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.     

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.     

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.     

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.     

Congenital choledochal cyst: Occurrence of primary intrahepatic stones and prevention of carcinogenesis

Intrahepatic biliary stones and carcinogenesis are two major complications seen after corrective surgery for congenital choledochal cyst. The incidence of these two complications was reviewed in patients seen at out-patient clinics at a mean follow-up time of 13 years and 6 months. Intrahepatic biliary stones developed in 5 of 56 patients who had undergone a standard excisional operation; in 4 of the 5 patients, the stones were found in the preexisting intrahepatic biliary dilatation (with or without a downstream stenosis), 7–15 years postoperatively. Biliary stricture with dilatation is a putative cause of intrahepatic bile duct stones, and this hypothesis was supported here, since stones developed in all 3 of the patients who had the peculiar configuration of intrahepatic biliary dilatation and stenosis. Biliary carcinoma was not observed in any of the 56 patients who had undergone standard excision of a choledochal cyst followed by hepatico-jejunostomy, but developed in 1 of 12 patients in whom complete excisional resection of the dilated extrahepatic bile duct had not been performed. The occurrence of carcinoma in choledochal cysts is reviewed.     

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.     

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.     

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)     

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.     

Choledochal cyst disease: A review

The authors present a review of the classification, etiology, presentation, treatment and long-term outcome of children and adults with choledochal cyst disease. Unlike children, adults often present with acute biliary tract symptoms or pancreatitis. The treatment of choice remains extrahepatic cyst excision and biliary bypass. This treatment has excellent long-term results that minimize the development of malignancy.     

A Case Report of Choledochocele (type III choledochal cyst of the Alonso-Lej classification)

Abstract: The case of a 33-year-old woman with a rare type of choledochal cyst (Choledochocele; Alonso-Lej's type III) is described. The patient had undergone prior cholecystectomy. Hypotonic duodenography showed a smooth defect in the duodenum. Endoscopic retrograde cholangiopancreato-graphy (ERCP) showed a cystic dilatation of the terminal portion of the common bile duct. Endoscopic ultrasonography (EUS) showed a cystic lesion at the same site. This case was preoperatively diagnosed by ERCP and EUS. Transduodenal sphincteroplasty was done and good results were obtained.     

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。     

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.     

Type IV-A choledochal cysts: a challenge

Background/Purpose

Type IV-A choledochal cysts are characterized by congenital cystic dilatation of the biliary tree extending to involve the intrahepatic biliary channels also. A single-center experience of the management of type IV-A choledochal cysts is presented.

Methods

Thirty-five out of 105 (33%) patients with choledochal cysts, who underwent surgery at a tertiary care center in northern India from January 1989 to December 2002, were found to have a type IV-A (Todani’s classification) cyst. The mean age of the patients was 24 years (range, 3 months to 60 years); 17 patients in the group were adults and 22 were females. Presenting features were abdominal pain, jaundice, cholangitis, and abdominal lump, in various combinations.

Results

Excision of the extrahepatic part of the cyst and a wide bilio-enteric anastomosis was achieved in 32 (91%) patients, while internal drainage of the cyst was necessitated in 3 patients, for technical reasons: collaterals due to portal hypertension (1 patient) and dense adhesions (2 patients). Six (17%) patients developed postoperative complications: 3 had bilio-enteric anastomosis leaks, with 2 requiring a percutaneous proximal biliary diversion; 2 had intraabdominal bleeds requiring re-exploration, and 1 had external pancreatic fistula that closed spontaneously. Follow-up information was available for 28 (80%) patients. Mean duration of follow up was 25 months (median, 12 months; range, 6 months to 9 years). Three patients required re-operation, for anastomotic stricture (n = 2) and hepatolithiasis and recurrent cholangitis (n = 1) during follow up.

Conclusions

Excision of the extrahepatic part of the cyst and drainage of the intrahepatic part by a wide hilar or subhilar anastomosis gave satisfactory results in the majority of patients with type IV-A choledochal cysts. Close long-term follow up of these patients is essential, because they are likely to present with complications related to the residual intrahepatic part of the disease.     

完全腹腔镜胆总管囊肿切除术和不同胆道重建术临床效果研究

目的 分析比较完全腹腔镜胆总管囊肿切除术联合不同胆道重建术治疗先天性胆总管囊肿(CCC)患者的临床效果。方法 2016年1月～2020年5月我院诊治的81例CCC患儿,接受腹腔镜胆总管囊肿切除术,在胆管重建时,其中28例(A组)接受肝管十二指肠吻合术(HA),30例(B组)接受肝管空肠Roux-en-Y吻合术(RH),23例(C组)接受改良胆管袢式吻合术(MCL)。结果 在术后3个月,C组临床显效率为69.6%,显著高于A组的35.7%或B组的43.3%(P<0.05);在术后3 d, C组血清CRP水平为(12.9±2.4)mg/L,显著低于A组【(14.6±2.9)mg/L,P<0.05】或B组【(16.7±2.8)mg/L,P<0.05】;术后,三组胆漏、胰漏、胆道出血、急性胆管炎和腹腔积液等并发症发生率分别为21.4%、16.7%和8.7%,无显著性差异(P>0.05)。结论 采用MCL吻合术治疗CCC患者总体临床疗效优于HA吻合术或RH吻合术,在手术过程中不游离空肠,可完全于腹腔镜下实施操作,创面小,能显著缩短手术时长,有利于肠蠕动恢复,减轻炎症反应,...     

Management of a ruptured bile duct cyst

A case of a ruptured bile duct cyst in a 25-year-old male patient is presented. The initial management of the clinical presentation of acute abdomen consisted of an exploratory laparotomy and a T-tube cystostomy of a choledochal cyst. Two months later, he was admitted to our surgical department. Preoperative evaluation showed a type IV-A choledochal cyst. The patient underwent excision of the choledochal cyst, cholecystectomy, and the construction of a Roux-en-Y end-to-side hepaticojejunostomy.