Variceal bleeding and portal hypertension: much to learn, much to explore

The newer diagnostic and therapeutic options continue to evolve and important developments have been made in the field of variceal bleeding and portal hypertension. A meeting was held at Baveno to update consensus on different terminologies in relation to portal hypertension. beta-blockers continue to be the mainstay for primary prophylaxis of variceal bleeding, and endoscopic variceal ligation (EVL) is fast emerging as a strong contender. The role of vasoactive drugs in the management of variceal bleeding was assessed. Octreotide and terlipressin were shown to be as effective as sclerotherapy in achieving initial hemostasis, and octreotide was shown to be safe and efficacious in the prevention of rebleeding. EVL was superior to endoscopic sclerotherapy (EST) for obliteration of esophageal varices. Sequential and simultaneous ligation and sclerotherapy were more effective than ligation alone, in reducing the recurrence rate after variceal obliteration. For gastric varices, cyanoacrylate glue continues to be the first line of treatment, and band ligation is being assessed further. Bleeding ectopic varices were dealt by appropriate endoscopic means. Endosonography has developed strongly in the assessment of variceal eradication and prediction of variceal recurrence. Transjugular intrahepatic portosystemic shunting (TIPS) significantly reduces rebleeding rates compared to EVL.     

Portal hypertension

Portal hypertension is a frequent syndrome characterized by a chronic increase in portal venous pressure and by the formation of portal-systemic collaterals. Its main consequence is massive bleeding from ruptured esophageal and gastric varices. Bleeding is promoted by increased portal and variceal pressure, and is favored by dilatation of the varices. The evaluation of the portal hypertensive patient should include the assessment of portal vein patency by ultrasonography, endoscopic evaluation of the presence, size, and extent of esophageal varices, and hemodynamic studies with measurements of portal pressure and of portal-collateral blood flow. The preferred techniques are hepatic vein catheterization and measurement of azygos blood flow. Endoscopic measurements of variceal pressure and estimations of portal blood velocity by the Doppler technique have recently been introduced, but are still research procedures. Acute variceal hemorrhage should be treated under intensive care. Specific therapy to arrest variceal bleeding includes balloon tamponade, vasopressin, somatostatin, sclerotherapy, and emergency surgery. Treatment of portal hypertension is aimed at preventing variceal hemorrhage and bleeding-related deaths. Pharmacologic prophylaxis is based on the use of drugs that cause a sustained reduction in portal pressure; most studies have used propranolol. Surgery and endoscopic sclerotherapy can also be used to prevent rebleeding.     

Variceal bleeding and portal hypertension

Within the short span of half a century, the treatment of variceal bleeding has become highly differentiated, with multiple treatment options. Pharmacological therapy with beta-blockers is well established for preventing the first variceal bleeding. The utility of adding a vasodilator to beta-blockers needs to be studied further. Octreotide is widely used as an adjuvant to standard endoscopic treatment to prevent variceal rebleeding, and the utility of this approach has been validated in several randomized controlled trials. Band ligation is well established, and its popularity has increased with the introduction of multiple ligation devices. The technical simplicity and safety of band ligation has sparked interest in using this technique for primary prophylaxis of variceal bleeding. However, randomized trials have not shown any advantage for band ligation over beta-blocker therapy, and the high variceal recurrence rate after band ligation may eliminate any theoretical advantage. A synchronous combination of band ligation and sclerotherapy has not been shown to improve the results of band ligation alone, but a metachronous approach using sclerotherapy to treat recurrent varices after band ligation has shown beneficial results. Histoacryl remains the best treatment option for gastric varices, but band ligation and loop ligation have shown promising results, and should be considered when Histoacryl is not available. Balloon-occluded retrograde transvenous obliteration is a new radiological modality for gastric varices, and one that sounds promising. TIPS is well established as an alternative to elective endoscopic treatment. Compared with endoscopic treatment, TIPS has been shown to improve the survival rate in one randomized trial. However, the cost and complications of TIPS have restricted its use. The use of endoscopic ultrasound for Doppler studies of blood flow in portal hypertension is currently investigational, but it may gain a role in selecting the optimal treatment approach for the individual patient.     

内镜超声评估门静脉高压性出血风险及静脉曲张复发进展

门静脉高压可导致食管胃底静脉曲张破裂出血(EVB),严重时危及生命,需尽快干预.近年来,内镜超声检查(EUS)技术发展迅速,可直接显示食管及胃黏膜表面曲张静脉和大部分门静脉属支及侧支循环,有助于预测EVB及静脉曲张复发.本文就EUS评估门静脉高压性出血及静脉曲张复发进展进行综述.     

Traitement endoscopique des varices gastriques

Gastric variceal bleeding is a challenge with worst outcomes than oesphageal variceal. The management needs pluridisciplinary discussions and correct appreciation of vascular portal hypertension shunts. Current guidelines recommend endoscopic therapy as first-line with cyanoacrylate obturation. This effective therapy is not perfect with up to 5 to 10% of rebleeding and serious complications. Recent therapies as ultrasound vascular interventional endoscopy with coils or cyano-acrylate guided injection appears as a really promising weapon. Without randomized and controlled studies, this endoscopic technic can’t appear in current guidelines. Future will show that recent endoscopic therapies are not just smokescreen. Interventional radiologists have come to the rescue of many endoscopists when managing bleeding varices. Transjugular intrahepatic portosystemic shunt is indeed effective in the prevention of gastric variceal rebleeding especially for high risk patients.     

A case of non-cirrhotic portal hypertension associated with anti-retroviral therapy in a Japanese patient with human immunodeficiency virus infection

The diagnosis of non-cirrhotic portal hypertension (NCPH), a rare but potentially life-threatening complication in human immunodeficiency virus (HIV)-positive individuals, often occurs only after the emergence of fatal manifestations such as bleeding of esophageal varices. We herein report a female Japanese HIV patient who developed NCPH approximately 4 years after discontinuation of 65 months of didanosine (ddI) administration. The patient presented with severe ascites, bloody bowel discharge, extreme abdominal swelling, and symptoms of portal hypertension but no sign of liver cirrhosis. Examination revealed esophageal varices, oozing-like bleeding from a wide part of the colon, significant atrophy of the right lobe of the liver, and arterio-portal shunting and recanalization from the left medial segment branch of the portal vein to a paraumbilical vein, but no visible obstruction of the main trunk of the portal vein. Treatment for esophageal varices consisted of coagulation therapy with argon plasma after enforcement by endoscopic sclerotherapy and oral administration of β-blockers for elevated portal blood pressure. The patient has not experienced gastrointestinal bleeding in the approximately 5 years since the diagnosis of NCPH. Reviewing this case suggests the importance of suspecting NCPH in HIV patients with liver dysfunction of unknown etiology with a history of ddI and other purine analogs use, as well as the importance of controlling portal hypertension and esophageal varices in the treatment of NCPH.     

H��mostase endoscopique par ent��roscopie double ballon de varices anastomotiques chol��docoj��junales

Gastrointestinal bleeding secondary to rupture from ectopic varices is an infrequent complication due to portal hypertension. The unusual happening of this emergency situation explains the lack of consensus regarding its management. The treatment may be medical, radiological, surgical or endoscopic. The endoscopy in this case can be technically difficult for accessibility reasons, as in the case of small-bowel varices. We present the case of a patient with bleeding varices from choledoco-jejunal anastomosis treated effectively by sclerotherapy with double balloon enteroscopy.     

内镜套扎联合部分脾动脉栓塞术治疗门静脉性上消化道急性出血的研究

目的　探讨急诊内镜套扎联合部分脾动脉栓塞术治疗门静脉高压并发上消化道急性出血的疗效。方法　对 4 8例门静脉高压并食管胃底静脉曲张破裂急性出血患者行急诊内镜下曲张静脉套扎术(EVL) ,联合部分脾动脉栓塞术 (PSE) ,观察近期止血效果和远期再出血发生率及外周血细胞等变化。结果　EVL PSE联合术近期止血效果显著 (10 0 % ) ,食管胃底曲张静脉消失率达 6 8.75 % (33/48) ;无手术死亡 ,无严重并发症 ,远期再出血率 6 .2 5 % (3/48) ,外周血白细胞及血小板均较术前明显回升 (P <0 .0 5 )。结论　联合术能有效地救治门静脉高压并食管胃底静脉曲张破裂急性出血 ,减少单纯内镜下套扎治疗的次数和复发再出血的风险。该法创伤小 ,安全有效。     

内镜治疗肝癌合并门静脉癌栓引起的食管胃底静脉曲张出血的疗效分析

目的明确内镜治疗肝癌合并门静脉癌栓患者发生食管胃底静脉曲张出血的止血效果与意义。方法回顾性分析2013年1月-2015年12月首都医科大学附属北京地坛医院住院收治食管胃底静脉曲张出血的肝癌伴门静脉癌栓的患者,比较非内镜治疗组和内镜治疗组患者止血治疗效果、主要死亡原因及生存时间。结果共纳入76例患者,非内镜治疗组27例,内镜治疗组49例。非内镜治疗组有40.7%(11/27)的患者1周内死亡,81.5%(22/27)死于食管胃底静脉曲张出血,平均生存期为(42.03±13.94)d;内镜治疗组仅16.3%(8/49)的患者1周内死亡(P0.05),55.1%(27/49)死于食管胃底静脉曲张出血(P0.05),平均生存期为(174.24±34.42)d(P0.05)。结论内镜下治疗能有效地降低患者死于食管胃底静脉曲张出血的风险,延长患者生存期,具有临床意义。     

Idiopathic esophageal varices with longitudinal erosion

A 60-year-old man with idiopathic esophageal varices is presented. Endoscopy and endoscopic ultrasonography clearly demonstrated a longitudinal erosion on one of two straight varices. There was no evidence of portal hypertension, and no abnormality in the superior vena cava system. Peritoneoscopy and liver biopsy were normal. These varices with longitudinal erosion represent unique endoscopic findings as compared with previously reported cases. There has been no variceal bleeding during a 6-year follow-up.     

内镜套扎联合部分脾栓塞术治疗门脉高压症及其对血流动力学的影响

目的 :探讨一种新的治疗门脉高压症的手术方法。方法 :对 41例合并食管静脉曲张和脾功能亢进的门脉高压症患者实施内镜套扎 -部分脾栓塞联合治疗 ,手术前后采用彩色多普勒超声检查门奇静脉侧支循环情况 ,与对照组进行对比研究。结果 :除 1例患者出现异位栓塞死亡 ,1例出现脾脓肿经开腹手术治疗得到治愈外 ,未发生其他严重并发症。联合术后患者食管曲张静脉得到根治 ,脾功能亢进缓解。手术后患者门静脉血流速度减慢 ,血流量减少 (P <0 .0 5) ,奇静脉血流量降低 (P <0 .0 1 ) ,胃左静脉血流速度减慢 (P <0 .0 5)。术后随访 2～ 2 4个月 ,未出现复发性出血。结论 :联合术能有效地治疗门脉高压症食管静脉曲张出血和脾功能亢进 ,减少了闭塞曲张静脉所需重复套扎次数及近期再出血 ,同时术后减少了门静脉血流速度 ,血流量 ,降低了套扎术后复发出血的风险 ,该方法操作简单 ,侵袭性小 ,尤其适应于肝功能较差 ,难以耐受分流及断流手术的门脉高压症患者。     

肝硬化食管胃静脉曲张的内镜治疗

肝硬化食管胃静脉曲张出血是危及生命的门脉高压并发症。食管静脉曲张一级预防策略为非选择性β受体阻滞剂（non selective beta blockers,NSBBs）或内镜下静脉曲张套扎术（endoscopic variceal ligation,EVL）,急性出血时首选EVL,其二级预防推荐NSBBs联合EVL。胃静脉曲张出血中,食管胃静脉曲张1型（gastroesophageal varices type 1,GOV1）应用EVL,食管胃静脉曲张2型（gastroesophageal varices type 2,GOV2）和孤立胃静脉曲张（isolated gastric varices,IGV）推荐内镜下组织胶注射术。预防胃静脉曲张再出血方面,内镜下组织胶注射术和经颈静脉肝内门体分流术（transjugular intrahepatic portosystemic shunt,TIPS）可应用于GOV2型和IGV,EVL、NSBBs或内镜下组织胶注射术可应用于GOV1型。胃静脉曲张一级预防可选用NSBBs或内镜下组织胶注射术。     

Temporary pacemaker protected transjugular intrahepatic portosystemic shunt in a patient with acute variceal bleeding and bradyarrhythmia: A case report

BACKGROUNDEsophagogastric varices are a common complication of cirrhosis with portal hypertension and endoscopic treatment has been recognized as a primary preventive and therapeutic option for such patients; however, it should be noted that bradyarrhythmia is regarded as one of the contraindications to endoscopic examination. Meanwhile, acute variceal bleeding may result in a high mortality rate in cirrhotic patients with portal hypertension accompanied by bradyarrhythmia. At present, there is an absence of reports concerning the treatment of such group of patients who underwent transjugular intrahepatic portosystemic shunt (TIPS). The present report details the case of a cirrhotic patient with acute variceal bleeding accompanied by bradyarrhythmia who underwent TIPS under temporary pacemaker protection.CASE SUMMARYWe report the case of a 64-year-old male patient who was confirmed with bradyarrhythmia by ambulatory electrocardiogram 24 h before the operation. The patient was successfully treated by TIPS under temporary pacemaker protection.CONCLUSIONIn terms of cirrhotic patients with abnormal cardiac electrophysiological conduction, TIPS may be effective in reducing the complications of portal hypertension following the exclusion of severe pulmonary hypertension and heart failure, showing moderate feasibility in clinical applications.     

Multifocal gastrointestinal varices: a rare manifestation of immunoglobulin G4-related disease

Multifocal gastrointestinal varices are uncommon and often associated with liver cirrhosis. They consist of varices at the gastroesophageal region and the other sites (i.e. ectopic varices) simultaneously. The etiology includes venous system anomalies or thrombosis (congenital or acquired), vascular injury (iatrogenic or traumatic), or portal hypertension (either intrahepatic or extrahepatic). The clinical manifestations vary from asymptomatic lesions to life-threatening variceal hemorrhage. The identification of bleeding foci, as well as the etiology of varices, can be challenging. The treatment necessitates a multidisciplinary approach. Here, we report a case with multifocal gastrointestinal varices involving the stomach, duodenum, and transverse colon. The patient presented with intermittent melena and has no history of liver or heart disease. Serial endoscopic examinations confirmed the multiple sites of the gastrointestinal varices. Abdominal computed tomography demonstrated that the liver parenchyma is normal, and the hepatic veins, intrahepatic portal system, as well as vena cava, are all unobstructed. Nevertheless, it revealed typical features of autoimmune pancreatitis, retroperitoneal fibrosis, and compromised splenic and superior mesenteric veins. After the pancreatic tissue sampling, we eventually confirmed the etiology as immunoglobulin G4-related disease. In addition to steroid treatment for immunoglobulin G4-related disease, we successfully treated variceal bleeding with band ligation and prevented rebleeding with propranolol. He had been convalescing and has received periodic follow-up in our outpatient clinic for more than 12 months uneventfully.     

药物联合内镜下治疗食管胃底静脉曲张破裂出血56例

目的观察药物联合内镜下治疗食管胃底静脉曲张破裂出血的临床疗效及安全性。方法对56例食管胃底静脉曲张破裂出血患者联合药物及内镜下治疗,单纯食管静脉曲张采用套扎或硬化治疗,但急性出血时首选组织粘合剂注射,对合并的胃底曲张静脉同时行组织粘合剂注射。术后观察不良反应、并发症和近期疗效。结果 53例止血成功,成功率94.64%,1例仍有便血,2例发生脑梗死,考虑组织粘合剂引起的异位栓塞。结论药物联合内镜下治疗食管胃底静脉曲张破裂出血安全、有效,但需警惕异位栓塞的发生。     

Variceal bleeding and portal hypertension: has there been any progress in the last 12 months?

A review of the literature on the management of esophagogastric varices published in the last 12 months shows that the data are still quite conflicting. In the primary and secondary prophylaxis of variceal bleeding, beta-blockers are still the mainstay of pharmacotherapy. Measurement of the hepatic portal venous pressure gradient is considered to be a reliable parameter for successful reduction of portal pressure using medical therapy. However, intolerance of propranolol requiring discontinuation of therapy has been observed in approximately 30 % of patients. Patients' compliance with medication may represent another drawback of medical therapy.The role of endoscopic band ligation in secondary prophylaxis is now indisputable, especially in comparison with sclerotherapy. In the primary prevention of variceal bleeding, band ligation is beginning to have a competitive edge over pharmacological therapy.Acute variceal bleeding is no longer a frequent morbid emergency. Most cases of bleeding can now be managed successfully with band ligation and N-butyl-2-cyanoacrylate obliteration. N-butyl-2-cyanoacrylate has come into increasingly widespread use in the treatment of bleeding gastric fundal varices in which surgery or transjugular intrahepatic portosystemic shunting were previously regarded as the preferred therapies.     

胃底静脉曲张出血33例临床分析

目的 分析胃底静脉曲张出血的病因、诊断和治疗方法。方法 回顾分析该院1994年元月～2003年12月检查确诊为胃底静脉曲张出血33例，对其临床资料进行分析讨论。结果 病因：肝硬化门脉高压23例，肝癌6例，脾静脉阻塞4例。治疗结果：内镜下套扎17例；5例于1～3d内复发大出血死亡，另有9例2个月内复发出血，硬化剂治疗4例，均有复发出血；17例行手术治疗，14例术后无再出血，3例虽有复发出血，但频率及出血量明显减少。结论 胃底静脉曲张是门脉高压，特别是脾胃区门脉高压的表现。胃底静脉曲张较食管静脉曲张出血率低，但出血更严重。内镜对胃底静脉曲张的诊断价值有限。对胃底静脉曲张不宜行套扎或硬化剂治疗。能手术者应首选手术治疗，不宜手术者可考虑介入治疗，栓塞胃底静脉。     

Traitement des complications de l’hypertension portale par TIPS en 2016

Portal hypertension (PHT) is defined by an increase of the pressure gradient between the sus-hepatic vena and the portal vein. PHT is most often due to liver cirrhosis. Transjugular intrahepatic portosystemic shunt (TIPS) is a percutaneously created low-resistance channel between the portal and the hepatic veins. The goal of TIPS is to reduce portal pressure by shunting blood from the portal to the systemic circulation, bypassing the liver. TIPS could permit to treat severe portal hypertension-related complication such as esophageal or gastric varices bleeding. TIPS is currently indicated as the salvage therapy in patients with bleeding esophageal varices who failed to respond to standard treatment. More recently, applying TIPS early has been shown to be an effective treatment to control bleeding and decrease mortality in patients with severe cirrhosis. TIPS is also recommended as the second-line treatment for secondary prophylaxis. TIPS is a promising treatment for refractory ascites or hepatic hydrothorax. TIPS should be considered in the treatment of Budd-Chiari syndrome. However, the role of TIPS in the treatment of hepatorenal syndrome is not well defined.     

Endosonographic Doppler-guided manometry of esophageal varices: experimental validation and clinical feasibility

BACKGROUND AND STUDY AIMS: The risk of variceal bleeding cannot be accurately predicted using endoscopy alone. Although variceal pressure has been demonstrated to be a major determinant for the rupture of esophageal varices, direct determination by needle puncture is unsuitable for routine clinical use. Due to their operator-dependency, current noninvasive endoscopic methods for determination of variceal pressure have not gained wide acceptance. We have developed a new method of measuring variceal pressure, using endoscopic power Doppler imaging to monitor the manometry of esophageal varices. The aims of this study were to test in vitro the accuracy of Doppler-guided manometry and to assess the clinical feasibility of this method. MATERIALS AND METHODS: Experimental validation of this technique was performed using an in vitro model of artificial varices of different sizes. A linear-array endosonography (EUS) probe with power Doppler capability was used to assess flow in the varices and a balloon for manometry of esophageal varices was attached to the tip of the probe. Pressure readings were made at the time of disappearance of the Doppler signal during variceal compression by the balloon. Linear regression analysis was used to compare the results of Doppler-guided and direct intraluminal pressure measurement in the artificial varices. Variceal pressure was then measured with this technique in 28 patients with portal hypertension and esophageal varices without previous bleeding, and the results were compared with portal pressure assessed according to the hepatic vein pressure gradient (HVPG). RESULTS: In vitro studies demonstrated a good correlation between the pressure measured with Doppler monitoring and the actual intravariceal pressure (r > or = 0.922; P < 0.001). The determination of variceal pressure with this method was technically successful in 26/28 patients (93 %). The intraoperator variance was 9.3 +/- 8.6 %. Overall, the mean variceal pressure was significantly lower than the mean HVPG (21.2 +/- 5.3 mmHg vs. 24.3 +/- 7.8 mmHg; P < 0.01). Variceal pressure and portal pressure (as assessed by the HVPG) correlated significantly (r = 0.64; P < 0.001). CONCLUSIONS: Our preliminary results indicate that EUS Doppler-guided manometry of esophageal varices is feasible and accurate. This technique may become a more reliable method for noninvasive measurement of variceal pressure and warrants further investigation.     

Ruptured gastric fundal varices treated with endoscopic variceal ligation and transjugular retrograde obliteration: case report

Therapeutic modalities for the obliteration of collateral vessels connecting the portal venous system with the systemic circulation, transjugular retrograde obliteration (TJO) and balloon-occluded retrograde transvenous obliteration have recently been developed, and several satisfactory results have been reported with their use. We report a case of ruptured gastric fundal varices treated with TJO after endoscopic variceal ligation (EVL). In our case, variceal bleeding was controlled successfully with EVL and varices were eradicated with TJO.