encompassing not only the use of grayscale imaging, but also the application of Doppler ultrasound, the measurement of hemodynamic parameters and the assessment of liver stiffness.

Liver Transplantation as Curative Treatment in Severe Pulmonary Hypertension due to Hepatic Vascular Malformations in Rendu-Osler-Weber Disease

Liver Transplantation as Curative Treatment in Severe Pulmonary Hypertension due to Hepatic Vascular Malformations in Rendu-Osler-Weber Disease

59-Year-Old Woman With Fatigue, Dyspnea, and Lower Extremity Edema

59-Year-Old Woman With Fatigue, Dyspnea, and Lower Extremity Edema