儿童门静脉海绵样变18例诊疗分析

目的探讨儿童门静脉海绵样变的临床特点及治疗方式。方法回顾性分析2012年至2018年浙江大学医学院附属儿童医院收治的18例门静脉海绵样变患儿临床资料、手术方式及预后情况,并进行总结分析。结果本组18例均手术成功。其中5例行Rex手术,选择的分流血管包括肠系膜上静脉、胃冠状静脉、胃右静脉以及门静脉扩张最显著的侧支血管。行Rex手术的患儿有1例术后因消化道出血、脾功能亢进而再次接受手术,发现为吻合口狭窄,术中切除狭窄段,取管径较大的一段空肠系膜血管作为补片搭桥,同时行食管胃底静脉结扎术。5例行Warren手术的患儿术后恢复良好,无一例消化道出血,脾功能亢进、脾大现象均明显改善,术后无一例并发症。4例行近端脾肾分流术(其中1例术后反复消化道出血,于二次手术时行肠系膜上静脉-下腔静脉分流术)。3例行肠系膜上静脉-下腔静脉分流术,同时切除脾脏,术后出现消化道出血,保守治疗后病情得到控制。结论儿童门静脉海绵样变需早期诊断,选择恰当的治疗措施对改善患儿的预后非常重要。外科治疗方法的选择应根据患儿的病情及门静脉系统血管的条件来决定,其中Rex手术是儿童门静脉海绵样变的首选治疗方法,此外Warren手术和近端脾肾分流术也是较为理想的术式。     

儿童门脉高压症病因和预后回顾分析

目的了解住院儿童门脉高压症的病因和预后。方法回顾性分析１９７８～１９９７年共２０年的住院病例５３例，随访４０例，随访时间为０５～１２年，中位数１３年。结果９０年代以后儿童门脉高压症的住院率有上升的趋势，诊断的病因亦增多；病因主要是门脉海绵样变、门脉性肝硬化和先天性胆道闭锁，遗传代谢病亦占有一定的比例；在主要病因中，以先天性胆道闭锁所致的门脉高压症预后最差，门脉性肝硬化的预后极不一致，部分预后良好，门脉海绵样变以分流术后的预后较好。结论儿童门脉高压症的病因构成与成人相比有明显不同的特点，其预后与病因和治疗方式有关，临床上应有所认识。     

小儿门静脉海绵样变术后并发乳糜腹水临床分析

目的 本文探讨小儿门静脉海绵样变术后并发乳糜腹水的发病原因,预防措施及治疗方法.方法 回顾性分析本院5年间收治因门静脉海绵样变门脉高压而反复上消化道出血患儿9例,均行手术治疗,术后4例出现乳糜腹,其中2例发生于脾脏切除加脾肾静脉分流术后,1例发生于肠系膜上静脉下腔静脉分流术后,1例发生于Warren术后.结果 4例乳糜腹水患儿有3例经过禁食及TPN等保守治疗后在术后1～4周乳糜腹水消失,1例患儿经过4周保守治疗后每天仍有2000 ml左右的乳糜腹水,在术后4周行腹腔大隐静脉转流术,术后1周乳糜腹水消失.随访1～6年,均无复发.结论 门静脉海绵样变术后并发乳糜腹水的主要原因为术中损伤淋巴管或乳糜池,保守治疗有效,必要时可手术治疗.     

小儿门静脉海绵样变治疗的思路

目的 探讨小儿门静脉海绵样变治疗的最佳术式。方法 对1990年～2002年收治的16例门静脉海绵样变的诊治过程进行回顾性分析。本组16例中13例因消化道出血入院，另3例因脾肿大、脾功能亢进人院。治疗方法有药物治疗，胃镜下注射硬化剂或套扎术，12例手术治疗。手术方式多种。7例行1次手术，3例行2次手术，1例行3次手术，1例行4次手术。结果 术后随访1～11年，3例仍有出血，非手术疗法可控制；4例经钡餐胃镜检查食道静脉曲张情况明显好转；4例B超下可测及吻合口的直径及血流速度；本组死亡3例，2例死于大出血、休克，1例死于术后败血症。结论 (1)小儿门静脉海绵样变所致的门脉高压症发病年龄早，消化道大出血为其主要症状，可危及生命。(2)治疗上应酌情选择降压效果明显、止血确切的手术方法。如未做过手术，脾大和脾亢不明显者，可首选肠腔分流；脾大和脾亢明显者在切脾同时作断流， 脾肾分流术；若已行脾肾分流术后仍出血，可选择肠腔分流。胃右静脉或胃冠状静脉与肝左静脉属支吻合法是一种新的门体分流术式，在条件允许时可试用。(3)对合并胆道疾病者，应先降低门脉压力、后行胆道手术。     

心血管系统疾病

012,82儿童门脉高压症7例临床分析/黄开宇…//温州医学院学报一2001,31(i)一51一52 结果:7例儿童门脉高压症,门脉海绵样变4例,肝硬化2例,先夭性肝纤维化1例。门脉海绵样变预后较肝硬化为好。结论:儿童门脉高压症的病因与成人不同,以门脉海绵样变为主,其预后与病因有关。参4(李瑛) 012783彩色多普勒超声心动图诊断多支肺静脉狭窄1例/张蓉//中国超声医学杂志一2000,16(12)一943一944 012784先天性心脏病免疫功能变化及其临床意义/杨林海…//山西医药杂志一2001,30(2)一102一103 对33例先心病患儿免疫球蛋白(I茄、I幼、IgM)及17例患儿T细胞亚…     

儿童门静脉海绵样变性的临床特点及手术治疗策略CSCD

目的探讨儿童门静脉海绵样变性的临床特点及手术治疗策略。方法回顾性分析湖南省儿童医院2010年5月至2022年1月收治的27例儿童门静脉海绵样变性患儿临床资料,其中男12例,女15例;年龄1岁7个月至11岁11个月。患儿均经腹部超声及CT检查确诊为儿童门静脉海绵样变性,并经数字减影血管造影(digital subtraction angiography,DSA)评估肝内外门静脉通畅情况,根据病变情况选取手术方式。结果27例患儿均实施手术。其中Rex手术19例,行Rex手术的患儿术后门静脉系统压力均明显下降;其中2例利用粗大肝外侧支静脉与门静脉矢状部行侧侧吻合,肝内门静脉获得满意灌注量;1例行巨脾切除后利用脾静脉行经典Rex手术;2例搭桥血管血栓形成,经抗凝治疗后失败,血管闭塞。3例因严重门静脉海绵样变性和反复消化道出血实施肝移植手术,其中1例早期发生肝动脉血栓,经抗凝治疗后再通。3例实施远端脾肾分流手术,分流血管通畅。2例实施近端脾肾分流手术,其中1例出现脾静脉附壁血栓,经抗凝治疗后好转。结论儿童门静脉海绵样变性常常以门脉高压相关症状为临床特点,手术方式应根据肝内外门静脉发育和代偿情况综合分析后进行选择,搭桥血管可以根据患儿自身血管特点进行选择。首选Rex手术的原因在于其能够恢复门静脉系统正常解剖通道和生理作用,改善肝脏灌注和生长发育,避免肝性脑病的发生。     

肠门分流（Rex手术）治疗肝外型门静脉高压的初步研究

目的探讨肠系膜上静脉门静脉左支分流术（Rex手术）治疗门静脉海绵样变的临床效果：方法2008年10月至2009年9月本院共收治6例门静脉海绵样变性并门脉高压患儿。年龄20～82个月,均为男性。4例有反复上消化道出血、呕血、输血史,输血量800～2400mL;2例表现为巨睥、脾功能亢进、进行性贫血。5例患儿术前红细胞、白细胞、血小板不同程度降低;肝功能各项指标均正常。术前影像学检查提示：食管胃底静脉曲张,门脉海绵样变;脾脏增大。手术采用上腹肋缘下横切口,测定肠系膜上静脉压力,并行门静脉造影。游离胃冠状静脉达食管裂孔水平,离断。游离肝门静脉左主支,阻断并纵行劈开矢状部,然后以6-0Prolene线将胃冠状静脉与门静脉左支行端侧吻合。再次测定肠系膜上静脉压力,并行血管造影。术后随访3～11个月。结果6例均成功实施手术,手术时间200．240min,出血约20～30mL,分流完成后造影显示分流血管均通畅无狭窄。分流前门静脉压力39～53cmH2O,分流后降为29～32cmH2O。术后住院时是7～12d：随访期间患儿未再出现呕血,影像学检查显示分流血管通畅,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,厚度3．5～4．0cm。结论Rex分流术治疗门静脉海绵样变性安全、可行,效果良好。     

小儿门静脉海绵样变性的外科治疗

目的 探讨小儿门静脉海绵样变性的外科治疗方法。方法 总结收治的16例小儿门静脉海绵样变性病例的临床表现、彩色多普勒的影像特征及手术中所见、实施手术的方法和效果。结果 首发症状多为呕血、便血，少数表现为顽固性贫血，脾脏肿大是其重要体征，均经彩色多普勒获得明确诊断。16例均行脾切除、贲门周围血管离断术有14例另加不同的分流手术。脾-肾分流手术适用于大多数门静脉海绵样变性的患儿，年龄及肾血管的直径不一定是限定手术的条件。术后随访显示患儿贫血纠正，上消化道出血明显改善。彩色多普勒显示分流处血流通畅。结论 该病治疗以手术治疗为主，在脾切除、贲门周围血管离断术的基础上，分流术可以获得良好的效果。     

改良Rex手术治疗小儿肝外门静脉高压的疗效及最佳方法探讨

目的 探讨改良Rex手术治疗小儿肝外门静脉高压的效果和预后,明确最佳Rex手术方法.方法 2008年2月至2016年3月,101例门静脉海绵样变患儿于我院接受Rex手术治疗,其中48例接受胃冠状静脉-门静脉左支分流术(CV-LPV),26例接受移植门静脉系统血管间置、门静脉主干-门静脉左支分流术(iPV-LPV),5例行保留脾脏的脾静脉近端-门静脉左支分流术(SV-LPV),6例接受血管间置的脾静脉-门静脉左支分流术(iSV-LPV),2例肠系膜下静脉-门静脉左支分流术(IMV-LPV),4例双冠状静脉-门静脉左支分流术(dCV-LPV),4例胃右静脉-门静脉左支分流术(RGV-LPV),4例胃网膜右静脉-门静脉左支分流术(RGEV-LPV),2例Roux-en-Y空肠支静脉-门静脉左支分流术(RYV-LPV).比较不同术式的手术时间、术后J静脉压力、脾功能亢进缓解程度、术后住院时间、术后再出血率、分流血管直径的差异.结果 101例患儿均成功接受Rex手术治疗.手术时间:CV-LPV明显高于iPV-LPV(P=0.036);iSV-LPV明显高于CV-LPV、iPV-LPV、RGV-LPV、RGEV-LPV和RYV-LPV(P=0.024、P-0.001、P=0.039、P=0.039和P=0.145).术后肠系膜上静脉压力:iSV-LPV明显高于iPV-LPV、dCV-LPV和RYV-LPV(P＜0.05);RGEV-LPV明显高于iPV-LPV、SV-LPV、dCV-LPV和RYV-LPV(P＜0.05).术后总体上消化道再出血发生率为18.8％,CV-LPV 22.9％,iPV-LPV 11.5％,SV-LPV 20.0％,iSV-LPV 0％,IMV-LPV 50.0％,dCV-LPV25.0％,RGV-LPV 0％,RGEV-LPV 50.0％,RYV-LPV 0％,组间比较差异无统计学意义(P=0.420).术后血小板:RGV-LPV明显高于CV-LPV、SV-LPV、iSV-LPV和dCV-LPV(P＜0.05);iPV-LPV明显高于SV-LPV和iSV-LPV(P＜0.05).各Rex手术方法间术后脾脏长度、厚度、术后住院时间、术后分流血管直径和流速、术后血HGB差异无统计学意义(P＞0.05).结论 改良Rex手术是治疗小儿肝外门静脉高压的有效方式,其中移植门静脉系统血管间置、门静脉主干-门静脉左支分流术是最佳改良Rex手术方法,应作为首选术式.     

小儿食管静脉曲张的内镜诊断与治疗

小儿食管静脉曲张发病率虽不高,但却是门脉高压症最严重的并发症之一,也是上消化道大出血最常见的原因,病死率甚高。引起门脉高压的常见原因,在小儿主要是肝硬化和肝外门静脉阻塞。过去常用三腔管压迫或脾切除,门脉分流术来治疗,但术后并发症的发生率很高,再出血率...     

Portal obstruction in children. II. Results of surgical portosystemic shunts

Seventy-six children with portal vein obstruction underwent surgical portosystemic shunt, for severe gastrointestinal tract bleeding in 64 and for prophylactic purposes in 12. Endoscopy and angiography or both showed shunt patency in 70 children; thrombosis occurred in the remaining six. The mean age at successful shunt surgery was 6 years 10 months. Early postoperative assessment of shunt patency was judged from regression of splenomegaly and thrombocytopenia when splenectomy was not performed; when done, early postoperative ultrasonography correctly indicated the result. Significant regression of endoscopy was most often delayed postoperatively for up to six months. Children with a proved patent shunt did not have any further episodes of gastrointestinal tract bleeding, displayed no clinical signs of encephalopathy, and often exhibited a striking increase in growth velocity. These results strongly support the contention that a portosystemic shunt is the best treatment for portal vein obstruction after the first spontaneous bleeding episode, even in young children.     

小儿门静脉海绵样变性、门静脉高压症

目的 探讨门静脉海绵样变性、门静脉高压症的病因、病理、临床表现、诊断方法、治疗及预后等问题。方法 总结我院７例经手术治疗病例的临床及影像学检查资料。结果 呕血伴排柏油样便为主要临床表现,脾脏增大为体验特征。本组均经彩色多普勒检查提示或明确诊断,４例术前行血管造影。胃底及下段食管血管断流、脾切除为共有手术方式,５例行不同形式的分流手术。结论 部分患儿可能为先天性因素所致。除门静脉高压症常见的临床表现     

64层CT在儿童门静脉海绵样变性手术治疗中的价值

目的 探讨64层CT在门静脉海绵样变性术前病情判断及手术方式选择中的作用.方法 情况,与患儿手术治疗方式对比分析.结果 12例患儿中5例食管胃底静脉曲张,行胃底及食管下段血管断流术和脾切除术,4例仅行脾切除术,3例病变累及肝内门静脉行活体肝移植.自发性脾/胃-肾静脉分流8例,腹膜后Retzius静脉丛(RV)开放4例,术中保留.术后1例肝移植出现门静脉血栓死亡,其余无明显并发症.结论 64层螺旋CT血管成像可清楚显示门静脉及分支,侧支循环建立情况,有助于术前手术方案选择.     

Focal nodular hyperplasia involving portal cavernoma]

Focal nodular hyperplasia is a rare hepatic tumor in children representing only 2% of all pediatric hepatic tumors. We report a case in teen-ager who had a symptomatic portal cavernoma at 9 years of age, and had been treated by porto-mesenteric surgical shunt. At 16 years, liver ultrasounds revealed multiple hepatic nodules. Focal nodular hyperplasia was suggested by CT scan and magnetic resonance imaging. Given the symptoms and the unusual and multiple lesions, diagnosis was confirmed by surgical biopsy. FNH pathogeny is discussed in this context.     

Extrahepatic portal hypertension in children: observations on three surgical procedures

This paper presents a comparative prospective study of three modalities of surgical treatment for extrahepatic portal hypertension in children: central splenorenal shunt after splenectomy (CSS), side-to-side lienorenal shunt (SSLR) without splenectomy, and splenectomy and gastroesophageal devascularization (SGD). In an 18-month period, 27 procedures were performed: 10 CSS, 10 SSLR, and seven SGD. The outcomes were evaluated by fall in portal pressures, hematological parameters, shunt patency, splenic regression, and disappearance of esophageal varices. All three procedures were comparable in the fall of portal pressure after surgery. The average blood loss and operating time were statistically significant in favor of SSLR compared with CSS. At 3-month follow-up, shunt patency was confirmed by duplex Doppler study in all the patients in the SSLR group and in nine out of 10 patients in the CSS group. In the CSS and SGD groups, hypersplenism resolved in all the patients. In the SSLR group, blood counts improved in only five out of eight affected children. No patient re-bled during a follow-up of 3–5 years. There were no cases of hepatic encephalopathy or overwhelming postsplenectomy sepsis. In conclusion, CSS is useful when there is a large spleen, severe hypersplenism, and a shuntable splenic vein. SSLR is suitable when there is only mild splenomegaly, mild hypersplenism, and a shuntable splenic vein. Splenectomy and devascularization is the choice when there is no shuntable splenic vein.     

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。     

Crohn's disease lesions in the upper gastrointestinal tract: correlation between clinical, radiological, endoscopic, and histological features in adolescents and children

The incidence of Crohn's disease (CD) lesions in the upper gastrointestinal (GI) tract of both adults and children is frequently underestimated. In this prospective study, a total of 31 children suspected of having Crohn's disease were systematically examined to identify upper digestive tract lesions. They all underwent barium transit endoscopy with multiple-level biopsies. Typical clinical symptoms suggestive of upper GI tract involvement were found in 5 children (16%), radiological signs in only one child (3%), endoscopic lesions in 13 children (42%), and specific granulomas in 12 children (39%). In eight of these 12 children, the biopsies were taken from macroscopically normal areas of the esophagogastroduodenal mucosa. One of the 31 children had no abnormal radiological and endoscopic features suggestive of CD on the distal small bowel and the colon. There was no correlation between the clinical, radiological, and histological data. Endoscopy plus biopsy provided a positive diagnosis in 39% of cases and a confirmation of the diagnosis in 87% of cases. Endoscopic and histological evidence of CD of the upper GI tract is often present despite an absence of clinical symptoms or radiological changes. Upper GI tract endoscopy with multiple biopsies may be important in the evaluation of this condition and even in some cases for the establishment of the diagnosis.