Non-cirrhotic portal fibrosis

Non-cirrhotic portal hypertension (NCPH) comprises of diseases having an increase in portal pressure (PP) due to intraheptic or prehepatic lesions, in the absence of cirrhosis. The lesions are generally vascular, either in the portal vein, its branches or in the perisinusoidal area. Because the wedged hepatic venous pressure (WHVP) is near normal, measurement of intravariceal or intrasplenic pressure is needed to assess portal pressure. The majority of the diseases included in the category of NCPH are well characterized disease entities where portal hypertension (PHT) is a late manifestation and hence, these are not discussed. Two diseases which present only with features of PHT and are common in developing countries are NCPF and extra-hepatic portal vein obstruction (EHPVO). Non-cirrhotic portal fibrosis is a syndrome of obscure etiology, characterized by 'Obliterative portovenopathy' leading to PHT, massive splenomegaly, repeated well tolerated episodes of variceal bleeding and anemia in young adults from low socio-economic strata of life. The hepatic parenchymal functions are nearly normal. Jaundice, ascites and hepatic encephalopathy are rare. Management of variceal bleeding remains the main concern as nearly 85% of patients with NCPF present with variceal bleeding. Endoscopic variceal ligation or sclerotherapy are equally effective in about 90-95% of the patients. Gastric varices are seen in about 25% patients and a bleed from them can be managed with cyanoacrylate glue injection or surgery. Other indications for surgery include failure of endoscopic therapy to control acute bleed and symptomatic hypersplenism. The prognosis of patients with NCPF is good and 5-years survival rates in patients in whom variceal bleeding can be controlled is about > 95%.     

Etiological spectrum of esophageal varices due to portal hypertension in Indian children: Is it different from the West?

Background and Aim:  Information about portal hypertension (PHT) in children is meagre. We therefore studied the spectrum and outcome of PHT in children (≤14 years of age) over a period of 9 years.
Methods:  PHT was diagnosed on endoscopy (presence of varices) in 517 cases during the study period. The diagnosis of extrahepatic portal venous obstruction (EHPVO) and Budd–Chiari Syndrome (BCS) were made on the basis of ultrasound examination. Cirrhosis was diagnosed on the basis of clinical, biochemical, ultrasound, and liver biopsy (whenever feasible). Noncirrhotic portal fibrosis (NCPF) and congenital hepatic fibrosis (CHF) were diagnosed on liver biopsy. Endoscopic sclerotherapy (EST) was done in all the patients who presented with variceal bleeding and surgery was performed whenever indicated.
Results:  Causes of PHT included EHPVO in 54%, cirrhosis in 39%, CHF in 3%, NCPF in 2%, and BCS in 2%. Of these, 279 (54%) patients presented with upper gastrointestinal bleeding and this group comprised of EHPVO in 85%, cirrhosis in 10%, CHF in 2.5%, NCPF in 2%, and BCS in 1%. Bleeding was the presenting feature in 85% of EHPVO cases and in 13% of cirrhosis cases. In EHPVO cases, variceal eradication was achieved in 95% of cases with a mean 5 ± 2.4 EST sessions. Surgery was required in 24 cases of EHPVO. Mortality due to bleeding was 1.7% in EHPVO and 30% in cirrhosis.
Conclusions:  EHPVO and cirrhosis are the two major causes of PHT in children. However, predominant cause of variceal bleeding is EHPVO. EST is an effective method of treatment in EHPVO.     

Pediatric non-cirrhotic portal hypertension:Endoscopic outcome and perspectives from developing nations

Non-cirrhotic portal hypertension(NCPH) forms an important subset of portal hypertension in children. Variceal bleed and splenomegaly are their predominant presentation. Laboratory features show cytopenias(hypersplenism) and preserved hepatic synthetic functions. Repeated sessions of endoscopic variceal ligation or endoscopic sclerotherapy eradicate esophageal varices in almost all cases. After variceal eradication, there is an increased risk of other complications like secondary gastric varices, cholangiopathy, colopathy, growth failure,especially in extra-hepatic portal vein obstruction(EHPVO). Massive splenomegaly-related pain and early satiety cause poor quality of life(QoL). Meso-Rex bypass is the definitive therapy when the procedure is anatomically feasible in EHPVO. Other portosystemic shunt surgeries with splenectomy are indicated when patients present late and spleen-related issues predominate. Shunt surgeries prevent rebleed, improve growth and QoL. Non-cirrhotic portal fibrosis(NCPF) is a less common cause of portal hypertension in children in developing nations.Presentation in the second decade, massive splenomegaly and patent portal vein are discriminating features of NCPF. Shunt surgery is required in severe cases when endotherapy is insufficient for the varices. Congenital hepatic fibrosis(CHF)presents with firm palpable liver and splenomegaly. Ductal plate malformation forms the histological hallmark of CHF. CHF is commonly associated with Caroli's disease, renal cysts, and syndromes associated with neurological defects.Isolated CHF has a favourable prognosis requiring endotherapy. Liver transplanta-tion is required when there is decompensation or recurrent cholangitis, especially in Caroli's syndrome. Combined liver-kidney transplantation is indicated when both liver and renal issues are present.     

Non-cirrhotic portal fibrosis (idiopathic portal hypertension): experience with 151 patients and a review of the literature

BACKGROUND: Non-cirrhotic portal fibrosis (NCPF), the equivalent of idiopathic portal hypertension in Japan and hepatoportal sclerosis in the United States of America, is a common cause of portal hypertension in India. The clinical features, portographic and histological findings, and management of 151 patients with non-cirrhotic portal fibrosis are presented. METHODS: The disease is diagnosed by the presence of unequivocal evidence of portal hypertension in the definite absence of liver cirrhosis and extrahepatic portal vein obstruction (EHPVO). Retrospective analysis of records of 151 patients with NCPF was analyzed for the clinical presentation, physical findings, laboratory tests, radiological and histological findings, and for the outcome of treatment. RESULTS: The disease is characterized by massive splenomegaly with anemia, preserved liver function and benign prognosis in a majority of patients. Splenoportovenography (SPV) showed massive dilatation of the portal and splenic veins, and the presence of collaterals. Twenty-four (15.9%) patients showed evidence of natural/spontaneous shunts (splenorenal 15, umbilical nine) on SPV; these patients had a lower incidence of variceal bleeding. Liver histology demonstrated maintained lobular architecture, portal fibrosis of variable degree, sclerosis and obliteration of small-sized portal vein radicles, and subcapsular scarring with the collapse of the underlying parenchyma. Piecemeal or hepatocytic necrosis was absent in all histology specimens. Three patients showed nodular transformation along with abnormal liver functions, and may represent late manifestation of NCPF where features are similar to those seen in patients with incomplete septal cirrhosis. In the initial part of the study, surgery (side-to-side lieno-renal shunt) was the preferred modality of treatment, however, endoscopic sclerotherapy or variceal ligation has now become the preferred first line of management of variceal bleeding. CONCLUSIONS: The epidemiological and clinical features of NCPF have more similarity to IPH than has previously been documented. The development of spontaneous shunts tends to protect these patients from variceal bleeding.     

Endoscopic variceal ligation for primary prophylaxis of oesophageal variceal bleed: preliminary report of a randomized controlled trial

BACKGROUND: Prevention of variceal bleeding, a major cause of morbidity and mortality, is an important goal in the management of patients with portal hypertension (PHT). Although propranolol has been found useful in preventing the first episode of variceal bleeding (primary prophylaxis) in cirrhotic PHT, it has limitations which include side effects, contraindications, non-compliance and failure in some patients. Endoscopic variceal ligation (EVL) has not been used for primary prophylaxis. METHODS: Thirty cirrhotic patients with PHT, grade III to IV oesophageal varices, hepatic venous pressure gradient > or = 12 mmHg and no prior history of upper gastrointestinal bleeding were randomized to receive propranolol (to reduce their pulse rate by 25% from baseline, n = 15) and EVL (weekly to fortnightly until variceal eradication, n = 15). The two groups were comparable. All the patients in EVL group had variceal eradication during 3.8 +/- 2.2 sessions. RESULTS: There was no major complication or interval bleeding. During a follow-up period of 17.6 +/- 4.7 months, varices recurred in three, two of which bled (successfully treated by EVL). In contrast, during this period of follow up one patient in the propranolol group had variceal bleeding (P=NS). Side effects of propranolol included symptomatic bradycardia requiring reduction of dose in one of 15 patients. CONCLUSIONS: Although sample size in this study is small, it seems that EVL may be a good option for primary prophylaxis for variceal bleeding in patients with cirrhotic PHT; further studies on a larger number of patients and longer follow up are required.     

重视非肝硬化性门静脉高压的诊断和治疗

非肝硬化性门静脉高压(NCPH)是指除肝硬化外多种疾病导致的门静脉高压症。NCPH常见的原因有门静脉血栓形成、先天性肝纤维化和特发性门静脉高压等。这组疾病的主要特点是门静脉高压相关的表现突出,而肝功能储备相对较好,鉴别该类疾病需要临床,影像学和病理学的深入检查。通过适当的内外科治疗,多数患者预后较好。     

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.     

Comparative efficacy of emergency endoscopic sclerotherapy for active variceal bleeding due to cirrhosis of the liver, non-cirrhotic portal fibrosis and extrahepatic portal venous obstruction

Patients with continued variceal bleeding due to portal hypertension (n = 202) were treated by endoscopic injection sclerotherapy after resuscitation. Portal hypertension was due to hepatic cirrhosis in 123, non-cirrhotic portal fibrosis (NCPF) in 49 and extrahepatic portal venous obstruction (EHO) in 30 patients. Polidocanol 1% was injected intravariceally. An adequate sclerotherapy was carried out in 97% of patients. Immediate haemostasis was achieved in 177 (88%) patients. Rebleeding occurred in 31 (17.5%) of 177 patients. By reinjection of varices, definitive control of bleeding occurred in 160 (79%) patients. There was no significant difference in terms of immediate control of bleeding in patients with different aetiologies of portal hypertension and hepatic functional status (Child's grade). Rebleeding episodes were lower in patients with EHO than cirrhosis of the liver and NCPF. Similarly, the Child's status significantly influenced the recurrence of bleeding which was lower in Child's A than B and B than C. The in-hospital mortality was 18.6%. This was also significantly related to Child's status and aetiology of portal hypertension. Minor complications occurred in 10.4% of patients. It is concluded that endoscopic sclerotherapy as the first line of treatment is an effective and technically feasible procedure for the control of active variceal bleeding, regardless of the cause of portal hypertension. Furthermore, the results were influenced by the aetiology of portal hypertension and hepatic functional status.     

Variceal pressure is a strong predictor of variceal haemorrhage in patients with cirrhosis as well as in patients with non-cirrhotic portal hypertension

BACKGROUND: Variceal pressure is a strong predictor for a first variceal bleed in patients with cirrhosis. AIMS: To evaluate whether variceal pressure is also a determinant of the risk of a first variceal bleed in patients with non-cirrhotic portal hypertension. METHODS: Variceal pressure was measured non-invasively in 25 patients with non-cirrhotic portal hypertension and large varices while receiving a stable therapeutic regimen. Factors predictive of bleeding were compared with those observed in 87 cirrhotics. RESULTS: The one year incidence of variceal bleeding was 32% (n=28) for the cirrhotic and 20% (n=5) for the non-cirrhotic patients. There was no difference in factors predicting the risk of bleeding between the groups, except for variceal pressure. For the same level of variceal pressure, the risk of variceal bleeding was lower in patients with non-cirrhotic portal hypertension. Multiple logistic regression analysis revealed the following variables as having a significant predictive power: variceal pressure (p=0.0001), red spots (p=0.004), and the time interval between the first observation of the varices and the moment of variceal pressure measurement (p=0. 0046). For the non-cirrhotics the risk of bleeding increased with higher Child-Pugh score (p=0.0024); this was not the case for the cirrhotic patients (p=0.9521). CONCLUSION: Variceal pressure is a major predictor of variceal bleeding in patients with cirrhosis as well as in patients with non-cirrhotic portal hypertension. The risk of bleeding in non-cirrhotics is less than in cirrhotics for the same level of variceal pressure. In patients with non-cirrhotic portal hypertension the risk of variceal bleeding increases more with advancing disease.     

Coagulation profile and platelet function in patients with extrahepatic portal vein obstruction and non-cirrhotic portal fibrosis

BACKGROUND AND AIMS: Coagulation disorders commonly develop in patients with cirrhosis of the liver. They have also been reported in patients with non-cirrhotic portal fibrosis (NCPF) and extra-hepatic portal venous obstruction (EHPVO); the two conditions with portal hypertension and near-normal liver functions. The spectrum and prevalence of coagulation abnormalities and their association with the pathogenesis of these diseases and with hypersplenism was prospectively studied. METHODS: Eighteen EHPVO patients that included an equal number of NCPF patients and 20 healthy controls were prospectively studied. The coagulation parameters assessed included: international normalized ratio, partial thromboplastin time, and fibrinogen and fibrinogen degradation products. Platelet aggregation and malondialdehyde levels were measured. RESULTS: Both EHPVO (83%) and NCPF (78%) patients had a significantly prolonged international normalized ratio and a decrease in fibrinogen and platelet aggregation. The EHPVO patients had a significant prolongation in partial thromboplastin time (67% patients), with increased levels of fibrinogen degradation product levels occurring in all patients; these were normal in NCPF patients. Platelet malondialdehyde levels were normal in both groups. Hypersplenism was present in four EHPVO and seven NCPF patients. It did not significantly influence the coagulation profile in either NCPF or EHPVO patients. CONCLUSIONS: Coagulation anomalies are common and significant in both NCPF and EHPVO patients, suggestive of a mild disseminated intravascular coagulation disorder. These imbalances could be caused by chronic subclinical endotoxemia and cytokine activation after the initial portal thromboembolic event. The persistence of these abnormalities in adolescent patients indicates an ongoing coagulation derangement.     

Endoscopic band ligation followed by sclerotherapy: Is it superior to sclerotherapy in children with extrahepatic portal venous obstruction?

Background and Aim: There is scarcity of data about children on a combination of endoscopic variceal ligation (EVL) and endoscopic sclerotherapy (EST). We assessed the efficacy of EVL followed by EST and EST alone in children with extrahepatic portal venous obstruction (EHPVO). Methods: From January 2000 to March 2007, 186 consecutive children (mean age 6.3 ± 4.2 years, 82% boys) with EHPVO with variceal bleeding were included. EVL followed by EST (Group I, n = 101) or EST alone (Group II, n = 60) was carried out at 3‐weekly intervals until eradication. Surveillance endoscopy was done at 3 to 6‐monthly intervals. In all cases, the number of sessions required to eradicate the esophageal varices, the volume of sclerosant, the complications and the endoscopic outcome on follow up were recorded. Results: Eradication was achieved in 158 of 161 (98%) children and 25 were lost to follow up. Group I required significantly fewer sessions (5.2 ± 1.8 vs 6.8 ± 2.8, P < 0.005), less sclerosant (13 ± 8.2 mL vs 30 ± 20 mL, P < 0.001) and had fewer complications (7% vs 28%, P < 0.001) as compared with Group II. On follow up (33 ± 17.6 months in Group I and 43 ± 16.7 months in Group II), there was a significant increase in the prevalence of portal hypertensive gastropathy as well as isolated gastric varices in both the groups. However, the prevalence of gastroesophageal varices decreased. Conclusions: EVL followed by EST is better than EST alone in children with EHPVO as it requires fewer sessions and has fewer complications. However, following eradication, evolution of gastric varices and portal hypertensive gastropathy was similar in the two groups.     

心得安联合内镜套扎术预防食管静脉曲张再出血的疗效分析

目的比较心得安联合内镜套扎治疗与单独内镜套扎治疗预防食管静脉曲张再出血的疗效。方法 65例食管静脉曲张破裂出血的患者随机分为心得安联合内镜套扎治疗组(33例),单独内镜套扎治疗组(32例),平均随访12个月,比较两组间再出血率,门脉高压性胃病,食管静脉曲张复发和胃底静脉曲张的发生率。结果两组治疗后随访第6,12个月显示,与单独内镜套扎治疗比较,心得安联合内镜套扎治疗显著降低再出血率(15.2%vs 37.5%,21.2%vs 46.9%,P<0.05),门脉高压性胃病(18.2%vs43.8%,30.3%vs 56.3%,P<0.05),食管静脉曲张复发(15.2%vs 37.5%,24.2%vs 50.0%,P<0.05)和胃底静脉曲张的发生率(12.1%vs 34.4%,21.2%vs 46.9%,P<0.05)。结论心得安联合内镜套扎治疗是二级预防食管静脉曲张出血的首选治疗方法。     

Effects of Esophageal Varice Eradication on Portal Hypertensive Gastropathy and Fundal Varices: A Retrospective and Comparative Study

Esophageal varice eradication results in gastric hemodynamic changes. The aim of this study was to detect the influence of variceal eradication on portal hypertensive gastropathy (PHG) and fundal varices and to compare the results of two therapeutic methods (endoscopic variceal ligation and endoscopic sclerotherapy). A total of 114 consecutive patients with cirrhosis and portal hypertension who underwent elective endoscopic variceal ligation (EVL) (85 patients) or endoscopic sclerotherapy (EST) (29 patients) for obliteration of esophageal varices were selected for this study. Both groups were compared for PHG and fundal varice formation before and after eradication. Fifty-eight (68.2%) patients in the EVL and 18 (62.1%) patients in the EST group had PHG before esophageal varice eradication (P > 0.05). PHG grade after eradication of esophageal varices by both EVL and EST was significantly higher compared to pre-eradication. PHG grade and aggregation were similar in both groups. Thirty-seven patients (34 F₁, 3 F₂) in the EVL group and 13 patients (10 F₁, 3 F₂) in the EST group had fundal varices before variceal eradication (P > 0.05). Fundal varices were detected in 46 (35 F₁, 11F₂) and 19 (11F₁, 8F₂) patients in the EVL and EST groups after eradication, respectively. There was a statistically significant increment in occurrence of fundal varices after eradication with EVL and EST groups. There was no significant difference regarding fundal varice development after esophageal variceal eradication in both groups. After varical eradication, PHG was found in 57 (87.7%) and 39 (79.6%) patients with and without fundal varices, respectively (P > 0.05). Esophageal eradication with EVL and EST increases both the incidence and the severity of PHG and fundal varice formation. Both methods have comparable influences on PHG and fundal varices.     

Band ligation plus sclerotherapy versus sclerotherapy alone in children with extrahepatic portal venous obstruction

Although endoscopic band ligation (EVL) plus sclerotherapy (EST) has shown to be superior to any individual method, there is no study in children. We therefore analyzed our experience of EST and EVL + EST in children with extrahepatic portal venous obstruction (EHPVO). Over a period of 8 years, 136 children who presented with a history of recent variceal bleeding due to EHPVO were studied; 30 of them received EVL + EST and 106 received EST alone. In the EVL + EST group, after the first session of EVL, EST was done three times weekly until variceal eradication was achieved. The mean age of these children was 7 +/- 3.6 years with a male-to-female ratio of 2.6:1. The rate of eradication was comparable in both groups (100% in EVL + EST and 96% in EST). However, EVL + EST group required a significantly fewer sessions and lower volume of sclerosant (2 +/- 1 vs. 4.4 +/- 2 sessions, P < 0.001 and 3.1 +/- 2 mL versus 7.5 +/- 4 mL, P < 0.001 respectively). There were significantly less complications in EVL + EST group (10% vs. 36%, P < 0.01). Over a mean follow-up of 27 months, varices recurred in 6.6% and 10% cases, respectively. EVL + EST is a better method in the treatment of esophageal varices in children with EHPVO than EST alone, as it required fewer session and had fewer complication.     

Development of Gastroesophageal Varices and Risk of Variceal Bleeding in Patients With Cirrhosis

Abstract: We studied the relationships between portal pressure measured using the portal venous pressure gradient, the development of gastroesophageal varices, and the risk of variceal bleeding in 56 patients with cirrhosis. Portal pressure was higher in patients with varices than in those without (P>0.01), and 11 mmHg was the lowest portal pressure measured in the patients with varices. The size of the varices was not associated with the portal pressure. There was no difference in the value of portal pressure measurements for the patients with variceal bleeding and those without and there was no linear-relationship between the degree of portal hypertension and the rate of variceal bleeding. 12 mmHg was the lowest portal pressure measured in the patients with variceal bleeding. The size of the varices was related to the rate of variceal bleeding (P>0.05). We conclude that (a) a portal pressure of 11 mmHg is necessary for the formation of varices, (b) 12 mmHg of portal pressure is necessary for variceal bleeding to occur but the degree of portal hypertension has no predictive value for the risk of variceal bleeding, and (c) the size of the varices does not depend on the degree of portal hypertension but is associated with the risk of variceal bleeding.     

Rabbit model of non-cirrhotic portal fibrosis with repeated immunosensitization by rabbit splenic extract

BACKGROUND: Non-cirrhotic portal fibrosis (NCPF) or idiopathic portal hypertension, a disease of unknown etiology, is a common cause of portal hypertension in developing countries. Attempts to understand the etiopathogenesis of NCPF by developing animal models have been made. We describe a novel approach using repeated injections of rabbit splenic extract that were obtained from a previously primed rabbit, to develop a model of NCPF. METHODS: Twenty-eight rabbits (1.5-2.0 kg) were divided into the control (group I, n = 13) and the experimental (group II, n = 15) groups. The supernatant obtained after centrifugation of a 20% splenic homogenate, containing 6 mg protein/mL, was mixed with Freund's complete adjuvant (1:1 ratio) and injected intramuscularly to the recipient rabbits every 2 weeks for 3 months. Portal pressure was measured by inserting a cannula into the gastrosplenic vein. RESULTS: The mean portal pressure in group II was significantly (P < 0.05) higher than group I at 1 (19.4 +/- 2.9 vs 10.4 +/- 2.2 mmHg), 3 (16.7 +/- 1.1 vs 7.2 +/- 3.6 mmHg), and 6 (20.3 +/- 5.4 vs 10.3 +/- 4.8 mmHg) months. The mean splenic weight in group II was significantly (P < 0.05) greater than group I at 1, 3 and 6 months. Histopathology of spleen showed medullary congestion, hemosidrin laden macrophages and mild fibrosis. Liver showed normal hepatocytes with mild portal lymphocytic infiltrates and Kupffer cell hyperplasia. No significant anomalies were observed in the tests of liver function at 1 and 6 months. CONCLUSIONS: This animal model showed significant splenomegaly, with persistent rise in portal pressure without hepatic parenchymal injury, quite akin to NCPF seen in humans. This study also proposes that repeated immunostimulation may have an important role in the pathogenesis of NCPF.     

Two surgical procedures for esophagogastric variceal bleeding in patients with portal hypertension

AIM:To determine the clinical value of a splenorenal shunt plus pericardial devascularization(PCVD)in portal hypertension(PHT)patients with variceal bleeding.METHODS:From January 2008 to November 2012,290 patients with cirrhotic portal hypertension were treated surgically in our department for the prevention of gastroesophageal variceal bleeding:207 patients received a routine PCVD procedure(PCVD group),and83 patients received a PCVD plus a splenorenal shunt procedure(combined group).Changes in hemodynamic parameters,rebleeding,encephalopathy,portal vein thrombosis,and mortality were analyzed.RESULTS:The free portal pressure decreased to 21.43±4.35 mmHg in the combined group compared with24.61±5.42 mmHg in the PCVD group(P<0.05).The changes in hemodynamic parameters were more significant in the combined group(P<0.05).The long-term rebleeding rate was 7.22%in the combined group,which was lower than that in the PCVD group(14.93%),(P<0.05).CONCLUSION:Devascularization plus splenorenal shunt is an effective and safe strategy to control esophagogastric variceal bleeding in PHT.It should be recommended as a first-line treatment for preventing bleeding in PHT patients when surgical interventions are considered.     

Portal vein thrombosis： Etiology and clinical outcome of cirrhosis and malignancy-related non-cirrhotic, non-tumoral extrahepatic portal venous obstruction

The etiology and pathogenesis of portal vein thrombosis are unclear. Portal venous thrombosis presentation differs in cirrhotic and tumor-related versus non-cirrhotic and non-tumoral extrahepatic portal venous obstruction (EHPVO). Non-cirrhotic and non-tumoral EHPVO patients are young and present with well tolerated bleeding.Cirrhosis and tumor-related portal vein thrombosis patients are older and have a grim prognosis. Among the 118 patients with portal vein thrombosis, 15.3% had cirrhosis, 42.4% had liver malignancy (primary or metastatic), 6% had pancreatitis (acute or chronic), 5% had hypercoagulable state and 31.3% had idiopathy,12% had hypercoagulable state in the EHPVO group.     

Hepatoportal sclerosis (obliterative portal venopathy) and nodular regenerative hyperplasia in a patient with myasthenia gravis: A case report and review of the published work

Nodular regenerative hyperplasia (NRH) and hepatoportal sclerosis, also known as obliterative portal venopathy (OPV), are two causes of non‐cirrhotic portal hypertension (NCPH). NCPH is an increasingly recognized entity that can be seen in association with collagen vascular diseases and with the use of medications such as azathioprine and didanosine, but oftentimes the etiology remains unidentified. We herein report a case of NCPH occurring due to OPV and NRH in a 64‐year‐old woman with myasthenia gravis (MG), status post‐thymectomy. Portal hypertension was diagnosed incidentally on computed tomography in the absence of predisposing factors. Extensive work‐up to determine the etiology of any underlying liver disease was unrevealing. NRH and OPV were identified on liver biopsy. Subsequently, the patient had variceal bleeding that necessitated transjugular intrahepatic portosystemic shunt placement. A few similar cases of NCPH occurring in the setting of MG have been previously reported, suggesting that the immunological mechanisms involved in the pathogenesis of myasthenia may also have contributed to the development of NCPH.     

ENDOSCOPIC SCLEROTHERAPY FOR ESOPHAGO-GASTRIC VARICES: A CRITICAL REAPPRAISAL

Amongst the many non-surgical techniques for the treatment of variceal bleeding, endoscopic sclerotherapy (EST) has shown great promise. EST can successfully obliterate esophageal varices and prevent variceal rebleeding. It is also very effective in the control of active bleeding from esophageal varices. The technique of EST is simple and can be carried out with a conventional, forward viewing, flexible endoscope and a teflon injector. Weekly intravariceal injections of an aqueous sclerosant are prefer-rable. Though complications of EST in experienced hands are low, prophylactic EST at present should be advocated only to patients at high risk of bleeding. While controversy exists, most reports indicate that EST improves survival of patients with portal hypertension who have bled from esophageal varices. With regular follow-up endoscopics, recurrence of varices and bleeding from them can be substantially reduced. Sclerotherapy may successfully obliterate gastric varices in some patients either following EST for esophageal varices or by direct gastric variceal injections. For the long-term management of portal hypertension, combination of pharmacotherapy before as well as after eradication of esophageal varices, needs proper evaluation.