Isolated Gastric Varices & Portal Hypertension

7/18/2005

Question:  What disease process might be the cause of splenomegaly, portal hypertension, and gastric varices?

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15906778[PMID] or 14962658[PMID] or 15340666[PMID] or 15538159[PMID] or 15030033[PMID] or 14665393[PMID] or 14599933[PMID] or 12870732[PMID] or 12605645[PMID] or 12568521[PMID] or 12556142[PMID] or 12408529[PMID] or 12084024[PMID] or 11928079[PMID] or 11895549[PMID]

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<1>

Unique Identifier [PMID]: 15906778

Authors: Koklu S. Yuksel O. Arhan M. Sahin C. Basar O. Yolcu OF. Ucar E. Ibis M. Ertugrul I. Sahin B.

Institution: Department of Gastroenterology, Turkiye Yuksek Ihtisas Hospital, Ankara, Turkey. gskoklu@yahoo.com

Title: Report of 24 left-sided portal hypertension cases: a single-center prospective cohort study.

Source: Digestive Diseases & Sciences. 50(5):976-82, 2005 May.

Abstract: Our aim was to analyze patients diagnosed with left-sided portal hypertension prospectively and to document the complications at follow-up. Twenty-four patients with isolated splenic vein thrombosis (diagnosed by ultrasonography or angiography or intraoperatively) and/or isolated fundal varices (diagnosed by endoscopy or endosonography) were involved in this study. Demographics, clinical presentation, diagnostic and therapeutic procedures, and morbidity and mortality were recorded in their follow-up. There were 11 and 13 left-sided portal hypertension cases associated with pancreatic diseases and nonpancreatic disorders, respectively. Chronic abdominal pain and gastrointestinal bleeding were the two most common complaints. All patients except one had isolated esophageal (2 cases) or fundal (21 cases) varices. Thirteen patients had splenomegaly on ultrasonography. On Doppler sonography, the splenic vein could be evaluated in 21 of the 24 patients (9 and 6 had complete and partial occlusion, respectively, and 6 had patent blood flow). Urgent intervention with therapeutic endoscopy and splenectomy was performed for two patients each. Medical therapy was begun for three patients according to the underlying diseases. Three patients underwent elective surgery. Two patients were lost to follow-up after the first visit and the mean follow-up of the remaining 22 patients after diagnosis of left-sided portal hypertension was 20 months. Only one patient (with pancreas cancer) had gastrointestinal bleeding at follow-up. All patients with pancreas and gastric cancer died within 2-12 months. Left-sided portal hypertension has various etiologies. It may be difficult to diagnose this entity both endoscopically and radiologically. Treatment should be directed at the underlying diseases. Recurrent hemorrhage due to left-sided portal hypertension is not usual and the prognosis depends mainly on the underlying etiology.

Publication Type: Journal Article.

<2>

Unique Identifier [PMID]: 14962658

Authors: Romano M. Giojelli A. Capuano G. Pomponi D. Salvatore M.

Institution: Department of Radiology and Radiotherapy, University 'Federico II', 5, Via Pansini, 80131 Naples, Italy. maurizio.romano@ibb.cnr.it

Title: Partial splenic embolization in patients with idiopathic portal hypertension.

Source: European Journal of Radiology. 49(3):268-73, 2004 Mar.

Abstract: PURPOSE: To evaluate the effectiveness of partial splenic embolization (PSE) in patients with idiopathic portal hypertension (IPH) in reducing variceal bleeding episodes, splenomegaly and thrombocytopenia. MATERIALS AND METHODS: Six patients (2M, 4F, mean age 30.3 years) with IPH presenting with splenomegaly, thrombocytopenia and recurrent variceal bleeding were treated with PSE using gelatin sponge (four patients) or Contour particles (two patients) as embolization material. RESULTS: PSE was performed successfully in all cases; 3F coaxial microcatheters were necessary in two patients due to extreme splenic artery tortuosity. The average amount of devascularized parenchyma at CT 1 week after PSE was 71%. Splenomegaly and thrombocytopenia improved in all cases, with a mean platelet count increase of 120,000/mm(3) and an average 68% reduction of spleen volume at follow up. Variceal bleeding did not recur after PSE. Esophageal or gastroesophageal varices disappeared (one patient) or significantly reduced (five patients) at endoscopic controls. No significant complications were noted. The follow up was of at least 18 months in all patients; mean follow up was 28.2 months. CONCLUSION: In patients with IPH PSE can be effective in preventing variceal bleedings, in reducing spleen volume and in significantly increasing platelet count; therapeutic results were durable in our population.

Publication Type: Clinical Trial. Journal Article.

<3>

Unique Identifier [PMID]: 15340666

Authors: Wong HK. Kuo HT. Tsai SL. Uen YH. Tzeng WS.

Institution: Division of Gastroenterology, Department of Internal Medicine, Chi-Mei Foundation Hospital, Tainan, Taiwan.

Title: Isolated gastric varices due to focal splenic vein stenosis.

Source: Journal of the Formosan Medical Association. 103(8):640-3, 2004 Aug.

Abstract: Left-sided portal hypertension due to splenic vein stenosis is a very rare disease. We report a case of this condition in a 21-year-old woman who suffered from a first episode of tarry stool passage with fresh blood vomiting. Panendoscopy showed isolated gastric varices while sonography showed a normal liver but the presence of splenomegaly with prominent collateral circulations. Further imaging studies, including abdominal computed tomography, splenoportography and percutaneous transhepatic portography, revealed a focal stenotic proximal splenic vein resulting in left-sided portal hypertension. The collateral circulation ran from the short gastric veins via the left gastric veins into the main portal vein. The intraportal venous pressure was within normal limits. Splenectomy was performed and near normal wedge liver biopsy pathology confirmed non-cirrhotic extrahepatic portal hypertension. The patient had no further variceal bleeding after surgery.

Publication Type: Case Reports. Journal Article.

<4>

Unique Identifier [PMID]: 15538159

Authors: Ishihara K. Ishida R. Saito T. Teramoto K. Hosomura Y. Shibuya H.

Institution: Department of Radiology, Sano Kosei General Hospital, Tochigi, Japan. ishrmrad@tmd.ac.jp

Title: Computed tomography features of portal hypertensive gastropathy.

Source: Journal of Computer Assisted Tomography. 28(6):832-5, 2004 Nov-Dec.

Abstract: OBJECTIVE: The aim of this study was to detect the computed tomography features in portal hypertensive gastropathy (PHG). METHODS: This study included 32 patients with portal hypertension. Ten of 32 patients were diagnosed with (PHG), and the other 22 patients were not diagnosed with PHG on the basis of upper endoscopic examination. Dynamic computed tomography studies were performed in all 32 patients. Each computed tomography scan was evaluated with regard to whether the gastric inner layer was enhanced or not. RESULTS: Nine patients with PHG had delayed enhancement on the inner layers of gastric walls, but in 17 of 22 patients without PHG, delayed enhancement was not observed. CONCLUSION: Enhancement on the inner layer of gastric walls may reflect gastric congestion. Portal hypertensive gastropathy should be suspected when this finding is detected on computed tomography scans in patients with portal hypertension.

Publication Type: Journal Article.

<5>

Unique Identifier [PMID]: 15030033

Authors: Shah SR. Mathur SK.

Institution: Gastroenterology Surgical Services, King Edward VII Memorial Hospital, Mumbai 400 012.

Title: Presentation and natural history of variceal bleeding in patients with portal hypertension due to extrahepatic portal venous obstruction.

Source: Indian Journal of Gastroenterology. 22(6):217-20, 2003 Nov-Dec.

Abstract: BACKGROUND: The natural history of portal hypertension due to extrahepatic portal venous obstruction (EHPVO) in the presence of a non-cirrhotic liver is not well understood. AIM: To evaluate the presenting features and the natural history of EHPVO by assessing the bleed patterns prior to definitive management in these patients. METHODS: Two hundred and seven consecutive patients presenting with history of variceal bleeding due to EHPVO were studied prospectively. Clinical, hematological and biochemical features on presentation, and pattern and severity of bleeding prior to institution of endoscopic therapy or surgery were recorded. RESULTS: Ascites was observed in 16% and was invariably transient. Splenomegaly was present in 82%, with hypersplenism in 22%; however, hypersplenism was symptomatic in only 6% of patients. In 127 patients having more than one episode of bleeding, the frequency of bleeding episodes was 0.94/year and transfusion requirment was 1.47 units/year. No fixed pattern of frequency of variceal bleeding was identified. However, number of bleed episodes and transfusion units requirement correlated with each other. The maximum severity of bleeding was between the ages of 12 and 19 years. CONCLUSION: Hypersplenism causing symptoms is rare in EHPVO presenting with variceal bleed.

Publication Type: Journal Article.

<6>

Unique Identifier [PMID]: 14665393

Authors: Tanabe M.

Institution: Department of Tropical Medicine and Parasitology, School of Medicine, Keio University, Tokyo 160-8582, Japan. tanabe@sc.itc.keio.ac.jp

Title: Haemostatic abnormalities in hepatosplenic schistosomiasis mansoni. [Review] [68 refs]

Source: Parasitology International. 52(4):351-9, 2003 Dec.

Abstract: Hepatosplenic schistosomiasis is a complex immuno-regulatory disease and is major health problem in endemic countries. Acute bleeding is one of its most serious complications and often life-threatening. Clinical studies have demonstrated that the patients with hepatosplenic schistosomiasis are prone to develop complex haemostatic abnormalities that may be linked to the potential risk of bleeding from ruptured esophageal varices in these patients. The deficit in haemostatic parameters is more pronounced with the advancement of the disease and is maximal in the patients with experience of haematomesis. Evidences of enhanced generation of thrombin and plasmin indicate the presence of low-grade DIC in advanced hepatosplenic schistosomiasis, which is considered as a principal cause of haemostatic abnormalities in this endemic disease. Demonstration of procoagulant expression in peripheral blood monocytes of the patients and in the livers, spleens and intestines of S. mansoni-infected mice suggest their possible implication in the causation of DIC in S. mansoni infections. Moreover, because in vitro analysis indicates a participation of immune mechanisms in the localized procoagulant expression, it seems likely that the immune responses to schistosomes play a major role in the pathogenic mechanisms of haemostatic abnormalities in hepatosplenic schistosomiasis. [References: 68]

Publication Type: Journal Article. Review. Review, Tutorial.

<7>

Unique Identifier [PMID]: 14599933

Authors: Zhou GW. Tao ZY. Peng CH. Li HW.

Institution: Surgical Department and Shanghai Digestive Surgical Institute, Ruijin Hospital, Shanghai Second Medical University, Shanghai 200025, China. gw_vrai@yahoo.com.cn

Title: Reasonable choice of surgical procedures for patients with portal hypertension. [Review] [11 refs]

Source: Hepatobiliary & Pancreatic Diseases International. 2(3):330-3, 2003 Aug.

Abstract: OBJECTIVE: To assess individualized therapeutic protocol for patients with portal hypertension on the basis of accumulated knowledge about the mechanism of portal hypertension. DATA SOURCES: Patients data on shunt and other surgical procedures from Ruijin Hospital, Shanghai, China and the published papers. RESULTS: The direction of blood flow of the collateral vessels in the gastro-splenic region is an important factor in deciding surgical strategy because there is a close relationship between surgical risk and the classification of liver function. Clinically it is confirmed that each patient needs an individualized surgical procedure and that prophylactic operation is suitable for patients with splenomegaly, splenism associated with serious esophageal varices and hemorrhagic tendency under endoscopy but acceptable liver function. The shunt diameter (SD) (SD = 0.67 x PVD) is determined in our patients according to individualized hemodynamics. The rehemorrhagic rate after shunt being higher than that in others may be related to lesioned gastric mucosa caused by portal hypertension or bleeding and temporary melena. This finding is good for prevention of hepatic encephalopathy. The life quality and labor ability of patients will be improved because of hepatopetal flow in the portal vein. With strict indications for reoperation, selective operation is performed as soon as possible when hemorrhage is controlled conservatively and liver function improved. Once the patient with cirrhosis associated with portal hypertension is scheduled for liver transplantation, treatment of hemorrhage should aim to keep the patient in good condition and to avoid the protocol that may be disadvantageous to liver transplantation in the future. CONCLUSION: Surgical procedures for patients with portal hypertension should follow the principle of individualization. To obtain the best outcome, the choice of reasonable surgical procedure is expected. [References: 11]

Publication Type: Journal Article. Review.

<8>

Unique Identifier [PMID]: 12870732

Authors: Thomopoulos KC. Labropoulou-Karatza C. Mimidis KP. Katsakoulis EC. Iconomou G. Nikolopoulou VN.

Institution: Department of Internal Medicine, Division of Gastroenterology. University Hospital of Patras, Patras, Greece.

Title: Non-invasive predictors of the presence of large oesophageal varices in patients with cirrhosis.

Source: Digestive & Liver Disease. 35(7):473-8, 2003 Jul.

Abstract: BACKGROUND/AIMS: The usual clinical practice is to screen all patients with established cirrhosis at the time of diagnosis by upper endoscopy for the presence of varices. Patients with large varices should be treated with non-selective beta blockers to reduce the incidence of first variceal bleeding. However, fewer than 50% of cirrhotic patients have varices at screening endoscopy and most have small sized varices, with a low risk of bleeding. The aim of the present study was to determine whether clinical or laboratory non-endoscopic parameters could predict the presence of large oesophageal varices. PATIENTS/METHODS: Seventeen variables considered relevant to the prevalence of oesophageal varices were tested in 184 patients with cirrhosis, who underwent screening endoscopy. Small varices were regarded as those which flatten with insufflation or slightly protrude into the lumen, while large varices are those which protrude into the lumen or touch each other. None of the patients was on beta blockers or other vasoactive drugs or had a history of variceal bleeding. RESULTS: Oesophageal varices were present in 92 patients (50%), and large varices in 33 patients (17.9%).Variables associated with the presence of large oesophageal varices on univariate analysis were the presence of ascites and splenomegaly either by clinical examination or by ultrasound (p < 0.01), the presence of spiders (p = 0.02), platelet count (p < 0.0001), and bilirubin (p = 0.01). Factors independently associated with the presence of large oesophageal varices on multivariate analysis were platelet count, size of spleen and presence of ascites by ultrasound. Using mean values as cut-off points, it is noteworthy that only five out of 39 patients (12.8%) with platelets > or = 18(x 10(9)/l), spleen length < or = 135 mm and no ascites had varices. Moreover, all these patients had small sized varices. On the other hand, 15 out of 18 patients (83.3%) with a platelet count < 118 x 10(9)/l, spleen length > 135 mm and ascites had varices. Moreover, five out of those 18 patients had large varices (28.3%). CONCLUSION: Thrombocytopenia, splenomegaly and ascites are independent predictors of large oesophageal varices in cirrhotic patients. We suggest that endoscopy could be avoided safely in cirrhotic patients with none of these predictive factors, as large varices are absent in this group of patients.

Publication Type: Journal Article.

<9>

Unique Identifier [PMID]: 12605645

Authors: Ibarrola C. Colina F.

Institution: Pathology Department, University Hospital Doce de Octubre, Madrid, Spain.

Title: Clinicopathological features of nine cases of non-cirrhotic portal hypertension: current definitions and criteria are inadequate.

Source: Histopathology. 42(3):251-64, 2003 Mar.

Abstract: AIMS: The clinicopathological features of nine patients with non-cirrhotic portal hypertension were studied and an attempt was made to apply the descriptive criteria of experts to the morphological alterations of the livers in order to classify them adequately. METHODS AND RESULTS: Clinical and biochemical data and the alterations in livers resected at transplantation (n=7) or at autopsy (n=2) were gathered in five males and four females (ages 15-78 years) without aetiological factors for chronic hepatic disease who had oesophageal varices and splenomegaly in the absence of typical cirrhosis. Noting the luminal obstruction of the three hepatic vascular trees, hyperplastic nodule size and distribution, and the density of fibrosis, an attempt was made to assign each case to one of the following diagnostic categories: idiopathic portal hypertension, diffuse nodular regenerative hyperplasia, partial nodular transformation and incomplete septal cirrhosis. When a case could not be categorized into one of these groups, it was listed as non-cirrhotic irregular architectural transformation. Only three cases could be assigned to one pure diagnostic category (two diffuse nodular regenerative hyperplasias and one incomplete septal cirrhosis). Three other cases could not be classified due to the heterogeneity of their lesions. In the remaining three cases, the hepatic morphology was a mixture of hilar partial nodular transformation combined with another abnormal architectural pattern in the peripheral parenchyma: diffuse nodular regenerative hyperplasia in two cases and idiopathic portal hypertension in the other. In seven cases, old thromboses in the hilar portal tree were observed. Stenoses were observed in some of the arterial branches in five cases and in some hepatic venous branches in four. However, no obstructions could be discovered in small or large portal veins in the two classical diffuse nodular regenerative hyperplasia cases. CONCLUSIONS: The hepatic morphology in this group of non-cirrhotic portal hypertension patients was an abnormal remodelling of the liver associated with the frequent development of irregular hyperplastic nodules and frequent obstructions of the pre- and intrahepatic vascular lumens. It was very difficult to apply the nomenclature proposed by international experts.

Publication Type: Journal Article.

<10>

Unique Identifier [PMID]: 12568521

Authors: Mene A. Sharma D. Raina VK.

Institution: GI Surgery Unit, Department of Surgery, NSCB Government Medical College and Allied Hospitals, Jabalpur (MP), India 482003.

Title: Correlation between serum-ascites albumin concentration gradient with gastrointestinal bleeding in patients of portal hypertension.[erratum appears in Trop Doct. 2003 Oct;33(4):254].

Source: Tropical Doctor. 33(1):39-41, 2003 Jan.

Abstract: Non-availability of endoscopic screening to detect portal hypertensive patients at a high risk of index gastrointestinal (GI) haemorrhage led us to examine the relationship between serum-ascitic albumin concentration gradient (SAAG) measurements and the occurrence of GI haemorrhage in patients with portal hypertension and ascites. Fifty-six consecutive patients of portal hypertension with ascites attending the GI surgery outpatients clinic were divided into two groups on the basis of history: (a) those who had no history of GI bleeding; and (b) those who had an episode of GI bleeding within the past 21 days. Child-Pugh score was calculated for all patients. All the patients were assessed by ultrasonography and SAAG was estimated. Groups (a) and (b) were compared and sensitivity, specificity, positive and negative predictive value of using SAAG as a possible screening test for GI bleeding were calculated. SAAG values correlated significantly with bleeding and splenomegaly. For prediction of bleeding, SAAG had a sensitivity of 100% and specificity of 33.33%, positive predictive value of SAAG for GI bleeding was 71.4% and the negative predictive value was 100%. Estimation of SAAG is possible even in a small, modestly equipped laboratory, and could provide a new means for the identification of high-risk patients for GI bleeding and define patients more accurately for future clinical studies. The results of the present study are encouraging for clinicians working in developing countries and may help in optimizing prophylactic therapy and where available, improving the cost effectiveness of screening endoscopy.

Publication Type: Evaluation Studies. Journal Article.

<11>

Unique Identifier [PMID]: 12556142

Authors: Chou YH. Chiou HJ. Tiu CM. Chiou SY. Lee SD. Hung GS. Wu SC. Kuo BI. Lee RC. Chiang JH. Chang T. Yu C.

Institution: Department of Radiology, Veterans General Hospital-Taipei and National Yang Ming University, School of Medicine, Taipei Taiwan. cmtiu@vghtpe.gov.tw

Title: Duplex Doppler ultrasound of hepatic Schistosomiasis japonica: a study of 47 patients.

Source: American Journal of Tropical Medicine & Hygiene. 68(1):18-23, 2003 Jan.

Abstract: This study describes the ultrasound (US) appearances of the liver with hepatic schistosomiasis japonica (HSJ), and studies the portal hemodynamics in 47 patients with HSJ using duplex Doppler US over a period of 15 years. All patients but two were Chinese war veterans seen in Taiwan about 35-55 years after their presumed infection in Mainland China. The US presentations were reviewed. The data from Doppler portal flow studies were available for 39 patients with HSJ, and compared to data from Doppler portal flow studies in 40 normal healthy volunteers and to this data in 40 patients with postnecrotic cirrhosis. A typical "coarse reticular pattern" due to fibrosis in the whole liver was noted in 40 patients (85%). Other findings included periportal fibrosis (15%), septum-like fibrous bands extending to the liver capsule (32%), and an apparent nodular liver surface (19%). Splenomegaly was noted in seven patients. While coexisting hepatocellular carcinomas (HCC) were evident in three patients, and esophageal varices were found in three others, yet both conditions were found only in patients with positive hepatitis-B-surface antigen (HBsAg). Doppler flowmetry of the portal veins in HSJ patients showed a mean flow rate of 15.34 +/- 6.82 cm/sec, and a mean flow volume of 993.21 +/- 290.63 ml/min, both showed no significant difference from those in normal adults (p > 0.5). HSJ can be confidently diagnosed in patients with hepatic fibrosis when the hepatic pathology is presented as a coarse reticular pattern. The portal hemodynamics in HSJ patients who have been isolated from the infection site (for more than 35 years) are significantly different from portal hemodynamics in cirrhotic patients and are similar to those in healthy volunteers.

Publication Type: Journal Article.

<12>

Unique Identifier [PMID]: 12408529

Authors: Piscaglia F. Donati G. Cecilioni L. Celli N. Stagni B. Pini P. Gaiani S. Gherlinzoni F. Bolondi L.

Institution: Dept of Internal Medicine and Gastroenterology, University of Bologna, Italy. Piscagl@med.unibo.it

Title: Influence of the spleen on portal haemodynamics: a non-invasive study with Doppler ultrasound in chronic liver disease and haematological disorders.

Source: Scandinavian Journal of Gastroenterology. 37(10):1220-7, 2002 Oct.

Abstract: BACKGROUND: Splanchnic haemodynamic parameters for the differential diagnosis of splenomegalies of different origins are still suboptimal and the role of spleen enlargement in cirrhosis remains controversial. In an attempt to elucidate these questions, we assessed splanchnic haemodynamics in chronic liver diseases and various other disorders with splenomegaly. METHODS: Study groups comprised: (i) patients with chronic liver disease (89 with cirrhosis, 35 with chronic hepatitis), (ii) patients with splenomegaly without relevant portal hypertension (14 with haematological splenomegaly and 25 liver transplant recipients without complications), (iii) 15 patients with arterial hypertension, (iv) 22 healthy controls. In all subjects, spleen size, portal flow parameters and splenic artery resistance index were measured using duplex-Doppler ultrasound. RESULTS: Splenic artery resistance index was significantly and selectively increased in patients with cirrhosis (0.63, whereas all other group means ranged between 0.53 and 0.56; P < 0.01). Portal flow velocity was significantly decreased in cirrhosis (P < 0.01). The combination of these two parameters provided an accuracy of 87.5% in distinguishing portal hypertensive from haematological splenomegaly. In patients with cirrhosis, the degree of spleen enlargement was positively correlated with increasing portal flow volume, portal vein diameter and variceal size, whereas splenic resistance index and portal velocity did not differ in connection with spleen size. CONCLUSIONS: Splenoportal Doppler sonography provides specific findings in cirrhosis and may therefore be a useful tool in differentiating between splenomegaly of portal hypertensive or haematological origin. In patients with cirrhosis, the presence of splenomegaly is associated with the presence of larger oesophageal varices.

Publication Type: Journal Article.

<13>

Unique Identifier [PMID]: 12084024

Authors: Sarin SK. Kapoor D.

Institution: Department of Gastroenterology, GB Pant Hospital, New Delhi, India. sksarin@nda.vsnl.net.in

Title: Non-cirrhotic portal fibrosis: current concepts and management. [Review] [61 refs]

Source: Journal of Gastroenterology & Hepatology. 17(5):526-34, 2002 May.

Abstract: Non-cirrhotic portal hypertension (NCPH) comprises 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 is near normal, measurement of intravariceal or intrasplenic pressure is needed to assess PP. The majority of 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 that present only with features of PHT and are common in developing countries are non-cirrhotic portal fibrosis (NCPF) and extrahepatic portal vein obstruction (EHPVO). Non-cirrhotic portal fibrosis is a syndrome of obscure etiology, characterized by 'obliterative portovenopathy' leading to PHT, massive splenomegaly and well-tolerated episodes of variceal bleeding in young adults from low socioeconomic backgrounds, having near normal hepatic functions. In some parts of the world, NCPF is called idiopathic portal hypertension (IPH) or 'hepatoportal sclerosis'. Because 85-95% of patients with NCPF and EHPVO present with variceal bleeding, treatment involves management with endoscopic sclerotherapy (EST) or variceal ligation (EVL). These therapies are effective in approximately 90-95% of patients. Gastric varices are another common cause of upper gastrointestinal bleeding in these patients and these can be managed with cyanoacrylate glue injection or surgery. Other indications for surgery include failure of EST/EVL, and symptomatic hypersplenism. The prognosis of patients with NCPF is good and 5 years survival in patients in whom variceal bleeding can be controlled has been reported to be approximately 95-100%. Copyright 2002 Blackwell Publishing Asia Pty Ltd [References: 61]

Publication Type: Journal Article. Review. Review, Tutorial.

<14>

Unique Identifier [PMID]: 11928079

Authors: Okudaira M. Ohbu M. Okuda K.

Institution: Department of Pathology, Kitasato University School of Medicine, Sagamihara, Kanagawa, Japan.

Title: Idiopathic portal hypertension and its pathology. [Review] [66 refs]

Source: Seminars in Liver Disease. 22(1):59-72, 2002 Feb.

Abstract: Idiopathic portal hypertension (IPH) is a disorder of unknown etiology, clinically characterized by portal hypertension (varices and portosystemic collateral vessels), splenomegaly, and anemia (hypersplenism). A similar disorder is called noncirrhotic portal fibrosis in India, and hepatoportal sclerosis seems to be the counterpart in the United States. This disease is uncommon in developed countries. Middle-aged women are more prone to IPH in Japan. The liver has no cirrhosis or pseudonodule formation, and the principal pathologic changes are considerable portal fibrosis, devastation of intrahepatic terminal portal radicles, and parenchymal atrophy of the liver secondary to portal malperfusion. The characteristic portal hemodynamics include intrahepatic presinusoidal portal hypertension, increased splenic and portal vein blood flow, and increased intrahepatic portal resistance. The prognosis is generally good depending on the management of bleeding varices. Although the etiology is obscure, certain immunologic abnormalities seem to play an etiologic role in Japanese patients, and the incidence has markedly declined in recent years in Japan, indirectly suggesting a role of infection. The theory that IPH represents an undiagnosed intrahepatic portal vein thrombosis is refuted. [References: 66]

Publication Type: Journal Article. Review. Review, Tutorial.

<15>

Unique Identifier [PMID]: 11895549

Authors: Dhiman RK. Chawla Y. Vasishta RK. Kakkar N. Dilawari JB. Trehan MS. Puri P. Mitra SK. Suri S.

Institution: Department of Hepatology, Postgraduate Institute of Medical Education and Research, Chandigarh, India. rkpsdhiman@hotmail.com

Title: Non-cirrhotic portal fibrosis (idiopathic portal hypertension): experience with 151 patients and a review of the literature.[see comment]. [Review] [57 refs]

Comments Comment in: J Gastroenterol Hepatol. 2002 Jan;17(1):1-5; PMID: 11895548

Source: Journal of Gastroenterology & Hepatology. 17(1):6-16, 2002 Jan.

Abstract: 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. [References: 57]

Publication Type: Journal Article. Review. Review, Tutorial.