A 15-year-old girl with recurrent upper abdominal pain was diagnosed with congenital biliary dilatation. Abdominal enhanced computed tomography (CT) showed the anterior segmental branch of the right hepatic artery (RHA) running across the ventral aspect of the dilated common hepatic duct (CHD). Laparoscopic extrahepatic dilated biliary duct excision and Roux-en-Y hepaticojejunostomy were planned. Intraoperatively, the dilated CHD was observed to bifurcate into the ventral and dorsal ducts, between which the anterior segmental branch of the RHA crossed through the CHD. The CHD rejoined on the distal side as one duct. We transected the CHD just above the cystic duct. The patency of the ventral and dorsal sides of the bifurcated CHD was confirmed. Laparoscopic hepaticojejunostomy was performed at the distal side of the rejoined CHD, without sacrificing the anterior segmental branch of the RHA. There was no postoperative blood flow impairment in the right hepatic lobe or anastomotic stenosis.

Congenital biliary dilatation (CBD) is a congenital malformation of focal dilatation of the extrahepatic bile ducts, including the common bile duct, and is often associated with pancreaticobiliary maljunction (PBM). In this article, we report a CBD case that presented with focal dilation of the common bile duct without PBM (Todani\'s classification type Ib). The patient was a 32-year-old man who visited a doctor with a chief complaint of abdominal distension. Computed tomography revealed cystic dilatation of the common bile duct, and the patient was referred to our institution. Magnetic resonance cholangiopancreatography showed cystic dilatation of the common bile duct with a maximum diameter of 7 cm; however, evaluating the presence of PBM was challenging. Endoscopic ultrasonography showed small gallstones and debris in the dilated common bile duct and no thickening of the gallbladder wall. Endoscopic retrograde cholangiopancreatography revealed no PBM or markedly elevated bile amylase levels. Based on these findings, the patient was diagnosed with Todani Type Ib CBD. Since this patient did not have pancreatobiliary reflux, it was unclear whether the risk of developing biliary tract cancer was high, and since the treatment was highly invasive, the decision was to follow up without surgical treatment.

Choledochocele is defined as a congenital dilatation of the distal intramural part of the common bile duct protruding into the wall of the descending duodenum, typically without pancreaticobiliary maljunction. However, some cases present with a similar pathophysiology to pancreaticobiliary maljunction, including reciprocal reflux of pancreatic juices and bile, leading to protein plugs, pancreatitis, and biliary tract carcinogenesis. Choledochocele is relatively rare and its anatomy, physiology, pathology, and clinical features are thus not well known. We describe a patient with choledochocele who suffered from repeated severe acute pancreatitis and underwent subtotal stomach-preserving pancreatoduodenectomy, in whom the pathological findings of choledochocele showed hyperplasia.

An otherwise healthy 45-year-old woman had been experiencing intermittent right upper abdominal pain for the past 1 year. Computed tomography showed pneumobilia and pancreatic duct emphysema despite a normal duodenal papilla. Magnetic resonance cholangiopancreatography and endoscopic ultrasound confirmed bile duct dilation but without a pancreaticobiliary maljunction. Duodenoscopy detected a slightly sunken, unfixed, and spontaneously enlarged duodenal papilla. During the cholangiogram, the Oddi sphincter was relaxed and the catheter could be easily inserted into the bile duct. Further, no findings suggestive of pancreaticobiliary maljunction were observed, and the contrast medium leaked spontaneously from the duodenal papilla. As biliary amylase level was high, we surmised the occurrence of occult pancreaticobiliary reflux due to relaxation of the Oddi sphincter. However, as there are no guidelines on the management of this condition, we did not offer any treatment. Nevertheless, the patient continued to experience similar symptoms and was retested 1 year later with similar results. As occult pancreaticobiliary reflux was reconfirmed, we suggested that the patient undergo laparoscopic extrahepatic bile duct resection and cholecystectomy, which is the standard treatment for pancreaticobiliary maljunction. Pathological evaluation revealed fibrous thickening of the bile duct wall and chronic cholecystitis, which are typical findings of pancreaticobiliary reflux. Even though pancreaticobiliary reflux is mainly observed in pancreaticobiliary maljunction, it has also been reported in normal patients. Here, we describe a novel mechanism of pancreaticobiliary reflux, namely, a relaxed or defective Oddi sphincter.

We report a case of hepatolithiasis 30 years after congenital biliary dilatation (CBD) surgery. A 31-year-old woman presented with fever and epigastric pain. She had a history of radical surgery for type I CBD at the age of 1 year and had no significant symptoms for approximately 30 years after surgery. Laboratory and imaging results showed hepatolithiasis at the common trunk of segments II and III with cholangitis. She was admitted to our hospital for antibiotics and underwent double-balloon endoscopic retrograde cholangiography (DBERC) to treat the hepatolithiasis. Roux-limb jejunum was perforated during DBERC; hence, emergent laparoscopic perforation site closure and simultaneous endoscopic lithotomy through the Roux-limb jejunum with exteriorization via umbilical incision were performed. The postoperative course was uneventful, and the patient was discharged without any complications.

BACKGROUND: An ectopic gallbladder is a rare anomaly and can result in the misinterpretation of imaging findings and clinical confusion. Knowledge of such anomalies facilitates accurate diagnoses and prompt management. We report a pediatric case of an ectopic gallbladder concomitant with congenital biliary dilatation (CBD).
METHODS: A 9-year-old girl was referred to our hospital for elevated liver enzyme levels. Following physical examination and a review of medical imaging findings, she was diagnosed with Todani type IV-A CBD. We could not visualize the gallbladder by abdominal ultrasonography, CT, and MRI scans; therefore, we suspected gallbladder agenesis. A laparoscopic excision of the extrahepatic bile duct was performed to treat the CBD. Neither a gallbladder nor a cystic duct were revealed on the liver undersurface. Therefore, gallbladder agenesis was considered as a diagnosis based on preoperative imaging and intraoperative findings. However, during dissection of the hepatic hilum, a cyst-like structure was found on the ventral side of the common hepatic duct, slightly to the right, and a small additional duct that originated from the cystic structure was found. Upon incision, a small amount of bile was drained from the small duct. Thus, the cystic structure was diagnosed as an ectopic gallbladder with hypoplasia. Following the removal of the ectopic gallbladder, the extrahepatic bile duct was excised. Subsequently, laparoscopic Roux-en-Y hepaticojejunostomy was performed without any complications. Postoperative histopathological evaluations of the resected specimen revealed Rokitansky-Aschoff sinuses in the resected cystic lesion. The pathological investigations confirmed the diagnosis of an ectopic gallbladder. Following an uneventful postoperative course, the patient was discharged on day nine.
CONCLUSIONS: To our knowledge, this is the first pediatric case report describing an ectopic gallbladder concomitant with CBD. If the gallbladder cannot be detected in a preoperative imaging examination, it is important to consider the possibility of an ectopic gallbladder.

The site of perforation is difficult to identify preoperatively in many cases with spontaneous perforation of congenital biliary dilatation (CBD). We report a case of spontaneous perforation of CBD in which the perforation site was identified preoperatively using thin-slice contrast-enhanced computed tomography (CT). The patient was a girl aged 1 year and 4 months. She was admitted to our hospital because of vomiting and diarrhea that had continued for 3 days prior to admission. Abdominal contrast CT on admission showed dilated common bile duct, thickening of the gall bladder wall, and marked ascites. In addition, an area of low density with a diameter of 1 cm was detected near the neck of the gallbladder. We evaluated the area via thin-slice contrast-enhanced CT and detected a defect in the wall of the bile duct. Cholangiography revealed abnormal confluence of the pancreaticobiliary duct and a protein plug in the common duct. A diagnosis of CBD with perforation of the bile duct was made, and surgery was performed. The intraoperative findings matched that seen on the enhanced CT. There are some reports of pseudocysts and fluid retention around the perforation site; however, no reports are found in which the perforation site was confirmed by preoperative CT. If localized fluid retention is observed in cases with biliary perforation, confirmation with thin-slice contrast-enhanced CT might be useful for identifying the perforation site.

A 34-year-old man presented to the emergency department with a chief complaint of epigastric pain. Endoscopic ultrasound detected a 5 mm stone in the common bile duct. After endoscopic sphincterotomy, the black stones and debris were removed with balloon catheter. Abdominal ultrasonography detected no gallbladder stones; hence, the patient was followed up. However, 3 months later, the patient again developed acute cholangitis caused by common bile duct stones and underwent endoscopic stone removal. Cholangiography under balloon occlusion revealed a left hepatic duct diverticulum with an internal defect. Intraductal ultrasonography showed a hyperechoic lesion with acoustic shadow in the diverticulum, suggesting a stone or debris. Therefore, the patient was considered to have had repeated acute cholangitis because of the presence of falling diverticular stones. The patient underwent left hemihepatectomy plus segmentectomy 1 and cholecystectomy. Histopathologically, it was a true diverticulum without internal epithelial atypia. Many debris were seen in the diverticulum. Gallbladder stones were not observed. Eventually, the patient was discharged from the hospital with no postoperative complications and no recurrence of cholangitis after 20 months.

OBJECTIVE: Pancreaticobiliary maljunction (PBM) is a malformation in which the pancreatic and bile ducts join outside the duodenal wall. It is associated with various biliary and pancreatic diseases. In addition, patients with PBM carry a substantial lifetime risk of developing biliary or gallbladder carcinoma. We aimed to present a multicenter case series of PBM from Turkey.
METHODS: This study was conducted in adult and pediatric PBM patients who were referred to three tertiary reference centers of Turkey for endoscopic retrograde cholangiopancreatography (ERCP) between July 2007 and May 2020. The clinical presentations, types of PBM, ERCP findings, surgical histories, and the postoperative courses, including the development of biliary malignancies, were retrospectively reviewed.
RESULTS: The study group included 47 (31 adult and 16 children) patients. Type D PBM was more frequent (13/41: 27.7%) than that reported in Eastern studies. Type A PBM was more common in the adults (51.6% vs. 12.5%, p < 0.05), whereas type C was more common in pediatric patients (31.3% vs. 13.2%, p < 0.05). Although fusiform anatomy was predominant in both of the groups, cystic dilatation was more common (25.8% vs. 12.5%) in adults and the common bile duct diameter was greater [22 mm (range 11-58) vs. 12 mm (range 5-33)] in adult patients compared to pediatric patients. Resective surgeries were more frequently done in pediatric patients (73.3% vs. 53.6%), whereas cholecystectomy was more frequently performed in adult patients (21.4% vs. 6.7%).
CONCLUSIONS: Although our findings were compatible with Eastern studies, type D PBM (associated with pancreas divisum) was more frequent in our study population.

UNASSIGNED: Pancreaticobiliary maljunction (PBM) is a congenital anomaly wherein the persistent reflux of the pancreatic juice into the biliary tract induces biliary tract cancer. The prediction criteria for gallbladder carcinogenesis have been reported previously through results obtained from examining carcinogenic and non-carcinogenic cases with the parameters that classified each confluent form in PBM. This study aimed to validate these previous study results and provide new recommendations for gallbladder carcinogenesis prevention.
UNASSIGNED: Twenty-four patients with PBM underwent hepaticojejunostomy. The prediction criteria for gallbladder carcinogenesis consist of three elements. The confluent forms that satisfied none or one of the three criteria were defined as a low score group, and those that satisfied two or three were defined as the high score group. Immunohistology and pathological search were performed on the gallbladders\' sections in both groups to evaluate chronic inflammation.
UNASSIGNED: The cases with dysplasia, positive Ki67 labeling index, and gallbladder cancer were more common in the high score group and tended to have more lymphocyte infiltration. These findings indicate that the degree of inflammation and cell proliferation might be more severe in the high score group than in the low score group.
UNASSIGNED: There is a close relationship between the confluent form and the histopathological findings of the gallbladder in patients with PBM. The confluent forms observed in the high score group might have an additional correlation with increased proliferation activity and subsequent malignant transformation of the gallbladder epithelium.