Essential thrombocythemia with abdominal thrombotic is frequently associated complications including portal cavernoma as a consequence of chronic portal vein thrombosis. Essential thrombocythemia in a latent form is d...Essential thrombocythemia with abdominal thrombotic is frequently associated complications including portal cavernoma as a consequence of chronic portal vein thrombosis. Essential thrombocythemia in a latent form is difficult to identify at onset due to the absence of an overt disease phenotype. In the presented case report, the diagnosis of essential thrombocythemia was initially missed because the typical disease phenotype was masked by bleeding and hypersplenism. The correct diagnosis was only reached when the patient experienced persistent thrombocytosis and pseudohyperkalemia after a shunt operation.展开更多
AIM:To evaluate the outcome of non-malignant and non-cirrhotic patients with portal cavernoma and to determine the predictors for survival.METHODS:Between July 2002 and June 2010,we retrospectively enrolled all consec...AIM:To evaluate the outcome of non-malignant and non-cirrhotic patients with portal cavernoma and to determine the predictors for survival.METHODS:Between July 2002 and June 2010,we retrospectively enrolled all consecutive patients admitted to our department with a diagnosis of portal cavernoma without abdominal malignancy or liver cirrhosis.The primary endpoint of this observational study was death and cause of death.Independent predictors of survival were identified using the Cox regression model.RESULTS:A total of 64 patients were enrolled in the study.During a mean follow-up period of 18±2.41mo,7 patients died.Causes of death were pulmonary embolism(n=1),acute leukemia(n=1),massive esophageal variceal hemorrhage(n=1),progressive liver failure(n=2),severe systemic infection secondary to multiple liver abscesses(n=1)and accident(n=1).The cumulative 6-,12-and 36-mo survival rates were 94.9%,86%and 86%,respectively.Multivariate Cox regression analysis demonstrated that the presence of ascites(HR=10.729,95%CI:1.209-95.183,P=0.033)and elevated white blood cell count(HR=1.072,95%CI:1.014-1.133,P=0.015)were independent prognostic factors of non-malignant and non-cirrhotic patients with portal cavernoma.The cumulative 6-,12-and 36-mo survival rates were significantly different between patients with and without ascites(90%,61.5%and 61.5%vs 97.3%,97.3%and 97.3%,respectively,P=0.0008).CONCLUSION:The presence of ascites and elevated white blood cell count were significantly associated with poor prognosis in non-malignant and non-cirrhotic patients with portal cavernoma.展开更多
Portal cavernoma cholangiopathy(PCC)is one of the most harrowing complications of extrahepatic portal venous obstruc-tion,as it determines the long-term hepatobiliary outcome.Although symptomatic PCC is rare in childr...Portal cavernoma cholangiopathy(PCC)is one of the most harrowing complications of extrahepatic portal venous obstruc-tion,as it determines the long-term hepatobiliary outcome.Although symptomatic PCC is rare in children,asymptomatic PCC is as common as that in adults.However,there are major gaps in the literature with regard to the best imaging strategy and management modality in children.Moreover,natural history of PCC and effect of portosystemic shunt surgeries in children are unclear.Neglected PCC would lead to difficult or recalcitrant biliary strictures that will require endoscopic ther-apy or bilioenteric anastomosis,both of which are challenging in the presence of extensive collaterals.There are limited studies on the effect of portosystemic shunt surgeries on the outcome of PCC in children compared to adults.In this review,we aimed to collate all existing literature on PCC in childhood and also compare with adult studies.We highlight the difficul-ties of this disease to provide a comprehensive platform to foster further research on PCC exclusively in children.展开更多
Biliary ductal changes are a common radiological finding in patients with portal hypertension, however only a small percentage of patients (5%-30%) develop symptomatic bile duct obstruction. The exact pathogenesis is ...Biliary ductal changes are a common radiological finding in patients with portal hypertension, however only a small percentage of patients (5%-30%) develop symptomatic bile duct obstruction. The exact pathogenesis is not clear, but an involvement of factors such as bile duct compression by venous collaterals, ischemia, and infection is accepted by most authors. Although endoscopic retrograde cholangiopancrea- tography was used to define and diagnose this condition, magnetic resonance cholangiopancreatography is currently the investigation of choice for diagnosing this condition. Treatment is indicated only for symptomatic cases. Portosystemic shunts are the treatment of choice for symptomatic portal biliopathy. In the majority of patients, the changes caused by biliopathy resolve after shunt surgery, however, 15%-20% patients require a subsequent bilio-enteric bypass or endoscopic management for persistent biliopathy. There is a role for endoscopic therapy in patients with bile duct stones, cholangitis or when portosystemic shunt surgery is not feasible.展开更多
Portal biliopathy(PB) is defined as the presence of biliary abnormalities in patients with non-cirrhotic/nonneoplastic extrahepatic portal vein obstruction(EHPVO) and portal cavernoma(PC). The pathogenesis of PB is du...Portal biliopathy(PB) is defined as the presence of biliary abnormalities in patients with non-cirrhotic/nonneoplastic extrahepatic portal vein obstruction(EHPVO) and portal cavernoma(PC). The pathogenesis of PB is due to ab extrinseco compression of bile ducts by PC and/or to ischemic damage secondary to an altered biliary vascularization in EHPVO and PC. Although asymptomatic biliary abnormalities can be frequently seen by magnetic resonance cholangiopancreatography in patients with PC(77%-100%), only a part of these(5%-38%) are symptomatic. Clinical presentation includes jaundice, cholangitis, cholecystitis, abdominal pain, and cholelithiasis. In this subset of patients is required a specific treatment. Different therapeutic approaches aimed to diminish portal hypertension and treat biliary strictures are available. In order to decompress PC, surgical porto-systemic shunt or transjugular intrahepatic porto-systemic shunt can be performed, and treatment on the biliary stenosis includes endoscopic(Endoscopic retrograde cholangiopancreatography with endoscopic sphincterotomy, balloon dilation, stone extraction, stent placement) and surgical(bilioenteric anastomosis, cholecystectomy) approaches. Definitive treatment of PB often requires multiple and combined interventions both on vascular and biliary system. Liver transplantation can be considered in patients with secondary biliary cirrhosis, recurrent cholangitis or unsuccessful control of portal hypertension.展开更多
Portal biliopathy refers to cholangiographic abnormalities which occur in patients with portal cavernoma. These changes occur as a result of pressure on bile ducts from bridging tortuous paracholedochal, epicholedocha...Portal biliopathy refers to cholangiographic abnormalities which occur in patients with portal cavernoma. These changes occur as a result of pressure on bile ducts from bridging tortuous paracholedochal, epicholedochal and cholecystic veins. Bile duct ischemia may occur due prolonged venous pressure effect or result from insufficient blood supply. In addition, encasement of ducts may occur due fibrotic cavernoma. Majority of patients are asymptomatic. Portal biliopathy is a progressive disease and patients who have long standing disease and more severe bile duct abnormalities present with recurrent episodes of biliary pain, cholangitis and cholestasis. Serum chemistry, ultrasound with color Doppler imaging, magnetic resonance imaging with magnetic resonance cholangiopancreatography and magnetic resonance portovenography are modalities of choice for evaluation of portal biliopathy. Endoscopic retrograde cholangiography being an invasive procedure is indicated for endotherapy only. Management of portal biliopathy is done in a stepwise manner. First, endotherapy is done for dilation of biliary strictures, placement of biliary stents to facilitate drainage and removal of bile duct calculi. Next portal venous pressure is reduced by formation of surgical porto-systemic shunt or transjugular intrahepatic portosystemic shunt. This causes significant resolution of biliary changes. Patients who persist with biliary symptoms and bile duct changes may benefit from surgical biliary drainage procedures(hepaticojejunostomy or choledechoduodenostomy).展开更多
Transjugular intrahepatic portosystemic shunt(TIPS) placement is a standard procedure for the treatment of portal hypertension complications. When this conventional approach is not feasible, alternative procedures for...Transjugular intrahepatic portosystemic shunt(TIPS) placement is a standard procedure for the treatment of portal hypertension complications. When this conventional approach is not feasible, alternative procedures for systemic diversion of portal blood have been proposed. A one-step interventional approach, combining minilaparotomy-assisted transmesenteric(MAT) antegrade portal recanalization and TIPS, is described in an adolescent with recurrent esophageal varice bleeding and portal cavernoma(PC). A 16-yearold girl was admitted to our Unit because of repeated bleeding episodes over a short period of time due to esophageal varices in the context of a PC. A portal vein recanalization through an ileocolic vein isolation with the MAT approach followed by TIPS during the same session was performed. In the case of failed portal recanalization, this approach, would also be useful for varice endovascular embolization. Postoperative recovery was uneventful. Treatment consisting of propanolol, enoxaparin and a proton pump inhibitor was prescribed after the procedure. One month post-op, contrast enhanced computed tomography confirmed the patency of the portal and intrahepatic stent grafts. No residual peritoneal fluid was detected nor opacification of the large varices. Endoscopy showed good improvement of the varices. Doppler ultrasound confirmed the accelerated flow in the portal stent and hepatopetal flow inside the intrahepatic portal branches. Three months post-op, TIPS maintained its hourglass shape despite a slight expansion. Portal hypertension and life threatening conditions related to PC would benefit from one-step portal recanalization. MAT-TIPS is feasible and safe for the treatment of PC even in children. This minimally invasive procedure avoids or delays surgical treatment or re-transplantation when necessary in pediatric patients.展开更多
Extrahepatic portal vein obstruction(EHPVO) is a primary vascular condition characterized by chronic long standing blockage and cavernous transformation of portal vein with or without additional involvement of intrahe...Extrahepatic portal vein obstruction(EHPVO) is a primary vascular condition characterized by chronic long standing blockage and cavernous transformation of portal vein with or without additional involvement of intrahepatic branches, splenic or superior mesenteric vein. Patients generally present in childhood with multiple episodes of variceal bleed and EHPVO is the predominant cause of paediatric portal hypertension(PHT) in developing countries. It is a pre-hepatic type of PHT in which liver functions and morphology are preserved till late. Characteristic imaging findings include multiple parabiliary venous collaterals which form to bypass the obstructed portal vein with resultant changes in biliary tree termed portal biliopathy or portal cavernoma cholangiopathy. Ultrasound with Doppler, computed tomography, magnetic resonance cholangiography and magnetic resonance portovenography are non-invasive techniques which can provide a comprehensive analysis of degree and extent of EHPVO, collaterals and bile duct abnormalities. These can also be used to assess in surgical planning as well screening for shunt patency in post-operative patients. The multitude of changes and complications seen in EHPVO can be addressed by various radiological interventional procedures. The myriad of symptoms arising secondary to vascular, biliary, visceral and neurocognitive changes in EHPVO can be managed by various radiological interventions like transjugular intra-hepatic portosystemic shunt, percutaneous transhepatic biliary drainage, partial splenic embolization, balloon occluded retrograde obliteration of portosystemic shunt(PSS) and revision of PSS.展开更多
Extensive vascular network and proximity to the gastrointestinal tract make the liver susceptible to abscess formation.While pyogenic liver abscesses account for the majority of liver abscesses in the Western world,am...Extensive vascular network and proximity to the gastrointestinal tract make the liver susceptible to abscess formation.While pyogenic liver abscesses account for the majority of liver abscesses in the Western world,amebic liver abscesses are more prevalent in tropical and developing nations.Most liver abscesses heal without complications.However,various vascular complications can occur in these patients,including compression of the inferior vena cava,thrombosis of the portal vein and/or hepatic veins,hepatic artery pseudoaneurysm,direct rupture into major vessels or the pericardium,and biliovascular fistula.These compli-cations can present significant clinical challenges due to the potential for hae-morrhage,ischemia,and systemic embolism,thereby increasing the risk of morbidity and mortality.Mechanical compression,flow stasis,inflammation,endothelial injury,and direct invasion are some of the proposed mechanisms that can cause vascular complications in the setting of a liver abscess.For the diag-nosis,thorough assessment,and therapeutic planning of vascular complications,more sophisticated imaging techniques such as multidetector computed tomo-graphy angiography or magnetic resonance angiography may be necessary.Although most vascular complications resolve with abscess treatment alone,additional interventions may be required based on the nature,severity,and course of the complications.This article aims to provide a systematic update on the spectrum of vascular complications of liver abscesses,offering insights into their pathogenesis,diagnosis,and management strategies.展开更多
目的总结Rex分流联合断流、脾动脉结扎术治疗小儿门静脉海绵样变的经验与疗效。方法回顾性分析2012年8月至2019年7月在安徽省儿童医院行Rex分流联合断流、脾动脉结扎术治疗的16例门静脉海绵样变、门静脉高压症患儿临床资料及随访结果。...目的总结Rex分流联合断流、脾动脉结扎术治疗小儿门静脉海绵样变的经验与疗效。方法回顾性分析2012年8月至2019年7月在安徽省儿童医院行Rex分流联合断流、脾动脉结扎术治疗的16例门静脉海绵样变、门静脉高压症患儿临床资料及随访结果。结果15例成功手术,分流前门静脉测压28.3~45.8 cm H 2O,平均(32.6±5.4)cm H 2O,分流后显著下降为17.2~27.8 cm H 2O,平均(23.4±3.7)cmH 2O,分流前后门静脉压力差异有统计学意义(P<0.01)。术后均恢复出院。1例术中吻合血管不通畅加行Warren术,术后脾肾静脉吻合口血栓形成,介入治疗效果不显著,自动出院后死亡。成功手术的15例患儿术后随访6个月至6年,平均随访31.5个月,3例术后有消化道出血复发,药物止血均较易控制,其中2例均复发1次,1例复发2次后未再出血,但出现脾大、脾功能亢进,术后5年行脾切除术。15例均生长发育良好。结论Rex分流联合断流、脾动脉结扎术治疗小儿门静脉海绵样变所致门静脉高压症效果确切。展开更多
目的探讨球囊扩张术在儿童门静脉海绵样变(cavernous transformation of the portal vein,CTPV)Rex手术后桥血管与肝内门静脉左支吻合口狭窄中的应用效果。方法收集2018年11月至2019年4月由广州市妇女儿童医疗中心收治的CTPV Rex手术后...目的探讨球囊扩张术在儿童门静脉海绵样变(cavernous transformation of the portal vein,CTPV)Rex手术后桥血管与肝内门静脉左支吻合口狭窄中的应用效果。方法收集2018年11月至2019年4月由广州市妇女儿童医疗中心收治的CTPV Rex手术后桥血管吻合口狭窄并行介入治疗的5例患儿作为研究对象,其中男童2例,女童3例,年龄1.9~11岁。术前超声或CT检查显示桥血管吻合口不同程度狭窄,狭窄口内径为1~3.5 mm。结果5例CTPV患儿共行8次经皮肝穿刺门静脉入路血管扩张介入术,其中3例患儿仅行1次介入术(2例成功,另1例未成功);1例行2次介入(第1次未成功,第2次成功实施血管狭窄吻合口球囊扩张术);1例患儿因吻合口极度狭窄,约1 mm,前2次均未成功将导丝通过血管吻合口狭窄部,第3次联合肝胆外科,成功实施球囊扩张术。球囊扩张术后随访6~9个月,超声复查显示血管吻合口内径较术前扩大。5例CTPV患儿8次介入术中均未出现并发症。结论经皮肝穿刺肝内门静脉入路,对门静脉海绵样变Rex手术后桥血管吻合口狭窄的患儿实施球囊扩张术创伤小,疗效显著,有助于完善儿童CTPV治疗措施。展开更多
目的探讨应用颈内静脉做移植血管的Rex手术治疗门静脉海绵样变的临床疗效。方法2015年5月至2018年5月东莞市儿童医院共收治12例门静脉海绵样变性患儿,年龄89~180个月,男童7例,女童5例。10例存在反复上消化道出血病史。12例均表现为脾功...目的探讨应用颈内静脉做移植血管的Rex手术治疗门静脉海绵样变的临床疗效。方法2015年5月至2018年5月东莞市儿童医院共收治12例门静脉海绵样变性患儿,年龄89~180个月,男童7例,女童5例。10例存在反复上消化道出血病史。12例均表现为脾功能亢进,术前红细胞、白细胞、血小板都呈现不同程度降低;肝功能各项指标均正常。术前影像学检查均提示:食管胃底静脉曲张,门静脉海绵样变;脾脏肿大。术后随访12~24个月。结果12例均成功实施手术,手术时间为380~480 min,出血量为30~150 mL,分流术前测肠系膜上静脉压力为24~37 cm H 2O,平均为(32.5±3.7)cm H 2O,分流术后降为17~34 cm H 2O,平均为(23.5±4.7)cm H 2O,分流术前后肠系膜上静脉压力差异有统计学意义(t=5.19,P=0.001)。术后复查血细胞计数较术前均有不同程度升高,手术前后白细胞分别为(2.1±0.6)×1012 g/L和(4.4±2.3)×1012/L,差异有统计学意义(t=3.29,P=0.003);手术前后血红蛋白分别为(65.2±19.5)g/L和(101.2±13.9)g/L,差异有统计学意义(t=5.70,P=0.001);手术前后血小板分别为(70.5±33.1)×109/L和(161.17±66.8)×109/L,差异有统计学意义(t=4.21,P=0.001)。术后12个月复查脾脏长度、厚度较术前均有缩小,差异有统计学意义(P=0.001)。随访期间患儿未再发生呕血,影像学检查显示分流血管通畅,肝功能各项指标正常。结论应用颈内静脉做移植血管的Rex分流术治疗门静脉海绵样变性效果良好。展开更多
文摘Essential thrombocythemia with abdominal thrombotic is frequently associated complications including portal cavernoma as a consequence of chronic portal vein thrombosis. Essential thrombocythemia in a latent form is difficult to identify at onset due to the absence of an overt disease phenotype. In the presented case report, the diagnosis of essential thrombocythemia was initially missed because the typical disease phenotype was masked by bleeding and hypersplenism. The correct diagnosis was only reached when the patient experienced persistent thrombocytosis and pseudohyperkalemia after a shunt operation.
文摘AIM:To evaluate the outcome of non-malignant and non-cirrhotic patients with portal cavernoma and to determine the predictors for survival.METHODS:Between July 2002 and June 2010,we retrospectively enrolled all consecutive patients admitted to our department with a diagnosis of portal cavernoma without abdominal malignancy or liver cirrhosis.The primary endpoint of this observational study was death and cause of death.Independent predictors of survival were identified using the Cox regression model.RESULTS:A total of 64 patients were enrolled in the study.During a mean follow-up period of 18±2.41mo,7 patients died.Causes of death were pulmonary embolism(n=1),acute leukemia(n=1),massive esophageal variceal hemorrhage(n=1),progressive liver failure(n=2),severe systemic infection secondary to multiple liver abscesses(n=1)and accident(n=1).The cumulative 6-,12-and 36-mo survival rates were 94.9%,86%and 86%,respectively.Multivariate Cox regression analysis demonstrated that the presence of ascites(HR=10.729,95%CI:1.209-95.183,P=0.033)and elevated white blood cell count(HR=1.072,95%CI:1.014-1.133,P=0.015)were independent prognostic factors of non-malignant and non-cirrhotic patients with portal cavernoma.The cumulative 6-,12-and 36-mo survival rates were significantly different between patients with and without ascites(90%,61.5%and 61.5%vs 97.3%,97.3%and 97.3%,respectively,P=0.0008).CONCLUSION:The presence of ascites and elevated white blood cell count were significantly associated with poor prognosis in non-malignant and non-cirrhotic patients with portal cavernoma.
文摘Portal cavernoma cholangiopathy(PCC)is one of the most harrowing complications of extrahepatic portal venous obstruc-tion,as it determines the long-term hepatobiliary outcome.Although symptomatic PCC is rare in children,asymptomatic PCC is as common as that in adults.However,there are major gaps in the literature with regard to the best imaging strategy and management modality in children.Moreover,natural history of PCC and effect of portosystemic shunt surgeries in children are unclear.Neglected PCC would lead to difficult or recalcitrant biliary strictures that will require endoscopic ther-apy or bilioenteric anastomosis,both of which are challenging in the presence of extensive collaterals.There are limited studies on the effect of portosystemic shunt surgeries on the outcome of PCC in children compared to adults.In this review,we aimed to collate all existing literature on PCC in childhood and also compare with adult studies.We highlight the difficul-ties of this disease to provide a comprehensive platform to foster further research on PCC exclusively in children.
文摘Biliary ductal changes are a common radiological finding in patients with portal hypertension, however only a small percentage of patients (5%-30%) develop symptomatic bile duct obstruction. The exact pathogenesis is not clear, but an involvement of factors such as bile duct compression by venous collaterals, ischemia, and infection is accepted by most authors. Although endoscopic retrograde cholangiopancrea- tography was used to define and diagnose this condition, magnetic resonance cholangiopancreatography is currently the investigation of choice for diagnosing this condition. Treatment is indicated only for symptomatic cases. Portosystemic shunts are the treatment of choice for symptomatic portal biliopathy. In the majority of patients, the changes caused by biliopathy resolve after shunt surgery, however, 15%-20% patients require a subsequent bilio-enteric bypass or endoscopic management for persistent biliopathy. There is a role for endoscopic therapy in patients with bile duct stones, cholangitis or when portosystemic shunt surgery is not feasible.
文摘Portal biliopathy(PB) is defined as the presence of biliary abnormalities in patients with non-cirrhotic/nonneoplastic extrahepatic portal vein obstruction(EHPVO) and portal cavernoma(PC). The pathogenesis of PB is due to ab extrinseco compression of bile ducts by PC and/or to ischemic damage secondary to an altered biliary vascularization in EHPVO and PC. Although asymptomatic biliary abnormalities can be frequently seen by magnetic resonance cholangiopancreatography in patients with PC(77%-100%), only a part of these(5%-38%) are symptomatic. Clinical presentation includes jaundice, cholangitis, cholecystitis, abdominal pain, and cholelithiasis. In this subset of patients is required a specific treatment. Different therapeutic approaches aimed to diminish portal hypertension and treat biliary strictures are available. In order to decompress PC, surgical porto-systemic shunt or transjugular intrahepatic porto-systemic shunt can be performed, and treatment on the biliary stenosis includes endoscopic(Endoscopic retrograde cholangiopancreatography with endoscopic sphincterotomy, balloon dilation, stone extraction, stent placement) and surgical(bilioenteric anastomosis, cholecystectomy) approaches. Definitive treatment of PB often requires multiple and combined interventions both on vascular and biliary system. Liver transplantation can be considered in patients with secondary biliary cirrhosis, recurrent cholangitis or unsuccessful control of portal hypertension.
基金Supported by Dr.Khuroo’s Medical Trust,a nonprofit organization which supports academic activities,disseminates medical education and helps poor patients for medical treatment
文摘Portal biliopathy refers to cholangiographic abnormalities which occur in patients with portal cavernoma. These changes occur as a result of pressure on bile ducts from bridging tortuous paracholedochal, epicholedochal and cholecystic veins. Bile duct ischemia may occur due prolonged venous pressure effect or result from insufficient blood supply. In addition, encasement of ducts may occur due fibrotic cavernoma. Majority of patients are asymptomatic. Portal biliopathy is a progressive disease and patients who have long standing disease and more severe bile duct abnormalities present with recurrent episodes of biliary pain, cholangitis and cholestasis. Serum chemistry, ultrasound with color Doppler imaging, magnetic resonance imaging with magnetic resonance cholangiopancreatography and magnetic resonance portovenography are modalities of choice for evaluation of portal biliopathy. Endoscopic retrograde cholangiography being an invasive procedure is indicated for endotherapy only. Management of portal biliopathy is done in a stepwise manner. First, endotherapy is done for dilation of biliary strictures, placement of biliary stents to facilitate drainage and removal of bile duct calculi. Next portal venous pressure is reduced by formation of surgical porto-systemic shunt or transjugular intrahepatic portosystemic shunt. This causes significant resolution of biliary changes. Patients who persist with biliary symptoms and bile duct changes may benefit from surgical biliary drainage procedures(hepaticojejunostomy or choledechoduodenostomy).
文摘Transjugular intrahepatic portosystemic shunt(TIPS) placement is a standard procedure for the treatment of portal hypertension complications. When this conventional approach is not feasible, alternative procedures for systemic diversion of portal blood have been proposed. A one-step interventional approach, combining minilaparotomy-assisted transmesenteric(MAT) antegrade portal recanalization and TIPS, is described in an adolescent with recurrent esophageal varice bleeding and portal cavernoma(PC). A 16-yearold girl was admitted to our Unit because of repeated bleeding episodes over a short period of time due to esophageal varices in the context of a PC. A portal vein recanalization through an ileocolic vein isolation with the MAT approach followed by TIPS during the same session was performed. In the case of failed portal recanalization, this approach, would also be useful for varice endovascular embolization. Postoperative recovery was uneventful. Treatment consisting of propanolol, enoxaparin and a proton pump inhibitor was prescribed after the procedure. One month post-op, contrast enhanced computed tomography confirmed the patency of the portal and intrahepatic stent grafts. No residual peritoneal fluid was detected nor opacification of the large varices. Endoscopy showed good improvement of the varices. Doppler ultrasound confirmed the accelerated flow in the portal stent and hepatopetal flow inside the intrahepatic portal branches. Three months post-op, TIPS maintained its hourglass shape despite a slight expansion. Portal hypertension and life threatening conditions related to PC would benefit from one-step portal recanalization. MAT-TIPS is feasible and safe for the treatment of PC even in children. This minimally invasive procedure avoids or delays surgical treatment or re-transplantation when necessary in pediatric patients.
文摘Extrahepatic portal vein obstruction(EHPVO) is a primary vascular condition characterized by chronic long standing blockage and cavernous transformation of portal vein with or without additional involvement of intrahepatic branches, splenic or superior mesenteric vein. Patients generally present in childhood with multiple episodes of variceal bleed and EHPVO is the predominant cause of paediatric portal hypertension(PHT) in developing countries. It is a pre-hepatic type of PHT in which liver functions and morphology are preserved till late. Characteristic imaging findings include multiple parabiliary venous collaterals which form to bypass the obstructed portal vein with resultant changes in biliary tree termed portal biliopathy or portal cavernoma cholangiopathy. Ultrasound with Doppler, computed tomography, magnetic resonance cholangiography and magnetic resonance portovenography are non-invasive techniques which can provide a comprehensive analysis of degree and extent of EHPVO, collaterals and bile duct abnormalities. These can also be used to assess in surgical planning as well screening for shunt patency in post-operative patients. The multitude of changes and complications seen in EHPVO can be addressed by various radiological interventional procedures. The myriad of symptoms arising secondary to vascular, biliary, visceral and neurocognitive changes in EHPVO can be managed by various radiological interventions like transjugular intra-hepatic portosystemic shunt, percutaneous transhepatic biliary drainage, partial splenic embolization, balloon occluded retrograde obliteration of portosystemic shunt(PSS) and revision of PSS.
文摘Extensive vascular network and proximity to the gastrointestinal tract make the liver susceptible to abscess formation.While pyogenic liver abscesses account for the majority of liver abscesses in the Western world,amebic liver abscesses are more prevalent in tropical and developing nations.Most liver abscesses heal without complications.However,various vascular complications can occur in these patients,including compression of the inferior vena cava,thrombosis of the portal vein and/or hepatic veins,hepatic artery pseudoaneurysm,direct rupture into major vessels or the pericardium,and biliovascular fistula.These compli-cations can present significant clinical challenges due to the potential for hae-morrhage,ischemia,and systemic embolism,thereby increasing the risk of morbidity and mortality.Mechanical compression,flow stasis,inflammation,endothelial injury,and direct invasion are some of the proposed mechanisms that can cause vascular complications in the setting of a liver abscess.For the diag-nosis,thorough assessment,and therapeutic planning of vascular complications,more sophisticated imaging techniques such as multidetector computed tomo-graphy angiography or magnetic resonance angiography may be necessary.Although most vascular complications resolve with abscess treatment alone,additional interventions may be required based on the nature,severity,and course of the complications.This article aims to provide a systematic update on the spectrum of vascular complications of liver abscesses,offering insights into their pathogenesis,diagnosis,and management strategies.
文摘目的总结Rex分流联合断流、脾动脉结扎术治疗小儿门静脉海绵样变的经验与疗效。方法回顾性分析2012年8月至2019年7月在安徽省儿童医院行Rex分流联合断流、脾动脉结扎术治疗的16例门静脉海绵样变、门静脉高压症患儿临床资料及随访结果。结果15例成功手术,分流前门静脉测压28.3~45.8 cm H 2O,平均(32.6±5.4)cm H 2O,分流后显著下降为17.2~27.8 cm H 2O,平均(23.4±3.7)cmH 2O,分流前后门静脉压力差异有统计学意义(P<0.01)。术后均恢复出院。1例术中吻合血管不通畅加行Warren术,术后脾肾静脉吻合口血栓形成,介入治疗效果不显著,自动出院后死亡。成功手术的15例患儿术后随访6个月至6年,平均随访31.5个月,3例术后有消化道出血复发,药物止血均较易控制,其中2例均复发1次,1例复发2次后未再出血,但出现脾大、脾功能亢进,术后5年行脾切除术。15例均生长发育良好。结论Rex分流联合断流、脾动脉结扎术治疗小儿门静脉海绵样变所致门静脉高压症效果确切。
文摘目的探讨球囊扩张术在儿童门静脉海绵样变(cavernous transformation of the portal vein,CTPV)Rex手术后桥血管与肝内门静脉左支吻合口狭窄中的应用效果。方法收集2018年11月至2019年4月由广州市妇女儿童医疗中心收治的CTPV Rex手术后桥血管吻合口狭窄并行介入治疗的5例患儿作为研究对象,其中男童2例,女童3例,年龄1.9~11岁。术前超声或CT检查显示桥血管吻合口不同程度狭窄,狭窄口内径为1~3.5 mm。结果5例CTPV患儿共行8次经皮肝穿刺门静脉入路血管扩张介入术,其中3例患儿仅行1次介入术(2例成功,另1例未成功);1例行2次介入(第1次未成功,第2次成功实施血管狭窄吻合口球囊扩张术);1例患儿因吻合口极度狭窄,约1 mm,前2次均未成功将导丝通过血管吻合口狭窄部,第3次联合肝胆外科,成功实施球囊扩张术。球囊扩张术后随访6~9个月,超声复查显示血管吻合口内径较术前扩大。5例CTPV患儿8次介入术中均未出现并发症。结论经皮肝穿刺肝内门静脉入路,对门静脉海绵样变Rex手术后桥血管吻合口狭窄的患儿实施球囊扩张术创伤小,疗效显著,有助于完善儿童CTPV治疗措施。
文摘目的探讨应用颈内静脉做移植血管的Rex手术治疗门静脉海绵样变的临床疗效。方法2015年5月至2018年5月东莞市儿童医院共收治12例门静脉海绵样变性患儿,年龄89~180个月,男童7例,女童5例。10例存在反复上消化道出血病史。12例均表现为脾功能亢进,术前红细胞、白细胞、血小板都呈现不同程度降低;肝功能各项指标均正常。术前影像学检查均提示:食管胃底静脉曲张,门静脉海绵样变;脾脏肿大。术后随访12~24个月。结果12例均成功实施手术,手术时间为380~480 min,出血量为30~150 mL,分流术前测肠系膜上静脉压力为24~37 cm H 2O,平均为(32.5±3.7)cm H 2O,分流术后降为17~34 cm H 2O,平均为(23.5±4.7)cm H 2O,分流术前后肠系膜上静脉压力差异有统计学意义(t=5.19,P=0.001)。术后复查血细胞计数较术前均有不同程度升高,手术前后白细胞分别为(2.1±0.6)×1012 g/L和(4.4±2.3)×1012/L,差异有统计学意义(t=3.29,P=0.003);手术前后血红蛋白分别为(65.2±19.5)g/L和(101.2±13.9)g/L,差异有统计学意义(t=5.70,P=0.001);手术前后血小板分别为(70.5±33.1)×109/L和(161.17±66.8)×109/L,差异有统计学意义(t=4.21,P=0.001)。术后12个月复查脾脏长度、厚度较术前均有缩小,差异有统计学意义(P=0.001)。随访期间患儿未再发生呕血,影像学检查显示分流血管通畅,肝功能各项指标正常。结论应用颈内静脉做移植血管的Rex分流术治疗门静脉海绵样变性效果良好。
