肝硬化和布加综合症的肝脏影像学表现

Liver Imaging: A Case of Liver Imaging: A Case of Cirrhosis and BuddCirrhosis and Budd--ChiariChiari Rachel Rachel WeinermanWeinerman HMS IVHMS IV BIDMC Radiology Core RotationBIDMC Radiology Core Rotation September 17, 2007September 17, 2007 AgendaAgenda  Patient presentationPatient presentation  Menu of testsMenu of tests  Interpretation of studies, part IInterpretation of studies, part I  Differential DiagnosisDifferential Diagnosis  AnatomyAnatomy  Interpretation of studies, part IIInterpretation of studies, part II  Diagnosis and outcomeDiagnosis and outcome Our Patient: Clinical PresentationOur Patient: Clinical Presentation  Mr. P is a previously healthy 30Mr. P is a previously healthy 30--year year old man who presents to the EDold man who presents to the ED  WeekWeek--long history of:long history of: •• Back painBack pain •• Abdominal distensionAbdominal distension •• 1515--lb weight gainlb weight gain  No significant PMHNo significant PMH  ROS significant for insomniaROS significant for insomnia Menu of TestsMenu of Tests Imaging options to evaluate back pain and Imaging options to evaluate back pain and abdominal distension:abdominal distension: •• CTCT  Test of choiceTest of choice •• MRIMRI  If CT equivocalIf CT equivocal •• UltrasoundUltrasound  If RUQ If RUQ biliarybiliary etiology suggested etiology suggested •• Plain filmsPlain films  Seldom usefulSeldom useful •• AngiographyAngiography  No longer used for diagnosis; used for therapeutic No longer used for diagnosis; used for therapeutic interventionsinterventions BWH Centricity Arrow: Recanalized umbilical vein Our Patient: Cirrhosis on Axial Abdominal CT Star: Ascites Other features of cirrhosis: Enlarged spleen, Enlarged liver caudate and left lobes, atrophied right lobe, heterogeneous parenchyma Radiographic Appearance of Radiographic Appearance of Cirrhosis on CTCirrhosis on CT  Change in liver sizeChange in liver size •• Atrophy of right lobeAtrophy of right lobe •• Enlargement of caudate and left lobeEnlargement of caudate and left lobe  Caudate:rightCaudate:right lobe > 0.65 is 90% specific for lobe > 0.65 is 90% specific for cirrhosiscirrhosis  Change in liver contourChange in liver contour •• Hobnail appearanceHobnail appearance  Associated changesAssociated changes •• AscitesAscites •• SplenomegalySplenomegaly •• Collateral vesselsCollateral vessels Differential DiagnosisDifferential Diagnosis  Alcohol abuseAlcohol abuse  Viral hepatitisViral hepatitis  Primary Primary biliarybiliary cirrhosiscirrhosis  HemachromatosisHemachromatosis  VenoVeno--occlusive diseaseocclusive disease •• BuddBudd--ChiariChiari •• CHFCHF  Drug toxicityDrug toxicity  Hereditary:Hereditary: •• WilsonWilson’’s, alphas, alpha--1 anti1 anti--trypsintrypsin, metabolic , metabolic disordersdisorders •Our patient had no history of alcohol abuse, no risk factors for hepatitis, and did not have any history of drug intake. Our Patient: Axial CT Scan BWH Centricity Arrow: Inferior vena cava The hepatic veins should be seen at this level and are not seen. Companion Patient #1: Normal Axial Abdominal CT http://radiographics.rsnajnls.org/cgi/content/full/21/suppl_1/S133/F17A Yellow arrow: Right hepatic vein Hepatic veins are seen at the same level Differential DiagnosisDifferential Diagnosis  Alcohol abuseAlcohol abuse  Viral hepatitisViral hepatitis  Primary Primary biliarybiliary cirrhosiscirrhosis  HemachromatosisHemachromatosis  VenoVeno--occlusive diseaseocclusive disease •• BuddBudd--ChiariChiari •• CHFCHF  Drug toxicityDrug toxicity  Hereditary:Hereditary: •• WilsonWilson’’s, alphas, alpha--1 anti1 anti--trypsintrypsin, metabolic , metabolic disordersdisorders Liver Vascular AnatomyLiver Vascular Anatomy  Portal veinPortal vein •• Right and left branchesRight and left branches  Hepatic veinsHepatic veins •• Right, left, andRight, left, and middlemiddle •• Combine toCombine to form the IVCform the IVC http://www.moondragon.org/images2/hepaticanatomy.jpg Our Patient: Coronal CT Scan BWH Centricity Arrow: Main Portal Vein The portal vein appears patent on this coronal CT Menu of Tests, Part IIMenu of Tests, Part II  Concern is for occlusion of the Concern is for occlusion of the hepatic veins.hepatic veins.  Imaging options for evaluating Imaging options for evaluating hepatic vasculature:hepatic vasculature: •• Ultrasound with DopplerUltrasound with Doppler •• MR angiogram/MR angiogram/venogramvenogram •• Conventional angiogram/Conventional angiogram/venogramvenogram Our Patient: Doppler US BWH CentricityFlow is seen only in the right hepatic vein; the left and middle hepatic veins are not visualized. Clinical DiagnosisClinical Diagnosis  Imaging up to this point suggested Imaging up to this point suggested venoveno--occlusive diseaseocclusive disease •• BuddBudd--ChiariChiari syndrome most likelysyndrome most likely  Occlusion of hepatic veinsOcclusion of hepatic veins  Laboratory studies showed a CBC as Laboratory studies showed a CBC as follows: follows: HctHct 62% 62% PltPlt 876876 •• Suggested Suggested PolycythemiaPolycythemia VeraVera  LFTLFT’’ss were elevated and risingwere elevated and rising  Ammonia level was 88Ammonia level was 88 BuddBudd--ChiariChiari SyndromeSyndrome  ThombosisThombosis of the hepatic veins and/or of the hepatic veins and/or intrahepaticintrahepatic or or suprahepaticsuprahepatic IVCIVC  EtiologyEtiology •• MyeloproliferativeMyeloproliferative disordersdisorders •• MalignancyMalignancy •• Infection/liver lesionsInfection/liver lesions •• OCP/pregnancyOCP/pregnancy •• HypercoagulableHypercoagulable statesstates Factor V Leiden, Factor V Leiden, ProthrombinProthrombin gene mutation, APLA, gene mutation, APLA, Protein C/S deficiency, ATIII deficiency, PNH, Protein C/S deficiency, ATIII deficiency, PNH, nephroticnephrotic syndrome)syndrome) •• BehcetBehcet’’ss syndrome, other autoimmune syndrome, other autoimmune disordersdisorders •• IdiopathicIdiopathic PolycythemiaPolycythemia VeraVera  MyelioproliferativeMyelioproliferative disorderdisorder •• Increased Increased hematocrithematocrit, white blood cell , white blood cell count, and plateletscount, and platelets  MechanismMechanism •• Red blood cell production in the absence Red blood cell production in the absence of erythropoietin stimulationof erythropoietin stimulation •• JAK2 mutation usually responsibleJAK2 mutation usually responsible  Can cause clotting and thrombosis Can cause clotting and thrombosis due to increased viscosity of blooddue to increased viscosity of blood BuddBudd--ChiariChiari: Radiologic : Radiologic AppearanceAppearance  Doppler USDoppler US •• Lack of flow in hepatic veinsLack of flow in hepatic veins  CT with IV contrastCT with IV contrast •• Thrombus may be visible within the hepatic Thrombus may be visible within the hepatic venous systemvenous system  MRVMRV •• Reduced caliber or absence of hepatic veinsReduced caliber or absence of hepatic veins  VenographyVenography •• ““Spider webSpider web”” appearance of the hepatic appearance of the hepatic vasculaturevasculature •• Thrombus or occlusion of IVC or hepatic veinsThrombus or occlusion of IVC or hepatic veins Our Patient: Venogram Performed during transjugular biopsy of the liver BWH Centricity Our Patient: Digital Subtraction Venogram BWH Centricity Arrow: “Spider-web” hepatic vasculature Absence of normal hepatic veins with spider web vasculature, characteristic of Budd-Chiari BuddBudd--ChiariChiari: Treatment: Treatment  ThrombolysisThrombolysis •• Interventional radiologyInterventional radiology •• Only if acute thrombus is presentOnly if acute thrombus is present  Angioplasty/Angioplasty/StentingStenting  ShuntShunt •• To relieve hepatic venous congestionTo relieve hepatic venous congestion  Medical therapyMedical therapy •• Treat underlying causeTreat underlying cause •• LactuloseLactulose, beta, beta--blocker, diureticblocker, diuretic  Liver transplantLiver transplant Our Patient: DiagnosisOur Patient: Diagnosis  Diagnosis:Diagnosis: •• FulminantFulminant hepatic failure, acute on chronichepatic failure, acute on chronic •• BuddBudd--ChiariChiari syndromesyndrome •• PolycythemiaPolycythemia veravera  Tissue diagnosis:Tissue diagnosis: •• Liver biopsyLiver biopsy  Compatible with cirrhosis and BuddCompatible with cirrhosis and Budd--ChiariChiari •• Bone marrow biopsyBone marrow biopsy  MyeloproliferationMyeloproliferation consistent with consistent with polycythemiapolycythemia veravera  JAK2 mutation presentJAK2 mutation present  PMH revisited:PMH revisited: •• prior records indicated elevated prior records indicated elevated hematocrithematocrit as early as as early as 20042004 •• Mother had essential Mother had essential thrombocytosisthrombocytosis (elevated platelets)(elevated platelets) Our Patient: TreatmentOur Patient: Treatment  Treatment for BuddTreatment for Budd--ChiariChiari:: •• LactuloseLactulose, , NadololNadolol, , LasixLasix •• Therapeutic Therapeutic paracentesisparacentesis  Treatment for Treatment for polycythemiapolycythemia veravera •• Therapeutic phlebotomyTherapeutic phlebotomy •• HydroxyureaHydroxyurea •• Heparin for antiHeparin for anti--coagulationcoagulation  Placed on liver transplant listPlaced on liver transplant list Thank you!Thank you! Dr. Gillian LiebermanDr. Gillian Lieberman Dr. Jacques Dr. Jacques ThamTham NycaNyca BowenBowen The nurses, residents, fellows,The nurses, residents, fellows, and attendings who helped me careand attendings who helped me care for Mr. P during my subfor Mr. P during my sub--internshipinternship . Liver Imaging: A Case of Cirrhosis and Budd-Chiari Agenda Our Patient: Clinical Presentation Menu of Tests Slide Number 5 Radiographic Appearance of Cirrhosis on CT Differential Diagnosis Our Patient: Axial CT Scan Companion Patient #1:�Normal Axial Abdominal CT Differential Diagnosis Liver Vascular Anatomy Our Patient: Coronal CT Scan Menu of Tests, Part II Our Patient: Doppler US Clinical Diagnosis Budd-Chiari Syndrome Polycythemia Vera Budd-Chiari: Radiologic Appearance Our Patient: Venogram�Performed during transjugular biopsy of the liver Our Patient: Digital Subtraction Venogram� Budd-Chiari: Treatment Our Patient: Diagnosis Our Patient: Treatment Thank you!