胆总管囊肿切除不同胆道重建术的远期疗效评价

目的分析和评价先天性胆总管囊肿切除后采用不同胆道重建手术方式的远期治疗效果。方法对1985-2000年所行121例胆总管囊肿切除、胆道重建手术治疗的患儿资料进行总结和长期随访观察，胆道重建手术包括单纯肝管空肠Roux-Y吻合27例、肝管十二指肠吻合黏膜乳头成形24例、回盲部肠段间置14例和肝管空肠Roux-Y吻合加曾氏防反流瓣56例四种术式。结果91例获得远期随访，平均时间11．7g。2例发生恶变，出现反复发作胆管炎13例(单纯吻合组7例、黏膜乳头成形组5例和加防反流瓣组1例)、吻合口狭窄8例和肝门胆管结石5例，11例再手术后症状消失。结论根治切除囊肿是治疗本病的基础，肝门大口肝管空肠Roux-Y吻合加抗反流瓣术是胆道重建手术可选择的最佳术式。     

囊肿切除、胆道重建治疗先天性胆总管囊肿

目的：总结24年囊肿切除,胆道重建手术治疗先天性胆总管囊肿的经验,探讨有关的治疗问题,方法：回顾1975－1999年124例囊肿切除胆道重建手术治疗的先天性胆总管囊肿患者,其中行囊肿切除,肝总管空肠Roux-y吻合97例（加曾氏防反流瓣87例）,囊肿切除,间置空肠十二指肠哈合14例（加入工套叠瓣4例）,囊肿切除,肝总管十二指肠吻合8例,囊肿大部切除,胆道重建5例,对手术方式,手术后近,远期并发症进行分析。结果：124例中I型118例（囊性扩张102例,形扩张16例）,II型2例,IV4例,近期并发症有：术后出血5例,胆汁漏7例,胆系感染2例,吻合口梗阻1例,肠梗阻4例,无围手术期死亡,远期并发症;反流性胆管炎26例,胆系结石5例,胆管癌3例,肠醒阻6例,结论：囊肿切除胆道重建术是治疗先天性胆总管囊肿的最佳方法,提高手术技巧,预防术后胆管炎的发生对提高生活质量有重要意义。     

Wide hilar hepatico-jejunostomy: the optimum method of reconstruction after choledochal cyst excision

Standard reconstruction after choledochal cyst excision is by Roux-en-Y hepaticojejunostomy to the common hepatic duct. Long-term follow up studies have shown a 10% incidence of late complications, including anastomotic stricture. By extending the bilio-enteric anastomosis along the left hepatic duct, a wide hilar bilio-enteric anastomosis is created which may help to minimize late anastomotic complications. Forty-one consecutive patients (24 girls, 18 infants) with a median age of 2.3 years (range 44 days to 15.6 years) and median weight 11.5 kg (range 2.1–59 kg) underwent radical choledochal cyst excision with a wide hilar hepticojejunostomy. Thirty-eight were followed-up both clinically and by ultrasound scan and biochemical liver function tests for a median of 2.7 years (range 0.1–12.5 years). The median width of the hilar hepaticojejunostomy was 8 mm (range 6–25 mm) in 18 infants, and 15 mm (range 10–25 mm) in 22 older children. In one patient it was not measured. Only one surgical complication occurred—a self-limiting bile leak which settled spontaneously. Median postoperative stay was 6 days (range 5–21 days). No patient has had an episode of cholangitis or adhesive small bowel obstruction to date. Postoperative biochemical liver function tests have remained normal in all but one child (with pre-existing biliary cirrhosis). After radical resection of a choledochal cyst, a wide hilar hepaticojejunostomy is a, safe, effective and durable reconstructive technique that can be performed at any age and may help to minimize the long-term risk of complications.     

Comparison of hepaticoantrostomy and hepaticojejunostomy for biliary reconstruction after resection of a choledochal cyst

Twelve infants operated upon for choledochal cyst (CC) are reviewed with emphasis on the operative technique of biliary tract reconstruction, incidence of cholangitis, postoperative hypergastrinemia, biliary excretion, and upper gastrointestinal (GI) motility in a follow-up of 24 to 35 months. In 7 patients biliary reconstruction was performed with a Roux-en-Y hepaticojejunostomy (HJ), and in 5 with a hepaticoantrostomy (HAST). In the HJ group 4 patients had recurrent episodes of cholangitis and intermittent diarrhea and serum gastrin levels were significantly elevated in 5. Hepatobiliary scintigraphy showed unobstructed excretion of labelled bile through bile ducts into the Roux-en-Y loop, but with significantly delayed emptying of bile into the distal jejunum in all patients. Gastric emptying and upper intestinal passage were normal. In the HAST group no episode of cholangitis occurred and serum gastrin levels were within the normal range. Scintigraphically, hepatobiliary excretion, and duodenojejunal passage of labelled bile was normal, except in 1 patient who developed a postoperative stenosis of the left hepatic duct. Upper GI contrast studies demonstrated normal gastric emptying without reflux into the biliary system. These results suggest that biliary reconstruction with HAST can be performed safely with a low incidence of complications HAST offers a more physiologic method of biliary reconstruction after resection of a CC that allows bile to drain directly into the duodenum.     

腹腔镜下胆总管囊肿切除、胆道重建术

目的 探讨腹腔镜下胆总管囊肿根治切除、胆道重建术的可行性。方法 腹腔镜辅助下胆囊或胆总管穿刺胆道造影，同时镜下行胆总管囊肿切除及肝管空肠Roux-Y吻合术。结果 13例胆道造影显示均伴有胆胰管合流异常。7例胆总管呈囊状扩张(6例KomiⅠa型、1例KomiⅠb型)；5例胆总管呈梭形扩张(2例KomiⅡa型、3例。KomiⅡb型)；1例胆总管远段胰腺内囊肿中转开腹手术。12例腹腔镜下顺利根治切除胆总管囊肿，4例经结肠后、8例经结肠前吻合胆道重建术，手术时间4．2～6．5h。结论 腹腔镜下胆道造影方法简便、诊断明确；胆总管囊肿根治切除及胆道重建手术安全可靠，值得临床进一步推广应用。     

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.     

Pancreatitis complicated with dilated choledochal remnant after congenital choledochal cyst excision

We describe here three cases of pancreatitis after congenital choledochal cyst excision. In these three cases, the choledochal remnant in the pancreas head was markedly dilated, probably because of an incomplete resection of the cyst at the primary operation, and an increase in intraluminal pressure of the pancreatic duct caused by a dynamic obstruction by a protein plug or a pancreatic calculus. Complete cyst excision, including the choledochal wall in the pancreas, is therefore strongly recomended.     

Nonrefluxing biliary appendicoduodenostomy for choledochal cyst

A new technique of biliary appendicoduodenostomy along with an antireflux procedure was used for the treatment of a choledochal cyst, with a satisfactory result.     

先天性胆总管囊肿囊肿切除空肠间置与肝总管空肠吻合的远期随访与评价

目的 研究先天性胆这囊肿囊肿切除空肠间置与肝总管空肠Ｒｏｕｘ－Ｙ吻合重建胆管的远期随访结果与疗效评价。方法 对２种常用的胆管重建术：空肠间置术（２０例）和肝总管空肠Ｒｏｕｘ－Ｙ吻合术（３０例）进行３￣１０年随访。结果 获得随访４４例,其中短段空肠间置伴抗掺流瓣术１４例,肝总管空肠Ｒｏｕｘ－Ｙ吻合短段桥袢伴抗反流瓣术１８例均无反流性胆管炎,钡餐检查剂反流,而短段空肠间置不伴抗反流瓣４例中１例,肝总管     

45例腹腔镜辅助胆总管囊肿根治术

目的本研究对腹腔镜先天性胆总管囊肿切除，肝管空肠Roux-Y吻合术进行探讨。方法从2001年6月至2003年9月，共收治先天性胆总管囊肿患儿45例，年龄2个月到12岩。其中42例为囊肿型，平均囊肿直径3.8cm（2-18cm）；其余3例为梭形，直径分别为1．5、2．0和2．2cm。本组45例患儿均经腹腔镜行先天性胆总管囊肿切除，肝管空肠Roux-Y吻合术。结果本组45例患儿手术全部成功，手术时间平均为4.3h（3．5～7．6h），术中出血量约5～10ml。本组8例患儿合并肝管狭窄，术中同时行腹腔镜胆总管囊肿切除及肝管成形；6例患儿合并共同管内蛋白栓，术中通过腹腔镜导入肠道或插管冲洗清除。其中1例患儿于术后第1d发生胆漏，术后第26d时自愈；其余44例患儿术后恢复顺利，住院时间3～6d。术后随访3～30个月，无肠粘连梗阻和吻合口狭窄等术后并发症发生。结论经腹腔镜先天性胆总管囊肿切除，肝管空肠吻合术是一种安全可靠的方法，术中胆道造影全面了解胆道的结构，清晰的肝门暴露，准确的囊肿分离和熟练的缝合技术是手术成功的关键。     

胆总管囊肿术后并发晚发枫糖尿症一例并文献复习

目的分析1例胆总管囊肿术后并发晚发枫糖尿症(maple syrup urine disease,MSUD)患儿的诊治资料并复习相关文献,以提高对此类疾病的认识。方法收集胆总管囊肿术后并发晚发MSUD患儿的临床资料,总结临床表现、检查方法、诊断和治疗经过。通过PubMed、中国知网、万方等数据库检索近10年相关文献,检索起止时间为2010年1月至2020年5月。结果患儿在行胆总管囊肿切除术后2周内出现顽固性呕吐。术后1个月再次行腹腔镜探查术,第二次术后第一天患儿突然出现嗜睡、神志淡漠、瞳孔对光反射减弱和无法对答。四肢肌力降低,腱反射减弱。头部磁共振成像(magnetic resonance imaging,MRI)检查显示双侧额颞叶皮质、乳头体、双侧壳核、丘脑及中脑、脑桥背侧面多发对称性片状等T1长T2信号,T2-flair为稍高信号,弥散加权成像(diffusion weighted imaging,DWI)示弥散受限。尿有机酸气相色谱-质谱(gas chromatography-mass spectrometry,GC/MS)联用技术检测显示α-酮-3-甲基戊酸显著增高,提示MSUD。经抗感染、补充维生素B1和B12、低支链氨基酸营养、高压氧治疗后,患儿病情逐渐好转。通过检索数据库,近10年国内外文献共报道9例晚发MSUD患儿的病例。结合本研究病例,共10例晚发MSUD。结论早期识别、合理治疗、多学科协作是决定MSUD患儿预后的关键。     

Spontaneous rupture of choledochal cyst

Choledochal cyst in an uncommon congenital anomaly with classic presentation triad of abdominal pain, jaundice and right upper abdominal mass. Presentation due to biliary peritonitis following cyst rupture is extremely rare. One such case which was successfully treated is being reported.     

囊肿型先天性胆总管扩张术中不结扎远端残端的探讨

目的 胆总管囊肿切除术中,远端胆总管残端需要游离和结扎,但游离和结扎存胰管有损伤的风险,甚至导致胰漏的发生.本研究旨在探讨囊肿型胆总管囊肿切除术中不结扎远端残端的可行性,从而减少术中胰腺实质损伤的风险.方法 将我院2001年10月至2010年10月实施胆总管囊肿切除术和Roux-en-Y肝管空肠吻合术的270例患儿,根据胆总管囊肿不同的影像学和形态学特点分成两组:①不结扎组(n=207),即远端残端狭窄,选择不结扎远端残端术式;②结扎组(n=63),即远端残端无狭窄,选择结扎远端残端术式.对比分析两组术后胰漏发生率.结果 不结扎组和结扎组分别随访36个月和33个月,两组均未发现胰漏.结论 在远端胆总管狭窄类型(囊肿型)的胆总管囊肿切除术中不结扎远端残端是可行的,减少了胰管受损的可能,对避免胰漏发生起到了一定的作用,同时简化了手术步骤,值得在临床中推广应用.     

Intracorporeal electrohydraulic lithotripsy for intrahepatic bile duct stone formation after choledochal cyst excision

An 18-year-old girl who had undergone excision of a choledochal cyst and Roux-en-Y hepatico-jejunostomy at another hospital when 23 months old was referred to our department because of recurrent cholangitis. Radiological investigations showed stones lying in minimally dilated, right posterior intrahepatic bile ducts (IHBD). At laparotomy, the hepatico-jejunostomy site was incised, and a flexible endoscope inserted into the IHBD. Multiple stones packed in the IHBD were easily fragmented using an electro-hydraulic lithotripsy (EHL) device inserted through the endoscope, and removed. There were no EHL-related complications, and her postoperative progress was uneventful. She is currently well with no episodes of cholangitis after a follow-up period of 3 years. EHL is a simple, effective alternative method for removing IHBD stones after choledochal cyst excision. To the best of our knowledge, this is the first report of EHL being used to remove stones that developed in the IHBD after choledochal cyst excision.     

24小时食管pH及压力测定对胆总管囊肿术后胃食管反流的监测

目的 24h食管pH及压力测定及胆总管囊肿术后胃食管反流的监测。方法 对10年期63例先天性胆总管囊肿患儿中18例术后进行了24h食管双极pH及压力测定。结果 8例患儿出现胃食管反流,行囊肿切除肝管空肠Roux-Y吻合加抗反流装置3例,囊肿空肠Roux-Y吻合,无人工套叠4例,有人工套叠1例。结论 先天性胆总管囊肿术后可出现胃食管反流,反流与食管廓清运动能力无关,可能与异常的十二指肠活动有关。     

Piezoelectric extracorporeal shockwave lithotripsy for bile duct stone formation after choledochal cyst excision

We report a case of bile duct stones in which piezoelectric extracorporeal shockwave lithotripsy (ESWL) was highly effective for the clearance of stones. A 16-year-old girl, who had undergone excision of a choledochal cyst when she was 3 years old, presented a spiking fever and colic abdominal pain. Radiological investigations showed two large stones incarcerating to the proximal end of hepatico-jejunostomy anastomosis. Massive debris was also present in intrahepatic bile duct proximal to the anastomosis. She underwent piezoelectric ESWL with an EDAP LT02 lithotripter. An average of 40 min ESWL session was repeated at intervals of 2 or 3 days. Neither anesthetic nor sedative treatment was required. By the end of the sixth session, the stones incarcerated were fragmented and the debris in the intrahepatic bile duct was completely eliminated. We conclude that piezoelectric ESWL is a less invasive, effective and repeatable method, therefore, it could be a treatment of choice for bile duct stone formation after choledochal cyst excision.     

胆总管囊肿切除术中胰管损伤的防治

目的探讨胆总管囊肿切除术中胰管损伤的处理及预防。方法 分析手术处理５例胰管损伤的临床资料。结果 ５例胰管损伤中行胰管修补３例、胰管十二指肠吻合１例及副胰管结扎１例;术后４例效果满意,１例胰漏经保守引流治愈。结论 术中造影可清楚显示胆胰管连接系统,指导胆总管囊肿根治切除术离断 胆胰管连接部水平,避免损伤胰管。对胰管损伤者应行胰管修复或重建术。     

Why not choledochal cyst?

With excision as a standard procedure for treating choledochal cyst, early diagnosis becomes a central issue in reducing morbidity and mortality from that disorder. In a retrospective analysis of 35 patients treated over a 10-year period, we found the diagnosis was delayed for an average of 11.9 months from the beginning of presenting symptoms. Three factors were responsible: (1) a random combination of non specific presenting symptoms such as pain and vomiting or jaundice and hepatomegaly frequently led to a diagnosis such as acute pancreatitis or hepatitis, in which conservative treatment was considered most appropriate; (2) the three parameters commonly used to diagnose acute pancreatitis (serum amylase, lipase, and Cam/Ccr) may all be elevated in patients with a choledochal cyst; and (3) a combination of several noninvasive examinations such as radionuclide cholescintigraphy, ultrasonography, and upper gastrointestinal X-rays may fail to diagnose some difficult cases of choledochal cyst. To obviate such problems, we recommend a routine ultrasound examination of biliary tract in children with recurrent or progressive obstructive jaundice or unexplained acute pancreatitis. When these noninvasive examinations fail to solve the problem, endoscopic retrograde cholangiopancreatography should be considered. Offprint requests to: J.-H. Chuang     

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     

Rhabdomyosarcoma of the common bile duct imitating choledochal cyst

A child with rhabdomyosarcoma of the choledochus is described. Because the clinical and radiological presentation was that of a choledochal cyst, cholecystectomy, resection of the choledochus, and hepaticoduodenostomy was performed. Unfortunately, the pathological examination was misdiagnosed and radio- and chemotherapy were administered only after a second operation 2 months later. Offprint requests to: J. A. Bar-Maor