Rectosigmoid Varices and Other Mucosal Changes in Patients with Portal Hypertension

A prospective study was performed to evaluate the prevalence of anorectal varices and their clinical significance as well as to study other proctosigmoidoscopic changes in 75 patients with portal hypertension of diverse etiology. Sixty-seven patients (89.3%) had lower gastrointestinal varices with no significant difference (p greater than 0.05) in prevalence between cirrhosis (92.1%), noncirrhotic portal fibrosis (87%), and extrahepatic portal venous obstruction (85.7%). The rectum was the most common site of lower gastrointestinal varices. External anal and sigmoid colonic varices almost always occurred in the presence of rectal and/or internal anal varices. There was no correlation between the presence of rectosigmoid varices and the severity of esophagogastric mucosal changes or portal hypertension. There was no suggestion that esophageal variceal sclerotherapy influenced the presence of anorectal varices. Seven patients (9.3%) had recent hematochezia, including three patients in whom it occurred in the absence of any upper gastrointestinal hemorrhage. Varices were the cause of bleeding in at least five patients. An abnormal mucosal vascular pattern in the form of telangiectasias or spiders was seen, irrespective of etiology of portal hypertension, in nine patients (12%). Hemorrhoids were present in 31 patients (41.3%) with an age-related difference (p less than 0.05) between patients with cirrhosis (55.3%) and extrahepatic portal venous obstruction (21.4%).     

PATHOPHYSIOLOGY OF PORTAL HYPERTENSION AND IMPLICATIONS FOR ITS PHARMACOLOGICAL CONTROL

Bleeding from esophageal varices complicating portal hypertension is a major cause of morbidity and mortality in patients with chronic liver disease. Therapy has been directed towards obliteration of esophageal varices (endoscopic sclerotherapy, transhepatic sclerosis, or esophageal transection) or decompression of the portal vascular bed by the creation of surgical portasystemic shunts. The use of pharmacological agents to lower portal venous pressure (PVP) was for many years limited to intravenous or intraarterial vasopressin in the setting of acute variceal hemorrhage. However, since Lebrec et al.,¹ reported the favourable effect of propranolol on PVP and on the incidence of rebleeding from the upper gastrointestinal tract in patients with portal hypertension,² there has been an increasing interest in the potential use of a number of pharmacological agents in the short and long term management of patients with portal hypertension. The purpose of such therapy would be to reduce the incidence of complications of portal hypertension. These may include upper gastrointestinal bleeding, ascites via reduced ascitic fluid formation, and even portasystemic encephalopathy via improvement of hepatic perfusion and reduction of collateral shunting of blood.     

Protein C deficiency related obscure gastrointestinal bleeding treated by enteroscopy and anticoagulant therapy

Obscure gastrointestinal bleeding is an uncommonly encountered and difficult-to-treat clinical problem in gastroenterology,but advancements in endoscopic and radiologic imaging modalities allow for greater accuracyin diagnosing obscure gastrointestinal bleeding.Ectopic varices account for less than 5% of all variceal bleeding cases,and jejunal variceal bleeding due to extrahepatic portal hypertension is rare.We present a 47-year-old man suffering from obscure gastrointestinal bleeding.Computed tomography of the abdomen revealed multiple vascular tufts around the proximal jejunum but no evidence of cirrhosis,and a visible hypodense filling defect suggestive of thrombus was visible in the superior mesenteric vein.Enteroscopy revealed several serpiginous varices in the proximal jejunum.Serologic data disclosed protein C deficiency(33.6%).The patient was successfully treated by therapeutic balloonassisted enteroscopy and long-term anticoagulant therapy,which is normally contraindicated in patients with gastrointestinal bleeding.Diagnostic modalities for obscure gastrointestinal bleeding,such as capsule endoscopy,computed tomography enterography,magnetic resonance enterography,and enteroscopy,were also reviewed in this article.     

Portal and mesenteric thrombosis associated with protein S deficiency]

INTRODUCTION: liver cirrhosis is the main cause of portal thrombosis (PT), while hypercoagulability syndromes are rarely found as the etiology of PT. We report a case of portal and mesenteric thrombosis secondary to protein S deficiency. CASE REPORT: a 74-year-old woman was admitted with melena secondary to upper gastrointestinal bleeding. She reported mild, diffuse abdominal pain in the last 2 weeks. Endoscopy revealed ruptured esophageal varices. Doppler ultrasonography and CT demonstrated a heterogeneous liver, splenomegaly and ascites, and complete non-occlusive PT involving the hilum and portal branches, as well as the superior mesenteric vein, with portosystemic collaterals. At this point a complete study for cirrhosis etiologies was negative, including a liver biopsy that showed nonspecific architectural changes secondary to diminished blood flow, which suggested non-cirrhotic portal hypertension. The search for hypercoagulability states determined a deficiency of S protein, with total pS = 107% and free pS = 56%. The patient was started on anticoagulant treatment and no other thrombotic events occurred. DISCUSSION: PT usually manifests without specific symptoms. The most common presentation is upper gastrointestinal bleeding, as occurred in our patient. Liver cirrhosis is one of the most frequent cause of PT. Up to 65% of these patients present an associated prothrombotic state, including protein S deficiency. Our case reminds us of the importance of a systematic search for hipercoagulability syndromes in patients with TP, even when the etiology can be conferred to liver cirrhosis.     

The prevalence and spectrum of colonic lesions in patients with cirrhotic and noncirrhotic portal hypertension

Portal hypertension diffusely affects the gastrointestinal tract. The frequency and profile of distinct colonic mucosal lesions (portal colopathy) and rectal varices (RV; veins >4 cm above the anal verge) is not well studied. Fifty consecutive patients with portal hypertension (25 with cirrhosis, 10 with noncirrhotic portal fibrosis [NCpf], and 15 with extrahepatic portal vein obstruction [EHPVO]) were assessed clinically and by upper and lower gastrointestinal (GI) endoscopy. Colorectal lesions were seen in 35 (70%) patients, significantly more often in bleeders than in nonbleeders. Rectal varices were detected in 22 (44%) patients; larger and more often seen in EHPVO (80%) than in cirrhosis (28%) and NCPF (30%) (P < .01) patients. Portal colopathy was seen in 26 (52%) patients, with nearly similar frequency in cirrhotics, NCPF, and EHPVO patients. Previous sclerotherapy or presence of gastric varices had little influence on the development of these lesions. An association (P < .01) was, however, seen between the presence of colopathy and portal gastropathy. Overt bleeding was seen in 8% and 4% of patients with RV and colopathy, respectively. In conclusion, our results demonstrate that colorectal lesions are present in about two thirds of patients with portal hypertension. Patients with portal hypertension and lower GI bleeding should be colonoscoped. Patients with extrahepatic portal vein obstruction may in turn benefit from baseline sigmoidoscopic examination to define the presence and size of rectal varices.     

Colorectal variceal bleeding in patients with extrahepatic portal vein thrombosis and idiopathic portal hypertension.

We report three patients with colonic variceal bleeding secondary to portal hypertension, 0.5% of all cases with hemorrhagic portal hypertension studied by us in the last 16 years. One patient had idiopathic portal hypertension, and the others had extrahepatic portal vein thrombosis. Colonic varices were documented in all three cases by angiogram; large arteriovenous fistulas in the territory of the superior mesenteric artery and between the inferior mesenteric artery and hemorrhoidal veins were demonstrated in one patient. Two patients underwent colonoscopy; colonic varices were seen in only one. Two patients also had bled from esophagogastric varices. One patient underwent descending colon and sigmoid resection after failure to control bleeding with ligation of arterial supply; one patient underwent the Sugiura procedure, plus transanal ligation of hemorrhoids and rectal varices. At 3 months, 2 years, and 4 years of follow-up, the patients were in good general condition without any evidence of rebleeding.     

Transhepatic portal venous angioplasty with stenting for bleeding jejunal varices

A 54-year-old woman, who had undergone pancreatoduodenectomy with resection of the portal vein and intraoperative radiation therapy for cancer of the lower bile duct 16 months before, visited our institution complaining of melena. To identify the cause of bleeding and severe anemia, we performed gastrointestinal endoscopy but could detect no obvious source. The portal phase of the superior mesenteric arteriography and percutaneous transhepatic portography revealed severe stenosis of the extrahepatic portal vein, which corresponded to the end-to-end anastomosis of the portal vein, and hepatofugal collaterals. Extravasations into the afferent loop of the jejunum were detected only with portography. These findings suggested that portal hypertension due to extrahepatic portal obstruction led to bleeding varices. Subsequent to percutaneous transhepatic portography, we dilated the stenosis of the extrahepatic portal vein using a balloon catheter and placed an expandable metallic stent there. Portography after the treatment revealed the disappearance of the hepatofugal flow to collaterals and extravasations, and the patient has had no further episodes of gastrointestinal bleeding since. In conclusion, for patients with bleeding varices due to extrahepatic portal obstruction, especially after abdominal surgery, percutaneous transhepatic angioplasty is considered to be the treatment of choice because of its efficiency and minimal invasiveness.     

Peripapillary duodenal varices as a rare cause of severe bleeding in a patient with no other signs of portal hypertension--successful endoscopic treatment with cyanoacrylate injection

Duodenal varices (DVs) are a rare cause of upper gastrointestinal bleeding and rather suspected in patients with portal hypertension. Bleeding DVs are difficult to manage and often fatal due to delayed diagnosis. We report on a 71-year-old patient with massive upper gastrointestinal haemorrhage, who did not show any clinical signs of portal hypertension; however, he had a history of duodenal segmental resection 8 years before. The source of bleeding could not be detected with different imaging methods such as angiography and computed tomography. Upper gastrointestinal endoscopy finally revealed DVs, which were located just adjacent to the papilla. After endoscopic injection therapy with n-butyl 2-cyanoacrylate the bleeding stopped immediately and the patient soon stabilised. Despite the peripapillar localisation no signs of pancreatitis or cholestasis occurred; during 10-month follow-up a marked regression of the varices without further signs of variceal bleeding was observed.     

Rectal varices in extrahepatic portal hypertension

Abstract Rectal varices, as distinct from haemorrhoids, occur due to high pressure in the inferior mesenteric venous system in patients with portal hypertension. The exact prevalence of rectal varices in extrahepatic portal hypertension is unknown. To determine this, 116 patients with extrahepatic portal hypertension were studied for the presence of rectal varices. These lesions were found in 103 (88.8%) patients. Bleeding from rectal varices occurred in 14.6% of patients. Massive bleeding requiring hospitalization and blood transfusion was not encountered. It is concluded that rectal varices are common in extrahepatic portal hypertension. Bleeding from them is uncommon, and often mild and self-limiting. The available literature is reviewed and the importance of recognizing the condition stressed.     

Non-cirrhotic portal hypertension versus idiopathic portal hypertension

Portal hypertension occurs in a number of disorders other than cirrhosis and they are collectively called non-cirrhotic portal hypertension (NCPH). The common causes of NCPH include idiopathic portal hypertension (IPH), non-cirrhotic portal fibrosis (NCPF) and extrahepatic portal venous thrombosis (EHPVT). Other causes include schistosomiasis, hepatic venous outflow tract obstruction, veno-occlusive disease and congenital hepatic fibrosis. Patients with IPH and EHPVT present with upper gastrointestinal bleeding, splenomegaly, ascites after gastrointestinal bleeding, features of hypersplenism, growth retardation and jaundice due to portal biliopathy. The diagnosis is usually made by abdominal ultrasound, upper gastrointestinal endoscopy, normal liver function tests and normal liver histology. Variceal bleeding in NCPH has lower mortality as compared with cirrhosis because of better liver functions in NCPH. Treatment for NCPH includes primary prophylaxis for variceal bleeding and prevention of repeat bleeding using drugs like beta-blockers, endoscopic sclerotherapy and endoscopic band ligation of varices. In patients with uncontrolled variceal bleeding or symptomatic hypersplenism, porto-systemic shunt surgery or splenectomy are required.     

Current outcome of portal vein thrombosis in adults: risk and benefit of anticoagulant therapy

BACKGROUND & AIMS: The outcome of portal vein thrombosis in relation to associated prothrombotic states has not been evaluated. We assessed current outcome and predictors of bleeding and thrombotic events in a cohort of 136 adults with nonmalignant, noncirrhotic portal vein thrombosis, of whom 84 received anticoagulant therapy. METHODS: Multivariate Cox model analysis for event-free survival and analysis taking into account multiple events were used. RESULTS: Median follow-up was 46 months. The incidence rate of gastrointestinal bleeding was 12.5 (95% confidence interval [CI], 10-15) per 100 patient-years. Large varices were an independent predictor for bleeding. Anticoagulant therapy did not increase the risk or the severity of bleeding. The incidence rate of thrombotic events was 5.5 (95% CI, 3.8-7.2) per 100 patient-years. Underlying prothrombotic state and absence of anticoagulant therapy were independent predictors for thrombosis. In patients with underlying prothrombotic state, the incidence rates of splanchnic venous infarction were 0.82 and 5.2 per 100 patient-years in periods with and without anticoagulant therapy, respectively (P = 0.01). Two nonanticoagulated patients died of bleeding and thrombosis, respectively. CONCLUSIONS: In patients with portal vein thrombosis, the risk of thrombosis is currently as clinically significant as the risk of bleeding. The benefit-risk ratio favors anticoagulant therapy.     

Incidence and fate of antral varices.

OBJECTIVE: To study the incidence of antral varices (AV) and their fate in patients with portal hypertension so as to formulate a management policy. DESIGN: Prospective cohort study. SETTING: Single surgical unit specializing in portal hypertension management in a tertiary level centre. PARTICIPANTS: Three hundred and seventy-one patients [cirrhosis 170, non-cirrhotic portal fibrosis (NCPF) 53, extrahepatic portal venous obstruction (EHPVO) 148] with history of bleeding from oesophageal varices were inducted in the chronic sclerotherapy programme. INTERVENTIONS: Protocol-based endoscopic sclerotherapy and management of bleeding for oesophageal varices. OUTCOME MEASURES: Development or disappearance of AV, bleeding from AV. RESULTS: No patient had AV on index endoscopy. Thirteen (3.5%) patients developed AV, in cirrhosis 2.9%, EHPVO 4.1%, NCPF 3.8% (P = 0.86). AV developed after a mean of 15 months. Oesophageal varices took a longer number of sessions to obliterate in patients with AV (11.1 vs 5.98 sessions, P<0.0001). Only one patient bled, having coexistent oesophageal varices and gastropathy. AV disappeared spontaneously in seven patients, recurring in only one. Of seven persisting AV, none have bled over a mean follow-up of 30 months (SD 23.2). CONCLUSIONS: AV are seen in a small proportion of patients, and are distributed equally amongst the aetiologies of portal hypertension. They rarely bleed and may be ignored during sclerotherapy of oesophageal varices.     

Unusual sites of upper gastrointestinal variceal bleeding

When patients with portal hypertension bleed from varices, these are most commonly located in the esophagus and gastric fundus. However, varices can develop anywhere in the upper or lower gastrointestinal tract. Oftentimes if an active upper gastrointestinal bleeding site is not evident at the time of endoscopy, bleeding is attributed to any esophageal or gastric varices that are present. This supposition may not always be true as illustrated in the two patients presented here. Likewise, the absence of esophagogastric varices in a patient with portal hypertension does not preclude the presence of varices elsewhere. Endoscopic examination of the second and third portion of the duodenum can sometimes be helpful in accurately locating the bleeding site.     

Endoscopic sclerotherapy for bleeding esophageal varices secondary to extrahepatic portal vein obstruction

Portal hypertension and variceal bleeding secondary to extrahepatic portal vein obstruction continue to present a therapeutic challenge. We performed endoscopic injection sclerotherapy in eight patients with extrahepatic portal vein obstruction and bleeding esophageal varices. In contrast to other reported series, all but one of our patients were adults at the time sclerotherapy was initiated. Six had episodes of continued bleeding after a variety of surgical procedures. After sclerotherapy, five had no further bleeding with a mean follow-up of 26 months. Three patients had episodes of bleeding prior to variceal obliteration; two of these patients underwent surgical intervention after emergency sclerosis to stabilize their condition. Transfusion requirements were less after sclerosis (p = 0.035), although the follow-up has been relatively short (mean, 24 months) compared to the duration of bleeding. Our results suggest that endoscopic sclerotherapy is an effective therapeutic alternative, and perhaps the initial treatment of choice, in patients with extrahepatic portal vein obstruction and bleeding esophageal varices.     

多排螺旋CT门静脉成像对门静脉高压食管、胃底静脉曲张的评价

肝硬化失代偿期可引起门静脉高压,食管胃底静脉曲张是门静脉高压的一个严重并发症,其破裂可引起胃肠道大出血,对门静脉高压侧支循环的显示对患者的治疗方式的选择及预后的评估具有重要意义.多排螺旋CT门静脉成像(CTPV)可显示胃底静脉曲张的部位、形态及侧支循环血供的关系,在GEVI型,GV多为LGV或以LGV为主来供应,胃和(或)脾-肾分流较少见,GV的形态多为迂曲型.在GEV2型,GV大部分由PGV和(或)SGV供血,部分病例伴胃和(或)脾-肾分流.IGV型多以PGV和(或)SGV为主要血供,且较多合并胃和(或)脾-肾分流,GEV2和IGV型GV的形态以结节型和瘤型较多.CTPV可显示食管静脉曲张分型与其侧支循环的关系,EV以位于食管黏膜下、食管壁为主时,其血供多为胃左静脉前支优势型;EV为食管管旁静脉曲张为主时,其血供多为后支优势型;EV管壁、黏膜下静脉曲张程度与管旁静脉曲张接近时,其血供多为前后支均衡型.     

Extrahepatic portal hypertension: spleno-portal thrombosis secondary to protein C deficiency

Portal vein thrombosis (PVT) is the most frequent cause of hypertension portal extrahepatic. It is a rare disorder an the main risk factors are cirrhosis, hepatobiliary malignancies and prothrombotic disorders, which have been identified as major risk. Therapy with anticoagulants must to be considered in acute portal thrombosis or chronic one and proven hypercoagulability. We present the case of a twenty-nine years old patient, with extrahepatic portal hypertension secondary to portal and splenic vein thrombosis, who was diagnosed because of splenomegaly and a coagulation disorder. A protein C deficiency were discovered and anticoagulation and beta-blocker therapy were initiated. One year later the patient had not presented complications concerning to the disease or to the treatment.     

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.     

Massive duodenal variceal bleed; complication of extra hepatic portal hypertension: Endoscopic management and literature review

Bleeding from duodenal varices is reported to be a catastrophic and often fatal event. Most of the cases in the literature involve patients with underlying cirrhosis. However, approximately one quarter of duodenal variceal bleeds is caused by extrahepatic portal hypertension and they represent a unique population given their lack of liver dysfunction. The authors present a case where a 61-year-old male with history of remote crush injury presented with bright red blood per rectum and was found to have bleeding from massive duodenal varices. Injection sclerotherapy with ethanolamine was performed and the patient experienced a favorable outcome with near resolution of his varices on endoscopic follow-up. The authors conclude that sclerotherapy is a reasonable first line therapy and review the literature surrounding the treatment of duodenal varices secondary to extrahepatic portal hypertension.     

Hepatic vascular disease and portal hypertension in polycythemia vera and agnogenic myeloid metaplasia: a clinicopathological study of 145 patients examined at autopsy

The pathogenesis of portal hypertension arising in patients with myeloproliferative disorders has been difficult to understand because liver biopsy findings often show minimal changes. It has been suggested that increased splenic blood flow, hepatic infiltration with hematopoietic cells or sinusoidal fibrosis may be important. We have reviewed the autopsy findings and clinical histories of 97 patients with polycythemia vera and 48 patients with agnogenic myeloid metaplasia collected from three institutions and from the Polycythemia Vera Study Group. Cirrhosis was present in seven patients, one of whom had bleeding varices. Esophageal varices were present clinically in 10 patients without cirrhosis (seven polycythemia and three agnogenic myeloid metaplasia). All of these patients had lesions in small or medium-sized portal veins and four had stenosis of the extrahepatic portal vein with histology compatible with organized thrombi. Nodular regenerative hyperplasia occurred in 14.6% and correlated closely with the presence of portal vein lesions. Thirty patients had greater than 500 ml of ascites, seven of these patients also had varices and six of them had hepatic vein thrombosis. Ascites also correlated with hepatic vein disease confined to small intrahepatic branches. No correlation was seen between hepatic hematopoietic infiltration and signs of portal hypertension. We conclude that esophageal varices are common and are almost always associated with portal vein lesions visible by light microscopy. These portal vein lesions, and the secondary effects of nodular regenerative hyperplasia and portal hypertension, are most likely a result of portal vein thrombosis in patients with myeloproliferative disorders.