Síndrome de Budd-Chiari: etiología, manejo y resultados en una cohorte de 35 pacientes del Hospital Pablo Tobón Uribe / Budd-Chiari Syndrome: Etiology, management, and outcomes in a study of 35 patients at the Hospital Pablo Tobón Uribe

Resumen El síndrome de Budd-Chiari (Budd-Chiari syndrome, BCS) es una entidad rara, definida por la obstrucción del flujo venoso del hígado y corresponde a una condición heterogénea tanto en presentación como en manejo terapéutico. A continuación, presentamos un estudio retrospectivo de pacientes con diagnóstico de BCS, atendidos por el grupo de hepatología del el Hospital Pablo Tobón Uribe. Los resultados se expresan como proporciones en las variables categóricas y como medias y rangos para las variables continuas. La edad promedio de presentación fue de 37,7 años y no hubo preponderancia por el género. Los principales síntomas fueron dolor abdominal (91,4 %), ascitis (71,4 %) y hepatomegalia (60 %). Asimismo, 11 pacientes (31,4 %) presentaron hipercoagulabilidad primaria, mientras que en 7 (20 %) se encontró algún síndrome mieloproliferativo. Además, 6 (17,1 %) tenían neoplasias extrahepáticas: 3 con compresión mecánica y 3 por hipercoagulabilidad asociada; solo en 1 caso se documentaron membranas en la vena cava inferior. Y en 5 casos no fue posible determinar la causa de trombosis. De igual forma, 32 pacientes (91,4 %) recibieron anticoagulación como primer manejo; solo 4 sujetos fueron remitidos a procedimientos: 2 a angioplastia, 1 a derivación portosistémica intrahepática transyugular (Transjugular Intrahepatic Portosystemic Shunt, TIPS) y 1 a trasplante ortotópico hepático (TOH). Luego de ello, 17 (48,5 %) recibieron procedimientos invasivos como segunda línea: 8 (22,8 %) se condujeron a angioplastia y endoprótesis (stent), 5 (14,29 %) a TIPS y 4 necesitaron TOH. Así, los 6 pacientes que se llevaron a TIPS no necesitaron trasplante hepático. El BCS continúa siendo una condición poco frecuente que afecta la salud de personas jóvenes, tanto hombres como mujeres, con una presentación clínica heterogénea. Sin embargo, en la mayoría de los casos ocurre por una causa desencadenante clara, entre las cuales predominan las trombofilias y los síndromes mieloproliferativos. El uso de medidas terapéuticas invasivas, especialmente de la selección temprana de los enfermos que se benefician de TIPS, ha cambiado la historia natural y el pronóstico de este grupo de pacientes.

Abstract Budd-Chiari syndrome (BCS) is a rare disorder characterized by the obstruction of the veins of the liver. Both its presentation and therapeutic management are heterogenous. The following is a retrospective study of patients diagnosed with BCS treated by the hepatology service at the Hospital Pablo Tobón Uribe. The results of the categorical variables are expressed as proportions and the continuous variables as means and ranges. The average age of onset of this disease was 37.7 years and it was not predominant in neither sex. The most common symptoms were abdominal pain (91.4%), ascites (71.4%) and hepatomegaly (60%). Out of 35 patients, 11 (31.4%) had primary hypercoagulability, 7 (20%) had some myeloproliferative disorder, 6 (17.1%) had extrahepatic malignancies -3 with mechanical compression and 3 due to associated hypercoagulability-, and 1 case had a membranous obstruction of the inferior vena cava. 32 patients (91.4%) received anticoagulation as first-line therapy and only 4 were taken to surgery: 2 to angioplasty, 1 to transjugular intrahepatic portosystemic shunt (TIPS) and 1 to orthotopic liver transplantation (OLT). On the other hand, 17 (48.5%) individuals underwent invasive procedures as a second-line therapy, of which 8 (22.8%) underwent angioplasty and stenting, 5 (14.29%) underwent TIPS and 4 needed OLT. The 6 patients who were taken to TIPS did not need liver transplants. Budd-Chiari syndrome remains a rare condition affecting the health of young men and women alike, with a heterogeneous clinical presentation but, in most cases, with a clear trigger where thrombophilic and myeloproliferative disorders predominate. The use of invasive therapeutic measures, especially the early selection of patients who may benefit from TIPS, has changed the natural course and prognosis of this group of patients.

CT guided interventional therapy for Budd-Chiari syndrome with inferior vena obstruction / 中国介入影像与治疗学

Objective To explore the clinical value of CT for guiding interventional therapy of Budd-Chiari syndrome (BCS) with inferior vena cava (IVC) obstruction.Methods A total of 329 consecutive BCS patients with IVC obstruction underwent endovascular treatment.All the patients underwent CT examination prior to endovascular treatment,and the data were retrospectively reviewed.The accuracy,sensitivity,specificity,positive predictive value (PPV) and negative predictive value (NPV) of CT were compared with results of DSA.Results DSA showed partial obstructive IVC in 108 BCS patients and complete obstructive IVC in 221 patients.CT demonstrated partially obstructive IVC in 99 patients and completely obstructive IVC in 230 patients.CT revealed 15 false-negative and 6 false-positive results.CT diagnosis of puncture or not yielded an accuracy of 94.19％,sensitivity of 97.29％,specificity of 86.11％,PPV of 93.49％ and NPV of 93.94 ％ for detection of BCS with partial IVC obstruction,while CT-based interventional treatment plan yield an accuracy of 97.55 ％,sensitivity of 100 ％,specificity of 92.52 ％,PPV of 96.49 ％ and NPVof 100％.Conclusion CT can provide high diagnostic accuracy and sensitivity for BCS patients with obstructed IVC,therefore being helpful to planning interventional treatment of BCS.

Clinical features of patients with Budd-Chiari syndrome caused by hepatic vein thrombosis / 中华肝胆外科杂志

Objective To study the clinical features and prognosis of patients with primary BuddChiari syndrome (BCS) caused by hepatic vein thrombosis.Method 16 patients with primary BCS caused by hepatic vein thrombosis treated in our hospital between June 2010 to December 2012 were used as the study group while 132 patients with primary BCS caused by other causes were used as the control group.A retrospective study was then employed to analyze the clinical data of the two groups of patients during hospitalization and on follow-up.The study was censored in June 2013.The median follow-up was 24 months (range,6 months to 36 months).The difference in quantitative data between the 2 groups were analyzed using the independent-samples t test and the Wilcoxon W rank sum test,and the difference in qualitative data were analyzed using the Chi-square test and the Fisher's exact test.The survival rates and recurrence rates were calculated using the Kaplan-Meier method.Result The study group was significantly lower than the control group in age,duration of symptoms,albumin level,diameter of spleen and survival rate,but it was significantly higher in the proportion of patients with ascites,average hospitalization time,alanine transaminase,aspartate aminotransferase,total bilirubin,carbohydrate antigen-125 and recurrence rate after percutaneous transluminal angioplasty.The differences were significant (P ＜ 0.05).The Rotterdam BCS prognosis grades of the study group were:9 patients grade Ⅱ and 7 patients grade Ⅲ.In the control group,there were 65 patients with grade Ⅰ,51 patients with grade Ⅱ,and 16 patients with grade Ⅲ.The prognosis grade of the study group was significantly higher than the control group (P ＜ 0.05).Conclusion When compared to the patients with BCS due to other causes,patients with BCS caused by hepatic vein thrombosis were more common in the young,most of them were diagnosed in the acute period,they had worse clinical outcomes and had more severe clinical symptoms and liver damage.

Application of vascular enhancement technology in the evaluation of therapeutic efficacy of stenting for Budd-Chiari syndrome / 中华超声影像学杂志

Objective To explore the value of vascular enhancement technology (VET) in the evaluation of therapeutic efficacy of stenting for Budd-Chiari syndrome (BCS).Methods Two-dimensional ultrasound,color Doppler and VET were performed on 39 BCS patients with 41 stents,the static and dynamic images of stents and vessels with stents were stored.VET and two-dimensional ultrasound imaging quality of intravascular stent were compared.Patients with stent stenosis found by ultrasound were examined by digital subtraction angiography(DSA).The ranges of stent thrombosis were measured by DSA and VET and the difference was compared.Results VET image quality scores of endovascular stent were higher than those of the two-dimensional ultrasound,the difference was statistically significant.The ranges of stent thrombosis measured by VET were similar to DSA.Conclusions VET can improve the clarity of intravascular stents.VET and color Doppler can enhance the clinical value of ultrasound in the evaluation of therapeutic efficacy of stenting for BCS.

Inhibition of endothelial progenitor cells against hepatic vein thrombosis after allogeneic bone marrow transplantation / 中华器官移植杂志

Objective To (e)xplore inhibition of endothelial progenitor cells (EPCs) against hepatic vein thrombosis after allogeneic bone marrow transplantation (BMT).Methods Balb/c mice were randomly divided into three groups: (1) BMT group [Balb/c mice were injected intravenously with 5 × 106 bone marrow cells after total body irradiation (TBI)]; (2) EPCs co-transfusion with bone marrow cells group: 5 × 105 EPCs were infused into recipient mice simultaneously; (3) Normal control group.Liver index was detected on the day 0,5,10,15 and 20 after transplantation.Hepatic vein thrombosis,hepatic cells and vascular endothelial damage were observed under the light microscopy after H&E staining.The injury of liver cells,liver veins,hepatic sinusoidal endothelial cells (SECs)and platelet adhesion conditions were observed under a transmission electron microscope (TEM).The proportion of activated platelets and TNF-α concentration in peripheral blood were detected by using flow cytometry.Results On the day 0,5,10,15 and 20 after transplantation,the proportion of activated platelets,liver index and TNF-α concentrations in BMT group and EPCs co-transfusion group showed an upward trend,peaked on the 15th day,and then decreased.However,they were still significantly higher than those in normal control group (P＜0.05).The above parameters in EPCs co-transfusion group at each time point were significantly lower than those in BMT group (P＜0.05).As compared with BMT group,platelet adhesion decreased,hepatic vein thromboses were reduced,hepatocyte swelling and necrosis were alleviated,and liver damage repaired rapidly in EPCs co-transfusion group.Conclusion EPCs co-transfusion with bone marrow cells could inhibit the hepatic veins thrombosis and ameliorate liver damage significantly.

Further study of sonographic examination skills and classifications of the inferior vena cava lesions in patients with Budd-Chiari syndrome / 中华超声影像学杂志

Objective To explore a compatible approach to detect and classify the lesions of inferior vena cavas (IVCs) on sonogram in patients with Budd-Chiari syndrome(BCS).Methods Ultrasonogram of the IVCs were observed detailedly in 300 patients with BCS by using trans-abdomen and trans-thorax-right atrium-inferior vena cava ingress sections.Transducers usually used for heart examination were applied in the latter.Lesions of the IVCs found in 277 out of 300 patients were classified.All lesions were confirmed by digital subtraction angiography (DSA) and among them,52 cases underwent computed tomography angiography (CTA).Results Lesions of IVCs were classified into 3 categories as follows:membranous type,segmental type,and ex-pressed type.① Membranous type (thickness ≤ 15 mm) included membranous stenosis type and membranous occlusion type.On the basis of the thickness,the membranous stenosis type was further classified into thinner membranous stenosis type (thickness ≤5mm) and thicker membranous stenosis type (5 mm＜thickness≤ 15 mm).The membranous occlusion type was further classified into thinner membranous occlusion type (thickness ≤5 mm) and thicker membranous occlusion type (5 mm＜thickness ≤15 mm).② Segmental type (lengtb ＞ 15 mm) was consist of segmental stenosis type and segmental occlusion type.Based on the length of the lesion,the segmental stenosis type was further divided into longer segmental stenosis type (length ＞ 30 mm) and shorter segmental stenosis type (15 mm＜length ≤30 mm).The segmental occlusion type was further divided into longer segmental occlusion type (length ＞ 20mm) and shorter segmental occlusion type (15 mm＜ length ≤20 mm).③ Ex-pressed type of IVCs was mainly caused by compression of intumescent caudate lobes.Corresponding sonographic features were demonstrated in each type.Conclusions Ultrasonogram of trans-abdomen and trans-thorax-right atrium-inferior vena cava ingress sections could accurately classify the lesions of IVCs.It is of important significance for the clinical treatment.

Color Doppler ultrasound diagnosis and classification with Budd-Chiari syndrome / 中华超声影像学杂志

Objective To study the color Doppler ultrasound image characteristics and ultrasonic diagnosis classification of Budd-Chiari syndrome (BCS),so as to facilitate the treatment choice.Methods The clinical material of 126 BCS patients were retrospectively analyzed.All patients were proved by angiography,intervention or surgical treatment,and were classified according to the main color Doppler ultrasound images.Results According to the image characteristics of color Doppler ultrasound,BCS patients were divided into eight types:type Ⅰ,incomplete membrane of inferior vena cava in 30 cases; type Ⅱ,complete membrane of inferior vena cava in 3 cases;type Ⅲ,stenosis of inferior vena cava in 8 patients;type Ⅳ,inferior vena cava obstruction in 3 patients;type Ⅴ,stenosis of big hepatic vein in 20 cases;type Ⅵ,big hepatic vein obstruction in 15 cases; type Ⅶ,extensive obstruction of small hepatic vein in 9 cases; type Ⅷ,38 cases of mixed lesions.Conclusions Color Doppler ultrasound can display the stenosis or obstruction of the inferior vena cava,hepatic vein with property,position,degree and the hemodynamic changes,which are accurate and reliable to the diagnosis and classification of BCS.

Clinical evaluation of interventional treatment for Budd-Chiari syndrome with hepatic vein thrombosis / 中华放射学杂志

Objective To evaluate the effect of interventional therapy for Budd-Chiari syndrome with hepatic vein thrombosis. Methods Twenty-five patients with Budd-Chiari syndrome complicated with hepatic vein thrombosis underwent catheter-directed urokinase thrombolysis, balloon dilation and/or stent placement. During follow-up, re-thrombosis and patency of the recanalized hepatic vein and inferior vena cava were evaluated by liver ultrasound. The pressure gradient of hepatic vein-right atrium or inferior vena cava-right atrium before and after interventional treatment was compared with paired t-test. ResultsTechnical success was obtained in 23 patients. Complete resolution and partial resolution of the thrombi were accomplished in 18 cases and 5 cases, respectively. The recanalized hepatic veins and inferior vena cava were patent. The mean pressure gradient of hepatic vein-right atrium dropped from (29±7) cm H2O to (8±3) cm H2O (1 cm H2O＝0.098 kPa) after the interventional treatment (t=13.7,P<0.01). The mean pressure gradient of inferior vena cava-right atrium dropped from (19±4) cm H2O to (5±2) cm H2O after the interventional treatment (t=13.3, P<0.01). Failures occurred in 2 patients. Over the follow-up period of 1 to 42 months[(18±10) months]after interventional treatment in the 23 patients, one late death occurred. Restenoses of hepatic veins were found in 2 patients, which were all redilated successfully. Neither restenosis of hepatic vein nor recurrence of thrombosis was found in the other 20 patients. Conclusion Interventional therapy could be effectively performed for the treatment of Budd-Chiari syndrome with hepatic vein thrombosis.

Analysis of expression of von Willebrand factor, endothelin-1, vascular endothelial growth factor in Budd-Chiari syndrome with membranous obstruction / 中华放射学杂志

Objective To investigate whether the injured vascular endothelial plays an important role in the membranous formation of the inferior vena cava (IVC). Methods There were 40 cases of membranous obstruction of inferior vena cava (MOVC) in the experimental group and 40 arrhythmic inpatients in the control group from affiliated hospital .There were 23 males and 17 females in experimental group while 21 males and 19 females in control group, and the age were (41.8±8.1) yrs and (43.2±7.6) yrs respectively. All of them had no anti-coagulation (clotting) drug history and smoking history, no hypertension, no pulmonary artery hypertension, no coronary heart disease, no valvular disease, no myocardial disease, no blood disease, no diabetes, no connective tissue disease and malignancy, and liver and renal function must be normal. And then the serum concentrations of von Willebrand factor(vWF), endothelin-1(ET-1), vascular endothelial growth factor(VEGF) were defined by enzyme-linked immunosorbent assay (ELISA). The results were analyzed with independent sample t-test. Results In MOVC patients, the serum concentrations of vWF, ET-1 and VEGF[ (37.8±6.6) μg/L, (31.9±6.0) ng/L, (20.84±5.78) μg/L] were higher than those in the control group[ (3.3±1.3) μg/L, (5.3±1.8) ng/L, (4.2±1.2) μg/L. t=32.65,26.70,17.85,P＜0.01, respectively]. ConclusionsThe injury of vascular endothelium is related to the formation of membrane in the IVC.

Ultrasound imaging features of hepatic regenerative nodules in Budd-Chiari syndrome after portacaval anastomosis / 中华超声影像学杂志

Objective To investigate the routine ultrasound and contrast-enhanced ultrasound (CEUS) features of hepatic nodules in Budd-Chiari syndrome(BCS) after portacaval anastomosis.Methods Routine ultrasonography and CEUS were performed in 18 BCS patients with hepatic nodules after portacaval anastomosis.Results Appearance after portosystemic anastomosis,multiplicity,small size,presence of peripheral rim and hypervascularization were important ultrasound imaging features of hepatic regenerative nodules in patients with BCS.Nodules showedquick wash-in and slow wash-out pattern in CEUS.Sixteen cases showed center-to-periphery enhancement pattern in arterial phase and hyper-enhancement in portal phase and late phase.Two cases showed periphery-to-center enhancement pattern in arterial phase and periphery enhancement in portal phase and late phase.Conclusions Hepatic regenerative have different features on routine ultrasound and CEUS in patients with BCS after portacaval anastomosis,which are useful for differential diagnosis.

Application of vascular enhancement technology in the diagnosis of Budd-Chiari syndrome and evaluation of the therapeutic efficacy of interventional therapy / 中华超声影像学杂志

Objective To explore the value of the application of vascular enhancement technology (VET) in the diagnosis of Budd-Chiari syndrome (BCS) and the evaluation of the therapeutic efficacy of interventional therapy.Methods B-mode ultrasound,color Doppler and VET were performed on 93 patients with BCS for the systematic detection of intrahepatic vessels and the inferior vena cava (IVC),with the static and dynamic images of these vessels stored prior to interventional therapy.Subsequent to the operation,the same procedures were repeated on the vessels concerned and the images were recorded.The definitions of B-mode and VET images of the detected vessels were compared by two sonography experts.Regarding DSA as standard,the diagnostic accordance rate of VET and B-mode imaging of the diseased vessels was compared.Results Of the 613 vessels detected prior to intervention therapy,440 vessels images were distinct by B-mode and 533 by VET(P<0.05).VET demonstrated 37 intrahepatic collaterals which were invisible by B-mode.Regarding DSA as the gold standard,the diagnostic accordance rate of affected vessels of B-mode and VET was 69.2% and 92.5%,respectively(P<0.05).In total 103 therapeutic vessels,B-mode sonography and VET revealed 81 and 95 distinct vessels,respectively(P<0.05).Conclusions VET can improve the definition of veins and venous stents.The combination of VET and color Doppler can improve the clinical value of ultrasound in the diagnosis of BCS and evaluation of the therapeutic efficacy of interventional therapy.

Image analysis of Budd-Chiari syndrome in Henan province of China / 中华放射学杂志

Objective To investigate the distribution of the obstructive lesion of hepatic vein and inferior cava vein in patients with Budd-Chiari syndrome in Henan Province of China.Methods A total of 231 cases with Budd-Chiari syndrome were diagnosed by combination of three imaging techniques which either included colour Dopple ultrasound,multislice CT and vasography or colour Dopple ultrasound,MR angiography and vasography.All the hepatic veins,accessory hepatic veins and inferior vena cavae were imaged and their obstructions were detected and analysed.Results Out of the 231 patients there were 5 cases(2.2%)with simple obstruction of inferior vena cava with normal hepatic branches.Thirty-three cases(14.3%)had simple hepatic vein obstruction with normal inferior vena cava.The remaining 193(83.5%)cases had vein obstruction both in hepatic vein and inferior vena cava.Conclusion The most frequent form of Budd-Chiari symdrome in Henan province of China is the complex obstruction of inferior vena cava and heptic vein,and simple obstruction of inferior vena cava occurs the least.

Retrospective Analysis of Budd-Chiari Syndrome in 135 Patients / 中华全科医师杂志

A total of 135 patients diagnosed with Budd-Chiari syndrome(BCS)during August 2004 and February 2007 were reviewed and analyzed retrospectively.The results showed that 105 subiects (77.8%)caused by malignances,while 29 participants(21.5%)had no definite pathogens.The most commonly seen symptom included jaundiee(69.6%),followed by ascites(48.2%),abdominal distention (40.7%)and lower extremity edema(37.0%).Hisher levels of STB,PT and γ-GT were determined.Color Dopple ultrasound,CT and MRI could be helpful in diagnosing BCS;angiography remained the most accurate measurement.Main treatment strategies were stent implantation and/or balloon dilation.BCS is a clinical syndrome with no typical symptoms.which makes its management very difficult.A complete understanding of the condition will help reduce misdiagnosis.Angiography and stent implantation are primary ways to diagnose or treat BCS.

Sonographic classification of blood-drainage in Budd-Chiari syndrome with hepatic vein obstruction / 中华超声影像学杂志

Objective To summarize sonographic classification of blood-drainage in Budd-Chiari syndrome (BCS)with hepatic vein(HV)obstruction in order to provide much information for clinical treatment.Methods A total of 185 patients with HV obstruction were underwent sonographic examination.The affection of HV,the courses,orifices,blood flow direction of drainage viens,the communicating branches among HVs and draining viens were observed detailedly.Classification of blood-drainage pattern were performed by sonogram in accordance with digital subtraction angiography(DSA)and computed tomography angiography(CTA).Results There were 490 HVs with affection in 185 patients.The blood drainage pattern were classified as the following six types:HV-accessory HV(AHV)type(119/185),HV-HV type(31/185),HV-AHV/HV type(14/185),inferior caval vein (IVC)-AHV-HV-right atrium type(9/185),HV-umbilical vein type(5/185)and HV-hepatic subeapsular vein type (7/185).Blood of obstructed HVs was drainaged to IVC,right atrium,umbilical veins or hepatic subcapsular veins through communicating branches which were in various amount and diameter.Haemodynamics was obtained from drainage veins correspondingly.Conclusions Sonographic classification of blood-drainage pattern in BCS with hepatic vein obstruction is of important clinical significance in the treatment.

Cytokines expression in the membranous tissue and organized thrombi in membranous obstruction of Budd Chiari syndrome / 中华普通外科杂志

Objective To explore the relationship between the membranous tissue(MT)and organized thrombus(OT)in membranous obstruction of the inferior vena cava(MOVC),we investigated the related cytokines expression in the membranous tissues in MOVC as well as venous organized thrombi. Methods Using immunohistochemical method the expression of TGFβR,PDGFR,ET-1,FⅧ-rAg, ferritin and α1-antitrypsin were observed in the membranous tissues in 11 cases with MOVC and organized thrombi in 8 cases with deep venous thrombosis(DVT). Results Expression rates of TGFβR,PDGFR,ET-1,FⅧ-rAg, and ferritin in membranous tissues in 11 cases with MOVC and organized thrombi in 8 cases with DVT were as follows: TGFβR:MT 72.3%,OT 50%(P＞0.05);PDGFR:MT 45.5%,OT 100%(P＜0.05＝;ET-1:MT 100%,OT 0(P＜0.05＝;FⅧ-rAg: MT 90.9%,OT 12.5%(P＜0.05＝;ferritin: MT 72.3%,OT 100%(P＞0.05).α1-antitrypasin was not detected in either membranous tissues of MOVC or organized thrombi of DVT. Conclusions ThrovIgh the investigation of the related cytokines expression, it is possible that membranous tissue formation in MOVC is related to the organized thrombus.

Interventional treatment of Budd-Chiari syndrome with inferior vena cava obstruction / 中国医师进修杂志

Objective To evaluate the significance and announcements of interventional treatment in Budd-Chiari syndrome with inferior vena cava (IVC) obstruction. Method Forty-five patients with Budd-Chiari syndrome with IVC obstruction were treated by oombined interventional methods such as percutaneous transluminal angioplasty (PTA) with balloon catheters and stents. Results After PTA with balloon catheters and stents, venography proved IVC were reopened. After the systemic treatment, clinical symptoms completely or partly disappeared in 24 hours. One patient with acute thrombosis was treated by PTA and stent, 2 patients failed because of IVC obstruction were too long, 2 patients recurred IVC obstruction after interventional treatment. No pulmonary embolism and hemorrhage occurred during the procedure oftreatment. Conclusion The interventional treatment in Budd-Chiari syndrome with IVC obstruction is safe and effective.

The analysis of misdiagnosis and mistreatment in Budd-Chiari syndrome with hepatic vein obstruction / 介入放射学杂志

Objective To investigate the clinical symptoms and imaging features of Budd-Chiari syndrome with hepatic vein obstruction (HVBCS) and the reasons of mistreatment. Methods Thirteen patients with HVBCS were misdiagnosed and mistreated as inferior vena cava (IVC) obstruction,including 8 patients treated with stent implantation in IVC once and 5 patients with balloon dilatation. After analysis of the clinical symptoms,signs and imaging features; hepatic vein obstruction was further confirmed by digital subtraction angiography (DSA)in all patients. Results All patients had variable degrees of portal hypertension and no apparent symptoms of IVC obstruction. CT or / and MRI showed obvious caudate lobe enlargement and DSA showed IVC narrowing with external compression. All patients were undertaken hepatic vein angiography including 4 with PTV and 9 with hepatic vein stent implantation. All patients' clinical symptoms and signs completely disappeared or markedly improved after the procedure. Conclusion The stenosis of IVC in HVBCS,caused by compression compensatory hypertrophy of hepatic caudate lobe can be cured by hepatic vein angioplasty which is the most correct and effective method.

A Case of Chronic Ulcerative Colitis Complicated by Budd-Chiari Syndrome and Colon Cancer / 대한소화기내시경학회지

Ulcerative colitis is a chronic inflammatory bowel disease and may have many intestinal and extraintestinal complications. Compared with general population, patients with longstanding ulcerative colitis have an increased risk of colorectal cancer. Patients with ulcerative colitis have an increased frequency of thromboembolism too. However, hepatic vein thrombosis is a very rare extraintestinal complication. This is the first reported case of a young patient with ulcerative colitis who developed synchronous colonic neoplasm and chronic Budd-Chiari syndrome manifested as esophageal variceal bleeding. We report a case of ulcerative colitis complicated by Budd-Chiari syndrome and colon cancer in a 28-year-old female.

Rapidly Progressing Budd-Chiari Syndrome Complicated by Hepatocellular Carcinoma

Budd-Chiari syndrome (BCS) is a disorder caused by occlusion of the hepatic vein or inferior vena cava. The clinical presentation include abdominal pain, hepatomegaly, ascites, leg edema, collateral venous dilatation of the body trunk, and portal hypertension. In addition, BCS can cause hepatocellular carcinoma (HCC) in some patients, although its pathogenesis is not yet completely understood. The average reported time lag from diagnosis of BCS to full-blown HCC ranges from several years to several decades. Hepatic carcinogenesis in patients with BCS perhaps reflects a prolonged and persistent liver injury in that it occurs in the primary inferior vena cava obstruction rather than the primary hepatic vein thrombosis. Among patients with BCS, membranous obstruction of the vena cava (MOVC) usually presents an insidious and chronic illness, whereas primary hepatic vein thrombosis presents an acute or subacute illness. We experienced a case of a patient with BCS, which progressed rapidly that HCC developed only nine months after the diagnosis of BCS. The factors causing this rapid progression are still unclear and remain to be investigated.

Radical surgery under genuine direct vision for the treatment of Budd-Chiari syndrome / 中国普通外科杂志

Objective To sum up our preliminary experience on radical surgery for the treatment of Budd Chiari syndrome under genuine direct vision. Method A total of 13 cases were enrolled in this study with age ranging from 17 to 48 years, and history from 3 months to 5 years. There was inferior vena cava (IVC) membranous obstruction in 3 cases, right hepatic venous membrane (HV) in 1 case, IVC membrane with distal thrombosis in 6 cases, long segment of thrombosis of IVC in 2 cases, IVC tumor thrombus extending to right atrium in 1 case of retroperitoneal tumor. Result All lesions were successfully resected. Extracorporeal circulation was used in one case, cell saver was used in 2 cases. No blood transfusion was needed except for one case receiving bank blood transfusion of 2000 ml, and the other one of 400 ml. One patient died of renal failure during perioperative period. Disappearance of the symptoms and sigh after operation was found in all the other cases. Conclusion This new radical surgery gives access to the lesions under clear direct vision facilitating the correction.