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Choledochal cysts (CCs) are cystic dilations of intrahepatic and/or extrahepatic bile ducts. Around 80% of CCs are diagnosed within the first decade of life. These complex clinical entities are extremely rare, especially in the Western population. CCs are frequently classified according to the Todani classification. CCs may be asymptomatic or present as acute pancreatitis and/or cholangitis, biliary obstruction, or malignancy. Therefore, the diagnosis relies primarily on abdominal imaging modalities, mainly magnetic resonance cholangiopancreatography. Management is tailored based on the cyst morphology and the patient's clinical characteristics, with surveillance, surgery, and interventional endoscopy being the most frequent management options. While the surgical approach is the most frequently employed, type III CCs (also known as choledochocele) are frequently managed endoscopically, and novel endoscopic, minimally invasive treatment options are rapidly emerging. [ABSTRACT FROM AUTHOR]

Introduction: Choledochal cysts (CCs) are rare congenital anomalies characterized by cystic dilatations in the bile ducts. This study reviews the demographic and clinical characteristics of patients diagnosed with CCs at Sardjito Hospital, Yogyakarta, Indonesia, from 2017 to 2023. Methods: A retrospective analysis of medical records was conducted, including data on demographics, symptoms, lab findings, imaging, and surgical interventions. Results: Among 29 patients, 21 were female, with an average age at diagnosis of 60.63 months. Over 50% presented with jaundice or abdominal pain. All patients underwent intraoperative cholangiogram (IOC), and 89% had magnetic resonance cholangiopancreatography (MRCP) or ultrasonography (US). The largest cyst measured 21.52 cm. Elevated levels of total bilirubin, direct bilirubin, SGOT, and SGPT were noted preoperatively. Surgical treatment involved cyst excision, with 28 patients undergoing Roux-en-Y hepaticojejunostomy and 1 patient undergoing hepaticoduodenostomy. Postoperative complications occurred in 7 patients, including two fatalities, two cases of pancreatitis, and three instances of anastomotic leakage. No significant correlations were found between clinical variables and complications. The survival rate was 93.1%, with an average hospital stay of 27.4 days. Conclusion: The study highlights the female predominance of CCs, common symptoms of jaundice and abdominal pain, and the importance of IOC, MRCP, and US in diagnosis. Complete cyst excision with biliodigestive reconstruction remains the recommended treatment approach. [ABSTRACT FROM AUTHOR]

Focal liver lesions (FLLs) have become an increasingly common finding on abdominal imaging, especially asymptomatic and incidental liver lesions. Gastroenterologists and hepatologists often see these patients in consultation and make recommendations for management of multiple types of liver lesions, including hepatocellular adenoma, focal nodular hyperplasia, hemangioma, and hepatic cystic lesions including polycystic liver disease. Malignancy is important to consider in the differential diagnosis of FLLs, and healthcare providersmust be familiarwith the diagnosis andmanagement of FLLs. This American College of Gastroenterology practice guideline uses the best evidence available to make diagnosis and management recommendations for the most common FLLs. [ABSTRACT FROM AUTHOR]

Abstract Background Biliary intraepithelial neoplasia (BilIN), a noninvasive precursor of cholangiocarcinoma, can manifest malignant transformation. Since cholangiocarcinoma (CCA) may progress due to chronic inflammation in the bile ducts and gallbladder, choledochal cysts are considered a precursor to CCA. However, BilIN has rarely been reported in children, to date. Methods We reviewed medical records of patients (

Bile duct injuries are rare complications of hepatobiliary pancreatic surgery, leading to severe complications if not timely diagnosed and treated, with surgery traditionally being the primary treatment option. However, percutaneous transhepatic or endoscopic interventions have recently gained widespread use. We present a case study of a patient with variant biliary anatomy, who suffered biliary tract injury postcholedochal cyst resection and Roux-en-Y hepaticojejunostomy; successfully treated with percutaneous transhepatic bilioenteric neoanastomosis, guided by ultrasound and digital subtraction angiography (DSA).

Background: Biliary intraepithelial neoplasia (BilIN), a noninvasive precursor of cholangiocarcinoma, can manifest malignant transformation. Since cholangiocarcinoma (CCA) may progress due to chronic inflammation in the bile ducts and gallbladder, choledochal cysts are considered a precursor to CCA. However, BilIN has rarely been reported in children, to date. Methods: We reviewed medical records of patients (< 18 years of age, n = 329) who underwent choledochal cyst excision at Asan Medical Center from 2008 to 2022. BilIN was diagnosed in 15 patients. Subsequent analyses were performed of the demographics, surgical procedures, clinical course, and outcomes in these patients. Subgroup analysis and multivariate logistic regression test were performed to identify factors influencing BilIN occurrence. Results: The mean age of the patients included in our study was 40.1 ± 47.6 months. In 15 patients, BilIN of various grades was diagnosed. Todani type I was prevalent in 80% of the patients. The median age at surgery was 17 months. During a mean follow-up of 63.3 ± 94.0 months, no adverse events such as stone formation in the remnant intrapancreatic common bile duct and intrahepatic duct or cholangiocarcinoma were observed, indicating a favorable outcome until now. Conclusions: The potential progression of choledochal cysts to BilIN in children was demonstrated. These results could underscore the importance of early and comprehensive excision of choledochal cysts, including resection margins for associated lesions and more thorough postoperative surveillance in patients with or at risk of BilIN. [ABSTRACT FROM AUTHOR]

Background: Choledochal cysts are rare congenital anomalies of the biliary tree that may lead to obstruction, chronic inflammation, infection, and malignancy. There is wide variation in the timing of resection, operative approach, and reconstructive techniques. Outcomes have rarely been compared on a national level. Methods: We queried the Pediatric National Surgical Quality Improvement Program (NSQIP) to identify patients who underwent choledochal cyst excision from 2015 to 2020. Patients were stratified by hepaticoduodenostomy (HD) versus Roux‐en‐Y hepaticojejunostomy (RNYHJ), use of minimally invasive surgery (MIS), and age at surgery. We collected several outcomes, including length of stay (LOS), reoperation, complications, blood transfusions, and readmission rate. We compared outcomes between cohorts using nonparametric tests and multivariate regression. Results: Altogether, 407 patients met the study criteria, 150 (36.8%) underwent RNYHJ reconstruction, 100 (24.6%) underwent MIS only, and 111 (27.3%) were less than one year old. Patients who underwent open surgery were younger (median age 2.31 vs. 4.25 years, p = 0.002) and more likely underwent RNYHJ reconstruction (42.7% vs. 19%, p = 0.001). On adjusted analysis, the outcomes of LOS, reoperation, transfusion, and complications were similar between the type of reconstruction, operative approach, and age. Patients undergoing RNYHJ had lower rates of readmission than patients undergoing HD (4.0% vs. 10.5%, OR 0.34, CI [0.12, 0.79], p = 0.02). Conclusions: In children with choledochal cysts, most short‐term outcomes were similar between reconstructive techniques, operative approach, and age at resection, although HD reconstruction was associated with a higher readmission rate in this study. Clinical decision‐making should be driven by long‐term and biliary‐specific outcomes. [ABSTRACT FROM AUTHOR]

Key Clinical Message: This case demonstrates an atypical presentation of choledochal cysts (CDCs) and elaborates on the diagnostic challenges encountered when presented with CDCs in adulthood, as it principally presents in children. A choledochal cyst is a rare congenital anomaly characterized by cystic dilations in the extrahepatic and intrahepatic biliary trees. These cysts are classified according to their location and characteristics. This case study aims to demonstrate how nonspecific clinical features can pose a diagnostic dilemma when presented in adults. Additionally, the case report provides an overview of diagnostic methods and treatment options. In this case, we discuss a 50‐year‐old female who presented with a 2‐ to 3‐day history of severe colicky pain in the right upper quadrant of her abdomen without any other symptoms or abnormal laboratory tests. In addition to ultrasonography evidence of CBD dilation and cholelithiasis, MRCP results confirmed the diagnosis. She underwent surgical intervention involving cyst excision, a Roux‐en‐Y hepatojejunostomy, and a cholecystectomy. The postoperative period was without significant complications. The case presented here illustrates the potential outcomes for individuals who present with choledochal cysts during adulthood. Often, these cases present with vague symptoms or as the underlying cause of a more severe condition. This case contributes to the existing knowledge of choledochal cysts by providing insight into the clinical presentation, diagnostic methods, and treatment options. [ABSTRACT FROM AUTHOR]

Objective To explore the safety and efficacy of single-port laparoscopic choledochal cyst excision and Roux-en-Y hepaticojejunostomy (SPCH). Methods Between January 1, 2018, and December 31, 2022, 62 consecutive cases of choledochal cysts were recruited. The procedure was either SPCH (n=43) or multiple-port laparoscopic choledochal cysts excision and Roux-en-Y hepaticojejunostomy (MPCH) (n=19). Magnetic resonance cholangiopancreatography (MRCP) was performed. Baseline clinical characteristics, short-term efficacy and safety outcomes were examined through reviewing the relevant medical records. Results No significant inter-group differences existed in such baseline clinical characteristics as gender, operative age, Todani classification, clinical presentation, prenatal diagnosis & longest diameter of cyst. Average postoperative hospitalization stay of SPCH group was shorter than that of MPCH group (7. 14±2. 70 vs. 7. 58±3. 27 day, P=0. 892). However, the inter-group difference was not statistically significant. Operative duration (266. 79±69. 39 vs. 277. 32±90. 23 min, P=0. 913) and volume of blood loss (8. 88±12. 37 vs. 16. 68±34. 84 mL, P=0. 758) were similar between two groups. MPCH group had a significantly higher number of drainage tubing than SPCH group (11 vs. 7;P=0. 0009). One child in SPCH group developed stenosis after hepaticojejunostomy. During follow-ups, no other complications occurred. Conclusions SPCH is as safe and effective as MPCH for choledochal cysts. It helps to lower the difficulty of single-incision laparoscopy. [ABSTRACT FROM AUTHOR]

This summary discusses a study conducted at Cerrahpa¿a Medical Faculty in Istanbul, Turkey, on adult patients with biliary cysts. The study analyzed 30 patients who underwent treatment for biliary cysts and found that surgical resection is the preferred method for treating certain types of cysts. The study emphasizes the importance of specialized facilities for the diagnosis and treatment of biliary cysts due to the complexity of the condition and potential complications. The risk of cholangiocarcinoma development is higher in Asian populations, but no cases were observed in the study. [Extracted from the article]

A 53-year-old male patient with a previous diagnosis of dilatation of the common bile duct was admitted to the hospital due to recurrent episodes of vague epigastric pain over a 4-month period. After undergoing abdominal CT, MRI, MRCP, ERCP examinations, together with joint diagnosis by the radiology department and the gastroscopy unit, the diagnosis of a cystic dilatation of the common bile duct was excluded, and to preliminarily diagnose as cystic lesion at the hepatoduodenum ligament. A nasobiliary tube was preset before the surgery, and it was found that the gallbladder, the cyst, and the common bile duct were connected in sequence during the surgery, leading to the definitive diagnosis of biliary cyst of the cystic duct. During the surgery, the anatomical position of the common bile duct was accurately identified, avoiding iatrogenic biliary injury and preserving the integrity of the common bile duct structure. The patient recovered and was discharged from the hospital on the 14th postoperative day. Cystic duct cysts are a relatively new and rare condition. This case demonstrates that clinical decision-making by a multidisciplinary team is of great significance for such diseases, and preoperative assessment of the anatomical relationship between cystic dilation lesions in the hepatic portal region and the biliary system and gallbladder is also crucial. [ABSTRACT FROM AUTHOR]

Choledochal cysts are rare congenital biliary cystic dilations. The US incidence rate varies between 5 and 15 cases per 1,000,000 people. In contrast, Asians, which are a large subset of the population of Hawaii, have an incidence of approximately one in every 1000 births. We report our experience with robot-assisted laparoscopic surgical management with biliary reconstruction of choledochal cysts which to date is the largest American case series to be reported. From 2006 to 2021, patients diagnosed with a choledochal cyst(s) at a tertiary children's hospital were retrospectively reviewed. Perioperative analysis was performed. Complications were defined as immediate, early, or late. The data underwent simple descriptive statistics. Nineteen patients underwent choledochal cystectomy and hepaticoduodenostomy. Thirteen underwent a robotic approach while the rest were planned laparoscopic. Eighteen of 19 were female with 15/19 of Asian descent. The ages ranged from 5 mo to 21 y. Presenting diagnoses included jaundice, primary abdominal pain, pancreatitis, and cholangitis. Sixty eight percent had type 1 fusiform cysts while the rest were type 4a. Operative time and length of stay for robotic versus laparoscopic were 321 versus 267 min and 8.2 versus 17.3 d, respectively. For the robotic group, there was one immediate complication due to peritonitis. One-year follow-up revealed two patients requiring endoscopic retrograde cholangiopancreatography with dilation/stenting for an anastomotic stricture. There were no anastomotic leaks. Robot-assisted laparoscopic choledochal cystectomy with hepaticoduodenostomy is associated with overall good outcomes with the most common long-term complication being anastomotic stenosis. [ABSTRACT FROM AUTHOR]

Background/purposeCholedochal cysts are congenital dilations of the bile ducts, and are associated with an increased risk of malignant transformation. The purpose of this study is to report the outcomes of a large series of patients with choledochal cysts and to highlight our analysis of one patient who developed malignancy after cyst resection.MethodsWe conducted a retrospective review of patients

Choledochal cysts (CCs) are rare occurrences presenting as dilatations of biliary structures, which can present as single or multiple dilatations and can appear as both intra- and extrahepatic anomalies. The most widespread classification of CCs is the Todani classification, but there have been numerous reports of cysts that do not fall into any of the types described. We present such a case—a male patient 36 years of age who underwent preoperative CT, MRCP, and ERCP, which mistakenly indicated a type II Todani CC, and intraoperatively was found to be located at the confluence of the hepatic ducts and encompassed the origin of the common bile duct. Complete resection of the cyst and the proximal segment of the common bile duct was performed, and reconstruction was carried out by Roux-en-Y double-tutorized hepaticojejunostomy. Considering the risk of malignant transformation, the frequent preoperative misdiagnosis, as well as the technically challenging surgery required in such cases, we advocate for a revision of the classification and raise awareness of the need for guidelines regarding the proper short-term and long-term management of this disease to ensure adequate quality of life and disease-free survival for patients. [ABSTRACT FROM AUTHOR]

Purpose: Surgery for choledochal cysts (CDC) in children younger than 6 months is relatively rare. We report our experience and compare the results between Da Vinci robot-assisted hepaticojejunostomy (RAHJ) and laparoscopic-assisted hepaticojejunostomy (LAHJ) in children younger than 6 months to treat CDC. Methods: A retrospective study was conducted on all children under 6 months of age who underwent RAHJ or LAHJ at the Children's Hospital, Zhejiang University School of Medicine, from July 2018 to November 2023. Results: We reviewed 34 patients who underwent RAHJ surgery and 50 patients who underwent LAHJ surgery ( P = .243). RAHJ group of the median operation time was 182 minutes (range 161-221), and LAHJ group was 168 minutes (range 143-191) ( P = .02). The RAHJ group had a significantly shorter median postoperative hospital stay of 9 days (range 7-10) than the LAHJ group, 11 days (range 10-14), p < .001. The median hospitalization cost in the RAHJ group was significantly higher than that in the LAHJ group (75,474 CNY versus 28,984 CNY, p < .01). The median follow-up time was 18 months in the RAHJ group and 48 months in the LAHJ group ( p < .01). All patients in the RAHJ group recovered well and were discharged. One patient in the LAHJ group developed biliary fistula 21 days after surgery and recovered well after reoperation. Conclusions: For children under 6 months old, on the basis of no consideration of cost, RAHJ has fast postoperative recovery and fewer postoperative complications, which is more recommended.

This study aimed to evaluate the safety and therapeutic effect of Robot-assisted surgery (RAS) for choledochal cysts (CCs) excisions. PubMed, EMBASE, Cochrane Library, Web of Science, CNKI, WanFang, VIP, and CBM were searched from database inception to 1 May 2022. The Newcastle-Ottawa scale (NOS) was used to conduct quality assessments, and RevMan (Version 5.4) was used to perform the meta-analysis. In all, 9 studies, involving 623 patients, were analyzed. RAS compared with LAS was associated with less intraoperative blood loss, shorter time to start solid diets, shorter postoperative hospital stay, and lower complications. There was no significant difference in operative time between the two groups, but the total costs were higher in RAS. Our subgroup analysis showed that RAS had significant advantages over LAS in the child group: minor bleeding, shorter length of hospital stay, and fewer postoperative complications. The available evidence indicates that the RAS system has the advantages of less intraoperative blood loss, minor tissue damage, quick recovery, and sound healing in treating choledochal cyst, which proves that the RAS is safely feasible. Especially in children, RAS tends to be a better choice. [ABSTRACT FROM AUTHOR]

Choledochal cysts are a rare malformation of the biliary tract with an unknown etiology, predominantly affecting Asians and females. Although they are more often diagnosed during childhood, symptoms typically present in young adulthood due to complications. There are no pathognomonic clinical manifestations; the clinical presentation is associated with gallstones, choledocholithiasis, pancreatitis, cholangitis, and an increased risk of malignancy. We report a rare case of a choledochal cyst in adulthood, with delayed diagnosis and management due to the COVID-19 pandemic. A 21-year-old woman, at 34.2 weeks of pregnancy, was diagnosed with cholangitis in 2022. She initially received minimally invasive treatment at another hospital but lost follow-up after discharge due to the COVID-19 pandemic. A year later, her family doctor referred her to our clinic, following protocol for gallstone disease. A diagnostic protocol was initiated, involving multiple imaging studies such as ultrasound, endoscopic retrograde cholangiopancreatography (ERCP), and abdominal computed tomography (CT). These studies allowed us to identify and classify the cyst, enabling proper surgical planning. Open cyst excision with Roux-en-Y hepaticojejunostomy was performed. Biliary cysts represent a rare pathology with potentially serious complications. They are not usually suspected at first in young adults, and the clinical presentation reflects both the cyst and its associated complications. A combination of clinical data, imaging studies, and endoscopy is essential for precise diagnosis, proper classification, and appropriate surgical planning. Total excision with biliodigestive reconstruction through Roux-en-Y bilioenteric anastomosis remains the preferred treatment. Long-term follow-up is necessary due to the potential for complications., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Villarino Zapata et al.)

A choledochal cyst (CC) is a common congenital biliary disease in children, yet the underlying molecular bases for the cystic and fusiform clinical subtypes are unknown. RNA sequencing (RNA-seq) has been performed on 22 high-quality CC samples, including 12 cystic CC and 10 fusiform CC samples, to search for molecular features. Weighted gene co-expression network analysis (WGCNA) was performed to identify key modules associated with clinical subtypes. Bioinformatic analyses were conducted to elucidate potential mechanisms. Then, we constructed protein–protein interaction (PPI) networks to identify candidate hub genes related to CC. Finally, we used the support vector machine (SVM) to eliminate redundant features and screen out the hub genes. The selected gene expression was determined in CC patients through quantitative real-time polymerase chain reaction (PCR). A total of 6,463 genes were found to be aberrantly expressed between cystic CC and fusiform CC. Twelve co-expression modules that correlated with clinical subtypes of CC were identified and assigned representative colors. Among the 12 modules, the blue module was considered the key module. Two functionally distinct sets of dysregulated genes have been identified in two major subtypes, metabolism-related genes in cystic CC and immune-related genes in fusiform CC. A total of 20 candidate hub genes that were correlated with clinical subtypes were found in the blue module. In addition, we found ERBB2 and WNT11 that have not been studied in CC and verified their differential expression in CC through quantitative real-time PCR experiments. For the first time, we have described the transcriptome characteristics of CC. These results suggest that cystic CC and fusiform CC have different molecular mechanisms. The bi-omics-identified novel candidate genes and pathways might be helpful for personalized treatment and are of great clinical significance for CC.

A choledochal cyst (CC) is a common congenital biliary disease in children, yet the underlying molecular bases for the cystic and fusiform clinical subtypes are unknown. RNA sequencing (RNA-seq) has been performed on 22 high-quality CC samples, including 12 cystic CC and 10 fusiform CC samples, to search for molecular features. Weighted gene co-expression network analysis (WGCNA) was performed to identify key modules associated with clinical subtypes. Bioinformatic analyses were conducted to elucidate potential mechanisms. Then, we constructed protein–protein interaction (PPI) networks to identify candidate hub genes related to CC. Finally, we used the support vector machine (SVM) to eliminate redundant features and screen out the hub genes. The selected gene expression was determined in CC patients through quantitative real-time polymerase chain reaction (PCR). A total of 6,463 genes were found to be aberrantly expressed between cystic CC and fusiform CC. Twelve co-expression modules that correlated with clinical subtypes of CC were identified and assigned representative colors. Among the 12 modules, the blue module was considered the key module. Two functionally distinct sets of dysregulated genes have been identified in two major subtypes, metabolism-related genes in cystic CC and immune-related genes in fusiform CC. A total of 20 candidate hub genes that were correlated with clinical subtypes were found in the blue module. In addition, we found ERBB2 and WNT11 that have not been studied in CC and verified their differential expression in CC through quantitative real-time PCR experiments. For the first time, we have described the transcriptome characteristics of CC. These results suggest that cystic CC and fusiform CC have different molecular mechanisms. The bi-omics-identified novel candidate genes and pathways might be helpful for personalized treatment and are of great clinical significance for CC. [ABSTRACT FROM AUTHOR]

A giant common bile duct (CBD) calculus is a rare occurrence, and the presence of a giant calculus within a choledochal cyst (CDC) is even more unusual. In this case report, we detail an instance of a giant CBD calculus measuring 7 cm x 3 cm found within a CDC, accompanied by multiple tiny calculi. Magnetic resonance cholangiopancreatography (MRCP) revealed the dilation of the bi-lobar intrahepatic biliary radical (IHBR) and the CBD. A large T2 hypointense and T1 hyperintense calculus occupied the dilated CBD and common hepatic duct (CHD), extending into the left hepatic duct (LHD) and right hepatic duct (RHD). There was a possibility of type 1c CDC with cystolithiasis, hepatolithiasis, and cholelithiasis. The patient underwent open cholecystectomy with choledochotomy, stone retrieval, excision of the CDC, and Roux-en-Y hepaticojejunostomy., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. All India Institute of Medical Sciences, Rishikesh, Institutional Ethics Committee issued approval AIIMS/IEC/24/214. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, V et al.)