Warren手术治疗小儿门脉高压症50例分析

目的 探讨保留脾脏、远端脾静脉肾静脉分流手术（Warren手术）治疗小儿门脉高压症的临床效果.方法 1999年10月至2012年12月,我们收治门脉高压症患儿50例,年龄3～15岁.男18例,女32例.患儿表现为反复消化道出血,食管静脉曲张,脾功能亢进.门静脉主干呈海绵窦样改变36例,Carolis病合并肝纤维化3例,先天性肝纤维化9例,门静脉发育不良1例,门静脉海绵样变Rex术后复发1例.Child-pugh评分均为A级（5～7分）,脾静脉直径6.0 ～ 15.0 mm,平均（9.8±2.2）mm.术中先经小肠系膜Ⅲ级静脉和脾静脉分支行静脉压力测定及造影,将脾静脉游离,于距肠系膜下静脉0.5 cm处切断,与左肾静脉端-侧吻合.结果 所有患儿实施远端脾-肾分流手术,手术时间255～ 370 min,平均手术时间232 min,术中失血10～ 30 mL.分流后门静脉和脾静脉压力均下降,门静脉压力分流后较分流前显著降低（P=0.026）;脾静脉压力分流后较分流前明显降低（P =0.007）.迄今为止随访时间跨度为0.5～11年.随访期间,患儿脾脏缩小,血红蛋白、白细胞、血小板恢复正常,无一例出现脑病.B超检查显示49例脾肾静脉吻合口通畅,1例术后1个月发现脾肾吻合口闭合,经再次手术治疗痊愈.结论 Warren手术治疗小儿门脉高压症效果显著,能有效降低小儿门脉高压术后食管静脉曲张再出血的发生率,保证入肝血流,减少肝性脑病的发生,是治疗小儿门脉高压症较理想的术式.     

Warren手术治疗小儿门脉高压

目的 总结过去8年采用保留脾脏、远端脾静脉肾静脉分流手术(Warren手术)治疗小儿门脉高压的效果.方法 本组患儿36例,年龄3～15岁,男12例,女24例.患儿表现为反复消化道出血,食管静脉曲张,脾功能亢进.门静脉主干呈海绵窦样改变29例,Carolis病合并肝纤维化2例,先天性肝纤维化5例.Child-pugh评分均为A级(5～7分),脾静脉的直径6.5～12.2 mm,平均为(8.6±2.3)mm.术中首先经小肠系膜山级静脉和脾静脉分支行静脉压力测定及造影.然后将整个脾静脉游离,距肠系膜上静脉0.5 cm处切断,与左肾静脉端一侧吻合.结果 36例患儿实施远端脾-肾分流手术,平均手术时间3.1 h,手术失血10～30 ml;无术中需输血者.分流前肠系膜上静脉压力为26.5～33.3 cmH2O,平均值为(28.9±4.8)cmH2O,脾静脉压力为26.2～33.5 cmH2O,平均值为(28.5±4.5)cmH2O;分流后肠系膜上静脉压力为17.2～26.4cmH2O,平均值为(23.8±3.9)cmH2O,脾静脉压力为10.5～16.cmH2O,平均值为(13.5±4.7)cmH2O,分流手术后脾静脉压力明显降低(P＜0.01).手术后2例患儿出现乳糜腹,保守治疗1个月后自然消失.随诊6～94个月,1例(1/36)手术后3个月吻合口闭合,行脾切除和断流手术.其他35例随访期内无再发消化道出血者,脾脏尽管比正常同龄儿童稍大,但均回缩,且血红蛋白、白细胞、血小板均在正常水平,未出现脑病者.B超检查,脾肾静脉吻合口通畅.结论 Warren手术是治疗小儿肝外型门静脉高压的有效途径之一,预防消化道再出血和脾功能亢进效果可靠,同时具有保留脾脏,贲门胃底血流可通过胃短动脉流向脾静脉使该区域保持低压状态,不降低门静脉灌注压力等优点.     

并发消化道出血的先天性门体分流畸形8例影像学特征病例系列报告

摘要目的：探讨合并消化道出血的先天性门体分流畸形的影像学特征,以提高对该畸形的认识。方法：纳入复旦大学附属儿科医院病历系统中先天性门体分流且病史中有消化道出血症状的病例,采集性别、年龄、临床表现、CTA和MRA影像学资料、外科手术结局。CTA为GE Light speed 64排螺旋CT扫描仪,MRA为Siemens Avanto 1.5 T 扫描仪,提取纳入病例的CTA原始图像,以1.25 mm层厚/0.625 mm层间隔重建,传入后处理工作站进行多平面重建;提取纳入病例的MRA原始图像,传入MR工作站进行后期多平面重建;由2名从事儿科影像诊断10年以上医生独立读片,观察门静脉及其属支的形态及走行,异常门体分流位置、途径,髂静脉、下腔静脉及肠管、肠壁血管分布。结果：2008年3月至2017年1月符合本文病例纳入标准的连续病例8例,男6例,年龄3月至10岁,7例贫血,7例便血,1例呕血。8例均行CTA检查,其中2例同时行MRA检查。截止出院时,6例行手术治疗,2例随访观察。8例先天性门体分流畸形患儿均为肝外型门体分流,2例肝外型Ⅰ型,6例为Ⅱ型。以便血为表现的7例中,髂内静脉、直肠上静脉及结肠静脉扩张扭曲;以呕血为表现的1例,脾静脉和肠系膜上静脉汇合后通过胃冠状静脉汇入左肾静脉,胃冠状静脉显著扩张、扭曲。结论：合并便血的先天性门体分流大部分通过肠系膜下静脉与髂静脉异常沟通,发生便血的比例高。     

Pathology of Noncirrhotic Portal Hypertension: Clinicopathologic Study in Pediatric Patients

From 1995–2002, 14 patients with predominantly prehepatic, noncirrhotic portal hypertension were evaluated. At presentation, the eight females and six males had a mean age of 9 years (range 2–18). Seven were admitted with gastrointestinal, mostly esophageal bleeding, three with splenomegaly, three with hepato-pulmonary syndrome, and one with hyperammonemia. Imaging studies showed portal vein obstruction in six patients and nonobstructed but frequently anomalous vascular patterns, including hypoplasia of the portal vein, in the remaining eight patients. At the onset, liver function was marginally abnormal in all patients, but thrombocytopenia of approximately 100 × 10⁹/L was consistently observed, probably reflecting chronic mild consumption coagulopathy and hypersplenism. The most striking and frequent histopathologic finding in 25 liver samples, was the presence of hypoplastic portal triads with collapsed portal vein radicles. In contrast, other triads showed markedly distended and misshapen portal vein radicles and likely lymphatics. These two patterns of collapse and distention presumably reflect areas of impaired versus overloaded intrahepatic portal venous flow. Some of the biopsies showed variable portal/sinusoidal fibrosis. Four patients (two with intestinal bleeding, two with hepato-pulmonary syndrome) required liver transplants and are doing well. Eight patients are doing well clinically after surgical or spontaneous vascular shunting. Two patients with intestinal bleeding and hepato-pulmonary syndrome, respectively) who had congenital dyskeratosis, underwent bone marrow transplantation and died of nonhepatic-related complications. It is possible to suggest prehepatic causes of portal hypertension even in needle biopsies when collapsed portal vein radicles are present in portal triads, but more than one biopsy sample with larger bore bioptomes may be required to see the changes. Conversely, identifying these changes may suggest to the clinicians the need to work-up a patient for portal hypertension. This study was presented in abstract form at the annual meeting of the Society for Pediatric Pathology, Chicago, Illinois, USA, February 2002.     

门静脉主干-门静脉右支搭桥治疗门静脉海绵样变

目的 Rex手术(肠系膜上静脉与门静脉左支矢状部搭桥分流手术)是治疗肝外门静脉海绵变导致门静脉高压的最流行术式,但是有大约14％的患者合并有门静脉矢状部闭锁或发育不良不能行此分流手术,并且此手术后的失败率在4％～28％,这限制了这种符合生理性分流手术的应用.本研究的目的是探索向肝内门静脉右支分流建立恢复门静脉血流的新途径.方法 2015年11月至2016年6月,对4例门静脉海绵样变性行Rex分流手术后门脉高压复发患儿行门静脉右支分流手术.患几年龄3.5～8.9岁,平均6.1岁,分别于1.1～2.5年前在我院行Rex手术,其中采用扩张的胃冠状静脉与肝内门静脉矢状部搭桥3例,扩张的门静脉主干分支与肝内门静脉矢状部搭桥1例,术后再发出血和脾功能亢进,经保守治疗无效.术前影像学检查提示:食管胃底静脉曲张,门静脉海绵样变存在;原搭桥血管显示不清,肝内门静脉血流少;脾脏增大.手术采用原上腹肋缘下横切口,首先游离肝门的了解原搭桥血管的形态;然后将胆囊从胆囊床上游离;在右侧肝门的后方切开Glisson氏鞘,显露门静脉右支,将其游离足够的长度;然后切开肝十二指肠韧带,游离扩张门静脉主干足够的长度;根据肝内门静脉右支至肝外门静脉主干之间的距离,截取肠系膜下静脉作为搭桥血管:血管壁外观正常,直径≥5 mm;以7-0 Prolene线将间置静脉的近心端和远心端分别与门静脉右支和肝外门静脉的主干行端侧吻合.术后1个月、3个月及6个月定期随访.结果 4例患儿中3例门静脉右支发育好、通畅,直径5～6 mm,成功实施门静脉右支搭桥手术;另外1例门静脉右支纤细放弃搭桥.手术时间2.3 h(1.9～3.5 h),术中出血约50 ml(30～80 ml),无一例需要输血.取肠系膜下静脉间置3例;搭桥后门静脉的平均压力由33 cmH2 O(26～45 cmH2 O)下降至18.7 cmH2O(14～24 cmH2O).术中发现肝内门静脉右支与左支延续相通,而与肝外门静脉主干中断不通;Glisson鞘内沿着胆管周围大量增生的小静脉;原搭桥血管呈条索状,一致性狭窄或闭锁.术后随访6～18个月,无患儿再出现呕血,影像学检查分流血管增粗通畅,肝内门静脉增粗,门静脉海绵样改变消失,肝功能各项指标正常;血小板、红细胞血红蛋白、白细胞正常.结论 门静脉主干与肝内门静脉右支分流是治疗肝外型门静脉高压的有效新方法,适用于门静脉左支发育不良或Rex手术失败的病例,目前该方法尚未见文献报告.     

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.     

Effectiveness of Rex shunt in children with portal hypertension following liver transplantation or with primary portal hypertension

Abstract:  Portal vein thrombosis can occur as a result of primary anomalies, after liver transplantation, and for other reasons. It may result in severe complications secondary to portal hypertension, such as bleeding from esophageal or gastric varices, hypersplenism, or impaired somatic growth. In this retrospective study, we analyzed the outcome of 25 children who underwent a Rex shunt procedure. The following venous grafts were used as the shunt: the autologous internal or external jugular vein (n = 17) or a cryopreserved graft (n = 5); in three patients the umbilical vein was recanalized. The median follow up time was 109 months (range 18 days–146 months). The best results were achieved in patients in whom an autologous jugular vein segment was used as a vascular graft for the Rex shunt (shunt patency of 88%). In patients with a functioning shunt no further lower or upper gastrointestinal bleeding occurred. And in the entire study population hypersplenism syndrome improved after surgery. In our large cohort of pediatric patients, the Rex shunt has shown to be an effective method to eliminate portal hypertension and to revascularize the liver and thereby prevents the possible consequences of long-term portosystemic shunting.     

Late complications in long-term survivors of biliary atresia.

Ninety patients with biliary atresia surviving more than 5 years were analysed with respect to late complications occurring after the age of 4 years. Thirty-five had complications including cholangitis, portal hypertension, hypersplenism, gastrointestinal bleeding, and esophageal varices. These complications occurred at various times. The background factors of late complications were past history of cholangitis soon after the operation, advanced age at operation, re-operation, high portal pressure at initial operation, and a long interval before disappearance of jaundice after surgery. These factors are mostly related to the first operation and its postoperative course. Therefore, we stress that late complications can be prevented by intensive treatment of the patient at the time of the first operation.     

颈内静脉搭桥Rex手术治疗小儿肝外门静脉梗阻的初步探讨

目的 探讨颈内静脉搭桥Rex手术(肠系膜上静脉-门静脉左支吻合术)治疗小儿肝外门静脉梗阻的手术效果.方法 2014年10月至2015年6月共收治肝外门静脉梗阻8例,行颈内静脉搭桥Rex手术治疗,随访1～9个月,评价手术效果.手术方法:取上腹正中纵行切口,首先切断肝圆韧带,并沿其向Rex窝分离,解剖出门静脉左支矢状部3 cm;沿结肠中血管找到肠系膜上静脉,分离出3 cm;取左侧颈部横切口,切取颈内静脉7～9 cm.将颈内静脉在门静脉左支和肠系膜上静脉之间搭桥行两个端侧吻合.结果 8例中男5例,女3例,平均年龄4岁(1岁4个月至7岁),表现为消化道出血6例,脾功能亢进6例,出血合并脾亢4例.术前肝功能及凝血大致正常,胃镜检查7例食管胃底静脉曲张,1例无曲张.超声及CT均诊断门静脉海绵样变性;行经肝静脉逆行门静脉系统造影,显示门静脉左支矢状部存在7例,未显示1例(术中证实为闭锁).7例手术探查Rex窝内有血流,完成Rex手术,1例门静脉左支闭锁,改行Warren手术.吻合后经肠系膜上静脉造影显示搭桥血管通畅,肝内门静脉系统显影,肝外侧枝曲张血管明显减少或未显示.吻合后门静脉压力明显降低,吻合前PVP为(27.00±3.74)mmHg,吻合后PVP为(19.00±2.71)mmHg,吻合前后PVP压力差为(8.00±3.42)mmHg,P=0.001.术后随访,全部患儿无再次出血,6例术前脾亢患儿5例得到缓解,1例未缓解;3例于术后半年复查胃镜,食管胃底静脉曲张由重度转为轻-中度;超声检查门静脉血流均通畅,无血栓及狭窄,术后门静脉左支直径为(7.10±2.47)mm(3.3～10.5 mm).结论 Rex手术对于小儿门静脉梗阻具有良好治理效果,在降低门静脉压力的同时,恢复了肝脏血流,是EHPVO根治性手术方式,应该作为治疗小儿肝前性门静脉高压的首选手术方法.     

肝前型门静脉高压症动物（犬）模型制作体会

目的 为使肝前型门脉高压症动物实验更可靠,建立符合或按近其临床表现的动物模型。方法 实验犬29只。开腹测脾体积、门静脉直径及压力。部分结扎门脉使其截面积缩小95．0％,门脉压上升到结扎前的3倍,胃肠无明显淤血时关腹。分别在术后2、4、8、12周及16周再次开腹观察胃肠淤血及侧支循环情况,测门脉压及脾体积。结果 4只死亡。2－8周的门脉压较高,腹膜后水肿、肠壁肥厚。随着侧支循环的建立,压力逐渐下降,12－16周脉压稳定,腹膜后水肿消失,交通血管清晰。12、16周门静脉的方法制作肝前型门脉高压症动物模型,缩窄门脉后12－16周为成功;更接近其临床表现,有一定的侧支循环的门脉高压症,此时做肝前型门脉高压症的实验数据最可靠。     

Congenital portocaval shunt

Several congenital anomalies of the portal vein and inferior vena cava (IVC) have been reported; however, reports of a congenital communication between the portal vein and IVC are few. We report a patient who was found to have a natural shunt between the extrahepatic portal vein and the IVC that was discovered when she underwent a right hepatic lobectomy for an undifferentiated liver sarcoma. The patient also had agenesis of the right kidney. We have not been able to find a similar case reported in English literature.     

Recanalized umbilical vein as a conduit for mesenterico/porto-Rex bypass for patients with extrahepatic portal vein obstruction

Purpose

Mesenterico-left portal vein (meso-Rex) bypass is as an effective modality for restoring intrahepatic portal perfusion in patients with extrahepatic portal vein obstruction. Achieving sufficient patency is difficult with end-to-side anastomosis of a bypass graft to a small or hypoplastic left portal vein in the Rex recessus. Here, we describe the use of a recanalized umbilical vein in the round ligament as a conduit for bypass construction in two patients.

Methods

Case 1 was an 11-year-old boy diagnosed with rupture of the esophageal varices and hypersplenism due to congenital extrahepatic portal hypertension. Because of persistent hypersplenism and thrombocytopenia, he underwent meso-Rex bypassing with a left iliac vein graft interposed between the umbilical vein and the superior mesenteric vein. Case 2 was a neonate with a large hepatic tumor (mesenchymal hamartoma) that developed abdominal compartment syndrome at birth. The tumor was removed by right hepatectomy with excision of the portal vein bifurcation at 3?days of age. Porto-Rex bypassing was accomplished by end-to-end anastomosis between the portal vein trunk and the umbilical vein.

Results

Sufficient hepatopetal portal flow through the umbilical vein was achieved in both patients and maintained for over 16 and 13?months, respectively. Although hypersplenism remained in Case 1, intrahepatic portal vein branches gradually widened and the cavernoma in the hepatic hilum disappeared within 2?months. Neither patient had symptoms or signs of portal hypertension at the most recent follow-up.

Conclusion

Using the umbilical vein as a vein conduit may facilitate construction of a meso/porto-Rex bypass and restore intrahepatic portal vein perfusion in patients with extrahepatic portal vein obstruction.     

Jejunal varices diagnosed by capsule endoscopy in patients with post‐liver transplant portal hypertension

Portal hypertension secondary to portal vein obstruction following liver transplant occurs in 5%‐10% of children. Jejunal varices are uncommon in this group. We present a case series of children with significant GI blood loss, negative upper endoscopy, and jejunal varices detected by CE. Case series of patients who had CE for chronic GI blood loss following liver transplantation. Three patients who had their initial transplants at a median age of 7 months were identified at our institution presenting at a median age of 8 years (range 7‐16 years) with a median Hgb of 2.8 g/dL (range 1.8‐6.8 g/dL). Upper endoscopy was negative for significant esophageal varices, gastric varices, and bleeding portal gastropathy in all three children. All three patients had significant jejunal varices noted on CE in mid‐jejunum. Jejunal varices were described as large prominent bluish vessels underneath visualized mucosa, one with evidence of recent bleeding. The results led to venoplasty of the portal vein in two patients and a decompressive shunt in one patient with resolution of GI bleed and anemia. CE is useful to diagnose intestinal varices in children with portal hypertension and GI bleeding following liver transplant.     

可恢复性肝外门静脉高压动物模型的建立及其实验研究

目的探讨采用套管将门静脉主干部分缩窄后再去除的方法制作一种可恢复性肝外门静脉高压动物模型的可行性及有效性。方法将18只新西兰大白兔按随机数字表法分为三组,每组6只:对照组(normal control group,NC)、肝外门静脉高压组(extra-hepatic portal venous obstruction,EHPVO)和可恢复性肝外门静脉高压组(reversible extra-hepatic portal venous obstruction,r-EHPVO)。NC组仅开腹后游离门静脉主干。EHPVO组采用套管将门静脉主干部分缩窄制作肝外门静脉高压动物模型。r-EHPVO组在肝外门静脉高压模型制备2周后将套管去除。检测所有动物模型手术前后的门静脉压力、血常规和脾脏大小,并比较各组手术前后门静脉压力、红细胞、白细胞、血红蛋白及血小板和脾脏大小的变化。多组间比较采用单因素方差分析(one-way ANOVA),同一组不同时间段间的数据比较采用配对样本的t检验,以P<0.05为差异有统计学意义。结果18只动物模型的制作均成功完成。EHPVO组和r-EHPVO组的术后门静脉压力和脾脏大小均显著高于NC组,差异有统计学意义(P<0.01);EHPVO组与r-EHPVO组套管拆除前门静脉压力和脾脏大小比较,差异无统计学意义;套管拆除后r-EHPVO组门静脉压力和脾脏大小显著低于EHPVO组,但其仍高于NC组(P<0.01)。EHPVO组和r-EHPVO组的术后血红蛋白、红细胞和血小板均显著低于NC组(P<0.05);去除套管后,r-EHPVO组血红蛋白、红细胞和血小板显著高于EHPVO组,但仍低于NC组。结论采用套管将门静脉主干部分缩窄后再去除的方法制作可恢复性肝外门静脉高压动物模型是可行的,该动物模型可缓解门静脉高压状态(降低门静脉压力、脾脏大小和改善脾功能亢进状态),具有Rex手术治疗肝外门静脉高压的相似效果,能作为模拟Rex手术研究的动物模型。     

Correlation of splenic volume with hematological parameters, splenic vein diameter, portal pressure and grade of varices in extrahepatic portal vein obstruction in children

Purpose  

To study the correlation between the volume of the spleen and hematological parameters, splenic vein diameter, portal pressure before shunt, portal pressure after shunt, reduction of portal pressure and grade of esophageal varices in patients with extrahepatic portal vein obstruction (EHPVO).     

Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and cystic dysplasia of the kidney

Congenital absence of portal vein is a rare malformation. To date, 16 cases have been reported – all in association with other anomalies, i.e. benign or malignant hepatic neoplasms in 6 cases and cardiac malformations in 12. This case report described a girl with congenital absence of portal vein, focal nodular hyperplasia of the liver and cystic kidney dysplasia. Angiography showed the splenic vein and superior mesenteric vein joining to form a common trunk that entered the inferior vena cava directly above the liver. A review of the other cases in the literature is provided and the clinical aspects of our patient are discussed. Received: 3 December 1996 / Accepted in revised form: 9 September 1997     

Unusual spontaneous porto‐systemic shunt: The importance of diagnosing non‐anatomical porto‐systemic shunts to improve portal flow in pediatric living–related liver transplantation. Case report

Collateral circulation secondary to liver cirrhosis may cause the development of large PSSs that may steal flow from the main portal circulation. It is important to identify these shunts prior to, or during the transplant surgery because they might cause an insufficient portal flow to the implanted graft. There are few reports of “steal flow syndrome” cases in pediatrics, even in biliary atresia patients that may have portal hypoplasia as an associated malformation. We present a 12‐month‐old female who received an uneventful LDLT from her mother, and the GRWR was 4.8. During the early post‐operative period, she became hemodynamically unstable, developed ascites, and altered LFT. The post‐operative ultrasound identified reversed portal flow, finding a non‐anatomical PSS. A 3D CT scan confirmed the presence of a mesocaval shunt through the territory of the right gonadal vein, draining into the right iliac vein, with no portal inflow into the liver. The patient was re‐operated, and the shunt was ligated. An intraoperative Doppler ultrasound showed adequate portal inflow after the procedure; the patient evolved satisfactorily and was discharged home on day number 49. The aim was to report a case of post‐operative steal syndrome in a pediatric recipient due to a mesocaval shunt not diagnosed during the pretransplant evaluation.     

临时性门腔静脉架桥联合流出道改良重建在部分供肝肝移植中应用的实验研究

目的 通过动物肝移植实验,探索门腔静脉架桥和改良肝静脉出口重建这一联合方法的可行性及价值.方法 选择20～25 kg和10～15 kg健康杂交犬各12只,组成供体组和受体组,并随机配对.供体手术取左外侧叶及左中央叶为供肝,然后完全阻断门静脉.受体犬先预置门腔静脉之间端侧吻合架桥的分流通道,切肝门静脉阻断时开放,供肝植入...     

Risk of gastrointestinal bleeding during adolescence and early adulthood in children with portal vein obstruction

OBJECTIVE: To estimate the risk of bleeding during adolescence and early adulthood in a group of children with portal vein obstruction who had not undergone an effective treatment beforehand. STUDY DESIGN: Children (n = 44) were followed up from age 12 years to a mean age of 20 years (range, 15-34 years). Actuarial risk of bleeding, related to previous occurrence of gastrointestinal bleeding and to pattern of varices at age 12, was calculated yearly. RESULTS: Twenty-four children presented with gastrointestinal bleeding after age 12, and 20 did not bleed. The overall actuarial probability of bleeding was 49% at age 16 and 76% at age 24. Probability of bleeding at age 23 was higher in children who had bled before age 12 than in children who had not bled (93% vs 56%; P =.007). Probabilities of bleeding at age 18 and at age 23 were 60% and 85%, respectively, in patients who had grade II or III esophageal varices at age 12. The 9 children without varices or with grade I varices only on endoscopy did not bleed between the ages of 12 and 20 years. CONCLUSIONS: Children with portal vein obstruction have a >50% risk of bleeding during adolescence; the pattern of varices on endoscopy at age 12 may have a prognostic value.