Intrahepatic portal venous systems in adult patients with cavernous transformation of portal vein: Imaging features and a new classification

Background: Cavernous transformation of the portal vein(CTPV) due to portal vein obstruction is a rare vascular anomaly defined as the formation of multiple collateral vessels in the hepatic hilum. This study aimed to investigate the imaging features of intrahepatic portal vein in adult patients with CTPV and establish the relationship between the manifestations of intrahepatic portal vein and the progression of CTPV. Methods: We retrospectively analyzed 14 CTPV patients in Beijing Tsinghua Changgung Hospital. All patients underwent both direct portal venography(DPV) and computed tomography angiography(CTA) to reveal the manifestations of the portal venous system. The vessels measured included the left portal vein(LPV), right portal vein(RPV), main portal vein(MPV) and the portal vein bifurcation(PVB). Results: Nine males and 5 females, with a median age of 40.5 years, were included in the study. No significant difference was found in the diameters of the LPV or RPV measured by DPV and CTA. The visualization in terms of LPV, RPV and PVB measured by DPV was higher than that by CTA. There was a significant association between LPV/RPV and PVB/MPV in term of visibility revealed with DPV( P = 0.01), while this association was not observed with CTA. According to the imaging features of the portal vein measured by DPV, CTPV was classified into three categories to facilitate the diagnosis and treatment. Conclusions: DPV was more accurate than CTA for revealing the course of the intrahepatic portal vein in patients with CTPV. The classification of CTPV, that originated from the imaging features of the portal vein revealed by DPV, may provide a new perspective for the diagnosis and treatment of CTPV.

Application of transmesenteric vein extrahepatic portosystemic shunt in treatment of symptomatic portal hypertension with cavernous transformation of portal vein

Purpose:To evaluate the feasibility and efficacy of a transmesenteric vein extrahepatic portosystemic shunt(TmEPS)for the treatment of cavernous transformation of the portal vein(CTPV).Materials and methods:The clinical data of 20 patients with CTPV who underwent TmEPS between December 2020and January 2022 at Henan Provincial People’s Hospital were retrospectively collected.The superior mesenteric vein(SMV)trunk was patent or partially occluded in these patients.An extrahepatic portosystemic shunt between the inferior vena cava and the SMV was established using a stent graft through an infraumbilical median longitudinal mini-laparotomy.The technical success,efficacy,and complication rates were evaluated,and the preand postoperative SMV pressures were compared.Patients’clinical outcomes and shunt patency were assessed.Results:TmEPS was successfully performed in 20 patients.The initial puncture success rate of the balloon-assisted puncture technique is 95%.The mean SMV pressure decreased from 29.1±2.9 mmHg to 15.6±3.3 mmHg(p<0.001).All symptoms of portal hypertension resolved.No fatal procedural complications occurred.During the follow-up period,hepatic encephalopathy occurred in two patients.The remaining patients remained asymptomatic.All shunts were patent.Conclusions:TmEPS is a feasible,safe,and effective treatment option for patients with CTPV.

Transjugular intrahepatic portosystemic shunt for the prevention of recurrent esophageal variceal bleeding in patients with cavernous transformation of portal vein

Background: Treatment options for patients with cavernous transformation of portal vein(CTPV) are limited. This study aimed to evaluate the feasibility, efficacy and safety of transjugular intrahepatic portosystemic shunt(TIPS) to prevent recurrent esophageal variceal bleeding in patients with CTPV. Methods: We retrospectively analyzed 67 consecutive patients undergone TIPS from January 2011 to December 2016. All patients were diagnosed with CTPV. The indication for TIPS was a previous episode of variceal bleeding. The data on recurrent bleeding, stent patency, hepatic encephalopathy and survival were retrieved and analyzed. Results: TIPS procedure was successfully performed in 56 out of 67(83.6%) patients with CTPV. TIPS was performed via a transjugular approach alone( n = 15), a combined transjugular/transhepatic approach( n = 33) and a combined transjugular/transsplenic approach( n = 8). Mean portosystemic pressure gradient(PSG) decreased from 28.09 ± 7.28 mmHg to 17.53 ± 6.12 mmHg after TIPS( P < 0.01). The probability of the remaining free recurrent variceal bleeding was 87.0%. The probability of TIPS patency reached 81.5%. Hepatic encephalopathy occurrence was 27.8%, and survival rate was 88.9% until the end of follow-up. Four out of 11 patients who failed TIPS died, and 4 had recurrent bleeding. Conclusions: TIPS should be considered a safe and feasible alternative therapy to prevent recurrent esophageal variceal bleeding in patients with CTPV, and to achieve clinical improvement.

How can portal vein cavernous transformation cause chronic incomplete biliary obstruction?

Biliary disease in the setting of non-cirrhotic portal vein thrombosis(and similarly in portal vein cavernous transformation) can become a serious problem during the evolution of disease.This is mostly due to portal biliary ductopathy.There are several mechanisms that play a role in the development of portal biliary ductopathy,such as induction of fibrosis in the biliary tract(due to direct action of dilated peribiliary collaterals and/or recurrent cholangitis),loss of biliary motility,chronic cholestasis(due to fibrosis or choledocholithiasis) and increased formation of cholelithiasis(due to various factors).The management of cholelithiasis in cases with portal vein cavernous transformation merits special attention.Because of a heterogeneous clinical presentation and concomitant pathophysiological changes that take place in biliary anatomy,diagnosis and therapy can become very complicated.Due to increased incidence and complications of cholelithiasis,standard treatment modalities like sphincterotomy or balloon sweeping of bile ducts can cause serious problems.Cholangitis,biliary strictures and hemobilia are the most common complications that occur during management of these patients.In this review,we specifically discuss important issues about bile stones related to bile duct obstruction in non-cirrhotic portal vein thrombosis and present evidence in the current literature.

Sixty-four-slice computed tomography in surgical strategy of portal vein cavernous transformation

AIM:To investigate the role of 64-slice computed tomography(CT) in portal vein cavernous transformation to determine surgical strategy.METHODS:The site of lesions and extent of collateral circulation in 12 pediatric cases of cavernous transformation of the portal vein with surgical treatment were analyzed.RESULTS:Eleven of 12 children had esophageal varices and were treated with lower esophageal and gastric devascularization and splenectomy,and the other case was only treated with splenectomy.There were eight cases with spontaneous spleen/stomach-renal shunt,four with Retzius vein opening,which was reserved during surgery.Three cases of lesions involving the intrahepatic portal vein(PV) were treated with living donor liver transplantation.One patient died from PV thrombosis after liver transplantation,and the rest had no significant complications.CONCLUSION:The PV,its branches and collateral circulation were clearly seen by 64-slice spiral CT angiography,which helped with preoperative surgical planning.

Sonographic features of umbilical vein recanalization for a Rex shunt on cavernous transformation of portal vein in children

BACKGROUND The Rex shunt was widely used as the preferred surgical approach for cavernous transformation of the portal vein(CTPV)in children that creates a bypass between the superior mesenteric vein and the intrahepatic left portal vein(LPV).This procedure can relieve portal hypertension and restore physiological hepatopetal flow.However,the modified procedure is technically demanding because it is difficult to make an end-to-end anastomosis of a bypass to a hypoplastic LPV.Many studies reported using a recanalized umbilical vein as a conduit to resolve this problem.However,the feasibility of umbilical vein recanalization for a Rex shunt has not been fully investigated.AIM To investigate the efficacy of a recanalized umbilical vein as a conduit for a Rex shunt on CTPV in children by ultrasonography.METHODS A total of 47 children who were diagnosed with CTPV with prehepatic portal hypertension in the Second Hospital,Cheeloo College of Medicine,Shandong University,were enrolled in this study.Fifteen children received a recanalized umbilical vein as a conduit for a Rex shunt surgery and were enrolled in group I.Thirty-two children received the classic Rex shunt surgery and were enrolled in group II.The sonographic features of the two groups related to intraoperative and postoperative variation in terms of bypass vessel and the LPV were compared.RESULTS The patency rate of group I(60.0%,9/15)was significantly lower than that of group II(87.5%,28/32)7 d after(on the 8th d)operation(P<0.05).After clinical anticoagulation treatment for 3 mo,there was no significant difference in the patency rate between group I(86.7%,13/15)and group II(90.6%,29/32)(P>0.05).Moreover,3 mo after(at the beginning of the 4th mo)surgery,the inner diameter significantly widened and flow velocity notably increased for the bypass vessels and the sagittal part of the LPV compared to intraoperative values in both shunt groups(P<0.05).However,there was no significant difference between the two surgical groups 3 mo after surgery(P>0.05).CONCLUSION For children with hypoplastic LPV in the Rex recessus,using a recanalized umbilical vein as a conduit for a Rex shunt may be an effective procedure for CTPV treatment.

CT diagnosis of cavernous transformation of portal vein

Objective: To study the CT features of cavernous transformation of the portal vein (CTPV) and to evaluate the diagnostic reliability of CT. Methods: Ten patients with CTPV underwent abdominal precontrast and contrast CT. The CT findings were compared with DSA and surgical findings. Results: Characteristic features included loss of the normal structure of the portal system and the presence of multiple, sinuous, periportal vascular structures which were enhanced during the portal phase. Transient abnormality of hepatic perfusion, characterized by the image of homogeneous bands, can be observed in arterial phase, which is not detectable in venous phase. Conclusion: CT proved to be reliable for the diagnosis of CTPV, and provides essential information for clinical treatment planning. Additional DSA examination and US were indicated for atypical patients for a more reliable diagnosis.

经颈静脉肝内门体分流术治疗门静脉高压伴门静脉血栓的疗效与安全性

目的探讨经颈静脉肝内门体静脉分流术(transjugular intrahepatic portosystemic shunt,TIPS)治疗门静脉高压伴门静脉血栓(portal vein thrombosis,PVT)的疗效及安全性。方法纳入2017年12月至2022年10月中国人民解放军总医院收治的符合TIPS治疗指征的31例门静脉高压伴PVT患者。收集患者临床资料,包括术前实验室检查、术式选择、术中门静脉压力(portal vein pressure,PVP)、术后随访超声或增强CT检查及有无肝性脑病(hepatic encephalopathy,HE)等。采用配对t检验比较支架植入前后PVP差异,Kaplan-Meier曲线分析患者术后分流道通畅率、再出血率、HE发生率及生存率。Log-rank检验分析伴或不伴有门静脉海绵样变性(cavernous transformation of portal vein,CTPV)患者的随访结果差异。结果TIPS成功率为93.55%(29/31)。手术成功的29例患者支架植入前后PVP由(30.15±4.61)mmHg降至(20.84±5.57)mmHg,差异有统计学意义(t=8.975,P<0.05)。术后随访时间为22.90(4.50,61.80)个月。随访期间,24.14%(7/29)的患者出现分流道功能障碍,17.24%(5/29)的患者出现再出血,17.24%(5/29)的患者出现HE,17.24%(5/29)的患者死亡。10例PVT患者伴有CTPV,伴有CTPV患者的分流道功能障碍5例、再出血3例、HE 1例、死亡3例,不伴有CTPV患者的分流道功能障碍2例、再出血2例、HE 4例、死亡2例。伴有CTPV的PVT患者的分流道功能障碍及再出血发生率高于不伴CTPV的患者(均P<0.05),而两组间HE发生率及术后病死率差异无统计学意义(均P>0.05)。结论TIPS可有效降低伴有PVT患者的PVP,PVT伴CTPV的患者TIPS后分流道功能障碍及再出血发生率高于不伴CTPV的患者。

门静脉海绵样变(CTPV)的介入治疗

目的总结良性病变所致门静脉海绵样变(cavernous transformation of portal vein,CTPV)介入治疗的经验及临床疗效。方法回顾性分析2000年至2012年24例在我科行介入治疗的CTPV患者的临床资料。所有患者发病时均有不同程度的呕血、黑便或腹痛等临床症状。经颈内静脉肝内门腔分流术(transjugularintrahepatic portosystemic shunt,TIPSS)联合置管局部溶栓14例,单纯TIPSS治疗或联合冠状静脉栓塞术2例,经皮穿刺脾静脉途径门静球主干囊扩张联合TIPSS治疗1例,经皮肝穿门静脉球囊扩张2例,经皮肝穿门静脉支架植入或联合胃冠状静脉栓塞术2例,脾动脉部分栓塞术(partial splenic embolization,PSE)2例。介入手术中途停止治疗1例。随访2个月～6年,观察门静脉及分流道通畅情况并随访上消化道出血及腹痛情况。结果随访时间内2例患者分别于术后2年及3年因TIPSS分流道狭窄或闭塞发生再次上消化道出血,行分流道修正术。1例行PSE于术后2年因上消化道出血转外科行脾切除加门奇静脉断流术;1例因无法开通闭塞的门静脉转外科行门奇静脉断流术联合分流;余所有患者未再出血或腹痛发生,门静脉或分流道通畅。结论介入手术是一种对CTPV安全、有效、微创的治疗方法,选择合适的介入手术方法是治疗成功的关键。

肝内外胆管结石合并CTPV外科治疗策略

目的研究肝内外胆管结石合并CTPV的外科治疗策略。方法收集我院自2010年1月至2014年3月共收治肝内外胆管结石患者670名,其中肝内外胆管结石患者合并门脉海绵样变性患者9例。分别采用ERCP/EST取石,胆总管切开取石,肝叶部分切除和分期手术方法。结果手术残石率44.4%(4/9),治疗总残石率22.2%(2/9)。ERCP取石成功2例,胆总管探查取石+T管外引流3例,肝叶部分切除3例,分期手术1例,术中输血率33.3%(3/9)。结论完善的术前影像学诊断,个体化的外科治疗方案,精准的外科操作,是诊治此类患者的要点。

Portal ductopathy:Clinical importance and nomenclature

Non-cirrhotic portal hypertension(PHT)accounts for about 20%of all PHT cases,portal vein thrombosis(PVT) resulting in cavernous transformation being the most common cause.All known complications of PHT may be encountered in patients with chronic PVT.However,the effect of this entity on the biliary tree and pancreatic duct has not yet been fully established.Additionally,a dispute remains regarding the nomenclature of common bile duct abnormalities which occur as a result of chronic PVT.Although many clinical reports have focused on biliary abnormalities,only a few have evaluated both the biliary and pancreatic ductal systems.In this review the relevant literature evaluating the effect of PVT on both ductal systems is discussed,and findings are considered with reference to results of a prominent center in Turkey,from which the term"portal ductopathy"has been put forth to replace"portal biliopathy".

Successful liver allograft inflow reconstruction with the right gastroepiploic vein

Portal vein thrombosis is a common complica- tion in cirrhotic patients. When portal vein thrombectomy is not a suitable option, a large collateral vessel can be used for allograft venous inflow reconstruction. We describe an unusu- al case of successful portal revascularization using the right gastroepiploic vein. The patient underwent a cadaveric or- thotropic liver transplantation with end-to-end anastomosis of the portal vein to the right gastroepiploic vein. Six months after liver transplantation the patient is well with good liver function. The use of the right gastroepiploic vein for allograft venous reconstruction is feasible and safe, with a great advan- tage of avoiding the need of venous jump graft.

JAK2V617F基因突变阳性骨髓增殖性肿瘤相关门静脉海绵样变性1例

骨髓增殖性肿瘤(myeloproliferative neoplasms,MPN)导致血细胞增加、血液高凝,是非肝硬化门静脉海绵样变性的重要病因。JAK2V617F基因突变可以帮助诊断MPN。我们报道1例JAK2V617F基因突变阳性的MPN患者发生门静脉海绵样变性(cavernous transformation of the portal vein,CTPV)。该例患者脾脏显著肿大,一方面是由于骨髓增殖性疾病本身的原因,另一方面是由于PVCT、门脉高压和脾脏淤血造成,表现为外周血血细胞计数基本在正常范围,亦是血细胞增殖和脾脏对血细胞的处理增加的共同作用的结果。抗凝治疗效果差,而应以预防食管曲张静脉破裂出血(EVB)为主。

血栓弹力图对肝硬化患者门静脉血栓形成的预测价值

目的探讨血栓弹力图(TEG)及常规凝血试验评价肝硬化患者门静脉血栓形成(PVT)的价值。方法选择2015年12月至2017年2月复旦大学附属中山医院消化科收治的肝硬化失代偿期患者,根据是否合并PVT分组。比较PVT组与非PVT组TEG参数、常规凝血指标、相关临床资料等的差异。结果共纳入199例患者,PVT组82例(41.2%)、无PVT组117例(58.8%)。两组患者TEG各项参数差异均无统计学意义。Logistic回归分析显示,女性(OR=3.061,95%CI 1.389~6.744,P=0.005)、脾切除史(OR=5.740,95%CI 1.876~17.568,P=0.002)、D-二聚体升高(OR=1.533,95%CI 1.182~1.989,P=0.001)是PVT的独立相关因素;Child B+C级(OR=12.844,95%CI 2.452~67.263,P=0.003)、R时间缩短(OR=0.526,95%CI0.294~0.942,P=0.031)、血小板增多(OR=1.013,95%CI 1.005~1.021,P=0.001)是门脉海绵样变的独立相关因素。结论TEG检测有助于预测PVT患者门脉海绵样变。

骨髓增生性疾病所致门静脉海绵样变性1例报告

1 病例资料患者男性,64岁,因“乏力1周,黑便3天”于2021年11月22日收入本院。患者于1周前无明显诱因下出现乏力,未重视未就诊,3天前进食辛辣食物后出现中上腹不适,后解黑色糊状便,每天2次,每次约200 mL,头晕乏力较前加重,无畏寒发热,无恶心呕吐,无呕血,无腹痛。患者20年前有胃溃疡伴出血病史,曾行胃镜检查明确,保守治疗后好转,曾因胃溃疡穿孔行修补手术治疗。既往否认慢性病史,否认肝炎、结核、吸烟嗜酒以及特殊服药史。

改良穿刺套件对肝硬化门静脉高压合并门静脉海绵样变上消化道大出血的疗效

目的探讨改良穿刺套件对肝硬化门静脉高压合并门静脉海绵样变(CTPV)急性上消化道大出血的临床疗效。方法收集2016年7月至2021年3月解放军联勤保障部队第九六〇医院收治的34例肝硬化门静脉高压合并CTPV急性上消化道大出血患者的临床资料,所有患者均行经颈静脉肝内门体分流术(TIPS),采用改良穿刺套件穿刺门静脉并植入直径均为8 mm的覆膜支架和裸支架,建立分流道后测量门静脉压力,观察患者的术中、术后情况,比较TIPS手术前后门静脉主干压力。通过随访复查彩色多普勒超声,观察患者的再出血、支架通畅性、肝性脑病及患者生存情况等。结果34例患者的手术成功率为94.1%(32/34),其中,32例患者均采用改良穿刺套件顺利完成手术。32例患者中,成功穿刺门静脉右支11例,成功穿刺门静脉左支21例,其余2例患者因门静脉完全闭塞而无法进行手术;24例CTPV患者均采用穿刺定位球囊的方法成功完成穿刺;25例患者同时行胃冠状静脉栓塞术,术中未出现手术相关并发症。TIPS术后门静脉主干压力明显低于术前,差异有统计学意义(P﹤0.01)。术后造影检查结果显示,分流道腔内血流通畅,侧支循环消失,24 h内消化道未再次出血,住院期间无患者死亡。术后3个月,32例患者均行彩色多普勒超声检查,分流道腔内血流通畅。术后6个月,1例患者失访,5例患者术后出现分流道狭窄或阻塞,支架狭窄程度为30%~50%,经球囊扩张治疗后支架内血流恢复通畅,其余26例患者术后分流道内血流通畅,临床疗效良好。结论采用改良穿刺套件治疗肝硬化门静脉高压合并CTPV所致的急性上消化道大出血的近期、中期疗效较好,安全可靠。

1例肝硬化门静脉高压合并门静脉海绵样变性病人行经肠系膜上静脉肝外门体分流术的术后护理

总结1例肝硬化门静脉高压合并门静脉海绵样变性病人行经肠系膜上静脉肝外门体分流术的术后护理经验。护理要点:腹部切口及颈部穿刺点的护理;抗凝管理;管道护理;营养支持;预防血栓形成,加强皮肤护理;术后并发症的预防及观察。经过精心的护理,病人术后恢复良好,术后28 d出院。

肝移植治疗小儿先天性门静脉海绵样变

目的探讨肝移植治疗小儿先天性门静脉海绵样变的临床效果。方法2例患儿经CT和CTA检查诊断为肝内门静脉系统海绵样变,并伴有严重的门静脉高压症,分别于2004年12月和2005年6月施行肝移植术治疗。从临床症状、血液学指标和影像学检查方面对手术疗效进行评价。结果2例患儿术后消化道出血症状均消失,且无复发;血红蛋白、血球压积、血小板水平等均接近或恢复正常;脾脏亦有一定程度缩小;1例患儿随访至今,情况良好;另1例患儿术后失访2月,3月后超声复查发现大量腹水,门静脉吻合口明显狭窄,再次手术重新植入另一供体门静脉,手术过程顺利,因术后出现严重肺部感染,同时合并肝、肾功能损害,于术后2周死亡。结论肝移植术可成为先天性门静脉海绵样变的有效治疗方法之一,其近期疗效良好。对肝移植的远期效果和并发症,尚需进一步积累病例和随访研究加以评价。

肝细胞癌门静脉癌栓致门静脉海绵样变性侧支循环的螺旋CT研究

目的 探讨肝细胞癌门静脉癌栓所致门静脉海绵样变性 (cavernoustransformationoftheportalvein ,CaTPV)侧支循环血管的构成及螺旋CT特征。资料与方法 搜集经组织病理学证实的肝细胞癌伴门静脉主干癌栓 5 7例进行回顾性分析 ,根据病理所见有无肝硬化背景分为肝硬化阳性组 (n =2 6 )和肝硬化阴性组 (n =31) ;另选择 15例肝硬化合并门静脉高压而无肝细胞癌和CaTPV的病例作为对照组。所有病例均行标准化上腹部螺旋CT双期增强扫描。观察CaTPV侧支血管的CT表现 ,对比分析各组螺旋CT对CaTPV侧支血管的显示率。结果 门静脉胆支(胆囊静脉和胆管周围静脉丛 )和胃支 (胃左、胃右静脉及属支 )是CaTPV最常显示的侧支血管。门静脉胆支的出现与CaTPV有关 ,而与肝硬化、门静脉高压无关 (CT显示率 81%～ 94%比 0 ) ;门静脉胃支的CT显示率 3组之间无差别 (胃左静脉 77%～ 87% ,胃右静脉 5 8%～ 6 0 % )。结论 门静脉胆支和胃支是构成CaTPV侧支循环的最主要的血管 ;胆支开放仅见于CaTPV ,而胃支开放与肝硬化并发门静脉高压以及CaTPV均有关系。

儿童门静脉海绵样变间接门静脉造影特点

目的探讨儿童门静脉海绵样变(CTPV)的间接门脉造影表现及其临床意义。方法回顾性分析了8例经超声诊断及造影证实的门静脉海绵样变患儿的间接门脉造影血管造影图像,观察其特征性的血管造影表现,并对其指导临床治疗的意义进行了评价。结果8例中6例清楚显示门脉主要分支近肝门处阻塞,2例门静脉主干阻塞,代之以肿块样纡曲血管并在其周围形成向肝内放射状的侧支血管影像,以及离肝性侧支循环静脉。8例血管造影均能对与分流或断流手术有关的血管解剖提供准确信息。结论儿童CTPV具有特征性的血管造影表现,间接门脉造影对CTPV的诊断和治疗有重要价值。