Familial occurrence of congenital bile duct cysts

Congenital bile duct cysts are now a well-documented anomaly of the biliary tree, and have become more common in Japan. Familial occurrence of congenital bile duct cysts, however, is extremely rare, with only six reported cases in the literature. We report a familial pattern of congenital bile duct cysts in a mother and her daughter. A 33-year-old female was admitted for evaluation of right upper quadrant abdominal pain and fever 6 days after an uneventful delivery of her second child. A com- puted tomography (CT) and ultrasound scan (US) revealed an obstructed biliary tract. Percutaneous transhepatic biliary drainage was then performed, and a cholangiogram revealed a Scholtz type B choledochocele without an anomalous connection of the pancreaticobiliary ducts. Endoscopic US demonstrated that the choledochocele was associated with a stone in the cyst. A pylorus-preserving pancreatoduodenal resection was performed, and a histological study revealed that the choledochocele was lined by biliary mucosa without evidence of malignancy. The newborn infant had an abdominal tumour. An US and CT revealed a congenital bile duct cyst. An operation was performed and the intraoperative cholangiogram showed an Alonso-Lej type I congenital bile duct cyst with an anomalous connection of the pancreaticobiliary ducts. Whether congenital bile duct cysts are hereditary remains to be elucidated.     

Double common bile duct： A case report

Double common bile duct （DCBD） is a rare congenital anomaly in which two common bile ducts exist. One usually has normal drainage into the papilla duodeni major and the other usually named accessory common bile duct （ACBD） opens in different parts of upper gastrointestinal tract （stomach, duodenum, ductus pancreaticus or septum）. This anomaly is of great importance since it is often associated with biliary lithiasis, choledochal cyst, anomalous pancreaticobiliary junction （APBJ） and upper gastrointestinal tract malignancies. We recently recognized a rare case of DCBD associated with APB3 with lithiasis in better developed common bile duct. The opening site of ACBD was in the pancreatic duct. The anomaly was suspected by transabdominal ultrasonography and finally confirmed by endoscopic retrograde cholangiopancreatography （ERCP） followed by endoscopic sphincterotomy and stone extraction. According to the literature, the existence of DCBD with the opening of ACBD in the pancreatic duct is most frequently associated with APB3 and gallbladder carcinoma. In case of DCBD, the opening site of ACBD is of greatest clinical importance because of its close implications with concomitant pathology. The adequate diagnosis of this rare anomaly is significant since the operative complications may occur in cases with DCBD which is not recognized prior to surgical treatment.     

Anomalous connection of the right hepatic duct into the cystic duct: utility of magnetic resonance cholangiopancreatography

A 41-year-old female was admitted to our hospital for treatment of uterus carcinoma. Abdominal ultrasound showed gallbladder stones. Although magnetic resonance cholangiopancreatography revealed the right intrahepatic bile ducts, left hepatic duct and the common bile duct, the confluence of the right and left hepatic ducts was not visualized. At surgery, intra-operative cholangiography showed a biliary anomaly of the right hepatic duct entering the cystic duct. Subsequently cholecystectomy was accomplished without any injury to the bile duct. Our case may be the eighth such case of this rare biliary anomaly. When magnetic resonance cholangiopancreatography does not show the confluence of the right and left hepatic ducts, biliary anomaly of the right hepatic duct should be suspected and careful dissection should be performed from the Hartman's pouch, followed by intraoperative cholangiography, in order to avoid unnecessary injury to the bile duct.     

Wire-guided intraductal US in the assessment of bile duct strictures with Mirizzi syndrome-like features at ERCP

BACKGROUND: Mirizzi syndrome refers to common hepatic duct obstruction caused by extrinsic compression that is usually from a stone impacted in the cystic duct. The utility of transpapillary intraductal US for assessment of biliary strictures with radiographic features of Mirizzi syndrome was studied retrospectively. METHODS: Intraductal US was performed in 16 patients with a common hepatic duct stricture caused by extrinsic compression and nonvisualization of the gallbladder by endoscopic retrograde cholangiography. An over-the-wire catheter US probe (20 MHz) was inserted into the bile duct. Intraductal US findings were compared with the final diagnoses at surgery. RESULTS: Intraductal US was successful in all patients. The extraluminal cause of the common hepatic duct stricture was detected in 14 patients (87.5%). In 9 patients, intraductal US detected an impacted stone outside the common hepatic duct. Intraductal US demonstrated extraluminal lesions without evidence of a stone in 5 patients, including a mass in 4 and asymmetrical, irregular thickening of the bile duct wall in 1 patient. In the remaining 2 patients, intraductal US demonstrated only a distended gallbladder. CONCLUSIONS: Transpapillary wire-guided intraductal US is useful for assessing biliary strictures with features that suggest Mirizzi syndrome and optimizes management of patients with these findings.     

Agenesis of gallbladder and multiple anomalies of the biliary tree in a patient with portal thrombosis: a case report.

A 55-year-old male patient was admitted to hospital because of splenomegaly. Abdominal ultrasonography and computed tomography failed to demonstrate the gallbladder. The diagnosis of right portal vein thrombosis was established by Doppler ultrasonography, splenoportography and computed tomography angiography. To investigate the biliary tree and evaluate the effect on the biliary tree of portal changes, endoscopic retrograde cholangiopancreatography was performed. Endoscopic retrograde cholangiopancreatography study revealed the absence of gallbladder, cystic duct and common bile duct together with the junction of the right and left hepatic ducts at the pancreatic head, with predominant left hepatic duct. To our knowledge, this is the first reported case of multiple congenital anomalies of the extrahepatic biliary tree associated with right portal vein thrombosis. The presence of these rare pathologies in two viscera running together in a patient with right portal vein thrombosis is a very rare condition.     

Right hepatic duct opening into the cystic duct: the role of pre- and intraoperative cholangiography

Although an aberrant hepatic duct entering the cystic duct is not especially rare, the main right hepatic duct entering the cystic duct is extremely rare. A 69-year-old woman developed severe intermittent right upper quadrant pain and high fever. A diagnosis of acute calculus cholecystitis was made by radiographic examinations. Magnetic resonance cholangiopancreatography demonstrated dilatation of the right hepatic duct, but could not identify the junction of the right hepatic duct and the cystic duct. Endoscopic retrograde cholangiopancreatography established that the right hepatic duct joined the cystic duct and that cholecystolithiasis was present. As the right hepatic duct entering the cystic duct can lead to ductal injury, this anomaly should be kept in mind when performing laparoscopic cholecystectomy. Pre- and intraoperative cholangiography contribute to the avoidance of iatrogenic bile duct injury. When the right hepatic duct drains into the cystic duct, the gallbladder should be removed distal to the junction of the hepatic and cystic ducts.     

Double cancer of the cystic duct and gallbladder associated with low junction of the cystic duct

We report a case of double cancer of the cystic duct and gallbladder associated with low junction of the cystic duct. A 73-year-old woman was admitted to the hospital complaining of upper abdominal pain. Endoscopic retrograde cholangiography showed a stenotic lesion in the lower common bile duct and no visualization of the cystic duct or gallbladder. Enhanced computed tomography revealed a heterogeneously enhanced tumorous lesion around the lower bile duct in the pancreatic head. A diagnosis of cancer arising from the cystic duct that entered the lower part of the common hepatic duct was made by intraductal ultrasonography, which showed an intraluminal protruding lesion in the cystic duct. Isolated gallbladder cancer was also diagnosed, by abdominal computed tomography. She underwent pancreaticoduodenectomy with dissection of regional lymph nodes. Histological examination revealed moderately differentiated adenocarcinoma of the cystic duct and well-differentiated adenocarcinoma of the gallbladder. Double cancer of the cystic duct and gallbladder is extremely rare, and this case also suggests a relationship between a low junction of the cystic duct and neoplasm in the biliary tract.     

Left-sided gallbladder associated with congenital liver cyst

Background. A left-sided gallbladder is a rare congenital anomaly defined as a gallbladder attached to the lower surface of the left lateral segment of the liver, i.e. to the left of the interlobar fissure and round ligament. Case outlines. In two women aged 42 and 70 years a left-sided gallbladder was associated with a congenital cyst of the liver. In the first patient, the ectopic gallbladder was an incidental finding at operation for a symptomatic liver cyst; as the gallbladder was normal it was not removed. The second patient underwent operation for chronic calculous cholecystitis, when the left-sided gallbladder and congenital liver cyst were found. An operative cholangiogram was normal, the cystic duct joining the common bile duct from the right side. The gallbladder was removed, and the cyst was de-roofed. Both patients had an uneventful recovery and remain symptom-free at 12 and 9 years respectively. Discussion. To the best of our knowledge, the association of these two congenital anomalies has not been described previously.     

Multiseptate gallbladder: Biliary manometry and scintigraphy

A 30-year-old man with multiseptate gallbladder, a very rare congenital anomaly, is presented. His presenting symptom was epigastric pain. A hypoplastic gallbladder with multiple septa was demonstrated by ultrasonography and endoscopic retrograde cholangiography. An injection of cerulein reproduced pain, and simultaneous biliary manometry and scintigraphy showed impairment of gallbladder filling and emptying. Laparoscopic cholecystectomy resulted in complete relief of the pain. Biliary manometry and scintigraphy are useful to determine the operative indication in a symptomatic patient with this entity.     

Gallbladder agenesis with a stone in the cystic duct bud

An 84-year-old woman was admitted to the hospital because of pyloric stenosis caused by gastric cancer. Abdominal computed tomography and magnetic resonance imaging failed to demonstrate the gallbladder, but showed a gallstone in a ductlike structure parallel to the common bile duct. When laparotomy was performed, the gallbladder and the fossa were not observed, and a blind-end duct, similar to a cystic duct, was found beside the common bile duct. Incisional exploration of the common bile duct was done after distal gastrectomy; the gallstone was not found in the common bile duct, but in the duct parallel to it. By observing the duct beneath the common bile duct with a cholangioscope, we considered it to be a hypoplastic cystic duct. After the gallstone was removed, a T-tube was placed into the common bile duct. Agenesis of the gallbladder is a rare congenital anomaly and is often asymptomatic. As far as we know, this is the first report of gallbladder agenesis with a hypoplastic cystic duct impacted with a stone. Careful intraoperative examination using a cholangioscope is useful to confirm the structure of the common bile duct.     

Carcinoma of the cystic duct

Two patients with carcinoma of the cystic duct presented with obstructive jaundice due to extrinsic compression of the common hepatic duct by the tumor. Sonography and computed tomography showed dilatation of the intrahepatic bile ducts and gallbladder. In one patient, a calculus seen in the gallbladder neck suggested Mirizzi syndrome. In the other, a small soft tissue mass was indistinguishable from a common duct tumor or an enlarged lymph node. In both cases, direct cholangiography demonstrated extrinsic compression and displacement of the common duct with proximal biliary dilatation and nonvisualization of the gallbladder. Carcinoma of the cystic duct should be considered whenever there is evidence of cystic duct obstruction and/or when cholangiography shows extrinsic mass effect on the common duct.     

Congenital left intrahepatic bile duct draining into gastric wall mimicking biliary reflux gastritis

Abnormalities and variations of the biliary ducts are not rare.Most aberrant bile ducts eventually drain into the descending part of duodenum through the papilla of vater.However,drainage of the left hepatic bile duct into the stomach is extremely rare.A 29-year old man was admitted to the hospital with the diagnosis of biliary reflux gastritis.Comprehensive imaging modalities were performed including electronic endoscopy,endoscopic ultrasonography,endoscopic retrograde cholangiopancreatography and magnetic resonance cholangio-pancreatography.Finally,congenital ectopic left intrahepatic bile duct draining into the stomach was found,which caused biliary reflux gastritis.The patient did not receive any surgery.Good recovery was achieved by medical treatment.     

A Patient with a Left Accessory Hepatic Duct with Gallstones

Abstract: Accessory hepatic ducts, especially the left ducts, are relatively rare anomalies of the biliary tract. We present here a patient with this anomaly complicated by the presence of multiple stones. Endoscopic retrograde cholangiography (ERC) and ultrasound (US) were very useful in establishing the diagnosis preoperatively and in determining our surgical strategy. ERC demonstrated the left accessory hepatic duct with multiple radiolucent stones at the level of the cystic duct on the opposite side of the gallbladder. US demonstrated a hyperechoic mass measuring 15×10 mm with an acoustic shadow at the hepatic hilus. It also showed the internal “honey-comb” structure of the mass which contained numerous stones measuring 5–6 mm in diameter and a fine tubular structure between the accessory hepatic duct and the caudate lobe. Intraoperatively the sac-like, dilated (35×10 mm), left accessory hepatic duct was filled with numerous bilirubin stones originating at the cystic duct from its contralateral side. A few fine bile ducts communicated with the accessory hepatic duct and the caudate lobe.     

Mirizzi syndrome Type IV: A rare entity

Mirizzi's syndrome, characterized by obstructive jaundice due to an extrinsic compression of common hepatic duct by an impacted gallstone in the cystic duct or the neck of the gallbladder, is a rare complication of gallstone disease. The present case describes Mirizzi's syndrome classified as Type IV in a 50‐year‐old man with obstructive jaundice. Abdominal computed tomography scan demonstrated a dilated intrahepatic biliary tree and a tumoral mass at the porta hepatis, suggesting cholangiocarcinoma. Endoscopic retrograde cholangiopancreatography also suggested cholangiocarcinoma involving the entire circumference of the common hepatic duct in porta hepatis. The diagnosis of Mirizzi's syndrome Type IV was confirmed during cholecystectomy, withdrawal of gallstone and Roux‐en‐Y hepaticojejunostomy.     

Asymptomatic adenomyoma of the common hepatic duct discovered during a medical checkup: report of a case

Although adenomyoma of the gallbladder is not so rare, adenomyoma arising in the bile duct is extremely rare. We herein report a case of partial biliary obstruction due to adenomyoma of the common hepatic duct. A 64-year-old woman was referred to the 1st Department of Surgery, Niigata University School of Medicine for surgical intervention, in whom a biliary stricture in the common hepatic duct was discovered incidentally during a medical check-up for hyperamylasemia in an affiliated hospital. She was asymptomatic without jaundice. Based on her past history of abdominal contusion, the insidious presentation and the smooth biliary stricture on cholangiography, a diagnosis of benign biliary stricture secondary to blunt abdominal trauma was made. A suspicion of malignancy, however, could not be ruled out. Subsequently, she underwent a resection of the extrahepatic bile duct with lymph node dissection in the hepatoduodenal ligament. The patient's postoperative course was uneventful. She was discharged 18 days after the operation. Histology of the resected specimen confirmed the diagnosis of adenomyoma in the common hepatic duct. Clinicians should note to avoid overlooking or misdiagnosing that adenomyoma is a cause of biliary obstruction, although extremely rare.     

A case of septum formation of the common hepatic duct combined with an anomalous hepatic duct of the caudate lobe

A 66-year-old Japanese woman was admitted to our hospital with upper right quadrant pain and jaundice. Laboratory data indicated the presence of obstructive jaundice. A diagnosis of cholecystocholedocholithiasis was made by ultrasonography. Endoscopic retrograde cholangiography revealed choledochal stones with septum formation in a portion of the common hepatic duct up to where an anomalous bile duct drained from the caudate lobe of the liver. We performed a cholecystectomy, T-tube drainage, and partial resection of the septum when these findings were confirmed at surgery. Histological examination of the removed septum showed fibrous stroma under atrophic mucosa. To our knowledge, this is the eighteenth reported case in Japan of septum formation of the bile duct. However, no other such case to date has been seen combined with an anomalous hepatic duct of the caudate lobe of the liver. Selective cholangiography by choledochoscope and computed tomography with a thin guide-wire inserted to the bile duct were useful in confirming the origin of the anomalous bile duct. This case may serve as further evidence of the assumption that septum formation of the common (hepatic) bile duct is a malformation of congenital origin. An erratum to this article is available at .     

A case of primary mucoepidermoid carcinoma arising from the common bile duct

Mucoepidermoid carcinoma of the bile duct is an extremely rare tumor. Seventeen cases originating from intrahepatic bile duct and 2 cases from common hepatic duct have been reported in the English literature. Mucoepidermoid carcinoma arising from the common bile duct has not been previously reported. A 68 year-old man was admitted due to obstructive jaundice. Computed tomography showed a malignant tumor of the common bile duct located in the intrapancreatic segment. Filling defects of the distal common bile duct was seen on endoscopic retrograde cholangiogram. Under the impression of bile duct cancer, pylorus-preserving pancreatoduodenectomy was performed. Histologic diagnosis of the resected specimen was mucoepidermoid carcinoma of the common bile duct. After surgery, the patient received concurrent chemoradiotherapy, and planned to receive additional chemotherapy. We herein report on a first case of primary mucoepidermoid carcinoma of the common bile duct, and review the literature.     

A case of primary small cell carcinoma arising from the common bile duct]

Small cell carcinoma is usually seen in the lung, but rarely involves the gastrointestinal tract including biliary tract. A 65 year-old man was admitted because of obstructive jaundice. A smooth-surfaced round intraluminal mass with proximal bile duct dilatation was seen in the proximal common bile duct on endoscopic retrograde cholangiogram. Under the diagnosis of bile duct cancer, pylorus-preserving pancreatoduodenectomy was done. Pathology revealed a 2 cm sized small cell carcinoma in the proximal common bile duct and distal common hepatic duct. On immunohistochemical stain, the tumor cells were positive for neuroendocrine markers CD56 and synaptophysin. After surgery, the patient received 5 cycles of adjuvant chemotherapy with VIP (etoposide, ifosfamide, and cisplatin) regimen. However, the patient died of liver metastasis 12 months after the diagnosis. We report a case of extrapulmonary small cell carcinoma arising from the common bile duct.     

Congenital cystic dilatation of the common bile duct, anomaly of the biliopancreatic junction and cancer of the bile ducts

The choledochus and pancreatic duct ordinarily enter the duodenum either separately or via a common channel located in the duodenal wall. The usual maximal length of the common channel is approximately 0.5 cm. An anomalous junction of the pancreaticobiliary tract is defined by the presence of an unusually long common channel whose length is 1 cm or longer. During the last 8 years, we studied 9 cases of congenital dilatation of the bile duct. Besides the choledochal cyst, all patients had a common channel which measured 1.5 cm or longer. The mean age of patients (6 females, 3 males) was 36.5 years. Three patients had extrahepatic bile tract carcinoma. One patient, a 51-year old man, had gallbladder carcinoma without stones while the two other patients, a 43-year old woman and a 32-year old man, had carcinoma arising from the wall of the cystic dilatation. These two patients had undergone a simple internal drainage procedure 16 and 8 years before, respectively. Our study suggests that the anomalous junction of the pancreaticobiliary tract, an embryonic anomaly which allows the reflux of pancreatic juice into the bile duct and gallbladder, may be responsible for congenital bile duct dilatation and biliary tract carcinoma. Total excision of the cystic dilatation and the gallbladder with hepaticojejunostomy is the appropriate surgical treatment.     

Mirizzi syndrome

Opinion statement Mirizzi syndrome is an important complication of gallstone disease. If not recognized preoperatively, it can result in significant morbidity and mortality. Preoperative diagnosis may be difficult despite the availability of multiple imaging modalities. Ul-trasonography (US), CT, and magnetic resonance cholangiopancreatography (MRCP) are common initial tests for suspected Mirizzi syndrome. Typical findings on US suggestive of Mirizzi syndrome are a shrunken gallbladder, impacted stone(s) in the cystic duct, a dilated intrahepatic tree, and common hepatic duct with a normal-sized common bile duct. The main role of CT is to differentiate Mirizzi syndrome from a malignancy in the area of porta hepatis or in the liver. MRI and MRCP are increasingly playing an important role and have the additional advantage of showing the extent of inflammation around the gallbladder that can help in the differentiation of Mirizzi syndrome from other gallbladder pathologies such as gallbladder malignancy. Endoscopic retrograde cholangiopancreatography (ERCP) is the gold standard in the diagnosis of Mirizzi syndrome. It delineates the cause, level, and extent of biliary obstruction, as well as ductal abnormalities, including fistula. ERCP also offers a variety of therapeutic options, such as stone extraction and biliary stent placement. Percutaneous cholangiogram can provide information similar to ERCP; however, ERCP has an additional advantage of identifying a low-lying cystic duct that may be missed on percutaneous cholangiogram. Wire-guided intraductal US can provide high-resolution images of the biliary tract and adjacent structures. Treatment is primarily surgical. Open surgery is the current standard for managing patients with Mirizzi syndrome. Good short-and long-term results with low mortality and morbidity have been reported with open surgical management. Laparoscopic management is contraindicated in many patients because of the increased risk of morbidity and mortality associated with this approach. Endoscopic treatment may serve as an alternative in patients who are poor surgical candidates, such as elderly patients or those with multiple comorbidities. Endoscopic treatment also can serve as a temporizing measure to provide biliary drainage in preparation for an elective surgery.