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
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
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
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
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。
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。
