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.     

Diagnosis and treatment of congenital choledochal cyst: 20 years' experience in China

AIM To summarize the experience of diagnosis andtreatment of congenital choledochal cyst in the past 20years(1980-2000).METHODS The clinical data of 108 patients admitted from1980 to 2000 were analyzed retrospectively.RESULTS Abdominal pain,jaundice and abdominal masswere presented in most child cases.Clinical symptoms inadult cases were non-specific,resulting in delayeddiagnosis frequently.Fifty-seven patients(52.7%)hadcoexistent pancreatiobiliary disease.Carcinoma of thebillary duct occurred in 18 patients(16.6%).Ultrasonicexamination was undertaken in 94 cases,ERCP performedin 46 cases and CT in 71 cases.All of the cases werecorrectly diagnosed before operation.Abnormalpancreatobiliary duct junction was found in 39 patients.Before 1985 the diagnosis and classification of congenitalcholedochal cyst were established by ultrasonographypreoperatively and confirmed during operation,the mainprocedures were internal drainage by cyst enterostomy.After 1985,the diagnosis was established by ERCP andCT,and cystectomy with Roux-en-Y hepaticojejunostomywas the conventional procedures.In 1994,we reported anew and simplified operative procedure in order to reducethe risk of choledochal cyst malignancy.Postoperativecomplication was mainly retrograde infection of biliarytract,which could be controlled by the administration ofantibiotics,there was no perioperative mortality.CONCLUSION The concept in diagnosis and treatment ofcongenital choledochal cyst has obviously been changedgreatly.CT and ERCP were of great help in theclassification of the disease.Currently,cystectomy withRoux-en-Y hepaticojejunostomy is strongly recommendedas the choice for patients with type Ⅰ and type Ⅳ cysts.Piggyback orthotopic liver transplantation is indicated intype Ⅴ cysts(Caroli's disease)with frequently recurrentcholangitis.     

Choledochal cyst,pancreatobiliary malunion,and cancer

A review of the clinical features of biliary cancer with choledochal cyst and pancreatobiliary malunion is presented, together with a recent case report. Biliary cancer develops in about 25% of patients with choledochal cyst and pancreatobiliary malunion, and usually occurs in younger patients (usually those in their 40s) than does biliary carcinoma in the general population. The risk of malignancy in the retained cyst with internal drainage is higher than that in the primary cyst. Early excision of the retained cyst should be performed as quickly as possible, even if the patient is symptomless. Some bile acid fractions and refluxed pancreatic enzymes in bile are possibly responsible for carcinogenesis. Carcinoma generally develops in the extrahepatic bile duct and gallbladder, and rarely in the intrahepatic bile duct. In cystic dilatation, cancer usually occurs in the common bile duct, while in diffuse or non-dilated type, it develops in the gallbladder. Multicentric carcinomas develop in the bile duct either synchronously or metachronously. The prognosis of biliary cancer is usually dismal. However, aggressive procedures are now gaining better results than conventional approaches. The procedure of choice for choledochal cyst or malunion is to prevent the development of cancer by performing an early excision. Removal of the entire extrahepatic bile duct is necessary, even in patients with malunion and no biliary dilatation. Carcinoma rarely arises in the intrahepatic bile duct after excisional surgery, probably due to the long-standing stricture of the bile duct. Capacious anastomosis and/or ductoplasty is essential. Carcinoma may also develop in the remnant bile duct. Excision of the distal duct extending into the pancreas is also necessary.     

A review of choledochal cyst in pediatric and adult patients

A review of 80 pediatric and 13 adult patients with choledochal cyst disease occurring in a multiracial population is presented to define the incidence, clinical pattern, surgical techniques, and complications seen over periods of 30 years (pediatric) and 15 years (adult patients). In pediatric patients, most cases are now being diagnosed at a much earlier age, usually in infancy or early childhood, presumably due to the ready availability of ultrasound. Adult patients, by contrast, present a complex picture of heptobiliary disease, and delay in diagnosis is not uncommon. Jaundice appears to be the predominant symptom and the classical triad (pain, jaundice, and mass) is seldom seen. The surgical technique emphasises complete excision of the cyst, including the distal end, as the critical factor in the prevention of late complications. Avoidance of anastomotic stricture depends on the apposition of gut to healthy duct wall and not on the size of the anastomosis alone. Postoperative results have been excellent with no immediate mortality and no evidence of cholangitis in patients with extrahepatic cysts. The morbidity is related to patients with persistent intrahepatic biliary dilatation, Caroli's disease, and carcinoma. While endoscopic intervention, hepatic resection, and liver transplantation have been utilized in the management of cystic disease in the liver, the outlook for patients with cholangiocarcinoma associated with choledochal cyst remains extremely poor, with no survivors in this series.     

Multiple early bile duct carcinoma associated with congenital choledochal cyst

Emergency ultrasonography showed a protruding tumor in the markedly dilated common bile duct of a 33-year-old Japanese woman. Magnetic resonance cholangiopancreatography also demonstrated the tumor clearly, almost as clearly as did percutaneous transhepatic cholangiography. With a diagnosis of common bile duct carcinoma associated with congenital choledochal cyst, pancreaticoduodenectomy was performed. In the resected specimen, as well as the protruding tumor, there was also a small slightly elevated lesion. Pathology examination showed adenocarcinoma limited to the fibromuscular layer in the protruding tumor, and adenocarcinoma limited to the mucosa in the elevated lesion. Prophylactic total excision of the choledochal cyst before the occurrence of malignant change is strongly recommended in patients with congenital choledochal cyst. However, in those who are reluctant to undergo the operation, periodic follow-up with ultrasonography and magnetic resonance cholangiopancreatography would be ideal to achieve early detection of malignant change. (Received May 9, 1997; accepted Sept. 26, 1997)     

Case Report: Giant choledochal cyst

We report an adult female with a rare giant choledochal cyst. The patient presented following a normal pregnancy with the classical triad of an abdominal mass associated with jaundice and right upper quadrant abdominal pain. The cyst was excised using an intramural technique and biliary reconstruction achieved with a Roux-en-Y hepaticojejunostomy. Our patient has remained well with no evidence of malignancy over a 12 year review period. The aetiology and current management of this condition are discussed.     

Cholecystoduodenal fistula associated with choledochal cyst: a rare clinical entity

A 61-year-old woman presented with recent history of cholangitis. On evaluation, she was found to have a type I choledochal cyst and a cholecystoduodenal fistula. She underwent excision of the choledochal cyst and disconnection of the fistula. In this case study, we present the diagnostic features and management of choledochal cyst associated with cholecystoduodenal fistula and a literature review of the condition.     

Classification of congenital biliary cystic disease: special reference to type Ic and IVA cysts with primary ductal stricture

Congenital biliary cysts occur not only in the choledochus but also everywhere in the biliary tree, and are frequently accompanied by pancreatobiliary malunion. Alonso-Lej and colleagues first classified choledochal cysts into three types, in 1959. Due to the recognition of intrahepatic involvement, we refined their classification into six types, in 1977, and this has become the reference. However, this classification does not include the concept of pancreatobiliary malunion and has caused some confusion, especially in regard to types Ic and IVA. Type Ic, showing fusiform dilatation, has a pancreatobiliary malunion and often extends continuously to the intrahepatic duct. Type IVA, with intrahepatic involvement, shows primary ductal stricture, especially around the hepatic hilum and umbilicus. The incidence of the stricture is considerably greater than was previously assumed, and upstream intrahepatic ducts often join the dilated duct, with distinct caliber change. Intrahepatic dilatation may develop primarily, due to the stricture, combined with weakness of the duct wall. This stricture can be corrected by portal dissection in the liver to obtain a large anastomosis. In patients with intrahepatic involvement, we should carefully find the stricture by suitable cholangiograms, or by intraoperative cholangioscopy. Detecting the ductal stricture is obviously important, rather than classifying the cyst as type Ic or IVA.     

Congenital choledochal dilatation: Classification, clinical features, and long-term results

Choledochal cyst is generally accompanied by pancreatobiliary malunion. Pancreatic juice usually refluxes into the bile duct via the malunion, and bile juice occasionally regurgitates into the pancreatic duct. As a result, various pathological conditions occur in the biliary tract, pancreas, and liver. We have revised our classification of choledochal cyst on the basis of malunion. Abdominal mass, pain, and jaundice are regarded as the triad of choledochal cysts, but symptoms are quite non-specific. In neonates and infants less than 12 months of age, choledochal cyst is usually huge and no cylindrical dilatation is observed. Vomiting and jaundice with acholic stool are often observed. No hyperamylasemia is seen, despite the presence of a malunion. Whereas children over 13 months of age tend to show diffuse dilatation, and an abdominal mass is rarely palpable. Epigastralgia with hyperamylasemia, fever, vomiting, and slight jaundice are often observed. Cyst excision is the treatment of choice to prevent the development of ascending cholangitis and cancer in the cyst. Late complications of excisional surgery include recurrent cholangitis, pancreatitis, and rarely, biliary malignancy. These complications usually develop 5 years or more after initial surgery and become the object of reoperation. Complete removal of the extrahepatic bile duct from the hepatic hilum to the intrapancreatic duct and a hilar hepaticoenterostomy with wide anastomosis after ductoplasty, including the coexisting primary stricture, are essential at the primary surgery. Provided a large anastomosis, favorable results can be obtained with either duodenostomy or Roux-Y jejunostomy. Surgical enlargement of the hilar ducts and careful lifelong follow-up should be routine in all patients who have undergone cyst excision. When ductal stricture with intrahepatic gallstones is confirmed, reoperation at an early stage is indicated to obtain a good quality of life with short hospitalization.     

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.     

Sphincter of Oddi dysfunction associated with choledochal cyst

The pathophysiology of choledochal cysts remains unclear, although an association with anomalous pancreato-biliary junction and the reflux of pancreatic enzymes into the biliary tree is known. Sphincter of Oddi (SO) manometry was performed in three patients with choledochal cysts. All patients exhibited an elevated basal pressure diagnostic of sphincter of Oddi dysfunction. Two patients exhibited anomalous pancreato-biliary junction. This report suggests an association between the choledochal cyst and sphincter of Oddi dysfunction, and may suggest that SO dysfunction plays a role in choledochal cyst formation.     

Mucinous cystadenoma of the pancreas 17 years after excision of gallbladder because of a choledochal cyst

A 56-year-old woman who had undergone excision of the gallbladder because of a choledochal cyst had a tumorous lesion of the pancreas identified by upper abdominal ultrasonography, but an operation was not carried out, because there was no apparent increase in the cystic mass and no elevation of serum tumor markers. In October 2001, she was admitted to our hospital to check for malignancy because of elevated levels of the tumor marker Dupan-2. Abdominal enhanced computed tomography and upper abdominal ultrasonography revealed a large multilocular cystic mass in the body to tail of the pancreas. Endoscopic retrograde cholangiopancreatography showed elongation of the common duct that communicates with the common bile duct and the main pancreatic duct, indicating an anomalous arrangement of the biliary and pancreatic duct system. No apparent communications between the cystic mass and the main pancreatic duct were observed. In January 2002, the patient underwent a spleen-preserving distal pancreatectomy, and histopathological and immunohistochemical examinations led to the diagnosis of pancreatic mucinous cystadenoma with ovarian-like stroma. The mucinous cystadenoma was detected 17 years after the operation for the choledochal cyst. To the best of our knowledge, no documented case reports of mucinous cystadenoma of the pancreas associated with a choledocal cyst have been reported to date. We present here the first case report of pancreatic mucinous cystadenoma occurring in the body to tail of the pancreas, associated with a choledocal cyst.     

Triple carcinomas of the biliary tract associated with congenital choledochal dilatation and pancreaticobiliary maljunction

We report a rare case of triple carcinomas of the biliary tract associated with congenital choledochal dilatation (CCD) and pancreaticobiliary maljunction (PBM). The patient was a 58-year-old Japanese man who complained of epigastralgia. Ultrasonography and computed tomography revealed an elevated lesion inside the markedly dilated extrahepatic bile duct, thickening of the gallbladder wall, and small polypoid lesions in the gallbladder. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography showed CCD and PBM. With a diagnosis of carcinoma of the bile duct and cholesterol polyps in the gallbladder, pylorus-preserving pancreaticoduodenectomy was performed. The resected specimen showed two elevated lesions in the dilated bile duct, cholesterol polyps, and an area of irregular mucosa in the gallbladder. Histopathological examination showed two carcinomas in the bile duct, an adenosquamous cell carcinoma, and a moderately differentiated tubular adenocarcinoma, and a well differentiated tubular adenocarcinoma of the gallbladder. Two years and 6 months after the operation, a solitary metastatic liver tumor was detected. Left hepatic lobectomy was performed. At present, 7 months after the second operation, the patient is doing well with no signs of recurrence. Multiple carcinomas in the biliary tract associated with CCD and PBM, including the details in the present patient, were reviewed. Received: March 31, 1999 / Accepted: October 22, 1999     

对肝内胆管结石外科治疗的再认识

肝内胆管结石系原发性胆管结石范畴,尽管其发病率有所下降,但因地区不同仍有差别。早期肝内胆管结石采用微创外科技术施行肝切除术治疗是正确选择,应保护Oddi括约肌的功能;但对复杂性肝内胆管结石所致的终末期肝病的外科治疗仍存在诸多问题。应提倡多学科合作医疗模式,进行综合性治疗,并加强肝内胆管结石预防、病因和发病机制等研究,为治疗肝内胆管结石努力探索。     

完全腹腔镜手术治疗成人先天性胆总管囊肿患者疗效及安全性分析*

目的 探究完全腹腔镜手术治疗成人先天性胆总管囊肿的疗效及安全性。方法 回顾性分析2012年2月~2018年5月在我院进行手术治疗的25例先天性胆总管囊肿患者的临床资料,其中接受传统开腹手术治疗者17),采用完全腹腔镜手术治疗者8例。结果 开腹组患者术后首次进食时间、肛门排气时间、排便时间、住院日和术中出血量分别为(5.0±1.7) d、(3.8±1.5)d、(4.7±1.8) d、(9.4±2.3) d和(241.6±15.3)mL,显著长于或多于腔镜组【分别为(3.0±1.1) d、(2.6±0.8) d、(3.1±1.3) d、(6.6±1.2) d和(94.0±9.7)mL,P<0.05】,但腔镜组手术时间为(278.3±60.5)min,显著长于开腹组【(231.7±41.2)min,P<0.05】;治疗后3 d,开腹组患者血清C反应蛋白(CRP)水平为(77.4±6.4)mg/L ,显著高于腔镜组【(30.8±3.5)mg/L,P<0.05】;腔镜组和开腹组手术后并发症发生率分别为37.5%和58.8%,无显著性差异(P>0.05)。结论 完全腹腔镜手术治疗成人先天性胆总管囊肿患者疗效较好,与传统开腹手术比,术中出血量少,术后恢复快,不加重肝功能损伤。     

规则肝切除术治疗复杂肝内胆管结石的临床体会

目的探讨复杂肝内胆管结石行规则肝切除术的临床效果。方法回顾性分析石首市人民医院2013年1-12月98例复杂肝内胆管结石患者的临床资料,分析患者肝内胆管结石分布特点、临床表现、手术范围、手术时间、并发症及手术治疗效果。结果98例患者均完成规则肝切除术,37例行胆总管切开取石T管引流加肝段切除,6例行左肝外叶切除,1例行左半肝切除,7例行右半肝单独肝段及联合肝段切除,45例行左右半肝肝段联合切除,2例行肝门部胆管整形后胆肠盆式吻合。围手术期未出现患者死亡,手术时间(65.0±5.0)min;术中出血量(83.0±6.2)ml,术后无结石残留患者。5例(5.1%)患者出现并发症,其中3例患者并发胆漏,2例患者并发肝脏创面渗血。3例患者术后病理检查发现肝内胆管癌。随访5年,11例(11.2%)患者术后结石复发。结论规则肝切除术治疗复杂肝内胆管结石患者安全且疗效较好,并发症少,结石复发率低,值得临床推广应用。     

Bacteriological and electron microscopic examination of primary intrahepatic stones

BACKGROUND: Primary intrahepatic cholelithiasis is usually combined with biliary tract infection. This research was undertaken to investigate the relationship between intrahepatic stones and biliary tract infection. METHODS: Thirty-five bile samples and 30 stones specimens were cultured for bacteria and 12 stones specimens were examined with a scan electron microscope (SEM) or a transmission electron microscope (TEM). RESULT: 94.2% bile samples and 96.7% stones specimens were positive in bacteria culture. Bacteria were found in stones under SEM and TEM. CONCLUSION: Bacteria in stones are associated with the infection of the biliary tract.     

Spontaneous perforation of right hepatic duct in a child with choledochal cyst

A 5-year-old girl with choledochal cyst (Alonso Lej, type I) and spontaneous perforation of the right hepatic duct during an attack of acute pancreatitis was treated by two-stage surgery. Biliary panperitonitis was diagnosed by abdominal paracentesis, bilirubin and amylase levels in the collected fluid being 41.3 mg/dl and 1812 Somogyi U, respectively. The initial surgery involved simple peritoneal drainage and decompression of the bile duct, followed by removal of a choledochal cyst and the common hepatic bile duct, including the biliary stricture of the left hepatic duct, and biliary reconstruction 5 months later. Cholangiogram after the initial surgery demonstrated pancreaticobiliary maljunction and stricture in the left hepatic duct and the common hepatic duct. Regurgitation of pancreatic juice into the bile duct was shown by the extremely high amylase level (90100 Somogyi U) in the bile drained through a T-tube. The patient is currently well, with normal liver and pancreatic function tests. The experience in this patient is significant in that it should be known that spontaneous perforation of the bile duct may occur at the right hepatic duct in choledochal cyst, causing biliary peritonitis.