经内镜逆行胰胆管造影术在儿童胰胆疾病中的应用进展

经内镜逆行胰胆管造影术(ERCP)是胰胆疾病诊断和治疗的重要手段,可对各种原因所致的梗阻性黄疸、胆管结石、胰胆管异常、急慢性胰腺炎等多种疾病进行诊断和治疗。随着内镜诊治技术的发展,ERCP在儿童胰胆疾病中的应用逐渐增多,主要集中在儿童先天性胰胆疾病和急慢性胰腺炎。现就近年来ERCP在儿童胰胆疾病诊治中的应用进展进行综述。     

小儿经内镜逆行胰胆管造影术的临床应用

随着儿科内镜技术的发展,小儿经内镜逆行胰胆管造影术(ERCP)开始得到重视,它具有提供详细胰胆管系统的解剖和功能信息优点,是胰胆管疾病因诊断及鉴别诊断的重要手段,而治疗性ERCP如括约肌切开术、取石术、支架植入及鼻胆管引流术也逐渐应用于临床,其并发症少、死亡率低、重复性好,具有广阔的应用前景.     

磁共振胰胆管造影诊断小儿先天性胆总管囊肿

磁共振胰胆管造影 (ｍａｇｎｅｔｉｃｒｅｓｏ ｎａｎｃｅｃｈｏｌａｎｇｉｏｐａｎｃｒｅａｔｏｇｒａｐｈｙ ,ＭＲＣＰ)是近年来开展的一项新的胰胆管影像学检查方法 ,国内自 1995年起相继开展了此项目 ,但都用于成人胰胆管病变的观察与诊断 ,对小儿胰胆管疾病的应用尚未见报道。总结我院 1999年 3月～2 0 0 0年 4月采用ＭＲＣＰ诊断并经手术治疗的 16例小儿先天性胆总管囊肿病例 ,报告如下。临床资料1.一般资料　患儿 16例 ,男 3例 ,女 13例 ,年龄 3个月～ 14岁 ,平均5 .3岁。经临床及超声检查可疑先天性胆总管囊肿而行ＭＲＣＰ检查。2 .…     

经内镜逆行胰胆管造影术治疗儿童胰胆管合流异常的临床研究

目的 探讨经内镜逆行胰胆管造影术(endoscopic retrograde cholangiopancreatography,ERCP)治疗胰胆管合流异常(pancreaticobiliary maljunction,PBM)的临床效果及预后。方法 回顾性分析2014年1月至2020年12月解放军总医院第七医学中心收治的60例PBM患儿的临床资料,根据治疗方法分为ERCP组(n=30)和外科手术组(n=30)。分析患儿的临床资料、辅助检查和预后情况。统计学方法采用Wilcoxon秩和检验、Mann-Whitney检验、χ²检验或Fisher确切概率法。结果 ERCP依据日本胰胆功能障碍研究小组(Japanese study group on pancreaticobiliary maljunction,JSGPM)-PBM分型标准,以A型(10例,33.3%)、B型(10例,33.3%)为主,而外科手术组以C型(11例,36.7%)、D型(14例,46.7%)为主,差异有统计学意义(P<0.05)。两组患者的手术成功率均为100%。ERCP组与外科手术组的...     

内镜下逆行胰胆管造影治疗儿童胆总管结石并发急性胰腺炎一例(附内镜下视频)

目的探讨内镜下逆行胰胆管造影(ERCP)在儿童胆总管结石并发急性胰腺炎中的应用价值。方法回顾性分析郑州大学附属儿童医院收治的1例胆总管结石并发急性胰腺炎患儿的诊断与治疗经过, 分析ERCP在儿童胆管结石诊治中的作用。结果患儿, 男, 13岁11个月, 因间断腹痛、呕吐1个月, 加重2 h就诊, 确诊为胆总管结石并发急性腹腺炎接受ERCP治疗。球囊扩张十二指肠乳头扩约肌, 应用取石网篮取石, 取出胆总管结石及大量泥沙样结石。术后置入鼻胆引流管一根, 于术后1周改为内支架引流, 患儿病情好转。结论 ERCP治疗儿童胆总管结石并发急性胰腺炎可快速缓解临床症状, 改善实验室检查指标。     

儿童胆胰疾病的内镜下逆行胰胆管造影介入治疗

内镜下逆行胰胆管造影(ERCP)及治疗技术是成人胆胰疾病微创治疗的重要手段。随着内镜技术的发展及儿科医生对儿童胆胰疾病认识的提高, ERCP技术逐渐成为儿童胆胰疾病不可或缺的诊治手段。本文重点阐述ERCP在儿童胆胰疾病中的适应证、手术要点等, 推动ERCP在儿童中的应用发展。     

十二指肠乳头位置与胰胆管合流异常

目的观察十二指肠乳头开口位置与胰胆管合流异常(PBM)的关系。方法对64例伴有PBM的先天性胆总管囊肿(CBD)患儿、10例伴有PBM和30例无PBM的成人胰胆疾病患者的胆道造影进行分析,乳头开口于十二指肠第2段中1/3定为正常开口组,开口于第2段下1/3、第2、3段交界处和第3段的定为易位开口组;测乳头距十二指肠第2、3段交界处距离,实测长度除以椎体高度取得校正值。结果CBD患儿十二指肠乳头开口于第2段中1/3、下1/3、第2段与第3段交界处和第3段的分别占42%、22%、27%和9%。易位开口组和正常开口组的共通管长度校正值分别为0.89±0.42和0.66±0.31,P=0.03,囊肿长度校正值分别为3.0±1.1和2.2±0.5,P=0.001。共通管长度与乳头距十二指肠第2、3段交界处距离的相关系数为0.297,P=0.038。成人10例PBM患者中8例(80%)存在乳头易位开口,30例无PBM患者中12例42%存在乳头易位开口,两组间差异有显著性(P=0.03)。结论在伴有PBM的CBD患儿和胰胆疾病成人患者中,十二指肠乳头向远端开口易位发生率显著高于无PBM的病人,乳头开口越远,共通管越长。     

内镜下逆行胰胆管造影及相关技术治疗慢性胰腺炎并发胰腺胸膜瘘一例(附内镜下视频)

目的探讨内镜下逆行胰胆管造影(ERCP)相关技术在慢性胰腺炎并发胰腺胸膜瘘诊疗中的应用价值。方法总结分析首都医科大学附属北京儿童医院收治的1例以呼吸道症状为首发临床表现的慢性胰腺炎合并胰腺胸膜瘘的患儿临床资料及诊疗过程, 复习相关文献。结果患儿, 女, 8岁1个月, 因慢性胰腺炎并胰腺胸膜瘘予ERCP治疗。术中取石, 见大量蛋白及黏液栓流出, 置入支架, 尾端位于胰体尾交界胰管瘘口处, 术后6 d, 病情好转出院。结论慢性胰腺炎合并胰腺胸膜瘘可引起呼吸道症状, 临床需注意鉴别诊断, 早期进行ERCP治疗, 效果好、创伤小、恢复快, 对改善患儿预后有重要作用。     

儿童内镜下逆行胰胆管造影术后并发症的预防和治疗

内镜下逆行胰胆管造影(ERCP)及相关技术在儿童胆胰疾病中逐渐开展起来, 临床医生应加强术后并发症的预防及管理, 使患儿全程获益。本文从ERCP及相关技术的操作方法入手, 阐述可能出现的并发症, 总结预防及治疗方法, 旨在为临床医生提供参考。     

Diagnostic and therapeutic ERCP in the pediatric age group

The role and value of endoscopic retrograde cholangiopancreatography (ERCP) in the pediatric age group is not well established, because pancreatic and biliary diseases are less common in children. This however is not the case in areas like the Eastern Province of Saudi Arabia where sickle cell disease (SCD) and other hemoglobinopathies are common, with increased frequency of cholelithiasis and choledocholithiasis. The purpose of this study was to evaluate the indications, findings, safety and therapies of ERCP in children. One hundred and twenty five children had diagnostic and/or therapeutic ERCP as part of their management at our hospital. Their medical records were reviewed for: age at diagnosis, sex, Hb electrophoresis, indication for ERCP, findings, therapy and complications. There were 77 males and 48 females. Their age at presentation ranged from 5–18 year (mean 13.25 year). The majority of them had sickle cell disease (77.6%). The indications for ERCP were: obstructive jaundice (67.2%), recurrent biliary colic with or without jaundice (10.4%), acute and chronic pancreatitis (7.2%), postoperative bile leak (2.4%), cholangitis with obstructive jaundice (2.4%), hepatitis of unknown etiology (3.2%), cirrhosis of unknown etiology (4%), thalassemia with jaundice (0.8%), hemobilia (0.8%), acute cholecystitis with jaundice (0.8%), and sickle cell disease with ulcerative colitis and obstructive jaundice (0.8%). In six children, ERCP was done following laparoscopic cholecystectomy. ERCP was carried out under sedation in 91 (72.8%) children and under general anesthesia in 34. It was successful in 121 (96.8%) children while cannulation of the Ampulla failed in four. ERCP was normal in 43 children, but eight of them showed evidence of recent stone passage and in six, there were gallstones. In the remaining children, ERCP revealed: normal CBD with stones (18 patients), dilated CBD with stones (17 patients), dilated CBD without stones (19 patients), dilated biliary tree with stones (10 patients), dilated biliary tree without stones (six patients), bile leak (two patients), dilated biliary tree with stones and choledocho-duodenal fistula (one patient), choledochal cyst (two patients), septate gallbladder (one patient), normal ERCP with multiple pancreatic cysts (one patient) and biliary stricture (one patient). The following procedures were carried out: 35 had endoscopic sphincterotomy and stone extraction, 20 had endoscopic sphincterotomy, four had CBD stenting, one underwent removal of a stent, two had insertion of a nasobiliary tube and one had biliary endoprosethesis. There was no mortality. One had bleeding from the site of sphincterotomy which stopped after adrenaline injection. Four patients (3.2%) developed transient mild pancreatitis which settled conservatively. ERCP in the pediatric age group is safe both as a diagnostic and therapeutic procedure. ERCP can provide valuable information which aid in the diagnosis of biliary and pancreatic diseases in children as well as therapy with the technical feasibility of endoscopic sphincterotomy. This is specially so in the era of laparoscopic cholecystectomy, where ERCP should be the treatment of choice in children with CBD stones who are going or have previously undergone laparoscopic cholecystectomy.     

Retained common-duct stones after open cholecystectomy and duct exploration in children

A retrospective study was performed of 250 patients with cholelithiasis treated at the Royal Children's Hospital, (RCH) Melbourne, over 25 years by open operation; 32 (12.8%) had proven choledocholithiasis on either preoperative imaging, operative cholangiography (OpCG), or postoperative investigation. A further 3 had underlying congenital biliary abnormalities and were excluded from further study. Thirty-one of the 32 were explored at open operation, 27 after OpCG and 4 on clinical grounds. One retained common-bile-duct (CBD) stone was undetected until the postoperative period (1/250, 0.25%). Seven ducts were not cleared, giving a duct exploration failure rate of 22.6% (7/31). All 8 retained CBD stones were identified in the early postoperative period and managed with a variety of techniques, including endoscopic retrograde cholangio-pancreatography (ERCP). The incidence of retained stones after open CBD exploration was high (22.6%), and can be attributed to difficulties in operative technique dealing with the smaller paediatric CBD. In addition, haemolytic disease seems to induce a propensity for choledocholithiasis. Given the small numbers presenting with cholelithiasis to RCH (10 per year), it is suggested that a selective approach to CBD exploration is appropriate in children. With the increasing use of laparoscopic cholecystectomy in children and the inherent technical difficulties of laparoscopic operative cholangiography, ERCP may offer an alternative solution in dealing with CBD stones rather than open or laparoscopic CBD exploration.     

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.     

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.     

先天性胆总管囊肿患儿胆汁成分分析及致石性研究

目的：探讨先天性胆总管囊肿患儿胆汁成分变化与结石形成关系。方法：对10例胆总管囊肿及9例非肝胆疾病手术患儿胆汁成分进行定量分析,包括总胆汁酸、4种初级胆汁酸、磷脂、胆汁蛋白、胆固醇及总脂含量等,并进行胆汁细菌培养。结果：①囊肿患儿胆汁中胆汁酸、磷酸、胆固醇、总蛋白及总脂浓度明显异常,以胆汁酸减少为显著。其肝胆汁、胆囊胆汁、囊肿胆汁中总胆汁酸浓度均明显降低,总脂浓度下降。易发生胆结石的主要原因之一;②囊肿患儿三种胆汁中四种初级结合型胆汁酸的含量及G／T,CA／CDCA均降低,胆汁酸组分比的变化,也是易产生结石的原因。囊肿中初级结合型胆汁酸的浓度相对降低,次级胆汁酸相对增加,可能对囊肿恶变发生的影响;③囊肿患儿肝细胞功能受损是导致CCC患儿胆汁成分发生改变的主要原因。结论：CCC患儿胆汁中主要固体成分存在明显异常,以胆汁酸减少及胆汁酸的组分比改变为著,是CCC患儿易发生结石的主要原因之一,导致CCC患儿胆汁成分改变的核心问题是肝功能受损所致。     

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.     

Chronic pancreatitis in a child with glycogen storage disease type 1

A case of chronic pancreatitis in an 8-year-old boy with glycogen storage disease type 1a (GSD 1a) is presented. This patient had a history of hyperlipidaemia unresponsive to dietary therapy, e.g., a carbohydraterich diet, uncooked cornstarch, and nocturnal intragastric tube feedings. He had recently suffered bouts of abdominal pain and diarrhoea. Serum amylase and trypsin were elevated, abdominal CT revealed the presence of a pseudocyst of the pancreas. The presence of chronic pancreatitis was confirmed by endoscopic retrograde cholangiopancreatography and an infected pseudocyst was removed at laparotomy.     

腹腔镜胆总管囊肿切除肝管空肠Roux-en-Y吻合手术总结

目的 本研究对腹腔镜胆总管囊肿切除肝管空肠Roux-en-Y吻合手术6年经验进行总结,探讨该技术的近期和远期疗效.方法 从2001年4月至2007年3月,本课题组采用腹腔镜技术治疗先天性胆总管囊肿患儿102例,平均年龄3.2岁.其中13～28d9例,29d～6个月23例,7个月～3岁28例,4～18岁42例.采用四trocars技术完成胆道造影、胆囊和胆总管囊肿壁全层切除;同时利用腹腔镜头对扩张的肝内胆管进行探查;从脐部切口提出空肠,直视下行Roux-en-Y空肠吻合,然后还纳肠管;经结肠后上提空肠的肝支,镜下将肝管与空肠连续吻合.结果 102例全部在腹腔镜下完成手术,无中转开放手术者,平均手术时间224min(155～456min),手术中出血5～10ml,无手术中需要输血者.21例患儿合并肝门部肝管狭窄,行肝管扩大成形术;其中1例患儿手术后并发胆漏,持续腹腔引流26d,自然愈合.2例患儿分别于手术后第二天至第五天和第七天并发消化道出血.1例5岁女孩手术后7.5h突发心跳骤停,高血钾(K+10.85mmol/L),死亡.其他98例患儿手术后恢复顺利,手术后3～6d出院.1例患儿于手术后6个月发生空肠肝支梗阻坏死,行坏死肠管切除,二次肝管空肠Roux-Y吻合手术.患儿手术后经3～72个月的随访肝功能正常,无结石和胰腺炎发生.结论 腹腔镜胆总管囊肿切除肝管空肠Roux-en-Y吻合手术是治疗先天性胆总管囊肿可靠的手段,镜下放大的手术视野有利于精确的手术操作,近期和远期疗效良好.手术后早期有必要密切观察血钾的变化.     

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.     

改良胆肠袢式吻合术在小儿先天性胆总管囊状扩张症中的应用

目的 探讨改良胆肠袢式(warren)吻合术治疗小儿先天性胆总管囊状扩张症的近期临床疗效.方法 回顾性分析我院2008年10月至2010年12月采用改良胆肠袢式(warren)吻合术治疗30例小儿胆道扩张症患儿的临床资料,并与同期所行胆管空肠Roux-en-Y吻合术的25例患儿临床资料进行比较.结果 改良胆肠袢式(warren)吻合组完成吻合术的时间(56±9)min、术后肠功能恢复时间(23.0±3.4)h及住院时间(9±1)d均短于胆管空肠Roux-en-Y吻合组(85±13)min,(42.3±4.2)h,(12±2)d,两种术式具有相同的胆汁引流效果,减黄的效果差别也显著,近期并发症发生率的差别无统计学意义.结论 改良胆肠袢式(warren)吻合术与传统的Y式吻合术相比有很多优点,并且操作简便易行,安全可靠,治疗小儿先天性胆道扩张症近期临床疗效好,长期疗效仍需进一步观察.