肠系膜上静脉门静脉左支分流术治疗肝外门脉高压

目的 探讨肠系膜上静脉门静脉左支分流术治疗肝外门静脉梗阻的效果.方法 2008年10月至2010年2月对12例肝外门脉梗阻并门脉高压患儿实施肠系膜上静脉门静脉左支分流术(mesenteric-to-left portal vein bypass,MLPVB;又称Rex Shunt,RS手术).2例因门静脉左支闭锁,无法吻合,实施远端脾肾分流术(Warren手术).RS手术患儿年龄1.6～12岁,平均(5.6±3.3)岁.男8例,女2例.7例患儿有反复呕血、黑便史,输血史,输血量800～2400 ml;另外3例患儿表现巨脾、脾功能亢进、进行性贫血.8例患儿术前红细胞、白细胞、血小板降低;2例AST轻度升高,1例TBIL轻度升高,其他患儿各项指标正常;无脑病表现.术前影像学检查提示:10例表现食道胃底静脉曲张,门脉海绵样变;脾脏增大,脾脏平均长径(n=8)(12.4±0.8)cm,厚度(5.1±0.57)cm.手术采用上腹肋缘下横切口,测定肠系膜上静脉压力,并行门脉造影.分离矢状部肝组织,游离肝门静脉左支,穿刺测压并造影,确认门静脉左支通畅,阻断钳阻断并纵行劈开矢状部.根据门脉造影及探查情况选择游离胃冠状静脉、或脾静脉或移植一段合适的回肠静脉.然后以6-0 Prolene线将胃冠状静脉、脾静脉与门静脉左支行端侧吻合,移植血管一端与门静脉左支矢状部吻合另一端与门静脉远端吻合.再次测定肠系膜上静脉压力,并行血管造影.术后随访4～20个月.结果 10例患儿均成功实施手术,平均手术时间(220±14.7)min,出血约10～50 ml,分流完成后造影显示分流血管均通畅无狭窄.分流前门脉平均压力(38.3±7.2)cm H2O,分流后降为平均(27.2±5.3)cm H2O,(P=0.001).平均住院时间(10±1.9)d.随访期间无患儿再出现呕血,影像学检查分流血管通畅,肝功能各项指标正常;血小板、红细胞血红蛋白、白细胞正常;脾脏较术前缩小,脾脏平均长径(n=8)(10.2±0.5)cm,较术前缩小(P=0.00),平均厚度(3.6±0.2)cm,较术前缩小(P=0.000),门静脉左支直径增大.结论 Rex分流术治疗门静脉海绵样变性安全、可行、效果良好.     

硬化剂治疗食管静脉曲张破裂出血的远期疗效分析

目的 探讨经硬化剂治疗食管静脉曲张破裂出血后的远期疗效。方法 1996-2003年在内镜下用硬化剂(1％乙氧硬化醇)注射治疗15例食管静脉曲张破裂出血患儿,年龄3—14岁,采用静脉内和静脉旁联合注射。注射后长期临床与内镜随访,观察再出血率及曲张静脉消失率与并发症发生率。结果 15例共作了43次硬化剂注射治疗,依治疗时间先后不同,随访时间从3年4个月到7年2个月不等,平均5年6个月。10例经2次治疗,5例经3次治疗,曲张静脉基本消退,内镜随访发现曲张静脉平均消退时间为3-6个月。15例长期随访发现仅3例曲张静脉复发。再出血率明显减少,15例中仅3例发生再出血,其中2例因长期失访,直至再出血来院检查,发现1例系十二指肠溃疡,1例为静脉曲张出血,而治疗前几乎每1-2个月出血1次。此外未见一例食管穿孔、食管狭窄的并发症。结论 食管静脉曲张硬化剂疗法治疗儿童食管静脉曲张安全有效,尤其是肝外血管畸形所致的门脉高压症效果显著,是挽救患儿生命,提高患儿生命质量的有效内科治疗方法。     

Non-conventional portosystemic shunts in children with extrahepatic portal vein obstruction

Portal hypertension due to extrahepatic portal vein obstruction is ideally treated by the use of a selective shunt. In a four year period between July 1987 and June 1992, 50 surgical procedures were carried out in 48 children with portal hypertension-related variceal hemorrhage. The portal, splenic, mesenteric, or coronary vein was not available for anastomosis in four children who, therefore, underwent non-conventional shunts which are described here. There was no postoperative mortality. One patient had a repeat gastrointestinal hemorrhage, probably due to stenosis of the shunt and failure to ligate the coronary vein. No rebleeding occurred on follow-up of at least 18 months. We conclude that in selected children requiring surgery for portal hypertension due to extrahepatic portal venous obstruction, a non-conventional shunt may be used with beneficial results.     

肝素联合波立维抗凝方案在Rex手术治疗小儿肝外门静脉梗阻中的应用及疗效分析

目的 探讨肝素联合波立维抗凝治疗方案对改善Rex手术预后和分流血管通畅性的效果.方法 以2010年1月至2019年9月首都儿科研究所收治的51例肝外门静脉梗阻(extra-hepatic portal venous obstruction,EHPVO)患儿为研究对象,其中男32例,女19例;51例均接受门静脉系统静脉间...     

儿童食管静脉曲张出血结扎治疗

食管静脉曲张（esophageal varices,EV）出血的内镜下诊断和治疗,近几年已取得很大进展,并且已经取代外科手术,作为首选的治疗方法。在内镜下食管静脉曲张硬化治疗（endoscopic variceal sclerotherapy,EVS）之后,由Stigmann首先进行了内镜下食管静脉曲张结扎治疗（endoscopic variceal ligation,EVL）,在成人目前已有大量报道,但在儿童报道不多。我院1999年1月至2003年12月共有287例患者行EVL治疗,其中小于14岁儿童有7例,并取得显著疗效。现报道如下。     

内窥镜下硬化剂注射治疗儿童门脉高压性食管静脉曲张出血的疗效

评价内窥镜下硬化剂注射治疗儿童高压性食管静脉曲张的疗效。方法回顾１９９６－１９９９年５例１１例的硬化剂注射。其中４男１女,平均年龄６．２岁。４例在外院行脾切除,２例脾切 后复发出血。１例行分流术,１例食管下端曲张静脉断流术。     

内镜下硬化剂注射治疗儿童食管静脉曲张的疗效观察

目的：评价内镜下硬化剂注射治疗儿童门脉高压性食管静脉曲张出血的疗效。方法：从1996年11月－2000年6月，行内镜下硬化剂注射治疗儿童门脉高压性食管静脉曲张10例，患儿平均年龄8．2岁，9例曾在外院做过脾切除手术。结果：10例患儿共注射22次，其中8例梅例注射2次，2例注射3次，术后再出血仅1例，其余9例经第1次注射后至今未再出血。随访发现每例患儿曲张静脉首次治疗后就明显消退，目前6例基本消迭，4例已完全消失。结论：硬化剂注射治疗门脉高压性食管静脉曲张出血是安全有效的。     

Spleen stiffness measurement as a non-invasive test to evaluate and monitor portal hypertension in children with extrahepatic portal vein obstruction

Pediatric Surgery International - The aim of the study was to test the feasibility of spleen stiffness measurement (SSM) by two-dimensional shear wave elastography (2D-SWE) and compare data on its...     

Ultrasound diagnosis of extrahepatic portal vein obstruction in childhood

Portal vein obstruction in childhood produces portal hypertension and hepatopetal collaterals surrounding the obstructed portal vein; this is the cavernomatous transformation of the portal vein which is a well defined angiographic entity. Clinically findings are the various manifestations of Banti's syndrome, especially splenomegaly and gastrointestinal bleeding. Ultrasonic diagnosis is possible on the associated findings of splenomegaly, normal liver, a subhepatic sponge like mass corresponding to the hepatopetal collaterals. These signs must be sought in the ultrasonic differential diagnosis of splenomegaly in pediatrics.     

Cholestasis in children with portal vein obstruction

We describe cholestasis as a result of bile duct abnormalities in 8 children with portal vein obstruction. In a clinical, biochemical and radiological investigation of 121 children with cavernous transformation of the portal vein seen between 1986 and 2000, 8 presented with jaundice, pruritus, and/or raised serum aminotransferases and/or gamma glutamyl transpeptidase (gamma GT) activities. Each displayed dilation and narrowing of intra- and/or extrahepatic bile ducts. Surgical decompression of the portal system (portal-systemic or Rex anastomosis) resulted in the regression of the signs of cholestasis in all children. We conclude that children with portal vein obstruction may exhibit clinically significant cholestasis as a result of external compression of the bile duct by the cavernoma.