Friday, December 5 th, 2008. The patient was appropriately resuscitated with crystalloid fluid and blood products Emergent endoscopy showed large.

Friday, December 5th, 2008

The patient was appropriately resuscitated with crystalloid fluid and blood products

Emergent endoscopy showed large gastric and esophageal varices with stigmata of recent bleeding. No endoscopic therapies or biopsies were performed at the time.

Once stable, a three-phase abdominal CT with IV contrast was performed. A diagnostic test/procedure was then performed.

Dr. Emma Robinson

Dr. Gerald VillanuevaDepartment of Medicine

Division of Gastroenterology

Dr. Sameer Dhalla

Stool Culture: Negative Fecal Leukocytes: Negative Stool Ova and Parasites: Negative Hepatitis Serologies: Negative ANA, AMA: Negative Ceruloplasmin, anti-trypsin: WNL Tests for Thrombophilia: All Negative Anti-Schistosomal Antibodies: Negative

A diagnostic liver biopsy was performed

Dr. Cristina Hajdu

Findings MINIMAL PORTAL AND LOBULAR INFLAMMATION

FOCAL PORTAL, PERIPORTAL AND PERICENTRAL VEIN FIBROSIS

MINIMAL MACROVESICULAR STEATOSIS

Final Diagnosis

Idiopathic Portal Fibrosis

Idiopathic Portal Fibrosis

Young previously healthy man from Hong Kong with short history of heavy alcohol use presents with UGIB and hypovolemia

Anemia and Hypoalbuminemia Clinical and radiographic evidence of

portal hypertension: variceal bleed, ascites, Splenomegaly. All out of proportion to mild hepatocellular disease

No cirrhosis on CT. No venous thrombosis

PrehepaticPortal vein thrombosis

Splenic vein thombosis

Splanchnic arteriovenous fistula

Splenomegaly (lymphoma, Gaucher's disease)

PosthepaticIVC obstruction

Cardiac disease (constrictive pericarditis, restrictive cardiomyopathy)

Intrahepatic

PresinusoidalSchistosomiasisIdiopathic portal hypertension/Noncirrhotic portal fibrosis/Hepatoportal sclerosis

Primary biliary cirrhosisSarcoidosisCongenital hepatic fibrosisSclerosing cholangitisHepatic arteriopetal fistula

SinusoidalArsenic poisoningVinyl chloride toxicityVitamin A toxicityNodular regenerative hyperplasia

PostsinusoidalSinusoidal obstruction syndrome (Veno-occlusive disease)Budd-Chiari syndrome

PrehepaticPortal vein thrombosis

Splenic vein thombosis

Splanchnic arteriovenous fistula

Splenomegaly (lymphoma, Gaucher's disease)

PosthepaticIVC obstruction

Cardiac disease (constrictive pericarditis, restrictive cardiomyopathy)

Intrahepatic

PresinusoidalSchistosomiasisIdiopathic portal hypertension/Noncirrhotic portal fibrosis/Hepatoportal sclerosis

Primary biliary cirrhosisSarcoidosisCongenital hepatic fibrosisSclerosing cholangitisHepatic arteriopetal fistula

SinusoidalArsenic poisoningVinyl chloride toxicityVitamin A toxicityNodular regenerative hyperplasia

PostsinusoidalSinusoidal obstruction syndrome (Veno-occlusive disease)Budd-Chiari syndrome

Historical 19th century term was Banti’s Syndrome: Anemia, thrombocytopenia, splenomegaly without hematological cause

Characterized simultaneuosly in the 1960’s-India (1962): Non-Cirrhotic Portal Fibrosis-Japan (1962): Idiopathic Portal hypertension-US (1965): Hepatoportal Sclerosis

After 30 years of competing names for the same disease, the above term has been “generally” adopted

Presence of portal hypertension Absence of liver cirrhosis Histological features of dense portal

fibrosis, marked phlebosclerosis, and dilated sinusoids.

Present worldwide but most focused in South Asia and East Asia, particularly Japan

Prevalence: 25-30% of non-cirrhotic portal hypertension in Asia. Dramatic decline in a more recent Japanese population survey.

Disparate Male to Female Ratios

Recurrent Infection Autoimmunity Genetic: HLA-DR3 Hypercoagulability HAART Miscellaneous Toxins

Variceal Bleed which is surprisingly well tolerated

Other signs of portal hypertension Preserved Liver Function Characteristic Hemodynamics Characteristic Path Findings

Diagnosis of exclusion

Histological feature*Frequency,

percent

Irregular intimal thickening of portal veins 75-100

Organizing thrombus and/or recanalization of portal veins

20-100

Intralobular fibrous septa 95

Abnormal blood vessels in the lobules 75

Subcapsular atrophy 70

Dense portal fibrosis and portal venous obliteration 32-52

Periductal fibrosis of interlobular bile ducts 50

Portal inflammation 47

Nodular hyperplasia of parenchyma 25-40

Few studies of IPF management exist Acute and Prophylactic regimens for

variceal bleed as with cirrhotics TIPS and surgical anastomosis is

often well tolerated

Small subgroup progress to nodular transformation of the liver with extensive subhepatic and portal fibrosis

HCC?

The Patient is doing well on his previous regimen of nadolol and esomeprazole

Furosemide and Aldactone were added for ascites

He is following regularly with a gastroenterologist and has had no recurrent bleeding events since his discharge in October 2008

Idiopathic Portal Fibrosis

Portal Hypertension

Alcohol Abuse

Asian Descent

Raised in Endemic Area

UnknownMechanisms

Gastric/Esophageal Varices complicated by recurrent UGIB

Multifactorial Anemia

OrthostasisSplenomegaly

Mild Elevation in Alk Phos and ALT

Steatosis and Mild peri-central vein fibrosis

Medication non-adherence

Ascites

Dr. Martin Blaser Dr. Anthony Grieco Dr. Emma Robinson Dr. Gerald Villanueva Dr. Cristina Hajdu Dr. Chirayu Gor Dr. Christina Yoon