Transcript Slide 1
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 Villanueva Department 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 Intrahepatic Prehepatic Portal vein thrombosis Splenic vein thombosis Splanchnic arteriovenous fistula Splenomegaly (lymphoma, Gaucher's disease) Posthepatic IVC obstruction Cardiac disease (constrictive pericarditis, restrictive cardiomyopathy) 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 (Venoocclusive disease) Budd-Chiari syndrome Intrahepatic Prehepatic Portal vein thrombosis Splenic vein thombosis Splanchnic arteriovenous fistula Splenomegaly (lymphoma, Gaucher's disease) Posthepatic IVC obstruction Cardiac disease (constrictive pericarditis, restrictive cardiomyopathy) 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 (Venoocclusive 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 Frequency, percent Histological feature* 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 Nodular hyperplasia of parenchyma 47 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 Raised in Endemic Area Unknown Mechanisms Idiopathic Portal Fibrosis Alcohol Abuse Steatosis and Mild pericentral vein fibrosis Asian Descent Medication non-adherence Mild Elevation in Alk Phos and ALT Portal Hypertension Gastric/Esophageal Varices complicated by recurrent UGIB Ascites Splenomegal y Multifactorial Anemia Orthostasis Dr. Martin Blaser Dr. Anthony Grieco Dr. Emma Robinson Dr. Gerald Villanueva Dr. Cristina Hajdu Dr. Chirayu Gor Dr. Christina Yoon