Accessory hepatic duct associated with a choledochal cyst

This case report describes an accessory hepatic duct (AHD) identified by intraoperative cholangiography during excisional surgery of a choledochal cyst (CC). The accessory duct was divided and reconstructed successfully to the Roux-en-Y jejunal loop. The postoperative course was uneventful, and follow-up abdominal sonography revealed neither evidence of biliary tract obstruction nor atrophic changes of the liver. It is advocated that an AHD should be meticulously reconstructed if it is divided during excisional surgery of a CC.     

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.     

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     

改良肝总管空肠Roux-Y型吻合术治疗小儿先天性胆总管囊肿

目的 探讨小儿先天性胆总管囊肿的治疗方法。方法 本组２８例,男９例,女１９例,年龄１０ｄ￣１４岁,均采用改良的肝总管空肠Ｒｏｕｘ－Ｙ型吻合术治疗先天性胆总管囊肿。结果 ２８例均痊愈出院。随访６个月￣８年,全组患儿未见出现腹痛、黄疸、发热。其中２０例患儿,经Ｂ超检查,未见胆管狭窄及扩张。Ｘ线钡餐检查,未见有钡剂反流入胆道。结论 改良肝总管空肠Ｒｏｕｘ－Ｙ型吻合术是治疗先天性胆总管囊肿的较好方法。     

经腹腔镜行先天性胆总管囊肿肝门部狭窄肝管扩大成形术

目的 经腹腔镜行先天性胆总管囊肿切除，肝管空肠Roux—Y吻合术在国内外已经开始应用，但是肝门部肝管狭窄一直被视为腹腔镜手术的禁忌，本次报道是对腹腔镜下行肝门部狭窄肝管扩大成形术可行性进行探讨。方法 从2001年7月至2003年1月，我院共经腹腔镜行胆总管囊肿切除，肝管空肠Roux—Y吻合术治疗先天性胆总管囊肿患儿35例，其中8例合并肝门部肝管狭窄，患儿年龄9个月至3．5岁(平均23岁)。肝总管近端狭窄7例，均合并左右肝管扩张；右肝管开口狭窄1例，合并右肝管扩张。手术中在腹腔镜监视下，从狭窄部的正中劈开狭窄环的前壁至扩张部，解除梗阻；然后将5mm直径的腹腔镜头导入肝内胆管，检查肝内胆管情况，明确有无肝内胆管狭窄及异物，指导冲洗胆道。最后用5—0可吸收缝线将空肠与扩大的肝管相吻合。结果 8例患儿均在腹腔镜监视下顺利完成肝门部狭窄肝管扩大成形肝管空肠Roux—Y吻合术，无须中转开腹手术者。狭窄部均为膜状，直径0．2～0．3cm，劈开狭窄环即见扩张的肝管。腹腔镜头导入肝内胆管发现球状蛋白栓1例，颗粒状蛋白栓2例，冲洗清除。整个手术花费时间3.8～5．6h，平均4．3h；出血量极少(约5～10m1)，无须输血者；术后无切口感染，胆漏和肠梗阻发生，均3d拔除腹腔引流管。术后住院时间4～6d。目前本组患儿随访3个月至18个月，无胆管炎和结石发生者，肝功能检查指标均在正常范围内。结论 本研究结果显示腹腔镜下行肝门部狭窄肝管扩大成形肝管空肠Roux—Y吻合术是安全可靠的途径，扩大了该技术应用于根治先天性胆总管囊肿手术的适应证。特别是术中腹腔镜替代胆道镜探查肝内胆管方便易行，可有效地术中同时处理肝内胆管并存的病变。     

Excision of a choledochal cyst and simultaneous hepatic lateral segmentectomy

We treated a 4-year-old girl with a choledochal cyst (CC) with bilateral intrahepatic involvement. A severe stricture between the enormously dilated left intrahepatic bile duct and the dilated common hepatic duct was found; this necessitated prophylactic hepatic lateral segmentectomy together with excision of the CC to avoid possible stone formation in the cystically dilated left intrahepatic duct. The choice of the combined procedures was based upon long-term results of other patients in our experience. This is the first such procedure to be reported.     

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。     

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.     

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。     

先天性胆总管囊肿胰管发育与十二指肠乳头异位的关系

目的十二指肠乳头的开口代表胚胎发育时期肝憩室的发生部位,而胰腺、胆总管和肝脏均起源于肝憩室。前续研究显示先天性胆总管囊肿、胰胆合流异常和十二指肠乳头异位密切相关。本文探讨胰管发育异常和十二指肠乳头异位之间的关系。方法先天性胆总管囊肿患儿118例,胰胆管造影了解其胰胆合流异常、十二指肠乳头异位及胰管发育异常情况,以11例年龄相仿球形红细胞增多症合并胆结石患儿的胆道造影结果为对照组。结果对照组中,11例患儿的十二指肠乳头均位于十二指肠降部。118例先天性胆总管囊肿患儿中,十二指肠乳头开口于降部38例,占32.2%,其余80例患儿,十二指肠乳头开口于降部以远,占67.8%。对于乳头开口于十二指肠降部以远的患儿,合并胰管发育异常者明显高于乳头开口位置正常者(十二指肠降部)(P<0.01)。乳头开口位置正常的先天性胆总管囊肿患儿,合并胰管发育异常仅占23.7%;乳头开口于十二指肠降部以远的患儿合并胰管发育异常仅占49.5%。乳头开口位置正常和开口于十二指肠降部以远的先天性胆总管囊肿患儿,合并胰管扩张的发生率分别为7.9%和28.7%,P<0.05。结论本研究结果表明胰管发育异常是先天性胆总管囊肿的重要病理改变,提示胚胎发育早期肝憩室的发育异常,可能是引起胆道扩张、胰管发育异常和乳头异位的原因。     

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.     

Modified Kasai's procedure for a choledochal cyst with a very narrow hilar duct

Excision has been widely recognized as the treatment of choice for choledochal cysts. For biliary reconstruction after cyst excision, we have strongly recommended hepaticoenterostomy at the hepatic hilum with a wide anastomotic stoma to prevent postoperative cholangitis. However, we recently treated two infants in whom a wide anastomotic stoma could not be made due to a narrow hilar duct. Therefore, a hepatic portojejunostomy with Roux-en-Y anastomosis (Kasai's procedure) was performed after cyst excision in both case to permit free drainage of bile. The mucosa of the hilar duct was everted and fixed to the liver parenchyma to prevent stricture formation. Both babies have done well since the surgery.     

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.     

Colonic atresia secondary to a choledochal cyst

Colonic atresia and choledochal cyst are both uncommon congenital abnormalities. We report a case in which the two conditions coexisted and propose an aetiological link. Accepted: 10 April 1997     

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     

Colonic atresia and choledochal cyst: a rare combination

Colonic atresia and choledochal cyst are rare congenital abnormalities and there are few cases of their coexistence reported in the literature. We report a case of choledochal cyst and colonic atresia in a term neonate. Our prenatal ultrasonographic and intraoperative findings support the idea that these two entities may be related.     

基于多层CT技术在儿童先天性胆总管囊肿手术方式制定中的应用

目的 探讨多层螺旋CT在先天性胆总管囊肿手术方式制定中的应用价值.方法 收集我院2006年9月至2009年3月间经手术及病理证实胆总管囊肿病例30例,根据手术中所见腹腔粘连情况,将病例分为粘连组及非粘连组,分别观察两组在多层螺旋CT双期增强检查中横断面图像及多平面重建图像(冠状面及矢状面)上胆总管囊肿分型及囊肿大小、形态、囊壁厚度、囊肿张力及囊壁附着血管数的差异,通过统计学分析筛选出与腹腔粘连有显著性相关的CT征象.结果 胆总管囊肿的囊壁厚度及囊壁附着血管数与腹腔粘连有显著性相关,粘连组壁厚(2.9±1.12)mm,附着血管数(4.9±1.4)个,P<0.05.而胆总管囊肿分型及其大小、囊肿张力则与腹腔粘连无显著性相关.结论 多层螺旋CT增强检查上胆总管囊肿壁厚大于或等于2.9 mm及囊壁附着的血管数大于或等于5支可以术前判断腹腔内感染或粘连较重.可为胆总管囊肿手术方式的选择提供指导.     

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.     

类似胆总管囊肿的胆总管横纹肌肉瘤：阻塞性黄疸的罕见原因

肝外胆道肿瘤罕见,其中以横纹肌肉瘤最常见。横纹肌肉瘤是一种软组织恶性肌肉骨骼肿瘤,是儿童非常罕见的一种胆总管恶性肿瘤。它通常表现为阻塞性黄疸和/或瘙痒。如果肿瘤没有局部浸润邻近组织,其影像学表现则类似胆总管囊肿。因此,确诊通常是在手术时或术前通过活检。早期诊断、与胆总管囊肿鉴别、尽早开始治疗对延长患者生存时间有重要意义。该文报道1例10岁男童,反复阻塞性黄疸和发热,术前诊断为胆总管囊肿,术后确诊为胆总管胚胎性横纹肌肉瘤。经手术切除、术后化疗,该患儿预后良好。故必须了解这种罕见的肿瘤可以类似先天性胆总管囊肿,并且在儿童阻塞性黄疸的鉴别诊断时应加以考虑。