Non-cirrhotic Portal Fibrosis Research Articles

Hepatoportal sclerosis in a child

Hepatoportal sclerosis (non-cirrhotic portal fibrosis) is characterised by portal hypertension, splenomegaly and variceal bleeding. It is rarely seen in childhood. We report on hepatoportal sclerosis in a patient with haematemesis as a presenting symptom. This 11-year-old male patient was admitted with upper gastrointestinal bleeding (UGB). He was observed for UGB for the first time at the age of 6 years at another hospital and was treated with sclerotherapy. Afterwards he had no bleeding from varices until admission to our hospital. At the age of 6 months, he had been admitted and treated with antibiotics and intravenous fluid because of diarrhoea. On physical examination, bodyweight and height were at the 10th–25th percentile, the spleen was enlarged (7 cm under the costal margin) but the liver was not palpable. Therewas no collateral circulation on the abdominal skin. On Doppler ultrasonography, the liver parenchyma was coarsely granular, periportal hyperechogenicity was detected, and the inferior vena cava and hepatic veins were patent.At the portal hilus, a vascular structure resembling cavernous transformation or a recanalised portal veinwas seen. The portal vein diameter was enlarged with multiple collaterals. Haemoglobin was 10.1 g/dl, leukocytes 4100/ mm, thrombocytes 145000/mm, prothrombin time 18 s (reference range 11–15 s), activated partial thromboplastin time 35 s (reference range 25–35 s), international normalised ratio (INR) 1.5, blood glucose, renal function (BUN 10 mg/dl, creatinine 0.6 mg/dl) and electrolytes

Cardiac dysfunction in portal hypertension among patients with cirrhosis and non-cirrhotic portal fibrosis

Background/Aims: In cirrhosis, diastolic dysfunction of heart is well documented. Contribution of portal hypertension towards cardiac changes in cirrhosis is difficult to assess. We examined the patients of non-cirrhotic portal fibrosis who have portal hypertension without liver insufficiency to understand the contribution of portal hypertension in causing cardiac changes. Methods: Cardiac function was studied in four groups of patients: normal controls, patients with non-cirrhotic portal fibrosis (having portal hypertension without liver dysfunction) and cirrhotics with and without ascites. Cardiac function was evaluated by echocardiography. Additional measurements of plasma renin activity and aldosterone levels were performed. Results: Diastolic function as assessed by the ratio between E wave and A wave (E/A ratio), was significantly lower in patients with non-cirrhotic portal fibrosis (median 1.3) compared to normal controls (median 1.52). However, even lower values were observed in cirrhotics without ascites (median 1.05) and with ascites (median 0.94). There was a significant correlation ( r=−0.75) between plasma aldosterone levels and the E/A ratio in cirrhotics. Conclusions: Diastolic dysfunction is not only present in cirrhosis but also in non-cirrhotic portal fibrosis. It indicates that portal hypertension is an important factor in the genesis of cardiac dysfunction.

286 Portal hypertensive gastropathy is prevalent amongst chronic hepatitis C non-responders with bridging fibrosis and early cirrhosis: results from the HALT-C trial

OBJECTIVE:The natural history and likelihood of bleeding from portal hypertensive gastropathy (PHG) present in patients with portal hypertension before endoscopic variceal obliteration may differ from that in patients who develop PHG during or after variceal eradication.METHODS:A total of 967 variceal bleeders who had achieved variceal eradication by endoscopic sclerotherapy in the recent past were prospectively studied. In all, 88 (9.1%) patients (cirrhosis in 54, noncirrhotic portal fibrosis in 18, and extrahepatic portal vein obstruction in 16) had distinct mucosal lesions. PHG alone was present in 78, PHG with gastric antral vascular ectasia (GAVE) in eight, and GAVE alone in two patients. PHG was graded as mild or severe and according to whether present before (group A) or after endoscopic intervention (group B). Patients underwent regular endoscopy at follow-up to see if the PHG was transitory (disappearing within 3 months), persistent (no change), or progressive. Bleeding from PHG lesions was defined as acute or chronic.RESULTS:Twenty-two (26%) patients had PHG before (group A) and 64 (74%) developed PHG after variceal eradication (group B). During a mean follow-up of 25.1 ± 14.2 months, PHG lesions disappeared in group A in only two patients (9%), but in group B in 28 (44%) patients (p < 0.05). PHG lesions more often progressed in the former as compared to the latter (18% vs 9.4%), p = NS). The incidence of bleeding was higher in group A than group B (32% vs 4.7%, p < 0.02). Bleeding from PHG occurred in 10 patients (11.6%); seven of them were from group A, and all had either progressive (n = 3) or persistent (n = 4) lesions.CONCLUSIONS:PHG developing after variceal eradication is often transitory and less severe. If PHG is pre-existing, endoscopic therapy for varices could worsen the PHG, with a likelihood of bleeding. Such patients may be benefited by concomitant beta-blocker therapy.

Isolated Gastric Varices: Prevalence, Clinical Relevance and Natural History

Background: Isolated ectopic gastric varices (IGV2) are present either in the body or antrum of the stomach or upper duodenum. The prevalence, natural history and clinical significance of these varices has not been adequately described. Materials and Methods: Consecutive patients with portal hypertension, prospectively studied and diagnosed to have IGV2, were assessed for their time of appearance – primary (at first presentation) or secondary (after obliteration of oesophageal varices), association with other varices, portal hypertensive gastropathy and any overt bleeding. Results: Fifty-three of the 1,128 (4.7%) patients had IGV2. The IGV2 were commonly seen in the antrum (53%), duodenum (32%), or at both sites (11%) and rarely in body and fundus (4%). IGV2 were predominantly (84.9%) secondary in origin, developing after oesophageal variceal obliteration. The median time for emergence of secondary IGV2 was 8.2 months for patients with cirrhosis, 12.8 months for non-cirrhotic portal fibrosis and 10.8 months for extra-hepatic portal vein obstruction. Eight (15%) patients had primary IGV2, 6 of them had underlying portal vein obstruction. Portal gastropathy (p < 0.05) and UGI bleeding were more common in the secondary than in primary IGV2. Bleeding due to IGV2 was seen only in 3 (5.7%) patients during a mean follow-up of 36.3 ± 12.1 months, and could be successfully managed with endoscopic ligation or obliteration. Conclusions: Isolated ectopic gastric varices are not uncommon and generally develop following obliteration of main variceal columns. They rarely bleed and often can be managed with endoscopic interventions.

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%.

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.

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

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. 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. 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. 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.

Endoscopic sclerotherapy of gastric variceal bleeding with N-butyl-2-cyanoacrylate.

bleeding from gastric varices is a life-threatening complication of portal hypertension. Fundal and isolated gastric varices are at high risk for variceal bleeding. In this study, we report our experience with n-butyl-2-cyanoacrylate (BC) in patients with large gastric varices. twenty-nine patients (15 male, 14 female) with large fundal varices (active bleed, 5; passive bleed after eradication of esophageal varices, 13; unbled fundal varices with red color sign, 11) underwent endoscopic sclerotherapy with BC. Cirrhosis was present in 13 patients; extrahepatic portal venous obstruction, in 13; and noncirrhotic portal fibrosis, in 3. N-Butyl-2-cyanoacrylate after mixing with lipiodol (1:1) was given to the initial 10 patients and was given in undiluted form to the remaining patients, followed by injection of 0.7 mL of distilled water to rinse the injection catheter. One to three injections (0.5-1 mL) were given until all gastric varices became hard. All patients were on long-term endoscopic sclerotherapy or variceal ligation programs for eradication of esophageal varices. acute variceal bleeding was controlled in all five patients with BC injections. Eradication of gastric varices was achieved in 27 (93.1%) patients (20 patients in 1 session, 4 patients in 2, and 3 patients in 3-6). Rebleeding occurred in three (10.3%) patients who responded to repeat BC injections. Complications related to the procedure occurred in two (6.9%) patients. In one patient, the needle became impacted into the tissue adhesive. This patient died 5 days later because of massive upper gastrointestinal bleeding. In the other patient, there was distal embolization. sclerotherapy of gastric varices with BC is a safe and an effective treatment for control of bleeding and eradication. The needle should be withdrawn immediately after the BC injection to prevent its impaction into the tissue adhesive.

Non-cirrhotic portal fibrosis: current concepts and management.

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%.

Prevalence of autonomic dysfunction in cirrhotic and noncirrhotic portal hypertension.

Autonomic dysfunction is common in patients with cirrhosis of the liver, but more so in patients with decompensated state, and is associated with increased mortality. We evaluated the presence and extent of autonomic dysfunction in patients with extrahepatic portal venous obstruction (EHPVO) and noncirrhotic portal fibrosis (NCPF), diseases with relatively preserved liver functions. Heart rate variability in response to standing, deep breathing, and Valsalva maneuver and blood pressure response to sustained handgrip and standing were studied in 18 patients with EHPVO (13 mol/L, 5 F, mean age 15.2 +/- 6 yr), 12 patients with NCPF (5 mol/L, 7 F, mean age 26.4 +/- 8 yr), 15 patients with cirrhosis (7 mol/L, 8 F, mean age 12.6 +/- 6 yr), and 17 healthy controls (11 mol/L, 6 F, mean age 18.6 +/- 3 yr). Time-domain parameters of heart rate variability on 24-h ambulatory monitoring were assessed in all the patients. Autonomic dysfunction was observed in 67% of EHPVO, 25% of NCPF, and 80% of cirrhotic subjects but none of the healthy controls (p < 0.05). Four of five time-domain heart rate variability indices showed significant abnormalities in patients with EHPVO (p < 0.05) and cirrhosis (p < 0.05), when compared with patients with NCPF and healthy controls. Autonomic dysfunction is frequently encountered in patients with EHPVO and cirrhosis, and the presence of autonomic dysfunction in patients with noncirrhotic portal hypertension suggests a primary role of portal hypertension per se in the dysfunction.

Prevalence of autonomic dysfunction in cirrhotic and noncirrhotic portal hypertension

OBJECTIVE: Autonomic dysfunction is common in patients with cirrhosis of the liver, but more so in patients with decompensated state, and is associated with increased mortality. We evaluated the presence and extent of autonomic dysfunction in patients with extrahepatic portal venous obstruction (EHPVO) and noncirrhotic portal fibrosis (NCPF), diseases with relatively preserved liver functions. METHODS: Heart rate variability in response to standing, deep breathing, and Valsalva maneuver and blood pressure response to sustained handgrip and standing were studied in 18 patients with EHPVO (13 mol/L, 5 F, mean age 15.2 ± 6 yr), 12 patients with NCPF (5 mol/L, 7 F, mean age 26.4 ± 8 yr), 15 patients with cirrhosis (7 mol/L, 8 F, mean age 12.6 ± 6 yr), and 17 healthy controls (11 mol/L, 6 F, mean age 18.6 ± 3 yr). Time-domain parameters of heart rate variability on 24-h ambulatory monitoring were assessed in all the patients. RESULTS: Autonomic dysfunction was observed in 67% of EHPVO, 25% of NCPF, and 80% of cirrhotic subjects but none of the healthy controls ( p < 0.05). Four of five time-domain heart rate variability indices showed significant abnormalities in patients with EHPVO ( p < 0.05) and cirrhosis ( p < 0.05), when compared with patients with NCPF and healthy controls. CONCLUSIONS: Autonomic dysfunction is frequently encountered in patients with EHPVO and cirrhosis, and the presence of autonomic dysfunction in patients with noncirrhotic portal hypertension suggests a primary role of portal hypertension per se in the dysfunction.

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

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. 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. 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. 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.

Idiopathic portal hypertension and its pathology.

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.

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

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. 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. 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. 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.

Non-cirrhotic portal hypertension in pregnancy

Objective: To study the outcome of pregnancy in women with non-cirrhotic portal hypertension (NCPH). Method: A retrospective analysis of 50 pregnancies in 27 women with NCPH was carried out. Pregnancy outcome was compared in extra hepatic portal vein obstruction (EHPVO) and non-cirrhotic portal fibrosis (NCPF). Results: The mean maternal age was 24.60±2.857 years, and the disease was diagnosed during pregnancy in 15 (55.6%) patients. Variceal bleeding occurred in 17/50 (34%) pregnancies and the majority (88.2%) of them responded to endoscopic sclerotherapy. Incidence of variceal bleeding during pregnancy was lower in pregnancies where the disease was diagnosed prior to pregnancy (8.6%), and it was 43.5% in EHPVO and 25.9% in NCPF. The mean birth weight of the neonates was 2668.4±427.42 g, and the incidence of abortion, prematurity, small for gestational age babies and perinatal death was 20, 17.5, 12.5 and 20%, respectively. Variceal bleeding during pregnancy was associated with a higher incidence of abortion (29.4%) and perinatal death (33.3%). Conclusion: Variceal bleeding is the most common complication in pregnancies with NCPH. Pregnancies can be allowed and managed successfully in patients with NCPH.

The natural history of portal hypertensive gastropathy: influence of variceal eradication.

The natural history and likelihood of bleeding from portal hypertensive gastropathy (PHG) present in patients with portal hypertension before endoscopic variceal obliteration may differ from that in patients who develop PHG during or after variceal eradication. A total of 967 variceal bleeders who had achieved variceal eradication by endoscopic sclerotherapy in the recent past were prospectively studied. In all, 88 (9.1%) patients (cirrhosis in 54, noncirrhotic portal fibrosis in 18, and extrahepatic portal vein obstruction in 16) had distinct mucosal lesions. PHG alone was present in 78, PHG with gastric antral vascular ectasia (GAVE) in eight, and GAVE alone in two patients. PHG was graded as mild or severe and according to whether present before (group A) or after endoscopic intervention (group B). Patients underwent regular endoscopy at follow-up to see if the PHG was transitory (disappearing within 3 months), persistent (no change), or progressive. Bleeding from PHG lesions was defined as acute or chronic. Twenty-two (26%) patients had PHG before (group A) and 64 (74%) developed PHG after variceal eradication (group B). During a mean follow-up of 25.1 +/- 14.2 months, PHG lesions disappeared in group A in only two patients (9%), but in group B in 28 (44%) patients (p < 0.05). PHG lesions more often progressed in the former as compared to the latter (18% vs 9.4%, p = NS). The incidence of bleeding was higher in group A than group B (32% vs 4.7%, p < 0.02). Bleeding from PHG occurred in 10 patients (11.6%); seven of them were from group A, and all had either progressive (n = 3) or persistent (n = 4) lesions. PHG developing after variceal eradication is often transitory and less severe. If PHG is pre-existing, endoscopic therapy for varices could worsen the PHG, with a likelihood of bleeding. Such patients may be benefited by concomitant beta-blocker therapy.

The natural history of portal hypertensive gastropathy: influence of variceal eradication

OBJECTIVE: The natural history and likelihood of bleeding from portal hypertensive gastropathy (PHG) present in patients with portal hypertension before endoscopic variceal obliteration may differ from that in patients who develop PHG during or after variceal eradication. METHODS: A total of 967 variceal bleeders who had achieved variceal eradication by endoscopic sclerotherapy in the recent past were prospectively studied. In all, 88 (9.1%) patients (cirrhosis in 54, noncirrhotic portal fibrosis in 18, and extrahepatic portal vein obstruction in 16) had distinct mucosal lesions. PHG alone was present in 78, PHG with gastric antral vascular ectasia (GAVE) in eight, and GAVE alone in two patients. PHG was graded as mild or severe and according to whether present before (group A) or after endoscopic intervention (group B). Patients underwent regular endoscopy at follow-up to see if the PHG was transitory (disappearing within 3 months), persistent (no change), or progressive. Bleeding from PHG lesions was defined as acute or chronic. RESULTS: Twenty-two (26%) patients had PHG before (group A) and 64 (74%) developed PHG after variceal eradication (group B). During a mean follow-up of 25.1 ± 14.2 months, PHG lesions disappeared in group A in only two patients (9%), but in group B in 28 (44%) patients ( p < 0.05). PHG lesions more often progressed in the former as compared to the latter (18% vs 9.4%), p = NS). The incidence of bleeding was higher in group A than group B (32% vs 4.7%, p < 0.02). Bleeding from PHG occurred in 10 patients (11.6%); seven of them were from group A, and all had either progressive (n = 3) or persistent (n = 4) lesions. CONCLUSIONS: PHG developing after variceal eradication is often transitory and less severe. If PHG is pre-existing, endoscopic therapy for varices could worsen the PHG, with a likelihood of bleeding. Such patients may be benefited by concomitant beta-blocker therapy.

Transabdominal extensive esophagogastric devascularization with gastroesophageal stapling for management of noncirrhotic portal hypertension: long-term results.

Outside Japan portosystemic shunts have been favored as the surgical procedure of choice for the management of portal hypertension of noncirrhotic etiology. Devascularization procedures have resulted in high rebleed rates probably owing to a limited extent of devascularization. We performed this study to assess the efficacy of our modification of Sugiura's procedure for long-term control of variceal bleeding in patients with noncirrhotic portal hypertension. Forty-six patients with extrahepatic portal venous obstruction (EHPVO) and 22 with noncirrhotic portal fibrosis (NCPF) were subjected to transabdominal extensive esophagogastric devascularization with esophageal or gastric stapled transection (modified Sugiura's procedure), 38 in an emergency situation and 30 electively. Follow-up endoscopies were performed every 6 months. Operative mortality, morbidity, variceal status, and causes of recurrent bleeding were evaluated. The postoperative mortality was 4%. Early procedure-related complications were seen in 6%, and esophageal strictures formed in 7 of 45 survivors undergoing esophageal transection (15%). Over a mean +/- SD follow-up of 53 +/- 34 months, 95% of patients were free of varices. Seven survivors (11%) had a rebleed, but only 5% were due to varices (two esophageal, one gastric). Six (9%) patients developed gastropathy. The 5-year survival was 88%. The modified Sugiura's procedure is safe and effective for long-term control of variceal bleeding especially in the emergency setting and in patients with anatomy unsuitable for shunt surgery or if surgical expertise for a shunt operation is not available.

Incidence and fate of antral varices.

To study the incidence of antral varices (AV) and their fate in patients with portal hypertension so as to formulate a management policy. Prospective cohort study. Single surgical unit specializing in portal hypertension management in a tertiary level centre. 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. Protocol-based endoscopic sclerotherapy and management of bleeding for oesophageal varices. Development or disappearance of AV, bleeding from AV. 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). 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.

Comparison of endoscopic variceal sclerotherapy with sequential endoscopic band ligation plus low-dose sclerotherapy for secondary prophylaxis of variceal hemorrhage: a prospective randomized study

Background: Endoscopic variceal sclerotherapy and band ligation both have certain limitations such as, respectively, esophageal complications and early recurrence of varices. Methods: From February 1994 to March 1996, all consecutive patients with portal hypertension due to either cirrhosis or noncirrhotic portal fibrosis and a history of variceal bleeding were included in a prospective study and randomly assigned to receive either endoscopic variceal sclerotherapy alone or endoscopic variceal band ligation plus low-dose endoscopic variceal sclerotherapy. Results: Of 69 patients, 34 were randomly assigned to receive endoscopic variceal sclerotherapy alone; 35 received endoscopic variceal band ligation plus endoscopic variceal sclerotherapy. Complete variceal eradication rates (85% vs. 80%) and the number of endoscopic sessions required for eradication (6.61 ± 2.94 vs. 7.85 ± 3.31) were similar in the endoscopic variceal sclerotherapy and endoscopic variceal band ligation plus endoscopic variceal sclerotherapy groups, respectively. The mean volume of sclerosant required in the combined group (54.94 ± 33.74 mL) was significantly less than that in the endoscopic variceal sclerotherapy group (81.91 ± 34.80 mL). The complication and recurrent bleeding rates were significantly higher in the endoscopic variceal sclerotherapy group than those in the combined group (20% and 16% vs. 3% and 3%, respectively). Conclusions: Both endoscopic variceal sclerotherapy and endoscopic variceal band ligation plus endoscopic variceal sclerotherapy were comparable in eradicating varices but the combined technique was associated with significantly lower complication and recurrent bleeding rates. (Gastrointest Endosc 1999;50:369-73.)