Clinical Pathological Conference

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Clinical Pathological Conference

• Friday, December 5th, 2008

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Hospital Course

• 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.

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Abdominal CT ScanArterial Phase
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Abdominal CT ScanPortal Venous Phase
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Radiology Discussant

• Dr. Emma Robinson

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Faculty Discussant

• Dr. Gerald Villanueva
• Department of Medicine
• Division of Gastroenterology

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Interval History

• Dr. Sameer Dhalla

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Interval History

• 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

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Pathology Discussant

• Dr. Cristina Hajdu

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• Findings
• MINIMAL PORTAL AND LOBULAR INFLAMMATION
• FOCAL PORTAL, PERIPORTAL AND PERICENTRAL VEIN
  FIBROSIS
• MINIMAL MACROVESICULAR STEATOSIS
• Final Diagnosis
• Idiopathic Portal Fibrosis

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Final Diagnosis

• Idiopathic Portal Fibrosis

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Key Clinical Elements of Case

• 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

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Intrahepatic

Presinusoidal
Schistosomiasis
Idiopathic portal hypertension/Noncirrhotic portal fibrosis/Hepatoportal sclerosis
Primary biliary cirrhosis
Sarcoidosis
Congenital hepatic fibrosis
Sclerosing cholangitis
Hepatic arteriopetal fistula

Sinusoidal
Arsenic poisoning
Vinyl chloride toxicity
Vitamin A toxicity
Nodular regenerative hyperplasia

Postsinusoidal
Sinusoidal obstruction syndrome (Veno-occlusive disease)
Budd-Chiari syndrome
Prehepatic
Portal vein thrombosis
Splenic vein thombosis
Splanchnic arteriovenous fistula
Splenomegaly (lymphoma, Gaucher's disease)
Posthepatic
IVC obstruction
Cardiac disease (constrictive pericarditis, restrictive cardiomyopathy)
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Intrahepatic

Presinusoidal
Schistosomiasis
Idiopathic portal hypertension/Noncirrhotic portal fibrosis/Hepatoportal sclerosis
Primary biliary cirrhosis
Sarcoidosis
Congenital hepatic fibrosis
Sclerosing cholangitis
Hepatic arteriopetal fistula

Sinusoidal
Arsenic poisoning
Vinyl chloride toxicity
Vitamin A toxicity
Nodular regenerative hyperplasia

Postsinusoidal
Sinusoidal obstruction syndrome (Veno-occlusive disease)
Budd-Chiari syndrome
Prehepatic
Portal vein thrombosis
Splenic vein thombosis
Splanchnic arteriovenous fistula
Splenomegaly (lymphoma, Gaucher's disease)
Posthepatic
IVC obstruction
Cardiac disease (constrictive pericarditis, restrictive cardiomyopathy)
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Idiopathic Portal Fibrosis

• Historical 19th century term was Bantis
  Syndrome Anemia, thrombocytopenia, splenomegaly
  without hematological cause
• Characterized simultaneuosly in the 1960s
• -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

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IPF Overview

• Presence of portal hypertension
• Absence of liver cirrhosis
• Histological features of dense portal fibrosis,
  marked phlebosclerosis, and dilated sinusoids.

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IPF Epidemiology

• 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

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IPF Etiology Unknown

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

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IPF Clinical Approach

• Variceal Bleed which is surprisingly well
  tolerated
• Other signs of portal hypertension
• Preserved Liver Function
• Characteristic Hemodynamics
• Characteristic Path Findings
• Diagnosis of exclusion

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IPH Histological Findings
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
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IPF Management

• Few studies of IPF management exist
• Acute and Prophylactic regimens for variceal
  bleed as with cirrhotics
• TIPS and surgical anastomosis is often well
  tolerated

IPF Prognosis

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

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Back to the Patient.

• 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

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Asian Descent
Raised in Endemic Area
Unknown Mechanisms
Idiopathic Portal Fibrosis
Alcohol Abuse
Medication non-adherence
Steatosis and Mild peri-central vein fibrosis
Portal Hypertension
Mild Elevation in Alk Phos and ALT
Gastric/Esophageal Varices complicated by
recurrent UGIB
Orthostasis
Multifactorial Anemia
Splenomegaly
Ascites
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Thank You

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