RESUMO
PURPOSE: To describe the technical aspects, feasibility, and outcomes in children with Budd-Chiari syndrome (BCS) undergoing transjugular intrahepatic portosystemic shunt (TIPS) creation during infancy. MATERIALS AND METHODS: A retrospective review of infants with BCS undergoing TIPS creation between January 2012 and December 2018 was performed. Eight infants (5 males) underwent TIPS creation (7 for refractory ascites and 1 for refractory variceal bleeding) during the study period. The median age at TIPS creation was 10.5 months (range, 8-16 months). The median elapsed time between presentation and TIPS creation was 6.5 months (range, 0-13 months). The median weight and median pediatric end-stage liver disease score of the infants at the time of TIPS creation were 6.7 kg (range, 5.4-10 kg) and 13 kg (range, 8-18 kg), respectively. RESULTS: TIPS creation was successful in all patients. There were no immediate postprocedural complications. An 18-gauge hollow needle was manually curved, through which a 21-gauge Chiba needle was inserted to access the portal vein. All patients received 1 or 2 overlapping bare metal stents. One patient was lost to follow-up after the procedure. The median follow-up duration was 32 months (range, 14-51 months). Four of 7 infants needed reintervention. Two children died during the follow-up period. Two children successfully underwent living donor liver transplant, whereas the remaining 3 children were asymptomatic at the follow-up. CONCLUSIONS: TIPS creation was found to be safe and efficacious in improving portal hypertension and growth in these children, although, with a higher rate of reinterventions, possibly due to the use of small, bare metal stents.
Assuntos
Síndrome de Budd-Chiari , Doença Hepática Terminal , Varizes Esofágicas e Gástricas , Transplante de Fígado , Derivação Portossistêmica Transjugular Intra-Hepática , Síndrome de Budd-Chiari/diagnóstico por imagem , Síndrome de Budd-Chiari/cirurgia , Criança , Hemorragia Gastrointestinal , Humanos , Transplante de Fígado/efeitos adversos , Doadores Vivos , Masculino , Derivação Portossistêmica Transjugular Intra-Hepática/efeitos adversos , Derivação Portossistêmica Transjugular Intra-Hepática/métodos , Estudos Retrospectivos , Índice de Gravidade de Doença , Resultado do TratamentoRESUMO
Refractory ascites post-liver transplantation can be a challenging problem. Causes of refractory ascites include venous outflow anastomotic stenosis, vessel kinking by the regenerating liver, pre-existing graft disease, and positional outflow obstruction. We present a case report of a child presenting with high drain output and refractory ascites post-LDLT secondary to a positional kinking. Repeating the Doppler studies with patients both supine and sitting may be helpful.
Assuntos
Ascite/terapia , Transplante de Fígado , Complicações Pós-Operatórias/terapia , Ascite/diagnóstico por imagem , Ascite/etiologia , Criança , Constrição Patológica , Veias Hepáticas/cirurgia , Humanos , Doadores Vivos , Masculino , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/etiologia , StentsRESUMO
Preoperative extensive PV thrombosis can pose a technical challenge during liver transplantation surgery. Several strategies adopted to mitigate this problem include creation of a superior mesenteric vein-PV jump graft, use of a polytetrafluoroethylene graft, renoportal anastomosis, or cavoportal hemitransposition. Extensive and diffuse thrombosis of the splanchnic venous system may even necessitate multivisceral transplantation. We describe the case of a pediatric patient with Budd-Chiari syndrome and decompensated cirrhosis, who developed extensive thrombosis of the porto-spleno-mesenteric venous system prior to liver transplantation. We used a combination technique of thrombus aspiration by a novel trans-TIPPS approach followed by thrombolysis. Complete preoperative resolution of the extensive thrombosis was achieved. This allowed the creation of a brief window to enable planned LDLT. In prudently selected patients, performing an early mechanical and chemical thrombolysis of an extensive acute splanchnic venous thrombosis can thus help expedite a planned LDLT.
Assuntos
Síndrome de Budd-Chiari/cirurgia , Transplante de Fígado , Veia Porta , Derivação Portossistêmica Transjugular Intra-Hepática , Complicações Pós-Operatórias/terapia , Veia Esplênica , Trombectomia , Terapia Trombolítica , Trombose Venosa/terapia , Vísceras/irrigação sanguínea , Doença Aguda , Criança , Terapia Combinada , Humanos , Doadores Vivos , Masculino , Período Pré-Operatório , Resultado do TratamentoRESUMO
Coil embolization of the atypical enlarged pulmonary artery/arteriole with visible shunting may improve hypoxemia in patients with hepatopulmonary syndrome (HPS). When used selectively in cases with large shunts, either pre- or post-liver transplantation (LT), it can aid an early recovery and reduce morbidity. We present a case where a large intrapulmonary shunt was embolized preoperatively to improve hypoxemia associated with HPS and enhance post-operative recovery.
Assuntos
Embolização Terapêutica/métodos , Doença Hepática Terminal/cirurgia , Síndrome Hepatopulmonar/cirurgia , Transplante de Fígado/métodos , Arteríolas/cirurgia , Ascite , Pré-Escolar , Humanos , Hipertensão Portal , Hipóxia/metabolismo , Hipóxia/cirurgia , Cirrose Hepática/fisiopatologia , Masculino , Metilenotetra-Hidrofolato Redutase (NADPH2)/genética , Mutação , Período Pós-Operatório , Artéria Pulmonar/cirurgia , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
BACKGROUND AND AIM: Hemobilia is a rare but potentially life threatening problem, which can be difficult to diagnose and treat. In the last few decades there has been a change in the etiologic spectrum and management of this problem in the West. The aim of this study was to analyze the etiology, clinical features, management and outcome of major hemobilia in a tertiary referral centre from western India. METHODS: A retrospective analysis was undertaken on 22 patients (16 males, 6 females; mean age 39 years, range 13 to 74) who presented with major hemobilia over a 5-year period. RESULTS: The etiology was iatrogenic in 13 patients (percutaneous transhepatic biliary drainage 8, post laparoscopic cholecystectomy 3, endoscopic retrograde cholangiopancreatography 1, and liver biopsy 1), liver trauma in 6 and liver tumors in 3 patients. Twenty patients presented with gastrointestinal bleeding (melena 20 patients, hemetemesis with melena 8 patients), 5 with jaundice and 8 had fever. Abdominal angiography was performed in 20 patients. Angiography revealed pseudoaneurysm of the right hepatic artery or its branches in 14 patients, left hepatic artery in 2, an arterio-biliary fistula in 1, tumor blush in 1 and the source could not be located in 2 patients. Seventeen of the 22 patients were treated with radiological intervention, 3 required surgery (liver resection for tumors 2, laparotomy for venous collateral bleeding of portal cavernoma 1) and two were managed conservatively. Radiological intervention involved embolisation with coils and/or glue in 16, and chemoembolisation in 1 patient. Sixteen of 17 patients responded to embolisation. Overall there were two deaths. CONCLUSION: The spectrum of hemobilia seen in India is now similar to that in the developed world with iatrogenic causes being the commonest. Interventional radiology can treat a majority of patients reducing the need and morbidity associated with surgery.
Assuntos
Hemobilia/etiologia , Hemobilia/terapia , Adolescente , Adulto , Idoso , Angiografia , Colangiografia , Embolização Terapêutica , Endoscopia Gastrointestinal , Feminino , Hemobilia/epidemiologia , Humanos , Doença Iatrogênica , Índia/epidemiologia , Masculino , Pessoa de Meia-Idade , Radiografia Intervencionista , Estudos RetrospectivosRESUMO
OBJECTIVES: Budd-Chiari syndrome (BCS) in children is not uncommon. Published literature on therapy for this condition is scarce. We therefore attempted radiological interventions in these patients to determine their efficacy and safety. PATIENTS AND METHODS: Fourteen of 16 children with a median age of 22 months diagnosed as having BCS were subjected to an inferior vena cava/hepatic venogram with the aim to establish a normal antegrade flow in at least 1 hepatic vein (HV). RESULTS: A normal antegrade flow in at least 1 of the HVs could be established in 11 children. Three patients had angioplasty of the HV (vein size
Assuntos
Angioplastia , Síndrome de Budd-Chiari/terapia , Veias Hepáticas/diagnóstico por imagem , Fígado/diagnóstico por imagem , Derivação Portossistêmica Transjugular Intra-Hepática , Radiografia Intervencionista , Stents , Síndrome de Budd-Chiari/diagnóstico por imagem , Síndrome de Budd-Chiari/cirurgia , Criança , Pré-Escolar , Feminino , Veias Hepáticas/cirurgia , Humanos , Lactente , Fígado/irrigação sanguínea , Masculino , Flebografia/métodos , Complicações Pós-Operatórias , Radiografia Intervencionista/efeitos adversos , Resultado do Tratamento , Veia Cava Inferior/diagnóstico por imagemRESUMO
Chronic mesenteric ischaemia is not an uncommon disorder. It is associated with high morbidity and mortality. It presents with chronic abdominal pain and the diagnosis is often missed because of nonspecific clinical findings and limitations of diagnostic studies. Although surgery has been considered to be the mainstay of treatment, it is associated with significant morbidity. We report two cases of chronic mesenteric ischaemia managed effectively with endovascular therapy with no morbidity and good long term pain relief.
Assuntos
Dor Abdominal/etiologia , Angioplastia com Balão , Oclusão Vascular Mesentérica/terapia , Stents , Dor Abdominal/diagnóstico por imagem , Idoso , Angiografia , Doença Crônica , Feminino , Humanos , Oclusão Vascular Mesentérica/complicações , Oclusão Vascular Mesentérica/diagnóstico por imagem , Pessoa de Meia-Idade , Resultado do TratamentoRESUMO
A 33-year-old man of a Middle Eastern origin presented to us with abdominal pain and distension secondary to refractory ascites of 1-month duration. The patient had a history of taking oral retinoic acid 25 mg for 4 months for mycosis fungoides. Investigations revealed thrombosis of hepatic veins with extensive thrombosis of the porto-mesenteric axis. A combination of transjugular intrahepatic portosystemic shunt, balloon angioplasty and thrombolysis with recombinant tissue plasminogen activator was successfully used to treat his condition.
Assuntos
Angioplastia com Balão , Antineoplásicos/uso terapêutico , Micose Fungoide/tratamento farmacológico , Derivação Portossistêmica Transjugular Intra-Hepática , Circulação Esplâncnica/fisiologia , Ativador de Plasminogênio Tecidual/uso terapêutico , Tretinoína/uso terapêutico , Trombose Venosa/terapia , Dor Abdominal , Adulto , Ascite , Humanos , Masculino , Circulação Esplâncnica/efeitos dos fármacos , Stents , Resultado do Tratamento , Trombose Venosa/etiologiaRESUMO
Transjugular intrahepatic portosystemic shunt (TIPSS) has important role in the management of Budd-Chiari syndrome (BCS). Stent dysfunction rate up to 80% at 2 years limits the use of TIPSS. Reintervention is required if shunt develops occlusion or stenosis. Different techniques have been reported for the catheterization of TIPSS in cases of an occluded stent. In those failing a standard transvenous approach, the Colapinto needle technique, a combined transvenous transhepatic approach or transplenic approaches have been described. Here, we report our experience in a challenging TIPSS recanalization using a transsplenic approach.
Assuntos
Derivação Portossistêmica Transjugular Intra-Hepática/métodos , Punções/métodos , Baço/cirurgia , Adulto , Síndrome de Budd-Chiari/cirurgia , Humanos , Masculino , Reoperação , Stents/efeitos adversos , Fatores de TempoRESUMO
BACKGROUND: Hepatocellular carcinoma is an uncommon complication described in patients with Budd-Chiari syndrome. CASE CHARACTERISTICS: A 12-year-old boy with Budd-Chiari syndrome, who was earlier treated with Transjugular intrahepatic porto-systemic shunt (TIPS), presented with acute onset hemoperitoneum and hypotension. OUTCOME: It was diagnosed to be a case of ruptured hepatocellular carcinoma. MESSAGE: Successful TIPS may not prevent the development of hepatocellular carcinoma, and children with Budd Chiari syndrome should be monitored for the same.
Assuntos
Síndrome de Budd-Chiari/complicações , Carcinoma Hepatocelular , Neoplasias Hepáticas , Carcinoma Hepatocelular/complicações , Carcinoma Hepatocelular/diagnóstico , Criança , Humanos , Neoplasias Hepáticas/complicações , Neoplasias Hepáticas/diagnóstico , Masculino , Derivação Portossistêmica Transjugular Intra-HepáticaRESUMO
OBJECTIVES: Budd-Chiari syndrome (BCS) is an uncommon cause of chronic liver disease in children. The literature on the management of pediatric BCS is scarce. Our aim was to determine the long-term outcome of patients undergoing a radiological intervention for the treatment of BCS. METHODS: Thirty-two children diagnosed with BCS between 2004 and 2014 were included. Data on the course of disease, medical management, response, and complications related to radiological interventions and outcome were collected. MAIN RESULTS: Twenty-five patients who were on regular follow-up were analyzed. The median age of the patients at presentation was 9 months (4.5-214). Sixteen patients initially received anticoagulation alone. This was associated with a high failure rate of 66%. Twenty patients underwent a radiological intervention in the form of angioplasty (n=7), hepatic vein stenting (n=3) or transjugular intrahepatic portosystemic shunt (TIPS) (n=14). Success with angioplasty was achieved in 43% of cases. Hepatic vein stenting was successful in 66%, whereas TIPS was successful in 72% of cases. TIPS was feasible in all patients. The median follow-up duration was 44 months (5-132). Four patients developed hepatopulmonary syndrome after a median period of 3 years (1.5-5.25) and one patient developed hepatocellular carcinoma. CONCLUSION: BCS commonly presents during infancy. Anticoagulation alone and angioplasty of the hepatic veins are associated with a high failure rate. Hepatic vein stenting or TIPS is feasible and efficacious in improving liver function, portal hypertension, and growth. It is associated with good long-term outcome and delays the need for liver transplantation, but may not prevent complications such as hepatopulmonary syndrome and hepatocellular carcinoma.
Assuntos
Angioplastia , Síndrome de Budd-Chiari/terapia , Veias Hepáticas , Derivação Portossistêmica Transjugular Intra-Hepática , Radiografia Intervencionista , Adolescente , Fatores Etários , Angioplastia/efeitos adversos , Angioplastia/instrumentação , Anticoagulantes/uso terapêutico , Síndrome de Budd-Chiari/diagnóstico por imagem , Síndrome de Budd-Chiari/fisiopatologia , Criança , Pré-Escolar , Feminino , Veias Hepáticas/diagnóstico por imagem , Veias Hepáticas/fisiopatologia , Humanos , Lactente , Masculino , Pressão na Veia Porta , Derivação Portossistêmica Transjugular Intra-Hepática/efeitos adversos , Radiografia Intervencionista/efeitos adversos , Estudos Retrospectivos , Stents , Fatores de Tempo , Resultado do TratamentoRESUMO
BACKGROUND: Hepatic vein thrombosis (Budd-Chiari Syndrome) is a rare disorder resulting from an obstruction to the outflow of blood from the liver. Early decompression is needed to prevent liver dysfunction and death. Radiological intervention includes angioplasty of stenosis and webs and the placement of transjugular intrahepatic portosystemic shunts (TIPPS). Side-to-side portacaval shunt (SSPCS) remains the gold standard for achieving good long-term results. CASE PRESENTATION: A 37-year old lady underwent side-to-side portacaval shunt for Budd Chiari syndrome. She had early shunt blockage and this was successfully treated with the placement of a metallic stent across the shunt. CONCLUSION: At five years, she remains asymptomatic, with normal liver functions, no ascites, and normal flow through the stent on Colour Doppler examination.