门静脉高压症门静脉高压症Portal HypertensionQuestionsWhere is the portal vein?What is portal hypertension?How do we handle with the patient of portal hypertension?Recently a talent artist died of the severe complication of liver cirrhosis combined with portal hypertension. 50 year old male, with history of hepatitis and liver cirrhosis upper digestive tract bleeding recently presenting with massive ascitesA typical caseHistory of portal hypertensionIn 1882 an Italian pathologist Banti first described this disease with unknown etiology named Banti Syndrome. In 1902 Gilbert first named this disease as portal hypertension. DefinitionPortal hypertension is defined as a portal vein pressure above the normal range, with clinical syndrome.Normal pressure of PV is 13-24cmH2O, and the average is about 18cmH2OuThe anatomy is relatively constant. uBy the confluence of the superior mesenteric and splenic veins behind the neck of the pancreas. uThe inferior mesenteric vein most often joins the splenic vein before PV is formed, but 1/3 of the inferior mesenteric vein joins the superior mesenteric vein. Anatomy a dual supply from both the HA and the PV. PV system is entirely devoid of valves. PV normally carries 75 per cent of the blood supply of the liver, with an average flow of 1200 ml/min.AnatomyAnatomyArteriesPortal VeinsHepatic VeinsPotential venous collaterals that develop with portal hypertension AnatomyThe colleteral network through the coronary and short gastric veins to the azygos vein is the most important one clinically because it results in formation of esophagogastric varices. Other sites include a recanalized umbilical vein from the left portal vein to the epigastric venous system (caput medusae), retroperitoneal colleteral vessels, and the hemorrhoidal venous plexus. Etiology1. Intrahepatic occlusionuPresinusoidal: SchistosomiasisuSinusoidal, post-sinusoidal: liver cirrhosis (alcoholic hepatitis, viral hepatitis, Wilson disease)2. Extraheptic diseasesuPV occlusion: stenosis, thrombosis, extrinsic compression, trauma,inflammationuHV occlusion: Budd-Chiari SyndromeuIncreased volume of PV: HA-PV、SA-SV ateriovenous fistulaBackward theory vs. Forward theoryHigh resistance Ohm Law P = QR High volume It is currently believed that the principle and initial abnormality is increased vascular resistance to portal flow and that portal hypertension is then maintained by increased blood flow into the portal circulation. Hyperdynamic circulation of portal hypertension PATHOLOGIC CHANGESCirrhosis was first described in a fourth century B.C., Hippocratic aphorism: “In cases of jaundice it is a bad sign when liver becomes hard.”Lannec introduced the term cirrhosis, which was derived from the Greek word kirrhos, meaning “orange-yellow”.Liver cirrhosisAlthough the mechanisms causing liver cirrhosis are diverse, the pathologic response is uniform: hepatocellular necrosis followed by fibrosis and nodular regeneration. Each of these three elements may exist alone, but all three are required for the development of cirrhosis. Cirrhosis causes two major phenomena:- hepatocellular failure - portal hypertensionThe spleen is enlarged from the normal 300 grams or less to between 500 and 1000 gm. Sensitivity: PLCWBC RBC (Hypersplenism)Splenomegaly&HypersplenismDilation of collateralEsophagus varicesRectal varicesGastric varicesThe increased pressure is transmitted to collateral venous channels. Sometimes these venous collaterals are dilated. Seen here is caput medusae which consists of dilated veins seen on the abdomen of a patient with cirrhosis of the liver.caput medusaeAscites 1. FPP （1）elevated filtrating pressure of capillary bed （2）disable of lymph refluence 2. deteriorated liver function （1）decreasing albumin production （2）inactivation of aldosterone and vasopressin Portal hypertensive gastropathy Hepatic encephalopathy asterixisCLINIC MANESTATION &DIAGONOSISDiagnosisMedical history: Hepatits, SchistosomiasisSplenomegaly, HypersplenismBleedingAscitesLab TestingBlood testingLiver functionAbdomen US, Duplex USX- ray, CTangiographyChild Classification MELD score as an allocation system in United States since Feb 28, 2002. MELD score = 10 ( 0.957 ln creatinine, mg/dL +0.378 ln bilirubin, mg/dL +1.12 ln INR +0.643)Where laboratory values less than 1.0 are set to 1.0 for purpose of the MELD score calculation and the maximum serum creatinine is 4.0mg/dL. Euro-transplant plans to switch form MUC to MELD within 2 years. MELD (Model for end-stage liver disease)MELD score: 29分；分；3月死亡率为月死亡率为58Child-Pugh: 10分；分；Child C3D CT ScanEyes-icterusSkin-spiderA major challenge to the physician or surgeon managing patients with cirrhosis is to determine when definite treatment (transplantation) rather than palliative treatment (e.g., intervention to prevent recurrent variceal hemorrhage) should be applied. TreatmentTreatment Conventional surgical treatment is aimed at the complication of portal hypertension No prophylactic operation Various interventions depending