计算机辅助手术系统在小儿巨大肝间叶性错构瘤诊治中的应用

目的探讨计算机辅助手术系统(海信CAS)在小儿巨大肝间叶性错构瘤(HMH)诊治中的临床价值。方法自2010年9月至2015年10月,我们收治5例HMH患儿,术前均行上腹部增强CT检查,3例(2013年7月至2015年10月)应用海信CAS对患儿CT资料行三维重建及模拟肝切除,制定手术计划,术中实施精准肝切除术。结果 2例(2010年9月至2013年5月)根据术前CT检查分别实施肝右叶切除术及肝左叶切除术。3例(2013年7月至2015年10月)应用海信CAS系统成功进行肝脏及肿瘤的三维重建,并进行模拟肝切除,根据术前制定的手术计划分别成功实施:肝Ⅱ、Ⅲ、Ⅴ、Ⅷ部分及Ⅳ全部切除术,肝Ⅴ段切除术,右半肝切除术。术后恢复好,病理检查证实为HMH。随访3个月至5年未见并发症及复发。结论 HMH影像学检查与肝囊肿相似,易误诊。海信CAS能清晰、直观显示HMH的位置、形态及其与肝内管道系统的关系,其术前规划功能利于术者制定最佳手术方案,实施个体化精准肝脏肿瘤切除。     

数字化医疗技术在小儿外科的应用展望

在21世纪短短的十几年时间里,数字医学已经逐渐广泛地应用于临床医学的各个方面,包括疾病诊断、术前评估、治疗方案、随访等.数字化医疗技术作为一种全新的疾病诊疗技术,让医师及患者更全面的了解疾病的发生发展,通过三维重建、模拟手术等方式改变传统的疾病诊疗模式,让高难度、高风险的手术能够更安全地开展,指引着当代医学向一个新的方向发展.目前总的来说,数字医学的基础研究、临床应用,发达国家早于且强于国内,成人医学早于且强于儿科,在小儿外科中,以骨科、神经外科的发展、应用较早.本文将对数字化医疗技术的概念、基础与临床研究的现状,及其在小儿外科的应用现状进行总结,并展望其在小儿外科的应用前景.     

小儿门静脉高压治疗新进展

门静脉高压(portal hypertension,PH)是各种原因导致门静脉系统压力升高引起的一组临床综合征,是儿童发病率较低的严重并发症.小儿门静脉高压症的病因和治疗有别于成人.本文综合近五年小儿PH治疗的文章,总结历年国际及国内范围对该病的病案报道,分别从药物、内镜、介入、手术、肝移植等方面做了详细的描述,理清了门静脉高压治疗的最新进展,有助于提高各种病因门静脉高压患儿的治疗效果.     

腹腔镜脾切除联合胃食管周围血管离断术治疗小儿门静脉高压症

目的 报告内结扎法腹腔镜脾切除联合胃食管周围血管离断术治疗小儿门静脉高压症合并出血和脾功能亢进的经验及效果.方法 本组6例,男5例,女1例,年龄8～17岁.采用丝线内结扎法结合超声刀进行腹腔镜脾切除和选择性贲门周围血管离断术,切除巨脾后,离断胃食管周围侧支血管和穿支静脉,保留胃冠状静脉和食管旁静脉向奇静脉的分流.结果 ...     

移植血管间置、门静脉主干-门静脉肝内左支分流术在治疗小儿肝外门静脉高压中的应用

目的 探讨移植血管间置、门静脉主干-肝内门静脉左支分流术在治疗小儿肝外门静脉高压中的效果和预后.方法 回顾性分析2010年1月至2014年12月9例(男6例,女3例)因“呕血、黑便”等上消化道出血表现入院,入院后完善腹部CT、超声及门静脉血管B型超声检查,诊断为门静脉海绵样变、门静脉高压和脾大患儿的临床资料.所有患儿均行移植血管间置、门静脉主干-肝内门静脉左支分流术治疗,术中造影发现胃冠状静脉直径较细或长度不够,不能行胃冠状静脉-门静脉左支分流术治疗,遂采用移植自体血管的方式,将其两端分别与门静脉主干和肝内门静脉左支吻合.其中采用移植空肠静脉方法治疗2例,回肠静脉4例,肠系膜下静脉3例.患儿术后均获随访,随访时间1～60个月,平均24.6个月.随访期间,采用超声评估脾大小及分流血管通畅情况.记录血常规、血生化和血氨,评估脾功能亢进情况和肝功能.结果 所有患儿均成功行移植血管间置、门静脉主干-门静脉肝内左支分流术治疗.手术时间105～360 min,平均218.3 min.其中3例住院期间有输血记录.术后住院时间6～10d,平均7.2d.术后复查B型超声示脾长径为9.6～14.7 cm,厚径为3.4～5.4 cm;较术前(长径10.4～17.6 cm,厚径3.2～6.8 cm)明显降低.血常规、生化和血氨均恢复正常.随访B型超声可见分流血管通畅,血管直径为0.5～0.7 cm,平均0.6 cm;分流血管入肝血流速度为0.14～0.18 m/s,平均0.16 m/s.1例患儿复发,采用保守治疗有效.结论 移植血管间置、门静脉主干-门静脉肝内左支分流术是治疗小儿肝外门静脉高压的有效方式之一,在患儿不能采用胃冠状静脉-门静脉左支分流术治疗时可考虑采用该方法治疗.     

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.     

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.     

Rex手术治疗小儿肝外门静脉高压的应用进展

肝外门静脉梗阻(Extra-hepatic portal venous obstruction,EHPVO)是引起小儿门静脉高压的常见原因之一.研究发现,大约66％～76.5％的小儿门静脉高压是由EHPVO引起的,其中以门静脉海绵样变多见.Rex手术已经成为当前治疗小儿肝外门静脉高压的重要手术方式之一,由于其重建入肝血流,恢复门静脉解剖结构和生理功能的作用,是小儿肝外门静脉高压的根治性手术方法.     

门静脉通畅状态对门静脉血流动力学和门静脉血管病理改变的影响研究

目的探讨门静脉通畅情况对门静脉血流动力学和门静脉血管病理生理的影响。方法选取18只4～5 kg雄性成年新西兰白兔作为研究对象, 采用随机数字表法将其分为3组:一组采用开腹后游离门静脉主干的方法, 为对照(normal control, NC)组;一组采用套管将门静脉主干部分缩窄的方法, 为肝外门静脉梗阻(extra-hepatic portal venous obstruction, EHPVO)组;一组采用在EHPVO模型制备14 d后将套管去除的方法, 为可恢复性肝外门静脉梗阻(reversible extra-hepatic portal venous obstruction, r-EHPVO)组。所有模型于制作第14天和第28天时, 测量门静脉压力、门静脉血流速度和门静脉血管直径。计算门静脉血管剪切力τ=4Q×η/π×r3[其中η为血黏度(Pa·s)、Q为血液流速(mL/s)、r为血管内半径(cm)]和门静脉血管周向应力T=ΔP×r/h[其中r为血管平均半径(cm), ΔP为管壁内外压强差(kPa), h为壁厚(cm)]。模型制作第28天时取门静脉主干血管制作病理切片, 显微镜...     

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.     

Abnormal direct entry of the umbilical vein into the portal vein: report of a case

Abnormalities of the intra-abdominal umbilical venous connections are rare congenital vascular anomalies. We report a case of an aneurismal dilatation of an umbilical vein directly connected to the portal vein, vital for the delivery of placental blood in the fetus. A term female newborn presented with a dilated umbilical vein detected by prenatal ultrasound. CT angiography revealed an aneurismal dilatation of a vascular structure, from the umbilical ring to the main portal vein. Follow-up CT angiography showed thrombus progression up to the level of SMV inlet. A laparotomy revealed that the normal course of the umbilical vein was absent. The aneurysm was resected and no thrombus was noted in the aneurysm. Post-operative ultrasound revealed an isolated thrombus at the right main portal vein that resolved spontaneously on the follow-up ultrasound. At the 6-month post-operative follow-up, the baby was well and without complications. Although not evaluated for the presence of a ductus venosus, this anomalous umbilical-portal venous connection might have been associated with an absent ductus venosus.     

联合肝脏离断和门静脉结扎二步肝切除术在儿童肝肿瘤治疗中的应用

联合肝脏离断和门静脉结扎的二步肝切除(associated liver partition and portal vein ligation for staged hepatectomy,ALPPS)手术方案具有短期内残肝体积迅速增大的特点,在预估残肝体积(future liver remnant,FLR)不足的成人肝肿瘤手术中已获得推广。儿童肝脏肿瘤往往体积相对较大,尤其是肝母细胞瘤常侵犯多个肝段,或占据肝脏中央解剖部位,存在根治性肝切除术导致FLR不足的情况,有实施这一术式的价值。目前ALPPS在儿童肝脏肿瘤中的应用尚处于起步阶段。一般认为,术前评估FLR30%的病例可考虑实施本术式;术前应对患儿肝体积、肝功能以及肿瘤的可切除性进行精准评估;两次手术间隔时间以7~14 d为宜。手术并发症主要包括肝功能不足、出血和胆漏。关于儿童肝肿瘤中该术式的临床疗效评估尚待进一步总结。     

Spontaneous perforation of the biliary tract and portal vein thrombosis in infancy

Spontaneous biliary perforation (SBP) is an uncommon cause of surgical jaundice in the first few weeks of life and is characterised by the occurrence of a punched-out defect in the bile duct, typically where the cystic duct joins the common hepatic duct. In most cases the site occurs anteriorly and bile leaks into the general peritoneal cavity. We now describe two cases of SBP where the perforation occurred posteriorly, limiting the leak and resulting in delayed recognition. Surgical management in both cases consisted of hepaticojejunostomy-en-Roux. Both cases were complicated, although not immediately, by portal vein thrombosis and one by chylous ascites. Posterior SBP, presumably due to their intimate anatomical relation with the portal vein, seem predisposed to such complications.     

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.     

Ultrasonographic assessment of the gallbladder in 21 children with portal vein thrombosis

Background: Portal vein thrombosis (PVT) is one of the most frequent causes of portal hypertension (PH) during childhood. Portal systemic collateral vessels occur at several locations, including the gallbladder (GB). Objective: To evaluate the GB in patients with PVT using US to assess GB wall thickness and its function, and the incidence of lithiasis and varices. Materials and methods: A prospective study was done on 21 children and young adults whose ages ranged from 17 months to 20 years and 10 months (mean age: 11 years and 7 months). A control group was matched for age and sex. All of the patients and controls fasted for at least 6 h prior to the US examination. The GB measurements included anterior wall thickness. These measurements were obtained before the ingestion and then 30 and 60 min after the ingestion of a meal containing at least 25 g of fat. The rate of GB contractility was calculated based on these results. The presence of varices in the GB wall was detected by the characteristic serpentine shape of the intramural vessels and by the venous flow using pulse duplex and color Doppler imaging. The presence of biliary lithiasis was confirmed by shadowing. The chi-square test, the exact Fisher test and the Mann–Whitney test were used to compare the results. Results: Biliary lithiasis occurred in 3 (14.2%) of the 21 patients. The GB wall was thickened in 13 (61.9%) of the 21 patients, which corresponded with the number of patients with GB varices. The wall dimensions of all the controls were within normal limits. In patients with PVT; GB contractility was lower than in the 21 patients used as control and resulted in a significant difference in all of the measurements. Conclusion: GB varices are very common in children with PVT, and it is noted especially in patients whose GB wall was thickened and in whom the GB contractility was reduced. Lithiasis could be a consequence of the decreased contractility of GB.     

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。     

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。     

Transileocolic venous balloon dilatation for the management of primary and recurrent portal venous stenosis after living donor liver transplantation in children

Portal venous stenosis is relatively a rare complication after liver transplantation in children and it sometimes leads to life threatening event due to gastrointestinal bleeding or graft failure. Recently, balloon dilatation has been widely accepted as a treatment of choice for the management of portal venous stenosis. The purpose of this study was to evaluate the feasibility of transileocolic venous balloon dilatation for the management of primary and recurrent portal venous stenosis after living donor liver transplantation (LDLT) in children. The records of 57 pediatric liver transplants were retrospectively reviewed. Nine patients (15.8%) with portal venous stenosis were identified. Seven symptomatic children with portal venous stenosis underwent balloon dilatation. Two approaches were employed for balloon dilatation; the transileocolic venous approach and the percutaneous transhepatic approach. In patients with recurrent stenosis, careful follow-up was carried out while they were asymptomatic. Twelve balloon dilatations were performed in seven children with primary or recurrent portal venous stenoses. The initial technical success rate was 91.7% (11/12), while 6 out of 12 (50.0%) procedures resulted in recurrent stenosis. Five out of six recurrent stenoses required repeated balloon dilatation. The clinical success rate of balloon dilatation in our study was 85.7% (6/7). Other than recurrent stenosis, two procedure-related complications occurred. In conclusion, transileocolic venous balloon dilatation was a safe and effective procedure for portal venous stenosis after LDLT in children.