A case report of Budd-Chiari syndrome with cardiac complications and low SPO2 successfully managed by an integrated-approach

Budd-Chiari syndrome [BCS] is a rare, potentially life-threatening condition characterized by the triad of abdominal pain, ascites, and hepatomegaly (with or without jaundice). There will be an underlying disorder in most cases. The diagnosis, as well as management of the case, requires a multidisciplinary approach. The treatment should aim at reducing the presenting symptoms as well as removing the underlying pathology. Here we explain a case report of a 21-year-old male patient in the subacute stage of BCS with its complications and considered as a candidate for liver transplantation. His liver enzymes, PT/INR, D- dimer, and homocysteine values were above normal levels. The arterial oxygen saturation level was subnormal, and he was on supportive oxygen supplement. Inferior venacava [IVC] Doppler revealed a non-obstructive intrahepatic thrombus. The patient was treated with Homoeopathic medicine Arsenicum album and Arnica montana, given as an adjuvant to conventional treatment.

Ascitis en paciente con colitis ulcerativa como presentación de síndrome de Budd-Chiari: reporte de caso y revisión de la literatura / Ascites in a patient with ulcerative colitis presenting Budd-Chiari syndrome: case report and literature review

La enfermedad inflamatoria intestinal es una patología crónica en la que participa el sistema inmune, que puede acompañarse de manifestaciones extraintestinales en el hígado y vías biliares. El síndrome de Budd-Chiari se ha descrito previamente como una complicación rara de la colitis ulcerativa. Se describe el caso de una paciente joven con antecedente de colitis ulcerativa y trombosis venosa de miembro inferior, en manejo crónico con aminosalicilatos y anticoagulada durante 6 meses, quien debutó con cuadro clínico de ascitis de dos meses de evolución, con líquido ascítico de características hipertensivas, documentación imagenológica y biopsia hepática compatibles con síndrome de Budd-Chiari. Se presenta reporte de caso y revisión breve de la literatura.

Inflammatory bowel disease is a chronic disease involving the immune system, which can be accompanied by extraintestinal manifestations in the liver and biliary tract. Budd-Chiari syndrome has previously been described as a rare complication of ulcerative colitis. We describe the case of a young patient with a history of ulcerative colitis and venous thrombosis of the lower limb, under chronic management with aminosalicylates, and anticoagulation therapy for the past 6 months, who consulted with a 2-month history of ascites, with hypertension-related ascitic fluid, imaging analysis and liver biopsy compatible with Budd-Chiari syndrome. A case report and a brief literature review are presented.

Manejo de pacientes con síndrome de Budd-Chiari. Revisión de tema / Treatment of patients with Budd-Chiari syndrome. Literature review

El síndrome de Budd-Chiari (SBC), descrito en 1845, se define como la obstrucción del flujo venoso hepático en ausencia de enfermedad cardíaca o pericárdica. En Colombia no se tienen datos epidemiológicos claros de esta patología, la cual alrededor del mundo se considera poco frecuente. Se diagnostica al demostrar la obstrucción del flujo de las venas hepáticas. Tiene diversas manifestaciones clínicas como fiebre, ascitis, dolor abdominal y circulación colateral, entre otras. En ciertos casos es asintomática y en su gran mayoría se acompaña de patologías protrombóticas. El manejo inicial depende de la condición del paciente; sin embargo, se ha propuesto el manejo escalonado, donde se inicia con anticoagulación, se continúa con angioplastia, luego con desvío portosistémico intrahepático transyugular (TIPS), y se termina con trasplante hepático. El pronóstico depende de un diagnóstico precoz y un tratamiento adecuado. En las mejores circunstancias se alcanza una sobrevida a cinco años en el 90% de los casos, mientras que en ausencia de manejo, la tasa de mortalidad a un año alcanza el mismo porcentaje.

Budd-Chiari syndrome (SBC), described in 1845, is defined as the obstruction of hepatic venous flow in the absence of heart or pericardial disease. In Colombia there are no clear epidemiological data of this pathology, that around the world is considered rare. It is diagnosed by demonstrating the obstruction of the flow of the hepatic veins. It has various clinical manifestations such as fever, ascites, abdominal pain and collateral circulation, among others. In certain cases, it is asymptomatic but in the great majority it is accompanied by prothrombotic pathologies. Initial management depends on the patient's condition; however, staggered management has been proposed, beginning with anticoagulation, continuing with angioplasty, then with transjugular intrahepatic portosystemic shunt (TIPS), and finally, with liver transplantation. The prognosis depends on an early diagnosis and proper treatment. In the best circumstances, a five-year survival is achieved in 90% of cases, while in the absence of treatment, the one-year mortality rate reaches the same percentage.

Acute Hepatitis Like Presentation of Budd Chiari Syndrome in a Child.

The patient presented with progressively increasing ascites and pain abdomen but without any bleeding tendency, or encephalopathy. The initial diagnosis of acute hepatitis was proved wrong by contrast enhanced CT scan of abdomen which showed atretic hepatic veins. Atretic hepatic veins causing Budd Chiari Syndroem (BCS) is a rarity in literature.

[Budd-chiari syndrome a rare complication of hepatic echinococcosis; about 3 cases]

Hydatid disease is a parasitic infestation due to the development of echinococcus granulosus in the organism. This disease is particularly frequent in Tunisia where echinococcosis is endemic. Liver is the most common organ to be involved by hydatidosis and several complications have been described. Vascular complications secondary to hepatic echinococcosis such as Budd-Chiari syndrome have rarely been reported. We present herein, three rare cases of hydatid cyst complicated by Budd-Chiari syndrome. The cyst occupied at least two segments of the hepatic dome in all patients and his diameter was 12, 5 cm, 21 cm and 12, 5 cm respectively. According to the Gharbi classification, the cysts were type III in one case and type IV in the two others. The Budd-Chiari syndrome was chronic in one patient and asymptomatic in the two others. His diagnosis was established by abdominal US in two cases, completed by US Doppler and CT angiogram in one patient and by CT angiogram in the other. The diagnosis was established only by CT angiogram in the later case. The Budd-Chiari syndrome was due to compression of two hepatic veins in one case, compression of inferior vena cava in one case and compression of one hepatic vein and inferior vena cava in one case. Laparotomy was performed in all patients. The surgical procedure consisted in resection of the protruding hepatic tissue after puncture aspiration of the cyst in the three cases. The postoperative period was complicated by abscess formation in residual cavity, requiring drainage and by development of ascitis, in one case. Hydatid cyst should be remembered amongst the causes of Budd-Chiari syndrome in countries where the disease is endemic. Conversely, this vascular complication should be looked for routinely in patients with hydatid disease of the liver

[Association Budd Chiari syndrome, antiphospholipid syndrome and Grave's disease]

Antiphospholipid syndrome is revealed by Budd Chiari syndrome in 5% of the cases. Antiphospholipid syndrome is characterized by venous or arterial thrombosis, foetal loss and positivity of antiphospholipid antibodies, namely lupus anticoagulant, anticardiolipin antibodies and anti-beta2-glycoprotein I. Anticardiolipin antibodies was reported in auto-immune thyroid disorders, particularly in Grave's disease. Antiphospholipid syndrom associated to Grave's disease was reported in only three cases. To describe a case report of association of Grave's disease and antiphospholipid syndrome. We report the first case of Grave's disease associated with antiphospholipid syndrome, revealed by Budd Chiari syndrome. Our observation is particular by the fact that it is about a patient presenting a Grave's disease associated with antiphospholipid syndrome revealed by Budd Chiari syndrome. This triple association has never been reported in literature. Although association between antiphospholipid syndrome and Grave's disease was previously described, further studies evaluating the coexistence of these two affections in the same patient would be useful

Changing profile of Budd Chiari syndrome in India.

There is increasing evidence that Budd Chiari syndrome occurs when acquired predisposing factor(s) affect a susceptible individual with one or more underlying thrombophilic conditions. Geographical variations in disease pattern of Budd Chiari syndrome exist, which may reflect differing predisposing factors. We review a change in disease profile of Budd Chiari syndrome in India over the past three decades. While earlier studies from India reported isolated inferior vena cava (IVC) obstruction as the commonest disease type, this is a minority in more recent reports where a combination of IVC and hepatic vein obstruction is the commonest type. Longer duration of illness has been shown to be associated with IVC obstruction and the recent change in disease profile in India may reflect earlier diagnosis of Budd Chiari syndrome. Poverty, malnutrition, recurrent bacterial infections and filariasis have been previously suggested as predisposing factors for IVC obstruction. Improvement in hygiene and sanitation may partly explain the recent change in disease profile of Budd Chiari syndrome in India.

Direct intrahepatic cavo-portal shunts in Budd-Chiari syndrome: Role of simultaneous fluoroscopy and trans-abdominal ultrasonography.

BACKGROUND: Transjugular intrahepatic porto-systemic shunt (TIPS) for Budd-Chiari syndrome (BCS) can be inserted from inferior vena cava or hepatic vein to portal vein. The former is performed when hepatic veins are not suitable and is technically more challenging. METHODS: In this retrospective study, 7 patients with chronic BCS needed cavo-portal shunt as hepatic veins were neither amenable to plasty nor provided access for TIPS placement. Simultaneous fluoroscopic and trans-abdominal ultrasound guidance was used at the time of portal vein puncture. RESULTS: Technical success and clinical improvement were obtained in all patients. Median 3 (range 1-4) attempts were needed to puncture the portal vein. There were no significant complications. Uncovered stents were used in six patients and stent occlusion was common, but could be managed by re-intervention. CONCLUSION: Cavo-portal shunt is an effective technique for patients with BCS uncontrolled by medical therapy. Additional trans-abdominal ultrasound in oblique parasagittal plane keeps the procedure safe.

Budd-Chiari syndrome--a case report.

A 21year old male presented with abdominal pain for 2 months and abdominal distension and swelling of lower limbs for 1 month. Ultrasonography of abdomen showing coarse echotexture of liver and intraluminal filling defect of inferior vena cava (IVC) and CT scan confirming the USG finding and showing enlarged caudate lobe of liver and thrombus in proximal IVC suggested the possibility of Budd-Chiari syndrome. Confirmation of diagnosis was done by inferior venacavography. The patient had nephrotic syndrome as the risk factor for thrombosis. The patient was portally decompressed by portocaval shunt with significant symptomatic relief.

Budd-Chiari syndrome.

A 30 years old, non-alcoholic farmer from Trishal, Mymensingh was admitted in Mymensingh Medical College Hospital on 7 February, 2004 with the complaints of gradual swelling of abdomen, both legs and upper abdominal pain for 3 months. For the last 6 years, he was treated as a case of chronic liver disease (CLD) with spironolactone and frusemide. He was non-icteric, mildly anaemic with mild oedema, clubbing, gynaecomastia and engorged vein over anterior abdominal wall, flanks and back. Direction of venous flow was from below upward. There were mild hepatosplenomegaly, ascites and bilateral testicular atrophy. He was diagnosed as a case of Budd-Chiari Syndrome (BCS) on the basis of physical examination and it was confirmed by the findings of ultrasonography, liver scan and doppler study. The patient was managed by medical therapy alone.

Budd chiari syndrome: experience at king faisal specialist hospital and research center, Riyadh, Saudi Arabia

Budd-chiari syndrome [BCS] is rare disorder, varied in etiology, inconsistent in presentation, unpredictable in progression, and challenging in therapy. Aim of study: The aim of this study is to review experience of Budd- Chiari syndrome at KFSH, Riyadh; over at ten-year period. In a retrospective study using a computer data search of the medical records from May 1990 to May 2000, 43 patients' charts suspected of BCS were reviewed. 29 patients were found to have BCS, diagnosed by Doppler ultrasound, venography, MRI, or CT with or without liver biopsy. Findings: Of the 29 patients, 18 were male [62.1%] and 11 were female [37.9%]; mean age was 35.4 [range 17-69 years]. Nine patients had Behect's disease seven patients had malignancy [five HCC, one melanoma and one hypernephroma]. Eight patients had antiphospholipid syndrome, two patients one melanoma and one hypernephroma]. Eight patients had antiphospholipid syndrome, two patients had no known cause and three [one related to trauma, the other related to protein C deficiency and the third related nephrotic syndrome]. Nineteen patients presented acutely with abdominal pain, progressive ascites and hepatomegaly. Ten patients had subacute presentation mimicking cirrhosis of liver. Five patients had web in the intrahepatic IVC or ostium HV and the remaining 24 patients had thrombosis of hepatic veins. Five patients received surgical shunts, three had balloon dilatation of webs, 11 had anticoagulant therapy and seven patients had symptomatic treatment. Eleven patients died, six of them had malignancy, two died while awaiting liver transplantation and three died with fulminant hepatic failure. Fourteen patients are still on follow-up. Budd Chiari syndrome in a Saudi Arabia is related to a defined cause in majority of patients [92%]. Behcet's disease is a dominant cause of BCS in Saudi Arabia. This is in contrast to world literature where around 50% of patients are of unknown etiology

Tratamento da síndrome de Budd-Chiari por meio da colocação de tips e de "stent" venoso supra-hepático / Transjugular intrahepatic portosystemic shunt (TIPS) and suprahepatic venous stenting in the management of Budd-Chiari syndrome

OBJETIVOS: Descrever o uso do "shunt" intra-hepático portossistêmico (TIPS) e do "stent" venoso supra-hepático no manejo da síndrome de Budd-Chiari, enfocando suas indicações, aspectos técnicos e benefícios do procedimento. MATERIAIS E MÉTODOS: De janeiro de 1999 a março de 2002, nove casos de síndrome de Budd-Chiari foram encaminhados ao Serviço de Hemodinâmica do Hospital São Lucas, Porto Alegre, RS. A obstrução venosa supra-hepática foi constatada em todos os casos por meio de ultra-sonografia com Doppler em cores. A criação de TIPS foi realizada entre o sistema venoso supra-hepático ou a veia cava inferior e a veia porta, posicionando-se a endoprótese entre as duas abordagens. Doppler em cores pós-procedimento foi efetuado em todos os pacientes em períodos seriados. RESULTADOS: Três casos foram tratados inicialmente com inserção de "stent" venoso por apresentarem estenose preponderante em veias supra-hepáticas. Em dois desses casos ocorreu trombose do "stent", sendo necessária colocação de TIPS. Os demais seis casos foram tratados primariamente com TIPS. Dos oito "shunts" criados, trombose da endoprótese foi constatada em três casos, resolvidas com limpeza dos trombos e dilatação com balão em um caso e inserção de novas próteses nos demais. Embolização com molas de colaterais venosas ectasiadas foi efetuada em um paciente. CONCLUSÕES: A colocação de TIPS constitui-se numa estratégia terapêutica segura e efetiva na síndrome de Budd-Chiari, promovendo uma significativa melhora clínica e hemodinâmica dos pacientes, evitando procedimentos mais invasivos e podendo, em casos sem cirrose estabelecida, servir de tratamento definitivo da hipertensão portal

OBJECTIVE: To evaluate the use of transjugular intrahepatic portosystemic shunt (TIPS) and suprahepatic venous stenting in the management of Budd-Chiari syndrome, emphasizing the indications, technical aspects and the advantages of the procedure. MATERIALS AND METHODS: Nine patients with Budd-Chiari syndrome were referred to the Hemodynamics Service of "Hospital São Lucas", Porto Alegre, RS, Brazil, for percutaneous procedure. Suprahepatic venous occlusion was detected in all cases using Doppler sonography. In the TIPS procedure, a snare loop was identified in the inferior vena cava just below the right atrium at the site of expected outflow of the occluded hepatic vein. After sonographic and fluoroscopic localization of the portal bifurcation, the needle was advanced into the portal vein and a stiff guide wire was introduced. Venography was performed and a stent was implanted. Follow-up color Doppler examinations were obtained in all cases at different intervals. RESULTS: Prominent hepatic venous stenosis was detected in three patients that were treated with suprahepatic stent implantation. Two of these patients required TIPS due to thrombosis of the stent. In the other six patients a TIPS creation was done. During follow-up, shunt dysfunction occurred in three of eight patients, requiring repeat intervention and insertion of another endoprostheses in two patients, mechanical thrombectomy and balloon dilatation in one and coil embolization of ectatic collaterals in another patient. CONCLUSION: TIPS is a safe and effective procedure for the treatment of portal hypertension caused by Budd-Chiari syndrome, allowing clinical and hemodynamic improvement and avoiding invasive approaches.

Portal system thrombosis: a complication of long-term residence at extreme altitude.

BACKGROUND: With induction of the Indian Army to heights over 5,000 meters above sea level, complications of long-term stay at extreme altitude have come to light. We describe our experience with patients developing portal system thrombosis. METHODS: Clinical data were collected over 2 years on patients hospitalized for thrombotic complications from high-altitude (HAA) and non-high altitude (non-HAA) areas. Site of thrombosis was confirmed by imaging or at surgery. Patients were investigated for conditions that predispose to venous thrombosis. RESULTS: Ten cases of portal system thrombosis were seen during the period; of these, 9 (mean age 28 [SD 3.9] years; all men) were from HAA. Mean duration of residence in HAA was 12.4 (4.9) months; two were smokers and six drank 7-22 g alcohol/day at least 5 times a week. The first symptom was abdominal pain; this was later complicated by gastrointestinal bleeding (n=5), fever (7), vomiting (7), and weight loss (5). Average time between onset of first symptom and reaching a tertiary-care hospital was 9.5 (4.7) days. Clinical examination showed ascites (8 cases), splenomegaly (7), and hepatomegaly (6). Mean hemoglobin level at admission was 15.8 (3.4) g/dL. Ascites was hemorrhagic; five cases also had large splenic hematoma. The site of thrombosis was splenic vein (7 cases), portal vein (6), and superior (4) and inferior (1) mesenteric vein. None of 5 patients investigated had any prothrombotic condition. Endoscopic/ sonographic evidence of development of collaterals appeared as early as 12-20 days after onset of symptoms. CONCLUSION: Residence in HAA for extended periods is a risk factor for development of portal system thrombosis. Persistent pain in abdomen in such individuals should raise the possibility of portal system thrombosis.

Leiomyosarcoma of inferior vein cava presenting as acute Budd-Chiari syndrome.

We report a 49-year-old lady who presented with acute Budd-Chiari syndrome. Spiral CT scan showed inferior vein cava (IVC) tumor and ischemia of the right liver secondary to hepatic vein blockage. These were confirmed by MRI scan and IVC gram, at which time tissue diagnosis was obtained. At surgery, the tumor was seen to originate from the infrahepatic IVC and extended to the level of the diaphragm, blocking the hepatic vein outflow. The tumor was excised completely. Histology confirmed it to be leiomyosarcoma of the IVC. The patient is well, without recurrence of symptoms or tumor, 10 months later.

Bleeding scrotal varices as presentation of Budd-Chiari syndrome.

Budd-Chiari syndrome presents with ascites, edema and bleeding from esophageal varices. Presentation as bleeding scrotal varices is rare. We report a patient with Budd-Chiari syndrome who presented with recurrent bleeding from scrotal varices for 20 years.

Fisiopatologia e diagnóstico da síndrome de Budd-Chiari / Physiopathology and diagnosis of the Budd-Chiari syndrome

A Síndrome de Budd-Chiari (SBC) compreende grupo heterogêneo de distúrbios caracterizados pela supressão parcial ou completa da drenagem venosa hepática. Sua apresentação clínica depende da causa e do sítio de obstrução venosa e as opções de tratamento são variáveis. Neste trabalho os autores revisam temas relacionados a fisiopatologia, diagnóstico, discutindo aspectos controversos publicados na literatura