Rare Cause Of Portal Hypertension Research Articles

Transhepatic ultrasound guided embolization as a successful novel technique in treatment of pediatric complex intrahepatic arterioportal fistula: a case report and review of the literature

IntroductionIntrahepatic vascular shunts “IHVS” are abnormal communications between intra-hepatic vasculature involving the arterial, portal, or hepatic venous system. Arterio-portal fistula “APF” is an intrahepatic communication between the hepatic arterial system and the portal venous system without any communication with the systemic venous circulation. APF is considered a rare cause of portal hypertension and gastrointestinal bleeding in infancy.Case presentationA 3-month-old Mediterranean female with known cardiac congenital anomalies presented to us with abdominal distension and diarrhea. Ultrasonography revealed massive ascites and computerized tomography (CT) abdomen with intravenous (IV) contrast revealed a left hepatic lesion. On further evaluation, an intrahepatic arterio-portal vascular malformation was detected. Attempted trans arterial embolization failed and radiology team successfully carried out direct trans hepatic ultrasound guided coiling of the aneurysmal venous sac followed by successful resection of segment 4 of the liver with the vascular malformation avoiding life threatening intra operative bleeding.ConclusionAny child with recurrent gastrointestinal bleeding, failure to thrive, vomiting, diarrhea, steatorrhea, splenomegaly, or ascites should be investigated for intrahepatic arterio-portal fistula “IAPF”. Our novel technique of direct trans hepatic ultrasound guided coiling is an alternative method if trans arterial embolization “TAE” failed.

Budd-Chiari Syndrome-A Single Center Experience From the United Kingdom.

Pediatric Budd-Chiari syndrome (BCS) is a rare cause of portal hypertension and liver disease in Europe and North America. In order to understand the long-term effect of radiological intervention on BCS we performed a single center retrospective review. Fourteen cases were identified; 6 of 14 (43%) had a congenital thrombophilia with many having multiple prothrombotic mutations. Two were managed with medical anticoagulation alone and two required super-urgent transplant for acute liver failure. The remaining 10 of 14 (71%) underwent radiological intervention: 1 of 14 thrombolysis, 5 of 14 angioplasty, and 4 of 14 transjugular intrahepatic portosystemic shunt (TIPS). Six of 14 (43%) patients required repeat radiological intervention (1 angioplasty, 5 TIPS) but none required surgical shunts or liver transplantation for chronic liver disease. The time between diagnosis and treatment did not predict the need for repeat radiological intervention. These data show that radiological intervention can be highly effective, and reduces the need for surgery, though it requires specialist multidisciplinary teams for monitoring.

Liver Stiffness by Transient Elastography to Detect Porto‐Sinusoidal Vascular Liver Disease With Portal Hypertension

Porto-sinusoidal vascular liver disease (PSVD) is a rare cause of portal hypertension. PSVD is still often misdiagnosed as cirrhosis, emphasizing the need to improve PSVD diagnosis strategies. Data on liver stiffness measurement using transient elastography (TE-LSM) in PSVD are limited. The aim of this study was to evaluate the accuracy of TE-LSM to discriminate PSVD from cirrhosis in patients with signs of portal hypertension. Retrospective multicenter study comparing TE-LSM in patients with PSVD, according to Vascular Liver Disease Interest Group criteria, with patients with compensated biopsy-proven cirrhosis associated with alcohol (n=117), HCV infection (n=110), or NAFLD (n=46). All patients had at least one sign of portal hypertension among gastroesophageal varices, splenomegaly, portosystemic collaterals, history of ascites, or platelet count < 150 × 109 /L. The 77 patients with PSVD included in the test cohort had lower median TE-LSM (7.9 kPa) than the patients with alcohol-associated, HCV-related, and NAFLD-related cirrhosis (33.8, 18.2, and 33.6kPa, respectively; P<0.001). When compared with cirrhosis, a cutoff value of 10kPa had a specificity of 97% for the diagnosis of PSVD with a 85% positive predictive value. A cutoff value of 20kPa had a sensitivity of 94% for ruling out PSVD with a 97% negative predictive value. Of the patients, 94% were well-classified. Even better results were obtained in a validation cohort including 78 patients with PSVD. This study including a total of 155 patients with PSVD and 273 patients with cirrhosis demonstrates that TE-LSM<10kPa strongly suggests PSVD in patients with signs of portal hypertension. Conversely, when TE-LSM is >20kPa, PSVD is highly unlikely.

Successful surgical treatment for intrahepatic arterioportal fistula with severe portal hypertension: a case report

BackgroundIntrahepatic arterioportal fistula (IAPF) is a rare cause of portal hypertension. Interventional radiology (IVR) is generally selected as the first-line therapeutic option. Surgical treatment for IAPF is required in refractory cases of IVR. As the treatment success rate with IVR is high, cases requiring surgical treatment are extremely rare.Case presentationA 54-year-old man was admitted to another hospital complaining of hematemesis due to rupture of the esophageal varices. A computed tomography revealed ascites and arterioportal fistula in the left lobe of the liver. Transcatheter arterial embolization (TAE) was performed to occlude the fistula; however, it could not reach complete occlusion. Thereafter, there were a total of four hematemeses, and six endoscopic variceal ligations were required. The second TAE also failed to reach complete occlusion. He was transferred to our hospital for further treatment. Because liver function was low due to frequent hematemeses and there was also uncontrollable ascites, it was confirmed that hepatectomy could not be performed safely at this time. Therefore, we ligated the left portal branch and ligated and dissected the left gastric vein to decrease portal vein pressure. However, on the 5th day after surgery, the esophageal varices reruptured. As the disappearance of ascites was observed in the postoperative course and the general condition also improved, left hepatectomy was performed to remove IAPF. There was no recurrence of portal hypertension for 1 year and 3 months since hepatectomy.ConclusionsThis case was difficult to treat with IVR and required surgical treatment. Our experience in the present case suggests that hepatectomy to remove arterioportal fistula was considered effective for improving portal hypertension due to IAPF. However, careful treatment selection according to the patient’s overall condition and clinical course is necessary for IAPF presenting with severe portal hypertension.

Its Congenital Hepatic Fibrosis; Not Cirrhosis At All

Congenital hepatic fibrosis is a rare condition characterized by extensive fibrosis of liver but with preserved normal lobular architecture inherited as autosomal recessive trait. We report a 19 year-old-female admitted to Bangabandhu Sheikh Mujib Medical University with the complaints of lump in upper abdomen since last 13 years and episodes of fever and abdominal pain for same duration. She was diagnosed with hepatic TB on hepatic histology. Congenital hepatic fibrosis is a rare cause of portal hypertension that presents during childhood. Prognosis of congenital hepatic fibrosis is good. Life threatening events in these patients are related with variceal bleeding and episodes of cholangitis. Owing to relatively good liver function these patients tolerate portosystemic shunt surgeries quite well.Though rare, congenital hepatic fibrosis should be included in the differential diagnosis of portal hypertension in early life.

Portal Hypertension and Unexplained Diarrhea in an 80-Year-Old Man

Question: An 80-year-old man was referred for evaluation of cirrhosis and chronic diarrhea of unknown etiologies. In the past year he had been diagnosed with ascites, esophageal varices, and splenomegaly. His history was notable for four loose, non-bloody stools per day, intermittent abdominal cramping, and thirty pound weight loss. He denied fever, night sweats, rash, itching, or flushing. Physical examination revealed cachexia, no jugular venous distension, and a distended abdomen with shifting dullness. There were no skin lesions. His only medications were loperamide and propranolol. Laboratory studies showed a white blood cell count 5.5 x 109/L [3.5-10.5 x 109/L], hemoglobin 11 g/dL [13.5-17.5 g/dL], platelets 93 x 109/L [150-450 x 109/L], ALT 111 U/L [7-55 U/L], AST 67 U/L [8-48 U/L], alkaline phosphatase 601 U/L [46-118 U/L], total bilirubin 0.9 mg/dL [0.1-1.0mg/dL], and INR of 3.3. An abdomen/pelvis CT demonstrated hepatosplenomegaly, ascites (Figure A), and punctate sclerotic bone lesions. Diagnostic paracentesis revealed a SAAG of 2.6 and total protein of 1.6 g/dL. Gram stain, bacterial culture, and cytology of the ascitic fluid were negative. Liver biopsy with hematoxylin and eosin staining showed nodules without surrounding fibrosis (Figure B). Serum tryptase was elevated at 243 ng/dL [<11.5 ng/dL]. What is the most likely cause of his diarrhea and portal hypertension?A.Congestive heart failureB.Felty’s syndromeC.Systemic mastocytosisD.Cirrhosis Look on page 819 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image(s) to Practical Teaching Cases. The correct answer is C. The liver biopsy (Figure B) shows hepatocellular nodules less than 3 mm in diameter with hypertrophic hepatocytes (arrows). These nodules are not surrounded by fibrosis but rather compressed hepatocytes (arrowheads). A reticulin stain (Figure C) highlights the changes of nodular hyperplasia (arrows) and atrophic hepatocytes (arrowheads) which are diagnostic of nodular regenerative hyperplasia (NRH). Given the history of chronic diarrhea, sclerotic bone lesions, and elevated serum tryptase, a bone marrow biopsy was performed. This revealed a hypercellular marrow with 20% involvement by atypical mast cells. Together these findings support a diagnosis of SM and secondary NRH. Congestive heart failure (Answer A) has been associated with NRH but the lack of jugular venous distension on physical exam makes this unlikely. NRH was first described in a patient with rheumatoid arthritis, neutropenia, and splenomegaly (Felty’s syndrome); however, in this case, there is no personal history of rheumatoid arthritis and neutropenia which excludes Felty’s syndrome (Answer B). While cirrhosis (Answer D) may present with esophageal varices, portal hypertension and hypersplenism; liver biopsy would demonstrate fibrosis rather than nodular regenerative hyperplasia. Occasionally, NRH and cirrhosis may be difficult to distinguish, particularly on clinical grounds and radiographic imaging. The diagnosis of NRH is made by liver biopsy. SM is characterized by excessive mast cell accumulation in multiple tissues, most commonly the skin, central nervous system, musculoskeletal system and gastrointestinal tract. Gastrointestinal manifestations such as diarrhea and malabsorption are likely a result of GI mucosal infiltration by mast cells with concomitant release of prostaglandin mediators.1Capron J.-P. Lebrec D. Degott C. et al.Portal hypertension in systemic mastocytosis.Gastroenterology. 1978; 74: 595-597Abstract Full Text PDF PubMed Scopus (28) Google Scholar SM is a rare cause of portal hypertension which may develop from arteriovenous shunting secondary to histamine release, increased intrahepatic resistance due to mast cell infiltration of sinusoids, and the development of NRH.2Grundfest S. Cooperman A.M. Ferguson R. Benjamin S. Portal hypertension associated with systemic mastocytosis and splenomegaly.Gastroenterology. 1980; 78: 370-373Abstract Full Text PDF PubMed Scopus (35) Google Scholar In this case, portal hypertension was caused by the development of NRH. NRH of the liver is an uncommon condition characterized by the diffuse transformation of normal hepatic parenchyma into small, regenerative nodules with no fibrosis. It is associated with systemic diseases including rheumatologic disorders, vascular disorders, myeloproliferative disorders, and certain medications.3Reshamwala P.A. Kleiner D.E. Heller T. Nodular Regenerative Hyperplasia: Not All Nodules Are Created Equal.Hepatology. 2006; 44: 7-14Crossref PubMed Scopus (189) Google Scholar NRH develops as a consequence of portal venopathy and altered hepatic circulation. The nodular areas are believed to develop as hyperperfused hepatocytes become hypertrophic while hypoperfused hepatocytes become atrophic.3Reshamwala P.A. Kleiner D.E. Heller T. Nodular Regenerative Hyperplasia: Not All Nodules Are Created Equal.Hepatology. 2006; 44: 7-14Crossref PubMed Scopus (189) Google Scholar Portal hypertension and its complications are the predominant clinical manifestations of NRH. Occasionally, as in this case, NRH and cirrhosis may be difficult to distinguish on clinical grounds and radiographic imaging. This case illustrates the importance of the gastroenterologist having knowledge of systemic diseases known to be associated with NRH and how liver biopsy is essential for diagnosis. Exam 2: Portal Hypertension and Unexplained Diarrhea in an 80-Year-Old ManGastroenterologyVol. 154Issue 4Preview Full-Text PDF

Peliosis Hepatis Complicating Pregnancy: A Rare Entity.

Peliosis hepatis (PH) is a rare, benign condition of the liver characterized by the presence of blood-filled lacunar spaces in the parenchyma. It usually has a chronic presentation and is a rare cause of portal hypertension reported in adult patients. Its etiology is diverse and ranges from infectious agents to tumors to toxic substances and anabolic steroids; however, the cause remains unclear in 25-50% of patients. Similarly, the symptomatology and imaging findings are diverse. Biopsy is the definitive test to diagnose the condition. Herein, we present a case of a young female presenting in her seventh month of gestational amenorrhea with signs of portal hypertension and subsequently diagnosed to have PH. She was managed conservatively and delivered her baby normally. Later, she presented with spontaneous bacterial peritonitis and hepatic encephalopathy and developed hepatorenal syndrome. She later succumbed to her illness. The condition should be kept in the differential diagnosis of the atypical liver masses and liver diseases causing portal hypertension.

Budd-Chiari syndrome: an update on imaging features

Budd-Chiari syndrome (BCS) is a rare cause of portal hypertension and liver failure. This condition is characterized by an impaired hepatic venous drainage. The diagnosis of BCS is based on imaging, which helps initiate treatment. Imaging findings can be categorized into direct and indirect signs. Direct signs are the hallmarks of BCS and consist of visualization of obstructive lesions of the hepatic veins or the upper portion of the inferior vena cava. Indirect signs, which are secondary to venous obstruction, correspond to intra- and extrahepatic collateral circulation, perfusion abnormalities, dysmorphy and signs of portal hypertension.

Hepatoportal Fistula in an 83-Year-Old Male, Presenting With Hematemesis 52 Years After Blunt Trauma

Hepatoportal arteriovenous fistulae are a rare cause of portal hypertension, which can have significant clinical manifestations. They have multiple etiologies, one of which includes hepatic trauma. We present a case of hepatoportal fistula presenting with bleeding esophageal varices in an 83-year-old man. The exact cause of fistula in this case is not entirely clear; however, hepatic trauma was noted in the patients' history, some 52 years before presentation. We also present a literature review on this rare and interesting phenomenon.

Metabolomics Discloses Potential Biomarkers for the Noninvasive Diagnosis of Idiopathic Portal Hypertension

Idiopathic portal hypertension (IPH) is a rare cause of portal hypertension that lacks a specific diagnostic test. Requiring ruling-out other causes of portal hypertension it is frequently misdiagnosed. This study evaluates whether using high-throughput techniques there is a metabolomic profile allowing a noninvasive diagnosis of IPH. Thirty-three IPH patients were included. Matched patients with cirrhosis (CH) and healthy volunteers (HV) were included as controls. Metabolomic analysis of plasma samples was performed using UPLC-time-of-flight-mass spectrometry. We computed Student's P-values, corrected by multiple comparison and VIP score (Variable Importance in the Projection). The metabolites were selected with an adjusted Benjamini Hochberg P value <0.05. We use markers with a greater VIP score, to build partial least squares projection to latent structures regression with discriminant analysis (PLS-DA) representative models to discriminate IPH from CH and from HV. The performance of the PLS-DA model was evaluated using R(2) and Q(2) parameter. An additional internal cross-validation was done. PLS-DA analysis showed a clear separation of IPH from CH with a model involving 28 metabolites (Q(2)=0.67, area under the curve (AUC)=0.99) and a clear separation of IPH from healthy subjects with a model including 31 metabolites (Q(2)=0.75, AUC=0.98). After cross-validation, both models showed high rates of sensitivity (94.8 and 97.5), specificity (89.1 and 89.7), and AUC (0.98 and 0.98), reinforcing the strength of our findings. A metabolomic profile clearly differentiating patients with IPH from CH and healthy subjects has been identified using subsets of 28 and 31 metabolites, respectively. Therefore, metabolomic analysis appears to be a valuable tool for the noninvasive diagnosis of IPH.

Rectal arterio-portal fistula: An unusual cause of persistent bleeding per rectum following a proximal spleno-renal shunt

Gastrointestinal arterio-venous malformations are a known cause of gastrointestinal bleeding. We present a rare case of persistent rectal bleeding due to a rectal arterio-portal venous fistula in the setting of portal hypertension secondary to portal vein thrombosis. The portal hypertension was initially surgically treated with splenectomy and a proximal splenorenal shunt. However, rectal bleeding persisted even after surgery, presenting us with a diagnostic dilemma. The patient was re-evaluated with a computed tomography mesenteric angiogram which revealed a rectal arterio-portal fistula. Arterio-portal fistulas are a known but rare cause of portal hypertension, and possibly the underlying cause of continued rectal bleeding in this case. This was successfully treated using angiographic localization and super-selective embolization of the rectal arterio-portal venous fistula via the right internal iliac artery.The patient subsequently went on to have a full term pregnancy. Through this case report, we hope to highlight awareness of this unusual condition, discuss the diagnostic workup and our management approach.

Splenic Arteriovenous Fistula: A Rare Cause of Portal Hypertension

Purpose: Portal hypertension is a common condition encountered by gastroenterologists. While this is most commonly encountered in the setting of cirrhosis, other conditions affecting portal venous blood flow dynamics can present with findings consistent with portal hypertension. We present a rare case of portal hypertension caused by a splenic arteriovenous (SAV) fistula. A 69-year-old female presented with hematemesis after a prolonged bout of nausea and vomiting. Endoscopic evaluation showed large esophagogastric varices which were amenable to esophageal banding. There was no evidence for alcohol abuse, hepatotoxin exposure, or risk factors for viral hepatitis. Serologic studies for viral hepatitis, antinuclear antibody, antismooth muscle antibody, antimitochondrial antibodies, and iron studies were normal. Initial radiographic imaging did not suggest cirrhosis and a percutaneous liver biopsy was normal. She was referred to our center for further management. Following initial esophageal banding, she did not rebleed. Our review of the previous CT images suggested a possible splenic arteriovenous (SAV) fistula. CT angiography showed contrast in the splenic and portal veins during the arterial phase and aneurysmal splenic vein enlargement confirming the diagnosis of a SAV fistula. Transcatheter arterial embolization was done with only a small branch of the splenic artery still feeding the dilated splenic vein postembolization. Upon further questioning, she denied any major abdominal trauma and had two normal pregnancies. She did not experience any further bleeding and was discharged. SAV fistula is a rare cause of portal hypertension and should be suspected in female patients who present with variceal bleeding, but have no signs of chronic liver disease. In a SAV fistula, portal hypertension is due to enhanced arteriovenous flow as blood bypasses the capillary bed of the spleen. The hemodynamic changes that ensue lead to dramatic and rapid increases in portal venous pressures and subsequent clinical sequellae. The etiology of an acquired SAV fistula may be spontaneous, iatrogenic, or traumatic. A significant female predominance of spontaneous cases and relatively young presentations that are temporally associated with pregnancy have suggested that hormonal factors (perhaps associated with pregnancy) lead to arterial wall elastin weakening. Gastrointestinal hemorrhage has been the most common presentation in the literature. Our patient demonstrates that an SAV fistula should be considered as a cause of noncirrhotic portal hypertension in females even well beyond the child-bearing years.

Role of hepatic vein catheterisation and transient elastography in the diagnosis of idiopathic portal hypertension

BackgroundIdiopathic portal hypertension is a rare cause of portal hypertension, frequently misdiagnosed as cryptogenic cirrhosis. This study evaluates specific findings at hepatic vein catheterisation or liver stiffness in idiopathic portal hypertension. Methods39 cases of idiopathic portal hypertension patients were retrospectively reviewed. Hepatic vein catheterisation and liver stiffness measurements were compared to matched patients with cirrhosis and portal hypertension, and non-cirrhotic portal vein thrombosis, included as controls. ResultsHepatic vein-to-vein communications were found in 49% idiopathic portal hypertension patients precluding adequate hepatic venous pressure gradient measurements in 12. In the remaining 27 patients, mean hepatic venous pressure gradient (HVPG) was 7.1±3.1mmHg. Only 5 patients had HVPG≥10mmHg. HVPG was markedly lower than in cirrhosis (17±3mmHg, p<0.001). Mean liver stiffness in idiopathic portal hypertension was 8.4±3.3kPa; significantly higher than in non-cirrhotic portal vein thrombosis (6.4±2.2kPa, p=0.009), but lower than in cirrhosis (40.9±20.5kPa, p=0.005). Only 2 idiopathic portal hypertension patients had liver stiffness >13.6kPa. ConclusionsPatients with idiopathic portal hypertension frequently have hepatic vein-to-vein communications and, despite unequivocal signs of portal hypertension, HVPG and liver stiffness values much lower than the cut-off for clinical significant portal hypertension in cirrhosis. These findings oblige to formally rule-out idiopathic portal hypertension in the presence of signs of portal hypertension.

Congenital arterioportal fistulas: radiological treatment and color Doppler US follow-up

Congenital intrahepatic arterioportal fistulas (APFs) are a rare cause of portal hypertension in children. Doppler US is a useful diagnostic imaging modality. Transarterial embolization is a minimally invasive and effective therapy allowing occlusion of the fistula and restoration of liver hemodynamics. To describe the clinical and radiologic findings, percutaneous treatment and role of D-US in the postembolization follow-up of children with APF. Between 2002 and 2011, four children with APF were treated. Initial diagnosis and follow-up was performed with D-US and confirmed by arteriography, followed by endovascular embolization in all patients. D-US demonstrated abnormal arterioportal communications in all patients. Six endovascular procedures were performed in these four children. In two children, no residual fistula was seen on D-US after the first procedure and symptoms resolved. In the other two children, D-US demonstrated residual flow through the fistula, with resolution of pathological D-US findings and symptoms after the second endovascular procedure. All four children were successfully treated and asymptomatic at the end of follow-up. The mean follow-up was 24 months. Interventional radiology has a key role in the treatment of congenital APF. D-US is a noninvasive and effective tool for the diagnosis and follow-up of these patients.

Gastric variceal bleeding caused by an intrahepatic arterioportal fistula that formed after liver biopsy: a case report and review of the literature

An intrahepatic arterioportal fistula is a rare cause of portal hypertension and variceal bleeding. We report on a patient with an intrahepatic arterioportal fistula following liver biopsy who was successfully treated by hepatectomy after unsuccessful arterial embolization. We also review the literature on symptomatic intrahepatic arterioportal fistulas after liver biopsy. A 48-year-old male with bleeding gastric varices and hepatitis B virus-associated liver cirrhosis was transferred to our hospital; this patient previously underwent percutaneous liver biopsies 3 and 6 years ago. Abdominal examination revealed a bruit over the liver, tenderness in the right upper quadrant, and splenomegaly. Ultrasonographic examination, computed tomography, and angiography confirmed an arterioportal fistula between the right hepatic artery and the right portal vein with portal hypertension. After admission, the patient suffered a large hematemesis and developed shock. He was treated with emergency transarterial embolization using microcoils. Since some collateral vessels bypassed the obstructive coils and still fed the fistulous area, embolization was performed again. Despite the second embolization, the collateral vessels could not be completely controlled. Radical treatment involving resection of his right hepatic lobe was performed. For nearly 6 years postoperatively, this patient has had no further episodes of variceal bleeding.

A Case of Idiopathic Non-Cirrhotic Portal Hypertension

Idiopathic Non-Cirrhotic Portal Hypertension (INCPH) is a rare cause of portal hypertension. Its etiology is still unknown but its prognosis is excellent. Herein, we report a case of a 21 year old gentleman who was found to be thrombocytopenic on routine lab work before right anterior cruciate ligament repair. He had no other symptoms on initial presentation. He denied any history of GI bleeding, encephalopathy, ascites, jaundice, or abdominal distention. His past medical and family history were negative.On examination his blood pressure was 140/80, HR 52/min, and he was afebrile. He was non-icteric. His exam was benign except for soft a palpable spleen 2 cm below the costal margin. He was hemoccult negative. He had a creatinine of 1.12, Bilirubin of 1.4, AST of 26 and ALT of 56. His platelet count was 76,000, hematocrit 48.9, and hemoglobin 16.5. His INR was 1.1. Hepatitis B surface antibody was positive due to vaccination. HBS negative core antibody, Hepatitis A and C serologies were negative. His EBV serologies were negative. A bone marrow biopsy did not reveal any hematological cause of his thrombocytopenia. He also underwent a CT scan of the abdomen which showed splenomegaly, enlarged intra-abdominal varices, and Grade II esophageal varices which required banding. Liver ultrasound showed patent vasculature. He underwent a transjugular liver biopsy with hepatic vein and wedge pressure measurements as well as hepatic vein venograms. His venography showed a normal course, caliber and branching, his hepatic vein and wedge pressures were 12 and 21 mm Hg respectively with a corrected sinusoidal gradient of 8. His biopsy showed vascular abnormalities (megasinusoids) and irregularly distributed vessels without fibrosis associated with focal nodular regenerative change suggestive of INCPH (Figure 1). He underwent varicial banding. He is currently doing very well. Portal hypertension is a clinical syndrome defined by a portosystemic venous pressure gradient exceeding 5 mm Hg.[1] Liver cirrhosis is the most frequent cause of portal hypertension in the western hemisphere. However, in a variety of disorders, portal hypertension develops in the absence of cirrhosis. This condition is referred to as noncirrhotic portal hypertension. [1] The most common cause of noncirrhotic portal hypertension worldwide is schistosomiasis. [2] In the Western world, chronic liver diseases, such as nonalcoholic steatohepatitis, primary biliary cirrhosis, primary sclerosing cholangitis and congenital hepatic fibrosis together with extrahepatic portal vein thrombosis and Budd-Chiari syndrome are common causes of noncirrhotic portal hypertension. Chronic arsenic toxicity is also one of the causes of non cirrhotic portal fibrosis [3]. If all these conditions have been ruled out, the diagnosis of INCPH can be made.[4-6] In the Western world INCPH might be responsible for 3%-5% of cases of portal hypertension.

Liver metastases, a rare cause of portal hypertension and stoma bleeding. Brief review of literature

INTRODUCTIONPortal hypertension is an unusual complication of liver metastases, which is frequently occurring in malignant disease. Portal hypertension may cause oesophageal varices and also stoma varices (colostomy and ileostomy). Oesophageal varices and bleeding from these varices have been frequently reported in literature. Stomal varices have also been reported in literature mostly associated with liver cirrhosis. These stomal varices lead to the massive bleeding causing morbidity and mortality.Portal hypertension is a pathological increase in portal pressure gradient (the difference between pressure in the portal and inferior vena cava veins). It is either due to an increase in portal blood flow or an increase in vascular resistance or combination of both. In liver cirrhosis, the primary factor leading to portal hypertension is increase in portal blood flow resistance and later on development of increased portal blood flow. It has been postulated that in liver metastasis the increase in portal flow resistance occurs at any site within portal venous system as a consequence of mechanical architectural disturbance. PRESENTATION OF CASEWe report a case of a 64 year old gentleman who developed portal hypertension due to secondary metastases from colorectal cancer. He subsequently developed bleeding varices in his end colostomy. DISCUSSIONWe believe that the combination of extensive metastases and chemotherapy induced portal hypertension in our patient. CONCLUSIONOur case and other literature review highlight that the recurrent bleeding stoma associated with colorectal cancer should be investigated for portal hypertension.

Seltene Ursache einer Fundusvarizenblutung

Agnogenic myeloid metaplasia (AMM) is a clonal stem cell disease, which is characterized by myelofibrosis, osteosclerosis and pancytopenia. Affected patients frequently develop portal hypertension secondary to extramedullary hematopoiesis, which rarely becomes clinically relevant. We here report on a 63-year-old patient with a first presentation of variceal bleeding. The patient was diagnosed with portal hypertension and due to marked splenomegaly, liver cirrhosis was suspected. Subsequently, an extramedullary hematopoiesis and AMM could be diagnosed. The variceal bleeding was initially treated endoscopically. The patient has received cytoreductive therapy and a splenorenal shunt, which has led to remission and long-term survival. Agnogenic myeloid metaplasia is a rare cause of portal hypertension and may present with variceal bleeding. Clinicians should be aware of this rare cause of portal hypertension, as treatment of the underlying disease with cytoreductive therapy and appropriate management of portal hypertension may lead to long-term survival.

Congenital intrahepatic arterioportal fistula presenting as severe undernutrition and chronic watery diarrhea in a 2-year-old girl

Intrahepatic arterioportal fistula (IAPF) is a rare cause of portal hypertension in young children. We report the case of a 2-year-old girl with severe undernutrition, chronic watery diarrhea, and gastrointestinal bleeding because of a congenital intrahepatic arterioportal fistula. Radiographic embolization and surgical ligation of the left hepatic artery were attempted, with no resolution of the symptoms. So, a left lobectomy was performed, with excellent results and prompt disappearance of the diarrhea. Hepatectomy should be considered as a definitive and reliable therapy for congenital IAPF.

A large congenital and solitary intrahepatic arterioportal fistula in an old woman

Arterioportal fistula (APF) is a rare cause of portal hypertension and may lead to death. APF can be congenital, post-traumatic, iatrogenic (transhepatic intervention or biopsy) or related to ruptured hepatic artery aneurysms. Congenital APF is a rare condition even in children. In this case report, we describe a 73-year-old woman diagnosed as APF by ultrasonography, computed tomography, and hepatic artery selective arteriography. The fistula was embolized twice but failed, and she still suffered from alimentary tract hemorrhage. Then, selective arteriography of the hepatic artery was performed again and venae coronaria ventriculi and short gastric vein were embolized. During the 2-year follow-up, the patient remained asymptomatic. We therefore argue that embolization of venae coronaria ventriculi and short gastric vein may be an effective treatment modality for intrahepatic APF with severe upper gastrointestinal bleeding.