First report of surgery for congenital biliary dilatation using the hinotori™ Surgical Robot System (with video).

Robot-assisted surgery for congenital biliary dilatation has been evolving primarily with the da Vinci® Surgical System. The hinotori™ Surgical Robot System, developed in Japan, received approval for gastroenterological surgery in 2022. We present the inaugural case of congenital biliary dilatation surgery utilizing the hinotori™ system. A 57-year-old woman was referred to our institution for evaluation and treatment of common bile duct dilatation classified under Todani Type Ia congenital biliary dilatation. Robotic resection of the extrahepatic bile duct and hepaticojejunostomy with Roux-en-Y were performed. The operation lasted 292 min with minimal blood loss (10 mL). The patient had an uneventful postoperative course and was discharged 10 days after surgery. Robotic surgery using the hinotori™ system for congenital biliary dilatation can be safely performed.

Colangiografía: duplicación vesicular verdadera / Cholangiography: true gallbladder duplication / Colangiografia: duplicação verdadeira da vesícula biliar

Se presenta una variante anatómica de la vía biliar extrahepática, como lo es la verdadera duplicación vesicular. Las malformaciones congénitas vesiculares se categorizan en anormalidades posicionales y morfológicas, la duplicación vesicular está incluida en este último. La clasificación aceptada es la de Boyden. Basado en la relación del conducto cístico con el cuerpo vesicular describió la "vesica fellea divisa" (vesícula bilobulada que drena a un solo conducto cístico y comparte origen embriológico común) y "vesica fellea duplex" (verdadera duplicación vesicular, dos vesículas separadas con conductos císticos independientes, origen embriológico doble).

A proposed feasible classification of common bile duct duplications based on a newly described variant and review of existing literature.

BACKGROUND: Duplication of the common bile duct (CBD) is extremely rare among the anatomical variations in the biliary tract system, which presents a septum within the CBD or an accessory CBD. In our study, we report a rare case of duplication of the common bile duct combined congenital biliary dilatation.we present a rare case of a septum in the dilated biliary tract. CASE PRESENTATION: We reported a 5-year-old Asian girl who had history of repeated abdominal pain for 4 days and aggravated for 1 day. Magnetic resonance cholangiopancreatography (MRCP) examination revealed duplicated common bile duct (DCBD) malformation with congenital biliary dilatation and distal cholelithiasis. The patient underwent choledochal cyst resection and biliary tract reconstruction and abdominal cavity irrigation and drainage under general anesthesia. A septum was found within the common bile duct during the operation. The septum divided the extrahepatic bile duct into two parts connected to the left and right hepatic ducts respectively and the gallbladder is attached to the repeated right bile duct which was not previously reported in the literature. CONCLUSIONS: We complement and adjust the classification of common bile duct duplication by reviewing the literature.

Extrahepatic biliary duplication with heterotopic gastric mucosa in a 46,XX male patient.

Extrahepatic biliary duplication is a rare congenital biliary malformation, even more so when associated with heterotopic gastric mucosa. This case report highlights the difficulty of diagnosing such biliary abnormalities, in particular when the duplicated extrahepatic bile duct is the only structure visible by imaging as it is masking the common bile duct. This report shows that extrahepatic bile duct duplication may be a cause of chronic biliary obstruction and secondary sclerosing cholangitis. It has to be considered as a differential diagnosis of primary sclerosing cholangitis in children and adolescents. Furthermore, a potential link between the 46,XX karyotype and biliary duplication is discussed.

Long-term outcomes of endoscopic treatment of aberrant hepatic duct injuries after cholecystectomy.

BACKGROUND AND AIMS: Right aberrant hepatic ducts are an anatomic variant with clinical relevance because of the risk of injury during cholecystectomy. Treatment options for aberrant hepatic duct injuries are not standardized. This study aims to analyze the long-term results of endoscopic treatment of aberrant hepatic duct lesions. METHODS: Patients who underwent ERCP for aberrant hepatic duct lesions were retrospectively identified. Demographic data, type of aberrant duct lesion according to the Strasberg classification, type of treatment (number of plastic stents inserted, treatment duration, and number of ERCPs), and adverse events were recorded. Follow-up was obtained by telephone contact or medical examinations. RESULTS: Between January 1996 and March 2019, 32 patients (78% women, mean age 51.7 years) with aberrant hepatic duct injuries underwent ERCP at our Endoscopy Unit. Six patients had Strasberg type B lesions, 11 patients had type C, and 8 patients had type E5, and 7 patients had a stenosis of the aberrant duct. A mean of 3.7 biliary plastic stents per patient were used; mean treatment duration was 6.3 months. All patients with isolated aberrant duct stenosis and 1 of 6 patients (17%) with type B Strasberg lesions achieved patency. Ten of 11 patients (91%) with type C Strasberg lesions achieved duct recanalization. After a mean follow-up of 109.3 ± 61.2 months, 29 of 32 patients (91%) were asymptomatic; 1 underwent surgery for recurrent cholangitis, 1 received a new endoscopic procedure because of cholangitis, and 1 reported episodic biliary colic without an increase in liver function test values and was successfully managed with a low-fat diet. CONCLUSIONS: An endoscopic approach to aberrant hepatic duct lesions after cholecystectomy can be considered an effective first-line therapy.

A quantitative image analysis using MRI for diagnosis of biliary atresia.

PURPOSE: Biliary atresia is a life-threatening disease that needs early diagnosis and management. Recently, MRI images have been used for the diagnosis of biliary atresia with improved accuracy of diagnosis when other imaging modalities such as ultrasonography are equivocal. This study aimed to evaluate the juxta-hilar extrahepatic biliary tree using MRI images to determine a quantitative value for diagnosing biliary atresia. MATERIALS AND METHODS: This retrospective study was approved by the Ethical Committee at Mackey Memorial Hospital (IRB Number: 15MMHIS149e). Between January 2010 and December 2015, twenty-five patients with surgically confirmed biliary atresia were enrolled (age 18-65 days). Another 25 patients with clinically or surgically diagnosed idiopathic neonatal hepatitis (age 6-64 days) and 20 patients with non-hepatobiliary disease (age 6-65 days) were considered control group and normal subjects, respectively. The diameter of the enlarged, T2-hyperintense structure was measured using MRI images by two radiologists both blinded. The cut-off value for a biliary atresia diagnosis was obtained by area under the curve analysis. RESULTS: The diameter of the T2-hyperintense structure at porta hepatis in biliary atresia (4.79 ±â€¯1.14 mm) is larger than in idiopathic neonatal hepatitis (1.72 ±â€¯0.42 mm) or in non-hepatobiliary disease (1.72 ±â€¯0.35 mm) (p < 0.05). The optimum cut-off value for diagnosing biliary atresia was 3.1 mm with 98% sensitivity and 98% specificity. CONCLUSION: The value of the enlarged, T2-hyperintense structure measured on MRI images was significantly increased in biliary atresia and may be useful in diagnosing biliary atresia.

The Double Trouble: A Case of Duplicated Extrahepatic Bile Duct with Choledochal Cyst.

Biliary tract shows a large number of anatomic variations and duplication of the bile duct is an extremely rare anomaly. It has been reported to be associated with other congenital conditions like Abnormal Pancreato Biliary Junction (APBJ), biliary atresia and choledochal cyst and may lead to complications like cholangitis, choledocholithiasis and malignancy. The clinical presentation may be with one of the above complications and the condition usually reveals itself only on imaging investigations, as a surprise to the radiologist and the surgeon. Its detection is important prior to any biliary tract surgery to prevent inadvertent bile duct injury. The authors report a case of a rare subtype of extrahepatic bile duct duplication with coexisting choledochal cyst.

Primary Langerhans Cell Histiocytosis of the Extrahepatic Bile Duct Occurring in an Adult Patient

Background: Langerhans cell histiocytosis is characterized by an abnormal proliferation of neoplastic Langerhans cells. Langerhans cell histiocytosis commonly affects the pediatric population, whereas presentation in adults remains a rare event. The presentation of Langerhans cell histiocytosis is highly variable, but the involvement of skin, bone, and lung is very common. Langerhans cell histiocytosis presenting as a bile duct mass is rare and usually occurs as part of a multiorgan system disease. Case Report: We present a case of Langerhans cell histiocytosis confined to the extrahepatic bile duct in a 62-year-old female patient with sclerosing cholangitis. The mass was composed of mononuclear cells with cleaved nuclei that were positive for CD68, S100, and CD1a as assessed by immunohistochemistry. Conclusion: This is the first report of Langerhans cell histiocytosis limited to the extrahepatic bile duct in an adult patient. We discuss the clinical manifestations and the challenges encountered in the diagnosis and treatment of this rare entity.

Duplication of the extrahepatic bile duct: A case report and review of the literatures.

RATIONALE: Duplication of the extrahepatic bile duct is an extremely rare congenital anomaly of the biliary system. PATIENT CONCERNS: A 44-year-old woman presented with a history of continuous upper abdominal pain and vomiting. DIAGNOSES: Magnetic resonance cholangiopancreatography (MRCP) disclosed diffuse dilatation of the intrahepatic and extrahepatic bile ducts. Endoscopic retrograde cholangiopancreatography (ERCP) showed the presence of two extrahepatic bile ducts with calculus at the distal end of the CBD. INTERVENTIONS: Laparoscopic cholecystectomy (LC) was performed after an ERCP. Choledochoscopy, performed during the operation, showed duplicated common bile duct and the cystic duct was seen opening at the right side of the extrahepatic duct. OUTCOMES: The patient was doing well after 6 months of follow-up. LESSONS: We reported a case of a double common duct with choledocholithiasis and gallstone. This rare anomaly may lead to cholangitis, common bile duct injury during surgery, malignancy occurrence, and should be treated with extreme care.

Telomere length of gallbladder epithelium is shortened in patients with congenital biliary dilatation: measurement by quantitative fluorescence in situ hybridization.

BACKGROUND: Congenital biliary dilatation (CBD) is a congenital malformation involving both dilatation of the extrahepatic bile duct and pancreaticobiliary maljunction. Persistent reflux of pancreatic juice injures the biliary tract mucosa, resulting in chronic inflammation and higher rates of carcinogenesis in the biliary tract, including the gallbladder. Telomeres are repetitive DNA sequences located at the ends of chromosomes. Chromosomal instability due to telomere dysfunction plays an important role in the carcinogenesis of many organs. This study was performed to determine whether excessive shortening of telomeres occurs in the gallbladder mucosa of patients with CBD. METHODS: Resected gallbladders were obtained from 17 patients with CBD, ten patients with cholecystolithiasis without pancreatic juice reflux, and 17 patients with normal gallbladders (controls) (median age of each group of patients: 37, 50, and 53 years, respectively). The telomere lengths of the gallbladder epithelium were measured by quantitative fluorescence in situ hybridization using tissue sections, and the normalized telomere-to-centromere ratio (NTCR) was calculated. RESULTS: The NTCRs in the CBD, cholecystolithiasis, and control groups were 1.24 [interquartile range (IQR) 1.125-1.52], 1.96 (IQR 1.56-2.295), and 1.77 (IQR 1.48-2.53), respectively. The NTCR in the CBD group was significantly smaller than that in the cholecystolithiasis and control groups (p = 0.003 and 0.004, respectively), even in young patients. CONCLUSIONS: Our findings indicate that telomere shortening in the gallbladder mucosa plays an important role in the process of carcinogenesis in patients with CBD. These results support the recommendation of established guidelines for prophylactic surgery in patients with CBD because CBD is a premalignant condition with excessive telomere shortening.

Symptomatic pancreatic duct stones in the disconnected bile duct: A case series.

BACKGROUND: Pancreaticobiliary maljunction (PBM) refers to the union of the pancreatic and biliary ducts outside of the duodenal wall. Patients are at increased risk of bile duct and gallbladder cancer, likely secondary to pancreatic juice refluxing into the biliary tree, and it is recommended that they undergo biliary diversion. METHODS: This is a case series of all patients in our institution with PBM and bilioenteric anastomosis who presented with symptomatic pancreatic duct stones in a disconnected bile duct. IRB approval was obtained prior to the initiation of the study. RESULTS: We describe eight cases of this finding. All patients underwent ERCP, with stones successfully removed from the disconnected bile duct in seven patients and from the pancreatic duct in one patient. CONCLUSION: This novel finding has not been described in the medical literature, and may become more prevalent as more patients with PBM undergo bilioenteric anastomosis.

Pancreaticobiliary maljunction and choledochal cysts: from embryogenesis to therapeutics aspects.

Pancreaticobiliary maljunction (PBM) and choledochal cysts (CC) are rare and little-known diseases. Several definitions have been proposed for the PBM, but the most widely accepted is an excessive length of the common pancreaticobiliary duct due to the abnormal convergence of the pancreatic and biliary ducts out of the duodenal wall. This anomaly, thought to develop during embryogenesis, is associated with a loss of regulation of the Oddi's sphincter leading to a pancreaticobiliary or biliopancreatic backflow. This reflux could be responsible, or associated with cystic dilatation of the bile ducts and biliary tract cancers, to various biliary or pancreatic events such as cholangitis or pancreatitis. For the diagnosis of PBM, magnetic resonance cholangiopancreatography has now become the gold standard as a noninvasive imaging tool. However, the main risk of PBM is the development of bile duct cancer, most often on a distended area. PBM without CC increase the occurrence of gallbladder cancer and require a preventive cholecystectomy. Surgical treatment of PBM with concomitant CC is more complex and depends on localization of the dilatation(s) as reported in the Todani's classification. This review describes the pathogenesis, embryogenesis, clinical features, investigation and management of PBM and CC.