Vascular Disorders of the Liver : VALDIG's Guide to Management and Causes

This book provides a comprehensive account on individually rare, but collectively frequent diseases of the liver. In the first part, conditions such as hepatic vascular malformations, ischemic cholangiopathy, hepatic artery occlusion, sinusoidal conditions and Budd-Chiari syndrome are discussed among others. The second part examines the causes of vascular liver disease namely, coagulation disorders, neoplasm disorders, non-malignant blood disorders, systemic diseases and toxins, among others. The unique expertise of the authors, who are all members of the Vascular Liver Disease Disorders Group, an independent network of researchers with a common interest in Vascular Liver Diseases, are merged for an optimal pragmatic and individualized approach. This book is of interest to a broad range of experts, such as hepatologists, internists, radiologists and angiologists. Contents Part I: Vascular Disorders of the Liver and Their Management Chapter 1: Role of Liver Biopsy in the Study of Vascular Disorders of the Liver Introduction Pathological Analysis of Liver Biopsy Elementary Morphological Features of Vascular Liver Disorders Pathological Diagnosis of Vascular Liver Diseases Budd-Chiari Syndrome Sinusoidal Obstruction Syndrome Sinusoidal Dilatation and Peliosis Hepatitis Porto-sinusoidal Vascular Disease Hepatocellular Nodules in VLD Indications of Liver Biopsy in Vascular Liver Diseases References Chapter 2: Role of Imaging in the Study of Vascular Disorders of the Liver Introduction Anatomy and Microcirculation Imaging Modalities Focal Vascular Anomalies of the Liver Transient Hepatic Parenchymal Enhancement Hepatic Arterioportal Shunts Subsegmental, Segmental or Lobar Portal Venous Thrombosis Obstruction of the Superior Vena Cava Increased Hepatic Enhancement Around Liver Tumors Increased Hepatic Enhancement Related to Inflammation Other Transient Hepatic Parenchymal Enhancement Pseudolesions Pseudolesions Around the Falciform Ligament Pseudolesion Adjacent to the Hilum Pseudolesions Around the Gallbladder Diffuse Vascular Liver Diseases Extrahepatic Portal Vein Thrombosis Budd-Chiari Syndrome Other Causes of Sinusoidal Dilatation Sinusoidal Obstruction Syndrome Portosinusoidal Disease Congenital Portosystemic Shunts Hereditary Hemorrhagic Telangiectasia Liver Cirrhosis Thrombosis, Stenosis, Dissection of the Hepatic Artery Hepatic artery Aneurysm Portal Vein Aneurysm Nodules Associated with Liver Diseases References Chapter 3: Hepatic Artery Occlusion and Ischemic Cholangiopathy Pathophysiology Conditions Associated with Injury of the Arterial Blood Supply of the Bile Ducts Susceptible to Induce Ischemic Cholangiopathy Conditions Associated with Definite Ischemic Cholangiopathy Conditions Associated with Possible Ischemic Cholangiopathy Clinical, Laboratory, Radiological, and Histological Findings Associated with Ischemic Cholangiopathy Differential Diagnosis Prognosis and Available Therapeutic Options Conclusion References Chapter 4: Hepatic Vascular Malformations in Hereditary Hemorrhagic Telangiectasia Liver VMs in HHT Pathogenesis Clinical Manifestations Diagnosis of Liver VMs in HHT Doppler US Evaluation of Liver VMs in HHT Non invasive and Invasive Assessment of Cardiac Hemodynamics CT MR Celiac Angiography Endoscopy and Invasive Evaluation of Portal Hypertension Liver Biopsy Differential Diagnosis Treatment References Chapter 5: Congenital Extrahepatic Portosystemic Shunts: Abernethy Malformation Introduction Etiology Diagnosis Anatomical Classification Liver Pathology CEPS Complications Hepatic Encephalopathy Pulmonary Arterial Hypertension (PaHT) and Hepatopulmonary Syndrome (HPS) Liver Nodules Management and Treatment References Chapter 6: Budd-Chiari Syndrome: Hepatic Venous Outflow Tract Obstruction Introduction Etiology and Risk Factors Manifestations Diagnosis Treatment Pregnancy Prognosis References Chapter 7: Extrahepatic Portal Vein Obstruction: Recent Portal Vein Thrombosis and Portal Cavernoma in the Absence of Cirrhosis Definition and Epidemiology of Recent (or Acute) and Chronic Extrahepatic Portal Vein Obstruction Manifestations, Outcome and Complications Recent EHPVO Chronic EHPVO (Portal Cavernoma) Diagnosis of EHPVO Therapy Anticoagulation, Thrombolysis, Surgery: Efficacy and Complications Recent EHPVO Chronic EHPVO Conclusion References Chapter 8: Portal Vein Thrombosis in Patients with Cirrhosis Epidemiology, Diagnosis and Classification Risk Factors Natural History Treatment Conclusion References Chapter 9: Porto-Sinusoidal Vascular Disorder Introduction Definition: Past and Present Epidemiology Etiology: Associated Conditions Drug Exposure Immunological Disorders Coagulation Disorders Infections Congenital and Hereditary Disorders Clinical Presentation and Features PSVD with Portal Hypertension PSVD Without Portal Hypertension Histopathological Findings Auxiliary Diagnostic Methods Imaging Elastography Hepatic Venous Pressure Gradient Measurement and Hepatic Venography Special Considerations Focal Liver Lesions Pregnancy Non-hepatic Abdominal Surgery Management Medical Anticoagulation Treatment of Portal Hypertension Transjugular Intrahepatic Porto-Systemic Shunt Surgery Current and Future Perspectives in Translational and Clinical Research Translational Clinical Conclusions References Chapter 10: Sinusoidal Obstruction Syndrome/Hepatic Veno-Occlusive Disease Introduction Disease State Incidence Pathophysiology Risk Factors Diagnosis Early Diagnosis Early Markers Hemodynamic Study of the Liver Biopsy Baltimore and Seattle Criteria European Society for Blood and Marrow Transplantation (EBMT) Adults Children Assessing Severity Treatment Non-pharmacologic Prevention Supportive Care Pharmacotherapy Prevention Treatment General Defibrotide References Chapter 11: Sinusoidal Dilatation and Peliosis Hepatis Normal Hepatic Sinusoids Sinusoidal Pressure and Compression Classifications of Sinusoidal Distortion Sinusoidal Dilatation Features of Sinusoidal Dilatation Etiologies of Sinusoidal Dilatation Outflow Obstruction Cardiac Conditions Budd-Chiari syndrome Sinusoidal Obstruction Syndrome Sickle Cell Anemia Non-Obstructive Sinusoidal Dilatation Isolated (Benign) Sinusoidal Dilatation Sinusoidal Dilatation Associated with Idiopathic Non-Cirrhotic Portal Hypertension Peliosis Hepatis Etiologies of Peliosis Infection Malignancy Drug exposure Management of Peliosis Hepatis Conclusions References Chapter 12: Hypoxic Hepatitis Introduction Definition Epidemiology Pathophysiology Clinical Manifestations Diagnosis Management Prognosis Conclusion References Chapter 13: Congestive Cardiac Hepatopathy Introduction The Liver in Chronic Congestive Heart Failure Epidemiology Mechanism of Liver Injury Histological Changes Clinical Manifestations Laboratory Findings Prognosis and Clinical Progression Treatment The Liver in Congenital Cardiac Disease Fontan-Associated Liver Disease (FALD) Fontan Circulation Pathophysiology of FALD Natural History of FALD Serological Biomarkers of FALD Radiological Findings in FALD Liver Stiffness in FALD Hepatic Hemodynamics in FALD Liver Biopsy in Fontan Patients Hepatic Nodules in FALD Hepatocellular Carcinoma Portal Hypertension-Related Complications after Fontan Surgery Follow-up and Treatment of FALD Liver Damage in Other Congenital Heart Diseases References Chapter 14: Regenerative Nodules and Liver Tumors in Vascular Liver Diseases Introduction Hepatocellular Nodules: A Wide Spectrum of Lesions Large Regenerative Nodules (LRN) (Fig. 14.1) Pathology Imaging LRN in VLD Focal Nodular Hyperplasia (FNH) (Fig. 14.2) Pathology Imaging FNH in VLD Hepatocellular Adenoma (HCA) (Fig. 14.3) Pathology Hepatocyte Nuclear Factor 1A (HNF1A) Inactivated HCA (H-HCA) Inflammatory HCA (IHCA) b-Catenin Mutated HCA (b-HCA) and b-Catenin Mutated Inflammatory HCA (b-IHCA) Sonic Hedgehog HCA (shHCA) Unclassified HCA Imaging HCA in VLD Hepatocellular Carcinoma (HCC) (Fig. 14.4) Pathology Imaging HCC in VLD Hepatocellular Nodules in Vascular Liver Diseases Budd Chiari Syndrome (BCS) (Figs. 14.4 and 14.5) Congenital Porto-Systemic Shunts (CPSS) Porto-sinusoidal Vascular Disease (PSVD) (Fig. 14.6) Hereditary Hemorraghic Telangiectasia (HHT) (Fig. 14.7) Sinusoidal Obstruction Syndrome (SOS) Congenital Hepatic Fibrosis (CHF) References Chapter 15: Pregnancy in Vascular Liver Disease Pregnancy-Related Changes in Circulation and Coagulation Outcomes of Pregnancy in Women with Vascular Liver Disease Budd-Chiari Syndrome Pregnancy and Post-Partum, a Risk Factor for Budd-Chiari Syndrome Budd-Chiari Syndrome Presenting During Pregnancy or Post-Partum Pregnancy in a Patient with Previously Recognized Budd-Chiari Syndrome Portal Vein Thrombosis Portal Vein Thrombosis Presenting During Pregnancy or Post-Partum Pregnancy in Patients with Previously Recognized Portal Vein Thrombosis Porto-Sinusoidal Vascular Disease Other Vascular Disorders Management of Liver Disease and Underlying Prothrombotic Disorders During Pregnancy, Delivery and Puerperium in Women with Vascular Liver Disease Early Counseling Management During Pregnancy Management of Portal Hypertension Management of Antithrombotic Therapy During Pregnancy Management of Underlying Prothrombotic Disorders Planning of Labor Phase and Birth Puerperium Period References Chapter 16: Antithrombotic Therapy and Liver Disease Introduction Primary Vascular Liver Diseases Requiring Anticoagulant Treatment Budd-Chiari Syndrome/Hepatic Outflow Tract Obstruction (BCS) Extrahepatic Portal Vein Obstruction (EHPVO) Acute/Recent EHPVO Thrombolysis in Recent EHPVO Chronic Extrahepatic Portal Vein Obstruction (EHPVO) Non-Cirrhotic, Nonmalignant Idiopathic Noncirrhotic Portal Hypertension/Porto-Sinusoidal Vascular Disease Sinusoidal Obstruction Syndrome (SOS) Antithrombotic Therapy in Parenchymal Liver Disease with Secondary Vascular Involvement Portal Vein Thrombosis (PVT) in Patients with Cirrhosis Anticoagulant Drugs: Mechanism of Action and Management References Part II: Causes for Vascular Disorders of the Liver Chapter 17: Hemostatic Disorders and the Liver Liver Diseases and Hemostatic Disorders Hemostatic Changes in Liver Disease The Concept of Rebalanced Hemostasis Hypercoagulable Features Unbalanced Hemostasis: A Contributor to Vascular Liver Disorders? Intrahepatic Thrombosis Portal Vein Thrombosis and Budd Chiari Syndrome Hepatic Artery Thrombosis after Liver Transplantation Effects of Coagulation Disorders on the Liver Disseminated Intravascular Coagulation Hemostatic Activation in Pregnancy-Associated Liver Diseases Conclusion References Chapter 18: Primary Hepatic Vascular Neoplasms and Hematologic Neoplasms Affecting Liver Vessels Primary Hepatic Vascular Neoplasms Cavernous Haemangioma Pathology Manifestations and Course Imaging Appearance Typical Aspect Variants Epithelioid Hemangioendothelioma Pathology Manifestations and Course Imaging Appearance Angiosarcoma Pathology Etiology Manifestations and Course Imaging Appearance Hepatic Small Vessel Neoplasm Hematologic Neoplasms Affecting Liver Vessels Malignant Lymphoproliferative Disorders Clinical Presentations Imaging Appearance Histopathological Features Mastocytosis Myeloid Metaplasia Amyloidosis Conclusion References Chapter 19: Primary Immunodeficiencies and Non Malignant Hematologic Disease Associated with Disorders of Hepatic Vessels Primary Immunodeficiencies Associated with Disorders of Hepatic Vessels Predominantly Antibody Deficiencies Common Variable Immunodeficiency Disorders Pathology Evolution and Outcome Primary Predominantly Antibody Deficiencies (PAD) Due to Monogenic Defects Treatment and Liver Transplantation Conclusion Chronic Granulomatous Disease Combined Immune-Deficiencies with Associated or Syndromic Features Telomere Biology Disorders Hepatic Veno-Occlusive Disease with Immunodeficiency Syndrome (VODI) Non Malignant Hematologic Disorders Associated with Hepatic Vessel Disorders Paroxysmal Nocturnal Hemoglobinuria Sickle Cell Disease References Chapter 20: Systemic Diseases Affecting Liver Vessels Introduction Proposed General Pathophysiology Vasculitis Behçet’s Disease Periarteritis Nodosa Other Vasculitis Connective Tissue Disease Anti-Phospholipid Syndrome Systemic Lupus Systemic Sclerosis Rheumatoid Arthritis Sarcoidosis Drug Toxicity References Chapter 21: Drugs and Toxins Affecting Liver Vessels Introduction Epidemiology and Diagnosis of Drug-Induced Vascular Liver Diseases Hormones-Associated Vascular Liver Lesions Oral Contraceptive Agents Budd-Chiari Syndrome and Portal Vein Thrombosis Sinusoidal Dilatation Anabolic Steroids Thiopurines Azathioprine Thioguanine Oxaliplatin Didanosine Medicinal Plants and Toxins Pyrrolizidine Alcaloids Vitamin A Vinyl Chloride Monomer Toxicity Conclusion References Chapter 22: Liver Transplantation and Hepatic Vessels Introduction Standard Vascular Anastomoses during Liver Transplantation Types of Complications and Risk Factors Post-operative Imaging Post-transplantation Arterial Complications Hepatic Artery Thrombosis (HAT) Hepatic Artery Stenosis (HAS) Hepatic Artery Pseudoaneurysms (HAP) Post-transplantation Portal Complications Portal Vein Thrombosis (PVT) Portal Vein Stenosis (PVS) Post Transplantation Hepatic Venous Outflow Obstruction References