Hepatic Circulatory Disturbances
Hepatic Circulatory Disturbances
Overview
Central veins = hepatic vein tributaries. Normal liver: NO hepatic-portal vein anastomoses (cirrhotic liver develops them). Normal free hepatic vein pressure ~6mmHg. 3 categories: hepatic venous, portal venous, hepatic arterial obstruction.
I. Hepatic venous obstruction (3 diseases)
Budd-Chiari syndrome (hepatic vein thrombosis) — pure form = slow hepatic vein + adjacent IVC thrombosis (some include veno-occlusive disease in this).
Etiology: ~1/3 idiopathic. Rest: ↑thrombotic tendency — polycythaemia vera, PNH, OCPs, pregnancy/postpartum, intra-abdominal cancer (esp. HCC), chemo/radiation, myeloproliferative disease, suprahepatic IVC membranous webs.
Morphology: Gross=enlarged, swollen, red-purple, tense capsule. Micro: sudden=centrilobular congestion/necrosis/sinusoidal rupture into space of Disse. Slow=chronic, centrilobular fibrosis→CARDIAC CIRRHOSIS.
Clinical: Acute=pain, vomiting, hepatomegaly, ascites, mild icterus→acute hepatic failure/death. Chronic(more usual)=tender hepatomegaly, ascites, portal HTN features, survives months-years.
Hepatic veno-occlusive disease — intimal thickening/stenosis/obliteration of terminal central+medium hepatic veins. Similar to Budd-Chiari BUT KEY DIFFERENTIATOR = NO major hepatic vein thrombosis.
Etiology: “bush tea” hepatotoxic alkaloids (Africa/India/tropics); high-dose chemo pre-BMT; rare hereditary form w/ immunodeficiency (gene mutation).
Peliosis hepatis + bacillary angiomatosis — peliosis = primary sinusoidal dilatation → outflow blockage, ±massive intraperitoneal haemorrhage. Both linked to HIV + CD4 <100/μl + Bartonella henselae opportunistic infection (poor hygiene) → blood-filled hepatic cysts, endothelial-lined, fibromyxoid inflammatory background. Also linked: anabolic steroids, OCPs — self-limiting on withdrawal.
II. Portal venous obstruction
Intrahepatic: CIRRHOSIS (commonest/most important) > tumour invasion > congenital hepatic fibrosis > schistosomiasis.
Extrahepatic: intra-abdominal cancer, intra-abdominal sepsis, direct tumour invasion, myeloproliferative disorders, post-upper-abdominal-surgery thrombosis.
Effects: regardless of site/cause, MOST IMPORTANT = PORTAL HYPERTENSION. Extrahepatic+splenic vein extension→bowel venous infarction. Pylephlebitis→multiple pyaemic liver abscesses.
III. Hepatic arterial obstruction
Uncommon. Main hepatic artery/right-lobe branch ligation→rare fatal infarction. Small intrahepatic branch obstruction→usually NO effect (good collaterals).
Clinical relevance
Budd-Chiari vs veno-occlusive disease = classic pair, identical downstream pathology, differentiated by ONE fact: major hepatic vein thrombosis present (Budd-Chiari) vs absent (veno-occlusive). Peliosis/bacillary angiomatosis HIV+CD4<100+Bartonella henselae = testable opportunistic-infection threshold. 3 obstruction levels differ sharply in severity: arterial=usually silent (collaterals protect), venous(either level)=converges on portal HTN/hepatic failure — “veins matter more than artery” for this organ.
Hepatic Circulatory Disturbances
Overview
Central veins = hepatic vein tributaries. Normal liver: NO hepatic-portal vein anastomoses (cirrhotic liver develops them). Normal free hepatic vein pressure ~6mmHg. 3 categories: hepatic venous, portal venous, hepatic arterial obstruction.
I. Hepatic venous obstruction (3 diseases)
Budd-Chiari syndrome (hepatic vein thrombosis) — pure form = slow hepatic vein + adjacent IVC thrombosis (some include veno-occlusive disease in this).
Etiology: ~1/3 idiopathic. Rest: ↑thrombotic tendency — polycythaemia vera, PNH, OCPs, pregnancy/postpartum, intra-abdominal cancer (esp. HCC), chemo/radiation, myeloproliferative disease, suprahepatic IVC membranous webs.
Morphology: Gross=enlarged, swollen, red-purple, tense capsule. Micro: sudden=centrilobular congestion/necrosis/sinusoidal rupture into space of Disse. Slow=chronic, centrilobular fibrosis→CARDIAC CIRRHOSIS.
Clinical: Acute=pain, vomiting, hepatomegaly, ascites, mild icterus→acute hepatic failure/death. Chronic(more usual)=tender hepatomegaly, ascites, portal HTN features, survives months-years.
Hepatic veno-occlusive disease — intimal thickening/stenosis/obliteration of terminal central+medium hepatic veins. Similar to Budd-Chiari BUT KEY DIFFERENTIATOR = NO major hepatic vein thrombosis.
Etiology: “bush tea” hepatotoxic alkaloids (Africa/India/tropics); high-dose chemo pre-BMT; rare hereditary form w/ immunodeficiency (gene mutation).
Peliosis hepatis + bacillary angiomatosis — peliosis = primary sinusoidal dilatation → outflow blockage, ±massive intraperitoneal haemorrhage. Both linked to HIV + CD4 <100/μl + Bartonella henselae opportunistic infection (poor hygiene) → blood-filled hepatic cysts, endothelial-lined, fibromyxoid inflammatory background. Also linked: anabolic steroids, OCPs — self-limiting on withdrawal.
II. Portal venous obstruction
Intrahepatic: CIRRHOSIS (commonest/most important) > tumour invasion > congenital hepatic fibrosis > schistosomiasis.
Extrahepatic: intra-abdominal cancer, intra-abdominal sepsis, direct tumour invasion, myeloproliferative disorders, post-upper-abdominal-surgery thrombosis.
Effects: regardless of site/cause, MOST IMPORTANT = PORTAL HYPERTENSION. Extrahepatic+splenic vein extension→bowel venous infarction. Pylephlebitis→multiple pyaemic liver abscesses.
III. Hepatic arterial obstruction
Uncommon. Main hepatic artery/right-lobe branch ligation→rare fatal infarction. Small intrahepatic branch obstruction→usually NO effect (good collaterals).
Clinical relevance
Budd-Chiari vs veno-occlusive disease = classic pair, identical downstream pathology, differentiated by ONE fact: major hepatic vein thrombosis present (Budd-Chiari) vs absent (veno-occlusive). Peliosis/bacillary angiomatosis HIV+CD4<100+Bartonella henselae = testable opportunistic-infection threshold. 3 obstruction levels differ sharply in severity: arterial=usually silent (collaterals protect), venous(either level)=converges on portal HTN/hepatic failure — “veins matter more than artery” for this organ.
Hepatic Circulatory Disturbances
Overview
The liver’s central veins are hepatic vein tributaries. Normal liver has no hepatic-vein-to-portal-vein anastomoses (cirrhotic liver develops such anastomoses). Normal free hepatic vein pressure ~6 mmHg. Circulatory disturbances of the liver are grouped as hepatic venous obstruction, portal venous obstruction, and hepatic arterial obstruction.
I. Hepatic Venous Obstruction
Three uncommon diseases:
Budd-Chiari Syndrome (Hepatic Vein Thrombosis)
In its pure form, slowly developing thrombosis of the hepatic veins and adjacent inferior vena cava (some workers also include hepatic veno-occlusive disease within this syndrome).
Etiology: ~1/3 idiopathic. Remainder linked to increased thrombotic tendency: polycythaemia vera, paroxysmal nocturnal haemoglobinuria, oral contraceptives, pregnancy/postpartum state, intra-abdominal cancers (e.g. hepatocellular carcinoma), chemotherapy/radiation, myeloproliferative diseases, membranous webs of the suprahepatic IVC (congenital or from organised thrombosis).
Morphology: Gross — enlarged, swollen, red-purple liver with a tense capsule. Micro — sudden occlusion: centrilobular congestion, necrosis, sinusoidal rupture into the space of Disse. Slowly developing thrombosis: more chronic changes, centrilobular fibrosing reaction that may progress to cardiac cirrhosis.
Clinical features: acute form — abdominal pain, vomiting, enlarged liver, ascites, mild icterus; leads to acute hepatic failure and death. Chronic form (more usual) — pain over an enlarged tender liver, ascites, other portal hypertension features; patient may survive months to a few years.
Hepatic Veno-occlusive Disease
Intimal thickening, stenosis, and obliteration of terminal central veins and medium-sized hepatic veins. Produces pathologic changes similar to Budd-Chiari syndrome, but is distinguished by the absence of thrombosis in the major hepatic veins.
Etiology:
- More widespread in Africa, India, and other tropical countries where hepatotoxic-alkaloid-containing “bush tea” is consumed — implicated.
- Occurs after high-dose chemotherapy before bone marrow transplantation.
- Also seen as part of a rare hereditary veno-occlusive disease with immunodeficiency (gene mutation).
Bacillary Angiomatosis and Peliosis Hepatis
Peliosis hepatis: uncommon primary sinusoidal dilatation causing blood-outflow blockage, sometimes leading to massive intraperitoneal haemorrhage (sinusoidal dilatation itself can occur secondary to many other liver diseases too). Etiology unclear, but peliosis hepatis and the related bacillary angiomatosis occur in HIV-infected patients with CD4+ counts <100/μl — opportunistic Bartonella henselae infection (in poor hygiene settings) produces blood-filled cysts in the liver, partly lined by endothelial cells, with mixed inflammatory cells in a fibromyxoid background. An etiologic association with anabolic steroid and oral contraceptive use has also been suggested; self-limiting upon withdrawing the offending agent.
II. Portal Venous Obstruction
Occurs at intrahepatic or extrahepatic sites.
Intrahepatic causes: cirrhosis (commonest and most important), then in decreasing frequency tumour invasion, congenital hepatic fibrosis, schistosomiasis.
Extrahepatic causes: intra-abdominal cancers, intra-abdominal sepsis, direct tumour invasion, myeloproliferative disorders, upper abdominal surgery followed by thrombosis.
Effects — depend on the obstruction site, but irrespective of site or cause, the most important effect is portal hypertension and its manifestations. If obstruction is extrahepatic and extends into the splenic vein, venous infarction of the bowel may result. Pylephlebitis may be followed by multiple pyaemic liver abscesses.
III. Hepatic Arterial Obstruction
Uncommon. Accidental ligation of the main hepatic artery or its branch to the right lobe may rarely be followed by fatal infarction. Obstruction of small intrahepatic arterial branches usually produces no effect, thanks to good collateral circulation.
Clinical Relevance
- Budd-Chiari syndrome versus hepatic veno-occlusive disease is a classic paired distinction: identical downstream pathology, but Budd-Chiari has demonstrable major hepatic vein thrombosis while veno-occlusive disease specifically lacks it — this single histologic/radiologic fact is the differentiator.
- Peliosis hepatis and bacillary angiomatosis sharing an HIV/CD4<100 + Bartonella henselae link is worth anchoring alongside other opportunistic-infection thresholds in immunocompromised patients, since the CD4 cutoff itself is a testable number.
- The three obstruction levels (hepatic venous, portal venous, hepatic arterial) differ sharply in clinical consequence severity — arterial obstruction is usually silent (collateral-protected), while venous obstruction at either level converges on the same endpoint of portal hypertension or hepatic failure, worth remembering as “veins matter more than the artery” for this organ specifically.
Hepatic Circulatory Disturbances
Overview
The liver’s central veins are hepatic vein tributaries. Normal liver has no hepatic-vein-to-portal-vein anastomoses (cirrhotic liver develops such anastomoses). Normal free hepatic vein pressure ~6 mmHg. Circulatory disturbances of the liver are grouped as hepatic venous obstruction, portal venous obstruction, and hepatic arterial obstruction.
I. Hepatic Venous Obstruction
Three uncommon diseases:
Budd-Chiari Syndrome (Hepatic Vein Thrombosis)
In its pure form, slowly developing thrombosis of the hepatic veins and adjacent inferior vena cava (some workers also include hepatic veno-occlusive disease within this syndrome).
Etiology: ~1/3 idiopathic. Remainder linked to increased thrombotic tendency: polycythaemia vera, paroxysmal nocturnal haemoglobinuria, oral contraceptives, pregnancy/postpartum state, intra-abdominal cancers (e.g. hepatocellular carcinoma), chemotherapy/radiation, myeloproliferative diseases, membranous webs of the suprahepatic IVC (congenital or from organised thrombosis).
Morphology: Gross — enlarged, swollen, red-purple liver with a tense capsule. Micro — sudden occlusion: centrilobular congestion, necrosis, sinusoidal rupture into the space of Disse. Slowly developing thrombosis: more chronic changes, centrilobular fibrosing reaction that may progress to cardiac cirrhosis.
Clinical features: acute form — abdominal pain, vomiting, enlarged liver, ascites, mild icterus; leads to acute hepatic failure and death. Chronic form (more usual) — pain over an enlarged tender liver, ascites, other portal hypertension features; patient may survive months to a few years.
Hepatic Veno-occlusive Disease
Intimal thickening, stenosis, and obliteration of terminal central veins and medium-sized hepatic veins. Produces pathologic changes similar to Budd-Chiari syndrome, but is distinguished by the absence of thrombosis in the major hepatic veins.
Etiology:
- More widespread in Africa, India, and other tropical countries where hepatotoxic-alkaloid-containing “bush tea” is consumed — implicated.
- Occurs after high-dose chemotherapy before bone marrow transplantation.
- Also seen as part of a rare hereditary veno-occlusive disease with immunodeficiency (gene mutation).
Bacillary Angiomatosis and Peliosis Hepatis
Peliosis hepatis: uncommon primary sinusoidal dilatation causing blood-outflow blockage, sometimes leading to massive intraperitoneal haemorrhage (sinusoidal dilatation itself can occur secondary to many other liver diseases too). Etiology unclear, but peliosis hepatis and the related bacillary angiomatosis occur in HIV-infected patients with CD4+ counts <100/μl — opportunistic Bartonella henselae infection (in poor hygiene settings) produces blood-filled cysts in the liver, partly lined by endothelial cells, with mixed inflammatory cells in a fibromyxoid background. An etiologic association with anabolic steroid and oral contraceptive use has also been suggested; self-limiting upon withdrawing the offending agent.
II. Portal Venous Obstruction
Occurs at intrahepatic or extrahepatic sites.
Intrahepatic causes: cirrhosis (commonest and most important), then in decreasing frequency tumour invasion, congenital hepatic fibrosis, schistosomiasis.
Extrahepatic causes: intra-abdominal cancers, intra-abdominal sepsis, direct tumour invasion, myeloproliferative disorders, upper abdominal surgery followed by thrombosis.
Effects — depend on the obstruction site, but irrespective of site or cause, the most important effect is portal hypertension and its manifestations. If obstruction is extrahepatic and extends into the splenic vein, venous infarction of the bowel may result. Pylephlebitis may be followed by multiple pyaemic liver abscesses.
III. Hepatic Arterial Obstruction
Uncommon. Accidental ligation of the main hepatic artery or its branch to the right lobe may rarely be followed by fatal infarction. Obstruction of small intrahepatic arterial branches usually produces no effect, thanks to good collateral circulation.
Clinical Relevance
- Budd-Chiari syndrome versus hepatic veno-occlusive disease is a classic paired distinction: identical downstream pathology, but Budd-Chiari has demonstrable major hepatic vein thrombosis while veno-occlusive disease specifically lacks it — this single histologic/radiologic fact is the differentiator.
- Peliosis hepatis and bacillary angiomatosis sharing an HIV/CD4<100 + Bartonella henselae link is worth anchoring alongside other opportunistic-infection thresholds in immunocompromised patients, since the CD4 cutoff itself is a testable number.
- The three obstruction levels (hepatic venous, portal venous, hepatic arterial) differ sharply in clinical consequence severity — arterial obstruction is usually silent (collateral-protected), while venous obstruction at either level converges on the same endpoint of portal hypertension or hepatic failure, worth remembering as “veins matter more than the artery” for this organ specifically.
Hepatic Circulatory Disturbances
- Three-level obstruction map of the hepatic circulation (arterial/portal venous/hepatic venous) with their differing severity of consequence
- Budd-Chiari syndrome vs hepatic veno-occlusive disease side by side, thrombosis-presence as the differentiating fact
Hepatic Circulatory Disturbances
- Three-level obstruction map of the hepatic circulation (arterial/portal venous/hepatic venous) with their differing severity of consequence
- Budd-Chiari syndrome vs hepatic veno-occlusive disease side by side, thrombosis-presence as the differentiating fact
My Sticky Notes
Personal revision notes, mnemonics and reminders.
