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Portal cavernoma

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https://www.readbyqxmd.com/read/27801752/survey-on-clinical-practice-of-primary-prophylaxis-in-portal-hypertension-in-children
#1
Odile Jeanniard-Malet, Mathieu Duché, Alexandre Fabre
Primary prophylaxis in portal hypertension in children is controversial, as there are few studies documenting its efficacy on the risk of bleeding.28 centres out of the 38 we contacted returned a completed questionnaire about their clinical practices. More than 75% of the centres use endoscopy to screen patients diagnosed with portal cavernoma, biliary atresia, cystic fibrosis and other fibrotic chronic liver diseases with suspected portal hypertension.In cases of grade 2 varices with red marks and grade 3 varices more than 90% of centres perform sclerotherapy or endoscopic variceal ligation (EVL)...
October 31, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27757214/positive-impact-of-eculizumab-therapy-on-surgery-for-budd-chiari-syndrome-in-a-patient-with-paroxysmal-nocturnal-hemoglobinuria-and-a-long-term-history-of-thrombosis
#2
Silvia De-la-Iglesia, Hugo Luzardo, Angelina Lemes, Melissa Torres, Maria Teresa Gómez-Casares, Naylen Cruz, Teresa Molero
Paroxysmal nocturnal hemoglobinuria (PNH) is associated with severe end-organ damage and a high risk of thrombosis. Budd-Chiari syndrome, which develops after thrombotic occlusion of major hepatic blood vessels, is relatively common in PNH and has been associated with increased mortality. We report the case of a 46-year-old male with PNH who presented with Budd-Chiari syndrome associated with portal cavernoma, portal hypertension and hypersplenism. In September 2010, the patient suffered gastrointestinal bleeding, hematuria, and elevated plasma lactate dehydrogenase; he started eculizumab therapy with a good response...
September 28, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27730821/spectrum-of-biliary-abnormalities-in-portal-cavernoma-cholangiopathy-pcc-secondary-to-idiopathic-extrahepatic-portal-vein-obstruction-ehpvo-a-prospective-magnetic-resonance-cholangiopancreaticography-mrcp-based-study
#3
Shumyla Jabeen, Irfan Robbani, Naseer A Choh, Obaid Ashraf, Feroze Shaheen, Tariq Gojwari, Sabeeha Gul
OBJECTIVE: To characterize biliary abnormalities seen in portal cavernoma cholangiopathy (PCC) on Magnetic Resonance Cholangiopancreaticography(MRCP) and elucidate certain salient features of the disease by collaborating our findings with those of previous studies. METHODS: We prospectively enrolled 52 patients with portal cavernoma secondary to idiopathic Extrahepatic portal vein obstruction(EHPVO) who underwent a standard MRCP protocol. Images were analysed for abnormalities involving the entire biliary tree...
October 12, 2016: British Journal of Radiology
https://www.readbyqxmd.com/read/27728350/is-it-apla-causing-portal-vein-thrombosis-with-portal-cavernoma
#4
Sivaji Patibandla, Jaya Singh K, Tumbanatham, Appikatla
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27728317/jack-2-negative-essential-thrombocytosis-with-portal-vein-thrombosis-and-portal-cavernoma-formation-a-case-report
#5
Ankit Sharma
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27728089/is-it-apla-causing-portal-vein-thrombosis-with-portal-cavernoma
#6
Sivaji Patibandla, Jaya Singh K, Tumbanatham Appikatla
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27689206/therapeutic-challenges-for-symptomatic-portal-cavernoma-cholangiopathy
#7
Adriana Cavași, Voicu Mercea, Ofelia Anton, Ion Cosmin Puia
Although transjugular intrahepatic portosystemic shunts are most frequently used for the management of portal hypertension, the surgical approach is preferred for symptomatic portal cavernoma cholangiopathy. We present the case of a 25-year old female patient with a portal cavernoma secondary to catheterization of the umbilical vein at birth. She had had two episodes of esophageal variceal bleeding, successfully treated by endoscopic banding. and an episode of acute cholangitis secondary to portal cavernoma cholangiopathy...
September 2016: Journal of Gastrointestinal and Liver Diseases: JGLD
https://www.readbyqxmd.com/read/27672292/portal-biliopathy
#8
REVIEW
Mohammad S Khuroo, Ajaz A Rather, Naira S Khuroo, Mehnaaz S Khuroo
Portal biliopathy refers to cholangiographic abnormalities which occur in patients with portal cavernoma. These changes occur as a result of pressure on bile ducts from bridging tortuous paracholedochal, epicholedochal and cholecystic veins. Bile duct ischemia may occur due prolonged venous pressure effect or result from insufficient blood supply. In addition, encasement of ducts may occur due fibrotic cavernoma. Majority of patients are asymptomatic. Portal biliopathy is a progressive disease and patients who have long standing disease and more severe bile duct abnormalities present with recurrent episodes of biliary pain, cholangitis and cholestasis...
September 21, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/27595034/use-of-left-gastric-vein-as-an-alternative-for-portal-flow-reconstruction-in-liver-transplantation
#9
Uirá Fernandes Teixeira, Mayara Christ Machry, Marcos Bertozzi Goldoni, Cristine Kruse, João Alfredo Diedrich, Pablo Duarte Rodrigues, Caroline Becker Giacomazzi, Estéfano Aurélio Negri, Matheus Koop, Carlos Gustavo Spode Gomes, José Artur Sampaio, Paulo Roberto Ott Fontes, Fábio Luiz Waechter
Portal vein thrombosis is observed in up to 10% of liver transplant candidates, hindering execution of the procedure. A dilated gastric vein is an alternative to portal vein reconstruction and decompression of splanchnic bed. We present two cases of patients with portal cavernoma and dilated left gastric vein draining splanchnic bed who underwent liver transplantation. The vein was dissected and sectioned near the cardia; the proximal segment was ligated with suture and the distal segment was anastomosed to the donor portal vein...
2016: Case Reports in Surgery
https://www.readbyqxmd.com/read/27358683/imaging-and-radiological-interventions-in-extra-hepatic-portal-vein-obstruction
#10
REVIEW
Sudheer S Pargewar, Saloni N Desai, S Rajesh, Vaibhav P Singh, Ankur Arora, Amar Mukund
Extrahepatic portal vein obstruction (EHPVO) is a primary vascular condition characterized by chronic long standing blockage and cavernous transformation of portal vein with or without additional involvement of intrahepatic branches, splenic or superior mesenteric vein. Patients generally present in childhood with multiple episodes of variceal bleed and EHPVO is the predominant cause of paediatric portal hypertension (PHT) in developing countries. It is a pre-hepatic type of PHT in which liver functions and morphology are preserved till late...
June 28, 2016: World Journal of Radiology
https://www.readbyqxmd.com/read/27346144/safety-of-supramesocolic-surgery-in-patients-with-portal-cavernoma-without-portal-vein-decompression-large-single-centre-experience
#11
Safi Dokmak, Béatrice Aussilhou, Alain Sauvanet, Philippe Lévy, Aurélie Plessier, Fadhel S Ftériche, Olivier Farges, Valérie Vilgrain, Dominique C Valla, Jacques Belghiti
BACKGROUND: Supra-mesocolic surgery (SMS) is complicated in patients with portal vein cavernoma (PC) and portal decompression is recommended. The aim of this study was to report a large single centre of SMS in patients with PC without portal decompression. METHODS: Between 2006 and 2013, all patients who met inclusion criteria were analyzed retrospectively. The primary endpoint was the feasibility rate, surgical and postoperative outcome. The secondary endpoints were the long-term outcome of patients who underwent biliary bypass for cholangitis...
July 2016: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
https://www.readbyqxmd.com/read/27118711/a-new-procedure-for-the-treatment-of-extrahepatic-portal-hypertension-in-children-portal-cavernoma-rex-shunt-with-interposition-of-grafted-portal-vessel
#12
Jin-Shan Zhang, Long Li, Wei Cheng
No abstract text is available yet for this article.
June 2016: Journal of the American College of Surgeons
https://www.readbyqxmd.com/read/26949386/outcome-of-the-pediatric-patients-with-portal-cavernoma-the-retrospective-study-for-10-years-focusing-on-recurrent-variceal-bleeding
#13
Hongjie Guo, Fabao Hao, Chunbao Guo, Yang Yu
Background. Portal cavernoma (PC) is the most critical condition with risk or variceal hemorrhage in pediatric patients. We retrospectively investigated the patients with PC focusing on the predictors for recurrent variceal bleeding. Methods. Between July 2003 and June 2013, we retrospectively enrolled all consecutive patients admitted to our department with a diagnosis of PC without abdominal malignancy or liver cirrhosis. The primary endpoint of this observational study was recurrent variceal bleeding. Independent predictors of recurrent variceal bleeding were identified using the logistic regression model...
2016: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/26903662/intraductal-sonography-in-patients-with-portal-cavernoma-cholangiopathy
#14
Vikram Bhatia, Saggere Muralikrishna Shasthry, Amar Mukund
Intraductal sonography generates high-resolution images of the entire length of the biliary tree and peribiliary tissues, including 3-dimensional dual- and muti-plane reconstructions to depict complex anatomy. Portal cavernoma cholangiopathy (previously called portal biliopathy) can have multiple etiologies of obstructive cholestasis in the same patient, which can be difficult to define even with advanced imaging techniques.We describe 2 difficult cases of portal cavernoma cholangiopathy in which intraductal sonography helped in clinical management decisions...
March 2016: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/26638882/genotype-phenotype-correlation-of-16p13-3-terminal-duplication-and-22q13-33-deletion-natural-history-of-a-patient-and-review-of-the-literature
#15
REVIEW
Marshall I B Fontes, Ana P Santos, Miriam C Molck, Milena Simioni, Diogo L L Nascimento, Ana K M Andrade, Carla Rosenberg, Ana C V Krepischi, Simone Appenzeller, Isabella L Monlleó, Vera Lúcia Gil-da-Silva-Lopes
This article reports a patient with a de novo ∼ 9.32 Mb duplication at 16p13.3 and a ∼ 71 Kb deletion at 22q13.33. The patient was followed from 1 month old to 3 years and 8 months of age and presented typical features of the 16p13.3 duplication syndrome. In addition, the patient presents a portal cavernoma, an alteration rarely reported in this condition. Renal agenesis was detected as additional developmental defect. After genomic array and FISH analysis, the karyotype was 46,XX,ins(22;16)(q13;p13.2p13...
March 2016: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/26387759/-natural-history-of-portal-cavernoma-without-liver-disease-a-single-centre-retrospective-study-of-32%C3%A2-cases
#16
A S Resseguier, M André, E A Orian Lazar, G Bommelaer, O Tournilhac, I Delèvaux, M Ruivard, M D'Incan, L Boyer, O Aumaître
PURPOSE: Portal cavernoma follows a chronic occlusion of the portal vein. The long-term consequences of portal cavernoma are not well known. The objective of this study was to report the aetiology of the portal cavernoma and its natural course after excluding liver diseases causes. METHODOLOGY: A single centre retrospective study based on the data collected from the radiology department of the Clermont-Ferrand hospital was conducted from 2000 to 2011. All the patients for whom an imagery found a portal cavernoma have been looked for excluding the patients having a liver disease whatever the aetiology and the Budd-Chiari syndrome...
June 2016: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/26378482/portal-cavernoma-in-the-era-of-mesoportal-shunt-rex-and-liver-transplant-in-children
#17
Gloria Chocarro, Paloma Triana Junco, Eva Dominguez, María Virginia Amesty, Vanesa Nuñez Cerezo, Francisco Hernandez, Javier Murcia, Juan Antonio Tovar, Manuel Lopez Santamaria
OBJECTIVE: The mesoportal shunt (MPS) and liver transplantation (LT) have changed the scenario of extrahepatic portal vein obstruction (EHPVO) since the MPS, the only "curative" technique, can now be offered in asymptomatic patients and also thrombotic complications of LT have increased the incidence of EHPVO. MATERIAL AND METHODS: A retrospective study of patients undergoing surgery for EHPVO was conducted between 1990 and 2015. An analysis was done for the shunt permeability and clinical evolution over time...
February 2016: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/26333179/well-tolerated-portal-hypertension-and-favorable-prognosis-in-adult-patients-with-extrahepatic-portal-vein-obstruction-in-japan
#18
Tadashi Sekimoto, Hitoshi Maruyama, Kazufumi Kobayashi, Soichiro Kiyono, Takayuki Kondo, Taro Shimada, Masanori Takahashi, Osamu Yokosuka
AIM: To evaluate the clinical features and prognoses in adult patients with extrahepatic portal vein obstruction (EHO) from the aspect of portal hypertension during the last 20 years in Japan. METHODS: There were 40 EHO patients (aged 21-77 years; mean ± standard deviation [SD], 54.6 ± 15.0). Clinical findings and prognoses were examined retrospectively during the median observation period of 71.6 months. RESULTS: Twenty-two patients (55%) showed positive signs of portal hypertension; 18 with esophageal varices (F0, one; F1, eight; F2, nine), two with gastric varices (F1, one; F2, one) and seven with mild ascites...
May 2016: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/26158259/modified-sugiura-operation-for-idiopathic-portal-hypertension-with-bleeding-oesophageal-varices-a-case-report
#19
A-V Schettini, R S Pinheiro, P Pescatore, J Lerut
A case of a 36 years old man presenting massive upper GI bleeding due to oesophageal varices developed in the context of an idiopathic portal cavernoma and extensive porto-splenic thrombosis is discussed. He underwent a successful modified Sugiura operation (oesophago-gastric devascularisation and splenectomy [OGDS]) completed with interventional endoscopic treatment of residual oesophageal varices. The benefit of the modified Sugiura procedure proposed for the treatment of upper GI variceal bleeding developed in the context of splanchnic venous thrombosis is discussed...
May 2015: Acta Chirurgica Belgica
https://www.readbyqxmd.com/read/26111208/multimodality-imaging-of-primary-extrahepatic-portal-vein-obstruction-ehpvo-what-every-radiologist-should-know
#20
A Arora, S K Sarin
Portal vein thrombosis (PVT) is a frequent complication of liver cirrhosis, but it can also occur as a primary vascular disorder amid absent liver disease. Extrahepatic portal vein obstruction (EHPVO) refers to the obstruction of the extrahepatic portal vein with or without involvement of the intrahepatic portal vein branches, splenic and/or superior mesenteric vein. It is a distinct disorder that excludes PVT occurring in concurrence with liver cirrhosis or hepatocellular carcinoma. The term "EHPVO" implies chronicity and is principally reserved for a long-standing condition characterized by cavernous transformation of the portal vein...
August 2015: British Journal of Radiology
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