Long-term survival with carcinoma arising from a congenital choledochal cyst

We report a case of 11-year survival with carcinoma arising from a congenital choledochal cyst. A 34-year-old Japanese woman was admitted due to epigastric discomfort. Ultrasonography revealed a choledochal cyst involving a polypoid tumor. Endoscopic retrograde cholangiopancreatography revealed pancreaticobiliary maljunction in which the main pancreatic duct joined the dilated common bile duct 2 cm above the papilla of Vater. A choledochal cyst containing a polypoid tumor was resected with the regional lymph nodes. Grossly, a 2×2-cm papillary tumor was noted in the posterior wall of the choledochal cyst. Microscopically, the tumor was well-differentiated tubular adenocarcinoma invading the mucosa and fibromuscular layer. Neither anticancer chemotherapy nor radiotherapy was performed. The patient recovered uneventfully after the operation and has been well for 11 years without evidence of recurrent cancer. The good results in this patient may have been due to the early stage of the cancer.     

Bile duct cancer developed after cyst excision for choledochal cyst

Oncogenesis after cyst excision for choledochal cyst and suitable surgical procedures for this operation are discussed. The clinical data of 23 patients with cancer of the biliary tree after excision of choledochal cyst reported in the English-language and Japanese literature were reviewed, and data for 1353 Japanese patients with choledochal cyst and/or pancreaticobiliary malunion were analyzed. In the 23 patients reported in the literature, age at cyst excision ranged from 1 to 55 years (average, 23.0 ± 13.7 years), and cancers were detected at age 18–60 years (average, 32.1 ± 12.2 years), with intervals between cyst excision and cancer detection of 1–19 years (average, 9.0 ± 5.5 years). Sites of cancer development were: intrahepatic, six; anastomotic, eight; hepatic side residual cyst, three; and the intrapancreatic duct, six. In the Japanese patients with choledochal cyst and/or pancreaticobiliary malunion, the incidence of cancer associated with primary choledochal cyst and/or pancreaticobiliary malunion was 16.2% (219/1353). The incidence of cancer development after cyst excision in this population, of whom 1291/1353 underwent surgery, was assumed to be 0.7%. Nearly half of the 23 patients in the literature had undergone inadequate cyst excision. Oncogenesis of cancers after cyst excision is possibly different from that of choledochal cysts. Received for publication on Feb. 17, 1999; accepted on March 29, 1999     

Choledochal cyst following operation for idiopathic perforation of the biliary tract in childhood

Three patients with idiopathic perforation of the biliary tract who had been treated by a drainage procedure, had a choledochal cyst during 2–8 years of follow up. In all patients, anomaly of pancreaticobiliary duct was revealed by cholangiography and/or retrograde cholangiopancreaticography. It was strongly suggested that biliary perforation in childhood and congenital choledochal cyst are common in pathogenesis and that an anomalous arrangement may play an important causative role. Presented at the 15th Annual Meeting of the Pacific Association of Pediatric Surgeons, Vancouver, British Columbia, Canada, May 11–14, 1982.     

Development of bile duct cancer in a 26-year-old man after resection of infantile choledochal cyst

Anomalous arrangement of the pancreaticobiliary duct is considered to be a high-risk factor for biliary tract malignancy. We report a case of intrahepatic cholangiocarcinoma in a 26-year-old man after total resection of choledochal cyst with anomalous arrangement of the pancreaticobiliary duct at the age of 5 months. He had been doing well after total resection of the choledochal cyst; however, he suddenly presented with a spiky fever and abdominal pain in the right upper quadrant at the age of 26 years. Computed tomographic scan and percutaneous transhepatic cholangioscope revealed multiple stones and stenosis of the hepatic duct and the left intrahepatic bile duct. Histologic examination of a biopsy specimen obtained from the stenotic site showed adenocarcinoma of the intrahepatic bile duct. Left lobectomy with re-reconstruction by right hepaticojejunostomy was performed, and his postoperative course was uneventful. One year after the operation, however, he died of carcinomatous peritonitis with recurrence of cholangiocarcinoma. This report warns us that bile stasis owing to stenosis of the intrahepatic bile duct and repeated cholangitis with multiple stones are high-risk factors for carcinogenesis of the intrahepatic bile duct even after total resection of the infantile choledochal cyst.     

Traumatic rupture of choledochal cyst in a child

Traumatic rupture of choledochal cyst is an extremely rare disorder. The current patient is a 4-year-old boy who fell in a bathroom and suffered a blow to the abdomen. Percutaneous transhepatic cholangiography revealed pancreaticobiliary maljunction. Inflammation of the peritoneal cavity was moderate. At first look, the choledochal cyst was excised and hepaticojejunostomy was performed. At this time, a rupture approximately 2 mm in diameter was recognized at the rear surface of the inferior part of the common bile duct.     

Congenital stenosis of the hepatic duct at the porta hepatis in children with choledochal cyst

Background

The intrahepatic gallstone is known as one of the complications of choledochal cyst. Stenosis of the hepatic duct may contribute to the formation of the stone. This report describes concurrent congenital stenosis of the hepatic duct identified during the primary operation of choledochal cyst.

Methods

Fourteen children were operated on for choledochal cyst. To identify the concurrent stenosis, cholangiography and inspection of the hepatic duct were performed during the operation. In those patients with identified membranous stenosis, the membrane was resected. In those with nonmembranous stenosis, the wide hilar hepaticojejunostomy was performed after hepatic duct plasty.

Results

Eleven sites of hepatic duct stenosis were identified, 8 membranous stenoses and 3 relative stenoses without membrane. The membranous stenoses were classified into 4 subtypes as follows: a small central opening, a marginal opening, 2 openings, and a bridgelike structure. At the primary surgery on choledochal cyst, those stenoses were released, and the formation of the gallstone has not been observed in 4 to 11 years in all cases.     

Histochemical and immunohistochemical studies on development of biliary carcinoma in forty-seven patients with choledochal cyst—Special reference to intestinal metaplasia in the biliary duct—

Histochemical and immunohistochemical studies on 47 consecutive specimens excised for choledochal cyst were performed to clarify possible metaplastic changes of the biliary duct in relation to carcinogenesis. An anomalous arrangement of the pancreaticobiliary ductal system was observed in all 39 cases examined. Among the 47 patients, 5 (10.6 per cent) had biliary carcinoma. 27.3 per cent mucous gland, 13.0 per cent goblet cell and 9.5 per cent argyrophil cell in 23 children. On the other hand, 81.8 per cent exhibited mucous gland, 41.7 per cent goblet cell and 27.3 per cent argyrophil cell in 24 adults. These metaplastic changes seemed to be an intestinal metaplasia and increased with age. Immunoreactive-gastrin or-somatostatin were evident immunohistochemically in 4 adults. These findings confirmed that intesinal metaplasia may develop in the biliary duct in cases of choledochal cyst. Although direct evidence between intestinal metaplasia and the development of biliary carcinoma was not found, reflux and stasis of pancreatic enzymes in the biliary duct may relate to the development of intestinal metaplasia and be an important factor related to the carcinogenesis of choledochal cyst.     

Surgical treatment for anomalous arrangement of the pancreaticobiliary duct with nondilatation of the common bile duct

Background/purpose

For anomalous arrangement of the pancreaticobiliary duct (AAPBD) with nondilatation of the common bile duct (CBD), the optimal surgical procedure remains controversial. The authors investigated which procedure would be most effective for AAPBD with nondilatation of the CBD.

Methods

The authors encountered 60 children with AAPBD in our institution between 1979 and 2002. Six of the 60 were classified as the nondilated type (CBD diameter; less than 8 mm), whereas the other 54 were classified as the dilated type (CBD diameter; more than 9 mm). Amylase levels in serum, CBD, and gallbladder were examined. Cellular activity of the resected gallbladder was examined for the incidence of hyperplasia and Ki-67 labeling index (Ki-67 LI).

Results

The amylase level in the nondilated type was elevated as in the dilated type. Epithelial hyperplasia of the gallbladder was present in 4 of the 6 with the nondilated type (67%). 10 of the 20 with the dilated type (50%), and none of the 6 controls (0%). The Ki-67 LI of the dilated type was significantly higher than that of control.

Conclusions

A free reflux of pancreatic juice into the biliary system was found regardless of dilatation, and cellular proliferative activity of the gallbladder mucosa was increased in both the nondilated and dilated type. Therefore, excision of the extrahepatic bile duct including cholecystectomy is recommended for AAPBD with nondilatation of the CBD.     

先天性胆总管囊肿多次手术原因分析

目的分析先天性胆总管囊肿(CCC)多次手术的原因。方法回顾性分析我院1995年1月至2004年12月因各种原因行再次及多次手术的CCC15例患者的临床资料。15例中,小儿7例,成人8例。首次手术行囊肿空肠吻合3例、囊肿十二指肠吻合3例(其中1例为妊娠期合并胆总管囊肿)。成人胆总管囊肿被误诊手术4例。囊肿自发破裂行外引流2例。胆漏2例。吻合口狭窄1例。结果13例行2次手术,2例行3次手术。本组1例术后发生胆漏患儿死亡,余均治愈。结论对CCC应尽可能地行囊肿切除,胆道重建,但在某些情况下可考虑先行外引流术以缓解症状,待病情稳定后再行囊肿切除,胆道重建。     

儿童自发性胆道穿孔诊治经验

目的 探讨儿童自发性胆道穿孔(spontaneous perforation of the bile duct,SPBD)的临床特点、诊断及治疗.方法 回顾性分析首都医科大学附属北京儿童医院2007年6月至2020年8月收治的36例SPBD患儿的临床资料,评价腹部超声和腹腔穿刺的诊断价值,比较胆囊置管引流、穿孔部位置管...     

Ciliated hepatic foregut cyst in a young child

Ciliated hepatic foregut cysts are a rare entity usually found in adults. We present a case of a 3-year-old boy incidentally noted to have a radiographically complex liver cyst on computed tomographic scan. Given the complex appearance, the cyst was excised. Pathology revealed a ciliated hepatic foregut cyst. This is the second child and youngest patient affected with this lesion reported in the literature. The etiology of the lesion and an argument for surgical removal in pediatric patients are presented.     

小儿先天性胆总管囊肿自发性穿孔的临床分析

目的：探讨先天性胆总管囊肿自发性穿孔的诊断方法和手术方式。方法：回顾性分析16例先天性胆总管囊肿自发性穿孔患儿的临床资料。结果：本组16例患儿年龄均小于4岁，平均年龄23个月。临床表现为胆胀，腹痛，呕吐及发热，同时有全腹压痛，10例术前经腹腔穿刺明确胆道自发性穿孔的诊断，其中5例经B超或CT检查证实有胆总管囊肿，采用囊肿外引流加腹腔引流治疗。16例患儿术后均恢复良好，3个月左右再地囊肿切除加胆道重建。结论：先天性胆总管囊肿穿孔与胆道梗阻有关，对小婴儿有腹膜炎表现，全腹压痛并以右上腹为著者，应常规行腹腔穿刺检查，急诊治疗宜选择囊肿外引流术，而后再行囊肿切除和胆道重建。     

LC引起的医源性右肝管、副肝管损伤的诊治

目的 分析腹腔镜胆囊切除术（LC）造成的医源性右肝管、副肝管损伤的特点及防治,探讨术中胆道造影（intraoperative cholangiography,IOC）对术后胆道通畅度评级的影响。方法 回顾性分析南京医科大学第一附属医院2014年5月至2021年11月诊治的20例医源性右肝管、副肝管损伤患者的损伤类型和损伤原因、治疗及随访结果。20例中11例未行术中胆道造影（非IOC组）,9例行术中行胆道造影（IOC组）,对比两组术后胆道通畅度评级。结果 右肝管损伤16例：8例行I期修补置管支撑引流,其中5例双T管置入;4例单纯修补;3例右肝管横断延期行胆肠吻合术;1例右肝管误缝扎,远期因梗阻行胆肠Roux-en-Y吻合术失败改行PTCD。4例副肝管损伤中1例修补并置管引流,1例术中误夹闭后胆瘘二期行胆肠吻合术,2例直接夹闭。IOC组术后胆道通畅度评级A级8例、C级1例,非IOC组GP A级3例、B级4例、C级3例、D级1例;IOC组评级优于非IOC组（P<0.05）。术后随访6~133个月,远期右肝管结石形成2例,胆管炎3例,右肝萎缩1例。结论 充分认识右肝管及副肝管的解剖特点,术中及时发现损伤,并根据损伤类型、IOC情况等制定相应策略是提高LC医源性胆管损伤疗效的关键。     

Neuroendocrine tumor of the common hepatic duct, mimicking a choledochal cyst in a 6-year-old child

We report a rare case of a neuroendocrine tumor of the extrahepatic biliary tree in a child. A 6-year-old girl who presented with jaundice and pruritus was found to have elevated liver enzymes along with a cystic dilatation of the common hepatic duct. After further diagnostic testing, a working diagnosis of a type I choledochal cyst was established. Laparotomy revealed hydrops of the gall bladder, an elongated and dilated cystic duct and a cystic dilatation of the common hepatic duct. An unusually thickened common bile duct was also noted at the time. Follow-up histology revealed an invasive neuroendocrine tumor which lead to a second look operation in which extended resection and lymphadenectomy was performed. No histological or gross pathological evidence of lymph node metastasis was found. Postoperatively the patient continues doing well 2 years after the original diagnosis was established. Neuroendocrine tumors of the extrahepatic bile duct are extremely rare with only 4 pediatric cases of a total of 51 cases published in the literature. To our knowledge, this is the youngest patient reported so far. The rarity of this entity made it challenging to diagnose a case of a neuroendocrine tumor in an atypical location that radiologically mimicked a choledochal cyst.     

Spontaneous rupture of choledochal cyst with pseudocyst formation-report on 2 cases and literature review

Spontaneous rupture and subsequent bile peritonitis are rare complications of choledochal cysts. Of these complications, the formation of a biliary pseudocyst is an unusual form, and its preoperative diagnosis is difficult. In this report, we describe 2 cases showing spontaneous rupture with biliary pseudocyst formation. Inflammatory tissue surrounded those pseudocysts, one of which was adjacent to the perforation and the other formed in the transverse mesocolon apart from the biliary tract. These pseudocysts were removed by careful dissection, and single-stage cyst excision with biliary reconstruction was successfully performed in both cases.     

Embryonal rhabdomyosarcoma of the common bile duct mimicking choledochal cyst

Embryonal rhabdomyosarcoma of the common bile duct is a very rare malignancy of childhood. The radiological appearance of the lesion is similar to that of congenital choledochal cyst if there is no local invasion to the adjacent tissues. The authors present a case of embryonal rhabdomyosarcoma of the common bile duct which was considered to be a congenital choledochal cyst preoperatively, and they discuss this very rare childhood tumor, with a brief survey of the literature. In conclusion, it is important to know that this rare tumor can simulate congenital choledochal cyst; it should be considered in the differential diagnosis of obstructive jaundice in children.     

凋亡抑制蛋白基因在先天性胆总管囊肿和胆管癌中的表达及意义

目的 探讨凋亡抑制蛋白基因(survivin)在先天性胆总管囊肿和胆管癌组织中的表达及意义.为进一步研究胆总管囊肿癌变的机制奠定理论基础.方法 采用免疫组化SP法检测Survivin在37例先天性胆总管囊肿组织(其中儿童20例,成人17例)和10例胆管癌组织中的表达,10例正常胆管作为对照.运用SPSS14.0统计学软件处理数据.结果 Survivin在37例先天性胆总管囊肿组织中,11例为阴性,15例弱阳性表达,9例阳性表达,2例强阳性表达.而在10例胆管癌组织中,Survivin有5例阳性和5例强阳性表达,未见阴性表达.正常胆管组织中仅有1例弱阳性表达,两两相比差异有统计学意义(P＜0.01).Survivin在成人胆总管囊肿中的表达强于儿童组,两者间差异有统计学意义(P＜0.01).结论 Survivin参与了胆总管囊肿癌变过程,可能与癌变的发生发展有关,在先天性胆总管囊肿癌变过程中可能起到促进作用.     

儿童及成人先天性胆总管囊肿对比临床分析

目的　研究儿童及成人先天性胆总管囊肿的不同特点 ,提高对先天性胆总管囊肿的诊治水平。方法　回顾分析 3 2例先天性胆总管囊肿的诊治资料 ,将其分为少儿 ( <14岁 )和成人( >14岁 )发病组 ,分析两者不同的临床表现、诊断和治疗特点。结果　少儿组 17例 ,临床表现为持续性黄疸、肝肿大、腹部包块 ,术前一般情况差 ,保守治疗 6例 ,其中 2例因肝硬化腹水 ,肝功能衰竭死亡 ,1例未愈出院 ,3例延期手术 ;手术 14例 ,13例行肝管空肠Roux -Y吻合 ,1例行肝管十二指肠吻合术。成人组 15例 ,以间歇性右上腹疼痛、畏寒发热、黄疸首次就诊 ,分别行囊肿切除肝管空肠吻合术 ( 12例 )、肝管十二指肠吻合术 ( 1例 )、肝左叶并囊肿切除肝管空肠吻合术 ( 2例 )。结论　先天性胆总管囊肿发病患儿起病急 ,病情发展快 ,易合并肝脏损害 ,应加强围手术期支持治疗 ;成人囊肿切除胆道重建术后 ,应密切随访 ,防治胆管炎及胆道癌变     

Spontaneous perforation of common bile duct in a young female: An intra-operative surprise

IntroductionSpontaneous CBD perforation is one of the rare causes of acute abdomen in infants and extremely rare in adults. It is rarely suspected and correctly diagnosed preoperatively.Presentation of caseA 17 year old female presented to Emergency Department with sudden onset of pain and distention of abdomen, associated with vomiting and non-passage of flatus and stool for 3 days and features of generalized peritonitis. On exploration, a perforation of size 0.5 cm in diameter was present on the antero-lateral surface of supraduodenal part of common bile duct (CBD) below the junction of cystic duct and common hepatic duct. Cholecystectomy done and the CBD repaired over a T-tube.DiscussionSpontaneous perforation of bile duct should ideally manage with T-tube drainage of the CBD along with cholecystectomy. In case with distal obstruction of the CBD, a biliary enteric bypass should be done.ConclusionDue to the paucity of cases, the index of suspicion for this diagnosis is low. But bilious peritoneal tap, features of generalized peritonitis and absence of free gas under diaphragm in abdominal x-ray may be considered as clues for suspicion. Accordingly, Surgery remains the mainstay of treatment.