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Portal cavernomatous transformation

Ruo-Yi Wang, Jun-Feng Wang, Qian Liu, Nan Ma, Wei-Xiu Chen, Jin-Liang Li
OBJECTIVE: To evaluate the effects of combined Rex-bypass shunt and pericardial devascularization on prehepatic portal hypertension secondary to cavernomatous transformation of portal vein (CTPV). METHODS: Forty-two patients aged from 3 years to 49 years (divided into 3 groups), 26 cases male and 16 female, with prehepatic vascular hepertention were treated with Rex-bypass shunt combined with pericardial devascularization. In each patient, preoperative assessment included ultrasound and computed tomographic angiography of the portal vein and blood analysis...
September 2017: Postgraduate Medicine
Zeeshan A Wani, Riyaz A Bhat, Ajeet S Bhadoria, Rakhi Maiwall
Extrahepatic portal vein obstruction is a vascular disorder of liver, which results in obstruction and cavernomatous transformation of portal vein with or without the involvement of intrahepatic portal vein, splenic vein, or superior mesenteric vein. Portal vein obstruction due to chronic liver disease, neoplasm, or postsurgery is a separate entity and is not the same as extrahepatic portal vein obstruction. Patients with extrahepatic portal vein obstruction are generally young and belong mostly to Asian countries...
May 2015: Saudi Journal of Gastroenterology: Official Journal of the Saudi Gastroenterology Association
Fabrizio di Francesco, Laura Del Prete, Chiara Grimaldi, Lidia Monti, Jean de Ville de Goyet
BACKGROUND: The association of pancreatitis and portal vein thrombosis (PVT) is extremely rare in children. We report on 3 cases which suggest that there may be a causal relation between the two. METHODS: Clinical characteristics and evolution of 3 children with this particular condition were analyzed retrospectively. In this group of patients, the strategy consisted in opting for early surgical decompression of the portal hypertension, which was followed by a favorable outcome, not only in terms of complications related to the portal hypertension but also of a contemporaneous spontaneous regression of the concurrent pancreatic disease, in absence of any other specific management of the latter problem...
April 2015: Journal of Pediatric Surgery
Fabrizio di Francesco, Lidia Monti, Chiara Grimaldi, Cristina Lo Zupone, Arianna Bertocchini, Jean de Ville de Goyet
A cavernomatous transformation of the extrahepatic portion of the portal vein is a common cause of chronic portal hypertension in children. A few attempts at radiological interventions have been reported, but have rarely been successful. In this report, a surgical Meso-Rex bypass was performed to treat complicated prehepatic portal hypertension, after the insertion of an intrahepatic stent for portosystemic shunting had failed. The review of this case nicely illustrates how differently effective are these two shunting procedures-in terms of restoring hepatopetal flow, managing portal hypertension, and establishing-or not-portosystemic connections...
January 2015: Pediatric Surgery International
Helena León-Brito, María Fraile-González, Irene Aresté-Anduaga, Juan Isidro Úriz-Otano, David Ruiz-Clavijo-García, José Carlos Súbtil-Íñigo, Eduardo Albéniz-Arbizu, Javier Eguaras-Ros, Carlos Jiménez-López
No abstract text is available yet for this article.
May 2014: Revista Española de Enfermedades Digestivas
Arianna Bertocchini, Pierluigi Falappa, Chiara Grimaldi, Giuseppe Bolla, Lidia Monti, Jean de Ville de Goyet
BACKGROUND: Children with extrahepatic portal hypertension typically present with cavernomatous transformation of the portal vein and a poorly defined intrahepatic portal vein system on conventional imaging. With the Meso-Rex Bypass becoming the gold-standard intervention for a cure, a precise assessment of the intrahepatic portal vein system provides helpful data for deciding whether a Meso-Rex Bypass is feasible or not. METHODS: All children with extrahepatic portal hypertension were prospectively assessed by wedged hepatic venous portography...
August 2014: Journal of Pediatric Surgery
Arianna Bertocchini, Giuseppe d'Ambrosio, Chiara Grimaldi, Laura del Prete, Fabrizio di Francesco, Piergiorgio Falappa, Lidia Monti, Jean de Ville de Goyet
INTRODUCTION: Portal vein aneurysms (PVAs) are usually located at the vein trunk or at its bifurcation, rarely intra-hepatic, or at the umbilical portion. Etiology remains unclear. METHODS: Three children with PVA were identified over a 5-year period. PVA anatomy was assessed by Doppler Ultrasound, Angio CT/MRI, and trans-jugular retrograde portography. RESULTS: Three children with intrahepatic PVA (including the umbilical portion) were identified during assessment for pre-hepatic portal hypertension: all had splenomegaly and hypersplenism...
March 2014: Journal of Pediatric Surgery
Fabiana Savoia, Cinzia Ferrara, Anna Sansone, Giuseppe Bifulco, Carmine Nappi, Costantino Di Carlo
Portal vein thrombosis (PVT) is characterized by the obstruction of the portal venous system. The venous obstruction can be partial or complete and it is caused by thrombogenic conditions (acquired or hereditary) or nonthrombotic factors. The acquired conditions include abdominal inflammation, infections, surgery, myeloproliferative disorders, obesity, oral contraceptive intake, pregnancy, and postpartum period. Occasionally, it is not possible to recognize any overt cause of PVT. During pregnancy there is an increased venous thromboembolism risk mainly in the systemic venous system and the PVT can occur, but there are no data about its exact prevalence, etiology, and outcome...
2013: Case Reports in Obstetrics and Gynecology
María Gabriela Delgado, Susana Seijo, Ismael Yepes, Linette Achécar, Maria Vega Catalina, Angeles García-Criado, Juan G Abraldes, Joaquín de la Peña, Rafael Bañares, Agustín Albillos, Jaume Bosch, Juan Carlos García-Pagán
BACKGROUND & AIMS: Portal vein thrombosis (PVT) is a frequent event in patients with cirrhosis; it can be treated with anticoagulants, but there are limited data regarding safety and efficacy of this approach. We evaluated this therapy in a large series of patients with cirrhosis and non-neoplastic PVT. METHODS: We analyzed data from 55 patients with cirrhosis and PVT, diagnosed from June 2003 to September 2010, who received anticoagulant therapy for acute or subacute thrombosis (n = 31) or progression of previously known PVT (n = 24)...
July 2012: Clinical Gastroenterology and Hepatology
Gabriela Picón-Coronel, Fredy Chablé-Montero, Arturo Angeles-Ángeles, Jorge Albores-Saavedra
Well established complications of essential thrombocythemia are multiple thrombohemorrhagic phenomena in various abdominal organs. We describe the case of a 22 year old man with essential thrombocythemia and thrombosis of the mesenteric and splenic veins as well as cavernomatous transformation of the portal vein. The patient also had a splenic infarction and a subphrenic hematoma. Additionally, he developed signs and symptoms of acute cholecystitis which in turn led to an open cholecystectomy. The gallbladder had a markedly thickened wall due to multiple recent and recanalized thrombi predominantly in subserosal veins...
July 2011: Annals of Hepatology
F Rotellar, J A Cienfuegos, A Bueno, P Martí, V Valentí, G Zozaya, F Pardo
Diffuse thrombosis of the entire portal system (PVT) and cavernomatous transformation of the portal vein (CTPV) represents a demanding challenge in liver transplantation. We present the case of a patient with nodular regenerative hyperplasia and recurrent episodes of type B hepatic encephalopathy concomitant with PVT as well as CTPV, successfully treated with orthotopic liver transplantation. The portal inflow to the graft was carried out through the confluence of 2 thin paracholedochal varicose veins, obtaining good early graft function and recovery of the encephalopatic episodes...
October 2010: Transplantation Proceedings
Guo-Hong Han, Xiang-Jie Meng, Zhan-Xin Yin, Jian-Hong Wang, Chuang-Ye He, Jie Liang, Xue-Gang Guo, Jie Liu, Lie Ding, Kai-Chun Wu, Dai-Ming Fan
OBJECTIVE: To evaluate retrospectively the feasibility, efficacy and safety of transjugular intrahepatic portosystemic shunt (TIPS) or percutaneous transhepatic or transsplenic approach to the portal vein with the combination of TIPS for the treatment of patients with portal vein thrombosis with or without cavernous transformation. METHODS: Sixty-five patients with portal vein thrombosis from July 2002 to August 2007 at our hospital were analyzed retrospectively...
June 9, 2009: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Sanjeev Kumar Jha, Ashish Kumar, Barjesh Chander Sharma, Shiv Kumar Sarin
BACKGROUND: Patients with cirrhosis and portal hypertension exhibit a hyperdynamic circulation manifesting as increased cardiac output, heart rate and plasma volume; and decreased arterial blood pressure, systemic vascular resistance, and pulmonary vascular resistance. It is believed that these changes are related to both hepatocellular dysfunction and portal hypertension. However, the role of portal hypertension per se in producing these changes in circulation has not been clear. Extrahepatic portal vein obstruction (EHPVO), a vascular disorder of the liver characterized by cavernomatous transformation of the main portal vein, is an excellent model to study the role of portal hypertension per se in producing these changes because there is no hepatic dysfunction in EHPVO...
June 2009: Hepatology International
F Streitparth, F Santosa, J Milz, B Gebauer, U Teichgräber, B Hamm, N Hidajat
PURPOSE: To evaluate the feasibility of TIPS for portal vein thrombosis (PVT) with/without portal vein occlusion/cavernomatous transformation and with/without underlying cirrhosis. MATERIALS AND METHODS: 13 patients with PVT and refractory ascites (n=7) and variceal bleeding (n = 6) received TIPS placement. The function of the TIPS was controlled with Doppler US at 1 day, 1 week, 4 weeks and 4 months after TIPS placement and subsequently every 3 months. The rates of technical success, revision and mortality after TIPS were evaluated...
October 2008: RöFo: Fortschritte Auf Dem Gebiete der Röntgenstrahlen und der Nuklearmedizin
Giovanni Galati, Umberto Vespasiani Gentilucci, Ilaria Sansoni, Sandro Spataro, Francesco M Di Matteo, Enrico M Zardi, Rosario F Grasso, Giuseppe Avvisati, Antonella Afeltra, Antonio Picardi
The most important systemic etiological factors for portal thrombosis are the thrombophilic conditions associated with myeloproliferative disorders (MPDs), either clinically manifest or so-called 'occult'. Latest studies have revealed a strong association between MPDs and a somatic point mutation (V617F) of the tyrosine kinase Janus kinase2. The 'pseudo-cholangiocarcinoma sign' is a well described radiological finding related to portal cavernoma. We describe the case of a 48-year-old man, presenting with cavernomatous transformation of an extrahepatic thrombotic obstruction of the portal vein mimicking a neoplastic mass of the hepatic hilum, who did not present any other cause for thrombosis except for the V617F Janus kinase2 mutation, suggesting an underlying MPD...
February 2009: European Journal of Gastroenterology & Hepatology
Catalin Vasilescu, Oana Stanciulea, Monica Popa, Anca Colita, Constantin Arion
UNLABELLED: An 8-year-old girl presented with a history of pain in the right hypocondrium, multiple petechiae in the skin, and ecchimoses at sites of minor trauma. Laboratory investigations showed severe thrombocytopenia. Doppler ultrasonography and magnetic resonance imaging showed portal and splenic vein cavernomatous transformation and splenomegaly. The patient underwent laparoscopic subtotal splenectomy with lower pole preservation and esophagogastric devascularization. The postoperative course was uneventful...
July 2008: Journal of Pediatric Surgery
E F Georgescu, Ligia Stănescu, Daniela Dumitrescu, Reanina Ionescu, Iuliana Georgescu
UNLABELLED: Sturge-Weber syndrome is a rare disorder consisting of a port-wine nevus in the distribution of the ophthalmic branch of the trigeminal nerve and central nervous system malformations. Facial cutaneous vascular malformation, seizures, and glaucoma are among the most common symptoms and signs. The syndrome results from malformation of the cerebral vasculature located within the pia mater, most commonly over the occipital region. These malformations led to venous hypertension and subsequent hypoperfusion on the underlying cortex, causing chronic cerebral ischemia, atrophy, calcification and neurological deterioration...
2007: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
Musturay Karcaaltincaba, Mithat Haliloglu, Erhan Akpinar, Deniz Akata, Mustafa Ozmen, Macit Ariyurek, Okan Akhan
Periportal region is an anatomic space around portal vein comprising hepatic artery, bile duct, nerves, lymphatics and a potential space. Periportal pathologies may involve any of these structures diffusely or focally with characteristic radiologic findings. Radiologic findings can be helpful in differential diagnosis of pathologies of periportal structures including periportal cavernomatous transformation, hepatic artery aneurysm, biliary diseases, neurofibromatosis, lymphoma, langerhans' cell histiocytosis, periportal fatty infiltration and other causes of periportal halo in adult and pediatric patients...
January 2007: European Journal of Radiology
Tutku Soyer, Arbay O Ciftci, F Cahit Tanyel, M Emin Senocak, Nebil Büyükpamukçu
BACKGROUND/PURPOSE: A retrospective study was performed to evaluate risk factors, clinical features, and treatment modalities of portal vein thrombosis (PVT) after splenectomy in pediatric hematologic disease. METHODS: Sixty-eight patients who underwent splenectomy for various hematologic diseases were evaluated with regard to age, sex, blood count, and splenic mass. Patients who developed PVT were also reviewed for clinical features, treatment modalities, and outcome...
November 2006: Journal of Pediatric Surgery
Thuong G Van Ha, Justin Hodge, Brian Funaki, Jonathan Lorenz, Jordan Rosenblum, Christopher Straus, Jeff Leef
PURPOSE: To determine the safety and efficacy of transjugular intrahepatic portosystemic shunt (TIPS) creation in patients with liver cirrhosis complicated by thrombosed portal vein. METHODS: This study reviewed 15 cases of TIPS creation in 15 cirrhotic patients with portal vein thrombosis at our institution over an 8-year period. There were 2 women and 13 men with a mean age of 53 years. Indications were refractory ascites, variceal hemorrhage, and refractory pleural effusion...
September 2006: Cardiovascular and Interventional Radiology
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