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1.
Medicine (Baltimore) ; 98(45): e17877, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31702657

RESUMO

RATIONALE: Budd-Chiari syndrome (BCS), which causes congestive hepatopathy and aggravates cirrhosis, is typically treated by interventional angioplasty to ameliorate blood flow. X-ray venography is useful for the evaluation of inferior vena cava (IVC) stenosis and determination of treatment timing, but it is invasive and thus unsuitable for repeated examinations. The development of a simple method for the prediction of IVC stenosis would reduce the burden on patients with BCS. PATIENT CONCERNS: We report here our experience of 2 patients with BCS who underwent percutaneous transluminal angioplasty (PTA). The first patient was a 39-year-old male who underwent PTA to expand his stenotic IVC. The second patient was a 19-year-old male who underwent PTA 3 times due to restenosis of his IVC. DIAGNOSES: Both patients were diagnosed with BCS with severe obstruction of the IVC. INTERVENTIONS: We evaluated the hepatic vein (HV) waveform by Doppler ultrasonography and measured liver stiffness (LS) using transient elastography (TE) before and after PTA. OUTCOMES: In case 1, the phasic oscillation of the HV waveform recovered and the LS value decreased after PTA. Both improvements were maintained for ∼3 years, reflecting the long-term patency of the IVC. In case 2, the HV waveform and the LS value improved temporarily after PTA, but then deteriorated gradually. Monitoring of the HV waveform and LS value allowed retreatment prior to total occlusion of the IVC and abrogated the risk of intravascular needle puncture. LESSONS: Monitoring of the HV waveform and the LS value enables safe management of patients with BCS who may require PTA.


Assuntos
Síndrome de Budd-Chiari/diagnóstico por imagem , Constrição Patológica/diagnóstico por imagem , Técnicas de Imagem por Elasticidade/métodos , Veias Hepáticas/diagnóstico por imagem , Adulto , Angioplastia com Balão , Síndrome de Budd-Chiari/complicações , Síndrome de Budd-Chiari/cirurgia , Constrição Patológica/etiologia , Constrição Patológica/cirurgia , Veias Hepáticas/cirurgia , Humanos , Masculino , Ultrassonografia Doppler , Adulto Jovem
2.
Transplant Proc ; 51(9): 3140-3146, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31611116

RESUMO

INTRODUCTION: Budd-Chiari syndrome (BCS) associated with hypereosinophilic syndrome (HES) is very rare, and only a few reports have described its treatment. Furthermore, no report to date has described the performance of liver transplantation for the treatment of BCS associated with HES. We herein describe a 54-year-old man who underwent deceased-donor liver transplantation (DDLT) for treatment of BCS associated with HES. CASE: A 54-year-old man was found to have an increased eosinophil count during a medical check-up. After exclusion of hematopoietic neoplastic diseases and secondary eosinophilia, idiopathic hypereosinophilia was diagnosed. Oral prednisolone was administered to the patient, and his eosinophil count immediately decreased to a normal level. He had an uneventful course without complications for 11 months but then presented with bloating and malaise. Imaging studies including ultrasonography, enhanced computed tomography, and angiography revealed BCS associated with HES. Transjugular intrahepatic portosystemic shunt failed because of complete obstruction of the hepatic veins. Therefore, the patient was introduced to our hospital for liver transplantation. DDLT was performed with venovenous bypass 1 month after the patient was placed on the DDLT waiting list. The explanted hepatic veins were completely occluded and organized. The patient's eosinophil count was maintained at a normal level with prednisolone treatment after DDLT. CONCLUSIONS: Liver transplantation can be a treatment option for BCS associated with HES if neoplastic diseases and secondary eosinophilia have been excluded. Life-long oral steroid therapy is required to control HES even after liver transplantation.


Assuntos
Síndrome de Budd-Chiari/complicações , Síndrome de Budd-Chiari/cirurgia , Síndrome Hipereosinofílica/complicações , Transplante de Fígado/métodos , Humanos , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
3.
Rozhl Chir ; 98(6): 239-244, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31331179

RESUMO

Closures in the splanchnic venous system (SVS) represent a broad medical problem. Anatomically, individual or even multiple sections of SVS may be affected at the same time. Main sections of SVS include the venous liver outflow system, the portal vein, and the upper mesenteric vein and its basin. Thrombosis is clearly the predominant cause of closure. The closures can present as acute, subacute, chronic occult or chronic manifest. The main pathological and anatomical units are the Budd-Chiari syndrome (BCS), extrahepatic portal vein obstruction (EHPVO) and mesenteric vein thrombosis (MVT). Advanced laboratory, imaging and intervention methods substantially modify the approach to prevention, diagnosis and treatment; surgical approach also plays a role. The problem of SVS closures is interdisciplinary.


Assuntos
Síndrome de Budd-Chiari , Circulação Esplâncnica , Trombose , Trombose Venosa , Síndrome de Budd-Chiari/diagnóstico , Síndrome de Budd-Chiari/cirurgia , Humanos , Veias Mesentéricas , Veia Porta
4.
Transplant Proc ; 51(5): 1621-1624, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31155205

RESUMO

Sporotrichosis is an infection caused by the fungus of the Sporothrix schenckii complex and can be particularly harmful in immunocompromised patients. We report the case of a 26-year-old male patient with a previous history of pulmonary infection who underwent a liver transplant for Budd-Chiari syndrome. After the procedure, he presented with persistent fever and leukocytosis. On the 13th post-operative day, he was diagnosed with thrombosis of the hepatic artery and underwent a second liver transplant 15 days after the first procedure. After the retransplant, he presented daily episodes of fever, even after the use of several antimicrobial, antiviral, and antifungal agents. A number of negative cultures from different sites were obtained. After an acute episode of mental confusion, the growth of S schenckii was observed in cultures from cerebrospinal fluid and ascites obtained from a diagnostic paracentesis. Treatment with amphotericin B was started but the patient died on the fourth day of antifungal treatment, from a massive gastrointestinal hemorrhage. We found no previous report in the literature of spontaneous dissemination of S schenckii to the abdominal cavity causing peritonitis.


Assuntos
Hospedeiro Imunocomprometido , Transplante de Fígado , Esporotricose/imunologia , Adulto , Antifúngicos/uso terapêutico , Síndrome de Budd-Chiari/cirurgia , Evolução Fatal , Humanos , Transplante de Fígado/efeitos adversos , Masculino , Peritonite/etiologia
5.
Cardiovasc Intervent Radiol ; 42(9): 1311-1321, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31218407

RESUMO

OBJECTIVE: This retrospective study evaluated interventional treatments (recanalization, balloon dilation, and/or stent placement) for Budd-Chiari syndrome (BCS), caused by combined obstruction of the inferior vena cava (IVC) and hepatic veins (HVs). METHODS: Before and after interventional therapy, patients with BCS (n = 162; asymptomatic 105.2 ± 103.3 mo; follow-up 15 [6-24] mo) underwent imaging studies (color Doppler ultrasound, CT, or MRI), and inferior vena cavography and manometry. Venous lesions were characterized by occlusion features, and presence of thrombosis and peripheral collateral vessels. RESULTS: One, 2, and 3 main HV occlusions were observed, respectively, in 25 (15.4%), 61 (37.7%), and 76 (46.9%) patients. Eighty-three (51.2%), 98 (60.5%), and 104 (64.2%) patients had, respectively, large accessory HVs, venous collaterals formed between the HVs, or venous communicating branches between the HV and the peritoneal veins. The middle, left, and right HV was patent in 32 (19.8%), 35 (21.6%), and 44 (27.2%) patients. Recanalization of both hepatic and caval occlusions was successful in 96% (51/53) of those attempted; recanalization of IVC occlusion was successful in 97% (106/109). Among 157 patients successfully treated, 146 were cured and 11 showed clinical improvement. Clinical symptoms were relieved in 82.4% after the initial intervention, and 94.2% after the second intervention. CONCLUSION: Recanalization and balloon angioplasty was effective for the management of BCS with concurrent HV and IVC occlusions. The majority of patients required only IVC recanalization. The outcome of patients treated only by IVC intervention was similar to that of patients given combined HV and IVC intervention.


Assuntos
Síndrome de Budd-Chiari/cirurgia , Procedimentos Endovasculares/métodos , Veias Hepáticas/patologia , Veias Hepáticas/cirurgia , Veia Cava Inferior/patologia , Veia Cava Inferior/cirurgia , Adolescente , Adulto , Idoso , Angioplastia com Balão/métodos , China , Diagnóstico por Imagem/métodos , Feminino , Veias Hepáticas/diagnóstico por imagem , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Stents , Resultado do Tratamento , Veia Cava Inferior/diagnóstico por imagem , Adulto Jovem
6.
Transplant Proc ; 51(4): 1196-1198, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30981407

RESUMO

Pulmonary hypertension is one of the problems that can be encountered before liver transplantation. It is not expected in cases with no additional disease in postoperative period. Herein, we report on a 43-year-old woman who developed idiopathic pulmonary hypertension in the early postoperative period. Further investigation both pathologically and clinically is needed in patients undergoing living donor liver transplantation that may help to solve the problems such as pulmonary arterial hypertension before it occurs and manage complex hemodynamic changes successfully in the future.


Assuntos
Hipertensão Pulmonar Primária Familiar/etiologia , Transplante de Fígado/efeitos adversos , Complicações Pós-Operatórias/etiologia , Adulto , Síndrome de Budd-Chiari/cirurgia , Hipertensão Pulmonar Primária Familiar/fisiopatologia , Feminino , Humanos , Doadores Vivos , Complicações Pós-Operatórias/fisiopatologia
7.
Surg Laparosc Endosc Percutan Tech ; 29(4): 304-307, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30629033

RESUMO

PURPOSE: To assess the safety and clinical effectiveness of catheter aspiration with recanalization in patients with Budd-Chiari syndrome (BCS) and inferior vena cava (IVC) thrombosis. MATERIALS AND METHODS: Between January 2010 and December 2017, 33 patients with BCS and IVC thrombosis were treated by catheter aspiration with IVC recanalization in our center. A 12 F angled-tip guiding catheter was used for the aspiration of thrombi in the IVC. Recanalization was conducted following thrombi aspiration. Rates of technical success, clinical success and long-term patency were calculated. RESULTS: Catheter aspiration with IVC recanalization was technically successful in all patients. After aspiration, no thrombi were detectable by IVC venography in 21 patients, while residual mural thrombi were found in 12 patients. IVC balloon dilation was performed in 18 patients, while stent insertion was performed in 15 patients. We also achieved clinical success in all patients. Symptomatic and asymptomatic pulmonary embolism were found in 1 patient each, respectively. The cumulative 1-, 3-, and 5-year patency rates were 93.6%, 93.6%, and 83.2%, respectively. All patients remained alive during the follow-up. CONCLUSIONS: Catheter aspiration with recanalization is a safe and efficacious approach to treating patients with BCS and IVC thrombosis.


Assuntos
Síndrome de Budd-Chiari/epidemiologia , Síndrome de Budd-Chiari/cirurgia , Cateterismo/métodos , Trombose Venosa/epidemiologia , Trombose Venosa/cirurgia , Adulto , Síndrome de Budd-Chiari/diagnóstico por imagem , Estudos de Coortes , Comorbidade , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Flebografia/métodos , Prognóstico , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Trombectomia/métodos , Resultado do Tratamento , Grau de Desobstrução Vascular/fisiologia , Veia Cava Inferior/diagnóstico por imagem , Veia Cava Inferior/cirurgia , Trombose Venosa/diagnóstico por imagem
8.
Medicine (Baltimore) ; 98(4): e14224, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30681600

RESUMO

RATIONALE: Diabetic foot ulcer is a severe complication of diabetes, and most patients with diabetic foot ulcer require amputation. The incidence of Budd-Chiari syndrome is low; it is relatively rare. Diabetic foot ulcer combined with Budd-Chiari syndrome has not been reported so far. PATIENT CONCERNS: A 52-year-old man presented with uncontrolled high body temperature, continued expansion of the lower leg and foot ulcer with increasing malodor. DIAGNOSIS: The patient was diagnosed with Wagner grade 4 diabetic foot ulcer combined with Budd-Chiari syndrome. INTERVENTIONS: Critical treatment was performed immediately after his admission to the hospital. After the patient's condition was stable, we performed an interventional procedure to relieve the inferior vena cava obstruction. Debridement was then performed on the diabetic foot ulcer. Finally, skin grafting was performed due to condition of the wound. We completed moist healing and vacuum sealing drainage throughout the treatment process. OUTCOMES: The patient was hospitalized for 56 days, and all his right lower extremity ulcers eventually healed. LESSONS: In the treatment of diabetic foot ulcer combined with Budd-Chiari syndrome, it is necessary to develop a unified treatment plan that includes the timely treatment of Budd-Chiari syndrome upon admission, the strategic use of debridement, and the application of moist healing and vacuum sealing drainage.


Assuntos
Síndrome de Budd-Chiari/cirurgia , Desbridamento/métodos , Pé Diabético/cirurgia , Drenagem/métodos , Transplante de Pele/métodos , Síndrome de Budd-Chiari/complicações , Pé Diabético/complicações , Humanos , Masculino , Pessoa de Meia-Idade
9.
Eur Radiol ; 29(6): 3273-3280, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30506220

RESUMO

OBJECTIVES: To evaluate the changes in arterial oxygenation after portal decompression in Budd-Chiari syndrome (BCS) patients with hepatopulmonary syndrome (HPS). METHODS: From June 2014 to June 2015, all patients with BCS who underwent balloon angioplasty or transjugular intrahepatic portosystemic shunt (TIPS) creation at our institution were eligible for inclusion in this study. Arterial blood gas analysis was performed with the patient in an upright position and breathing room air at 2-3 days and 1 and 3 months after the procedure. RESULTS: Eleven patients with HPS and 14 patients without HPS were included in this study. The procedure was technically successful in 24 patients. One patient with HPS had technically unsuccessful TIPS creation. Reobstruction or TIPS dysfunction was not detected in any patient within 3 months after the procedure. For patients with HPS, the alveolar-arterial oxygen gradient (A-aO2) remained comparable to baseline 2-3 days after the procedure (-3.2 ± 11.9 mmHg; p = .412), significantly improved 1 month after the procedure (-11.7 ± 6.4 mmHg; p < .001), and then returned to baseline 3 months after the procedure (-1.3 ± 12.5 mmHg; p = .757). For patients without HPS, the A-aO2 remained comparable to baseline at all three time points after the procedure (+1.4 ± 8.3 mmHg, +3.5 ± 8.1 mmHg, and +1.3 ± 8.2 mmHg; p = .543, p = .137, and p = .565). CONCLUSIONS: Arterial oxygenation transiently improves after portal decompression in BCS patients with HPS. KEY POINTS: • Intrapulmonary vascular dilation and hepatopulmonary syndrome are common in patients with Budd-Chiari syndrome. • Arterial oxygenation transiently improves after portal decompression in Budd-Chiari syndrome patients with hepatopulmonary syndrome.


Assuntos
Angioplastia com Balão , Síndrome de Budd-Chiari/complicações , Síndrome de Budd-Chiari/cirurgia , Descompressão Cirúrgica , Síndrome Hepatopulmonar/complicações , Oxigênio/sangue , Derivação Portossistêmica Transjugular Intra-Hepática , Adulto , Síndrome de Budd-Chiari/sangue , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
10.
Ann Vasc Surg ; 57: 274.e1-274.e3, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30500636

RESUMO

A case with an extremely rare intravenous cystic lesion in the suprahepatic inferior vena cava was reported, which originated from the lymphatic system and had induced Budd-Chiari syndrome. To the best of our knowledge, this is the first report of a benign cystic lesion originating from the wall of a suprahepatic inferior vena cava which results in Budd-Chiari syndrome.


Assuntos
Síndrome de Budd-Chiari/etiologia , Linfocele/complicações , Veia Cava Inferior , Biópsia , Síndrome de Budd-Chiari/diagnóstico por imagem , Síndrome de Budd-Chiari/patologia , Síndrome de Budd-Chiari/cirurgia , Angiografia por Tomografia Computadorizada , Humanos , Linfocele/diagnóstico por imagem , Linfocele/patologia , Linfocele/cirurgia , Masculino , Pessoa de Meia-Idade , Flebografia/métodos , Resultado do Tratamento , Veia Cava Inferior/diagnóstico por imagem , Veia Cava Inferior/patologia , Veia Cava Inferior/cirurgia
11.
Ann Surg ; 269(4): e43-e45, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30080720

RESUMO

OBJECTIVE: We aimed to describe our living-donor liver transplantation (LDLT) surgical technique and its long-term patency for patients with Budd-Chiari syndrome (BCS) and retrohepatic inferior vena cava (IVC) obstruction that extends up to the atrium. BACKGROUND: From a technical perspective, LDLT for BCS with an IVC obstruction up to the right atrium is one of the most challenging surgical procedures. Consequently, the optimal surgical technique for patients with BCS has not yet been elucidated. METHODS: A durable LDLT technique without piggy-back hepatectomy was designed using a large-caliber synthetic interposition vascular graft between the right atrium and the infrahepatic IVC for reconstructing the hepatic outflow tract in patients with BCS. RESULTS: Between May 2006 and May 2017, 5 of 17 BCS patients who underwent LDLT required the described technique. All patients with a median follow-up of 10.5 years (range, 9.2-11.5 years) demonstrated the patent IVC grafts and no recurrence of BCS. CONCLUSIONS: Our refined technique does not require unnecessary and dangerous dissection of the diseased IVC, and eliminates the residual suprahepatic vena cava with the possibility of BCS recurrence by connecting the graft to the healthy atrium.


Assuntos
Prótese Vascular , Síndrome de Budd-Chiari/cirurgia , Transplante de Fígado/métodos , Veia Cava Inferior/cirurgia , Átrios do Coração/cirurgia , Humanos , Doadores Vivos , Fatores de Tempo , Resultado do Tratamento
12.
J Laparoendosc Adv Surg Tech A ; 29(3): 309-315, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30036126

RESUMO

PURPOSE: To report the long-term results of endoluminal sharp recanalization of occluded inferior vena cava (IVC) in patients with the Budd-Chiari syndrome (BCS). PATIENTS AND METHODS: Seventy-two consecutive patients with BCS and IVC occlusion underwent endoluminal recanalization of the IVC occlusion during a 6-year period. BCS with occlusive IVC was detected by reviewing patient history and color Doppler ultrasonography. Data on technical success, morbidity, mortality, complications, and color Doppler sonographic outcome were collected and analyzed. RESULTS: Seventy-seven recanalizations were performed in 72 patients. Technical success (patent IVC with good blood flow) was achieved in 70 (97.4%) patients. No perioperative death was observed. Six complications were found during recanalization, and 10 complications were found for total interventional procedures. The complication rates were 7.8% and 7.3% for recanalization and total procedure, respectively. Bleeding of access veins was the most common complication, and 2 patients showed slight rupture of IVC. The postoperative IVC diameter and blood speed of IVC increased significantly. Fifty-seven patients (79.2%) were clinical cured, and 2 patients (2.8%) showed no improvement. The 1-year, 3-year, and 5-year primary patency rates were 92.5%, 86.8%, and 77.3%, respectively. The second patency rates were 100.0%, 97.8%, and 91.8% for 1, 3, and 5 years, respectively. There were 2 deaths during follow-up. CONCLUSION: Endoluminal sharp recanalization of occluded IVC in patients with BCS is safe and effective with good long-term outcomes.


Assuntos
Síndrome de Budd-Chiari/cirurgia , Procedimentos Endovasculares/efeitos adversos , Procedimentos Endovasculares/métodos , Grau de Desobstrução Vascular , Veia Cava Inferior/cirurgia , Adulto , Idoso , Perda Sanguínea Cirúrgica , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Ultrassonografia Doppler em Cores , Adulto Jovem
13.
Indian Pediatr ; 55(10): 871-873, 2018 Oct 15.
Artigo em Inglês | MEDLINE | ID: mdl-30426953

RESUMO

OBJECTIVE: To study the diagnostic methods and treatment outcomes in children with Budd- Chiari syndrome. METHODS: Case records of 25 patients with Budd-Chiari syndrome were evaluated retrospectively. These patients were investigated with imaging techniques and underwent balloon angioplasty or surgical management. RESULTS: 21 patients underwent balloon angioplasty, of which 17 had good medium- to long-term results, while only one out of four patients who underwent a portocaval shunt survived. CONCLUSIONS: The balloon angioplasty has satisfactory outcome in the treatment of acute Budd-Chiari syndrome. In failed cases, the surgical therapy may be attempted, but the outcomes do not appear rewarding.


Assuntos
Angioplastia com Balão/métodos , Síndrome de Budd-Chiari/diagnóstico , Derivação Portocava Cirúrgica/métodos , Angiografia/métodos , Angioplastia com Balão/efeitos adversos , Síndrome de Budd-Chiari/cirurgia , Criança , Feminino , Humanos , Masculino , Derivação Portocava Cirúrgica/efeitos adversos , Estudos Retrospectivos , Resultado do Tratamento
14.
Transplant Proc ; 50(9): 2715-2717, 2018 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-30401383

RESUMO

BACKGROUND: Our aim in this study was to evaluate long-term efficiency of hepatic venous balloon angioplasty (BA) and stent placement (SP) for hepatic venous outflow obstruction (HVOO) in pediatric liver transplantation (LT). METHODS: From January 1999 to September 2016, 262 pediatric patients underwent LT at our hospital. Ten were diagnosed with HVOO, which included 8 living donor grafts and 2 split liver grafts. BA and SP were used in management of these 10 patients with HVOO. After intervention, Doppler ultrasound (DUS) was the major follow-up modality for comparing efficiency of BA and SP. RESULTS: The incidence of HVOO was 3.8% (10 of 262) in our pediatric LTs. Of the 10 HVOO cases, 5 had SP, 3 had BA once, 1 had BA twice, and 1 had BA twice along with SP. The patent hepatic vein was maintained after a mean follow-up of 7.4 (range, 0.04-17) years. Recurrent rate of HVOO after BA was 42%. Neither recurrent HVOO nor stent migration occurred after SP and throughout long-term follow-up. CONCLUSION: Hepatic venous SP was found to be more effective and safe than BA for treatment of HVOO in pediatric LT for long-term follow-up.


Assuntos
Angioplastia com Balão/métodos , Síndrome de Budd-Chiari/etiologia , Síndrome de Budd-Chiari/cirurgia , Procedimentos Cirúrgicos do Sistema Digestório/instrumentação , Transplante de Fígado/efeitos adversos , Stents , Adolescente , Angioplastia com Balão/mortalidade , Síndrome de Budd-Chiari/epidemiologia , Criança , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Feminino , Veias Hepáticas/cirurgia , Humanos , Incidência , Doadores Vivos , Masculino , Recidiva , Resultado do Tratamento
16.
Am J Case Rep ; 19: 767-772, 2018 Jun 30.
Artigo em Inglês | MEDLINE | ID: mdl-29959308

RESUMO

BACKGROUND Acute-on-chronic liver failure was first defined within the last 10 years as acute decompensation of chronic liver disease accompanied by multiorgan failure and poor outcome. Budd-Chiari syndrome is a rare and potentially deadly hepatic condition. To the best of our knowledge, this is the first case report of a live liver donor recipient with antiphospholipid antibody syndrome. CASE REPORT A 47-year-old woman from Sudan with acute-on-chronic liver failure and subacute Budd-Chiari syndrome triggered by active pneumonia was evacuated to Amman, Jordan. In Amman, she was transferred to our hospital for liver transplant evaluation. She presented with progressive liver failure, acute kidney failure, acute respiratory failure, and encephalopathy stage IV. Multidisciplinary therapy was initiated with IV anti-infective drugs and optimizing mechanical ventilation. Clinically, we stopped her progressive deterioration after 48 h and she improved slightly in our ICU. Accelerated work-up for donors and recipient was completed and her daughter was selected as a medically appropriate donor despite the fact that she was found to have heterozygote factor V Leiden mutation and antiphospholipid antibody syndrome, similar to her mother. A lifesaving live-donor liver transplantation was carried out after 72 h. Donor and recipient were discharged in good condition with normal liver function and both were discharged on anticoagulant Rivaroxaban 20 mg. CONCLUSIONS We present the first case of a patient with acute-on-chronic liver failure with subacute Budd-Chiari syndrome, which was triggered by bacterial pneumonia and was successfully treated by live-donor liver transplantation from a donor with antiphospholipid antibody syndrome.


Assuntos
Insuficiência Hepática Crônica Agudizada/cirurgia , Síndrome Antifosfolipídica/complicações , Síndrome de Budd-Chiari/cirurgia , Transplante de Fígado , Doadores Vivos , Insuficiência Hepática Crônica Agudizada/etiologia , Síndrome de Budd-Chiari/complicações , Feminino , Humanos , Pessoa de Meia-Idade
18.
Radiol Med ; 123(10): 799-807, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29856000

RESUMO

PURPOSE: To determine the clinical effectiveness and long-term outcomes of endovascular treatment for hepatic vein (HV)-type Budd-Chiari syndrome (BCS). MATERIALS AND METHODS: From June 2011 to August 2016, 68 consecutive patients with symptomatic HV-type BCS underwent endovascular treatment in our center. Data on the baseline characteristics, technical success, clinical success, and long-term outcomes were collected and analyzed retrospectively. RESULTS: The technical success rate of endovascular treatment was 100%. Fifty patients underwent HV recanalization, and 18 underwent accessory HV (AHV) recanalization. The clinical success rate was 95.6% (65/68). During a mean follow-up period of 29.4 ± 13.6 months, 19 patients experienced re-obstruction of either the HV (n = 18) or the AHV (n = 1). The cumulative 1-, 2-, and 5-year primary patency rates were 80.0, 72.8, and 67.9%, respectively. The cumulative 1-, 2-, and 5-year secondary patency rates were 93.8, 90.3, and 82.9%, respectively. Univariate and multivariate analyses revealed that the independent predictor of a prolonged primary patency duration was recanalization of the AHV. Five patients died 1-28 months (median, 15 months) after treatment. The cumulative 1-, 2-, and 5-year survival rates were 96.9, 93.4, and 91.2%, respectively. There was no significant difference in survival between the HV and AHV recanalization groups. CONCLUSION: Endovascular treatment is effective for patients with HV-type BCS. It can result in excellent long-term patency and survival rates. If it is applicable, AHV recanalization should be considered prior to treatment in order to achieve a longer patency.


Assuntos
Síndrome de Budd-Chiari/cirurgia , Procedimentos Endovasculares , Adulto , Feminino , Humanos , Masculino , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
19.
J Laparoendosc Adv Surg Tech A ; 28(11): 1346-1351, 2018 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-29851361

RESUMO

PURPOSE: To evaluate the safety and efficacy of percutaneous transhepatic route creation for hepatic vein (HV) angioplasty in Budd-Chiari syndrome (BCS). PATIENTS AND METHODS: Between April 2012 and August 2016, a total of 19 BCS patients underwent percutaneous transhepatic route creation for HV angioplasty after transvenous catheterization failure in this study. Color Doppler ultrasonography was required in all patients after admission and during follow-up. Data were retrospectively collected, and follow-up observations were performed. RESULTS: Technical and clinical success was achieved in 18 patients. Except for 1 failure of route creation, 19 routes were successfully created in 18 patients, with a technology success rate of 95.0%. Twenty-two balloon angioplasties were performed in 18 patients, with a mean balloon diameter of 13.6 ± 0.5 mm. Blood pressure and length of occlusive HV decreased significantly, and blood flow velocity and diameter of HV increased significantly after procedure. Abdominal distension/pain and ascites decreased significantly after procedure. One procedure-related death occurred, who died of gastrointestinal bleeding 6 days later. Except for the failure case, the rest of 18 patients were successfully followed up. The remaining 18 patients survived during follow-up, with a 5-year survival rate of 94.9%. One patient had a restenosis of HV after 47 days, and had undergone successful dilation. The 5-year primary and second patency rates were 94.1% and 100%, respectively. CONCLUSION: Percutaneous transhepatic route creation is safe and effective for HV angioplasty, and can be used to treat BCS patients after transvenous catheterization failure.


Assuntos
Angioplastia com Balão/métodos , Síndrome de Budd-Chiari/cirurgia , Veias Hepáticas/cirurgia , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taxa de Sobrevida , Ultrassonografia Doppler em Cores , Veia Cava Inferior/cirurgia
20.
Sci Rep ; 8(1): 7393, 2018 05 09.
Artigo em Inglês | MEDLINE | ID: mdl-29743653

RESUMO

This study aimed to present long-term results of a 12-year patient follow-up of recoverable stents for BCS complicated by inferior vena cava (IVC) thrombosis. Forty consecutive patients with BCS complicated by IVC thrombosis were treated with recoverable stents. The median duration of symptoms was 24 months. Recoverable stents was placed after predilation of the obstructed IVC, and then agitation thrombolysis or catheter-directed thrombolysis of IVC was performed. The recoverable stents was removed eventually after thrombus disappeared. Clinical patency was defined as absence or improvement of symptoms. Patients were subsequently followed-up by color Doppler ultrasound. Recoverable stents placement, balloon angioplasty and thrombolysis were technically successful in all patients. Stents were successfully removed in 92.1% of patients. A few serious related complications including one acute pulmonary thromboembolism, one stent migration, and one failure retrieval stents occurred. The median follow-up was 43.7 months. The long-term results were satisfactory except 2 patients who presented with a restenosis or re-obstruction and underwent additional therapy. There were 5 deaths owing to pulmonary embolism or underlying malignant disease 0.4-101.8 months after the procedures, including one procedure-related death. In conclusion, Recoverable stents treatment is safe and effective for BCS complicated by IVC thrombosis, with a good long-term outcome.


Assuntos
Síndrome de Budd-Chiari/cirurgia , Stents , Trombose Venosa/complicações , Adulto , Idoso , Síndrome de Budd-Chiari/complicações , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Embolia Pulmonar/etiologia , Resultado do Tratamento , Ultrassonografia Doppler em Cores , Veia Cava Inferior
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