经脐单切口腹腔镜肝管空肠Roux-en-Y吻合术治疗儿童胆总管囊肿的探讨

目的 评价经脐单切口腹腔镜治疗胆总管囊肿患儿的安全性和有效性.方法 回顾性分析2011年4月至7月间在我中心经脐单切口腹腔镜技术治疗的19例胆总管囊肿患儿预后和短期随访结果,并与我中心以往传统4孔腹腔镜手术治疗胆总管囊肿的疗效对比.结果 中位随访时间为3个月.其中2例患儿巾转为传统4孔腹腔镜手术,其余17例患儿成功完成经脐单切口腹腔镜下胆总管囊肿根治手术(平均手术年龄:3岁,男/女:5/12).早期单切口手术中1例患儿术后出现胆漏,腹腔引流l0d后自愈.单切口组的平均手术时间3.06 h与我院传统腹腔镜胆总管囊肿根治术时间3.04 h相比,差异无统计学意义(P=0.9t09).两组的术后平均住院时间(6.23 d比7.41d)、术后恢复进食时间(2.64d比2.86d)、腹腔引流留置时间(3.23d比3.05d)亦无明显差异(P=0.056、0.472、0.619).结论 经脐单切口腹腔镜应用传统腹腔镜器械治疗胆总管囊肿是安全的,短期随访结果与传统腹腔镜疗效相仿,为胆总管囊肿外科治疗提供了新的途径.     

伴有并发症的儿童胆总管囊肿的临床处理

目的 讨论合伴有各类并发症的儿童胆总管囊肿的临床处理及手术时机和方法.方法 回顾性分析2013年1月至2015年12月上海新华医院小儿外科收治的45例合伴有各类并发症的儿童胆总管囊肿患儿的临床资料.其中,胆道穿孔7例,胆源性胰腺炎9例,胆管炎、阻塞性黄疸、肝功能受损29例,保守治疗效果不佳.7例胆道穿孔中,5例胆汁性腹膜炎行囊肿外引流和二期根治术;2例隐匿性胆道穿孔一期行根治术.9例胆源性胰腺炎给予内镜下鼻胆管引流,待淀粉酶正常后一期行根治术.29例阻塞性黄疸伴肝功能受损的患儿中,17例发病年龄小于3个月行一期根治手术;12例发病年龄大于6个月患儿先行ERCP置鼻胆管引流,待黄疸消退、转氨酶正常后一期行根治性手术.结果 伴胆道穿孔的7例胆总管囊肿患儿中,2例在外引流期间出现水电解质紊乱,1例T管脱落,1例隐匿性穿孔在根治术后出血再次手术,余者术后均痊愈出院.9例胆源性胰腺炎患儿置鼻胆管引流后淀粉酶均恢复正常,根治术后均痊愈出院.并发急性胆管炎、伴有梗阻性黄疸、肝功能受损29例中,12例发病年龄大于6个月,其中11例行内镜下置鼻胆管引流后并发症改善行根治手术,1例ERCP失败后改行外引流和二期根治术;小于3月龄婴儿直接行一期根治性手术,术后均痊愈出院.所有患儿保持随访,术后随访时间1～3年.1例术后慢性胰腺炎史,1例胆管炎史,均通过药物治疗缓解症状.结论 对合伴有各类并发症的儿童胆总管囊肿选择合理的处理手段和合适的手术方式将有效减少并发症所造成的危害.     

紧贴囊壁剥离法切除胆总管囊肿的临床评价

目的：对紧贴囊壁剥离法（或称疝囊剥离法）切除胆总管囊肿的安全性及可行性进行评估。方法：回顾1984－1999年的胆总管囊肿113例，其中106例行囊肿切除、胆肠Roux-en-Y吻合术，均是采用敞开囊腔紧贴囊壁剥离法切除囊肿。结果：106例手术均顺利，无意外损伤，失血量、输血量绝大多数在200ml以内，3例未输血，无严重并发症及死亡.结论：敞开囊腔紧贴囊壁剥离法简化了手术操作和降低了手术难度，增加了手术安全性，因而被认为是切除胆总管囊肿的安全、有效、易掌握的方法。     

胆总管囊肿根治术后胆道梗阻的原因分析和治疗策略

目的 本研究旨在分析胆总管囊肿根治术后胆道梗阻的原因, 提出相应的治疗策略.方法 回顾2006年1月至2016年1月间47例在外院接受胆总管囊肿根治术后胆道梗阻,转入我院成功行腹腔镜二次手术的患儿,对其病因进行观察,分析及治疗.结果 出现胆道梗阻症状,即反复胆管炎或持续肝功能异常距离第一次手术的时间为术后2周~10.8年(平均1.7年).在外院接受抗炎保肝利胆PTD治疗时间2周~10年(平均2.7年),未见明显好转.接受二次手术的间隔时间为1个月~11年(平均4.5年).病理显示57.4% (27/47)患儿存在I~IV级肝纤维化.本研究发现术后胆道梗阻的原因如下:①异位肝右动脉前置压迫肝管空肠吻合口近端导致梗阻占27.6% (13/47).术中将异位肝右动脉重置于肝管空肠吻合口后方解除梗阻;②初次手术未解决伴发的单一或多处肝管狭窄占29.8% (14/47).二次手术中行肝管扩大成型+肝管空肠再吻合;③吻合口狭窄占42.6% (20/47).二次手术中行肝管空肠再吻合.中位随访时间为48个月(1~120个月),无1例发生胆道再梗阻或胆管炎.肝功能于二次术后1个月~2年恢复正常.结论 异位前置肝右动脉压迫、合并单一或多处肝管狭窄、吻合技术是胆总管囊肿术后胆道梗阻的原因.应尽早外科手术解除梗阻避免肝功能损伤及肝硬化.     

Choledochal cysts: a nine-year review

Hospital records of 24 Chinese infants and children (7 males and 17 females) with choledochal cysts were reviewed. The patients were aged 1 day to 17 years. Abdominal pain, palpable abdominal mass and jaundice, either alone or in combination, accounted for most of the initial presentations. Twenty-two patients were classified as type I with surgery, while the other two patients were type V without surgery. Ultrasonography is a rapid and accurate diagnostic method in the initial evaluation. Cyst excision wth Roux-en-Y hepaticojejunostomy was the treatment of choice in this series. Chronic cholecystitis with or without inflammation of choledochal cyst was the most common pathological finding. Five patients underwent liver biopsies, showing four biliary cirrhosis and one portal fibrosis. So far, there is no evidence of malignancy in any patient.     

Contemporary surgical treatment for choledochal cysts

Congenital choledochal cyst is an uncommon anomaly of the common bile duct, and the pathogenesis is uncertain. Although various surgical approaches have been used, cyst excision with a Roux-en-Y anastomosis has proved to be the most successful. A total of 21 patients (17F:4M) have been treated by this method with minimal surgical morbidity at the Royal Children's Hospital, Melbourne. Diagnosis proved difficult in some cases, and in 2 patients was delayed for some years because of misinterpretation of radiological and pathological findings. Offprint requests to: A. W. Auldist     

Experience with choledochal cysts in infants

The purpose of this analysis is to study the clinical features, liver histology and the outcome after surgery in infants with choledochal cysts (CDCs), and to draw conclusions on the management strategy accordingly. The clinical features, liver histology and the outcome after surgery in 14 cases of CDCs treated before 1 year of age (the “infantile group”) were compared with 52 cases that were diagnosed and treated between 1 and 12 years of age (the “classical pediatric group”). The differentiation of the “infantile choledochal cysts” from “biliary atresia with cystic dilatation” was based on the configuration of intrahepatic bile ducts on cholangiogram. Jaundice was the universal presenting feature in 14 patients in the “infantile group”, the clinical presentation in 6 infants was indistinguishable from biliary atresia. On the other hand, pancreatitis as a presenting complication was seen exclusively in those presenting later, i.e. the “classical pediatric group”. Fibrosis, as early as 4 weeks of age, was seen in all nine (100%) infants in whom a liver histology was available. Seven of these nine infants had bridging fibrosis (n = 4) or cirrhosis (n = 3) on liver histology. The outcome was satisfactory in 9 of the 14 (64.3%) infants despite bridging fibrosis (n = 4) or cirrhosis (n = 1) in 5 of these 9 babies. The CDCs presenting in infancy differ from the classical variety of later presenting pediatric CDCs in terms of their presentation, liver histology and outcome. They merit an emergent surgical treatment following which the outcome is satisfactory. Delay results in progression to end stage liver disease.     

Ultrasonographic spectrum of choledochal cysts in children

Four different sonographic patterns which may be encountered in choledochal cyst are described. The most common form is concentric dilatation of the common bile duct (Type I). Rarely eccentric dilatation of the common bile duct, diverticulum, may be seen (Type II). Both forms of the disease may (Types IB and IIB) or may not (Types IA and IIA) be associated with intrahepatic biliary dilatation.     

基质胶原酶、明胶酶在先天性胆总管囊肿壁的表达及意义

目的观察先天性胆总管囊肿壁基质胶原酶（MMP-1）、明胶酶（MMP-2）蛋白的表达，探讨其与先天性胆总管囊肿发病的关系。方法收集53例先天性胆总管囊肿患儿临床资料以及囊肿壁和胆囊组织标本，其中男21例，女32例，年龄4个月～17岁，平均年龄6．5岁，另取正常胆囊组织28例作为对照组。免疫组化方法检测MMP-1、MMP-2蛋白表达，图像分析技术进行定量分析。结果MMP-1、MMP-2蛋白表达于胆总管囊肿壁黏膜上皮细胞、基质细胞及炎性细胞胞浆、胞膜；胆总管囊肿胆囊黏膜上皮细胞内也见不同程度表达。正常对照组胆囊表达阴性或弱阳性。定量分析显示胆总管囊肿壁MMp-1、MMP-2蛋白表达强度明显高于胆总管囊肿胆囊及对照组胆囊（P〈0．01）；胆总管囊肿胆囊与对照组胆囊组织间差异无显著性意义（P〉0．05）。结论胆总管囊肿MMP-1、MMP-2的表达高于其胆囊组织和对照组胆囊；MMP-1及MMP-2的高表达可能参与了先天性胆总管囊肿的形成过程。     

Laparoscopic excision of choledochal cysts in children: an intermediate-term report

Purpose  To assess the intermediate-term result of the use of the minimally invasive technique in the treatment of choledochal cysts in children. Methods  This is a retrospective review of all cases of choledochal cyst treated in the tertiary referral centre. The surgical technique is described and all the medical records were reviewed to assess the intraoperative and postoperative complications and follow-up problems. Results  From October 2000 to April 2008, laparoscopic excision and reconstruction were attempted in 37 patients. Laparoscopic surgery was successfully performed in 34. Conversion was required in three patients due to oozing on cyst dissection (two) and bleeding from small bowel mesentry (one) during the early part of the series. Postoperative complications were observed in 15 patients including subhepatic collection (6), bile leakage (3), minor wound infection (4) and prolonged ileus (2). At a mean follow-up of 4.2 years, four patients developed surgical complications including intrahepatic ductal strictures in a type IV cyst (one), stump pancreatitis (one), terminal ileal obstruction from internal herniation (one) and cholangitis (one). The cosmetic result was good in all patients. Conclusion  We conclude that laparoscopic excision and reconstruction can be safely performed in children with a choledochal cyst with satisfactory intermediate-term results.     

Rapid enlargement of a choledochal cyst: antenatal diagnosis and delayed primary excision

A case of choledochal cyst (CC) antenatally diagnosed at 29 weeks' gestation is reported. Rapid enlargement of the cyst soon after delivery resulted in complete gastric outlet obstruction (GOO). The lesion was treated by external drainage as a temporary maneuver, with delayed cyst excision and hepaticoduodenostomy at the hepatic hilum performed at 81 days of age. Surgical treatment of CC in early infancy has been reported to be safe and effective. However, delayed primary excision would be an alternative procedure, especially in rare cases showing rapid enlargement resulting in GOO, since this choice has the potential advantage of allowing weight gain and improved nutritional status without risking interim complications due to the drainage procedure. Accepted: 24 March 1997     

Laparoscopic management of pediatric choledochal cysts in developing countries: review of ten cases

We report laparoscopic management of choledochal cysts (CDC) in 10 children. We dissect the CDC using conventional mono- and bi-polar diathermy up to the lower end, ligate or clip it at the lowest possible level and divide it. The proximal end is divided after leaving a sufficient cuff for anastomosis. In the first three cases, we formally opened to complete the biliary-enteric anastomosis. However, in the subsequent seven cases, we made a small midline incision to develop a Roux-en Y loop, and the anastomosis was then completed by intra-corporeal suturing after re-creating the pneumo-peritoneum. Apart from biliary leak in one case, we did not encounter any major complications. The mean operative time was 4.2 h. We have discussed the technical points in the study. We conclude that CDC is eminently suitable for laparoscopic correction; it requires advanced skills and expertise for precise dissection and meticulous suturing in restricted spaces. If the case selection is good and if the team is experienced, CDC can be effectively managed using laparoscopy even without a sophisticated equipment. The wound- and scar-related morbidity is minimized.     

da Vinic机器人腹腔镜治疗小儿先天性胆总管囊肿

目的总结da Vinci机器人腹腔镜治疗小儿先天性胆总管囊肿的近期疗效和经验。方法 2015—2016年我们实施3例da Vinci机器人腹腔镜先天性胆总管囊肿根治术。患儿年龄分别为8个月、8岁和4岁。3例均为Ⅰa型囊肿,其中1例为巨大囊肿。3例均实施机器人囊肿完整切除+肝总管空肠.Roux-en-Y吻合术,于腹腔外完成Roux空肠端侧吻合。结果机器人操作时间分别为304min、226 min和245 min。术后开始进食时间为3 d、3 d和4 d。术后住院时间分别为7 d、9 d和10 d。随访时间分别为10个月、8个月和3个月,无术中、术后并发症。结论 da Vinci机器人治疗Ⅰa型先天性胆总管囊肿是安全、可行的。三维显像系统和灵活的机器手更加有利于囊肿切除和胆肠重建。     

Late follow-up following internal drainage of choledochal cysts reassessment of necessity for reoperation

To evaluate late results in children who had been treated with an internal drainage procedure for a choledochal cysts, we carried out follow-up examinations and performed endoscopic retrograde cholangiopancreatography (ERCP). Internal drainage procedures were performed on a total of 17 patients prior to 1972. The follow-up period ranged from 18 to 30 years. ERCP was performed on 8 of 10 patients followed up. All had dilatation of the common bile duct; 7 had an anomalous pancreatobiliary junction; and there were no carcinomas detected. The procedures consisted of choledochocystoduodenostomy (CCD) in 12 patients, choledochocystojejunostomy (Roux-Y) (CCJRY) in 4, and cholecystoduodenostomy (CYD) in 1. Of the 12 patients who were treated with CCD, 3 were lost to follow-up, 3 died of liver cirrhosis, and 6 survived. Five of the 6 surviving patients needed a reoperation for cholangitis; in 1 of these severe atypical abnormalities of the cyst were found on pathologic examination. Of the 4 patients who were treated with CCJRY, 1 died of liver cirrhosis but the 3 survivors had an asymptomatic postoperative course common bile duct and an anomalous pancreatobiliary junction noted during the current work-up. The patient who was treated with CYD needed another operation for choledocholithiasis. Our conclusions for following patients who were treated with an internal drainage procedure in childhood are twofold: (1) for patients who had CCD, cyst excision is recommended; and (2) for patients who had CCJRY performed at the age of 10 years or less, cautious observation is recommended as long as the patient remains asymptomatic. Offprint requests to: Masafumi Naito     

Choledochal cysts: A clinical and radiological evaluation of 21 cases

Choledochal cysts are predominantly a disease of children, the majority of which are female. The classical symptoms usually form part of a triad of jaundice, abdominal pain and a mass. The radiological features may be non specific and specific. Our experience of 21 cases with particular emphasis on the specific features which outline the cyst are presented.     

Magnetic resonance cholangiopancreatography on postoperative work-up in children with choledochal cysts

This study aimed to assess the clinical usefulness of magnetic resonance cholangiopancreatography (MRCP) as a postoperative diagnostic tool in children with choledochal cysts. Magnetic resonance cholangiopancreatography was performed postoperatively in 19 patients and image quality was compared with that obtained by intravenous cholangiography spiral computed tomography (IVC-SCT). While the detectability by MRCP was inferior to that by IVC-SCT, MRCP highly (84.2%) depicted the anastomotic site together with the reconstructed bowel and intrahepatic bile ducts. Magnetic resonance cholangiopancreatography also clearly delineated the postoperative condition of pancreaticobiliary maljunction (PBM), residual distal common bile duct, common channel, and pancreatic duct. Since MRCP is noninvasive and requires neither radiation exposure nor a contrast agent, and is useful for detecting both anastomosis and pancreatico-biliary ducts around PBM, MRCP might be superior to IVC-SCT as an imaging technique for outpatient clinics performing long-term follow-up studies in children with choledochal cysts.     

影响胆总管囊肿手术疗效的因素分析

目的探讨手术方式、年龄及囊肿扩张形状与胆总管囊肿手术疗效的关系。方法先天性胆总管囊肿患儿83例（男23例,女60例,平均年龄3．8岁）,采用囊肿切除、肝管空肠Roux-Y吻合术治疗,其中腹腔镜下手术59例,开腹手术24例。对83例患儿手术前后血生化指标进行分析,包括总胆红素（TBIL）、直接胆红素（DBIL）、AST、ALT、GGT和ALP。根据手术年龄将患儿分为三组：〈1岁组,1～3岁组和〉3岁组。根据囊肿扩张形状分为囊形扩张组和梭形扩张组。分析手术方式、手术年龄及囊肿扩张形状与手术前后血生化变化的关系,探讨手术方式、手术年龄及囊肿扩张形状与手术疗效的关系。结果所有患儿术后血TBIL、DBIL和ALP水平较术前显著降低;腹腔镜手术与开放手术比较,术后各生化指标降低程度无显著差别。手术年龄越小,术前血胆红素水平越高,肝功能指标越高。3岁以内手术者术后TBIL和DBIL降低程度显著高于手术年龄〉3岁者。、囊形扩张组和梭形扩张组比较,术后肝功能降低程度无显著差别;囊形扩张组术后TBIL、DBIL和ALP较术前显著降低。结论腹腔镜胆总管囊肿根治术手术疗效与开放手术无差别。早期发病的胆总管囊肿患儿（3岁以内）肝损伤较严重,应及早手术,术后效果明显。囊肿扩张形状不影响胆总管囊肿根治术的手术疗效,但囊形扩张者手术效果更明显。     

Bile infection contributes to intrahepatic calculi formation after excision of choledochal cysts

Intrahepatic calculi complicate choledochal cysts in 7–8% of patients. Although congenital stenoses and dilatation of the intrahepatic bile ducts are considered responsible for calculi formation, intrahepatic calculi are usually formed after cyst excision. In this study, bile specimens from patients with choledochal cysts were cultured for bacteria. Results were retrospectively analyzed among the following groups: the primary excision group, consisting of 97 patients undergoing cyst excision as a primary treatment (mean age 5.0 years), the internal drainage group, consisting of 13 patients who had previous cyst-enterostomy at cyst excision (mean age 20.2 years); and the hepatolithiasis group, consisting of 12 patients with postoperative hepatolithiasis (mean age 24.2 years). Bacteria were present in the bile of 10 patients (76.9%) in the internal drainage group and in all patients (100%) in the hepatolithiasis group, but present in only 17 patients (17.5%) in the primary excision group (p<0.01). Polymicrobial infection with Gram-negative enterobacteria such as Escherichia coli and Klebsiella species was predominant in the internal drainage and hepatolithiasis groups, while nonenteric bacteria were found in the primary excision group. Bile infection through bilioenterostomy may play an important role in intrahepatic calculus formation after excision of a choledochal cyst.     

Children presenting with hydatid cysts in common bile duct and choledochal cyst

Hydatid cysts developing primarily in the common bile duct are extremely rare. Occasionally, hydatid cysts rupture into the extrahepatic biliary ducts and cause obstruction [1–7]. Two children with intact hydatid cysts in the common bile duct and choledochal cysts were seen during a 2-year period in the Paediatric Surgery Division of the Children's Hospital, Banaras Hindu University, Varanasi. The details of their presentation and operative management are reported.     

Synchronous Todani types I and III choledochal cysts in a 10-month-old-infant: type IVb

A 10-month-old, previously healthy boy presented with one week of mild jaundice, light-colored stools and irritability. Abdominal sonography showed a large type I choledochal cyst and a separate, distinct cystic mass at the head of the pancreas. Magnetic resonance cholangiopancreatography was performed to evaluate the relationship of the two lesions. A type I choledochal cyst was confirmed, and a coexisting type III choledochocele was identified as the second cystic structure in conjunction with pancreaticobiliary malunion. The infant had complete resection of the type I choledochal cyst with Roux-en-Y hepaticojejunostomy, and anterior duodenotomy with marsupialization of the choledochocele. After five years of follow-up, the child is thriving and has had no recurrence of his symptoms. An exhaustive review of the literature identifies only one previous case of synchronous types I and III choledochal cysts, and this association is not clearly defined among the traditional classifications of type IV multiple choledochal cysts. Because operative management of a type III cyst requires the addition of a transduodenal approach, we encourage accurate reporting of mixed type choledochal cysts for the benefit of surgical planning, epidemiologic tracking and outcomes.