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胰源性门静脉高压症诊治规范(草案)

2016-11-23 赵玉沛 李晓斌 李宏为 姜洪池 窦科峰 董家鸿 别平 乔海泉 王宏光 廖泉 丛林 中华普通外科杂志

胰源性门静脉高压(pancreatic sinistral portal hypertension,PSPH)是指由于胰腺疾病及其并发症致使睥静脉受压、扭曲、管壁炎性增厚或管内堵塞,脾静脉血栓形成,影响脾静脉回流,导致脾静脉压力增高,最终脾脏肿大和脾胃区静脉压增高。在门静脉高压症中,仅有5%患者系胰源性门静脉高压症。 一、病因 胰腺疾病引起脾静脉血栓形成(s

胰源性门静脉高压(pancreatic sinistral portal hypertension,PSPH)是指由于胰腺疾病及其并发症致使睥静脉受压、扭曲、管壁炎性增厚或管内堵塞,脾静脉血栓形成,影响脾静脉回流,导致脾静脉压力增高,最终脾脏肿大和脾胃区静脉压增高。在门静脉高压症中,仅有5%患者系胰源性门静脉高压症。
 
一、病因
 
胰腺疾病引起脾静脉血栓形成(splenic vein thrombosis,SVT)所致脾静脉回流受阻是导致PSPH的主要原因,引起PSPH的胰腺疾病可大致分为3类:炎症、肿瘤及其他胰腺疾病。胰腺炎症包括急性胰腺炎、慢性胰腺炎、家族性胰腺炎及创伤性胰腺炎等。胰腺炎症渗出,胰蛋白酶释放、血液高凝状态、脾静脉发生痉挛、血流受阻、血管内皮细胞表改变、相对低灌注、纤维化挛缩等多种因素参与了胰腺炎引起SVT的病理过程。胰腺肿瘤尤其是生长在体尾部的肿瘤,脾静脉容易受到侵犯、压迫,从而导致脾静脉血栓形成,回流受阻。其他引起PSPH的病因包括:胰腺假性囊肿、胰腺脓肿、先天性囊肿等。
 
二、病理生理
 
因脾静脉回流受阻导致脾胃区静脉压力增高,可以发牛以下病理变化:
1.脾肿大和脾功能亢进。
2.侧支循环建立:脾静脉高压时,压力相对较低的脾静脉的属枝(如胃网膜左静脉、胃短静脉等)可转变为脾脏血流的流出道,它们与呈相对低压的门静脉近肝门段或奇静脉属枝问的侧支循环也逐渐开放。这些侧支循环大多是流向肝脏的,包括胃网膜静脉、胃短静脉、胃冠状静脉、左侧结肠静脉,少数情况下可自发形成脾肾分流:(1)脾静脉血经胃短静脉流至黏膜下静脉网,引起胃底静脉曲张,随后经胃底静脉,回流至胃冠状静脉,进一步流入门静脉,另一方面则与奇静脉、半奇静脉问形成侧支循环直接流回心脏,这一通路造成食管下端静脉曲张甚至出血。(2)脾静脉血经胃网膜左静脉流胃网膜右静脉,然后直接入门静脉或经肠系膜上静脉入门静脉。(3)胃网膜左静脉血亦可经曲张的大网膜静脉,流人肠系膜下静脉致结肠静脉曲张甚至出血。(4)脾静脉血经膈血管到肋问血管再到上腔静脉。
 
三、临床表现
 
主要为胰腺疾病和局部(脾胃区)门静脉压力升高而出现的临床表现:
(1)胰腺本身的疾病表现:如慢性胰腺炎常有慢性上腹痛及腰背部疼痛表现;急性胰腺炎多有典型的腹痛、腹胀、恶心、啾吐等消化道症状及腹膜炎体征,部分可继发感染;胰腺似性囊肿多继发于急性胰腺炎或是胰腺外伤后,逐渐出现腹胀,压迫胃肠道时可有恶心、呕吐等症状,继发感染时有发热和疼痛;胰腺体尾部肿瘤早期多无明显表现,晚期可…现消瘦、食欲不振,当肿瘤侵犯神经时叮出现腹部或腰背部疼痛。
(2)脾脏疾病表现:明显的脾功能亢进,脾脏肿人。
(3)胃底或兼有食管下端静脉曲张表现:胃底和食管下段静脉曲张,消化道出血多主要是由于胃底曲张静脉破裂引起,少数情况下食管静脉曲张也可破裂出血,表现为呕血或黑便,并伴有贫血。
(4)肝功能情况:通常化验结果正常,只有同时合并有肝脏疾患或有其他原因所致肝损害者肝功能才会f1{现异常。
 
四、诊断
 
根据临床表现、生化检查和影像学评价等进行综合判断,PSPH的主要表现为胰腺疾病和局部(脾胃区)门静脉高压两组症状。胃底和胃大弯侧曲张血管明显,食管下段静脉曲张不明显,是胰源性门静脉高压症区别于一般门静脉高压症的一大特征。PSPH另一特点是肝功能通常正常。常用检查方法有以下几种:


(1)血液检查:脾功能亢进时,血细胞计数减少,以白细胞和血小板降低最为明显;血清淀粉酶检查有助于明确有无胰腺炎的存在。
(2)B超检查:了解胰腺的病变情况、肝脏及脾脏大小,脾静脉内有无血栓及血流情况。
(3)钡餐或内镜检查:了解有无胃底或食管下段静脉曲张及程度。
(4)CT或MRI检查:胰腺、脾脏、肝脏的病变情况以及门静脉系统成像判断门静脉系统血流受阻的情况。

诊断应依据下列原则:
(1)既往有胰腺病史或胰腺外伤史。
(2)无肝硬变、血液系统疾病及血吸虫病史,肝功能正常(但肝功能异常亦不应盲目排除PSPH的诊断)。
(3)脾脏增大或脾功能亢进表现。
(4)B超或MRA检查示门静脉不增宽,脾静脉血流受阻及脾门及脾周迂曲扩张的静脉团形成。
(5)胃镜下可见胃底静脉曲张。具备上述临床特点病例可确诊为PSPH。
符合其中3项时应高度怀疑PSPH,行进一步检查。

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    2017-01-20 fusion
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    2016-11-26 雅文博武

    学习了很多的医学知识讲座

    0

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    2016-11-25 虈亣靌

    讲的好,学习了

    0

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    2016-11-23 ylzr123

    胰源性门静脉高压是指由于胰腺疾病及其并发症致使睥静脉受压、扭曲、管壁炎性增厚或管内堵塞,脾静脉血栓形成,影响脾静脉回流,导致脾静脉压力增高,最终脾脏肿大和脾胃区静脉压增高。

    0

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