1、门静脉高压症门静脉高压症Portal HypertensionQuestionsQuestions Where 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 hist
2、ory of hepatitis and liver cirrhosis upper digestive tract bleeding recently presenting with massive ascitesA typical caseA typical caseHistory of portal hypertension In 1882 an Italian pathologist Banti first described this disease with unknown etiology named Banti Syndrome. In 1902 Gilbert first n
3、amed this disease as portal hypertension. Definition Portal 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 18cmH2Ou The anatomy is relatively constant. u By the confluence of the superior
4、 mesenteric and splenic veins behind the neck of the pancreas. u The 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 de
5、void 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 Anatomy The colleteral network through the coronary and short gastri
6、c 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 hemorrho
7、idal 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:
8、 Budd-Chiari SyndromeuIncreased volume of PV: HA-PV、SA-SV ateriovenous fistulaBackward theory vs. Forward theory High 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
9、 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 t
10、he 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 element
11、s 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&
12、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 cirrh
13、osis 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 asterixis
14、CLINIC MANESTATION &DIAGONOSISDiagnosis Medical history: Hepatits, Schistosomiasis Splenomegaly, Hypersplenism Bleeding AscitesLab Testing Blood testing Liver function Abdomen US, Duplex US X- ray, CT angiographyChild Classification MELD score as an allocation system in United States since Feb 28, M
15、ELD score as an allocation system in United States since Feb 28, 2002. 2002. MELD score = 10 MELD score = 10 ( 0.957 ln creatinine, mg/dL +0.378 ln ( 0.957 ln creatinine, mg/dL +0.378 ln bilirubin, mg/dL +1.12 lnbilirubin, mg/dL +1.12 ln INR +0.643) INR +0.643)Where laboratory values less than 1.0 a
16、re set to 1.0 for purpose of Where laboratory values less than 1.0 are set to 1.0 for purpose of the MELD score calculation and the maximum serum creatininethe MELD score calculation and the maximum serum creatinine is is 4.0mg/dL. 4.0mg/dL. Euro-transplant plans to switch form MUC to MELD within 2
17、years. 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-spider A major challenge to the physician or surgeon managing patients with cirrhosis is to determine whe
18、n 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 interventi
19、ons depending on liver function New horizon is liver transplantationHistory of Treatment of Portal HypertensionHistory of Treatment of Portal HypertensionInvestigators, Year of PublicationInvestigators, Year of PublicationContributionContributionEck, 1877Eck, 1877PortacavalPortacaval shunt (dog) shu
20、nt (dog)Pavlov, 1893Pavlov, 1893Encephalopathy (dog)Encephalopathy (dog)Vidal, 1903Vidal, 1903Clinical portacavalClinical portacaval shunt (ascites shunt (ascites) )WestphalWestphal, 1930, 1930Balloon tamponadeBalloon tamponadeCrafoordCrafoord & Frenckner & Frenckner, 1939, 1939Endoscopic sclerother
21、apyEndoscopic sclerotherapyBlakemore et al, 1945Blakemore et al, 1945Clinical portacavalClinical portacaval and splenorenal and splenorenal shunts (bleeding) shunts (bleeding)SengstakenSengstaken & Blakemore, 1950 & Blakemore, 1950Balloon tamponadeBalloon tamponadeKehneKehne, 1956, 1956VasopressinVa
22、sopressinWarren et al, 1967Warren et al, 1967Distal splenorenalDistal splenorenal shunt shuntStarzlStarzl, 1967, 1967First successful liver transplantationFirst successful liver transplantationInokuchiInokuchi, 1968, 1968Left gastricvena cavalLeft gastricvena caval shunt shuntRoschRosch, 1969, 1969T
23、IPS in animalsTIPS in animalsJohnston & Rodgers, 1973Johnston & Rodgers, 1973Reintroduction of endoscopic sclerotherapyReintroduction of endoscopic sclerotherapySugiuraSugiura & Futagawa & Futagawa, 1973, 1973Extensive esophagogastricExtensive esophagogastric devascularization devascularizationCalne
24、Calne, 1980, 1980Cyclosporine for transplantationCyclosporine for transplantationLebrecLebrec, 1981, 1981PropranololPropranolol for bleeding for bleedingColapintoColapinto, 1983, 1983TIPS in humansTIPS in humansTIPS, transjugularTIPS, transjugular intrahepatic intrahepatic portosystemic portosystemi
25、c shunt. shunt.from Chen TS, Chen PS: Understanding the Liver. Westport, CT, Greenwood Press, 1984, pp 154155.from Chen TS, Chen PS: Understanding the Liver. Westport, CT, Greenwood Press, 1984, pp 154155.Acute Upper GI HemorrhageEndoscopyBleeding esophageal varicesBleeding gastric varices or portal
26、 gastropathyEmergency SclerotherapyBleeding stopsElective managementBleeding persist or recursRepeat SclerotherapyBleeding persistBalloon tamponade or phamacotherapyphamacotherapyBleeding stopsBleeding persistConsider early definite therapyGood risk patientshunt or devascularizationPoor risk patient
27、TIPSLiver TransplantationGood risk patientshunt or devascularizationRupture of Gastroesophagus varices Non-surgical treatment Transfusion and preventing shock Drug: vasopressin Endoscopy Sengstaken-Blakemore tube TIPS (transjugular intrahepatic portosystemic shunt) The highest priority in emergency
28、management is restoration of circulating blood volume, which should be accomplished before upper gastrointestinal endoscopy. Volume status is assessed by central venous pressure measurements, urinary output, and a Swan-Ganz pulmonary artery catheter if necessary. Vasopressin is usually administered
29、intravenously as a bolus dose of 20 units over 20 minutes and then as a continuous infusion of 0.2 to 0.4 unit/min. Because vasopressin also constricts systemic arterioles, it frequently causes hypertension, bradycardia, decreased cardiac output, and coronary vasoconstriction. Randomized trial have
30、shown that somatostatin and its longer-acting analogue octreotide are as efficacious as endoscopic treatment for control of acute variceal bleeding. Endoscopic Sclerotherapy & Variceal Ligation To tamponade acutely bleeding gastroesophageal varices. The tube has three luminaone to aspirate the stoma
31、ch, another to inflate the gastric balloon, and a third to inflate the esophageal balloon. Patients treated with balloon tamponade should be in an intensive care unit, and endotracheal tubes should be placed in almost all to prevent aspiration.Sengstaken-Blakemore tubeTIPS At the present time, TIPS
32、should not be recommended as initial therapy for acute variceal hemorrhage. Mortality is related to the status of hepatic function. One clear indication for TIPS is as a short-term bridge to liver transplantation for patients in whom endoscopic treatment has failed. TIPS A similar frequency of encep
33、halopathy after TIPS as has been previously reported after nonselective shunts. Shunt stenosis or occlusion develops in as many as half of patients within 1 year of TIPS insertion. Absolute contradications to TIPS include right-sided heart failure and polycystic liver disease. Relative contradicatio
34、ns are portal vein thrombosis, hyper-vascular liver tumors, and encephalopathy, which can be worsened by diversion of portal flow. TIPS Surgical treatment Portosystemic shunts Devascularization SplenectomyThe portal vein is divided, the hepatic limb of the portal vein is ligated, and the splanchnic
35、end of the portal vein is anastomosed end-to-side to the vena cava. All portal blood is necessarily diverted into the vena cava.End-to-side portacaval shuntAn anastomosis is made between the side of the portal vein and the side of the inferior vena cava. With a shunt of standard diameter, almost all
36、 splanchnic blood is diverted around the liver into the low-pressure vena cava. Side-to-side portacaval shuntA plastic prosthesis or an autogenous internal jugular vein is used for the shunt. One end is anastomosed to the inferior vena cava, and the other end is anastomosed to the trunk of the super
37、ior mesenteric vein. The shunt curves around the lower edge of the third portion of the duodenum and is sometimes called a C-shunt.Interposition mesocaval shuntA vascular prosthesis measuring 8 to 10 mm in diameter is interposed between the side of the vena cava and the side of the portal vein. Smal
38、l-diameter interposition portacaval shuntDistal splenorenal Warren shuntSplenorenal shuntTransection and reanastomosis of the distal esophagus with the stapling device to control variceal hemorrhage. (A) A stapling device is inserted through a small gastrotomy incision. (B) When the device is fired,
39、 the esophagus is simultaneously transected and reanastomosed with staples. (C) If the device fires correctly, a complete ring of esophageal tissue is excised.DevascularizationLigation of grastric varicesSugiura esophageal transection and devascularization operation TIPS LeVeen peritoneovenous shunt
40、 used for routing ascitic fluid into the systemic circulation. The shunt consists of fenestrated tubing for insertion into the peritoneal cavity, a one-way valve, and a length of venous tubing for insertion into the superior vena cava.Treatment of poorly controlled ascites女性，女性， 58岁，肝硬化数年，近三月来二次上消化道
41、出血。岁，肝硬化数年，近三月来二次上消化道出血。术前肝功能分级术前肝功能分级Child A级。级。术中发现胃底迂曲成团的曲张静术中发现胃底迂曲成团的曲张静脉丛，与术前脉丛，与术前CT所示一致。所示一致。 TIPS LeVeen peritoneovenous shunt used for routing ascitic fluid into the systemic circulation. The shunt consists of fenestrated tubing for insertion into the peritoneal cavity, a one-way valve, an
42、d a length of venous tubing for insertion into the superior vena cava.Treatment of poorly controlled ascites资料来源：北京、上海、长春、南京、武汉的资料来源：北京、上海、长春、南京、武汉的 八所医院，床位均在八所医院，床位均在1000张以上张以上我国门静脉高压症手术现状我国门静脉高压症手术现状Liver TransplantationYears after OLTLog Rank: p = 0.0001Survival rate after OLT Univ. of PittsburghClassic OLT谢谢 谢谢
