Assessment of a postoperative anastomotic stricture following correction surgery of a type IVa choledochal cyst using Gd-EOB-DTPA-enhanced magnetic resonance cholangiography

Choledochal cyst is a relatively uncommon disease which is characterized by congenital dilatation of the intra and/or extrahepatic part of the biliary tree. Type IVa choledochal cysts are managed surgically through total excision of the entire extrahepatic part of the abnormal bile ducts and a simultaneous hepaticoenterostomy. Postoperative anastomotic stricture after excision of choledochal cysts and hepaticojejunostomy is a well-known late complication. We report a case of a 17-year-old female in whom gadoxetic acid-enhanced magnetic resonance cholangiography assisted in the evaluation of a biliary stricture following bile duct procedures after choledochal cyst correction surgery.     

Diagnosis and management of choledochal cysts

BackgroundCholedochal cysts (CCs) are rare cystic dilatations of the intrahepatic and/or extrahepatic bile ducts. We review the pathophysiology, diagnosis, and management of CCs.MethodsMEDLINE/PubMed and Web of Science databases were queried for “choledochal cyst”, “bile duct cyst”, “choledochocele”, and “Caroli disease”. Data were synthesized and systematically reviewed.ResultsClassified according to the Todani Classification, CCs are generally believed to arise secondary to reflux of pancreatic enzymes into the biliary tree due to anomalous pancreaticobiliary duct union. Complications of CCs include abdominal pain, jaundice, cystolithiasis, cholecystitis, pancreatitis, liver abscess, liver cirrhosis and malignant transformation (3–7.5%). Radiological and endoscopic imaging is the cornerstone of CC diagnosis and full delineation of cyst anatomy is imperative for proper management. Management is generally guided by cyst classification with complete cyst excision necessary for CCs with high potential of malignant transformation such as types I and IV. 5-year overall survival after choledochal cyst excision is 95.5%.ConclusionMost CCs should undergo surgical intervention to mitigate the risk of cyst related complications such as cholangitis and malignant transformation.     

Angeborene Anomalien und Malformationen der Gallenwege

Congenital anomalies and malformations may affect the intra- and/or extrahepatic bile duct system and present with different symptoms and impact depending on the specific type. Mere anomalies of the bile duct system are common and are generally asymptomatic but may cause substantial problems during hepatobiliary surgery. Severe malformations such as complete or partial biliary atresia are rare and present with prolonged and progressive icterus in the neonatal period requiring surgical reconstruction of the bile duct system or even liver transplantation. Fibrocystic diseases affecting the liver are caused by ductal plate malformations and are characterized by dilation of the intrahepatic bile duct system and focal liver fibrosis. Autosomal recessive and dominant polycystic kidney disease, progressive destructive cholangitis, congenital hepatic fibrosis, Caroli disease and biliary (von Meyenburg complex) as well as mesenchymal hamartomas belong to this heterogeneous group of disorders. Choledochal cysts are usually diagnosed in childhood; however, in about 20?% of patients they are detected in adulthood. According to Todani’s classification they are graduated into 5 categories. Choledochal cysts are common in Asia and show a high prevalence in Japan. An anomalous long common orifice of the extrahepatic bile and pancreatic duct is considered an important pathoanatomical substrate. Adults mainly suffer from symptoms due to cholelithiasis, pancreatitis, biliary cirrhosis and carry a high risk for developing cholangiocarcinoma. Therefore, early and complete excision of the choledochal cysts and long-term surveillance are warranted.     

Outcomes of choledochal cysts with or without intrahepatic involvement in children after extrahepatic cyst excision and Roux-en-Y hepaticojejunostomy

Background. Type I and type IV-A choledochal cysts (CC) in Todani’s classification are the most frequent types of CC. Unlike type I cyst, in which the dilatation is confined to the extrahepatic bile duct, type IV-A affects both extra and intrahepatic ducts.Aim. To review our experience of complete cyst excision with Roux-en-Y hepaticojejunostomy for the treatment of type I and type IV-A CC in childhood, in order to better characterize these entities.Material and methods. Data was collected retrospectively from a cohort of children who underwent cyst resection for CC from 1989 to 2011 in our institution.Results. Twelve patients were submitted to surgical excision of extrahepatic cyst and hepaticojejunostomy for treatment of type I (n = 6) and type IV-A (n = 6) cysts, with a complication rate of 25% (n = 3) and no mortality. Long term follow-up was available in 92% of patients, with a median of 10 years (2-22 years). Morbidities consisted of bile leak (2 patients) and late-onset cholestasis (1 patient); two of these required anastomotic revision. The results did not reveal any significant differences between the groups regarding postoperative outcomes (P > 0.05). Preoperative intrahepatic dilatation was found to permanently vanish in 83% of patients diagnosed with type IV-A cyst after operative repair.Conclusions. Intrahepatic dilatation of type IV-A cyst in children did not adversely affect the postoperative outcome after conventional surgical repair. This operative approach was effective in the management of type I and type IV-A cysts.     

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.     

Extrahepatic cyst excision and partial hepatectomy for Todani type IV-A cysts

Background

Extrahepatic cyst excision and Roux-en-Y hepaticojejunostomy is not satisfactory in many patients with complex Todani type IV-A choledochal cysts.

Aims

To report the results of combined extrahepatic cyst excision, partial hepatectomy, and Roux-en-Y hepaticojejunostomy for type IV-A choledochal cysts.

Methods

The records of patients who received extrahepatic cyst excision, partial hepatectomy, and Roux-en-Y hepaticojejunostomy for type IV-A choledochal cysts from January 2002 to December 2011 were retrospectively reviewed, and surgical outcomes analysed.

Results

59 patients (30.5% males; mean age, 43.2 ± 18.4 years) were included. Radical excision of cystically dilated bile ducts was achieved in 53 patients (89.8%). Bile leakage, delayed wound healing, and abdominal infection occurred in 5 (8.47%), 7 (11.86%), and 3 (5.08%) patients, respectively. Forty-nine patients (83.1%) were followed for an average of 42.6 ± 15.3 months. During the follow-up, 6 patients (12.2%) experienced recurrent cholangitis. Long-term biliary function was excellent in 33 (67.4%), good in 9 (18.4%), fair in 5 (10.2%), and poor in 2 (4.1%) patients.

Conclusion

Combined extra-hepatic cyst excision, partial hepatectomy, and Roux-en-Y hepaticojejunostomy is effective for the treatment of complex Todani type IV-A choledochal cysts with substantial intrahepatic bile duct involvement and hilar bile duct stenosis.     

Biliary cystic disease

Biliary cystic disease, though uncommon, can present at a wide range of ages with a wide range of symptoms. Choledochal cysts are associated with the development of both cholangiocarcinoma and gallbladder cancer. Thus, most biliary cystic disease is best managed operatively. Many factors should be considered when performing surgery on patients with choledochal cysts, including age, presenting symptoms, cyst type, associated biliary stones, prior biliary surgery, intrahepatic strictures, hepatic atrophy/hypertrophy, biliary cirrhosis, portal hypertension, and associated biliary malignancy. When feasible, surgical treatment should consist of cholecystectomy and complete surgical excision of extrahepatic cysts with Roux-en-Y reconstruction. Because the risk of recurrent cholangitis is significant and additional symptoms and problems are common, the use of long-term soft Silastic biliary stents (Dow Corning Corp., Midland, MI) should be considered when complex intrahepatic and extrahepatic cystic disease is present. Alternatively, the Roux-en-Y jejunal limb can be marked at the fascia for future percutaneous access. Reconstruction via hepaticoduodenostomy and jejunal interposition has been associated with increased postoperative pain due to bile reflux gastritis. Thus, hepaticojejunostomy reconstruction is recommended. For choledochal cysts involving the distal bile duct, the bile duct should be excised at the intrapancreatic portion. Resection of the pancreatic head should be reserved for patients with established malignancies. Surgical excision of the intrahepatic portion of the bile duct should be individualized to include preservation of hepatic parenchyma when the liver is not cirrhotic. If cirrhosis is advanced, hepatic transplantation may be indicated, but this is rare. Oncologic principles should be followed in the presence of a malignancy. Lifelong follow-up is required because of the possibility of a "field" defect increasing susceptibility to cancer throughout the biliary tract epithelium.     

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.     

Computed tomography virtual endoscopy with angiographic imaging for the treatment of type IV-A choledochal cyst

Type IV-A choledochal cysts (CCs) are a congenital biliary anomaly which involve dilatation of the extrahepatic and intrahepatic bile ducts. We present the case of a 30-year-old woman with type IV-A CC, on whom three-dimensional computed tomography (3D CT) and virtual endoscopy were performed. 3D CT revealed partial dilatation in the posterior branch of the intrahepatic bile duct and a relative stricture between it and the extrahepatic bile duct. Virtual endoscopy showed that this stricture was membrane-like and separated from the surrounding blood vessels. Based on these image findings, complete cyst resection, bile duct plasty for the stricture, and hepaticojejunostomy were safely performed. To the best of our knowledge, there are no reports of imaging by virtual endoscopy of the biliary tract which show the surrounding blood vessels running along the bile duct.     

Choledochal Cysts : A Review of Literature

Choledochal cysts are cystic dilation of extrahepatic duct, intrahepatic duct, or both that may result in significant morbidity and mortality, unless identified early and managed appropriately. The incidence is common in Asian population compared with western counterpart with more than two third of the cases in Asia being reported from Japan. The traditional anatomic classification system is under debate with more focus on etiopathogenesis and other aspects of choledochal cysts. Even though categorized under the same roof, choledochal cysts vary with respect to their natural course, complications, and management. In this review, with the available literature on choledochal cysts, we discuss different views about the etiopathogenesis along with the natural course, complications, diagnosis, and surgical approach for choledochal cysts, which also explains why the traditional classification is questioned by some authors.     

A case of intrahepatic cholangiocarcinoma associated with Type IV choledochal cyst

Anomalous union of the pancreaticobiliary duct (AUPBD) is a congenital anomaly that is defined as a junction of the bile duct and pancreatic duct outside the duodenal wall. This anomaly results in a loss of normal sphincteric mechanisms at the pancreaticobiliary junction. As a result, regurgitation of pancreatic juice into the biliary system develops and causes choledochal cysts, choledocholithiasis, cholangitis, pancreatitis and malignancy of the biliary tract. Gallbladder cancer or common bile duct cancer associated with AUPBD and choledochal cysts have been frequently reported. But, intrahepatic cholangiocarcinoma associated with this condition has been only rarely reported. Here, we report a case of intrahepatic cholangiocarcinoma associated with AUPBD and choledochal cyst.     

Choledochocele imaged with magnetic resonance cholangiography

Choledochal cysts are rare developmental malformations of the biliary tree. Percutaneous and endoscopic ultrasound, as well as endoscopic retrograde cholangiopancreatography, are recommended diagnostic tools. Magnetic resonance cholangiography may also contribute to the workup and treatment plan of patients with choledochal cysts. We report a 25-yr-old white woman with episodic nausea and moderate epigastric discomfort. Magnetic resonance cholangiography showed a rather spherical, cyst-like, fluid-filled structure in continuity with the prepapillary segment of the common bile duct, thus making a choledochocele (type III choledochal cyst) likely. The patient was treated by endoscopic sphincterotomy and, after a 6-month follow-up, is without complaints.     

A case of choledochal cyst associated with adenocarcinoma exhibiting sarcomatous features

One complication of choledochal cyst in adulthood is the development of secondary carcinoma, usually well-differentiated adenocarcinoma. We report a case of extrahepatic bile duct carcinoma associated with choledochal cyst and presenting predominantly sarcomatous features. The patient was a 52-year-old female who presented with abdominal fullness and pain. Ultrasound (US), computed tomography (CT), and cholangiography revealed a choledochal cyst with a tumor. The resected choledochal cyst was 10×7.5 cm and contained a protruding, ulcerated tumor, measuring about 4.0×2.5 cm. Histologically, the tumor was composed of spindle-shaped or fusiform cells with occasional pleomorphic or bizarre giant cells and abundant fibrous stroma, similar to malignant mesenchymal tumors. However, further histologic and immunohistochemical examination revealed that the tumor was adenocarcinoma with prominent sarcomatous features. To our knowledge, sarcomatous change in extrahepatic bile duct carcinoma associated with choledochal cyst has not been previously described.     

Squamous Cell Carcinoma Arising Within a Choledochal Cyst

Choledochal cysts are rare congenital or acquired cystic dilatations of the intra- or extrahepatic bile ducts. Their exact pathophysiology remains unclear. Extensive complications exist with untreated cysts. including biliary stasis and inflammation, with resultant stricture and stone formation, cholangitis, pancreatitis, and malignant degeneration. Most commonly reported malignancies include cholangiocarcinoma, adenocarcinoma, and gallbladder cancer. We report the case of a 41-year-old female with a history of chronic calcific pancreatitis, choledocholithiasis, and a large type I choledochal cyst, who presented with flu-like symptoms followed by painless jaundice and weight loss. Endoscopic retrograde cholangiopancreatography and direct cholangioscopy revealed a mass at the biliary bifurcation, a 4-cm choledochal cyst with multiple calculi, absence of anomalous pancreaticobiliary ductal union, and multiple calcifications in the pancreatic head. Pathology demonstrated a synchronous choledochal cyst and squamous cell carcinoma, an infrequently reported phenomenon. We report the associated rare finding of chronic calcific pancreatitis, without anomaly of the pancreatic biliary junction. This suggests that the formation of calculi within the choledochal cyst may contribute to chronic calcific pancreatitis, possibly by virtue of papillary stenosis.     

Histological features of precancerous and early cancerous lesions of biliary tract carcinoma

Biliary tract carcinoma develops within the intrahepatic or extrahepatic biliary tree and gallbladder. Primary sclerosing cholangitis, hepatolithiasis, congenital choledochal cyst, liver fluke infection, pancreatobiliary maljunction, toxic exposures and hepatitis virus infection are risk factors for the development of human biliary carcinoma. The precise molecular abnormalities of biliary carcinogenesis are still unknown, but chronic inflammatory conditions induce the production of reactive oxygen or nitrogen species leading to DNA damage. Recent studies indicate that cholangiocarcinoma of the large bile duct may arise in premalignant lesions such as biliary intraepithelial neoplasm (BilIN) and intraductal papillary neoplasm of the bile duct (IPNB). BilIN and IPNB are generally confined to the large and septal‐sized bile duct. BilINs are occasionally observed in non‐biliary liver cirrhosis as well as chronic biliary disease. In contrast, the precursor lesion of intrahepatic cholangiocarcinoma of the small bile duct type remains unclear. We herein demonstrated the histological characteristics of different tumor development pathways from premalignant lesion to carcinoma in different sites of the biliary tree.     

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.     

Increased secondary bile acids in a choledochal cyst. Possible role in biliary metaplasia and carcinoma

Choledochal cysts are uncommon congenital or acquired lesions of the biliary tree. The incidence of biliary tract carcinoma in patients with choledochal cysts is 5-35 times greater than that of the general population. Factors responsible for the increased risk of carcinoma are unknown. The case of a young woman who underwent excision of a choledochal cyst 16 years after initial diagnosis and treatment by choledochocystduodenostomy is reported. Metaplasia of the epithelial lining of the cyst was found in the resected specimen. The relative composition of bile acids in cyst contents was as follows: lithocholate, 2%; deoxycholate, 88%; chenodeoxycholate, 5%; and cholate, 5%. Virtually all bile acids were recovered in unconjugated form. In contrast, the bile acid composition of hepatic bile was as follows: lithocholate, 0%; deoxycholate, 34%; chenodeoxycholate, 43%; and cholate, 23%. Bile acids were fully conjugated. These data suggest that stasis of bile within choledochal cysts contributes to bacterial overgrowth and generation of unconjugated secondary bile acids.     

Clinical aspects of congenital cystic dilatation of the common bile duct in adults

We reviewed the data for 155 patients with congenital cystic dilatation of the common bile duct (CCDB; Alonso-Lej Type I) treated at our institute in the past 12 years. Our definition of CCDB was segmental enlargement of the common bile duct or enlargement involving the proximal bile duct, visualized on cholangiograms. CCDB was subclassified as infant or adult type cyst, based on the extent of the enlargement and the clinicopathologic findings. The former is typically an enlargement of the common bile duct or an enlargement involving the common hepatic duct, while the latter is an enlargement of the entirety of the extrahepatic ducts or involves the intrahepatic bile ducts. Of the 155 patients, 5 (including 1 child) had infant type cysts and 150 (including 1 child) had adult type cysts; 125 patients had benign CCDB (3 with infant type cyst) and 30 exhibited CCDB biliary malignancy (2 with infant type). Anomalous arrangement of the pancreaticobiliary ductal system (AAPB) and congenital biliary stricture were frequently associated with CCDB and these features played an important role in the presenting symptoms of CCDB: AAPB was detected in 100 of the 155 patients, and stricture in 51. AAPB caused disturbance of the passage of bile from the terminal bile duct to the duodenum and cholestasis in the enlargement, with reciprocal regurgitation of bile into the pancreas and pancreatic juice into the bile duct, and the AAPB appeared to precede obstructive jaundice, acute pancreatitis, or biliary malignancies. Stricture was observed at one to all of six sites in the hepatic hilum. Intrahepatic stones were seen in 30 patients, and intrahepatic bile duct cancer was seen in 8 patients; in all these 8 patients, the cancer was associated with primary intrahepatic stones.     

Smallest Todani’s type II choledochal cyst

A choledochal cyst is defined as an isolated or combined congenital dilation of the extra hepatic or intrahepatic biliary tree. Todani and colleagues proposed the five types of congenital choledochal cysts which have gained widespread acceptance. Type II choledochal cyst, a diverticulum of common bile duct, is rarest, and most reported cases of Type II were as large as several centimeters in size. We herein report the case of a small Type II choledochal cyst which was resected at pancreatoduodenectomy for carcinoma of the papilla of Vater. A 58-year-old Japanese male was referred to our hospital for the evaluation of jaundice. Preoperative cholangiogram via the percutaneous transhepatic biliary drainage tube revealed a complete obstruction at the narrow terminal segment. Furthermore, a small diverticular protrusion was demonstrated on the lower part of the common bile duct. The resected specimen showed a 2.2 x 1.7 x 1.2 cm carcinoma of the major papilla, and a deep, 2 mm in diameter and 5 mm in depth, depression on the posterior wall of the common bile duct. The anomalous pancreatobiliary duct was not seen. The deep depression was confirmed microscopically to penetrate the fibromuscular layer of the common bile duct and diagnosed as a Todani's Type II choledochal cyst. To our knowledge, the current case is the smallest Type II choledochal cyst which was completely resected.     

Choledochal cyst during pregnancy: case report and literature review of treatment

Choledochal cysts are uncommon conditions, usually diagnosed during childhood, but rarely during pregnancy. Choledochal cysts during pregnancy carry several risks, including development of biliary tract cancer and peritonitis due to rupture of dilated cysts induced by pregnancy itself. We present here a case of choledochal cyst during pregnancy, and discuss appropriate treatments for choledochal cysts first presenting during pregnancy. A 25-year-old primigravida at 15 weeks’ gestation was admitted to our hospital with abdominal pain. Magnetic resonance cholangiopancreatography diagnosed a type 1 choledochal cyst without a mass lesion. A healthy baby arrived without complication at 38 weeks’ gestation. The patient underwent cholecystectomy and choledochal cyst excision. The postoperative course was uneventful with discharge on day 8. Pathological examination diagnosed no malignant feature. Surgery may be performed after delivery for choledochal cysts first presenting during pregnancy, and monitoring with magnetic resonance imaging, blood tests, and ultrasonography is necessary during observation.