Meso-Rex bypass for the management of extrahepatic portal vein obstruction in adults(with video)

Background:Extrahepatic portal vein obstruction(EHPVO)results in severe portal hypertension(PHT)leading to severely compromised quality of life.Often,pharmacological and endoscopic management is unable to solve this problem.Restoring hepatic portal flow using meso-Rex bypass(MRB)may solve it.This procedure,uncommon in adult patients,is considered the treatment of choice for EHPVO in children.Methods:From 1997 to 2018,8 male and 6 female adults,with a median age of 51 years(range 22-66)underwent MRB procedure for EHPVO at the University Hospitals Saint-Luc in Brussels,Belgium.Symp-toms of PHT were life altering in all but one patient and consisted of repetitive gastro-intestinal bleedings,sepsis due to portal biliopathy,and/or severe abdominal discomfort.The surgical technique consisted in interposition of a free venous graft or of a prosthetic graft between the superior mesenteric vein and the Rex recess of the left portal vein.Results:Median operative time was 500 min(range 300-730).Median follow-up duration was 22 months(range 2-169).One patient died due to hemorrhagic shock following percutaneous transluminal interven-tion for early graft thrombosis.Major morbidity,defined as Clavien-Dindo score≥III,was 35.7%(5/14).Shunt patency at last follow-up was 64.3%(9/14):85.7%(6/7)of pure venous grafts and only 42.9%(3/7)of prosthetic graft.Symptom relief was achieved in 85.7%(12/14)who became asymptomatic after MRB.Conclusions:Adult EHPVO represents a difficult clinical condition that leads to severely compromised quality of life and possible life-threatening complications.In such patients,MRB represents the only and last resort to restore physiological portal vein flow.Although successful in a majority of patients,this procedure is associated with major morbidity and mortality and should be done in tertiary centers expe-rienced with vascular liver surgery to get the best results.

Etiology and long-term outcome of extrahepatic portal vein obstruction in children

AIM:To study the management and outcome of children with extrahepatic portal vein obstruction(EHPVO) in a whole country population.METHODS:A nationwide multicenter retrospective case series of children with EHPVO was conducted.Data on demographics,radiographic studies,laboratory workup,endoscopic and surgical procedures,growth and development,were extracted from the patients' charts.Characteristics of clinical presentation,etiology of EHPVO,management and outcome were analyzed.RESULTS:Thirty patients,13 males and 17 females,19(63.3%) Israeli and 11(36.7%) Palestinians,were included in the analysis.Age at presentation was 4.8± 4.6 years,and mean follow-up was 4.9±4.3 years.Associated anomalies were found in 4 patients.The incidence of EHPVO in Israeli children aged 0-14 years was 0.72/million.Risk factors for EHPVO were detected in 13(43.3%)patients,including 9 patients(30%) with perinatal risk factors,and 4 patients(13.3%) with prothrombotic states:two had low levels of protein S and C,one had lupus anticoagulant,and one was homozygous for methyltetrahydrofolate reductase mutations.In 56.6% of patients,no predisposing factors were found.The most common presenting symptoms were an incidental finding of splenomegaly(43.3%),and upper gastrointestinal bleeding(40%).No differences were found between Israeli and Palestinian children with regard to age at presentation,etiology and clinical symptoms.Bleeding occurred in 18 patients(60%),at a median age of 3 years.Sclerotherapy or esophageal banding was performed in 20 patients.No sclerotherapy complications were reported.Portosystemic shunts were performed in 11 patients(36.6%),at a median age of 11(range 3-17)years:splenorenal in 9,mesocaval in 1,and a meso-Rex shunt in 1 patient.One patient underwent splenectomy due to severe pancytopenia.Patients were followed up for a median of 3(range 0.5-15)years.One patient died aged 3 years due to mucopolysaccharidase deficiency type Ⅲ.None of the patients died due to gastrointestinal bleeding.CONCLUSION:EHPVO is a rare disorder.The etiological factors are still mostly unknown,and the endoscopic and surgical treatment options ensure a good long-term prognosis.

Portal vein thrombosis: Etiology and clinical outcome of cirrhosis and malignancy-related non-cirrhotic, non-tumoral extrahepatic portal venous obstruction

The etiology and pathogenesis of portal vein thrombosis are unclear. Portal venous thrombosis presentation differs in cirrhotic and tumor-related versus non-cirrhotic and non-tumoral extrahepatic portal venous obstruction (EHPVO). Non-cirrhotic and non-tumoral EHPVO patients are young and present with well tolerated bleeding. Cirrhosis and tumor-related portal vein thrombosis patients are older and have a grim prognosis. Among the 118 patients with portal vein thrombosis, 15.3% had cirrhosis, 42.4% had liver malignancy (primary or metastatic), 6% had pancreatitis (acute or chronic), 5% had hypercoagulable state and 31.3% had idiopathy, 12% had hypercoagulable state in the EHPVO group.

Management of splenic artery aneurysm associated with extrahepatic portal vein obstruction

BACKGROUND: Splenic artery aneurysms although rare are clinically significant in view of their propensity for spontaneous rupture and life-threatening bleeding. While portal hypertension is an important etiological factor, the majority of reported cases are secondary to cirrhosis of the liver. We report three cases of splenic artery aneurysms associated with extrahepatic portal vein obstruction and discuss their management. METHODS: The records of three patients of splenic artery aneurysm associated with extrahepatic portal vein obstruction managed from 2003 to 2010 were reviewed retrospectively. The clinical presentation, surgical treatment and outcome were analyzed. RESULTS: The aneurysm was >3 cm in all patients. The clinical symptoms were secondary to extrahepatic portal vein obstruction (hematemesis in two, portal biliopathy in two) while the aneurysm was asymptomatic. Doppler ultrasound demonstrated aneurysms in all patients. A proximal splenorenal shunt was performed in two patients with excision of the aneurysm in one patient and ligation of the aneurysm in another one. The third patient had the splenic vein replaced by collaterals and hence underwent splenectomy with aneurysmectomy. All patients had an uneventful post-operative course. CONCLUSIONS: Splenic artery aneurysms are associated with extrahepatic portal vein obstruction. Surgery is the mainstay of treatment. Although technically difficult, it can be safely performed in an experienced center with minimal morbidity and good outcome.

Treatment of a patient with obstructive jaundice and extrahepatic portal hypertension

BACKGROUND: Recurrence of inflammation in the extrahepatic bile duct can lead to bile duct stenosis' obstructive jaundice and cavernous transformation of the portal vein. The latter can develop into extrahepatic portal hypertension (PHT). It is difficult to establish the correct method for treating these conditions. METHODS: At another hospital, a 51-year-old man developed PHT as a result of endoscopic retrograde cholangiopancreatography and endoscopic nasobiliary drainage to relieve cholelithiasis and obstructive jaundice. We dealt with the biliary tract obstruction through percutaneous transhepatic cholangial drainage (PTCD), followed by selective devascularization and a shunt operation 2 weeks after the disappearance of jaundice. Three months after cholecystojejunostomy, there were no obvious changes around the bile duct. RESULT: The patient recovered uneventfully and was discharged 14 days after operation. CONCLUSION: For this patient, surgery in stages was the best choice. The most suitable method to decrease jaundice is PTCD.

Catheter tract implantation metastases associated with percutaneous biliary drainage for extrahepatic cholangiocarcinoma

AIM： To estimate the incidence of catheter tract implantation metastasis among patients undergoing percutaneous transhepatic biliary drainage （PTBD） for extrahepatic cholangiocarcinoma, and to provide data regarding the management of this unusual complication of PTBD by reviewing cases reported in the literature. METHODS： A retrospective analysis of 67 consecutive patients who underwent PTBD before the resection of extrahepatic cholangiocarcinoma was conducted. The median follow-up period after PTBD was 106 too. The English language literature （PubMed, National Library of Medicine, Bethesda, MD, USA）, from .lanuary 1966 through December 2004, was reviewed. RESULTS： Catheter tract implantation metastasis developed in three patients. The cumulative incidence of implantation metastasis reached a plateau （6%） at 20 mo after PTBD. All of the three patients with implantation metastasis died of tumor progression at 3, 9, and 20 mo after the detection of this complication. Among the 10 reported patients with catheter tract implantation metastasis from extrahepatic cholangiocarcinoma （including our three patients）, two survived for more than 5 years after the excision of isolated catheter tract metastases. CONCLUSION： Catheter tract implantation metastasis is not a rare complication following PTBD for extrahepatic cholangiocarcinoma. Although the prognosis for patients with this complication is generally poor, the excision of the catheter tract may enable survival in selected patients with isolated metastases along the catheter tract.

Feasibility and efficacy evaluation of metallic biliary stents eluting gemcitabine and cisplatin for extrahepatic cholangiocarcinoma

BACKGROUND Effective endoscopic management is fundamental for the treatment of extrahepatic cholangiocarcinoma(ECC).However,current biliary stents that are widely used in clinical practice showed no antitumor effect.Drug-eluting stents(DESs)may achieve a combination of local chemotherapy and biliary drainage to prolong stent patency and improve prognosis.AIM To develop novel DESs coated with gemcitabine(GEM)and cisplatin(CIS)-coloaded nanofilms that can maintain the continuous and long-term release of antitumor agents in the bile duct to inhibit tumor growth and reduce systemic toxicity.METHODS Stents coated with different drug-eluting components were prepared by the mixed electrospinning method,with poly-L-lactide-caprolactone(PLCL)as the drug-loaded nanofiber membrane and GEM and/or CIS as the antitumor agents.Four different DESs were manufactured with four drug-loading ratios(5%,10%,15%,and 20%),including bare-loaded(PLCL-0),single-drug-loaded(PLCL-GEM and PLCL-CIS),and dual-drug-loaded(PLCL-GC)stents.The drug release property,antitumor activity,and biocompatibility were evaluated in vitro and in vivo to confirm the feasibility and efficacy of this novel DES for ECC.RESULTS The in vitro drug release study showed the stable,continuous release of both GEM and CIS,which was sustained for over 30 d without an obvious initial burst,and a higher drug-loaded content induced a lower release rate.The drug-loading ratio of 10%was used for further experiments due to its ideal inhibitory efficiency and relatively low toxicity.All drug-loaded nanofilms effectively inhibited the growth of EGI-1 cells in vitro and the tumor xenografts of nude mice in vivo;in addition,the dual-loaded nanofilm(PLCL-GC)had a significantly better effect than the single-drug-loaded nanofilms(P<0.05).No significant differences in the serological analysis(P>0.05)or histopathological changes were observed between the single-loaded and drug-loaded nanofilms after stent placement in the normal porcine biliary tract.CONCLUSION This novel PLCL-GEM and CIS-eluting stent maintains continuous,stable drug release locally and inhibits tumor growth effectively in vitro and in vivo.It can also be used safely in normal porcine bile ducts.We anticipate that it might be considered an alternative strategy for the palliative therapy of ECC patients.

A pilot study using lactulose in management of minimal hepatic encephalopathy in children with extrahepatic portal vein obstruction

Background:Minimal hepatic encephalopathy(MHE)is not associated with overt neuropsychiatric symptoms but rather with subtle changes in psychometric and/or neurOphysiolOgic tests.We aimed to diagnose MHE in children with extrahepatic portal vein obstruction fEHPV01 and to evaluate the el№ct of lactulose on MHE.Methods:A prospective study was carried out on 30 patients with EHPVO(21 males;mean age 10+2.5 years).The study was carried out in the Pediatric Hepatology Unit.Cairo University Pediatric Hospital,Cairo,Egypt,between 2011 and 2013.AII patients were subjected to clinical and Iaboratory assessment.neuropsychmetric testin2 using the arabic version of Wechsler intelligence tests.neurophysiological testing by visual electroencephalogram and P300 event related potentials(ERP).

Imaging and radiological interventions in extra-hepatic portal vein obstruction

Extrahepatic portal vein obstruction(EHPVO) is a primary vascular condition characterized by chronic long standing blockage and cavernous transformation of portal vein with or without additional involvement of intrahepatic branches, splenic or superior mesenteric vein. Patients generally present in childhood with multiple episodes of variceal bleed and EHPVO is the predominant cause of paediatric portal hypertension(PHT) in developing countries. It is a pre-hepatic type of PHT in which liver functions and morphology are preserved till late. Characteristic imaging findings include multiple parabiliary venous collaterals which form to bypass the obstructed portal vein with resultant changes in biliary tree termed portal biliopathy or portal cavernoma cholangiopathy. Ultrasound with Doppler, computed tomography, magnetic resonance cholangiography and magnetic resonance portovenography are non-invasive techniques which can provide a comprehensive analysis of degree and extent of EHPVO, collaterals and bile duct abnormalities. These can also be used to assess in surgical planning as well screening for shunt patency in post-operative patients. The multitude of changes and complications seen in EHPVO can be addressed by various radiological interventional procedures. The myriad of symptoms arising secondary to vascular, biliary, visceral and neurocognitive changes in EHPVO can be managed by various radiological interventions like transjugular intra-hepatic portosystemic shunt, percutaneous transhepatic biliary drainage, partial splenic embolization, balloon occluded retrograde obliteration of portosystemic shunt(PSS) and revision of PSS.

Therapeutic Mechanisms of Single Chinese Medicine Herb or Their Extracts for Extrahepatic Obstructive Jaundice

Obstructive jaundice （O J） is classified as extrahepatic OJ or intrahepatic OJ. Extrahepatic OJ is attributed to a variety of intricate etiological factors. Research has begun with Chinese medicine （CM）, which can be used as an adjunctive therapy for extrahepatic OJ. Particular attention has been paid to the therapeutic effects and their mechanisms of single CM herb and relevant extracts. The roles of single CM or their extracts during adjunctive therapy for extrahepatic OJ have been described briefly, This review focuses on the effects and their mechanisms of relevant herbal medicines.

Current approaches to the management of jejunal variceal bleeding at the site of hepaticojejunostomy after pancreaticoduodenectomy

Jejunal variceal bleeding at the site of hepaticojejunostomy after pancre-aticoduodenectomy due to portal hypertension caused by extrahepatic portal vein obstruction is a life-threatening complication and is very difficult to treat.Pharma-cotherapy,endoscopic methods,transcatheter embolization of veins supplying the jejunal afferent loop,portal venous stenting,and surgical procedures can be used for the treatment of jejunal variceal bleeding.Nevertheless,the optimal mana-gement strategy has not yet been established,which is due to the lack of ran-domized controlled trials involving a large cohort of patients necessary for their development.

磁共振胰胆管成像对胆道梗阻性疾病定位与定性诊断的价值

目的 探讨磁共振胰胆管成像 (MRCP)对胆道梗阻性疾患定位和定性诊断的价值。方法 对 85例梗阻性黄疸病人的MRCP进行分析 ,并与CT、US、直接胆道造影和临床手术病理结果对照。结果 全部病例MRCP检查均一次成功 ,其中 48例行直接胆道造影 ,与MRCP对照 ,二者所获图像极其相似。且对ERCP或PTC显示不完全的病例MRCP可补充有价值的诊断信息。MRCP对梗阻定位诊断准确率为 10 0 %。对梗阻定性诊断准确率为 89.4% ,结合MRI可提高至95 .3 %。结论 MRCP对梗阻性黄疸定位诊断与定性诊断准确率均高于CT及US。MRCP图像与直接胆道造影图像基本一致 ,方便易行无损伤 ,应列为胆道梗阻性疾病的术前常规检查方法。

高位胆管梗阻的介入治疗和近期疗效分析

目的回顾性分析高位梗阻性黄疸患者介入治疗方法及近期疗效。方法100例接受经皮肝穿刺胆汁引流或胆道支架置入治疗的高位梗阻性黄疸患者,其中胆管癌39例,转移瘤22例,肝移植后胆管病变18例,原发性肝癌15例,胆囊癌6例。测定术前,术后3～7d、8～14d血胆红素水平并进行显著性检验。结果79例行单纯外引流或内外引流,21例行胆道支架置入术,所用支架4种31枚。术前血清胆红素含量与术后3～7d胆红素水平差别有显著性(P<0.05),与术后8～14d胆红素水平比较有非常显著的差异(P<0.01)。结论介入治疗高位梗阻性黄疸方法简单、近期疗效满意。

丹参注射液对梗阻性黄疸大鼠肝内NO、MDA、ET的影响及其意义

目的 探讨丹参注射液对梗阻性黄疸 (Obstructivejaundice ,OJ)大鼠肝组织中一氧化氮(NO)、丙二醛 (MDA)、内皮素 (ET)的影响及其意义。方法 采用双重结扎切断大鼠胆总管造成OJ模型 ,分别给大鼠腹腔注射生理盐水 (模型组 )和丹参注射液 (治疗组 ) ,各组于术后 7d和1 4d观察肝组织匀浆NO2 - +NO3- 、MDA、ET含量 ,肝功能及肝组织形态改变。结果 胆道梗阻 7d和 1 4d后大鼠肝组织中NO2 - +NO3- 、MDA含量较正常鼠明显增加 ,两者不随梗阻时间延长而升高 ;肝组织中ET含量亦明显增高 ,且随时间延长而增高。丹参治疗组大鼠肝组织中NO2 - +NO3- 与模型组无差异 ,而MDA和ET含量明显低于模型组 ,血浆ALT、AST明显降低 ,肝组织损伤减轻。结论 丹参注射液可降低OJ大鼠肝内MDA、ET含量 。

经皮胆道引流术治疗恶性梗阻性黄疸

目的 回顾性分析恶性梗阻性黄疸的介入性经皮胆道引流治疗方法及其疗效。方法 对4 7例患者采用经皮肝穿刺胆道引流术治疗恶性梗阻性黄疸。先行PTC后探查通过阻塞段 ,如不能越过阻塞段 ,则直接进行外引流 ;如能越过阻塞段 ,则置入内外引流管作内外引流或置入金属支架或塑料内涵管作内引流。结果 内引流组 18例 ,15例置入金属支架 ,共 18枚 ,3例置入塑料内涵管 ,共 4根 ;内外引流组 15例 ,置入 18根内外引流管 ;外引流组 14例 ,置入 17根外引流管。术后 1周总胆红素从术前的 (5 14 .1± 2 0 4 .3) μmol/L降至 (2 38.4± 14 2 .8) μmol/L(P <0 .0 0 1) ,碱性磷酸酶与丙氨酸转氨酶均下降明显 (P均 <0 .0 0 1)。 4例于术后 1个月内死亡。跟踪随访 37例 ,平均随访 5 .3个月 ,8例仍存活。随访期内 18例 (48.6 % )总胆红素降至正常范围。结论 经皮肝穿刺胆道引流术是对恶性梗阻性黄疸的一种安全、有效的姑息性治疗方法 ,可明显缓解黄疸、减轻痛苦、提高生存质量 ,并可改善肝脏功能 ,有限地延长生存时间。

MRCP在肝外胆管梗阻中的诊断价值

目的 探讨磁共振胰胆管成像(magnetic resonance cholangiopancreatography, MRCP)技术在肝外胆 磁共振水成像 肝外胆管梗阻中的诊断价值。方法 收集我院2004年收治的42例胆管梗阻患者的ＭＲＣＰ检查临床资料,分析良、恶性肝外胆管梗阻在ＭＲＣＰ上的不同表现。结果 ＭＲＣＰ检查在30例良性梗阻患者主要表现为胆管均匀扩张及逐渐狭窄,管壁均匀增厚,断端可呈“鸟嘴样”,其在良性梗阻中的定位及定性准确率分别为100%(30/30)和97%(29/30);12例恶性梗阻患者中主要表现为胆管不均匀对称扩张及突然狭窄或截断,管壁不均匀增厚,可出现“双管截断征”,其在恶性梗阻中的定位及定性准确率分别为100%(12/12)和92%(11/12)。结论 ＭＲＣＰ是一种无创性显示胰胆管管腔形态学改变的影像学方法,在评价肝外胆管梗阻中具有较好的定位及定性准确率。

阻塞性黄疸对肠道细菌及小肠粘膜组织的影响

为探讨阻塞性黄疸（简称阻黄）对肠道细菌及粘膜组织的影响，通过建立阻黄动物模型，观察阻黄大鼠肠道细菌移位以及小肠粘膜组织学变化。结果发现：阻黄组（ＢＤＬ）术后３周厌氧性细菌移位的阳性率（４３．７５％）显著高于假性手术组（ＳＬ）（０％），Ｐ＜０．０５；组织学检查显示ＢＤＬ组肠粘膜发生了实质性损害。提示：阻黄时肠道内胆盐缺乏导致肠道常驻菌过度繁殖，肠道粘膜屏障的损害以及机体免疫功能抑制可能是促进肠道细菌移位。

磁共振胰胆管成像在诊断胆胰系疾病中的应用

目的评价磁共振胰胆管成像（ＭＲＣＰ）技术在胆胰系疾病诊断中的应用价值。方法对３８例患者进行了ＭＲＣＰ的检查，并与Ｂ超、ＣＴ比较，其中梗阻性黄疸３２例，２８例进行了手术治疗。结果ＭＲＣＰ能够清楚地显示胆胰管的结构，对胆道梗阻的部位确诊率达１００％，梗阻的病因确诊率达９６４３％，优于Ｂ超和ＣＴ。结论ＭＲＣＰ在胆胰系疾病中，特别是梗阻性黄疸的定位和定性诊断上有着良好的准确性。

PTCD治疗高位恶性梗阻性黄疸的临床应用(附16例分析)

目的:探讨PTCD术治疗高位恶性梗阻性黄疸的疗效及影响因素。方法:回顾性分析16例高位恶性梗阻性黄疸患者经PTCD术前后血清总胆红素(TB)的变化。结果:16例患者中12例黄疸症状得到改善。2周后TB均值由术前419.82umol/l降至233.3umol/l,方差分析,F=5.932,P<0.01。4例因胆道梗阻复杂或术后胆道感染,疗效不佳。结论:PTCD术治疗高位恶性梗阻性黄疸具有一定的近期疗效。

ERCP后CT扫描对阻塞性黄疸诊断价值的研究

目的 探讨 ERCP后 CT扫描的临床价值。材料与方法 34例阻塞性黄疸患者进一步作 ERCP后 CT扫描。方法为ERCP拔管后 1～ 2小时行 CT扫描。对比评价 ERCP后 CT扫描与单纯 ERCP检查有无不同。结果 34例患者两种检查方法正确诊断 19例 ,ERCP误诊或诊断不全面 ,由 ERCP后 CT扫描诊断正确 13例 ,ERCP后 CT扫描误诊或诊断不全面 ,由 ERCP作出正确诊断 1例 ,两种方法共同误诊 1例。经配对 χ2检验 ,P<0 .0 5。结论 ERCP后 CT扫描将 ERCP和 CT二者结合起来 ,可取长补短 ,提高诊断正确率 。