Hepaticoportal fistula and portal hypertension

Summary A large intrahepatic fistula between the hepatic artery and portal vein was detected at angiography in a patient with portal hypertension and bleeding esophageal varices. Hemodynamic studies demonstrated that increased vascular resistance in the liver rather than increased flow through the fistula was responsible for the portal hypertension and a portocaval shunt was performed. A repeat angiogram two months after the operation showed that the fistula had closed spontaneously.     

门静脉收缩器建立犬门静脉高压食管静脉曲张模型的实验研究

目的 探讨建立稳定的门脉高压食管静脉曲张犬模型的可行性.方法 12只杂交犬,采用门静脉.下腔静脉侧侧分流术+门静脉主干环置amemid收缩器+下腔静脉头侧双线交叉缝合结扎的方法制作动物模型.6周后,胃镜和门静脉造影检查观察食管曲张静脉形成情况.结果 造模手术前门静脉压力为(11.0 ±1.1)mm Hg,造模后门静脉压力升高至(22.9±1.2)mm as(P=0.010).胃镜发现所有的杂交犬均形成了轻-中度食管曲张静脉,门静脉造影检杳证实食管中下段有曲张静脉形成,同时腹壁形成了肉眼可见的曲张静脉.结论 门静脉收缩器法能成功地建立门脉高压食管静脉曲张动物模型.     

Effects of Esophageal Transection Combined with Splenectomy on Portal Hemodynamics

Portal hemodynamics were studied in 69 patients with cirrhosis and 29 patients with idiopathic portal hypertension to investigate the effects of an operative procedure for varices that consists of transabdominal esophageal mucosal transection, paraesophagogastric devascularization, pyloroplasty, and splenectomy. Portal venous flow measured by the pulsed Doppler flowmeter in 14 patients with cirrhosis and nine patients with idiopathic portal hypertension, who underwent operation 2-5 yr earlier, was significantly reduced compared with that in unoperated 49 patients with cirrhosis and 17 patients with idiopathic portal hypertension who had esophageal varices (410 +/- 158 versus 660 +/- 263 ml/min in cirrhosis; 443 +/- 185 versus 912 +/- 189 ml/min in idiopathic portal hypertension). In nine patients (six cirrhosis, three idiopathic portal hypertension), portal venous flow and portal vein pressure were measured before and after operation. In patients with cirrhosis, portal vein pressure did not change significantly postoperatively even though portal venous flow was reduced. In contrast, portal vein pressure decreased in two patients with idiopathic portal hypertension in whom portal venous flow was reduced. Portal vein pressure was elevated in one patient with idiopathic portal hypertension in whom portal venous flow was increased postoperatively as a result of resection of a large gastro- and splenorenal shunt conducted additionally.     

Portal hemodynamics in patients with gastric varices. A study in 230 patients with esophageal and/or gastric varices using portal vein catheterization

The hemodynamic features of gastric varices are not well documented. The purpose of this study was to investigate the nature of hepatofugal collateral veins, their origins, the direction of blood flow in the major veins and collateral veins, and portal venous pressure. To this end, 230 patients, mostly cirrhotic, who had esophageal or gastric varices, or both, demonstrated by endoscopy were investigated by portal vein catheterization. The findings were correlated with endoscopically assessed degrees of varices. Gastric varices were seen in 57% of the patients with varices due to portal hypertension. In most of the patients with advanced gastric varices, esophageal varices were minimal or absent. When patients with gastric varices were compared with those having predominantly esophageal varices, it was found that advanced gastric varices were more frequently supplied by the short and posterior gastric veins, they were almost always associated with large gastrorenal shunts, and portal venous pressure in patients with large gastric varices was lower. Chronic portal systemic encephalopathy was more common in patients with large gastric varices due to hepatofugal flow of superior mesenteric venous blood in the splenic vein than in patients with predominantly esophageal varices. Thus, the hemodynamics in patients with large gastric varices are distinctly different from those in patients with mainly esophageal varices, and such differences seem to account for the differing incidence of chronic encephalopathy and variceal bleeding.     

门脉高压性小肠病

小肠改变是门脉高压门静脉高压症是一组由门静脉压力持续增高引起的症候群,最常见表现是消化道出血,特别是食管胃静脉曲张破裂出血.随着小肠检查手段的发展,特别是胶囊内镜和双气囊小肠镜,发现门脉高压下小肠发生了病变,被定义为门脉高压性小肠病(PHE),这种改变也是消化道出血的重要原因.消化道出血的患者,胃或食管没有静脉曲张情况...     

Arterioportal Fistula: A Role for Pre-TIPSS Arteriography and Hepatic Venous Pressure Measurements

A 70-yr-old male presented with massive upper gastrointestinal bleeding secondary to esophageal varices. Because the bleeding was not controlled by sclerotherapy or vasopressin and nitroglycerin, the patient was evaluated for a transjugular intrahepatic portosystemic shunt. Preprocedure arteriography was performed because the etiology of the portal hypertension was uncertain. The arteriogram revealed a hepatic artery to portal vein fistula. Hepatic venous pressure measurements documented an elevated hepatic venous pressure gradient, which diminished dramatically upon embolization of the fistula. Rebleeding from the varices was associated with reestablishment of the fistula via collaterals and elevation of the hepatic venous pressure gradient. The case is presented to establish a role for arteriography prior to transjugular intrahepatic portosystemic shunting, especially in patients with unexplained portal hypertension, and to establish the potential value of hepatic venous pressure measurements in the treatment of arterioportal fistulas.     

晚期血吸虫病门脉高压症术后门静脉血栓形成的危险因素分析

目的探讨晚期血吸虫病门脉高压症脾切除贲门周围血管离断术后门静脉血栓(PVT)形成的危险因素。方法收集2004年8月至2014年3月期间本院外科收治的211例晚期血吸虫病门静脉高压症患者的临床资料,对可能影响术后PVT形成的因素进行单因素分析和Logistic回归分析。结果 211例患者中,59例术后PVT形成,发生率为27.96%(59/211)。单因素分析显示术前上消化道出血史、门静脉直径、脾静脉直径、食管静脉曲张程度、腹水、门脉高压性胃病、胃底静脉曲张、血氨水平是患者术后PVT形成的影响因素。Logistic回归分析显示门静脉直径增宽(OR=1.763,P=0.000)和门脉高压性胃病(OR=1.089,P=0.037)是患者术后PVT形成的独立危险因素。结论晚期血吸虫病门脉高压症术后PVT形成的发生率较高,门静脉直径增宽和门脉高压性胃病是PVT形成的独立危险因素。     

The epidemiology and pathogenesis of gastrointestinal varices

Gastrointestinal varices are a consequence of portal hypertension that can occur in the setting of cirrhosis or extrahepatic portal vein obstruction. Increased intrahepatic vascular resistance, a hyperdynamic circulation, and increased flow through the portal and collateral venous system lead to persistently elevated portal pressures that result in angiogenesis and formation of collaterals between the portal and systemic circulation. Despite this physiological attempt at decompression, portal hypertension persists as collateral vessels have higher resistance than the normal liver. Variceal wall tension is the main factor that determines vessel rupture and bleeding occurs when tension in the wall exceeds the limit of elasticity of the vessel. Progressive distension leads to increasing resistance to flow and hemorrhage ensues when the limits of resistance to further dilation are surpassed. Gastroesophageal varices are present in 50% of patients with cirrhosis and progress in size at a rate of 8%-10% per year. Hemorrhage occurs at a rate of approximately 12% per year and large esophageal varices carry a higher risk of rupture. Gastric varices occur in 20% of patients with portal hypertension and bleed less frequently, but more severely. Cardiofundal varices have a complex vascular anatomy that is important to consider as it pertains to the effectiveness of strategies used for management. Ectopic varices make up 2%-5% of all variceal bleeding, occur more frequently in patients with extrahepatic portal hypertension, and their identification should prompt assessment of the intra-abdominal vasculature. Varices in the setting of splenic vein thrombosis should be considered a distinct entity owing to their disparate etiologic basis and treatment approach.     

肝硬化门静脉高压患者胃镜与B超表现相关性研究

目的 通过对肝炎肝硬化门静脉高压患者胃镜、B超检测结果行相关性分析，为临床判断肝硬化程度、早期预防并发症提供参考。方法 选择肝炎肝硬化门静脉高压患者192例，根据食管静脉曲张程度分为轻、中、重度3组。比较胃底静脉曲张在各组中发生比例，并测量门静脉主干宽度、脾静脉宽度、脾脏厚度，研究其与食管静脉曲张程度相关性。结果 3组患者胃底静脉曲张发生比例存在显著差异，食管静脉曲张程度越重，伴胃底静脉曲张比例越高；随食管静脉曲张程度不同，门静脉主干内径、脾静脉内径及脾脏厚度之间存在差异，脾脏厚度与脾静脉内径之间存在直线相关关系。结论 门静脉主干内径、脾静脉内径、脾脏厚度可为判断门静脉高压提供参考，综合上述3点并结合胃镜检查结果可较准确判断有无食管静脉曲张及程度。     

Non-invasive diagnosis of portal hypertension in cirrhosis. Application to the primary prevention of varices

One of the major complications of cirrhosis is the occurrence of portal hypertension and esophageal varices. At present, universal endoscopic screening of esophageal varices is recommended in association to primary prophylaxis in patients at high risk of variceal bleeding. But this screening is invasive and could be not cost-effective. Besides, pre-primary phrophylaxis is not effective and hampared by side effects. So, non invasive diagnosis of portal hypertension might be useful. This one could depend on non invasive measurement of hepatic venous pressure gradient, but its application to screening is not well-documented and its use in treatment monitoring is debated. A second way could be non invasive diagnosis of large esophageal varices because of prognostic and economic issues. Indirect echographic markers of portal hypertension and esophageal varices (ascites, portal vein diameter > or = 13 mm, spleen length, maximal and mean velocimetry of portal vein flow, respectively < 20 cm/s and < 12 cm/s) could be useful. Among this parameters, spleen length is an independent predictive marker of esophageal varices. Besides, several direct or indirect blood markers of fibrosis have been tested. Platelet count is repeatedly a predictive marker of esophageal varices in multivariate analysis. The other predictive factors of esophageal varices could be: prothrombin time, splenomegaly, spider naevi, Child-Pugh class, bilirubinemia, platelet count/spleen diameter ratio and Fibrotest, but these data require validation. In summary, in regard to actual results, non invasive diagnosis of portal hypertension might be useful in esophageal varices screening, but the substitutes to endoscopy have limited place actually in clinical practice, and exclusive non invasive diagnosis of portal hypertension is not applicable; the only test that seems to be useful in clinical practice is conventional endoscopy awaiting the results of videocapsule.     

螺旋CT门静脉造影对门脉高压侧支循环的研究

目的探讨多排螺旋CT门静脉造影(CT portal venography,CTPV)显示肝硬化门脉高压侧支循环血管的临床应用价值。方法对92例肝硬化门脉高压的患者分别进行CT门脉造影,获得门脉侧支循环血管的清晰图像,测量门静脉主干和胃左静脉直径,将胃镜与CT门静脉造影两种技术进行比较。结果应用CT门静脉造影能清晰显示和测量门脉侧支循环的血管。CT门静脉造影与胃镜两种方法对食管和胃底曲张静脉的显示能力具有一致性,Kappa值分别为0.502和0.478。结论应用多排螺旋CT门静脉造影能很好显示和测量门体间侧支循环血管。联合应用多排螺旋CT门静脉造影与胃镜两种方法,对于肝硬化门静脉高压患者的诊断、病情判断和估计预后有帮助。     

Experimental prehepatic portal hypertension and esophageal varices in the rabbit

Prehepatic portal hypertension was induced in rabbits by constricting the vein, creating a pressure approximately 100% higher than the preoperative value. Angiographic, endoscopic, and pressure studies confirmed the portal hypertension to be decompressed, with shunting of blood through esophageal varices. Small periportal collaterals were insufficient to prevent the esophageal shunting. The esophageal varices developed within 4 weeks and were a consistent finding throughout the observation period. No relation between the extent of portal hypertension and the size of the varices was found.     

Portal vein thrombosis following sclerotherapy.

We present the case of a woman with idiopathic portal hypertension who underwent sclerotherapy for bleeding esophageal varices. She had a rebleed 27 months after complete eradication of esophageal varices. Endoscopy showed bleeding gastric varices. Ultrasonography, and later splenoportography, revealed a large thrombus in the right branch of the portal vein causing gross dilation of the portal and splenic vein. A proximal splenorenal shunt was done to decompress the portal system and hence gastric varices. Repeat endoscopy 4 weeks after surgery revealed complete disappearance of the gastric varices, while ultrasonography at 38 weeks showed marked decompression of the portal system with complete disappearance of the thrombus from the right branch of the portal vein. No new thrombus formation was seen.     

CURATIVE TREATMENT OF BLEEDING ESOPHAGEAL VARICES SECONDARY TO A SPLENIC ARTERIO-VENOUS FISTULA

A 59 year old female, who presented with abdominal pain, diarrhea, and ascites, developed major bleeding from esophageal varices. Celiac angiography demonstrated a splenic arterio-venous fistula with early filling of an enlarged splenic vein and esophageal varices (pre-sinusoidal extra hepatic portal hypertension). The patient underwent splenectomy and resection of the fistula with resultant disappearance of the varices and presenting symptoms.     

Is portal vein cavernous transformation a component of congenital hepatic fibrosis？

Congenital hepatic fibrosis （CHF） is an autosomal recessive disorder that belongs to the family of fibropolycystic liver diseases. This family includes a spectrum of disorders which are usually found in combination with each other and are usually inherited. Clinically fibropolycystic diseases have three effects being present in different proportions, those of a space occupying lesion, of portal hypertension and of cholangitis. In most patients, the first manifestations of CHF are signs and symptoms related to portal hypertension such as splenomegaly and varices. Portal hypertension in these patients has been attributed to the hypoplasia or compression of the portal vein radicles in the fibrous bands. Cavernous transformation of the portal vein （CTPV） is a relatively rare condition resulting from extrahepatic portal vein obstruction with recanalization or collateral vein formation to bypass the obstruction. It has been found that patients with CHF having an accompanying CTPV have relatively large splenomegaly and suffers more frequent episodes of bleeding from esophageal varices.We believe that CTPV is a congenital component of CHF and also one of the important causative factors of portal hypertension in these patients.     

Utility of endoscopic ultrasound in patients with portal hypertension

Endoscopic ultrasound(EUS) has revolutionized the diagnostic and therapeutic approach to patients with gastrointestinal disorders. Its application in patients with liver disease and portal hypertension is increasing. Patients with chronic liver disease are at risk for development of portal hypertension sequale such as ascites, spontaneous bacterial peritonitis and gastroesophageal varices. Bleeding esophageal and gastric varices are among the most common causes of mortality in patients with cirrhosis. Thus, early detection and treatment improve the outcome in this population. EUS can improve the detection and diagnosis of gastroesophageal varices and collateral veins and can provide endoscopic therapy of gastroesophageal varices such as EUS-guided sclerotherapy of esophageal collateral vessels and EUS-guided cynoacrylate(Glue) injection of gastric varices. EUS can also provide knowledge on the efficacy of pharmacotherapy of portal hypertension. Furthermore, EUS can provide assessment and prediction of variceal recurrence after endoscopic therapy and assessment of portal hemodynamics such as E-Flow and Doppler study of the azygous and portal veins. Moreover, EUS-guided fine needle aspiration may provide cytologic diagnosis of focal hepatic tumors andanalysis of free abdominal fluid.Using specialized EUSguided needle biopsy,a sample of liver tissue can be obtained to diagnose and evaluate for chronic liver disease.EUS-guided fine needle injection can be used to study portal vein pressure and hemodynamics,and potentially could be used to assist in exact measurement of portal vein pressure and placement of intrahepatic portosystemic shunt.     

Splenectomy with endoscopic variceal ligation is superior to splenectomy with pericardial devascularization in treatment of portal hypertension

INTRODUCTION Recurrent bleeding occurs in over 70% of portal hypertension patients with a variceal bleeding history[1]. It is a general consensus that all patients with a variceal bleeding history should accept further treatment to prevent re-bleeding. Su…     

Splenic arteriovenous fistula and sudden onset of portal hypertension as complications of a ruptured splenic artery aneurysm： Successful treatment with transcatheter arterial embolization. A case study and review of the literature

Splenic arteriovenous fistula (SAVF) accounts for an unusual but well-documented treatable cause of portal hypertension. A case of a 50-year-old multiparous female who developed suddenly portal hypertension due to SAVF formation is presented. The patient suffered from repeated episodes of haematemesis and melaena during the past twelve days and thus was emergently admitted to hospital for management. Clinical and laboratory investigations established the diagnosis of portal hypertension in the absence of liver parenchymal disease. Endoscopy revealed multiple esophageal bleeding varices. Abdominal computed tomography (CT) and transfemoral celiac arteriography documented the presence of a tortuous and aneurysmatic splenic artery and premature filling of an enlarged splenic vein, findings highly suggestive of an SAVF. The aforementioned vascular abnormality was successfully treated with percutaneous transcatheter embolization. Neither recurrence nor other complications were observed.     

Portal hypertensive gastropathy with portal thrombosis successfully treated with partial splenic embolization

A 60-year-old man with alcoholic liver cirrhosis was admitted to our hospital with severe anemia and tarry stool. Upper gastrointestinal endoscopy revealed grade 4 esophageal varices without bleeding and severe portal hypertensive gastropathy (PHG) of the fornix of the stomach with oozing. These findings suggested that PHG was the cause of progression of anemia. Abdominal computed tomography demonstrated no enhancement of the main portal vein and its first branches, indicating portal thrombosis and cavernous transformation. The patient underwent partial splenic embolization (PSE) to reduce portal hypertension. Two months after PSE was performed, upper gastrointestinal endoscopy showed improvement of PHG and endoscopic variceal ligation was performed to treat the esophageal varices. Contrast-enhanced CT revealed partial enhancement of the main portal vein indicating improvement of portal thrombosis. One year after PSE, hemoglobin had increased from 6.0 to 11.0 g/dl without blood transfusion. Moreover, albumin level had risen from 2.8 to 3.7 g/dl, cholinesterase from 51 to 150 IU/l, and prothrombin time from 47% to 66%. PSE can be an effective alternative for the management of severe PHG with portal vein thrombosis, and it might also be effective in improving liver function.     

Endoscopic, portographic, and hemodynamic evaluation of prolonged propranolol administration in pigs with experimental portal hypertension and esophageal varices

The effect of long-term propranolol administration on esophageal varices, portocollateral shunting, portal pressure, hepatosplanchnic hemodynamics, and liver function was studied in a pig model with experimentally induced prehepatic portal hypertension and esophageal varices. Five pigs were treated with 160 mg propranolol daily from week 5 to week 24 after portal-vein banding, and five pigs served as nontreated controls. Administration of propranolol caused an initial, significant reduction (20%) of portal venous pressure, followed by a gradual increase to levels not different from control pressures. In contrast, a marked reduction of the caliber of the coronary vein and size of the esophageal varices was noticed. Twenty weeks of propranolol treatment did not change liver blood flow or liver function. We conclude that the size of the varices rather than portal venous pressure depicts the effect of propranolol treatment and suggest that the beneficial effect of propranolol on variceal bleeding can be explained by a reduction in the wall tension of the varices, initiated and maintained by a diminution of splanchnic blood flow.