Extrahepatic portal vein aneurysm： Two case reports of surgical intervention

We report two cases of extrahepatic portal vein aneurysm, and both of them underwent surgical intervention. The first case had a mild pain in right upper quadrant of the abdomen; the second had no obvious symptoms. Physical examination revealed nothing abnormal. Both of them were diagnosed by magnetic resonance imaging angiography (MRA). One of the aneurysms was located at the main portal vein, the other, at the confluence of the superior mesenteric vein and the splenic vein, and these two places are exactly the most common locations of the extrahepatic portal vein aneurysm reported in the literature (30.7% each site). The first case underwent aneurysmorrhaphy and the second case, aneurysm resection with splene-ctomy. Both of them recovered soon after the operation, and the symptom of the first case was greatly alleviated. During the follow-up of half a year, no complication and adverse effect of surgical intervention was found and the color Doppler ultrasonography revealed no recurrence of the aneurysmal dilation. We suggest that surgical intervention can alleviate the symptom of the extrahepatic portal vein aneurysm and prevent its complications effectively and safely for low risk patients.     

Splenic-intrahepatic left portal shunt in an adult patient with extrahepatic portal vein obstruction without recurrence after pancreaticoduodenectomy

In the last decade, a superior mesenteric-intrahepatic left portal shunt (Rex shunt) has been reported for successful management of extrahepatic portal vein obstruction in children. However, in adults, a mesocaval shunt has been generally performed for the surgical management of extrahepatic portal vein obstruction because of the complexity of the underlying disease and the difficulty of the superior mesenteric-intrahepatic left portal shunt. We herein report an adult patient who was successfully treated by splenic-intrahepatic left portal shunt with an artificial graft (6-mm polytetrafluoroethylene) for complete obstruction of the extrahepatic portal vein following pancreaticoduodenectomy. The shunt procedure not only relieved portal hypertension but also restored hepatic portal flow. In the near future, the Rex shunt should be considered for a beneficial management of extrahepatic portal vein obstruction, even in adults.     

Extrahepatic portal vein obstruction in adults detected by ultrasound with frequent lack of portal hypertension signs

Extrahepatic portal vein obstruction (EHPO) was seen in 54 adult patients at the Chiba University Hospital and affiliated hospitals from 1978 to 1991. They were classified according to the background disease (Group A, unknown aetiology; Group B, benign disease; Group C, malignant disease). Among the initial symptoms and signs, abdominal pain was the most frequent in Group A (37%), and symptoms attributable to the primary disease in Groups B (44%) and C (75%). Definite or probable diagnosis was made in 45 of the 54 patients (81.8%) by ultrasound (US) examination carried out because of these symptoms and signs. Signs of portal hypertension were observed in 67% of patients; oesophageal varices were seen in 60%. Extrahepatic portal vein obstruction without portal hypertension signs was characterized by thick extensive hepatopetal collaterals or patency of some intrahepatic portal veins. Extrahepatic portal vein obstruction patients without portal hypertension remained free of its signs for more than 3 years of follow up and, in fact, EHPO without portal hypertension signs was a common occurrence. Emphasis is made on the diagnostic value of US examination which was useful in identifying the relation of clinical manifestation of EHPO to pathophysiology, and on the frequent lack of portal hypertension signs in this disease.     

肝硬化非常见侧支循环临床特点研究

目的 探讨肝硬化患者门体循环之间非常见侧支循环形成的临床特点及意义。方法 对临床确诊为肝硬化的患者运用64排螺旋CT和三维血管成像结合电子胃镜检查,观察其门体循环之间非常见侧支循环的形成。结果 ①700例肝硬化患者中118例(16.86%)存在非常见侧支循环,依次为脾肾静脉分流、胃肾静脉分流、椎旁静脉分流、腹膜后静脉分流、胃脾分流和心膈角静脉分流。②非常见侧支循环形成与肝硬化Child-Pugh分级相关(P<0.01)。③与常见侧支循环形成组比较,非常见侧支循环组较少出现重度食管和(或)胃底静脉曲张、重度门静脉高压性胃病及大量腹水(P<0.01)。④非常见侧支循环组中肝性脑病和慢性血氨升高的发生率高于常见侧支循环组(P<0.01)。结论 ①肝硬化患者中非常见侧支循环并不"非常见";②非常见侧支循环形成与肝功能Child-Pugh分级有关;③非常见侧支循环形成可缓解门静脉高压引起的相关并发症,但增大了肝性脑病和慢性血氨升高的发病率。     

MRA对门静脉和下腔静脉系统疾病的诊断价值

应用磁共振血管造影行门静脉和下腔静脉检查４０例、４３例次,包括三维相位对比３０次,动态增强１３次。结果表明：ＭＲＡ能较好地同时显示门静脉和下腔静脉,在门静脉和下腔静脉系统多种疾病的显示和诊断方面具有较高使用价值。     

Reconstruction of the main portal vein for a large saccular aneurysm

BackgroundA large aneurysm of the main portal vein is rare, and the appropriate surgical procedure is uncertain. Reconstruction of a main portal vein affected by a large saccular aneurysm is described.Case outlineAbdominal pain led to the diagnosis of a large saccular aneurysm of the main portal vein in a 58-year-old woman who had undergone cholecystectomy 10 years earlier. At laparotomy a dorsolateral approach to the hepatoduodenal ligament was performed with no attempt at extensive separate exposure of the anatomical structures in the hepatoduodenal ligament, so as to avoid the devascularisation of the common hepatic duct and additional weakening of the portal vein wall. The aneurysm was longitudinally incised, and the portal vein was reconstructed from the walls of the aneurysm with a longitudinal running suture. The rest of the aneurysmal wall was wrapped around the portal vein, leaving it normal in size and contour. Recovery was uneventful. Follow-up CT scan showed a patent portal vein in the region of the former aneurysm.DiscussionLarge saccular aneurysms can rupture, bleed and cause death. The potential hazards of manipulation of large portal vein aneurysms are negligible in comparison with the possible complications of the aneurysm itself. In our opinion the ease with which the main portal vein was dissected and reconstructed make an elective operation in such cases a reasonable approach.     

Insight into congenital absence of the portal vein： Is it rare？

Congenital absence of portal vein （CAPV） was a rare event in the past. However, the number of detected CAPV cases has increased in recent years because of advances in imaging techniques. Patients with CAPV present with portal hypertension （PH） or portosystemic encephalopathy （PSE）, but these conditions rarely occur until the patients grow up or become old. The patients usually visit doctors for the complications of venous shunts, hepatic or cardiac abnormalities detected by ultrasonography （US）, computed tomography （CT） and magnetic resonance imaging （MR1）. The etiology of this disease is not clear, but most investigators consider that it is associated with abnormal embryologic development of the portal vein. Usually, surgical intervention can relieve the symptoms and prevent occurrence of complications in CAPV patients. Moreover, its management should be stressed on a case-by-case basis, depending on the type or anatomy of the disease, as well as the symptoms and clinical conditions of the patient.     

多层螺旋CT门静脉血管成像在胰源性门脉高压症诊断中的应用

目的 研究多层螺旋CT门静脉血管成像在胰源性门静脉高压患者诊断中的应用.方法 应用16排多层螺旋CT门静脉血管成像,对47例临床怀疑胰腺体尾部病变的患者的门静脉系统形态改变与126例肝源性门脉高压患者和47例正常对照组进行形态学对比观察,并测量胃冠状静脉、门静脉、脾静脉、肠系膜上静脉内径、门静脉期肝实质和门静脉主干CT值,对比肝脏、脾脏体积.结果 在47例胰腺体尾部病变中发现有脾静脉狭窄、闭塞者38例,其中胰腺肿瘤患者27例(71.1%),急慢性胰腺炎患者11例(28.9%).38例胰源性门脉高压患者中,发现食管静脉曲张5例(13.2%),胃底静脉曲张25例(65.8%),胃体静脉曲张22例(57.9%),胃短-胃后静脉显示26例(68.4%),胃冠状静脉显示26例(68.4%),发现胃网膜静脉曲张24例(63.2%),肠系膜静脉曲张1例.脾静脉闭塞14例(36.8%),脾静脉狭窄23例(63.2%).结论 胰源性门脉高压在影像学上表现为脾静脉栓塞,脾脏增大,脾门处大量曲张静脉,胃后-胃短静脉及胃网膜静脉增粗迂曲,胃底和胃体静脉曲张,较少合并食管静脉曲张,肝脏形态大小亦无异常.多层螺旋CT门静脉血管成像检查可为胰源性门脉高压患者提供血管形态、病因诊断等多方面有价值信息,为临床诊断和治疗提供客观的影像学依据.     

Left-sided gallbladder： Its clinical significance and imaging presentations

AIM： To assess the importance of preoperative diagnosis and presentation of left-sided gallbladder using ultrasound （US）, CT and angiography.
METHODS： Retrospective review of 1482 patients who underwent enhanced CT scanning was performed. Left-sided gallbladder was diagnosed if a right-sided ligamentum teres was present. The image presentations on US, CT and angiography were also reviewed.
RESULTS： Left-sided gallbladder was diagnosed in nine patients. The associated abnormalities on CT imaging included portal vein anomalies, absence of umbilical portion of the portal vein in the left lobe of the liver, club-shaped portal vein in the right lobe of the liver, and difficulty in identifying segment Ⅳ. Angiography in six of nine patients demonstrated abnormal portal venous system （trifurcation type in four of six patients）. The main hepatic arteries followed the portal veins in all six patients. The segment Ⅳ artery was identified in four of six patients using angiography, although segment Ⅳ was difficult to define on CT imaging. Hepatectomy was performed in three patients with concomitant liver tumor and the diagnosis of left-sided gallbladder was confirmed intraoperatively.
CONCLUSION： Left-sided gallbladder is an important clinical entity in hepatectomy due to its associated portal venous and biliary anomalies. It should be considered in US, CT and angiography images that demonstrate no definite segment Ⅳ ,absence of umbilical portion of the portal vein in the left lobe, and club-shaped right anterior portal vein.     

肝前性门静脉高压症：9年诊治经验

目的探讨肝前性门脉高压综合征（PPH）诊断、临床特征、治疗方法的选择和疗效观察。方法回顾分析PPH40例患者的临床资料,依照间接门静脉造影和/或门静脉系统CT血管成像,全部确诊为PPH。施肠系膜上静脉-下腔静脉转流术31例,脾切除、脾静脉-肾静脉转流术4例,门静脉-下腔静脉转流术1例,肠系膜上静脉门静脉探查术、门奇静脉断流术2例,未行手术2例,仅给与保肝等内科治疗。结果 39例术后随访3个月～8年,1例未手术患者失访。36例经肠腔、脾肾和门腔转流手术治疗的患者术后脾亢消失,未再发生上消化道出血;2例断流手术患者,术后8月1例死亡,2例保守治疗1例死亡。结论间接门静脉系造影或CT门静脉系血管成像检查是诊断PPH的标准方法,分流手术安全有效。     

Surgical management of intraoperatively diagnosed portal annular pancreas: Two case reports

Rationale:Portal annular pancreas (PAP) is a rare pancreatic anomaly characterized by portal vein encasement in the pancreatic parenchyma. Due to its rarity, PAP may often be missed on preoperative computed tomography (CT) review, and surgeons may face challenges in dealing with an unexpected intraoperative encounter with PAP. We documented 2 such intraoperatively diagnosed cases and illustrated their surgical management.Patients’ concerns:In case 1, a 70-year-old man was found to have a 15-mm mass in the pancreatic body and dilatation of the peripheral main pancreatic duct on enhanced CT. Case 2 involved a 46-year-old woman with a history of familial adenomatous polyposis, and rectal cancer with a mass in the duodenal papilla.Diagnoses:The patient in case 1 was diagnosed with resectable pancreatic cancer. In case 2, the patient was diagnosed with duodenal papillary carcinoma.Interventions:In case 1, the patient underwent distal pancreatectomy with lymph node dissection. In case 2, the patient underwent pancreaticoduodenectomy. Intraoperatively, PAP was observed in both cases. In case 1, after the usual transection at the right border of the portal vein, an additional dissection was performed on the dorsal pancreas using a powered linear stapler. In case 2, an additional section was made in the pancreatic body caudal to the cricoid pancreatic junction so that the pancreatic cross-section was oriented in 1 plane.Outcomes:The patient in case 1 was discharged without complications. In case 2, although the patient had a grade-B pancreatic fistula (International Study Group of Pancreatic Fistula Classification), the patient recovered conservatively and was discharged without significant complications. In both cases, a retrospective review identified PAP in patients’ preoperative CT images.Lessons:Both cases required ingenuity during pancreatectomy. Awareness about PAP and its management will enable surgeons to prepare for unexpected encounters with the condition. Moreover, surgeons (especially pancreatic surgeons) should consider the possibility of PAP while managing pancreatic anomalies to make appropriate treatment decisions.     

In vivo assessment of the hepatic microcirculation after mesenterico‐portal bypass (REX‐shunt) using orthogonal polarization spectral imaging

Background: Extrahepatic portal vein thrombosis, not associated with cirrhosis or tumours, is the second most frequent cause of portal hypertension worldwide. Especially in children, anatomic mesenterico‐portal interposition (REX‐shunt) has become an established treatment. The changes in hepatic microcirculation after reperfusion of the shunt have not been investigated so far. Aims: This study investigates the hepatic microcirculation before and after REX‐shunt interposition using orthogonal polarization spectral imaging (OPS). Patients and methods: Since 2004, three consecutive patients with extrahepatic portal vein thrombosis underwent REX‐shunt interposition. We measured the hepatic microcirculation by OPS before and directly after REX‐shunt reperfusion and analysed the capillary vessel diameter, red blood cell velocity, functional capillary density and volumetric blood flow. Furthermore, we compared our values with the physiological values of the hepatic microcirculation defined previously by other investigators. Results: All shunts showed an excellent function in the follow‐up investigations. The intra‐individual microcirculatory analysis revealed a reduction in the red blood cell velocity after shunt reperfusion in particular. Conclusions: Our results provide preliminary evidence for the reversal of the hepatic arterial buffer response following the restoration of the portal venous blood flow. This may be a short‐term effect because of the restored portal venous blood flow.     

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.     

门脉CTA及内镜对胃静脉曲张诊断及疗效评估的比较研究

目的探讨多层螺旋CT血管造影（门脉CTA，简称CTPA）在诊断门脉高压胃静脉曲张及内镜下胃静脉曲张黏合剂联合硬化剂治疗疗效评估中的作用。方法对20例内镜下证实存在胃静脉曲张的患者予以黏合剂联合硬化剂治疗，治疗前后分别进行多层螺旋CT血管门脉造影检查。结果20例门脉CTA检查均发现胃静脉曲张。GOV-型门脉CTA示血流来源以胃左静脉为主，GOV-2型则由胃左静脉、胃短／胃后静脉混合供血，IGV-型以胃短／胃后静脉供血为主，此型分流发生率较高。经内镜下黏合剂联合硬化剂治疗，孤立性瘤状胃静脉曲张（IGV-型）10例，总体有效率为90．00％，而非孤立性瘤状胃静脉曲张（GOV-型和GOV-型）9例，总体有效率为44．45％。结论门脉CTA能准确、直观、立体地显示血管解剖特点，为门脉高压胃静脉曲张的诊断、治疗方案的选择提供重要依据，并且可作为评估内镜下胃静脉曲张黏合剂联合硬化剂治疗疗效的一种重要手段。     

Porto-hepatic venous shunt via portal vein aneurysm with splenomegaly

A 59-year-old woman presented with consciousness disturbance with flapping tremor. Laboratory examinations revealed normal liver functions, and imaging studies, ultrasonography, magnetic resonance imaging, and angiography, showed portal vein aneurysm communicating from the portal vein to the hepatic vein, splenomegaly, and splenic artery aneurysm. These examinations confirmed porto-systemic shunt leading to hepatic encephalopathy. Porto-hepatic venous shunt via portal vein aneurysm is extremely rare, and there are few reports that it causes encephalopathy; therefore, little is known about the entity and this association. The literature in English is reviewed and the etiology, clinical features, and prognosis discussed.     

合并门静脉栓塞的肝移植受体术前诊断与术式研究

肝移植受体并门静脉栓塞原因通常分为门静脉血栓（PVT）和门静脉癌栓（PVTT）。术前准确诊断评价门静脉系统,对鉴别门静脉栓子性质及肝移植手术方式指导意义重大。本文对当前合并门静脉栓塞肝移植受体的术前诊断方法及伴PVT肝移植术中重建门静脉的术式进展作一简要综述。     

216例肝硬化门静脉高压症患者CT血管成像门静脉侧支血管表现研究

目的探讨肝硬化患者CT 门静脉血管成像中门静脉侧支血管表现,为临床诊断提供依据。方法回顾性研究2013年1月~2014 年1月本院收治的 216 例临床诊断为肝硬化门静脉高压症患者的临床和CT检查资料,针对患者CT门静脉血管成像和门静脉侧支血管三维重建图像进行分析。结果216例患者中,肝硬化门体分流侧支血管的分布、走行及解剖毗邻关系在CT 门静脉血管成像图像上都能得到良好、直观的显示,其中胃左静脉曲张者172例（79.63%）,食管下段静脉曲张者100例（46.30%）,食管旁静脉曲张者 51例（23.61%）,胃/脾肾静脉分流者50例（23.15%）,附脐静脉及腹壁静脉曲张者36例（16.67%）;胃/脾肾静脉分流患者门静脉和脾静脉直径分别为（12.64±1.12） mm和（18.72±3.48） mm,与无分流患者比较有统计学差异[分别为（19.56±5.64） mm和（13.47±2.35）mm,P<0.05]。结论对肝硬化门脉高压患者行CT 门静脉血管成像检查能够对患者侧支循环的部位、严重程度等进行观察,并作出准确的判断。     

Power Doppler ultrasonography for the assessment of vascular invasion by pancreatic cancer

Aim: To investigate the diagnostic accuracy of power Doppler ultrasonography (US) in assessing the vascular invasion by pancreatic cancer. Methods: A prospective study of 40 consecutive patients with pancreatic cancer (head 35, body 5) was performed. All patients underwent surgery. The relationships between tumor and each vessel were classified into four types according to the closest circumferential contact of the tumor with the vessel. A type 0 indicated no contact; type 1 indicated less than one third contact; type 2 indicated one third to 99% contact, and type 3 indicated encasement. Vascular invasion was diagnosed in types 2 and 3. The diagnostic accuracy was evaluated in the portal vein and in the splanchnic arteries (celiac artery, common hepatic artery, and superior mesenteric artery). The power Doppler US findings were confirmed by the operative findings. The results of power Doppler US were compared with those of CT scan and angiography. Results: Portal vein invasion was confirmed in resected specimens in 23 cases and by operative findings in 5 cases. For the diagnosis of portal vein invasion, sensitivity, specificity, and overall accuracy of power Doppler US were, respectively, 79.3, 90.9, and 82.5%. The respective values were 79.3, 100, and 85% for CT and 72.4, 81.8, and 75% for angiography. For the diagnosis of arterial invasion, sensitivity, specificity, and overall accuracy of power Doppler US were 80, 92, and 90%, respectively. The corresponding values were 47, 88, and 73% for CT and 47, 100, and 80% for angiography. Conclusion: Power Doppler US proved to be useful for the diagnosis of vascular invasion by pancreatic cancer.     

Ultrasonography in portal hypertension: A sensitive noninvasive test to demonstrate portal-vascular anatomy

The accuracy of ultrasonography (US) in delineating the portal vascular anatomy was assessed clinically by the clinician in 30 cases of portal hypertension due to noncirrhotic portal fibrosis and extra hepatic portal venous obstruction. Ultrasonography detected portal vein block in 19 and in 11 patients it was found to be patent. These ultrasonic diagnoses were confirmed by spleno-portovenography (SPV) in all, except in 2 cases due to technical failure. Ultrasononic assessment of the splenic vein was found to be accurate in 93.3% (28/30) of cases. SPV also had similar accuracy of splenic vein assessment when compared with the surgical findings. In one patient, intraperitoneal haemorrhage was encounted following SPV, necessitating emergency surgery. Thus, US was found to be as accurate as splenoportovenography in the assessment of portal vascular anatomy. The imaging technique is cheap, easy, safe, and can be repeated as often as necessary. It should be the procedure of choice in assessing the anatomy of portal vascular system.     

Is congenital hepatic fibrosis a pure liver disease?

OBJECTIVES: An association between congenital hepatic fibrosis (CHF) and several different conditions is being increasingly recognized. We aimed to investigate, prospectively, these associated disorders and the clinical consequences for patients with CHF. MATERIALS AND METHODS: CHF was diagnosed using liver biopsy, abdominal ultrasound (US), Doppler US, upper endoscopy, and abdominal computed tomography (CT) in 19 patients (13 women, 6 men). CT portography and splenoportography with digital subtraction angiography were performed if indicated. Endoscopic retrograde cholangiopancreatography (ERCP) was performed to investigate the extent of portal vein involvement of the common bile duct if it existed, to remove a stone located in the common bile duct when documented, and to confirm the diagnosis of Caroli's syndrome. Cranial MRI was done when clinical findings suggested brain involvement. RESULTS: The mean age of the patients was 29.47+/-12.06, ranging from 13 to 57. CHF-associated diseases were Caroli's syndrome, polycystic kidney disease, cavernous transformation of the portal vein, Joubert's syndrome, von Meyenburg complex, polydactyly, medullary sponge kidney, and pancreatic duct atrophy. In two cases, cholangiocarcinoma had developed. There was only one case with pure CHF. Portosystemic shunt, TIPS, or splenectomy were performed in some cases to control bleeding from esophageal varices. Papillotomy and stone extraction from the common bile duct were performed in four patients with Caroli's syndrome complicated by cholangitis. Three patients died of complications of CHF. Two patients with Caroli's syndrome underwent liver transplantation. CONCLUSION: In this prospective study, it seems that CHF is not a pure liver disease but rather a multiorgan disorder involving the brain, portal vein, kidneys, and bile ducts. In most cases, the clinical picture includes other organ involvement, rather than purely the liver parenchyma.