累及肝门部的小儿巨大肝脏肿瘤手术

小儿肝脏肿瘤并非罕见,其中以恶性肝母细胞瘤和肝细胞癌占多数.由于小儿特殊的肿瘤生物学特性,多分化低、增生快,至患儿出现明显症状而就诊时,常已发展为巨大的肝脏肿瘤,病变累及半肝以上,给手术治疗带来极大难度.     

不阻断肝门小儿巨大肝肿瘤切除术探讨

目的小儿巨大肝肿瘤切除术中，常规行肝门阻断，本研究对不阻断肝门行巨大肝肿瘤切除术进行探讨。方法从2001年7月至2004年12月，我院对16例患儿施行不阻断肝门巨大肝肿瘤切除术，患儿年龄28d～14岁，平均3．4岁。其中男12例，女4例，肿瘤直径11～23cm，平均14．6cm，其中右半肝和左外叶内分别有一肿块1例。肝母细胞瘤9例，肝错构瘤4例，肝血管瘤3例。结果本组16例患儿手术全部成功，不阻断肝门行右半肝（Ⅴ～Ⅷ）切除8例，右三叶切除4例，左三叶切除1例，左半肝切除1例，第二肝门及右下叶肝段切除各1例。患儿术中无死亡，血流动力学指标稳定。本组良性巨大肝肿瘤患儿术后随访7个月～3．5年，目前均健康生存，无肿瘤复发，生长发育正常；肝母细胞瘤9例患儿术后常规化疗，患儿已经随访6～45个月，目前无瘤生存8例，2年以上4例，肝功能正常，正常生活。另外1例左外叶和右叶同时发现肝母细胞瘤，手术分别切除后5个月脑肺转移，于术后7个月死亡。结论不阻断肝门巨大肝肿瘤切除术，是一种安全可行的手术。术者熟练的肝脏解剖知识和肝切除技术、紧密结合术前和术中影像学技术了解肿瘤与大血管关系、彻底结扎肿瘤侧入肝肝动脉和门静脉及肝左右静脉共干或肝右静脉，是手术成功的关键。     

计算机手术模拟系统指导累及肝门部巨大肿瘤的精准肝脏肿瘤切除手术

小儿肝脏肿瘤瘤体巨大,病理类型多,部位复杂,累及肝脏体积较大,且多累及肝门部或肝门部淋巴结、门静脉等,给手术带来了挑战.当肿瘤累及肝门部,尤其是累及多个部位时,术中易并发难以控制的大出血,在过去被认为是手术禁区.随着医学影像技术的发展,这类肿瘤从不可切除向可切除转变.     

不阻断肝门肝切除在小儿肝脏肿瘤切除术中的应用

小儿肝脏肿瘤主要以肝母细胞瘤、肝血管瘤、肝错构瘤多见。肝叶切除目前仍是肝脏原发和继发性肿瘤治疗的最佳治疗手段。常规阻断肝门会造成肝脏缺血、回心血量下降，影响肝脏功能恢复和引起血液动力学改变。我们自2001年1月至2007年5月采用不阻断肝门规则性肝切除技术治疗小儿肝脏肿瘤37例，手术肿瘤完整切除率100％，手术期间的死亡率为零，术后无肝功能衰竭，现总结探讨不阻断肝门肝切除的应用。     

累及第二、三肝门巨大肝母细胞瘤手术切除41例

目的：探讨累及第二、三肝门的巨大肝母细胞瘤手术切除的可行性及手术方法,以期扩大手术适应证,提高小儿肝母细胞瘤的治疗效果。方法我们对41例累及第二、三肝门的巨大肝母细胞瘤施行肿瘤切除手术,患儿年龄3个月至14岁,平均年龄3.8岁;其中男25例,女16例;肿瘤直径8.5～21.6 cm,平均13.5 cm;均行手术前化疗,化疗3至7个疗程不等,直至肿瘤不再缩小。采用Kap-lan-Meier生存分析计算本组患儿生存率。结果41例均顺利实施肝脏肿瘤切除术,手术时间125～350 min（平均220 min）,术中出血40～500 mL,8例未输血。第一肝门阻断时间15～35 min （平均22 min）,其中阻断1次者12例,2次者23例,2次以上者6例,每次阻断时间一般不超过20 min。手术切除方式包括V-Ⅷ段切除8例;Ⅳ-Ⅷ段切除9例;Ⅰ-Ⅳ段切除4例;Ⅰ、Ⅳ、Ⅷ段切除9例;Ⅰ、Ⅳ、Ⅴ、Ⅷ段切除6例;Ⅰ段＋Ⅴ-Ⅷ段切除2例;Ⅰ段＋Ⅳ-Ⅷ段切除3例;合并下腔静脉瘤栓7例。41例患儿1年、3年、5年无瘤生存率分别为92.7％、72.8％和33.5％。其中最长无瘤生存期为90个月。41例术后均经规范化疗,化疗后定期门诊复查,其中20例（48.8％）复发,继续化疗。5例出现肺转移病例中,2例经术后化疗转移灶消失,3例于术后1年内死亡。结论累及第二、三肝门的巨大肝母细胞瘤患儿手术切除虽然具有一定挑战性,但在详细、准确的术前评估,及对肝脏解剖的熟练掌握下,肿瘤切除仍具有一定的可行性和安全性。完整切除肿瘤可有效提高肝母细胞瘤的长期生存率。     

小儿肝门部肝母细胞瘤的手术策略

目的探讨侵犯肝门血管的小儿肝母细胞瘤的手术策略。方法回顾性分析2006年6月至2010年6月作者收治的68例肝门部肝母细胞瘤患儿的临床资料,均使用微波切割技术,在不阻断肝门血流的状态下精细解剖分离,在发生血管损伤出血时则暂时性阻断肝门血管,如影响显露,则分块切除肿瘤。结果68例患儿均接受术前化疗,其中侵犯第一肝门17例,侵犯第二肝门51例。15例患儿第一肝门阻断时间在5min以内,2例患儿全肝血流阻断时间为5min。术中平均失血量25mL,6例因失血较多予输血治疗。无手术死亡病例。并发胆瘘3例,肝周积液5例,乳糜腹1例。结论对于肝门部肝母细胞瘤,采取术前化疗可降低手术难度,精细解剖是基本措施,分块切除是可选方法,肝门血管阻断无法保障安全,不损伤肝门血管是患儿生命安全的重要保障。     

小儿巨大肝脏肿瘤的诊断和处理

介绍５例小儿罕见肝脏巨大肿瘤的临床特点，Ｂ超、ＣＴ、选择性动脉造影的改变。提出术前术中诊断的注意点。术中根据肿瘤大小、部位以及与下腔静脉、肝静脉的关系选择恰当的术式。认为全肝血流阻断低温灌注对完成巨大的肝肿瘤切除是一种较好的方法。     

小儿肝肿瘤的诊断与外科治疗

近１０多年来由于影像学检查手段的进步,小儿肝肿瘤确诊日渐增多。为提高小儿肝肿瘤的诊断与治疗水平,我们总结了１９８４～１９９７年收治的２６例小儿肝肿瘤资料,现报告如下。临床资料性别与年龄：本组男１８例,女８例,男∶女为２．２５∶１,年龄最小５４天,最大８岁,平均年龄３．５岁。诊断：患儿多因上腹部不适,隐痛,在Ｂ超体检时无意发现,或以上腹部逐渐膨胀及上腹包块就诊,２例患儿伴有消瘦、食少、发热。肝良性肿瘤患儿ＡＦＰ测定值正常,６例肝恶性肿瘤患儿ＡＦＰ均高于正常值２０μｇ／Ｌ,定位诊断依靠Ｂ超及ＣＴ扫描…     

常温下长时间阻断入肝血供和选择性阻断肝静脉在小儿肝脏肿瘤切除术中的应用

小儿肝脏肿瘤切除术中的主要危险是大出血，笔者根据小儿肝脏解剖和生理特点，采用长时间阻断入肝血供及选择性阻断肝静脉行肝脏肿瘤切除术１５例。该方法便于操作，术中出血量明显减少，术后患儿恢复顺利，无并发症。临床观察小儿肝脏温缺血时限可达５２分钟。     

Bile-duct diameter at the porta hepatis and liver biopsies in Indian children with biliary atresia

Fibrous remnants from the porta hepatis and wedge biopsies of the liver were studied in 60 patients with biliary atresia. The patients were divided into three groups on the basis of bile duct diameter — I: no ducts; II: <50 m; III: >50 m. The liver damage was classified into three grades on the basis of ductular and parenchymal histopathologic changes and extent of inflammatory pathology. There was no correlation between severity of hepatic damage and duct diameter, although the levels of serum bilirubin and alkaline phosphatase varied in direct proportion to the degree of hepatic damage. There was an inverse relationship between age and duct diameter, but neither correlated with bile flow at surgery or in the postoperative period. The data suggest that Indian children with biliary atresia follow a different clinical course compared to those from Japan and Western countries. Correspondence to: V. Bhatnagar     

Ⅲ、Ⅵ期小儿肝母细胞瘤术中肝内血管的处理

目的 探讨小儿肝母细胞瘤累及肝内重要血管术中处理方法及其疗效.方法 对2005年4月至2008年8月25例小儿肝母细胞瘤切除术中对累及肝内重要血管的具体处理方法进行分析,按术中受累血管处理不同分为2组,血管结扎组10例、血管修补组15例,血管结扎组:累及的肝内重要血管连同瘤体、受累肝叶/肝实质一并结扎切除;血管修补组:对受累肝内重要血管分离,尽可能保留受累肝内重要血管及所支配之正常肝叶/实质.结合术后随访情况分析疗效.结果 血管结扎组中放弃手术1例,姑息性切除3例,完整切除6例,术后肝功能衰竭死亡2例,痊愈出院4例;血管修补组均完整切除痊愈出院.随访5～36个月(中位随访时间20个月),血管结扎组生存1例,血管修补组生存11例.结论 辅以血管修补的肿块切除术可扩大肝母细胞瘤手术适应证,有效预防术后肝功能衰竭,安全度过围手术期,为早期化疗提供时机.     

Imaging of liver tumours in childhood

Primary liver tumours account for 6% of all paediatric neoplasms. In a child with a clinical abdominal mass, imaging (in consultation with a paediatric surgeon) aims to confirm the intrahepatic site, determine its likely resectability, exclude metastatic abdominal disease, and characterise the mass. The imaging in 44 patients with primary liver tumour over a 33-year period was reviewed and correlated with surgical/pathological findings. Characterising hepatic masses with ultrasound, computed tomography, nuclear medicine, and angiography is less important than determining its resectability and alerting the surgeon to vascular anomalies and the presence of metastatic disease. We conclude that a chest X-ray and ultrasound study are the primary methods for evaluation of a child with suspected hepatic mass. With careful attention to technique, the mass can be evaluated and an assessment made of tumour resectability preoperatively. Based on this review, we propose a schema for the initial evaluation of suspected hepatic masses in children. Offprint requests to: J. F. de Ocampo     

Endoscopic findings at porta hepatis and clinical outcome after portoenterostomy in biliary atresia

Postoperative endoscopic examination of the porta hepatis through an exteriorized enterostomy was undertaken in 19 children with biliary atresia (BA). Endoscopic findings were classified into three types: (1) ductal (D) type with distinct bile-duct orifice and good bile drainage; (2) oozing (O) type without distinct bile-duct orifice but good bile flow; and (3) covered (C) type with no bile-duct orifice and no bile discharge. The postoperative course of the D-type patients was excellent with immediate disappearance of jaundice and good liver function. In the O type, all but 1 patient became jaundice-free, but the duration of disappearance of jaundice after the operation was significantly longer than for the D type (P <0.05). All of the patients with type C died within 3 years after portoenterostomy. Histologic studies of the extrahepatic biliary remnant showed no significant difference in the area of the largest bile-duct lumen or the total area of all bile duct openings among the three types. The age at operation was youngest in type D, and there was a significant difference between types D and C (P <0.05). Endoscopic examination of the porta hepatis reaffirmed the importance of early diagnosis and early operation in the treatment of BA. Type D may have an excellent prognosis while type C may require liver transplantation. Correspondence to: S. Matsuo at his present address     

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。     

Anatomy of the porta hepatis (PH) as rational basis for the hepatoporto-enterostomy (HPE).

PURPOSE: In a prospective study it was investigated whether and to what degree the morphology of the porta hepatis has a bearing on the early prognosis of children with EHBA. The results will point to consequences of surgery and to the formulation of a rational understanding of the successes and failures of HPE. METHODS: The macroscopy and the microscopy of the liver was studied on native specimen and corrosion preparations of deceased adults, children and embryos. The area potentially draining the bile was determined in excised PH specimen of children with EHBA. The histologically and planimetrically measured areas were correlated to clinical follow-up studies. In order to be able to study exclusively the influence of the PH morphology, the conditions of the intrahepatical structural changes (level of fibrosis, metric and morphic changes of biliary ductules in the Glisson's triads) had to be equal. Under these conditions 61 excised PH specimen were evaluated. The level of fibrosis was determined according to the definition of Schweizer/Müller 1984 (9). RESULTS: 1) Biliary ductules of the PH end in three definable zones according to a regular pattern. 2) Biliary ductules of the PH run in a narrow and immediate relation to the vascular structures. 3) Biliary ductules of the PH are often hidden between the vascular structures of the PH and are not always accessible to preparatory exposure for the anastomosis. 4) The quantity of the bile flow is a significant function of the total area of the biliary ductules secured in the excised PH specimen. 5) Incised biliary ductules at the excision edge may be obstructed again in the scarring of the anastomosis, leading to an early interruption of bile flow. 6) The opening-up of the biliary ductules in the central zone alone is not enough to guarantee a permanent bile flow. Only if intact biliary ductules of the two lateral hepatical lobes are secured, can a positive prognosis be made.     

新生儿含肝组织的巨型脐膨出的临床治疗抉择探讨

目的:回顾分析含有肝脏组织的巨型脐膨出(GO)的临床特征,探讨最佳治疗方式,改善预后,提高GO患儿救治率。方法:收集2009年1月至2019年5月在武汉儿童医院新生儿外科住院的GO患儿,治疗方法包括：1.使用类似Silo袋的方法,分期手术;2.一期直接或联合补片修补;3.保守治疗2～8周后延期一期手术。收集的数据包括患...