Hepatic vena cava syndrome(HVCS) also known as membranous obstruction of inferior vena cava reported mainly from Asia and Africa is an important cause of hepatic venous outflow obstruction(HVOO) that is complicated by...Hepatic vena cava syndrome(HVCS) also known as membranous obstruction of inferior vena cava reported mainly from Asia and Africa is an important cause of hepatic venous outflow obstruction(HVOO) that is complicated by high incidence of liver cirrhosis(LC) and moderate to high incidence of hepatocellular carcinoma(HCC). In the past the disease was considered congenital and was included under Budd-Chiari syndrome(BCS). HVCS is a chronic disease common in developing countries, the onset of which is related to poor hygienic living condition. The initial lesion in the disease is a bacterial infection induced localized thrombophlebitis in hepatic portion of inferior vena cava at the site where hepatic veins open which on resolution transforms into stenosis, membrane or thick obstruction,and is followed by development of cavo-caval collateral anastomosis. The disease is characterized by long asymptomatic period and recurrent acute exacerbations(AE) precipitated by clinical or subclinical bacterial infection. AE is managed with prolonged oral antibiotic. Development of LC and HCC in HVCS is related to the severity and frequency of AEs and not to the duration of the disease or the type or severity of the caval obstruction. HVOO that develops during severe acute stage or AE is a pre-cirrhotic condition. Primary BCS on the other hand is a rare disease related to prothrombotic disorders reported mainly among Caucasians that clinically manifest as acute, subacute disease or as fulminant hepatic failure; and is managed with life-long anticoagulation, portosystemic shunt/endovascular angioplasty and stent or liver transplantation. As epidemiology, etiology and natural history of HVCS are different from classical BCS, it is here, recognized as a separate disease entity, a third primary cause of HVOO after sinusoidal obstruction syndrome and BCS. Understanding of the natural history has made early diagnosis of HVCS possible. This paper describes epidemiology, natural history and diagnosis of HVCS and discusses the pathogenesis of LC in the disease and mentions distinctive clinical features of HVCS related LC.展开更多
BACKGROUND Budd-Chiari syndrome(BCS)is a challenging indication for liver transplantation(LT)due to a combination of massive liver,increased bleeding,retroperitoneal fibrosis and frequently presents with stenosis of t...BACKGROUND Budd-Chiari syndrome(BCS)is a challenging indication for liver transplantation(LT)due to a combination of massive liver,increased bleeding,retroperitoneal fibrosis and frequently presents with stenosis of the inferior vena cava(IVC).Occasionally,it may be totally thrombosed,increasing the complexity of the procedure,as it should also be resected.The challenge is even greater when performing living-donor LT as the graft does not contain the retrohepatic IVC;thus,it may be necessary to reconstruct it.CASE SUMMARY A 35-year-old male patient with liver cirrhosis due to BCS and hepatocellular carcinoma beyond the Milan criteria underwent living-donor LT with IVC reconstruction.It was necessary to remove the IVC as its retrohepatic portion was completely thrombosed,up to almost the right atrium.A right-lobe graft was retrieved from his sister,with outflow reconstruction including the right hepatic vein and the branches of segment V and VIII to the middle hepatic vein.Owing to massive subcutaneous collaterals in the abdominal wall,venovenous bypass was implemented before incising the skin.The right atrium was reached via a transdiaphragramatic approach.Hepatectomy was performed en bloc with the retrohepatic vena cava.It was reconstructed with an infra-hepatic vena cava graft obtained from a deceased donor.The patient remains well on outpatient clinic follow-up 25 mo after the procedure,under an anticoagulation protocol with warfarin.CONCLUSION Living-donor LT in BCS with IVC thrombosis is feasible using a meticulous surgical technique and tailored strategies.展开更多
Polycystic liver disease (PLD) is characterized by the presence of multiple bile duct-derived epithelial cysts scattered in the liver parenchyma. PLD can manifest itself in patients with severe autosomal dominant poly...Polycystic liver disease (PLD) is characterized by the presence of multiple bile duct-derived epithelial cysts scattered in the liver parenchyma. PLD can manifest itself in patients with severe autosomal dominant polycystic kidney disease (ADPKD). Isolated autosomal dominant polycystic liver disease (ADPLD) is genetically distinct from PLD associated with ADPKD, although it may have similar pathogenesis and clinical manifestations.Recently, mutations in two causative genes for ADPLD,independently from ADPKD, have been identified. We report here a family (a mother and her daughter) with a severe form of ADPLD not associated with ADPKD produced by a novel missense protein kinase C substrate 80K-H (PRKCSH) mutation (R281W). This mutation causes a severe phenotype, since the two affected subjects manifested signs of portal hypertension. Doppler sonography, computed tomography (CT) and magnetic resonance (MR) imaging are effective in documenting the underlying lesions in a non-invasive way.展开更多
文摘Hepatic vena cava syndrome(HVCS) also known as membranous obstruction of inferior vena cava reported mainly from Asia and Africa is an important cause of hepatic venous outflow obstruction(HVOO) that is complicated by high incidence of liver cirrhosis(LC) and moderate to high incidence of hepatocellular carcinoma(HCC). In the past the disease was considered congenital and was included under Budd-Chiari syndrome(BCS). HVCS is a chronic disease common in developing countries, the onset of which is related to poor hygienic living condition. The initial lesion in the disease is a bacterial infection induced localized thrombophlebitis in hepatic portion of inferior vena cava at the site where hepatic veins open which on resolution transforms into stenosis, membrane or thick obstruction,and is followed by development of cavo-caval collateral anastomosis. The disease is characterized by long asymptomatic period and recurrent acute exacerbations(AE) precipitated by clinical or subclinical bacterial infection. AE is managed with prolonged oral antibiotic. Development of LC and HCC in HVCS is related to the severity and frequency of AEs and not to the duration of the disease or the type or severity of the caval obstruction. HVOO that develops during severe acute stage or AE is a pre-cirrhotic condition. Primary BCS on the other hand is a rare disease related to prothrombotic disorders reported mainly among Caucasians that clinically manifest as acute, subacute disease or as fulminant hepatic failure; and is managed with life-long anticoagulation, portosystemic shunt/endovascular angioplasty and stent or liver transplantation. As epidemiology, etiology and natural history of HVCS are different from classical BCS, it is here, recognized as a separate disease entity, a third primary cause of HVOO after sinusoidal obstruction syndrome and BCS. Understanding of the natural history has made early diagnosis of HVCS possible. This paper describes epidemiology, natural history and diagnosis of HVCS and discusses the pathogenesis of LC in the disease and mentions distinctive clinical features of HVCS related LC.
文摘BACKGROUND Budd-Chiari syndrome(BCS)is a challenging indication for liver transplantation(LT)due to a combination of massive liver,increased bleeding,retroperitoneal fibrosis and frequently presents with stenosis of the inferior vena cava(IVC).Occasionally,it may be totally thrombosed,increasing the complexity of the procedure,as it should also be resected.The challenge is even greater when performing living-donor LT as the graft does not contain the retrohepatic IVC;thus,it may be necessary to reconstruct it.CASE SUMMARY A 35-year-old male patient with liver cirrhosis due to BCS and hepatocellular carcinoma beyond the Milan criteria underwent living-donor LT with IVC reconstruction.It was necessary to remove the IVC as its retrohepatic portion was completely thrombosed,up to almost the right atrium.A right-lobe graft was retrieved from his sister,with outflow reconstruction including the right hepatic vein and the branches of segment V and VIII to the middle hepatic vein.Owing to massive subcutaneous collaterals in the abdominal wall,venovenous bypass was implemented before incising the skin.The right atrium was reached via a transdiaphragramatic approach.Hepatectomy was performed en bloc with the retrohepatic vena cava.It was reconstructed with an infra-hepatic vena cava graft obtained from a deceased donor.The patient remains well on outpatient clinic follow-up 25 mo after the procedure,under an anticoagulation protocol with warfarin.CONCLUSION Living-donor LT in BCS with IVC thrombosis is feasible using a meticulous surgical technique and tailored strategies.
基金Supported by a grant from the Instituto de Ciencias de la Salud,Consejeria de Sanidad de Castilla La Mancha (Grant EQ03016)Joost PH Drenth is a recipient of a NOW-VIDI grant
文摘Polycystic liver disease (PLD) is characterized by the presence of multiple bile duct-derived epithelial cysts scattered in the liver parenchyma. PLD can manifest itself in patients with severe autosomal dominant polycystic kidney disease (ADPKD). Isolated autosomal dominant polycystic liver disease (ADPLD) is genetically distinct from PLD associated with ADPKD, although it may have similar pathogenesis and clinical manifestations.Recently, mutations in two causative genes for ADPLD,independently from ADPKD, have been identified. We report here a family (a mother and her daughter) with a severe form of ADPLD not associated with ADPKD produced by a novel missense protein kinase C substrate 80K-H (PRKCSH) mutation (R281W). This mutation causes a severe phenotype, since the two affected subjects manifested signs of portal hypertension. Doppler sonography, computed tomography (CT) and magnetic resonance (MR) imaging are effective in documenting the underlying lesions in a non-invasive way.
