The Hepatic Vasculature: Missed Lesions, Missed Diagnoses James M. Crawford, M.D., Ph.D. Department of Pathology and Laboratory Medicine North Shore-Long Island Jewish Health System • • • • • • • • • • • • • • • ♦ Case presentation 73 yo female -h/o hypertension, GE reflux -Elective EGD for abd pain, nausea, distention, bloating, constipation x months -No abnormalities identified on esophagogastroduodenoscopy -Video capsule placed without complications -6h later: returned for download of video capsule -Complained of epigastric pain, chest and back. Admitted for further eval. -Cardiac enzymes negative -CT: multiple foci of sub-diaphragmatic free air lateral to esophagus -Presumed diagnosis of esophageal perforation -CT follow-up at 2 days: ‘perforation improving’ -5 th day of admission: syncopal episode, thought due to dehydration (was NPO) -6 th day of admission: Hct declined from 29.6 to 11.9; Hb from 11.1 to 4.2 g/dl -transferred to MICU, treated for dehydration and hypotension (no blood given) -cardiac arrest, unable to be resuscitated Case 3 Case 3
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The Hepatic Vasculature:Missed Lesions, Missed Diagnoses
James M. Crawford, M.D., Ph.D.Department of Pathology and Laboratory Medicine
North Shore-Long Island Jewish Health System
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Case presentation73 yo female
-h/o hypertension, GE reflux
-Elective EGD for abd pain, nausea, distention, bloating, constipation x months-No abnormalities identified on esophagogastroduodenoscopy-Video capsule placed without complications
-6h later: returned for download of video capsule-Complained of epigastric pain, chest and back. Admitted for further eval.-Cardiac enzymes negative-CT: multiple foci of sub-diaphragmatic free air lateral to esophagus
-Presumed diagnosis of esophageal perforation-CT follow-up at 2 days: ‘perforation improving’
-5th day of admission: syncopal episode, thought due to dehydration (was NPO)
-6th day of admission: Hct declined from 29.6 to 11.9; Hb from 11.1 to 4.2 g/dl-transferred to MICU, treated for dehydration and hypotension (no blood given)-cardiac arrest, unable to be resuscitated
Case 3 Case 3
Case 3
Case presentation (Cont’d)Autopsy findings:-2 liters free blood in abdomen-Subcapsular hepatic hematoma (10.0 x 8.0 cm), left lobe of liver
-ruptured capsule-Left hepatic artery dissection, communicating with subcapsular hematoma
-rupture of left hepatic artery, into dissection-Main hepatic artery, right hepatic artery: dissection, without rupture
Hepatic Vascular LesionsClinical History: often not suspected
Pathology Examination (gross and microscopic): - may be missed- if seen, may be misinterpreted- if correctly diagnosed, may be completely change course
of clinical management
Hepatic Vascular LesionsTwo angles on hepatic vascular disease:
- Histopathologic examination of percutaneous liver biopsies
- Examination of explanted liver
- Post-mortem examination
Generally not an issue in partial hepatectomy resections
NOTE: Transplant pathology and nodular lesions are not covered in this presentation
Vascular Pathology of the Liver:a conceptual framework
Inflow: Hepatic ArteryPortal VeinPresinusoidal
Sinusoidal: CirrhosisOther
Outflow: Hepatic VenousOutflow Obstruction
Ca: 1550
Liver Vasculature: Basic facts
Normal Liver: 1200-1400 g = 2.5% of body weight
Blood Flow: 25% of cardiac output2/3 via portal vein1/3 via hepatic artery
Portal vascular tree: 17 to 20 orders of dichotomous branchesPortal vein blood enters parenchyma directly via “septal venules”Hepatic arterial blood supplies: bile ducts, capsule, vasa vasorum
enters parenchyma as post-capillary blood in portal blooddirect arteriolar supply of parenchyma is negligible in humans
Hepatic venous tree: fewer ramifications than portal treedrains into: right, middle, and left hepatic veins
Liver Vasculature: “third inflow”- Cholecystic veins through the gallbladder bed*- Extrahepatic parabiliary venous system- Extragastric paraumbilical venous system through falciform ligament
Implications: - Apparent “pseudolesions” on CT perfusion studies,
which are, in fact, artefact of differential blood flow- May rarely create local metabolic changes, e.g. differential steatosis
Yoshimitsu et al, Radiographics 2001; 21: S81-S96
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PortalVein
Bile HepaticDuct Artery
The perfect Portal Tract
Approximately 11 portal tracts/biopsy (range: 3-23) = 6 per cm (14G)62% contain a portal vein, 91% a hepatic artery, and 93% a bile ductBUT: 2.3 bile ducts, 2.6 hepatic arteries, and 0.7 portal veins per PT
Intraabdominal inflammationulcerative colitis, peritonitisintra-arterial chemotherapyendoscopic injection of sclerosing agents
Hepatic Artery Thrombosis
Cholangitis Infarction
Hepatic artery occlusion in the liver transplant: infarction of the biliary tree
Parenchymalinfarction
Hilarinfarction
Hepatic Artery Thrombosis
Polyarteritis Nodosa
Systemic Vascular Disease:Polyarteritis nodosaRheumatologic disorders with vasculitisSystemic amyloidosisHyaline arteriosclerosis
Portal Vein Obstruction
Portal vein thrombosis:Surgical complications, traumaIntrabdominal sepsis, phlebitisHepatocellular carcinomaMalignancy in pancreas, hepatic hilumThrombogenic disorders, Pancreatitis(can occur in setting of cirrhosis)
acute thrombosis of portal vein
Case presentationHistory:21 yo female Norwegian MIT student, in good health.2w PTA developed “upper respiratory syndrome” with cough,
sore throat, myalgias. No fevers, chills, chest pain.Day of admission: fever, chills, severe occipital headache,
lower back pain, diffuse abdominal pain, nauseated
Physical Examination:Lethargic and disoriented, fever of 104ºF, tachycardic,
tachypneic, hypotensive, orthostatic: admit to MICULUQ scar, no history available
Case presentationHistory:21 yo female Norwegian MIT student, in good health.2w PTA developed “upper respiratory syndrome” with cough,
sore throat, myalgias. No fevers, chills, chest pain.Day of admission: fever, chills, severe occipital headache,
lower back pain, diffuse abdominal pain, nauseated
Physical Examination:Lethargic and disoriented, fever of 104ºF, tachycardic,
tachypneic, hypotensive, orthostatic: admit to MICULUQ scar, no history available
Hospital Course:Developed respiratory distress, acidosis, oliguria, hypotensionExpired 14h after admission, 24h after onset of acute symptoms
Cavernous transformation of the portal vein
umbilical vein catheterization 2d →portal vein thrombosis, splenectomy age 5, Age 18: pneumococcal sepsis → death
Hepatoportal Sclerosis:Dense sclerosis of portal tractsObliteration of portal vein radiclesNo fibrous septa or cirrhosisIdiopathic vs. smoldering inflammation
1985: phlebotomy x 28 years, age 65 Hepatocellular carcinoma in “reversed cirrhosis”Blumberg RS, Gastroenterology 1988; 95:1399-1402
Incomplete Septal Cirrhosis:No substantive portal tract fibrosisFibrous septa (complete bridging and incomplete): no cirrhosis
note: approximation of portal tracts and terminal hepatic veinsDe novo parenchymal fibrosing injury vs. partially resorbed cirrhosis
Hepatoportal Sclerosis:Dense portal tract fibrosis, obliteration of portal vein radiclesNo significant parenchymal fibrosisDe novo portal fibrosing injury vs. partially resorbed cirrhosis
Non-cirrhotic portal hypertensionde novo injury vs. reversal of cirrhosis
Wanless IR et al., Arch Pathol Lab Med 2000; 124: 1599-1607
Case 2
Incomplete Septal Cirrhosis ???
Presinusoidal Obstruction
Nodular Regenerative Hyperplasia:No fibrosis at allNodules throughout the liverSmoldering vascular injuryCompensatory hyperplasia
Rheumatologic conditions, BMTx, Solid organ TxDiagnosis very difficult on liver biopsy
(AASLD Post-graduate Course 1987)
(Non-cirrhotic portal hypertension)
Incomplete Septal Cirrhosis:No substantive portal tract fibrosisFibrous septa (complete bridging and incomplete): no cirrhosis
note: approximation of portal tracts and terminal hepatic veinsDe novo parenchymal fibrosing injury vs. partially resorbed cirrhosis
Hepatoportal Sclerosis:Dense portal tract fibrosis, obliteration of portal vein radiclesNo significant parenchymal fibrosisDe novo portal fibrosing injury vs. partially resorbed cirrhosis
Nodular Regenerative Hyperplasia:No fibrosis of portal tracts or parenchymaRegenerative nodules with intervening parenchymal atrophyDe novo smoldering vascular injury vs. partially resorbed cirrhosis
Non-cirrhotic portal hypertensionde novo injury vs. reversal of cirrhosis
Wanless IR et al., Arch Pathol Lab Med 2000; 124: 1599-1607
Consideration of vascular disease is critical in differential diagnosisof virtually every hepatic pathology
Hepatic artery compromise:non-transplant setting: usually a smoldering subclinical processtransplantation setting: severe, usually leads to loss of organ
Portal vein compromise:large vessel: dramatic, may be life-threateningsmall vessel: subclinical, may give rise to pre-sinusoidal hypertension
Sinusoidal obstruction:Cirrhosis: sinusoids, small portal veins, small hepatic veinsAssortment of infiltrative disorders