scholarly journals MPL-Positive Essential Thrombocytosis Presenting as Budd-Chiari Syndrome in a Middle-Aged Woman with an Initially Normal Platelet Count

2021 ◽  
Author(s):  
Mohammad Ammad Ud Din ◽  
Hania Liaqat ◽  
Muhammad Osama
Keyword(s):  
Platelet Count ◽  
Vena Cava ◽  
Hepatic Veins ◽  
Budd Chiari Syndrome ◽  
Essential Thrombocytosis ◽  
Venous Flow ◽  
Normal Platelet Count ◽  
Normal Platelet ◽  
Aged Woman ◽  
Chiari Syndrome

Budd-Chiari syndrome (BCS) results from an occlusion of the hepatic venous flow which in turn leads to portal hypertension causing ascites and other signs of liver dysfunction. Here, we present the case of a 43-year-old woman with recurrent ascites who was found to have BCS secondary to an inferior vena cava thrombosis extending into the hepatic veins. Although she had a normal platelet count on admission, additional laboratory investigations revealed an MPL mutation. She was discharged on anticoagulation with apixaban and later found to have thrombocytosis on repeat blood work, confirming the diagnosis of essential thrombocytosis, following which she was started on myelosuppressive therapy with hydroxyurea.

Recanalization and Reconstruction of a Chronically Occluded Inferior Vena Cava Through an Existing Transjugular Intrahepatic Portosystemic Shunt in the Setting of Budd-Chiari Syndrome

2021 ◽  
pp. 153857442110020
Author(s):  
Reza Talaie ◽  
Hamed Jalaeian ◽  
Nassir Rostambeigi ◽  
Anthony Spano ◽  
Jafar Golzarian
Keyword(s):  
Inferior Vena Cava ◽  
Transjugular Intrahepatic Portosystemic Shunt ◽  
Inferior Vena ◽  
Vena Cava ◽  
Portosystemic Shunt ◽  
Hepatic Veins ◽  
Budd Chiari Syndrome ◽  
Chiari Syndrome ◽  
Intrahepatic Portosystemic Shunt ◽  
The Right

Budd-Chiari syndrome (BCS) results from the occlusion or flow reduction in the hepatic veins or inferior vena cava and can be treated with transjugular intrahepatic portosystemic shunt when hepatic vein recanalization fails.1-3 Hypercoagulable patients with primary BCS are predisposed to development of new areas of thrombosis within the TIPS shunt or IVC. This case details a patient with BCS, pre-existing TIPS extending to the right atrium, and chronic retrohepatic IVC thrombosis who underwent sharp recanalization of the IVC with stenting into the TIPS stent bridging the patient until his subsequent hepatic transplantation.

Budd-Chiari Syndrome in Childhood Secondary to Inferior Vena Caval Obstruction

PEDIATRICS ◽  
1979 ◽  
Vol 63 (5) ◽  
pp. 808-812
Author(s):  
Arvind Taneja ◽  
S. K. Mitra ◽  
P. D. Moghe ◽  
P. N. Rao ◽  
N. Samanta ◽  
...  
Keyword(s):  
Inferior Vena ◽  
Vena Cava ◽  
Hepatic Veins ◽  
Budd Chiari Syndrome ◽  
Venous Outflow ◽  
Massive Ascites ◽  
Inferior Vena Caval ◽  
Uncommon Disease ◽  
Chiari Syndrome ◽  
Hepatic Venous Outflow

Budd-Chiari syndrome is an uncommon disease caused by an obstruction to hepatic venous outflow either at the level of the hepatic veins or in the hepatic part of the inferior vena cava. Clinically, it presents with ascites, abdominal pain, hepatomegaly, edema, and occasionally jaundice. The syndrome was first recognised by Lamboran1 in 1842 and later described by Budd2 in 1846 and Chiari3 in 1899. The syndrome is caused by obstruction to the hepatic veins. In the Fig 1. Photograph showing massive ascites and dilated superficial abdominal veins. majority of cases, the obstruction is ascribed to obliterative thrombophlebitis of unknown cause.4

scholarly journals Balloon cavoplasty in membranous obstruction of vena cava

2017 ◽  
Vol 5 (7) ◽  
pp. 3239
Author(s):  
Sunil Abhisek B. ◽  
Kumar B.
Keyword(s):  
Right Atrium ◽  
Femoral Vein ◽  
Vena Cava ◽  
Hepatic Veins ◽  
Budd Chiari Syndrome ◽  
Case Scenario ◽  
Effective Treatment Option ◽  
Chiari Syndrome ◽  
Membranous Obstruction ◽  
Percutaneous Transluminal

A hepatic venous outflow tract obstruction at any level is considered as Budd Chiari syndrome (BCS). Primary BCS is usually due to a congenital membrane causing the obstruction; referred to as membranous obstruction of vena Cava (MOVC). In the past MOVC was predominantly treated through surgery, percutaneous transluminal balloon angioplasty (PTBA) is an alternative and effective form of treatment. Case scenario of a 32-year-old gentleman presented himself with complaints of hematemesis for one year and hematochezia for three months. Hepatomegaly was noted. An ultrasonogram revealed a dilated IVC, till its hepatic portion, and also the Hepatic Veins. There was a fibrous membrane like structure of about 5 mm thickness that was obstructing the IVC flow. Patient was taken up for venogram for conformation of diagnosis and intervention. 6F NIH catheter was introduced from Femoral vein, an injection into the IVC showed contrast not flowing into right atrium and there was a membranous obstruction for the forward flow. A Brockenbrough’s trans-septal needle with Mullins Sheath was used to puncture the membrane and right atrium was entered. A 22 mm Inouye mitral valvuloplasty balloon (Single Balloon, Toray Medical, Tokyo) was taken and positioned at the membrane and inflated and deflated several times with incremental pressures till the waist of the balloon disappeared. Conclusion membranous obstruction of vena cava (MOVC) is a common cause of primary Budd Chiari syndrome. Percutaneous transluminal angioplasty using Inoue or mansfield balloon with or without stenting is a safe and effective treatment option.

scholarly journals A Case of Budd-Chiari Syndrome Associated with Erythrocytosis and Homozygous MTHFR C677T Mutation

2021 ◽  
pp. 24-30
Author(s):  
Leilane Bentes De Sousa ◽  
Dayane Ferreira Aguiar ◽  
José Pereira de Moura Neto
Keyword(s):  
Case Report ◽  
Randomized Clinical Trials ◽  
Methylenetetrahydrofolate Reductase ◽  
Factor V Leiden ◽  
Mthfr C677t ◽  
Hepatic Veins ◽  
Factor V ◽  
Budd Chiari Syndrome ◽  
Venous Flow ◽  
Chiari Syndrome

An erythrocytosis describes an increased erythrocyte, subclassified into relative due to hemoconcentration or absolute by an increase in erythrocyte mass, defined as an increase in hemoglobin concentration and/or hematocrit in the peripheral blood above the sex-specific normal range. Budd-Chiari Syndrome (BCS) is related to an obstruction of the hepatic venous flow leading to occlusion of hepatic veins and their tributaries. Genetic and environmental factors can interact for risk determination of venous thromboembolism. The risk associated with SNP 677C>T and 1298A>C of the methylenetetrahydrofolate reductase (MTHFR), 1691G>A of the Factor V Leiden (FVL) and 20210G>A of the prothrombin (FII) genes were investigated in many studies involving thrombosis. This case report describes the clinical, hematological and biochemistry data about a 48-year-old woman diagnosed with PV and a BCS associated, also carrying 677C>T SNP in homozygosity. The patient started therapy with phlebotomy, hydroxyurea and oral anticoagulant. Currently, she presents a better clinical and laboratory condition with normalized values of hematological and platelet indices. This case report aims to contribute with evidence of related comorbidities and makes it possible to report that genetic factors are involved since the patient's mother had already been diagnosed with absolute erythrocytosis in 2016 at 78 years old. For this main result, we understand that it is clear that a family genetic study can reveal clinical modifying factors in these patients, as there are different clinical severities in the family. Furthermore, we believe in the need for a greater number of randomized clinical trials to add better evidence to complement an ideal therapeutic approach in these patients.

scholarly journals Acute Budd–Chiari syndrome due to a simple liver cyst

2014 ◽  
Vol 96 (1) ◽  
pp. 000-000 ◽  
Author(s):  
J Long ◽  
H Vaughan-Williams ◽  
J Moorhouse ◽  
H Sethi ◽  
N Kumar
Keyword(s):  
Liver Failure ◽  
Vena Cava ◽  
Liver Cyst ◽  
Organ Support ◽  
Hepatic Veins ◽  
Emergency Laparotomy ◽  
Budd Chiari Syndrome ◽  
Simple Cyst ◽  
Cystic Fluid ◽  
Chiari Syndrome

Simple liver cysts are common, rarely causing significant morbidity or mortality. Budd–Chiari syndrome (BCS) is caused by obstruction of hepatic venous outflow and is the leading cause of postsinusoidal liver failure. We present a rare case of BCS caused by a simple hepatic cyst. A 16cm × 16cm liver cyst was found on computed tomography of a 66-year-old woman presenting with abdominal pain. The cyst had become infected, thus enlarged, exerting mass effect with almost complete compression of the inferior vena cava. Shortly after admission, the patient developed acute liver failure, with deranged clotting and hepatic encephalopathy requiring full organ support on the intensive care unit. Cardiac output studies showed a low cardiac index of 1.4l/min/m2. An emergency laparotomy with fenestration of the cyst and drainage of 2l of purulent material led to a full recovery. Intraoperative cystic fluid aspirates later confirmed no evidence of Echinococcus. Histology confirmed a simple cyst. Liver biopsies showed severe, confluent, bridging necrosis, without background parenchymal liver disease. Acute BCS due to rapid compression of all major hepatic veins leading to fulminant hepatic failure is rare. Our case highlights a clinically significant complication of a simple liver cyst of which clinicians should be aware when managing these ‘innocent’ lesions.

Numerical Study of Blood Flow in the Hepatic Portion of the Inferior Vena Cava

2007 ◽  
Author(s):  
M. Matsubara ◽  
M. Watanabe ◽  
S. Watanabe ◽  
K. Konishi ◽  
S. Yamaguchi ◽  
...  
Keyword(s):  
Blood Flow ◽  
Inferior Vena Cava ◽  
Vessel Wall ◽  
Inferior Vena ◽  
Numerical Study ◽  
Vena Cava ◽  
Hepatic Veins ◽  
Partial Occlusion ◽  
Budd Chiari Syndrome ◽  
Chiari Syndrome

Budd-Chiari syndrome (BCS) is complete or partial occlusion in the hepatic veins and the hepatic portion of the inferior vena cava (IVC). The cause of BCS is not well known yet, however: abnormal vessel wall shear stress caused by blood flow is thought to increase the likelihood of developing BCS. In order to reveal the formation mechanism of BCS, we construct several vessel models of the IVC and hepatic veins from medical images and study the characteristics of the blood flow in the vicinity of the junctions of the hepatic veins with the IVC numerically.

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