scholarly journals Mesoatrial shunt in Budd-Chiari syndrome

2009 ◽  
Vol 66 (1) ◽  
pp. 69-73 ◽  
Author(s):  
Darko Mirkovic ◽  
Nebojsa Stankovic ◽  
Miodrag Jevtic ◽  
Miroslav Mitrovic ◽  
Milan Jovanovic
Keyword(s):  
Computed Tomography ◽  
Vena Cava ◽  
Factor V Leiden ◽  
Lower Limbs ◽  
Portal System ◽  
Hepatic Veins ◽  
Budd Chiari Syndrome ◽  
Factor V Leiden Mutation ◽  
Chiari Syndrome ◽  
The Right

Background. Budd-Chiari syndrome (BCS) represents partial or total occlusion of the hepatic veins with or without simultaneous obstruction of vena cava inferior (VCI). The symptoms of BCS are abdominal pain, hepatomegaly, ascites, varices of the abdominal wall, sometimes bleeding from the upper part of gastointestinal tract (GIT), lower limbs swelling and jaundice. Primary BSC is a relatively rare condition occurring in one per 100 000 of the population worldwide. Case report. A male patient, 25-year-old, facing tooth postextraction complications, was presented with acute BCS. On admission, physical examination revealed pale-grayish complexion, more pronounced veins over the thorax and abdomen, ascites, enlarged liver rising 8 cm below the right costal arch and having a minor pleural effusion by the right side. The patient was submitted to Doppler sonography and computed tomography (CT) that verified the right leg deep veins thrombosis, as well as the presence of a thrombus in the intrahepatic portion of the VCI. Multislice computed tomography (MSCT) showed occlusion of hepatic veins (Budd-Chiari syndrome) and thrombosis of the VCI in the retrohepatic part 6 cm long. Also, increased values of transaminases and gamma GT and reduced values of albumines and serum ferrum were registered. Molecular examination revealed Factor V Leiden mutation - heterozygote. After preoperative preparations a mesocaval shunt was made using Gore- Tex ring graft of 12 mm. Intraoperatively, the blue enlarged liver was found with almost black zones of tense capsule. After a graft making, liver congestion decreased followed by the change of colour and volume. Within postoperative course metabolic and synthetic liver functions were obvious. Conclusion. In patients with BCS medicamentous treatment does not yield adequate results, but even causes worsening of general condition. Surgical therapy in the presented patient was performed timely regarding the stage of the disease due to which irreversible liver changes were prevented while decompression of the portal system provided time overbridging up to liver transplantation.

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.

scholarly journals A Case Series of Budd-Chiari Syndrome in Nigerian Patients: Diagnosis, Associations, Management, and Outcomes

2021 ◽  
Author(s):  
Hammed Ninalowo ◽  
Aderemi Oluyemi ◽  
Omodele Olowoyeye ◽  
Abisoye Ajayi
Keyword(s):  
Inferior Vena ◽  
Vena Cava ◽  
Case Series ◽  
Hepatic Veins ◽  
Budd Chiari Syndrome ◽  
Resource Limited ◽  
Clinical Series ◽  
Chiari Syndrome ◽  
The Right ◽  
Invasive Techniques

AbstractBudd-Chiari syndrome (BCS) is defined as hepatic outflow obstruction regardless of the cause or level of obstruction—from small hepatic veins to the opening of the inferior vena cava in the right atrium. BCS could be primary or secondary. Very few cases of this syndrome have been reported in Nigeria and there is no local clinical series documenting the noninvasive and invasive imaging findings and importance of interventional radiology techniques in its diagnosis and management.We report three cases of patients diagnosed with BCS in Lagos, Nigeria. We hope this work will raise awareness about the condition and its associations and show that much can be done to establish diagnosis and care with minimally invasive techniques in our resource-limited environment.

scholarly journals Familial Budd-Chiari Syndrome in China: A Systematic Review of the Literature

2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Xingshun Qi ◽  
Juan Wang ◽  
Weirong Ren ◽  
Ming Bai ◽  
Man Yang ◽  
...  
Keyword(s):  
Familial Aggregation ◽  
Factor V Leiden ◽  
Lower Limbs ◽  
Prothrombin G20210a ◽  
Factor V ◽  
Review Of The Literature ◽  
Budd Chiari Syndrome ◽  
Factor V Leiden Mutation ◽  
Congenital Vascular Malformation ◽  
Chiari Syndrome

Familial occurrence of Budd-Chiari syndrome (BCS) has been reported in scattered cases, which potentially favors the congenital theory. A review of the literature was conducted to demonstrate this phenomenon in China. PubMed, VIP, and CNKI databases were searched for studies describing at least two Chinese BCS patients from the same one family. In the 18 eligible papers, 30 siblings or first-degree relatives from 14 families were diagnosed with BCS at 9 different centers. Common clinical presentations included varices of abdominal wall and lower limbs, edema of legs, and ascites. Type and location of obstruction were similar among these patients from the same one family. Screening for BCS was conducted in 65 family members from 3 families, demonstrating that 2 asymptomatic siblings from one family were further diagnosed with BCS. Factor V Leiden mutation was found in 3 of 4 patients from one family and in one of 2 patients from another one family. Prothrombin G20210A gene mutation was found in none of the 4 patients from the 2 families. In conclusion, our study showed the possibility of familial aggregation in Chinese BCS patients, but these available data cannot support the previous hypothesis that familial BCS originates from congenital vascular malformation.

scholarly journals Acute Budd Chiari Syndrome

2014 ◽  
Vol 31 (4) ◽  
pp. 223-226
Author(s):  
AFMH Uddin ◽  
M Wareshuzzaman ◽  
RK Banik ◽  
SK Saha ◽  
NC Nath ◽  
...  
Keyword(s):  
Laboratory Data ◽  
Vena Cava ◽  
Protein S ◽  
Abdominal Distension ◽  
Hepatic Veins ◽  
Budd Chiari Syndrome ◽  
Abdominal Ultrasonography ◽  
Factor Deficiency ◽  
Chiari Syndrome ◽  
The Right

Budd-Chiari syndrome (BCS) is a rare disease in population characterized by hepatic venous outflow obstruction at a site that may vary from the level of the small hepatic veins up to the junction of the inferior vena cava with the right atrium . Here we presented a case of 35 year old male who presented with abdominal pain, abdominal distension and leg oedema . Subsequent physical examination, laboratory data, abdominal ultrasonography and duplex study all confirmed the diagnosis of BCS with Protein C , protein S , and antithrombin III deficiency . But we were unable to detect whether these factor deficiency were primary or secondary. Genetic study may be used to elucidate the cause. Whether primary or secondary, standard protocol should be used to treat Budd Chiari syndrome with these factor deficiency. DOI: http://dx.doi.org/10.3329/jbcps.v31i4.21009 J Bangladesh Coll Phys Surg 2013; 31: 223-226

Budd-Chiari Syndrome:Rare Cause, But Important of Portal Hypertension

2017 ◽  
Vol 68 (3) ◽  
pp. 608-611
Author(s):  
Gabriela Balan ◽  
Ana Maria Pelin ◽  
Luana Andreea Macovei ◽  
Alina Plesea Condratovici ◽  
Catalin Plesea Condratovici ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Vena Cava ◽  
Abdominal Ultrasound ◽  
Caudate Lobe ◽  
Lower Limbs ◽  
Budd Chiari Syndrome ◽  
Dyspeptic Symptoms ◽  
Chiari Syndrome ◽  
The Right ◽  
Early Diagnose

Budd-Chiari syndrome (BCS) (artery-occlusive hepatic disease) is a rare disease characterized by the obstruction of the blood flow at the level of the suprahepatic veins till their flow in the inferior vena cava (IVC) or at the level of IVC on the segment between the suprahepatic veins and the right atrium. The most frequent symptoms are abdominal pains, hepatomegaly and ascites. The imagistic investigations have an essential role in the early establishment of the diagnostic, evaluating the extension of disease and the management of BCS. Treatment depends on the presence or absence of symptoms and how acute the disease is.We present the case of a 43-years old woman, who had had for two month dyspeptic symptoms, increase of volume of the abdomen and oedema in the lower limbs. The biological investigations indicated hepatic dysfunction and thrombocytosis. The abdominal ultrasound showed modifications of chronic hepatopathy with signs of portal hypertension. The abdominal computer-tomography emphasized hepatomegaly with multiple nodules of regeneration, signs of portal hypertension (splenomegaly, moderate ascites), caudate lobe hypertrophy and thrombosis IVC. The patient was diagnosed with BCS and essential thrombocythemia. She started the medical treatment and was listed for liver transplantation. Budd-Chiari syndrome has to be taken into account every time we investigate the etiology of an acute or chronic hepatopathies, because the early diagnose can improve the patient�s prognostic.

scholarly journals Budd Chiari Syndrome in Children

KYAMC Journal ◽  
2020 ◽  
Vol 11 (1) ◽  
pp. 46-49
Author(s):  
Mukesh Khadga ◽  
Mahmudul Hasan ◽  
AZM Raihanur Rahman ◽  
Nazmul Ahamed ◽  
Md Benzamin ◽  
...  
Keyword(s):  
Treatment Options ◽  
Vena Cava ◽  
Hepatic Veins ◽  
Budd Chiari Syndrome ◽  
Delay In Diagnosis ◽  
Surgical Measures ◽  
Chiari Syndrome ◽  
The Right ◽  
End Stage ◽  
High Index Of Suspicion

Budd-Chiari syndrome (BCS) is a rare disorder presenting with hepatic venous outflow obstruction from the level of the hepatic veins to the junction of the inferior vena cava (IVC) with the right atrium. It is rare in infants and children so, delay in diagnosis or misdiagnoses are very commonly seen. It results from either primary or secondary cause. Hypercoagulable state can be identified in majority of the cases. The clinical presentations may vary from asymptomatic to fulminant liver failure. A high index of suspicion and imaging findings may help in early diagnosis of this condition.Doppler ultrasonography can be the initial diagnostic investigation of choice.The treatment options include medical therapy along with surgical measures including liver transplantation for end-stage liver disease. KYAMC Journal Vol. 11, No.-1, April 2020, Page 46-49

scholarly journals Budd-Chiari syndrome due to giant hydatid cyst: a case report and brief literature review

2013 ◽  
Vol 7 (06) ◽  
pp. 489-493 ◽  
Author(s):  
Sami Akbulut ◽  
Mehmet Yilmaz ◽  
Aysegul Kahraman ◽  
Sezai Yilmaz
Keyword(s):  
Inferior Vena Cava ◽  
Hydatid Cyst ◽  
Hydatid Disease ◽  
Inferior Vena ◽  
Vena Cava ◽  
Hepatic Veins ◽  
Budd Chiari Syndrome ◽  
Chiari Syndrome ◽  
Hepatic Venous Outflow ◽  
The Right

Budd-Chiari syndrome is an uncommon disorder characterized by the thrombotic or non-thrombotic obstruction of hepatic venous outflow anywhere along the venous course from the hepatic venules to the junction of the inferior vena cava and the right atrium. The etiology of Budd-Chiari syndrome is classified as primary, attributable to intrinsic intraluminal thrombosis or the development of venous webs; or secondary, caused by intraluminal invasion by a parasite or malignant tumor or extraluminal compression by an abscess, solid tumor, or cyst, such as a hydatid cyst. In this study, we present a case of a giant hydatid cyst manifesting Budd-Chiari syndrome symptoms and signs by compressing the inferior vena cava and hepatic veins. In brief, the case demonstrates that hydatid disease should be considered in the differential diagnosis of Budd-Chiari Syndrome in areas such as Turkey, where hydatid disease is endemic.

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 Intrahepatic Cholangiocarcinoma Presenting as the Budd-Chiari Syndrome: A Case Report and Literature Review

2005 ◽  
Vol 19 (12) ◽  
pp. 723-728 ◽  
Author(s):  
Joanna K Law ◽  
Jenny Davis ◽  
Anne Buckley ◽  
Baljinder Salh
Keyword(s):  
Inferior Vena Cava ◽  
Intrahepatic Cholangiocarcinoma ◽  
Inferior Vena ◽  
Primary Tumour ◽  
Vena Cava ◽  
Biliary Tree ◽  
Budd Chiari Syndrome ◽  
Radiological Findings ◽  
Chiari Syndrome ◽  
The Right

Intrahepatic cholangiocarcinoma, an increasingly recognized primary tumour of the liver, is associated with a very poor prognosis. A patient with this tumour who presented with Budd-Chiari syndrome (the first to the authors' knowledge in Western literature and only the third patient overall) secondary to extensive thrombosis in his inferior vena cava extending from the right atrium down to his iliac vessels is described. Neither curative nor palliative intervention was deemed to be an option in this patient, who deteriorated rapidly while on anti-coagulants. Postmortem examination confirmed the radiological findings, and histological analysis revealed characteristic appearances of this tumour within the biliary tree and invasion into the inferior vena cava. Furthermore, biliary dysplasia, which can be a precursor to this cancer, was also noted within some of the bile ducts.

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