门静脉高压症

单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,门静脉高压症,Portal Hypertension,吴国洋,厦门大学附属中山医院,教学大纲,目 的：,1. 了解门静脉系统的解剖特点,2熟悉门静脉高压症的病因，病理和临床 表现,3掌握门静脉高压症的诊断和治疗原则,讲授内容：,门静脉的解剖概要，门静脉高压症的病因，病理、临床表现、诊断、鉴别诊断和治疗。,Anatomy,Liver Is a Unique Organ in That It Has a Dual Blood Supply,1500,ml/min (,25%,of the cardiac output),Portal vein contributes,2/3,of the total hepatic blood flow,Hepatic arterial perfusion accounts for over,50%,of the,livers oxygen supply,Portal Vein,肠系膜上静脉,肠系膜下静脉 门静脉 肝窦,脾静脉,下腔静脉 肝静脉 中央静脉,肝的显微解剖,门静脉在解剖上有三个特点：,门静脉无瓣膜，其压力通过,(A),流入的血量,和,(B),流出的阻力,形成并维持；,门静脉系位于两个毛细血管网之间，即一端是胃、肠、脾、胰的毛细血管网，另一端是肝小叶内的肝窦,(,毛细血管网,),；,门静脉系与腔静脉系之间存在有四个交通支，,即胃底、食管下段交通支,，,直肠下端、肛管交通支,，,前腹壁交通支,及,腹膜后交通支,。,正常门静脉压力1324,cmH,2,O，,平均18,cmH,2,O,Definition of Portal,Hypertention,是指由于各种原因致门静脉内压力升高而引起门静脉血流受阻、血液瘀滞时，临床上出现,脾肿大及脾功能亢进,食管胃底静脉曲张、呕血和黑便,腹水,等症状的一系列临床表现,Pathophysiology,Ohm law V = IR,( V - voltage, I - current, and R- resistance ),P = FR,P - the pressure gradient through the portal venous system,F -the volume of blood flowing through the system,R-the resistance to flow,1,门脉血流阻力增加：,即后向血流学说 ,(1)、肝细胞损害，纤维增生，门脉,血流阻力增加。,(2)、肝动脉门静脉交通支开放。,(3)、肠源性血管活性物质直接进入,体循环，致肝小静脉收缩。,2门脉血流量增加： 即前向血流学说 一些扩血管物质直接进入体 循环，导致内脏血流量增加，而 外周血管阻力降低，心输出量增 加，使全身和内脏处于高动力状 态。,门静脉高压症分型,肝前型,肝外门静脉血栓形成、先天性畸形和外在压迫,Splenic,Vein Thrombosis (Left-sided PH，,左侧门静脉高压症),Cause,:,Abdominal Trauma, Tumors, or,pancreatitis,.,Character,: Pressure Is Increased in Areas Drained by the,splenic,Vein, While Pressure in the Portal Vein Remains Normal.,Diagnosis,: Patients With Gastric And/or Esophageal,varices,and Normal Liver Biopsy Results.,Therapy,:,Splenectomy,Is Curative.,肝内型,肝炎后性肝硬化酒精性和胆汁性肝硬化（窦后及肝窦型）血吸虫性肝硬化（窦前型）,肝硬化患者的肝脏,Intrahepatic,predominantly,presinusoidal,Schistosomiasis,(early stage),Primary,biliary,cirrhosis (early stage),Idiopathic portal hypertension (early stage),Hepatic metastasis,Nodular regenerative hyperplasia,predominantly sinusoidal and/or,postsinusoidal,Acute , chronic and,fulminant,hepatitis,Acute alcoholic hepatitis,Primary,biliary,cirrhosis (advanced stage),Venoocclusive,disease,Idiopathic portal hypertension (advanced stage),Congenital hepatic fibrosis,Vitamin A toxicity -,noncirrhotic,portal fibrosis.,Portal Hypertension in Cirrhosis,Perisinusoidal,deposition of collagen,is the basis of PH in cirrhosis , it increase resistance to portal venous flow at the level of the sinusoids. This deposition results in narrowing and compression of the central veins caused by fibrosis.,Regenerative nodules,contributes pressure to this compression.,Arteriovenous,anastomoses,in a fibrous scar also contribute to the increased portal venous pressure.,肝后型,Budd,Chiari,综合征、缩窄性心包炎及严重右心衰等,门静脉系统病理与门静脉高压,脾源理论：1894年开始，班替(,Banti,),综合征。,门静脉系统病理与门静脉高压,机械梗阻理论：1900年开始，,Preble,等美国东北部长老会学派提出,治疗上以分流为主,机械梗阻理论的质疑：,1 门静脉压力不与肝硬化或脾大小成正比,二氧化硅门静脉注射等机械梗阻的方法未能造成门静脉高压的模型,门静脉系统病理与门静脉高压,递质机制的建立：,临床上发现肿大的脾脏随肝功能改善而缩小,组胺和去甲肾上腺素增加肝阻力，多巴胺与胰高血糖素使门静脉血流增加,治疗上以改善肝功能和清除、抑制或拮抗的方式来控制递质,A.,清除血浆置换,B.,抑制生长抑素类,C.,拮抗心得安等,Complications,Gastrointestinal tract hemorrhage,（,消化道出血）,Ascites,（,腹水）,Splenomegaly,and,hypersplenism,（,脾大伴脾亢）,Encephalopathy（,肝性脑病）,Splenomegaly,Splenomegaly,Splenomegaly,The spleen is enlarged and firm.,The,splenic,artery and vein are enlarged and tortuous, and they may be,aneurysmal,Histologically, sinusoids are dilated and lined by thickened epithelium.,Histiocytes,proliferate in the sinusoids with occasional,erythrophagocytosis,.,Hypersplenism,As the spleen enlarges, it can sequester,erythrocytes,leukocytes,platelets,resulting in mild-to-moderate decreases in some or all of these cell lines.,病因,1、门脉压力升高 脉系毛细血管床滤过压,增 高,组织液漏入腹腔,2、窦后阻塞、肝内淋巴产生增多、输出不畅,淋巴自肝包膜漏入腹腔,3、肝功能减退白蛋白合成障碍血浆胶体,渗透压降低血浆外渗,4、醛固酮、抗利尿激素体内灭活减少,钠水潴留,Pathogenesis of,Ascites,Decreased,oncotic,pressure,Increased hydrostatic pressure,Varices,of the,gastroesophageal,Junction,Why will,varices,form?,When portal pressures rise, blood flow is diverted to venous collaterals that dilate to form,varices,.,When will,varix,rupture and bleed?,The likelihood that any one,varix,will rupture and bleed depends on its wall tension.,In practice, a large, long,varix,with a high flow rate and a thin wall is most likely to rupture and bleed.,食管胃底曲张静脉破裂出血原因,胃酸反流，腐蚀食管下段粘膜,粗糙食物摩擦,咳嗽、呕吐、用力排便、重负等腹内压升高,Varices,of the,gastroesophageal,Junction,How to treat the,varix,in portal,hypertention,?,Therapies for portal hypertension aim to decrease,variceal,flow.,三个恶性循环圈,第一个是由脾脏肿大与门静脉的压力升高的恶性循环,第二个是由脾功能亢进与肝脏功能恶化、肝硬化和门静脉高压的恶性循环,第三个是食管胃底曲张静脉破裂出血与肝硬化程度和门静脉高压恶性循环,Encephalopathy,（,肝性脑病）,Encephalopathy,Pathophysiology,Portosystemic,shunting,Hepatic insufficiency,Toxins:,Ammonia,Endogenous Benzodiazepines,Diagnosis of Encephalopathy,Identify precipitating factor,Infection,GI bleed,Renal insufficiency,Drugs,Worsening liver function,Physical Exam,Asterixis,(,扑,翼性震颤,),Clinical Grade,Stage 0: No clinical signs,Stage 1:,Sleep-wake reversal,Stage 2:,Lethargy,（,嗜睡）, Slurred speech,（,言语含糊）,Stage 3: Stupor,（,木僵）,Stage 4: Coma,（,昏迷）,Clinical Presentation,and,Diagnosis,Clinical Presentation,Hematemesis,（呕血）,or,melena,（黑粪）,，,Hematochezia,（便血）,Increasing abdominal girth,（腹围）,(,ascites,formation),Splenomegaly,and,hypersplenism,(,脾肿大伴脾亢),Mental status changes such as lethargy and altered sleep patterns (presence of,portosystemic,encephalopathy),Abdominal pain and fever (spontaneous bacterial peritonitis SBP, which also presents without symptoms),Clinical Presentation,Signs of,portosystemic,collateral formation include the following:,Dilated veins in the anterior abdominal wall (umbilical,epigastric,vein shunts),Caput medusa (tortuous collaterals around the umbilicus),（水母头）,Rectal hemorrhoids,（痔疮）,Ascites,- Shifting dullness and fluid wave (if significant amount of,ascitic,fluid is present),Clinical presentation,Signs of liver disease include the following:,Ascites,（腹水）,Jaundice（,黄疸）,Spider,angiomas,（蜘蛛痣）,Palmar,erythema,（肝掌）,Asterixis,(,扑,翼性震颤,),Testicular atrophy,（睾丸萎缩）,Gynecomastia,(,乳房女性化,),Splenomegaly,（脾大）,Caput,Medusae,、,Palmar,Erythema,and Spider,Angiomas,Diagnosis,The medical history from a patient with portal hypertension should be directed towards determining the cause of portal hypertension （,病史）,The presence of the complications of portal hypertension（,并发症）,Lab and imaging studies（,实验室及影像）,（一）、血 象：,全血细胞减少,（二）、肝功能,：肝功受损，白,/,球（,A/G）,比例倒置，肝炎免疫等。,（三）、食管,X,线吞钡检查：,7080%,有食道静脉曲张,（四）、超声扫描,（,Duplex-Doppler,ultrasonography,）,：,1,肝硬变表现,早期可见肝脏肿大，实质回声致密，回声增强增粗。,晚期肝脏缩小，肝表现凹凸不平呈结节状、锯齿状、台阶状变化、不规则萎缩变形，弥漫性回声增强，分布不均匀。,2,腹水,3,脾肿大，长宽20,cm,4,门静脉内径及血流量测定,门静脉主干内径,13mm,，脾静脉内径大于,8mm,，门脉右支大于,10mm,，左支大于,llmm,。,（五）、食管内镜检查：,食道静脉曲张 轻：蛇形，,6mm，,波及食道 上段。,食道下端静脉曲张造影,（六）、,CT、MRI,等,1肝硬变 2腹水 3脾肿大（超过5个肋单位）,Endoscopy,All patients with cirrhosis should be considered for the presence of,varices,at the time of the initial diagnosis of cirrhosis.,Gastroesophageal,varices,confirm the diagnosis of portal hypertension; However, their absence does not rule it out.,Diagnosis of,Ascites,Physical exam,Shifting dullness,Imaging,Ultrasound,Paracentesis,Cell count,Culture,Ascites,albumin,鉴别诊断,（与食管胃底静脉曲张破裂出血鉴别）,1,、消化性溃疡（,peptic ulcer),：,发生率，年龄，部位，,出血血管,2,、出血性胃炎（,hemorrhagic gastritis),，糜烂性胃炎（,erosive gastritis),，应激性溃疡（,stress ulcer),：,发生率，,酒，非甾体抗炎药，皮质激素，休克，脓毒血症，,烧伤，手术后，,CNS,损伤,3,、胃癌（,gastric cancer),：,24%,4,、胆道出血（,hemobilia,),：,病因：肝外伤，肝血管瘤，,肝肿瘤，肝脓肿，胆管结石，胆道蛔虫症，胆管炎，,胆道出血三联征：绞痛，黄疸，出血，可以后继发,热,鉴别诊断,（与脾肿大鉴别）,1. 感染性脾肿大,病毒性肝炎、败血症等所致的急性感染脾肿大。,脾脓肿。,2. 血液病性脾肿大,慢性白血病、骨髓纤维化、慢性溶血性贫血，多有较明显的脾肿大。,慢性原发性血小板减少性紫癜。,3. 肿瘤性脾肿大,原发性脾肿瘤：,脾囊肿：真，假性囊肿。,Treatment,Histotry,of Treatment of Portal Hypertension,Year,Author,Contribution,1877,Eck,Portacaval,shunt(dog),1893,Pavlov,Encephalopathy(dog,),1903,Vidal,Clinical,portacaval,shunt(ascites,),1930,Westphal,Ballon,tamponade,1939,Crafoord,Frenckner,Endoscopic,sclerotherapy,1956,Kehne,Vasopressin,1967,Warren et al.,Distal,splenorenal,shunt,1967,Starzl,First successful liver transplant,1973,Sugiura,et al.,Extensive,esophagogastric,devascularization,1983,Colapinto,TIPS in human,原则：,第一， 分别对待两种不同原因的肝硬变,血吸虫性(,schistosomiasis,),窦前阻塞,脾肿大，脾功能亢进为主，,肝功良好 脾切除即获得良效。,肝炎后(,viral hepatitis),窦后阻塞脾,肿大，脾功能亢进不显著，肝功损害,明显手术效果较差。,第二， 外科治疗的目的是对症处理,1抢救食道胃底曲张,V,破裂所致的,大出血,2纠正脾亢,3顽固性腹水的处理,第三， 在抢救治疗中必须分别对待两种情况,1肝功良好,Child A,B,2,肝功不良,Child C,(一)、门脉高压食管胃底曲张,静脉破裂出血的治疗,黄疸，腹水，肝功能严重受损的病人,（,Child C, ,级）非手术疗法。,1输血：新鲜血,2药物治疗：,(1)、内脏血管收缩选择性降低门,V,压：,垂体加压素：20,u+5%GS200ml20-30,分钟,VD，,善得定(,Sandostatin)：100ug IV400ug/24h VD 35,天，,施他宁(,Stanlarnin)：300ug IV 6mg/24h VD,维持，,(2)、减低血排出量及内脏血管减压：,心得安：20,mg 3/,日，心率比原来慢25%为宜。,3三腔管气囊压迫,利用充气的气囊分别压迫胃底和食管下段破裂,的曲张静脉，达到止血目的。,(1)、 充气试验,(2)、 置放,(3)、 充气压迫,胃囊150-200,ml,空气，食道囊100-150,ml,空气,(4)、 牵引 0.5,Kg,(5) 、,注意事项：,A.,一般需压迫24-48,h，72h，,B. 12-24h,放空气囊，观察，若再出血重新压迫，,C.,加强护理，严防气囊上滑，填塞咽喉部引起窒息，,D.,适度牵引，注意前后鼻孔压迫坏死。,应用三腔二囊管注意事项,病人应侧卧或头部侧转。,三腔管一般放置24小时；如出血停止，可先排空食管气囊，后排空胃气囊，再观察1224小时，如确已止血，才将管慢慢拉出；,在进行三腔管压迫止血期间，要加强护理慎防窒息。,放置三腔管的时间不宜持续超过35天，每隔12小时应将气囊放空1020分钟；如有出血即充气压迫。,预防肝昏迷的发生。,4纤维内窥镜下硬化剂注射,内镜下将硬化剂直接注射到曲张静脉内，,近期疗效好，再出血率高，可高达45%。,5内镜下食管曲张静脉套扎 (,EVL),Band,Ligation,6放射介入行食道胃底曲张静脉栓塞术,经皮肝穿刺门脉分支栓塞法(,PTE),途径：皮肝门,V,系统冠状,V,栓塞剂,栓塞剂：凝血酶、95%无水乙醇、,十四烃硫酸钠,（二）、没有黄疸，腹水，肝功能尚正常,Child A，B(，),手术治疗,1,分流手术（,Portacaval,shunt）,门脉系统腔,V,系统减压,（1）、直接分流,a.,门腔静脉端侧分流术,b.,门腔静脉侧侧分流术,c.,肠系膜上、下腔静脉分流术（,H,架桥手术）,d.,中心性脾肾静脉分流术,共同特点：,降低了门静脉压力，控制出血，,但同时减少了向肝血流，肝功受损，,肝性脑病发生率高。,（2）选择性分流,a.,选择性远端脾肾分流术,(,warrens operation),不切除脾脏，而将脾静脉的远端与左肾,静脉的侧面作吻合。,b.,冠腔静脉分流术（,coronary -,caval,shunt）,冠状,V（,胃左,V）,下腔,V,c.,经颈静脉肝门门体分流术(,Transjugular,Intrahepatic,povtasystemic,stent,shunt,Tipss,),Tipss,是利用穿刺针，球囊导管和金属支架,在肝内建立人工门脉的静脉通道，治疗门,脉高压的介入放射新疗法。,适应证：,食道,V,曲张反复出血、曾行分流术、伴,顽固性腹水、不能耐受手术的急诊大出,血、等待肝移植预防消化道出血。,禁忌证：,严重心、肾功能不全，肝功衰竭，败,血症，胆道感染,疗效：,成功率90%-100%,食道,V,曲张好转 90%,急性出血控制 88-100%,腹水消失 70-75%,中远期疗效：1年生存68%,3年生存42%,近 期： 再出血4.5%,死亡5.3%,术后处理： 平卧48小时、抗凝治疗、,抗炎、保肝。,并发症及其处理：,术 中： 主要有胆道损伤、胆道出血、,肝外穿刺、腹腔出血。,近期： 肝性脑病,Partial portal systemic shunts（,限制性门腔分流）,Graft must be placed between the portal vein and the IVC,These reduce the size of the,anastomosis,of a side-to-side shunt to 8 mm in diameter. Portal flow is maintained in 80% of patients.,90% rate for control of bleeding.,Decrease the incidence of encephalopathy and liver failure.,2断流手术（,Portoazygos,disconneetion,Hassab,operation）,脾切除，同时结扎、切断冠状静脉及,食道贲门周围的门奇静脉间的反常血流，,达到止血目的。,注意点：,贲门周围血管可分四组，彻底切断这些,血管可能有效阻断门奇,V,间的反常血流。,a.,冠状静脉：胃支、食管支、高位食管支，,有时还出现异位高位食管支,b.,胃短,V,脾,V,c.,胃后静脉脾,V,d.,左膈,V,单支或分支进入食管下段左侧肌层,贲门周围血管离断术示意图高位食管支已离断,合理性：,a.,断流后门脉压力更高，入,肝门脉血供增加，改善肝,脏血供。,b.,贲门周围血管术是针对胃,脾区高压的手术，止血效,果明显。,3严重脾肿大，合并明显的脾 功能亢进、血吸虫性肝硬化， 脾亢单纯脾切除； 肝炎后肝硬化，脾亢脾切 除 +断流；4肝硬变引起的顽固性腹水 腹腔静脉转流术。,治疗门静脉高压症的理想术式应具备下述条件,能有效地预防和治疗食管、胃底曲张静脉破裂出血；,手术操作简单，手术死亡率及并发症低；,术后能恢复病人的劳动力，肝性脑病发生率低；,远期疗效好。,门静脉高压症并发食管胃底曲张静脉破裂出血的外科治疗,肝硬化病人中有,40%,发生食管胃底部静脉曲张，有上述部位静脉曲张者,50%,60%,可病发大出血,外科治疗的目的：紧急制止出血,采取手术的方法要依据门静脉高压症的病因，肝功能储备，门静脉系统主要血管可利用的情况和医师的操作技能及经验,Liver Transplantation,The ultimate shunt.,It is the treatment modality that has significantly improved the outcome of patients with child-Pugh class C disease and,variceal,bleeding.,Therapy of,Ascites,Medical Management:,Dietary Salt Restriction,Diuretic Therapy,Surgical Therapy:,Peritoneovenous,Shunt （,腹腔静脉转流术）,Side-to-side,portosystemic,Shunts,Tips,Liver,trransplantation,布加综合征（,Budd-,Chiari,Syndrome,）,