Ectopic pancreas associated with a choledochal cyst and extrahepatic biliary atresia

Two children with incidentally-diagnosed ectopic pancreatic tissue in the jejunum at surgery for extrahepatic biliary atresia (EHBA) and choledochal cyst (CC) are reported. No case has been reported in the literature describing the association of a CC with ectopic pancreas, and only one case of EHBA associated with ectopic pancreas has been reported. We believe that incidentally-detected ectopic pancreatic tissue should be excised, even though the patient is symptom-free, in order to prevent the risk of serious complications due to either the mass effect or the potential for acute pancreatitis, cystic degeneration, or malignant transformation at a later date. Accepted: 12 December 2000     

Aplasia of the dorsal pancreas and choledochal cyst

The authors describe a rare case of choledochal cyst and aplasia of the dorsal pancreas complicated with chronic pancreatitis. A 9-year-old boy presented with obstructive jaundice. After biliary drainage using pericutaneous transhepatic gallbladder drainage (PTGBD) technique, the patient underwent choledochal cyst excision with Roux-en-Y hepaticojejunostomy. The association of choledochal cyst and aplasia of the dorsal pancreas with chronic pancreatitis has never been reported previously.     

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.     

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.     

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。     

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.     

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。     

Abnormal pancreatic isoamylases in the serum of children with choledochal cyst.

PURPOSE: To investigate the changes of pancreatic isoamylases in the serum of children with choledochal cyst. METHODS: Serum and bile from 20 children with choledochal cyst were studied for total amylase activity and isoamylases. Using electrophoresis, the assay of pancreatic isoamylases was expressed by peak appearance rate (PAR) and ratio of isoenzyme activity (RIA). Serum from 20 healthy children and 12 adults with acute pancreatitis served as a comparison. RESULTS: The total amylase activity in the serum of children with choledochal cyst was slightly higher than that of healthy controls, but dramatically lower than that of acute pancreatitis. The serum of children with choledochal cyst showed 5 pancreatic peaks (PAR: P1, 100 %; P2, 100 %; P3, 100 %; P4, 75 %; P5, 45 %) without any major peak. In healthy controls, only P1 and P2 were present (PAR: P1, 40 %; P2, 100 %) with the latter being a major peak (RIA: 81.8 +/- 25.3 %). These results in children with choledochal cyst differed from those of patients with acute pancreatitis who presented with the less common P4 and no P5 (PAR: P1, 100 %; P2, 100 %; P3, 91.6 %; P4, 8.3 %). The pattern of pancreatic isoenzymes in bile was similar to that in the serum from patients with choledochal cyst. These abnormal pancreatic isoamylases gradually disappeared after operation. CONCLUSIONS: Abnormal pancreatic isoamylases, especially P4 and P5, are found in the serum of patients with choledochal cyst, which would point to a reflux of pancreatic juice into the biliary tract.     

Preoperative evaluation of choledochal cyst with MR cholangiopancreatography]

The choledochal cyst is a rare congenital disorder usually diagnosed in childhood. It requires a complete surgical resection to prevent complications, particularly the risk of malignant changes. At present, the preoperative examination requires a direct opacification of the biliary tree, but this is an invasive technique with a high risk of infection, especially in pediatric patients. CASE REPORT: A choledochal cyst was diagnosed in a five-year-old girl with recurrent abdominal pain. Diagnosis was made by ultrasound and preoperative evaluation by magnetic resonance-cholangiopancreatography using single-shot fast-spin echo sequences. A complete correlation was observed between surgical, preoperative cholangiography and MRCP data. CONCLUSION: Recent improvement in MRCP techniques provide a complete anatomic analysis of choledochal cysts, enabling one to diagnose an anomalous junction of the pancreaticobiliary duct, even the presence of stones within the biliary tree. This short and noninvasive examination should in the future replace direct opacification of the biliary tree for the preoperative assessment of choledochal cysts.     

Hepatic fibrosis with choledochal cyst in infants and children - an immunohistochemical assessment.

METHODS: The study population was divided into 4 groups: 12 infants with choledochal cyst, aged 4 m to 12 m, were classified as the infant choledochal cyst (ICC) group; 36 children, aged 1 y to 14 y, were classified as the children with choledochal cyst (CCC) group; while 18 patients, aged 2 m to 5 m, with biliary atresia (BA) were included as positive controls; and 14 infants, aged 1 d to 3 y, who died from non-liver diseases served as negative controls (CON). Liver specimens were examined using H&E sections to score fibrosis by means of Ohkuma's classification, and immunohistochemical sections were evaluated by counting the cells positive for cytokeratin (CK) and human leukocyte antigen-DR (HLA-DR) to discover the pathogenic factors of fibrosis. RESULTS: Most ICC patients had clinical biliary obstruction. The liver fibrosis score was highest in the BA group (2.9 +/- 0.7). The fibrosis score in the ICC group was higher (2.5 +/- 0.9) than that of the CCC (1.5 +/- 1.2; p < 0.05) and of the CON (0.1 +/- 0.4; p < 0.01) groups. The densities of CK-positive cells were 164 +/- 80/HP, 253 +/- 165/HP, 70 +/- 57/HP and 23 +/- 12/HP in the BA, ICC, CCC and CON groups, respectively, and differed significantly (p < 0.01) with the exception of the ICC vs. the BA group. The densities of HLA-DR positive cells were 130 +/- 72/HP, 98 +/- 54/HP, 96 +/- 50/HP and 36 +/- 13/HP in the portal area in the BA, ICC, CCC and CON groups, respectively. The density was lowest in the CON group (p < 0.01). CONCLUSION: In patients with choledochal cyst, liver fibrosis is more common and severe in infants than in children. Obstruction of the bile duct and proliferation of bile duct cells were the main pathogenic factors for fibrosis, while HLA-DR mediating immuno-injury may play a limited role.     

Small cell carcinoma of the pancreas in association with a choledochal cyst: immunohistochemical characterization and complete response to combination chemotherapy

Carcinomas arising in choledochal cysts are well recognized; however, small cell carcinomas have not been reported to arise in choledochal cysts. Extrapulmonary small cell carcinomas have been recognized as unique clinicopathologic entities for decades, and have been found arising in numerous sites. Recently there have been several reviews of the subject (Remick et al.: Medicine 66:457-71, 1987; Richardson and Weiland: Seminars in Oncology 9:484-496, 1982; Levenson et al.: Journal of the National Cancer Institute 67:607-612, 1981). Small cell carcinoma of the pancreas has only rarely been described (Reyes and Wang: Cancer 47:2500-2502, 1981; Corrin et al.: Cancer 31:1523-1527, 1973). These tumors must be distinguished from carcinoid tumors and islet cell tumors, which can often lead to histochemical and histologic confusion (Richardson and Weiland: Seminars in Oncology 9:484-496, 1982). Their response to therapy has not been well documented. We report here a small cell carcinoma of the pancreas that arose within a choledochal cyst and that had immunohistochemical characteristics supporting a neuroendocrine origin but that lacked evidence of islet cell differentiation. A complete response to chemotherapy is also described.     

Laparoscopic surgery for biliary atresia and choledochal cyst

Minimally invasive surgery in children has evolved to the extent that complex procedures can be performed with safety and outcome comparable with open surgery, with the advantage of minimal scarring. Here we describe the latest laparoscopic techniques used by us at the Juntendo University Hospital, Japan, for treating biliary atresia and choledochal malformation, with presentation of our postoperative management and discussion of preliminary outcomes.     

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。     

Laparoscopic treatment of biliary atresia and choledochal cyst

Minimally invasive surgery (MIS) has overcome many technical limitations and has evolved into a safe alternative for the treatment of many complex pediatric surgical procedures. The introduction of this approach for the correction of congenital biliary tract anomalies had to wait until instrumentation and surgeons' skills improved enough. This happened not so long ago: less than 10 years have elapsed since the first reported case of a minimally invasive operation for choledochal cyst and less than 3 years since the first reported case of a laparoscopic Kasai. This article summarizes the experience gained by the authors in laparoscopic treatment of 41 patients with biliary atresia and 15 patients with choledochal cyst with similar surgical techniques, which are described in detail. Based on the encouraging results, the authors believe that MIS will soon become the gold standard for the correction of congenital biliary tract anomalies.     

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.     

Sonographic and computerized tomography study of congenital choledochal cyst

In the neonatal period ultrasound and hepatobiliary functional scintigraphy are used to diagnose choledochal cysts. Initial sonography demonstrates hepatobiliary anatomy, hepatobiliary function is assessed by subsequent scintigraphy. The diagnosis can be confirmed by additional computed tomography as shown in this case report.     

先天性胆总管囊肿43例诊治分析

目的　探讨先天性胆总管囊肿 (CCC)的诊断和治疗方法。方法　对 1998年 1月～ 2 0 0 3年 1月我院收治 43例 14岁以下CCC患儿性别、年龄、诊断依据、囊肿类型、手术时机和术式进行分析。结果　CCC发病年龄在 2岁以下者占 72 % ;男女比为 1:3 ;本组入院前后均经B超、CT、磁共振胆管成像 (MRCP)等检查 ,诊断符合率分别为 83 .7%、80 %、78.9% ,共 40例患儿行手术治疗 ,术式为囊肿切除 肝总管空肠Roux -en -Y吻合术 ,无 1例发生死亡或严重并发症。结论　B超是首选的检查手段 ,囊肿切除、胆道重建是理想的手术方法 ,胆肠吻合口的直径大小与是否发生吻合口狭窄有直接关系     

磁共振在先天性胆总管囊肿诊治中的应用

磁共振胆胰管成像(MRCP)是目前几年快速发展起来的一种非损伤性胆胰管成像技术，已广泛应用于临床，但应用于小儿胰胆管病变领域的报道较少。我院1999年12月～2003年5月对12例先天性胆总管囊肿(congenital chotedochalcyst，CCC)行MRCP检查，并行手术治疗，现报道如下。