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Introduction: Type IV-A choledochal cyst (CDC) has been considered to have a poor prognosis due to the high incidence of postoperative anastomotic strictures and intrahepatic stones. This study aimed to evaluate the surgical outcomes of children with type IV-A CDC and to provide insights for clinical diagnosis and treatment. Methods: The study retrospectively analyzed patients from June 2015 to December 2018 at our center, 76 children were diagnosed with type IV-A CDC. All patients underwent single-incision laparoscopic choledochal cyst excision and Roux-en-Y hepaticojejunostomy. The decision to perform ductoplasty was made by comprehensively considering the intrahepatic duct dilatation (IHDD) and stricture. All patients were followed up 1, 3, and 6 months postoperatively, and then every 1 year thereafter. Patients were categorized into two groups based on IHDD changes postoperatively: the long-term group (LTG), with IHDD persisting for over a year, and the short-term group (STG), where IHDD normalized within a year. Single/multiple factor logistic regression was used to analyse the factors influencing postoperative IHDD. Results: The median follow-up period was 80 months, with a range from 64 to 101 months. The decrease in postoperative liver function parameters, compared to preoperative levels, was statistically significant. Two patients (2.63%) developed bile leaks. One patient (1.32%) developed anastomotic stricture. All patients' IHDD returned to normal size. Fifty-six (73.7%) patients showed normalization of IHDD within one-year postoperatively. The median recovery time for IHDD in patients was 1.65 months, ranging from 3 days to 74 months postoperatively. There were significant differences in intrahepatic biliary sludge and stones and maximum diameter of IHDD between STG and LTG. Logistic regression was used to analyse the factors and found that intrahepatic biliary sludge and a wider maximum diameter of IHDD were risk factors for postoperative long-term IHDD in patients. Conclusion: Single-incision laparoscopic hepaticojejunostomy with selective ductoplasty are safe and effective for children with Type IV-A CDC. Long-term follow-up is recommended for children presenting with intrahepatic biliary sludge and larger IHDD. [ABSTRACT FROM AUTHOR]

Choledochal cysts (CCs), a congenital anomaly resulting in the abnormal dilation of the biliary ductal system, are most often identified in patients younger than 10 years of age. Regardless of clinical presentation, the cornerstone of therapy for CCs is complete surgical excision with reconstruction with either hepaticoduodenostomy or hepaticojejunostomy. Although both procedures are used by surgeons for the correction of CCs, evidence on clinical outcomes following both approaches is inconclusive as to which may offer superior reconstruction. This narrative review aims to compare the current literature regarding both approaches by evaluating their anatomic and operative considerations, as well as their perioperative, postoperative, and long-term outcomes. Future studies should closely focus on long-term, comparative outcomes, including the risk of biliary malignancy, and refine techniques to minimize complications, such as biliary reflux and bowel obstruction, in order to improve care for pediatric patients undergoing treatment for CCs. [ABSTRACT FROM AUTHOR]

Objectives: To analyze the ability of magnetic resonance (MR) to identify cystic biliary atresia (CBA) and choledochal cyst (CC). Methods: Infants (≤ 1 year old) who were diagnosed with CBA or CC type I/IV from January 2010 to July 2023 were retrospectively reviewed. Imaging characteristics on MR were compared between the CBA and CC groups. Binary logistic regression and the area under the receiver operating characteristic curve (AUC) were analyzed for the identification of CBA. Results: Sixty-three patients with CBA (median age, 30 days) and 172 patients with CC (median age, 60 days) were included. Gallbladder (GB) wall thickness (cutoff, 1.2 mm) showed 98.4% sensitivity and 100% specificity (AUC, 0.998). MR-triangular cord thickness (MR-TCT) (cutoff, 4.1 mm) showed 100% sensitivity and 95.9% specificity (AUC, 0.986). The bile duct loop visualization showed 96.8% sensitivity and 100% specificity (AUC, 0.984). Proximal bile duct (PBD) diameter (cutoff, 1.3 mm) showed 92.1% sensitivity and 95.3% specificity (AUC, 0.977). Cyst wall thickness (cutoff, 1 mm) showed 77.8% sensitivity and 95.3% specificity (AUC, 0.942). The combination of GB wall thickness > 1.2 mm and MR-TCT > 4.1 mm, GB wall thickness > 1.2 mm and loop visualization, GB wall thickness > 1.2 mm, and cyst wall thickness > 1 mm showed 100% sensitivity and 100% specificity (AUC, 1.000). Conclusions: Imaging characteristics on MR might be used to identify CBA and CC, and the combination of GB wall thickness and MR-TCT, or loop visualization, or cyst wall thickness, has a perfect diagnostic value. Clinical relevance statement: Early and accurate differentiation of CBA and CC is essential, but current methods rely on inherently subjective ultrasound. Biliary features on MRI allow for an objective, accurate diagnosis. Key Points: Cystic biliary atresia and choledochal cysts have similar clinical presentations, but require different treatments at different ages. Examined MR features eliminate ultrasound related subjectivity and diagnose cystic biliary atresia with nearly perfect accuracy. Early and accurate differentiation between these entities allows for timely life-saving intervention. [ABSTRACT FROM AUTHOR]

Purpose Hepaticojejunostomy anastomosis (HJA) is the most challenging aspect in single-port laparoscopic choledochal cystectomy and Roux-en-Y hepaticojejunostomy (SPCH) in children, especially in small-diameter anastomoses (diameters less than 5 mm), which are more susceptible to anastomotic stricture. We developed the continuous submucosal technique for HJA (CS-HJA) to lessen postoperative complications. The purpose of this study is to introduce our preliminary experiences with CS-HJA. Methods We retrospectively analyzed all available clinical data of children who underwent SPCH surgery between March 2020 and October 2022. We operated with CS-HJA on 10 children who were diagnosed with small-diameter hepaticojejunostomy (diameter less than 5 mm). Data collection mainly included demographic information, imaging data, perioperative details, and postoperative outcomes. Ten patients were included in this study. The average patient age was 55.2 months; the age range was 3 to 120 months, and the average weight was 11.6 kg; male–female ratio was 1:9. The choledocho had fusiform dilatation in five cases and cystic dilatation in five cases. There was no dilatation of the left and right hepatic ducts or intrahepatic bile ducts in all patients. All patients had no dilatation of the left and right hepatic ducts or intrahepatic bile ducts. All patients underwent a single-port laparoscopic bile-intestinal anastomosis using a submucosal jejunal anastomosis technique. Analysis of the duration of the bile-intestinal anastomosis, the length of the child's stay in the hospital after surgery, the intraoperative complications, and the postoperative complications was performed. Results All the 10 patients underwent successful SPCH by CS-HJA technique. The average length of time for hepaticojejunostomy ranged from 22 to 40 minutes, and the postoperative hospital stay was 5.2 to 9.2 days. There were no instances of bile leakage following the operation. At 17 to 30 months of follow-up, there was no abdominal pain or jaundice, and the reexamination of transaminases, bilirubin, and amylase were normal. Ultrasonography showed no bile duct stricture or dilated bile ducts, and the incision is elegant, and the families of the patients were satisfied. Conclusion In SPCH surgery in children, the CS-HJA technique is safe and feasible for small-diameter hepaticojejunostomy. [ABSTRACT FROM AUTHOR]

Objective: Rare disease Background: A choledochal cyst (CC), or biliary cyst, is a congenital or acquired anomaly of the biliary tree. Pancreas divisum (PD) is a rare congenital anomaly due to incomplete fusion of pancreatic ducts, which can complicate the clinical course of choledochal cysts. This rare combination is a surgical management challenge. This report presents the diagnosis and management of a 23-year-old woman with a combined choledochal cyst and pancreas divisum treated with pancreaticoduodenectomy. Case Report: This article presents the case of a 23-year-old woman who presented with severe, stabbing abdominal pain radiating to the back and epigastric tenderness and was diagnosed with pancreatitis. Initial imaging revealed a choledochal cyst, prompting further investigation with ERCP that showed concomitant PD. She was treated via pancreaticoduodenectomy. During the following 9 years, she was hospitalized over 2 dozen times for recurrent pancreatitis. Conclusions: This report presents a complex case of a combined choledochal cyst and pancreas divisum, which was surgically managed by pancreaticoduodenectomy. The association of CC with PD should be suspected in patients with recurrent acute pancreatitis and/or cholangitis with no identifiable cause. Surgical treatment of CC with PD depends on the classification of the CC, and complications can include recurrent pancreatitis, although the prognosis is often favorable. The purpose of this report is to emphasize that pancreaticoduodenectomy is unlikely to provide favorable outcomes for CC with PD, especially considering there is evidence that less extensive surgical interventions produce better outcomes. [ABSTRACT FROM AUTHOR]

Background: Biliary dilatation without obvious etiology on cross sectional imaging warrants further investigation. This study aimed to assess yield of endoscopic ultrasound in providing etiologic diagnosis in such situation. Methods: Prospective cohort of consecutive patients with biliary dilatation & non diagnostic computed tomography (CT) and /or magnetic resonance imaging (MRI) underwent endoscopic ultrasound (EUS) with/without fine needle aspiration cytology (FNAC) and were followed clinically, biochemically with/without radiology for up to six months. The findings of EUS were corroborated with histopathology of surgical specimens and endoscopic retrograde cholangiography (ERCP) findings in relevant cases. Results: Median age of 121 patients completing follow up was 55 years. 98.2% patients were symptomatic and median common bile duct (CBD) diameter was 13 mm. EUS was able to identify lesions attributable for biliary dilatation in (67 out of 121) 55.4% cases with ampullary neoplasm being the commonest (29 out of 67 i.e. 43%). Multivariate logistic regression analysis identified jaundice as the predictor of positive diagnosis on EUS, of finding ampullary lesion and pancreatic lesion on EUS. EUS had sensitivity, specificity, positive predictive value and diagnostic accuracy of 95.65%, 94.23%, 95.65% and 95.04% respectively in providing etiologic diagnosis. Threshold value for baseline bilirubin of 10 mg%, for baseline CA 19.9 of 225 u/L and for largest CBD diameter of 16 mm were determined to have specificity of 98%, 95%, 92.5% respectively of finding a positive diagnosis on EUS. Conclusion: EUS provides considerable diagnostic yield with high accuracy in biliary dilatation when cross sectional imaging fails to provide etiologic diagnosis. [ABSTRACT FROM AUTHOR]

Background: Prenatally diagnosed hepatic hilar cysts are a challenging finding for the clinician. They can either be a sign of cystic biliary atresia (BA) or a choledochal cyst (CC), two diagnoses with different postnatal management and prognosis. Based on a case report of four patients, we aim to propose a management algorithm for prenatally diagnosed "hepatic hilar cysts". Case presentation: A hepatic hilar cyst, ranging from 5 to 25 mm, was detected prenatally in all four girls confirmed postnatally along with the presence of a gallbladder. Stool color was normal until two weeks of life at which time the stool color became lighter, and the patients developed cholestasis. All were operated before seven weeks of life: Case 1 had a CC with patent but irregular intrahepatic bile ducts at intraoperative cholangiogram, and no communication with the duodenum. A Roux-en-Y bilioenteric anastomosis was performed. The cyst showed complete epithelial lining loss, and liver pathology showed BA features. Case 2 had the final diagnosis of cystic BA with patent but abnormal intrahepatic bile ducts. She underwent two operations: the first operation at four weeks as described for case 1, since intraoperative findings were similar, as was histology. As cholestasis increased postoperatively, she underwent a Kasai hepato-porto-enterostomy six weeks later, where distinct BA findings were found with complete scarring of the hilar plate. Case 3 had a cystic BA with the cyst located within the common bile duct and atretic bile ducts proximal to the porta hepatis. It exhibited no communication with the liver or duodenum. A Kasai operation was performed, with histology showing complete epithelial loss within the cyst wall and scarring of the hilar plate. Case 4 had a cystic BA presenting a completely obliterated hepatic duct with the cyst lying within the common bile duct. A Kasai procedure was performed. Histology showed a common bile duct with a residual lumen of 0.1 mm. Conclusions: The spectrum of disease from CC to BA in the setting of a prenatally discovered hepatic hilar cyst is emphasized. Even if cholangiogram differentiates most patients with BA from those with CC, caution is advised for transitional types. [ABSTRACT FROM AUTHOR]

Background and aims: Biliary atresia (BA) is a progressive fibro-obliterative cholangiopathy. The histopathological diagnosis is often challenging and an immunohistochemical marker is often sought as an adjunct. We evaluated MMP7 immunohistochemistry in BA and other non-BA pediatric cholestatic liver diseases. Materials and methods: MMP7 immunohistochemistry was applied in 5 age-matched normal control, 23 cases of BA and 43 cases of non-BA pediatric cholestasis including 16 cases of choledochal cyst (CC), and a multiplication score was obtained by multiplying the intensity and percentage positivity in the cholangiocytes. Results: BA showed a high mean MMP7 multiplication score which was significantly different from the normal control and other non-BA pediatric cholestatic diseases including CC (p value < 0.001). The sensitivity, specificity, positive, and negative predictive values of MMP7 immunohistochemistry were 91.3%, 93.02%, 87.5%, and 95.2% respectively. Conclusion: MMP7 immunohistochemistry may be an adjunct to histomorphology in BA. [ABSTRACT FROM AUTHOR]

Cyst excision and Roux-en-Y hepaticojejunostomy (RYHJ) is the standard treatment for choledochal cysts (CCs). In the present study, the results of totally laparoscopic surgery for CCs (TLCCs) in a pediatric population were evaluated. The clinical data of 28 children with CCs between June 2020 and June 2023 were retrospectively reviewed. All patients underwent TLCCs involving cyst excision and RYHJ. The jejunojejunal anastomosis was completed laparoscopically using manual sutures. Age at operation, operative time, postoperative recovery and complications were evaluated. The 28 patients comprised 8 boys and 20 girls who underwent TLCCs at a mean age of 4.2 years (range, 1 month-12.3 years) with a mean weight of 15.9 kg (range, 4.6-43 kg). All patients received ultrasound and magnetic resonance cholangiopancreatography examinations, which revealed a mean cyst diameter and length of 1.74±0.76 cm and 3.85±1.25 cm, respectively. The mean operative time was 214±43.8 min. The mean time until starting an oral diet after surgery was 2.89±1.23 days. Apart from bile leakage and wound infection, no other complications occurred during a median follow-up period of 18 months (range, 4-42 months). TLCCs can be performed safely by skilled surgeons in pediatric patients. TLCCs may be more physiologically compatible and accelerate recovery of intestinal function with reduced trauma and better esthetic outcomes than conventional laparoscopic surgery for CCs. Therefore, TLCCs with manual sutures may be considered as an option for minimally invasive surgery in pediatric patients with CCs. [ABSTRACT FROM AUTHOR]

An increasing proportion of congenital choledochal malformation (CCM) are being detected on antenatal ultrasound. However, the actual timing of its surgical correction remains controversial with some series showing an excess of complications the earlier the operation. The aim of this study was to characterize the pathophysiological aspects of this cohort from the perspective of age at surgery in order to inform a more rational basis for clinical decision-making. We analysed a prospective database of CCM acquired over a 26-year period (Jan. 1997 to Dec. 2022) for patient demography; details of pre- and post-natal imaging; age at surgical intervention; operative complications (Clavien-Dindo classification) and outcome. Data are quoted as median (IQR). All comparisons are non-parametric. A P value of 0.05 was accepted as significant. There were 58 (72% female) children with an antenatally-detected CCM from a total of 265 (21.8%) in the series. These were classified as Type 1C (n = 47; 81%), type 4C (n = 3; 5%) and Type 5 (n = 8; 14%). There were no Type 1F lesions in this cohort. Median age at surgery was 113 (IQR 57–198) days. Postnatal cyst (US) size varied from 12 to 130 mm but there was little evidence of a relationship between this and post-natal liver biochemistry (e.g. bilirubin r S = 0.01, P = 0.44; AST r S = 0.14, P = 0.19). Choledochal pressure measurements (n = 46) showed resting pressures of 12 (9–21) mmHg with no significant correlation with age (P = 0.4) or aspartate aminotransferase (P = 0.2) or γ-glutamyl transferase (P = 0.06). The cohort was divided into 2 groups (Early and Late) based on the median age at surgery (all open) (113 days). Biliary obstruction was more common in the Early group (10 vs. 2; P = 0.01). Two perforations occurred, both in the Early group. With a deliberate policy of regular ultrasound-based follow-up we have seen no anastomotic complications (leak, stenosis, persistent intrahepatic biliary dilatation or stones) or post-operative cholangitis in any child [median follow-up 3.42 (1.30–8.05) years]. This is one of the largest series documenting the outcomes of antenatally-detected CCMs, certainly in Europe and North America. Such lesions are invariably cystic in nature, and either Types 1C, 4C or 5. The absence of complications using a policy of early intervention (where possible) in experienced hepatobiliary units was shown. II. [ABSTRACT FROM AUTHOR]

Abstract Introduction Type IV-A choledochal cyst (CDC) has been considered to have a poor prognosis due to the high incidence of postoperative anastomotic strictures and intrahepatic stones. This study aimed to evaluate the surgical outcomes of children with type IV-A CDC and to provide insights for clinical diagnosis and treatment. Methods The study retrospectively analyzed patients from June 2015 to December 2018 at our center, 76 children were diagnosed with type IV-A CDC. All patients underwent single-incision laparoscopic choledochal cyst excision and Roux-en-Y hepaticojejunostomy. The decision to perform ductoplasty was made by comprehensively considering the intrahepatic duct dilatation (IHDD) and stricture. All patients were followed up 1, 3, and 6 months postoperatively, and then every 1 year thereafter. Patients were categorized into two groups based on IHDD changes postoperatively: the long-term group (LTG), with IHDD persisting for over a year, and the short-term group (STG), where IHDD normalized within a year. Single/multiple factor logistic regression was used to analyse the factors influencing postoperative IHDD. Results The median follow-up period was 80 months, with a range from 64 to 101 months. The decrease in postoperative liver function parameters, compared to preoperative levels, was statistically significant. Two patients (2.63%) developed bile leaks. One patient (1.32%) developed anastomotic stricture. All patients’ IHDD returned to normal size. Fifty-six (73.7%) patients showed normalization of IHDD within one-year postoperatively. The median recovery time for IHDD in patients was 1.65 months, ranging from 3 days to 74 months postoperatively. There were significant differences in intrahepatic biliary sludge and stones and maximum diameter of IHDD between STG and LTG. Logistic regression was used to analyse the factors and found that intrahepatic biliary sludge and a wider maximum diameter of IHDD were risk factors for postoperative long-term IHDD in patients. Conclusion Single-incision laparoscopic hepaticojejunostomy with selective ductoplasty are safe and effective for children with Type IV-A CDC. Long-term follow-up is recommended for children presenting with intrahepatic biliary sludge and larger IHDD.

Abstract Background Biliary dilatation without obvious etiology on cross sectional imaging warrants further investigation. This study aimed to assess yield of endoscopic ultrasound in providing etiologic diagnosis in such situation. Methods Prospective cohort of consecutive patients with biliary dilatation & non diagnostic computed tomography (CT) and /or magnetic resonance imaging (MRI) underwent endoscopic ultrasound (EUS) with/without fine needle aspiration cytology (FNAC) and were followed clinically, biochemically with/without radiology for up to six months. The findings of EUS were corroborated with histopathology of surgical specimens and endoscopic retrograde cholangiography (ERCP) findings in relevant cases. Results Median age of 121 patients completing follow up was 55 years. 98.2% patients were symptomatic and median common bile duct (CBD) diameter was 13 mm. EUS was able to identify lesions attributable for biliary dilatation in (67 out of 121) 55.4% cases with ampullary neoplasm being the commonest (29 out of 67 i.e. 43%). Multivariate logistic regression analysis identified jaundice as the predictor of positive diagnosis on EUS, of finding ampullary lesion and pancreatic lesion on EUS. EUS had sensitivity, specificity, positive predictive value and diagnostic accuracy of 95.65%, 94.23%, 95.65% and 95.04% respectively in providing etiologic diagnosis. Threshold value for baseline bilirubin of 10 mg%, for baseline CA 19.9 of 225 u/L and for largest CBD diameter of 16 mm were determined to have specificity of 98%, 95%, 92.5% respectively of finding a positive diagnosis on EUS. Conclusion EUS provides considerable diagnostic yield with high accuracy in biliary dilatation when cross sectional imaging fails to provide etiologic diagnosis.

Abstract Background Prenatally diagnosed hepatic hilar cysts are a challenging finding for the clinician. They can either be a sign of cystic biliary atresia (BA) or a choledochal cyst (CC), two diagnoses with different postnatal management and prognosis. Based on a case report of four patients, we aim to propose a management algorithm for prenatally diagnosed “hepatic hilar cysts”. Case presentation A hepatic hilar cyst, ranging from 5 to 25 mm, was detected prenatally in all four girls confirmed postnatally along with the presence of a gallbladder. Stool color was normal until two weeks of life at which time the stool color became lighter, and the patients developed cholestasis. All were operated before seven weeks of life: Case 1 had a CC with patent but irregular intrahepatic bile ducts at intraoperative cholangiogram, and no communication with the duodenum. A Roux-en-Y bilioenteric anastomosis was performed. The cyst showed complete epithelial lining loss, and liver pathology showed BA features. Case 2 had the final diagnosis of cystic BA with patent but abnormal intrahepatic bile ducts. She underwent two operations: the first operation at four weeks as described for case 1, since intraoperative findings were similar, as was histology. As cholestasis increased postoperatively, she underwent a Kasai hepato-porto-enterostomy six weeks later, where distinct BA findings were found with complete scarring of the hilar plate. Case 3 had a cystic BA with the cyst located within the common bile duct and atretic bile ducts proximal to the porta hepatis. It exhibited no communication with the liver or duodenum. A Kasai operation was performed, with histology showing complete epithelial loss within the cyst wall and scarring of the hilar plate. Case 4 had a cystic BA presenting a completely obliterated hepatic duct with the cyst lying within the common bile duct. A Kasai procedure was performed. Histology showed a common bile duct with a residual lumen of 0.1 mm. Conclusions The spectrum of disease from CC to BA in the setting of a prenatally discovered hepatic hilar cyst is emphasized. Even if cholangiogram differentiates most patients with BA from those with CC, caution is advised for transitional types.

Pancreaticobiliary maljunction (PBM) is a congenital anomaly where the pancreatic and bile ducts join outside the duodenal wall, resulting in formation of an elongated common channel. In normal physiology, the sphincter of Oddi regulates the junction between the pancreatic and bile ducts. Individuals with PBM lack this regulatory mechanism resulting in reflux of pancreatic juices into the biliary tract. Activated pancreatic enzymes result in chronic inflammation of biliary tract resulting in choledochal cysts which places patients at risk for eventual development of cholangiocarcinoma.A 45-year-old white male presented with jaundice and dark urine. Laboratory tests showed elevated liver enzymes and bilirubin. Diagnostic imaging revealed anomalous biliary anatomy, a Type 1A choledochal cyst, and a mass in the common hepatic duct. Extrahepatic cholangiocarcinoma was confirmed by ERCP and biopsy, with PET/CT indicating localized disease without distant metastases.Treatment included a biliary sphincterotomy, stone drainage, and stenting. The patient underwent a robotic-assisted bile duct resection, cholecystectomy, hepatic lobectomy, and Roux-en-Y hepaticojejunostomy. The surgical specimen showed an invasive, poorly differentiated adenosquamous carcinoma with perineural invasion, but no regional lymph node involvement.PBM is a rare condition, and its diagnosis and management necessitate a multidisciplinary team, including pancreaticobiliary surgeons, endoscopists, and radiologists. Accurate diagnosis hinges on the team's expertise. Radiologists must be aware of PBM and have a thorough understanding of the associated anatomy and imaging characteristics that may indicate high-risk dysplasia or malignancy.

A choledochal cyst (CC) is one of the rare congenital diseases of the biliary tract and is more common in females. The association of ectopic pancreatic and splenic tissue with CC is an extremely rare condition. An 11-year-old girl child presented with a vague abdominal lump and intermittent dull abdominal pain for 5 months with early satiety and weight loss in the current case scenario. Her laboratory findings were normal. A type IV CC was discovered on ultrasonography, and the distal common bile duct displayed significant cystic dilatation on magnetic resonant cholangiopancreatography. After the CC was surgically removed, it was sent for histopathological examination. Sections revealed dual heterotropic elements, which included pancreatic and splenic tissues in a CC. It was very uncommon for both to exist independently in the CC and in this instance, both were observed together. This will be the first documented case, in which the CC contained dual ectopic tissue from the spleen and pancreas. Ectopic splenic tissue in the CC has never been documented in the literature. It is crucial to be aware of this entity when splenic or pancreatic tissue experiences changes due to inflammation, even though its clinical significance is uncertain. In the ectopic rests, bleeding, blockage, and malignant transformation are the possible outcomes.

Abstract Introduction and importance Choledochal cysts are congenital conditions that affect the biliary tree, typically identified during childhood. These cysts present as dilations in the biliary system, occurring either in the extrahepatic or intrahepatic regions or in some cases, in both areas. Occasionally, it may have associated chronic pancreatitis posing a challenge to the management of both conditions. In the case reported, a female child was diagnosed with a choledochal cyst along with chronic pancreatitis. She had an excision of cyst and lateral pancreaticojejunostomy simultaneously for both conditions resulting in favorable outcomes. Case presentation An 8-year-old child visited the clinic with a complaint of upper abdominal pain accompanied by intermittent vomiting. An ultrasound was performed which showed a dilated common bile duct. To confirm the diagnosis, an MRCP and baseline labs including liver function tests were ordered. The findings were suggestive of a type IA choledochal cyst with concomitant chronic pancreatitis. Subsequently, she had choledochal cyst excision with Roux-en-Y hepaticojejunostomy reconstruction plus lateral pancreaticojejunostomy using the classical Puestow’s technique. The patient’s recovery post-surgery was uneventful, resulting in discharge on the fifth post-operative day for routine outpatient follow-up. Clinical discussion Patients afflicted with choledochal cysts are prone to recurrent pancreatitis, though the precise underlying mechanism is unknown. Certain studies propose an association between abnormal pancreatico-biliary junction and recurrent pancreatitis, often defined as a common channel surpassing 15 mm in length. However, this aberration could not be definitively established in the current case. Diagnosis of choledochal cysts can be confirmed through either ERCP or MRCP, with the latter preferred due to its non-invasive nature. The optimal management strategy for choledochal cysts necessitates cyst resection and Roux-en-Y hepaticojejunostomy reconstruction Moreover, in cases associated with chronic pancreatitis, longitudinal pancreatojejunostomy may be undertaken. Conclusion Thus, it is concluded that although rare choledochal cysts may have associated chronic pancreatitis. Surgical excision of the cyst and lateral pancreaticojejunostomy can be performed simultaneously resulting in favorable post-operative outcomes.

Abstract Objective Laparoscopic choledochectomy and hepatojejunostomy have been reported in children since 1995, but this procedure is technically demanding. Robotic surgical systems can simplify complex minimally invasive procedures. Currently, few reports have been made on neonates. We present the experience of 6 cases of neonatal CC(choledochal cysts). Study design Between January 2022 and December 2023, 6 neonates underwent robotic resection of choledochal cyst and hepaticojejunostomy using the Da Vinci surgical system at Children’s Hospital, Zhejiang University School of Medicine, a high-volume hepatobiliary disease center. demographic data of the patients and surgical outcomes were collected and analyzed. Results All 6 patients were successfully treated by robotic cystectomy and hepaticojejunostomy. The mean age was 17.3 days (range 4–25) and the mean weight was 3.6 kg (range 2.55–4.4). 5 cysts were type Ia and 1 was type Iva. The mean diameter of the cysts was 3.8 cm (range 1.25-5). The mean time to establish feeding was 4.83 days (range 4–6), and patients were discharged after a median time of 16.83 days (range 7–42) without postoperative complications. Conclusions This procedure is safe and effective for neonates. The authors found that the use of robot-assisted surgery has ergonomic advantages in this delicate, minimally invasive procedure.

Abstract Background Matrix metalloproteinase-7 (MMP-7) is associated with biliary injury. This study aimed to evaluate the relationships of serum MMP-7 with clinical characteristics in choledochal cysts (CDC) children. Methods Between June 2020 and July 2022, we conducted a prospective study of CDCs who underwent one-stage definitive operation at our center. Serum MMP-7 was measured using an enzyme-linked immunosorbent assay. We evaluated the relationships between serum MMP-7 and age, laboratory tests, imaging examinations, liver fibrosis, MMP-7 expression, and perforation. Results A total of 328 CDCs were enrolled in the study, with a median serum MMP-7 of 7.67 ng/mL. Higher serum MMP-7 was correlated with younger age at diagnosis (p

Introduction and importance: Choledochal cysts are congenital conditions that affect the biliary tree, typically identified during childhood. These cysts present as dilations in the biliary system, occurring either in the extrahepatic or intrahepatic regions or in some cases, in both areas. Occasionally, it may have associated chronic pancreatitis posing a challenge to the management of both conditions. In the case reported, a female child was diagnosed with a choledochal cyst along with chronic pancreatitis. She had an excision of cyst and lateral pancreaticojejunostomy simultaneously for both conditions resulting in favorable outcomes. Case presentation: An 8-year-old child visited the clinic with a complaint of upper abdominal pain accompanied by intermittent vomiting. An ultrasound was performed which showed a dilated common bile duct. To confirm the diagnosis, an MRCP and baseline labs including liver function tests were ordered. The findings were suggestive of a type IA choledochal cyst with concomitant chronic pancreatitis. Subsequently, she had choledochal cyst excision with Roux-en-Y hepaticojejunostomy reconstruction plus lateral pancreaticojejunostomy using the classical Puestow's technique. The patient's recovery post-surgery was uneventful, resulting in discharge on the fifth post-operative day for routine outpatient follow-up. Clinical discussion: Patients afflicted with choledochal cysts are prone to recurrent pancreatitis, though the precise underlying mechanism is unknown. Certain studies propose an association between abnormal pancreatico-biliary junction and recurrent pancreatitis, often defined as a common channel surpassing 15 mm in length. However, this aberration could not be definitively established in the current case. Diagnosis of choledochal cysts can be confirmed through either ERCP or MRCP, with the latter preferred due to its non-invasive nature. The optimal management strategy for choledochal cysts necessitates cyst resection and Roux-en-Y hepaticojejunostomy reconstruction Moreover, in cases associated with chronic pancreatitis, longitudinal pancreatojejunostomy may be undertaken. Conclusion: Thus, it is concluded that although rare choledochal cysts may have associated chronic pancreatitis. Surgical excision of the cyst and lateral pancreaticojejunostomy can be performed simultaneously resulting in favorable post-operative outcomes. [ABSTRACT FROM AUTHOR]

Objective: Laparoscopic choledochectomy and hepatojejunostomy have been reported in children since 1995, but this procedure is technically demanding. Robotic surgical systems can simplify complex minimally invasive procedures. Currently, few reports have been made on neonates. We present the experience of 6 cases of neonatal CC(choledochal cysts). Study design: Between January 2022 and December 2023, 6 neonates underwent robotic resection of choledochal cyst and hepaticojejunostomy using the Da Vinci surgical system at Children's Hospital, Zhejiang University School of Medicine, a high-volume hepatobiliary disease center. demographic data of the patients and surgical outcomes were collected and analyzed. Results: All 6 patients were successfully treated by robotic cystectomy and hepaticojejunostomy. The mean age was 17.3 days (range 4–25) and the mean weight was 3.6 kg (range 2.55–4.4). 5 cysts were type Ia and 1 was type Iva. The mean diameter of the cysts was 3.8 cm (range 1.25-5). The mean time to establish feeding was 4.83 days (range 4–6), and patients were discharged after a median time of 16.83 days (range 7–42) without postoperative complications. Conclusions: This procedure is safe and effective for neonates. The authors found that the use of robot-assisted surgery has ergonomic advantages in this delicate, minimally invasive procedure. [ABSTRACT FROM AUTHOR]

Purpose: To describe clinical features of choledochal cyst (CC) patients in terms of demographic data, clinical presentation, investigations, treatment, and outcomes among children and adults. Methods: The medical records of patients undergoing choledochal cyst (CC) surgery from 2002 to 2021 at a university hospital were retrospectively reviewed. The patients were divided into two groups: children (< 15 years) and adults (≥ 15 years). Descriptive statistics were used. Results: There were 106 cases of CC (Female/male = 88/18, children/adult = 53/53). Abdominal pain was the predominant presenting symptom, followed by jaundice in both groups. Adults were significantly more prone to present with abdominal pain compared to children (86.8% vs. 52.8%; p < 0.001), while children were more likely to experience acholic stool than adults (22.6% vs. 3.8%; p = 0.004). Ultrasound was the preferred investigation screening modality (75.5%). Most patients were presented with type I CC (71.7%). Laparoscopic-assisted approach was performed in 8.5%. CC excision with roux-en-y hepatico-jejunostomy was the main procedure (88.7%). Adults had a higher incidence of post-op complications, including stones, anastomosis stricture, abdominal collection, and cholangitis. Adults were significantly more likely to require intervention after surgery, compared to children (26.4% vs. 5.7%; p = 0.04). Conclusions: Ultrasound was the most common screening tool for diagnosis. Postoperatively, adults with CC experience more serious post-op complications compared to children. This could be attributed to long-standing cystic inflammation. Therefore, prompt definitive surgery is recommended for CC patients. [ABSTRACT FROM AUTHOR]

Background: Matrix metalloproteinase-7 (MMP-7) is associated with biliary injury. This study aimed to evaluate the relationships of serum MMP-7 with clinical characteristics in choledochal cysts (CDC) children. Methods: Between June 2020 and July 2022, we conducted a prospective study of CDCs who underwent one-stage definitive operation at our center. Serum MMP-7 was measured using an enzyme-linked immunosorbent assay. We evaluated the relationships between serum MMP-7 and age, laboratory tests, imaging examinations, liver fibrosis, MMP-7 expression, and perforation. Results: A total of 328 CDCs were enrolled in the study, with a median serum MMP-7 of 7.67 ng/mL. Higher serum MMP-7 was correlated with younger age at diagnosis (p < 0.001), larger cyst sizes (p < 0.001), higher liver fibrosis stages (p < 0.001), and higher incidence of perforation (p < 0.01). Liver MMP-7 was mainly expressed in intrahepatic and extrahepatic biliary epithelial cells. The area under the receiver operating characteristic curve (AUROC) was 0.630 (p < 0.001) for serum MMP-7 in predicting perforation. When serum MMP-7 was combined with γ-glutamyl transferase (GGT), the AUROC increased to 0.706 (p < 0.001). Conclusions: Serum MMP-7 was associated with biliary obstruction in CDCs. Patients with high serum MMP-7 were more likely to have severe liver damage and biliary injury, with higher incidences of liver fibrosis and perforation. [ABSTRACT FROM AUTHOR]

Introduction: The pediatric choledochal cyst with hepatic duct stenosis occurs postoperative hepatolithiasis, recurrent cholangitis, or pancreatitis. The laparoscopic hepatic ductoplasty can prevent these incidences. Objectives: To determine the characteristic of hepatic duct stenosis, laparoscopic treatment, and outcomes in systematic review and meta-analysis. Methodology: We searched the published studies on PubMed, Scopus, and Cochrane Library databases from January 1985 to April 2022 in English language. This protocol was registered to PROSPERO (CRD42022332145). Results: Nine published studies and 412 patients were included. The meta-analysis revealed that the locations were the confluence of the left and right hepatic ducts 43.1%, the left or/and the right hepatic duct 8.3%, and the unclassified location 60.4%. These characteristics included a membranous/septum appearance (44.7%) and a circumferential/relative stenosis (22.7%). The laparoscopic techniques were the wide hilar Roux-en-Y hepaticojejunostomy (28.5%), the excision of membranes/septum (26.5%), and the mixed hepatic ductoplasty (45.0%). The outcomes revealed a minor bile leakage of 3.8% and minimal bleeding. The meta-analysis showed no statistical difference between laparoscopic and open techniques in hepatolithiasis (0% versus 2.00%), anastomosis stricture (4.83% versus 10.00%), and no recurrent cholangitis. There was no conversion rate but showed a trend the prolonged operating time in laparoscopy. Conclusion: Laparoscopic hepatic ductoplasty is safe and effective. The characteristics and location can be feasible laparoscopic procedures. So, hepatic ductoplasty decreases hepatolithiasis, anastomosis stricture, or recurrent cholangitis and may increase minor bile leakage. The systematic review registration was PROSPERO system with CRD42022332145. [ABSTRACT FROM AUTHOR]

OBJECTIVES To assess the effectiveness of retro pyloric Roux-En-Y Hepaticojejunostomy in cases of choledochal cysts and bile duct injuries to reduce complications, such as delayed gastric emptying and acute cholangitis. METHODOLOGY This retrospective cohort study was conducted in the surgical C unit of Hayatabad Medical Complex, Peshawar (January 2019 - December 2022). This study included 37 patients treated for bile duct injuries and choledochal cysts. All patients had an ASA III or above, aged 5 to 36 years. Amongst the 35 patients, 21 patients underwent surgery for choledochal cysts, while 14 patients were treated for bile duct injuries. Patients with type 1choledochal cysts were admitted through OPD after getting diagnosed by ultrasound abdomen and, in some cases, MRCP. RESULTS Out of 36 patients, 23 (63.71%) presented with type I choledochal cysts, while 14 (40%) exhibited bile duct injuries (BDI). Among the 14 BDI cases, 57.14% underwent open cholecystectomy, 42.85% underwent laparoscopic cholecystectomy, and 5.71% received immediate repair during the primary surgery. Conversely, 8.57% of patients with CBD ligation underwent exploration on the 3rd postoperative day upon early detection of the injury. Among the patients diagnosed with BDI after the 7th postoperative day, 25.71% underwent delayed repair at three months. Postoperatively, patients were authorized for fluid consumption within 24-48 hours. Moreover, 4 (11.42%) patients developed a fever of approximately 100°F, managed with intravenous Paracetamol in 2 (5.71%) cases. Patients also presented with subsequent wound infections, which were treated on an outpatient basis through oral antibiotics and wound dressing. CONCLUSION The Retropyloric Roux-en-Y hepaticojejunostomy is a modified biliary reconstruction procedure with minimal morbidity and better outcomes than traditional methods. It reduces complications like delayed gastric emptying and acute cholangitis. We recommend its frequent use for biliary reconstruction when appropriate.

ObjectiveTo compare the efficacy of robotic-assisted single-incision-plus-one-port laparoscopic choledochal cyst excision (R-SILC + 1) and single-incision laparoscopic choledochal cyst (SILC) in treating pediatric choledochal cyst (CDC).MethodsWe retrospectively analyzed the clinical data of patients diagnosed with CDC in our hospital from June 2021 to October 2023. Among them, patients underwent either R-SILC + 1 or SILC procedures. Demographic parameters, operative details, and postoperative outcomes were studied.ResultsA total of forty-nine patients were included, with 23 children undergoing R-SILC + 1 and 26 children undergoing SILC. There were no statistically significant differences in demographic data, postoperative pain scores, and postoperative complication rates between the two groups (all p > 0.05). Compared with the SILC group, the R-SILC + 1 group demonstrated less intraoperative bleeding volume (10.4 ± 3.6 vs. 15.0 ± 3.6 ml, p

Aim: The aim is to study the various histopathological changes in the liver in pediatric patients with choledochal cyst (CC) and correlate with the presentation and type of cyst. Methods: In a prospective observational study including all pediatric patients who underwent CC excision, histopathological changes of the liver in the form of cholestasis (CHS), portal inflammation (PI), bile duct proliferation (BDP), and fibrosis were studied and graded using a scoring system. They were analyzed in relation to age, sex, symptoms, and type of the cyst. Results: All 30 patients of CC showed various degrees of histopathological changes in the liver in the form of CHS, PI, BDP, and liver fibrosis. Patients 1 years had 17/17 (100%) fusiform variety of CC (P < 0.001). Patients 1 years presented with pain abdomen (P < 0.002). Higher grades of liver fibrosis and BDP were seen in the cystic variety compared to the fusiform variety (P < 0.001). However, no significant association was found with CHS and PI (P > 1.23). Conclusions: Histopathological changes in the liver of varying grades are seen in all patients of CC. Patients of CC

Abstract Objective There are no clear evidence-based recommendations concerning when patients with prenatally diagnosed choledochal cysts (CCs) should undergo surgery. This study was primarily designed to explore the proper timing of minimally invasive surgery for prenatally diagnosed CC patients. Methods Seventy-three patients with prenatally diagnosed CC were enrolled in this study and divided into 4 subgroups according to age at surgery (15 patients in the 3 months group). Eighty-five healthy infants were recruited and divided into 4 age groups (29 in the 3 month group). Preoperative data were collected and compared between CC patients and healthy controls in 4 age groups. Additionally, 73 patients were divided into laparoscopic and open groups to compare postoperative recovery indices and the occurrence of complications to determine the safety and feasibility of laparoscopic CC application in neonates and young infants. Results Twenty-one of 73 (28.8%) patients who were prenatally diagnosed with CCs experienced various clinical symptoms, and 15 of 21 (71.4%) patients experienced clinical symptoms less than 2 months after birth. No differences were found in alanine transaminase (ALT), aspartate transaminase (AST) or aspartate transaminase (APRI) levels between CC patients and controls at ≤ 1 month or 1–2 months of age (all p > 0.05), while higher levels were found in CC patients at 2–3 months or > 3 months of age (all p 2 months of age, while DBIL levels 1 week after surgery were also significantly lower than those before surgery in patients who underwent CC excision at ≤ 2 months of age. The initial oral feeding time in the laparoscopic surgery group was significantly earlier than that in the open surgery group for both CC patients who underwent CC excision at ≤ 2 months of age and those > 2 months of age (all p 2 months of age. Conclusion Most clinical symptoms attributed to CC occur less than 2 months after birth, while liver function and liver fibrosis may deteriorate after 2 months of age in patients with prenatally diagnosed CC. Laparoscopic surgery for CC in newborns and young infants (either less than or more than 2 months old) is safe and feasible and can shorten the initial oral feeding time without increasing complications such as postoperative anastomotic leakage or stricture. Thus, performing laparoscopic CC excisions within 2 months after birth in patients with prenatally diagnosed CC may be appropriate.

Abstract Cholangiocarcinoma in patients with Choledochal cysts is rare in childhood; however, it seriously affects the prognosis of the disease. The key to addressing this situation lies in completely removing the extrahepatic cyst. We herein present a case report of a 3-year-old boy with cholangiocarcinoma associated with a choledochal cyst (CDC). Preoperative 3D simulation, based on CT data, played an important role in the treatment of this patient.

Aim: The aim is to study the various histopathological changes in the liver in pediatric patients with choledochal cyst (CC) and correlate with the presentation and type of cyst. Methods: In a prospective observational study including all pediatric patients who underwent CC excision, histopathological changes of the liver in the form of cholestasis (CHS), portal inflammation (PI), bile duct proliferation (BDP), and fibrosis were studied and graded using a scoring system. They were analyzed in relation to age, sex, symptoms, and type of the cyst. Results: All 30 patients of CC showed various degrees of histopathological changes in the liver in the form of CHS, PI, BDP, and liver fibrosis. Patients <1 years had 9/13 (69.2%) cystic variety and those >1 years had 17/17 (100%) fusiform variety of CC (P < 0.001). Patients <1 years frequently presented with jaundice and hepatomegaly and those >1 years presented with pain abdomen (P < 0.002). Higher grades of liver fibrosis and BDP were seen in the cystic variety compared to the fusiform variety (P < 0.001). However, no significant association was found with CHS and PI (P > 1.23). Conclusions: Histopathological changes in the liver of varying grades are seen in all patients of CC. Patients of CC <1 year presented frequently with jaundice, had the cystic type, and had a higher degree of liver damage on histopathology. [ABSTRACT FROM AUTHOR]

Objective: There are no clear evidence-based recommendations concerning when patients with prenatally diagnosed choledochal cysts (CCs) should undergo surgery. This study was primarily designed to explore the proper timing of minimally invasive surgery for prenatally diagnosed CC patients. Methods: Seventy-three patients with prenatally diagnosed CC were enrolled in this study and divided into 4 subgroups according to age at surgery (15 patients in the < 1 month group, 27 in the 1–2 months group, 14 in the 2–3 months group and 17 in the > 3 months group). Eighty-five healthy infants were recruited and divided into 4 age groups (29 in the < 1 month group, 20 in the 1–2 month group, 19 in the 2–3 month group and 17 in the > 3 month group). Preoperative data were collected and compared between CC patients and healthy controls in 4 age groups. Additionally, 73 patients were divided into laparoscopic and open groups to compare postoperative recovery indices and the occurrence of complications to determine the safety and feasibility of laparoscopic CC application in neonates and young infants. Results: Twenty-one of 73 (28.8%) patients who were prenatally diagnosed with CCs experienced various clinical symptoms, and 15 of 21 (71.4%) patients experienced clinical symptoms less than 2 months after birth. No differences were found in alanine transaminase (ALT), aspartate transaminase (AST) or aspartate transaminase (APRI) levels between CC patients and controls at ≤ 1 month or 1–2 months of age (all p > 0.05), while higher levels were found in CC patients at 2–3 months or > 3 months of age (all p < 0.05). ALT, AST and DBIL levels 1 week after surgery were significantly lower than those before surgery in CC patients who underwent laparoscopic CC excision at > 2 months of age, while DBIL levels 1 week after surgery were also significantly lower than those before surgery in patients who underwent CC excision at ≤ 2 months of age. The initial oral feeding time in the laparoscopic surgery group was significantly earlier than that in the open surgery group for both CC patients who underwent CC excision at ≤ 2 months of age and those > 2 months of age (all p < 0.05). No differences were found in the rates of anastomotic leakage or stricture formation between the laparoscopic and open surgery groups at ≤ 2 months or > 2 months of age. Conclusion: Most clinical symptoms attributed to CC occur less than 2 months after birth, while liver function and liver fibrosis may deteriorate after 2 months of age in patients with prenatally diagnosed CC. Laparoscopic surgery for CC in newborns and young infants (either less than or more than 2 months old) is safe and feasible and can shorten the initial oral feeding time without increasing complications such as postoperative anastomotic leakage or stricture. Thus, performing laparoscopic CC excisions within 2 months after birth in patients with prenatally diagnosed CC may be appropriate. [ABSTRACT FROM AUTHOR]

Choledochal cysts are rare congenital anomalies characterized by dilatation of the bile ducts. The most frequent subtype of choledochal cyst is type I, which has fusiform dilatation of the common bile duct and poses significant challenges in diagnosis and management, particularly, in pediatric patients. We present a case report of a child diagnosed with a Type I choledochal cyst, detailing the clinical presentation, diagnostic workup, surgical intervention, and postoperative outcome. Early recognition and appropriate surgical management are crucial for optimal outcomes in children with this rare entity. [ABSTRACT FROM AUTHOR]

Cholangiocarcinoma in patients with Choledochal cysts is rare in childhood; however, it seriously affects the prognosis of the disease. The key to addressing this situation lies in completely removing the extrahepatic cyst. We herein present a case report of a 3-year-old boy with cholangiocarcinoma associated with a choledochal cyst (CDC). Preoperative 3D simulation, based on CT data, played an important role in the treatment of this patient. [ABSTRACT FROM AUTHOR]

Purposes: Dilatation of cystic duct is very rare and had been classified as Todani type VI choledochal cyst. Choledochal cyst combined with dilatation of cystic duct is difficult to diagnose preoperatively. The purpose of this study is to report the rare variants and discuss the significance and laparoscopic management strategy in children. Methods: The subjects for this study were 10 consecutive patients with type VI choledochal cyst who had laparoscopic procedures at our institute between January 2009 and January 2023. Laparoscopic cholecystectomy, excision of the dilated cystic duct, and choledochal cyst were carried out, and the continuity of the biliary duct was re-established through a Roux-en-Y hepaticojejunostomy. Results: Cystic duct combined with the common bile duct dilatation was revealed in all the patients intraoperatively. Laparoscopic procedures were completed with no conversions. The postoperative recovery was uneventful. The mean follow-up duration was 27 ± 12.7 months (range 5–36 months) with no postoperative complications encountered. Conclusions: The rare entity of type VI choledochal cyst should be recognized as a distinct type of choledochal cyst and need to be given enough attention clinically. The laparoscopic procedure is a feasible option for experienced surgeons. [ABSTRACT FROM AUTHOR]

This study aimed to develop a postnatal treatment strategy for infants with prenatally diagnosed congenital biliary dilatation. We performed a retrospective study of patients with prenatal diagnosed congenital biliary dilatation (CBD), aged <1 year who underwent surgery at our hospital between 2013 and 2023. We classified the patients into two groups, the "early group," consisting of patients who could not wait for growth, and required early surgery, and the "scheduled group," consisting of patients who were asymptomatic and could undergo scheduled surgery, and compared them. The parameters for early surgical prediction were AST, ALT, TB, DB, and CRP levels at birth, 1 week, 2 weeks, 1 month, 2 months, and 3 months after birth, and immediately before surgery, as well as the cyst diameter, presence of intrahepatic bile duct dilation, and presence of debris in the common bile duct. During the study period, 15 patients were diagnosed prenatally. The cyst diameter was significantly larger at all points in the early group. Patients with a cyst diameter of >30 mm at birth, intrahepatic bile duct dilatation at birth, and postnatal enlargement of the common bile duct to >30 mm are more likely to develop symptoms early. Blood biochemistry tests showed no significant differences between the two groups. Patients with a cyst diameter >30 mm in the early postnatal period require careful postnatal management and parents should be counseled regarding the high likelihood of their child needing surgery within the first 3 months of life. Level IV. • The postnatal cyst diameter is the key to the treatment strategy for prenatally diagnosed choledochal cysts. • Infants with cyst diameters greater than 30 mm require early surgery. • Neonatal Choledochal cysts with cyst diameters greater than 30 mm require careful management. [ABSTRACT FROM AUTHOR]

ObjectiveThe aim of the study was to develop a prediction nomogram based on clinical factors to assess the risk of postoperative complications in children with congenital choledochal cyst.MethodsThe clinical data from 131 children who underwent choledochal cyst resection and Roux-en-Y hepaticojejunostomy in our hospital between January 2016 and December 2022 were retrospectively analyzed. The general information, clinical symptoms, procedure, biochemical indicators, and imaging data were recorded. A prolonged hospital stay induced by postoperative complications or a follow-up over 6 months was assessed as the event outcome. A logistics regression analysis was performed to screen for risk factors with statistical significance in inducing postoperative complications. Then, with the dataset split into the training group and internal validation group, the nomogram for the prediction of postoperative complications was developed based on a computer algorithm. In addition, the receiver operating characteristic (ROC) curve and calibration curve were performed for nomogram verification.ResultsOf 131 children, the multivariate logistics regression analysis suggested that age ≤2 years [odds ratio (OR) 0.93; 95% confidence interval (CI) 0.15–5.65; p = 0.938], Todani classification type 1 (OR 36.58; 95% CI 4.14–871.74; p = 0.005), cyst wall thickness >0.4 cm (OR 10.82; 95% CI 2.88–49.13; p

ObjectiveThe purpose of this study is to compare the intraoperative and postoperative outcomes of a trans-umbilical single-site plus one robot-assisted surgery and a trans-umbilical single-site laparoscopic surgery in the treatment of choledochal cysts.MethodsWe retrospectively analyzed clinical data from 49 children diagnosed with choledochal cysts who were admitted to our hospital between June 2020 and December 2023. Among these patients, 24 underwent a trans-umbilical single-site plus one Da Vinci robot-assisted surgery (the robot group) and 25 underwent a trans-umbilical single-site laparoscopic-assisted surgery (the laparoscopic group). We compared differences in intraoperative and postoperative outcomes between the two groups.ResultsThere was no significant difference between the two groups of patients in terms of gender, age, weight, clinical symptoms, maximum cyst diameter, type, postoperative complications, and facial expression, leg movement, activity, crying, and comfortability (FLACC) scoring (p > 0.05). Compared with the patients in the laparoscopic group, those in the robot group had less intraoperative bleeding [10 (8–12) vs. 15 (11.5–18) ml, p

Background: The two most commonly performed methods of biliary-enteric reconstruction following choledochal cyst resection are Roux-en-Y hepaticojejunostomy (HJ) and hepaticoduodenostomy (HD). There is a lack of consensus regarding the better technique between them. This study aimed to evaluate the outcomes, efficacy and early complications of HD as a mode of biliary reconstruction after surgical resection of a choledochal cyst. Materials and Methods: This was a multi-institutional prospective study carried out in high-volume tertiary care teaching institutes from January 2010 to December 2022. All children managed with HD following choledochal cyst resection were analysed for their early complications and outcomes. Results: A total of 74 patients were included in this study. There were 59 (79.73%) females and 15 (20.27%) males. Thirty-nine (52.70%) patients had jaundice at the time of presentation. Magnetic resonance cholangiopancreatography was performed in 57 (77.03%) patients following ultrasonography. Intraoperatively, malrotation was present in 2 (2.70%) patients. In our study, operating time ranged from 60 to 195 min (mean: 118 min). Hospital stays ranged from 8 to 17 days (mean: 11.5 days). The post-operative biliary leak was seen in 7 (9.50%) patients, out of which 6 (8.11%) minor leaks were managed conservatively. Roux-en-Y HJ was performed on 1 (1.35%) patient with a major leak. In our series, 4 (5.40%) patients developed cholangitis; post-operative haemorrhagic nasogastric aspirate 5 (6.76%), post-operative pancreatitis 3 (4.05%) and wound infection 4 (5.40%) were observed and managed conservatively. None of the patients in our study developed an anastomotic stricture, bile gastritis and adhesive small bowel obstruction. Conclusion: Resection of choledochal cyst with HD reconstruction is safe and feasible with short operative time. HD is a viable option for operative management of choledochal cyst with low complication rates and faster recovery.

Hepatobiliary scintigraphy using radiopharmaceuticals (RPh) labeled with short-lived technetium‑99m (99mТс) has been used since the mid‑1970s and still retains its diagnostic value. The lecture focuses on key aspects of the pharmacokinetics of lidocaine analogs based on N-substitutions of iminodiacetic acid. The methodology and diagnostic significance of hepatocholescintigraphy with Bromesida® 99mTс (mebrofenin), including computer registration of RPh passage, anatomical and functional characteristics of liver, bile ducts, gallbladder and duodenum, are considered. Scintigraphic semiotics in choledochal cysts, acute and chronic cholecystitis, gallbladder and duodenum dyskinesia, are discussed. The presented clinical case illustrates the possibilities of Bromesida® 99mTс scintigraphy in the gallbladder form anomaly.

A choledochal cyst is a rare condition that requires surgical treatment to prevent complications, such as obstructive jaundice, cyst rupture, cholangitis, and the risk of malignancy. Complete cyst excision is considered the best option, as it reduces the risk of inflammation and the development of cholangiocarcinoma. Therefore, cholecystectomy and complete cyst resection followed by reconstruction with a Roux-en-Y hepaticojejunostomy is the treatment of choice. We present a case (with video) that shows the complete resection of a type I choledochal cyst with Roux-en-Y reconstruction of two separate ducts since the right posterior duct reached the cyst independently. The laparoscopic approach offers all the advantages of mini-invasive surgery and better visualization of the structures; however, biliary reconstruction to fine ducts implies a surgical challenge that requires high training in mini-invasive surgery. [ABSTRACT FROM AUTHOR]

The aim of the study is to clarify the clinicopathological and biliary morphological characteristics in reported cases of diverticular congenital biliary dilatation (CBD). Using PubMed and the Japan Medical Abstracts Society, articles on possible diverticular CBD were extracted and the clinical pictures examined. We also sought evidence for definitions of diverticular CBD and the associated condition of pancreaticobiliary maljunction (PBM) using the original articles by Alonso-Lej and Todani. The characteristic biliary morphologies of cases with images were also investigated. Analyses of 211 possible cases superficially demonstrated multiple diverticula in 12 (12%) and single diverticulum in 89 (88%), with diverticula located in the upper (n = 38, 38%), middle (n = 32, 32%), or lower (n = 26, 26%) biliary tract in and presence of intra-diverticular stones, PBM, and biliary carcinoma in 23% (n = 18), 39% (n = 25), and 11% (n = 14), respectively. However, evidence defining diverticular CBD or justifying the lack of associated PBM was not demonstrated even in the original articles. Scrutiny of the biliary anatomy in 59 cases with images showed incorrect inclusions of types I or IV-A with an irregular biliary duct wall or dilated cystic duct, periampullary choledochal diverticula, or even solitary biliary cysts. Authentic diverticular CBD, representing the diverticulum connected to the middle of the common bile duct via a thin, patent stalk was seen in only 6 cases. Real diverticular CBD appears extremely rare. The lack of an objective definition allows wide interpretations of clinical pictures, creating inconsistencies in the diagnosis and treatment of CBD and raising questions regarding the utility of conventional classifications. Level III. • Diverticular CBD is included in the classic Alonso-Lej and Todani classifications of congenital biliary dilatation (CBD) as a Type 2 cyst. • Scrutiny of previous publications on potential diverticulae of the common bile duct showed a lack of evidence for this type suggesting extreme rarity, and rather overdiagnosis and erroneous inclusion of various biliary morphologies. • The inclusion of this diverticular type in conventional classifications of congenital biliary dilatation has created uncertainties and inconsistency and should probably be removed. [ABSTRACT FROM AUTHOR]

The approach to patients with choledochal cysts (CCs) remains varied and subject to institutional practices. Owing to the rarity of the disease, the optimal treatment remains poorly defined, particularly in the adult population. This study aimed to review the literature on adult patients with CCs to evaluate trends of diagnosis and management in Western countries. A literature search of 3 electronic databases was performed on adult patients diagnosed with CCs in Western institutions. A review of published literature was completed with comprehensive screening by 2 independent reviewers. Studies were analyzed, and data on surgical approach, malignancies, and follow-up were collected. Findings are presented in concordance with the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. Of the 3488 articles retrieved, 21 studies evaluated Western adults with CCs for a combined population of 1337 patients. The most common Todani subtypes included types I (64%) and IV (22%). Symptoms at presentation included abdominal pain and jaundice, although many were asymptomatic. Ultrasound was used most frequently for diagnosis, followed by computed tomography and endoscopic cholangiopancreatography. The combined malignancy rate was 10.9%, with cholangiocarcinoma being the most prevalent. Complete extrahepatic cyst resection was standard for type I and IV CCs. Among malignancies, 18.5% and 16.4% were observed in patients with prior resection and internal drainage, respectively. A significant proportion of patients who undergo resection of CC disease harbor malignancy. Cancer risk seems reduced but not eliminated with complete resection, which remains the standard treatment. Additional studies are needed to standardize guidelines for the diagnosis and postoperative care of patients in Western countries. [ABSTRACT FROM AUTHOR]

In this study, the biliary-enteric anastomosis following choledochal cyst excision was studied using continuous suture and interrupted suture procedures in a pediatric population. Patients and methods In this study, information on 57 children who received continuous suture choledochal cyst surgery at our facility between 2015 and 2020 was gathered retrospectively. The patients that were enrolled were divided into two groups: continuous suture (n=24) and interrupted suture (n=33). Anastomotic leakage, anastomotic duration in minutes, hospital stay in days, and cost were all compared between these groups. Results The incidence of anastomotic leakage was higher in the interrupted suture group (4.17 vs. 6.6% for continuous suture and interrupted suture, respectively), but the difference was statistically insignificant (P=0.63). The groups did not differ regarding hospital stays (P=0.25). The mean time required to complete the anastomosis in the continuous suture group was 15.3±4.1 min, compared with 23.5±5.2 min in the interrupted suture group (P<0.0001). The costs were €5 ±0.0 in the continuous suture group and €30±6.4 in the interrupted suture group (P<0.0001). Conclusion The anastomotic leakage and hospital stay did not differ between the continuous suture and interrupted suture groups. In contrast, the continuous suture group had a considerable advantage over the interrupted suture group in terms of anastomotic duration and cost. [ABSTRACT FROM AUTHOR]

Background: The two most commonly performed methods of biliary-enteric reconstruction following choledochal cyst resection are Roux-en-Y hepaticojejunostomy (HJ) and hepaticoduodenostomy (HD). There is a lack of consensus regarding the better technique between them. This study aimed to evaluate the outcomes, efficacy and early complications of HD as a mode of biliary reconstruction after surgical resection of a choledochal cyst. Materials and Methods: This was a multi-institutional prospective study carried out in high-volume tertiary care teaching institutes from January 2010 to December 2022. All children managed with HD following choledochal cyst resection were analysed for their early complications and outcomes. Results: A total of 74 patients were included in this study. There were 59 (79.73%) females and 15 (20.27%) males. Thirty‑nine (52.70%) patients had jaundice at the time of presentation. Magnetic resonance cholangiopancreatography was performed in 57 (77.03%) patients following ultrasonography. Intraoperatively, malrotation was present in 2 (2.70%) patients. In our study, operating time ranged from 60 to 195 min (mean: 118 min). Hospital stays ranged from 8 to 17 days (mean: 11.5 days). The post-operative biliary leak was seen in 7 (9.50%) patients, out of which 6 (8.11%) minor leaks were managed conservatively. Roux‑en‑Y HJ was performed on 1 (1.35%) patient with a major leak. In our series, 4 (5.40%) patients developed cholangitis; post‑operative haemorrhagic nasogastric aspirate 5 (6.76%), post‑operative pancreatitis 3 (4.05%) and wound infection 4 (5.40%) were observed and managed conservatively. None of the patients in our study developed an anastomotic stricture, bile gastritis and adhesive small bowel obstruction. Conclusion: Resection of choledochal cyst with HD reconstruction is safe and feasible with short operative time. HD is a viable option for operative management of choledochal cyst with low complication rates and faster recovery. [ABSTRACT FROM AUTHOR]