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1.
Article in Chinese | WPRIM | ID: wpr-910570

ABSTRACT

Objective:To investigate the changes of liver volume and liver function in patients with extensive hepatic vein occluded Budd-Chiari syndrome (BCS) treated with transjugular intrahepatic portosystem shunt (TIPS).Methods:The clinical data of 29 BCS patients from Affiliated Hospital of Xuzhou Medical University during March 2016 to June 2019 were retrospectively collected and analyzed. The BCS was caused by extensive hepatic vein occlusion and patients were treated with TIPS. Pre- and postoperative abdominal CT/MRI images were collected and analyzed, and hepatic volume was measured with 3D-reconstruction. The liver volume and liver function during before and post the surgery were also collected and analyzed with preoperative value.Results:Patients including 8 males and 21 females, aged (33.3±6.3) years, were enrolled in this study. TIPS was successfully performed in all patients, with a technical success rate 100%. No serious complications related to TIPS occurred. Patients were followed up for 12-33 months (median, 16 months). Compared with preoperative [(2 124.6±420.9) cm 3] , the hepatic volume of time points after operation [1 week: (1 926.3±372.3) cm 3; 3 months: (1 480.6±183.1) cm 3; 6 months: (1 461.9±153.0) cm 3; 12 months: (1 469.3±148.5) cm 3] were all significantly reduced, and the differences were statistically significant ( P<0.05). Compared with preoperative values, the hepatic function indexes at each time point after operation were significantly improved ( P<0.05). The complete remission rate of ascites was 96.4% (27/28), 100.0% (28/28) and 100.0% (28/28) at 3, 6 and 12 months, respectively. Conclusion:The extensive hepatic vein occlusive BCS patients were benefit from TIPS therapy. Six months after operation, the hepatic volume and the hepatic function returned to normal level.

2.
Article in Chinese | WPRIM | ID: wpr-911580

ABSTRACT

Objective:To evaluate the clinical significance of patent accessory hepatic vein (AHV) in treatment of Budd-Chiari syndrome (BCS) with hepatic vein occlusion.Methods:The clinical data of 21 BCS patients treated from Jan 2010 to June 2019 were retrospectively analyzed. All patients underwent AHV and IVC venography.Results:Angiography showed that the diameter of AHV was 6-13mm after the procedure, the angle between AHV and the distal end of IVC was (106.9±27.7)°, and 57.1% of the AHV were opened at the right side of IVC, 9.5% at the front, and 1.4% at the left side, respectively. The technical success rate was 100%. Thirteen patients with AHV disease underwent balloon dilatation angioplasty, and their preoperative AHV pressure dropped from (41.6±6.4) cm H 2O to (22.2±5.5) cm H 2O ( t=11.966, P<0.01). The preoperative AHV and intrahepatic IVC pressure decreased from (29.1±3.3) cm H 2O to (19.1±8.8) cm H 2O ( t=8.136, P<0.01) and from (25.5±6.1) cm H 2O to (13.8±4.0) cm H 2O ( t=5.536, P<0.01), respectively. All patients were of no symptom during follow up for 6 months and ultrasound showed that the patency rate of original lesion was 100%. Conclusion:A patent AHV helps alleviate the symptoms and blood congestion of BCS patients with hepatic vein obstruction.

3.
Article in Chinese | WPRIM | ID: wpr-910647

ABSTRACT

Objective:To study the use of three-dimensional (3D) visualization in diagnosis and interventional treatment of patients with Budd-Chiari syndrome (BCS) presenting with inferior vena cava obstruction and dangerous collateral branches.Methods:The data of 28 patients with BCS presenting with inferior vena cava obstruction and dangerous collateral branches treated at the Affiliated Hospital of Xuzhou Medical University from September 2018 to January 2021 were retrospectively analyzed. There were 11 males and 17 females with a mean age of 49.0 years. Enhanced MR images of these 28 patients were used to build 3D visualization of inferior vena cava. Anteroposterior and left lateral digital subtraction angiography (DSA) of inferior vena cava were performed. The inferior vena cava of these patients was recanalized under guidance of 3D visualization, and patency of inferior vena cava was determined on follow up.Results:3D visualization of inferior vena cava was successfully constructed in all the 28 patients, and 51 dangerous collateral branches were displayed. One, 2, 3 and 4 dangerous collateral branches were found in 13, 8, 6 and 1 patients, respectively. The average angle between the preoperative planning puncture route and the long axis of the proximal end of inferior vena cava was 22.2°. The orifices and courses of the dangerous collaterals and the shape of inferior vena cava could be clearly displayed on 3D visualization in all the 28 patients (100.0%), which were significantly better than the 6 patients (21.4%) using DSA obtained in the anteroposterior and left lateral positions (χ 2=20.045, P<0.05). The inferior vena cava was successfully recanalized in all the 28 patients without complications. On follow up of these patients for 2 to 30 months (mean 18.4 months), the inferior vena cava was patent in 25 patients. Three patients developed inferior vena cava re-obstruction at 3, 4 and 14 months after interventional treatment, respectively. Conclusion:3D visualization was useful in the diagnosis and interventional treatment of patients with BCS presenting with inferior vena cava obstruction and dangerous collateral branches.

4.
J. vasc. bras ; 20: e20200133, 2021. graf
Article in Portuguese | LILACS-Express | LILACS | ID: biblio-1287075

ABSTRACT

Resumo A síndrome de Budd-Chiari é uma doença venosa hepática rara, mais incidente em adultos jovens, podendo se apresentar na forma aguda, subaguda ou crônica, o que resulta em hipertensão portal. O tratamento tradicional consiste em técnicas de trombólise e de shunts portossistêmicos intra-hepáticos, como pontes para o transplante hepático. Recentemente, técnicas de angioplastia com balão ou stents têm sido relatadas para o tratamento dessa afecção. Neste artigo, é relatado e discutido um caso de síndrome de Budd-Chiari por obstrução membranosa da via de saída da veia supra-hepática com trombose da veia hepática média em uma paciente de 24 anos. O tratamento estabelecido foi a angioplastia transjugular com balão, que obteve resultados satisfatórios e boa evolução clínica.


Abstract The Budd-Chiari syndrome is a rare hepatic venous disease. It is more prevalent in young adults and may present in acute, subacute, or chronic forms, causing portal hypertension. Traditional treatment consists of thrombolysis techniques and transjugular intrahepatic portosystemic shunt, as a bridge to liver transplantation. Recently, use of balloon or stent angioplasty techniques has been reported for treatment of this condition. In this article, we report and discuss a case of BCS by membranous obstruction in the hepatic vein outflow tract, with middle hepatic vein thrombosis, in a 24-year-old patient. The treatment chosen and employed was transjugular balloon angioplasty, which achieved satisfactory results and good clinical evolution.

5.
Rev. colomb. gastroenterol ; 35(3): 280-286, jul.-set. 2020. tab, graf
Article in Spanish | LILACS | ID: biblio-1138784

ABSTRACT

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.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Patients , Therapeutics , Diagnosis , Budd-Chiari Syndrome , Hospitals , Methods , Ascites , Signs and Symptoms , Abdominal Pain , Hepatomegaly
6.
Article in Chinese | WPRIM | ID: wpr-870426

ABSTRACT

Objective To explore the clinical significance of large spontaneous portosystemic shunts (L-SPSS) in Budd-Chiari syndrome (B-CS).Methods Clinical data of 382 B-CS patients treated at the First Affiliated Hospital of Zhengzhou University from Apr 2012 to Dec 2018 were analyzed retrospectively and we included 32 patients with B-CS complicated L-SPSS (diameter > 8 mm).65 patients without L-SPSS were selected randomly to form the control group.The correlation between L-SPSS and liver function,upper gastrointestinal bleeding,ascites,hepatic encephalopathy,inferior vena cava thrombosis,portal vein diameter and splenic vein diameter were analyzed.Results TBil,Child-Pugh scores,incidence of hepatic encephalopathy,ascites and inferior vena cava thrombosis in the B-CS complicated L-SPSS group were higher than that in the B-CS without L-SPSS group.The percentage of Child-Pugh grade B and C patients in the B-CS complicated L-SPSS group were also higher than that in the B-CS without L-SPSS group.The level of ALB and hepatic volume per unit surface area were both significantly less in the experimental group.(P <0.05).There were no statistics differences in incidence of upper gastrointestinal bleeding and MELD scores between the two groups (P > 0.05).Conclusions For those B-CS patients complicated L-SPSS,hepatic encephalopathy and ascites are more frequent,and they usually have poorer liver function.

7.
Article in Chinese | WPRIM | ID: wpr-861990

ABSTRACT

Objective: To observe the safety and efficacy of large-lumen catheter aspiration combined with catheter-directed thrombolyses (CDT) and angioplasty for Budd-Chiari syndrome (BCS) with inferior vena cava (IVC) thromboses. Methods: A total of 74 patients with BCS combined with IVC fresh or mixed mainly fresh thromboses were selected, 32 cases were enrolled into thrombi aspiration group and 42 cases into simple thrombolytic group. Patients in thrombi aspiration group underwent CDT and endovascular angioplasty combined with large-lumen catheter aspiration, while those in simple thrombolytic group underwent only CDT and endovascular angioplasty. The thrombolytic effect, thrombolytic time, the amount of thrombolytic medicine and complications were compared between the two groups. Results: The technical success rate of the two groups were both 100%. The average thrombolytic time and the average dosage of urokinase in the thrombi aspiration group were all less than those in simple thrombolytic group (both P<0.05). In thrombi aspiration group, grade III, grade Ⅱ and grade thrombectomy were observed in 12, 19 and 1 cases, while in simple thrombolytic group were found in 17, 20 and 5 cases, respectively, and there was no significant difference between the two groups (P=0.33). Complications occurred in 2 patients (2/32, 6.25%) in thrombi aspiration group and 3 patients (3/42, 7.14%) in simple thrombolytic group, and no significant difference was found between 2 groups (P=1.00). Conclusion: Large-lumen catheter aspiration combined with catheter-directed thrombolysis can shorten the thrombolytic time, reduce the amount of thrombolytic drugs, therefore being safety for treating BCS with IVC thromboses.

8.
Chinese Journal of Surgery ; (12): E007-E007, 2020.
Article in Chinese | WPRIM | ID: wpr-811581

ABSTRACT

Prevention and control about the situation of 2019 coronavirus disease (COVID-19) are grim at present. In addition to supporting the frontline actively, medical workers in general surgery spare no efforts in making good diagnosis and treatment of specialized diseases by optimizing treatment process, providing medical advice online, mastering indications of delayed operation and emergency operation reasonably, etc. Budd-Chiari syndrome is a complex disorder, and severity of the disease varies, serious cases can be life threatening. While fighting the epidemic, medical workers should also ensure the medical needs of patients. However, instead of continuing the traditional treatment, a new management system should be developed. Based on the characteristics of Budd-Chiari syndrome patients in China and our experience, we divide the patients into ordinary and critical cases, and treatment strategies suitable for the epidemic period of COVID-19 are put forward for reference and discussion by physicians.

9.
Journal of Clinical Hepatology ; (12): 908-911, 2020.
Article in Chinese | WPRIM | ID: wpr-819196

ABSTRACT

Budd-Chiari syndrome (BCS) is a clinical syndrome of portal hypertension caused by the occlusion of the hepatic vein or the inferior vena cava, with or without inferior vena cava hypertension, and the formation of inferior vena cava diaphragm and/or hepatic venous obstruction are the main features in the Chinese population. At present, little is known about the etiology and pathogenesis of BCS, and most of the studies are clinical studies of diagnosis and treatment strategies. There is a lack of experimental exploration and research on the pathogenesis of BCS, and stable models are a necessary condition for the research on pathogenesis. This article reviews and analyzes the methods for establishing BCS models, the features of each model, and related research advances, in order to provide a reliable experimental basis for the research on the etiology and pathogenesis of BCS.

10.
Article | IMSEAR | ID: sea-207215

ABSTRACT

Presenting an unusual case of 27 years old female who presented at 18 weeks of pregnancy with second trimester bleeding per vaginum. Patient had history of recurrent abortions on examination was found to have hypertension and thrombocytopenia. Usg done revealed severe oligohydramnios. Patient was managed conservatively but aborted spontaneously at 22 weeks of gestation. Post-abortionl on day 2 patient developed abdominal distension and liver function tests were found to be deranged. USG and CT abdomen and pelvis was done, which revealed Budd chiari syndrome due to inferior vena cava (IVC) web. This extremely rare condition is characterized by obstruction of inferior vena cava by membrane or fibrous band. This condition is diagnosed by radiological techniques which in our patient revealed classical findings of caudate lobe hypertrophy, non-visualization of hepatic veins, moderate hepatomegaly and spleenomegaly and multiple collaterals. Esophagogastroduodenoscopy done which revealed large varieces for which endoscopic variceal ligation was done. IVCgram and IVC plasty was done by interventional radiology department 6 weeks after abortion. The aim of this case report is to highlight an extremely rare cause of Budd Chiari syndrome and IVC web in patient with recurrent abortion with spleenomegly leading to thrombocytopenia. It is important to rule out other differential diagnosis in these patients like APLA, ITP.

11.
Rev. colomb. gastroenterol ; 34(3): 297-302, jul.-set. 2019. tab, graf
Article in Spanish | LILACS | ID: biblio-1042818

ABSTRACT

Resumen El síndrome de Budd-Chiari es una entidad definida como la obstrucción al flujo sanguíneo en el tracto de salida hepático. Esta obstrucción se puede localizar desde las pequeñas venas hepáticas hasta la entrada de la vena cava inferior a la aurícula derecha. En la mayoría de los casos, se ocasiona por una trombosis endoluminal conocida como Budd-Chiari primario y secundario, cuando ocurre por una compresión extrínseca asociada a las lesiones que ocupan espacio como malignidad. Por tanto, los estados trombofílicos hereditarios se conocen como los principales factores de riesgo. El embarazo y el puerperio, al ser estados hipercoagulables, se pueden asociar al síndrome de Budd-Chiari, sin embargo, la prevalencia reportada en la literatura es muy variable dependiendo de la población estudiada. En Colombia no hay estudios de incidencia o prevalencia de la enfermedad; existen varios reportes de caso, pero no relacionados con el embarazo. Reportamos el caso de una paciente, que 12 semanas posparto presentó un síndrome de Budd-Chiari; además presentamos el manejo ofrecido, la evolución clínica y una revisión de la literatura de los casos asociados con el embarazo.


Abstract Budd-Chiari syndrome is defined as obstruction of hepatic blood outflow. This obstruction can be located anywhere from the small hepatic veins at the entrance of the inferior vena cava to the right atrium. Most cases are primary Budd-Chiari syndrome which is caused by endoluminal thrombosis. Secondary Budd-Chiari syndrome occurs as the result of extrinsic compression associated with space-occupying lesions such as malignant tumors. Hereditary thrombophilic states are the main risk factors, but since pregnancy and the puerperium are hypercoagulable states, they can be associated with Budd-Chiari syndrome. Nevertheless, the prevalence of this type of case in the literature varies according to the population studied. There have been no studies on the incidence or prevalence of this disease in Colombia. The small number of case reports here have not been related to pregnancy. We report the case of a patient who developed Budd-Chiari syndrome 12 weeks postpartum. Our report includes management and clinical evolution as well as a review of the literature of cases associated with pregnancy.


Subject(s)
Humans , Female , Adolescent , Postpartum Period , Budd-Chiari Syndrome , Thrombosis , Literature
12.
Article in Chinese | WPRIM | ID: wpr-778910

ABSTRACT

Post-hepatic portal hypertension refers to portal hypertension caused by the obstruction of liver blood flow between the extrahepatic liver vein and the right heart due to the obstruction of the extrahepatic liver vein. Common etiologies include Budd-Chiari syndrome, right heart failure, and constrictive pericarditis. With the development of medicine, great progress has been made in the diagnosis and treatment of these diseases in recent years.

13.
Article in Chinese | WPRIM | ID: wpr-802975

ABSTRACT

Objective@#To study the application of multiple row spiral CT (MSCT) in the diagnosis of inferior vena cava lesions in Budd Chiari syndrome (BCS) and its clinical value.@*Methods@#Eighty patients with BCS admitted to the Central Hospital of Shan County from May 2017 to May 2018 were divided into two groups by digital grouping method, with 40 cases in each group.The control group was diagnosed by ultrasound, and the study group was diagnosed by multi-slice CT.The pathological changes, tissue contrast of inferior vena cava and hepatic vein and the diagnostic sensitivity, specificity, positive predictive value and negative predictive value of inferior vena cava obstruction were compared between the two groups.@*Results@#The pathological diagnosis rate in the study group was significantly higher than that in the control group(χ2=4.562, 4.695, 4.125, 5.124, all P<0.05). The contrast of inferior vena cava and hepatic vein tissues in the study group was significantly higher than that in the control group (t=12.897, 13.214, all P<0.05). The sensitivity, specificity, positive predictive value and negative predictive value of the diagnosis of inferior vena cava occlusion in the study group were higher than those in the control group (the control group: 91.2%, 98.6%, 97.3%, 80.3%, the study group: 100.0%, 99.5%, 98.2%, 100.0%, χ2=11.897, 10.214, 11.235, 13.564, all P<0.05).@*Conclusion@#The application of MSCT in the diagnosis of BCS-inferior vena cava lesions, can display the lesions comprehensively and intuitive guidance in the treatment of inferior vena cava lesions, has high diagnostic value, it is worthy of popularization and application in clinic.

14.
Article in Chinese | WPRIM | ID: wpr-745334

ABSTRACT

Objective To explore the feasibility of interventional therapy in rat models of Budd-Chiari syndrome (BCS).Methods A total of 50 male clean SD rats were divided into model group and control group using random number table method,with 25 rats in each group.In the model group,BCS rat model was constructed by adopting partial ligation of the inferior vena cava (IVC),and the control group only had separation of surrounding tissues of IVC.Liver function was studied 12 weeks after postoperative raising,and digital subtraction angiography (DSA) and interventional therapy were performed,coupled with liver histopathology staining.Results Twenty BCS rats survived till the 12th week of raising,with the survival rate reaching 80.0%,and 22 survived in the control group.Compared with the control group,ALT [(43.1±5.5) U/L vs.(62.6±4.6) U/L] and AST [(84.7±26.5) U/L vs.(161.7±25.8) U/L)] in serum of rats in the model group increased,the differences were statistically significant (P<0.05).HE staining showed that obvious hepatocyte necrosis and inflammatory cell infiltration were observed in BCS rats,and liver fibrosis was spotted via Masson staining.DSA examination found IVC obstruction in the model group,among which 14 (70.0%,14/20) received interventional therapy after successful probing of IVC obstructed segment,and 7 had balloon dilatation with a diameter of 3.5 mm,with 6 (85.7%,6/7) successfully dilatatedand the other 1 (14.3%,1/7) failed;the remaining 7 had balloon dilatation with a diameter of 4.5 mm,with 2 (28.6%,2/7) successfully dilatated,and the other 5 (71.4%,5/7) died of IVC rupture.Conclusion The BCS rat models by partial ligation of IVC can well simulate the pathophysiological changes and angiopathy characteristics of IVC obstructive BCS patients,which provide a platform for the basic research of interventional therapy of BCS.

15.
Article in Chinese | WPRIM | ID: wpr-743988

ABSTRACT

Objective To investigate the application value of inferior vena cava venography in correlation between the subtypes of inferior vena cava obstruction and calcifications at the obstruction in Budd-Chiari syndrome (BCS).Methods The retrospective cross-sectional study was conducted.The clinical data of 41 patients with BCS who were admitted to the Affiliated Hospital of Xuzhou Medical University between January 2009 and December 2016 were collected.There were 29 males and 12 females,aged (53±10)years,with a range of 34-70 years.Forty-one BCS patients underwent computed tomography (CT),inferior vena cava CT venography and digital subtraction angiography (DSA) within 2 weeks.Balloon dilatation and (or) endovascular stent implantation of inferior vena cava were performed according to calcification morphology and location of the inferior vena cava obstruction detected by DSA.Observation indicators:(1) calcifications of inferior vena cava obstruction;(2) intraoperative situations of interventional therapy;(3) correlation between the subtypes of inferior vena cava obstruction and calcifications at the obstruction;(4)follow-up and survival situations.Follow-up using outpatient examination of inferior vena cava venography was performed at 3,6,12,24,36,48 months postoperatively to detect postoperative clinical manifestations,complications and survival situations up to December 2018.Measurement data with normal distribution were represented as Mean±SD.Count data were represented as absolute number and comparison between groups was analyzed using the chi-square test.The likelihood ratio test was used to analyze the correlation between the subtypes of inferior vena cava obstruction and calcifications at the obstruction.The degree of correlation was detected by Cramer's V contingency coefficient.Results (1)Calcifications of inferior vena cava obstruction:of 41 patients,17 had no calcification at the inferior vena cava obstruction and 24 had calcifications at the obstruction.Calcification location in 24 patients:there were 17,4 and 3 patients with proximal,distal,both proximal and distal calcifications at the inferior vena cava obstruction,respectively.Calcification morphology:punctate and irregular calcifications were detected in 20 and 4 patients,respectively.Calcification distribution:20,3 and 1 patients had scattered,cluster and diffuse distribution,respectively.(2) Intraoperative situations of interventional therapy:of 41 patients,21 underwent balloon dilatation and 20 underwent balloon dilatation combined with endovascular stent implantation.Two patients were detected hematoma at the puncture site of right femoral vein and treated using pressure dressing.One patient encountered rupture of balloon due to diffuse calcifications at the inferior vena cava obstruction and was improved after continual replace of balloon for 3 times.One patient had pulmonary embolism caused by detachment of the thrombosis at the distal obstruction during the balloon dilatation and was given anticoagulation therapy combined with thrombolytic therapy using large-dose of urokinase.The other 37 patients underwent successful interventional therapy without exceptional circumstances.(3) Correlation between the subtypes of inferior vena cava obstruction and calcifications at the obstruction:of 24 patients with calcifications at the inferior vena cava obstruction,13 had membrane obstruction,7 had segmental obstruction and 4 had fenestrated membrane obstruction.Of 17 patients without calcifications at the inferior vena cava obstruction,3 had membrane obstruction,13 had segmental obstruction and 1 had fenestrated membrane obstruction.The likelihood ratio test showed that the subtypes of inferior vena cava obstruction were associated with calcifications at the obstruction (x2=9.293,P<0.05),with the correlation coefficient V as 0.466.Further analysis showed a correlation between membrane obstruction of inferior vena cava and calcifications at the inferior vena cava obstruction (x2=8.121,P<0.05),no correlation between segmental obstruction and calcifications at the inferior vena cava obstruction,also no correlation between fenestrated membrane obstruction and calcifications at the inferior vena cava obstruction (x2=3.395,0.004,P>0.05).(4) Follow-up and survival situations:41 patients were followed up for 24.0-48.0 months,with a median time of 37.1 months.Postoperative ultrasound showed smooth backflow in the inferior vena cava,different degree of improvements in the lower limb swelling and varicosity in 38 patients.Embolisms in the inferior vena cava obstruction remained existent in 3 patients,1 of whom showed significant decreasing of embolisms.There were 2 patients found restenosis and undergoing endovascular stent implantation.All the 41 patients survived.Conclusions The subtypes of inferior vena cava obstruction are associated with calcifications at the obstruction in BCS.Inferior vena cava venography evaluating calcifications at the inferior vena cava obstruction in BCS can be helpful for diagnosing the subtypes of inferior vena cava obstruction and guiding its interventional therapy.

16.
Article in Chinese | WPRIM | ID: wpr-743981

ABSTRACT

Objective To investigate the clinical efficacy of vena cava-atrium anastomosis liver transplantation (VCAALT) for Budd-Chiari syndrome (BCS).Methods The retrospective descriptive study was conducted.The clinicopathological data of 18 BCS patients who underwent VCAALT in the Zhongnan Hospital of Wuhan University (6 cases),the Third Xiangya Hospital of Central South University (8 cases) and Tongji Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology (4 cases) from May 1996 to December 2012 were collected.All the 18 patients were males,aged from 29 to 61 years,with an average age of 42 years.According to characteristics and invasion extent of hepatic vein and vena cava after preoperative examinations,patients were performed different surgical procedures of VCAALT,including bridge piggyback liver transplantation (BPBLT),hanging atrium liver transplantation (HALT) and cava vena resection bridge liver transplantation (CVRBLT).Observation indicators:(1) surgical and postoperative situations;(2) typical case analysis;(3) follow-up situations.Follow-up using outpatient examination and telephone interview was performed to detect patients' survival up to December 2018.Measurement data with normal distribution were represented as Mean±SD and measurement data with skewed distribution were described as M (range).Results (1) Surgical and postoperative situations:of 18 patients,11 underwent BPBLT,3 underwent HALT,4 underwent CVRBLT.The operation time and volume of intraoperative blood loss were (6.0± 1.3)hours and (1 264±435)mL.One patient died of bilateral pulmonary diffuse inflammation and sepsis due to severe infection.The duration of postoperative hospital stay was (18±5) days.(2) Typical case analysis:one 47-year-old male BCS patient was detected retrohepatic vena cava plaques and thrombus and hepatic venous thrombus by exploratory laparotomy,and underwent BPBLT.A 43-year-old male BCS patient was detected hepatic and retrohepatic vena cava plaques,thrombus,concomitant cavernous transformation,and underwent HALT.A 32-year-old male BCS patient was detected plaques and thrombus with red thrombus in the hepatic vein,from right renal vein to right atrium,and underwent CVRBLT.All the 3 patients underwent VCAALT successfully with a satisfactory recovery.(3) Followup situations:18 patients were followed up for 3.0-60.0 months,with a median time of 51.7 months.During the follow-up,3 patients died of acute rejection,biliary complications and chronic graft dysfunction at 1,3,5 years postoperatively.The 1-,3-,5-year survival rates were 16/18,15/18,14/18,respectively.Conclusion Different surgical procedures of VCAALT for BCS are selected according to different situations of patients,which are safe and feasible with a satisfactory efficacy and beneficial to long-term survival of patients.

17.
Article in Chinese | WPRIM | ID: wpr-743282

ABSTRACT

Objective To explore the clinical manifestation, diagnosis and treatment of Budd-Chiari syndrome in neonates. Method The clinical data of Budd-Chiari syndrome in a neonate were retrospectively reviewed and relevant literature was reviewed. Results The 21-day-old girl was born through vaginal delivery with gestational age of 39 weeks and birth weight of 3150 g. Her clinical manifestations included abdominal distention, hepatosplenomegaly, ascites, repeated hypoproteinemia and low platelet count, similar to sepsis. Imaging examination indicated hepatic segment stenosis of the inferior vena cava (the stenosis segment was about 24 mm in length and 1.59 mm in diameter at the narrowest place) . The girl was diagnosed with BuddChiari syndrome and died after abandoning treatment. Conclusion Neonatal Budd-Chiari syndrome is rare, easily misdiagnosed and has a high mortality.

18.
Article in Chinese | WPRIM | ID: wpr-743177

ABSTRACT

Objective To investigate the expression and significance of inducible nitric oxide synthase (iNOS), platelet-derived growth factor (PDGF)-B and lipopolysaccharide (LPS) in rat models with Budd-Chiari syndrome (BCS) . Methods BCS model was established by partial ligation of inferior vena cava in the posterior segment of the liver. The experimental rats were divided into control group (n=20), model group (n=20) and sham group (n=20) . Liver tissues were collected for immunohistochemistry, HE and Masson staining, and the expression levels of iNOS, PDGF-B and LPS were determined. Results The LPS value in model group was higher than that in both control group and sham group (P=0.001) . The mRNA and protein expressions of iNOS and PDGF-B in model group were higher than those in both control group and sham group (P=0.001) . Statistically significant differences in mRNA and protein expressions of iNOS and PDGF-B existed between each other among the subgroups (P=0.001) . In model group iNOS was positively correlated with PDGF-B and LPS; liver fibrosis was positively correlated with LPS and negatively correlated with PDGFB. Conclusion The damage and repair of BCS is a complicated process. The iNOS, PDGF-B and LPS may play different roles in different stages of BCS. How to regulate their balance in liver fibrosis may be a direction that deserves further study.

19.
Article in English | WPRIM | ID: wpr-741455

ABSTRACT

OBJECTIVE: To assess segmental liver stiffness (LS) with MRI before and after endovascular intervention in patients with Budd-Chiari syndrome (BCS). MATERIALS AND METHODS: Twenty-three patients (13 males and 10 females; mean age, 42.6 ± 12.6 years; age range, 31–56 years) with BCS as a primary liver disease were recruited for this study. Two consecutive magnetic resonance elastography (MRE) examinations were performed before the endovascular treatment. Fifteen patients who underwent endovascular intervention treatment also had follow-up MRE scans within three days after the procedure. LS was measured in three liver segments: the right posterior, right anterior, and left medial segments. Inter-reader and inter-exam repeatability were analyzed with intraclass correlation coefficients (ICCs) and Bland-Altman analysis. Segmental LS and clinical characteristics before and after the intervention were also compared. RESULTS: Within three days of the endovascular intervention, all three segmental LS values decreased: LS of the right posterior segment = 7.23 ± 0.88 kPa (before) vs. 4.94 ± 0.84 kPa (after), LS of the right anterior segment = 7.30 ± 1.06 kPa (before) vs. 4.77 ± 0.85 kPa (after), and LS of the left medial segment = 7.22 ± 0.87 kPa (before) vs. 4.87 ± 0.72 kPa (after) (all p = 0.001). There was a significant correlation between LS changes and venous pressure gradient changes before and after treatments (r = 0.651, p = 0.009). The clinical manifestations of all 15 patients significantly improved after therapy. The MRE repeatability was excellent, with insignificant variations (inter-reader, ICC = 0.839–0.943: inter-examination, ICC = 0.765–0.869). Bland-Altman analysis confirmed excellent agreement (limits of agreement, 13.4–19.4%). CONCLUSION: Segmental LS measured by MRE is a promising repeatable quantitative biomarker for monitoring the treatment response to minimally invasive endovascular intervention in patients with BCS.


Subject(s)
Budd-Chiari Syndrome , Elasticity Imaging Techniques , Female , Follow-Up Studies , Humans , Liver Diseases , Liver , Magnetic Resonance Imaging , Male , Venous Pressure
20.
Article in Chinese | WPRIM | ID: wpr-755112

ABSTRACT

Objective To analyze the application of three-dimensional visualization in interventional treatment of Budd-Chiari syndrome (BCS) with the hepatic vein occlusion type.Methods A retrospective study was conducted on 15 patients with BCS of the hepatic vein occlusion type who were admitted to the Affiliated Hospital of Xuzhou Medical University from January 2017 to June 2018.The study included 8 males and 7 females with a mean age of (36.7±14.4) years.All patients were examined with MRI enhanced scanning and the original MRI data of the DICOM format were extracted.The digital data were then extracted and reconstructed by the Mimics software to obtain a three-dimensional visualization model.The hepatic vein was recanalized under the guidance of the three-dimensional visualization model.Results The three-dimensional visualization model was successfully constructed.Of all the 15 three-dimensional visualization models,right hepatic vein ostial stenosis occurred in 4 patients and occlusion in 11 patients.The middle hepatic vein and the left hepatic vein formed a common trunk in 15 patients,and ostial stenosis and occlusion of the common trunk occurred in 3 and 12 patients,respectively.Communicating branches between the right and middle hepatic veins occurred in 6 patients.Accessory hepatic veins occurred in 3 of 15 patients,and the accessory hepatic vein ostium was stenosed in 3 patients.Communicating branches between the accessory hepatic vein and the right hepatic vein occurred in 2 patients,and communicating branches between the accessory hepatic vein and the middle hepatic vein occurred in 1 patient.All patients were treated successfully with interventional treatment without any complications.One,2,and 3 hepatic veins were recanalized in 7,5 and 3 patients,respectively.Conclusion The three-dimensional visualization was valuable in displaying the location,pattern and collateral vessels of the hepatic veins in BCS patients with the hepatic vein occlusion type.It was helpful for accurate preoperative assessment and to make individualized interventional treatment plans.

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