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Purpose: Laparoscopic resection of choledochal cyst (CC) has become a popular approach. As the discussion about optimal treatment and technical strategies continues, we aimed to investigate perspectives of IPEG members., Methods: An online survey was conducted in 2023 on behalf of the IPEG Research Committee. IPEG members were asked to complete an anonymous questionnaire that included 36 items on the management of CC., Result: 148 members responded to the survey (North America:49/Asia:44/Europe:23/South America:21/Others:11) and 116 completed all questions. Most surgeons (92.5%) operate on less than 5 cases annually. Diagnostic tools of choice were Magnetic Resonance Imaging (MRI, 95.9%) and ultrasonography (US, 74.5%). Regarding fusiform-type CC, operative indications were cyst size greater than 10 mm (68.9%), typical symptoms (78.5%), or anomalous pancreatico-biliary junction (63.8%). In unilateral intrahepatic biliary cysts (type IVa) cases, 81.3% of respondents do not perform a simultaneous liver resection with the initial cyst resection. While 22.0% resect the CC at diagnosis, even if asymptomatic, a larger group of surgeons (41%; 49/118) wait until the infant reaches six months. Intraoperative cholangiography and choledochoscopy are performed routinely by 38.9% and 13.7%, respectively. The majority (52.5%) ligates the common bile duct stump just below the CC. Laparoscopic reconstructions are performed by retrocolic hepatico-jejunostomy (48.3%) or hepatico-duodenostomy (45.8%) at similar rates, but when done open, 71.2% of respondents prefer retrocolic hepatico-jejunostomy. For the laparoscopic anastomosis, interrupted sutures with intracorporeal knot tying were most often utilized (48.3%)., Conclusion: Inidividual pediatric surgeons treat a small number of patients with CC each year. Laparosopic and open reconstruction techniques vary, likely due to technical challenges., Level of Evidence: III., Competing Interests: Conflict of interest The authors declare no conflict of interest associated with this study., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

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., (© 2024. The Author(s), under exclusive licence to European Society of Radiology.)

A choledochal cyst is a rare condition with a variable incidence and different modalities of presentation according to the context and the age of patients. Early diagnosis and complete resection are the only therapeutic options to prevent complications and malignancies. Type Ia, cystic dilatation of the entire extrahepatic duct, is the most observed, and a giant size (more than 10 cm in diameter) may rarely be reached. This patient group often has a long-lasting clinical history and a shared experience of uneasy access to specialist care. Huge dimensions are a complex challenge for complete resection, and the risk of permanent liver functional damage must be considered, as in the current case., (Copyright © 2024 Copyright: © 2024 Annals of African Medicine.)

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., (© 2024. The Author(s).)

Introduction: 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., Methods: 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., Results: 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]., Conclusions: 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., Evidence Level: II., (Copyright © 2024 Elsevier Inc. All rights reserved.)

We report the case of a 91-year-old female with acute cholangitis and long-standing symptoms of recurrent colic- related abdominal pain after cholecystectomy. She was diagnosed by abdominal CT of saccular dilation of the intramural bile duct in the duodenum suggesting choledococele. ERCP was performed in which the presumptive diagnosis was confirmed, and choledochotomy and choledochoplasty were done with excellent subsequent progress. Choledococele is the least common type of biliary cyst. It consists of a cystic dilation of the intramural portion of the common bile duct that protrudes into the duodenal lumen and causes symptoms of recurrent abdominal pain and biliary events. ERCP is a key test in both diagnosis and treatment except when they cannot be approached by this technique or malignancy is suspected.

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., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Giant choledochal cysts are rare, and so little data exist on the best surgical treatment method. We present here, a case of a giant choledochal cyst that was successfully excised by laparoscopic resection. A 37-year-old female presented with right upper abdominal pain and mild jaundice. On examination she had a right upper abdominal mass which on imaging was observed to be a giant choledochal cyst of type IVa, measuring approximately 129 mm × 190 mm. Her blood test results showed abnormal liver function. We successfully performed laparoscopic resection of the cyst, the patient recovered well and was discharged from hospital eight days post-operation without any complications. We wish to share the experience of this rare case and provide some clinical basis for future diagnosis and use of laparoscopic resection in the treatment of giant choledochal cysts., Competing Interests: Declaration of conflicting interestsThe authors declare that there are no conflicts of interest.

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., (© 2024. The Author(s).)

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., (© 2024. The Author(s).)

Several diseases originate from bile duct pathology. Despite studies on these diseases, certain etiologies of some of them still cannot be concluded. The most common disease of the bile duct in newborns is biliary atresia, whose prognosis varies according to the age of surgical correction. Other diseases such as Alagille syndrome, inspissated bile duct syndrome, and choledochal cysts are also time-sensitive because they can cause severe liver damage due to obstruction. The majority of these diseases present with cholestatic jaundice in the newborn or infant period, which is quite difficult to differentiate regarding clinical acumen and initial investigations. Intraoperative cholangiography is potentially necessary to make an accurate diagnosis, and further treatment will be performed synchronously or planned as findings suggest. This article provides a concise review of bile duct diseases, with interesting cases., Competing Interests: Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article., (©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.)

The objective of this study was to analyse the prevalence, risk factors and need for intervention in a sample of Indian children with choledochal cyst (CDC) complicated by pancreatitis with a special focus on chronic pancreatitis. A retrospective review of medical records of children admitted with CDC over 11 years was done and pancreatitis identified using INSPPIRE guidelines. Children were divided into two groups-one having choledochal cyst alone and the other choledochal cyst along with pancreatitis to determine associated risk factors. 40.2% of children with CDC had pancreatitis based on elevation of enzymes or radiological imaging. Age, total bilirubin and indirect bilirubin, requirement of intervention was significantly higher in the group with pancreatitis. 47% of those with radiological features of pancreatitis had imaging features of chronic pancreatitis. Chronic pancreatitis has not been reported previously in children with CDC and maybe peculiar to the Indian subcontinent., (© 2024 European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Background: This study aimed to develop a postnatal treatment strategy for infants with prenatally diagnosed congenital biliary dilatation., Methods: 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., Results: 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., Conclusions: 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 of Evidence: Level IV., Competing Interests: Declarations of interest None., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Choledochocele is defined as a congenital dilatation of the distal intramural part of the common bile duct protruding into the wall of the descending duodenum, typically without pancreaticobiliary maljunction. However, some cases present with a similar pathophysiology to pancreaticobiliary maljunction, including reciprocal reflux of pancreatic juices and bile, leading to protein plugs, pancreatitis, and biliary tract carcinogenesis. Choledochocele is relatively rare and its anatomy, physiology, pathology, and clinical features are thus not well known. We describe a patient with choledochocele who suffered from repeated severe acute pancreatitis and underwent subtotal stomach-preserving pancreatoduodenectomy, in whom the pathological findings of choledochocele showed hyperplasia., (© 2023. Japanese Society of Gastroenterology.)

Aim of the Study: The aim of the study is to clarify the clinicopathological and biliary morphological characteristics in reported cases of diverticular congenital biliary dilatation (CBD)., Method: 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., Results: 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., Conclusion: 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 of Evidence: Level III., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: 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., Methods: 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., Results: 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., Conclusions: 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., (Copyright © 2023. Published by Elsevier Inc.)

Background: This study aimed to screen the impact factors for clinical symptoms of prenatally diagnosed choledochal cysts (CDCs), to warn about the occurrence of clinical symptoms and the timing of surgery., Methods: Medical records of patients with prenatally diagnosed CDCs admitted to our hospital from April 2013 to April 2018 were retrospectively reviewed. Fetal hilar or abdominal cysts were found by prenatal ultrasonogram. All patients underwent laparoscopic cyst excision and hepaticojejunostomy in our center. Univariate analysis and multivariate logistic regression analysis were performed to screen the factors related to clinical symptoms intimately., Results: Two hundred eighteen cases were included. One hundred thirty-four patients (134/218, 61.5%) presented clinical symptoms before surgery. The results of univariate analysis showed that patients with clinical symptoms had earlier time of prenatal diagnosis (P = 0.002), higher values of GGT, TBIL, DBIL (P < 0.001, P < 0.001, P < 0.001, respectively) and larger maximum diameter of cyst before surgery (P = 0.012). Multivariate logistic regression analysis suggested that the time of prenatal diagnosis (P = 0.001, OR = 0.898, 95% CI: 0.845 ~ 0.955) and the GGT value within one week of life (P = 0.028, OR = 1.002, 95% CI: 1.000 ~ 1.003) were independent influencing factors for symptoms., Conclusions: For children with prenatally diagnosed CDCs, approximately 2/3 patients presented noticeable clinical symptoms before surgery. The time of prenatal diagnosis and the GGT value within 1 week of life were independent impact factors for the occurrence of clinical symptoms., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Background: The aim was to investigate the characteristics, surgical management, and outcomes of asymptomatic patients with antenatally diagnosed choledochal cysts (ADCCs) and to justify the strategy of laparoscopic surgery (LS) for them in our hospital., Methods: We developed our LS strategy for asymptomatic ADCCs. Patients with asymptomatic ADCCs who underwent LS or open surgery (OS) during January 2010 and January 2020 were included. Patients with recorded symptomatic ADCCs were exclude. All data of group LS and group OS were statistically compared and analyzed., Results: Twenty-five patients received LS and 18 patients received OS were included. There was no significant difference in baseline characteristics between the groups. A total of 65.1% of biliary sludge formation was detected preoperatively by ultrasonography (US) (72.0% in group LS, 55.6% in group OS, P = 0.26). Compared to the OS group, the LS group had a longer operative time (313.4 ± 27.2 vs. 154.0 ± 11.9 min, P = 0.02), shorter postoperative fasting period (3.1 ± 1.2 vs. 6.2 ± 2.3 days, P = 0.03), and shorter postoperative hospital stay (5.1 ± 1.9 vs. 9.2 ± 1.1 days, P = 0.03). The incidence of late complications, such as reflux cholangitis, adhesive intestinal obstruction, and biliary enteric anastomotic stricture with stone formation, was not significantly different between the two groups. The liver function and liver stiffness of all patients in the two groups were normal., Conclusions: Based on the strategy for asymptomatic ADCCs in our hospital, the perioperative safety and midterm follow-up results after LS were satisfactory and comparable to those after OS., (© 2023. The Author(s).)

Choledochal cyst (CC) or congenital biliary dilatation, has a skewed distribution with hereditary features that is far more common in East Asian females. CC is usually associated with pancreaticobiliary malunion (PBMU) forming a common channel. CC requires early definitive diagnosis, since there is a risk for malignancy occurring in the CC and/or intrahepatic bile ducts (IHBD). Complete CC excision and Roux-en-Y hepaticoenterostomy is required and can be performed by open or minimally invasive surgery with hepatojejunostomy the recommended procedure of choice. Principles of open surgical intervention form the basis of minimally invasive management with laparoscopy and robotic assistance. Current surgical management is associated with fewer early and late complications, such as hepaticoenterostomy anastomotic leakage, cholangitis, anastomosis stricture, and cholangiocarcinoma. Specific features of CC management at Juntendo include: intraoperative endoscopy of the common channel and IHBD for inspecting and clearing debris to significantly reduce post-operative pancreatitis or stone formation; near infra-red fluorescence with indocyanine green for visualizing tissue planes especially during minimally invasive surgery for CC; and a classification system for CC based on PBMU that overcomes inconsistencies between existing classification systems and clinical presentation., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: The purpose of this study was to analyze the outcomes of the combination of ultrasound (US)-guided percutaneous external drainage and subsequent definitive operation to manage complicated choledochal cyst in children., Methods: This retrospective study included 6 children with choledochal cyst who underwent initial US-guided percutaneous external drainage and subsequent cyst excision with Roux-en-Y hepaticojejunostomy between January 2021 and September 2022. Patient characteristics, laboratory findings, imaging data, treatment details, and postoperative outcomes were evaluated., Results: Mean age at presentation was 2.7 ± 2.2 (0.5-6.2) years, and 2 patients (2/6) were boys. Four patients (4/6) had a giant choledochal cyst with the widest diameter of ≥ 10 cm and underwent US-guided percutaneous biliary drainage on admission or after conservative treatments. The other 2 patients (2/6) underwent US-guided percutaneous transhepatic cholangio-drainage and percutaneous transhepatic gallbladder drainage due to coagulopathy, respectively. Five patients (5/6) recovered well after US-guided percutaneous external drainage and underwent the definitive operation, whereas 1 patient (1/6) had liver fibrosis confirmed by Fibroscan and ultimately underwent liver transplantation 2 months after external drainage. The mean time from US-guided percutaneous external drainage to the definitive operation was 12 ± 9 (3-21) days. The average length of hospital stay was 24 ± 9 (16-31) days. No related complications of US-guided percutaneous external drainage occurred during hospitalization. At 10.2 ± 6.8 (1.0-18.0) months follow-up, all patients had a normal liver function and US examination., Conclusions: Our detailed analysis of this small cohort suggests that US-guided percutaneous external drainage is technically feasible for choledochal cyst with giant cysts or coagulopathy in children, which may provide suitable conditions for subsequent definitive operation with a good prognosis., Trial Registration: Retrospectively registered., (© 2023. The Author(s).)

Over the past two decades, there have been an increasing number of reports describing a sixth type of choledochal cyst (cystic duct cyst) in adults that was not included in Todani's classification. This sixth entity has not yet been systematically reviewed in the literature. We therefore explored this condition in adults from the perspective of the clinical presentation, diagnosis and treatment through a systematic review of the evidence. The final analysis included 33 reported cases, with 55% of them reported in Asia. The mean age was 39.3 years old, with a female-to-male ratio of 1.5:1. Magnetic resonance cholangiopancreatography was accurate in establishing the diagnosis in 69% of cases. Where reported, standard laparoscopic/open cholecystectomy was performed in about 74% of patients, while around 25% of them needed extensive surgery. Associated malignancy was reported in 6.1% of cases, while 28% of patients had co-existent gallstone-related disease. No significant post-operative morbidity or mortality was reported. In this era of emergency laparoscopic cholecystectomy, surgeons should be aware of this rare condition, with the particular understanding that it is associated with gallstone-related disease in a significant number of reported cases., (© 2022. The Author(s) under exclusive licence to Springer Nature Singapore Pte Ltd.)

Background/purpose: Congenital biliary dilatation (CBD) is a disease associated with pancreaticobiliary maljunction. The most frequent postoperative complication is intrahepatic stones, which are caused by hilar bile duct stenosis (HBDS)., Methods: We retrospectively reviewed the records of patients who underwent primary surgery for CBD between 2013 and 2021. We evaluated images and videos of HBDS, the laparoscopic technique of releasing the stenosis, and its occurrence rate and compared intraoperative bile duct findings with those of preoperative magnetic resonance cholangiopancreatography (MRCP)., Results: There were 87 CBD cases in this study. HBDS occurred in 52% and preoperative MRCP for HBDS showed a 96% sensitivity and 74% specificity in this study. Bile duct plasty was performed in 45% of the cases and videos demonstrated typical methods of laparoscopic bile duct plasty. The mid- to long-term complications were hepatolithiasis in three patients, anastomotic site stricture in three, and postoperative obstruction in two., Conclusions: Our study revealed that preoperative imaging studies are useful for the prediction of HBDS. Our laparoscopic meticulous probing method for finding BDS reveals more intrahepatic BDS through magnification. Therefore, this may reduce the incidence of intrahepatic stones. Preoperative imaging and intraoperative findings can be complemented to plan management that reduces long-term complications., (© 2022 Japanese Society of Hepato-Biliary-Pancreatic Surgery.)

Purpose: This study aimed to evaluate the effect of prenatal diagnosis at different gestational times on the clinical features of patients with choledochal cysts (CDCs)., Methods: Medical records of patients with prenatally diagnosed CDCs admitted to our hospital (April 2013-April 2018) were retrospectively reviewed. The clinical characteristics and pathological CDC features were analyzed., Results: Two hundred eighteen cases were included. Patients were divided into two groups. Group 1 and group 2 had a prenatal diagnosis at ≤ 27 weeks of gestation (second trimester of gestation, n = 157) and > 27 weeks (third trimester of gestation, n = 61), respectively. The incidence of jaundice and the TBIL, IBIL and GGT levels were higher in Group 1 (P = 0.021, P = 0.029, P = 0.042, P = 0.007, respectively). The maximum cyst diameter at the time of surgery was larger in Group 1 (P = 0.015). An association study showed that the time of prenatal diagnosis was negatively correlated with the maximum cyst diameter both postnatally (r = - 0.223, P = 0.001) and at the time of surgery (r = - 0.268, P < 0.001)., Conclusion: Unlike patients diagnosed at a late prenatal age, patients diagnosed at an early prenatal age tend to present clinical symptoms (jaundice, manifested as high indirect bilirubin), hepatic function damage, and large cysts at the time of surgery., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Choledochal cysts (CCs) are rare cystic dilatations of the intrahepatic and/or extrahepatic bile ducts. We review the pathophysiology, diagnosis, and management of CCs., Methods: MEDLINE/PubMed and Web of Science databases were queried for "choledochal cyst", "bile duct cyst", "choledochocele", and "Caroli disease". Data were synthesized and systematically reviewed., Results: Classified according to the Todani Classification, CCs are generally believed to arise secondary to reflux of pancreatic enzymes into the biliary tree due to anomalous pancreaticobiliary duct union. Complications of CCs include abdominal pain, jaundice, cystolithiasis, cholecystitis, pancreatitis, liver abscess, liver cirrhosis and malignant transformation (3-7.5%). Radiological and endoscopic imaging is the cornerstone of CC diagnosis and full delineation of cyst anatomy is imperative for proper management. Management is generally guided by cyst classification with complete cyst excision necessary for CCs with high potential of malignant transformation such as types I and IV. 5-year overall survival after choledochal cyst excision is 95.5%., Conclusion: Most CCs should undergo surgical intervention to mitigate the risk of cyst related complications such as cholangitis and malignant transformation., (Copyright © 2022 International Hepato-Pancreato-Biliary Association Inc. Published by Elsevier Ltd. All rights reserved.)

Background/purpose: There is currently no consensus on the timing of operative correction for patients with a prenatal diagnosis of choledochal cyst (CDC). This study aims to retrospectively analyze patients with prenatally diagnosed CDCs to identify the optimal timing of operative correction and the importance of cyst size as a predictor of the appearance of symptoms related to the CDC., Methods: We reviewed 125 patients with a prenatal diagnosis of CDC who were admitted to Guangzhou Women and Children's Medical Center from July 2015 to July 2020. After dividing the patients into a symptomatic group (n = 37) and an asymptomatic group (n = 88), according to whether they had any clinical symptoms at the time of their operation, we compared their clinical data and postoperative outcomes. The asymptomatic group was divided into a <1 month group; a ≥1 month and <4 months group; and a ≥4 months group according to their postnatal age at operation; postoperative complications of the three groups were then compared. We were also interested in the effect of cyst size (width and length) for predicting the development of symptoms related to the CDC., Results: The time of onset of symptoms after birth was mainly concentrated in the first 3 months (48.6%). The median width and length of cysts measured by preoperative magnetic resonance cholangiopancreatography in the symptomatic group were greater than those in the asymptomatic group (43 mm vs 28 mm and 71 mm vs 45 mm, respectively; P < .05). The serum levels of the liver-related enzymes ALT, AST, and GGT, and the serum level of DBIL, were greater in the symptomatic group than in the asymptomatic group (P < .05). The operative time, intraoperative blood loss, and duration of postoperative hospital stay in the symptomatic group were greater than those in the asymptomatic group (P < .05). In the asymptomatic group, there were no statistically significant differences in the surgical data and postoperative complications between the <1 month group, the ≥1 month and <4 months group, and the ≥4 months group. The area under the receiver operating characteristic curve (AUROC) of the length of the cyst in predicting symptoms was 0.747, the best cut-off point was 5.2 cm, and the sensitivity and specificity were 78% and 70%, respectively. The AUROC of the width of the cyst was 0.704, the best cut-off point was 4.1 cm, and the sensitivity and specificity were 68% and 75%, respectively., Conclusion: We maintain that it is advantageous to receive surgical treatment in the asymptomatic period for patients with a prenatally diagnosed CDC. A cyst size of length >5.2 cm and width >4.1 cm suggested that clinical symptoms might appear, and that surgery should be carried out as soon as possible, even in the neonatal period., (© 2022 Japanese Society of Hepato-Biliary-Pancreatic Surgery.)

Abstract: Duplication of the gallbladder represents a variant of anomalous biliary anatomy that is rarely encountered on hepatobiliary scintigraphy. We describe the case of 76-year-old man with a gallbladder fold mimicking duplication of the gallbladder or an associated choledochal cyst on scintigraphy. Correlative imaging with CT and ultrasound helped elucidate the true anatomy. This case demonstrates the type of difficult scintigraphic patterns that can arise with variant anatomy and the necessary role of cross-sectional, anatomic imaging in unraveling such situations., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Competing Interests: Declaration of Competing Interest None declared.

Purpose: Hepatobiliary scintigraphy is a minimally invasive imaging method that evaluates bile flow dynamics. At our hospital, it has been performed for postoperative evaluation of patients with choledochal cysts (CC). This study evaluated the usefulness of biliary scintigraphy for predicting late complications in patients with CCs., Methods: The study included pediatric patients with CC who underwent surgery at Chiba University Hospital from 1978 to 2020, followed by postoperative biliary scintigraphy and subsequent radiologic evaluation. The patients were divided into two groups according to the presence or absence of "biliary cholestasis" on biliary scintigraphy., Results: The study included 108 patients, with a median age at surgery of 2 years and 11 months. The median follow-up period was 5203 days, with 11 hepatolithiasis cases and 8 cholangitis cases. No patients had cholangiocarcinoma. Twelve patients were considered to have "cholestasis" following biliary scintigraphy evaluation. There was no significant difference in the occurrence of hepatolithiasis between the cholestasis and non-cholestasis groups (p = 0.47), but cholangitis was significantly more common in the cholestasis group (p = 0.016)., Conclusion: Biliary cholestasis on postoperative hepatobiliary scintigraphy was a risk factor for cholangitis in patients with CCs. These particular patients should be monitored carefully., (© 2022. The Author(s).)

Background: Duplication of the common bile duct (CBD) is extremely rare among the anatomical variations in the biliary tract system, which presents a septum within the CBD or an accessory CBD. In our study, we report a rare case of duplication of the common bile duct combined congenital biliary dilatation.we present a rare case of a septum in the dilated biliary tract., Case Presentation: We reported a 5-year-old Asian girl who had history of repeated abdominal pain for 4 days and aggravated for 1 day. Magnetic resonance cholangiopancreatography (MRCP) examination revealed duplicated common bile duct (DCBD) malformation with congenital biliary dilatation and distal cholelithiasis. The patient underwent choledochal cyst resection and biliary tract reconstruction and abdominal cavity irrigation and drainage under general anesthesia. A septum was found within the common bile duct during the operation. The septum divided the extrahepatic bile duct into two parts connected to the left and right hepatic ducts respectively and the gallbladder is attached to the repeated right bile duct which was not previously reported in the literature., Conclusions: We complement and adjust the classification of common bile duct duplication by reviewing the literature., (© 2022. The Author(s).)

Background: The purpose of this study is to evaluate the accuracy of prenatal MRI in diagnosing choledochal cysts (CDC), evaluate the sensitivity and specificity of MRI signs in the diagnosis of fetal CDC, and first compare the trend of size of CC between prenatal and postpartum., Methods: A total of 18 fetal who were diagnosed with CDCs through prenatal MRI were enrolled in the study. We summarized and analyzed the prenatal clinical data and prognosis information of prenatal and postpartum surgery, then compared the sensitivity, specificity, and diagnostic accuracy of various signs of MRI and postpartum MRCP diagnosis of CC. Finally, we tried to compare the earliest prenatal detection of common bile duct cysts with the size of surgery, and calculated the growth rate of common bile duct cysts for the first time., Results: All 18 patients were delivered in our institution. Among these patients, 14 were confirmed with CDCs after postpartum surgery, two patients had CDCs that disappeared, and two patients were confirmed with cystic biliary atresia (CBA) through the Kasai operation. Furthermore, 13 patients with CDCs and two patients with CBA underwent MRCP before the operation, and one patient with CDCs ruptured at birth and underwent ultrasound diagnosis. The sensitivity and diagnostic compliance of prenatal MRI signs for the location were higher when compared to postnatal MRCP (100% vs. 76.9% and 83.3% vs. 66.7%): the cyst was located at the porta hepatis, which was higher than the lowest edge of the liver, and parallel to the hepatoduodenal ligament., Conclusion: Prenatal MRI is higher than that of US for diagnosing CDCs, specifically in identifying the location of the cyst and confirming the origin of the cyst. The length, width, and size of the CDC become slightly bigger in our study., Competing Interests: All authors have contributed significantly to the manuscript and declare that the work is original and has not been submitted or published elsewhere. None of the authors have any financial disclosure or conflict of interest., (Copyright © 2022 Huiying Wu et al.)

Cholestatic jaundice characterized by elevated conjugated bilirubin can be due to multitude of factors in neonates and childhood. Extrahepatic biliary atresia (EHBA), choledochal cyst, neonatal hepatitis, cytomegalovirus (CMV), and biliary plug are some of the common causes in neonate and early infancy. Causes in late infancy and childhood comprises viral hepatitis, choledochal cyst, cholelithiasis, worm infestation, and biliary compression secondary to extrinsic causes (node, collection, tumor). Some serious disorders like biliary atresia must be considered with the emphasis on early diagnosis of treatable causes. In the modern era, with multiple diagnostic modalities available including high-resolution ultrasonography, magnetic resonance imaging (MRI), CT scan, and nuclear imaging [hepatobiliary iminodiacetic acid (HIDA) scan], rapid diagnosis can be made in many surgically treatable cases. The authors will discuss the imaging modality available with advantages, disadvantages, and common indications of each modality, and overview of obstructive jaundice discussing the wide spectrum of causes in neonates and late childhood. Combining available knowledge with careful and meticulous search can help narrow down the diagnosis and initiate prompt treatment., (© 2022. The Author(s), under exclusive licence to Dr. K C Chaudhuri Foundation.)

Kato and colleagues present prenatal cases with transitional features between congenital biliary dilatation and biliary atresia. They propose that all prenatal biliary cysts originate from choledochal cysts with a narrow segment, but present on a spectrum from congenital biliary dilatation to biliary atresia depending on the severity of reactive biliary sclerosis., (© 2022 Japanese Society of Hepato-Biliary-Pancreatic Surgery.)

Objectives: The aim of this study was to establish an automatic classification model for chronic inflammation of the choledoch wall using deep learning with CT images in patients with pancreaticobiliary maljunction (PBM)., Methods: CT images were obtained from 76 PBM patients, including 61 cases assigned to the training set and 15 cases assigned to the testing set. The region of interest (ROI) containing the choledochal lesion was extracted and segmented using the UNet++ network. The degree of severity of inflammation in the choledochal wall was initially classified using the ResNeSt network. The final classification result was determined per decision rules. Grad-CAM was used to explain the association between the classification basis of the network and clinical diagnosis., Results: Segmentation of the lesion on the common bile duct wall was roughly obtained with the UNet++ segmentation model and the average value of Dice coefficient of the segmentation model in the testing set was 0.839 ± 0.150, which was verified through fivefold cross-validation. Inflammation was initially classified with ResNeSt18, which resulted in accuracy = 0.756, sensitivity = 0.611, specificity = 0.852, precision = 0.733, and area under curve (AUC) = 0.711. The final classification sensitivity was 0.8. Grad-CAM revealed similar distribution of inflammation of the choledochal wall and verified the inflammation classification., Conclusions: By combining the UNet++ network and the ResNeSt network, we achieved automatic classification of chronic inflammation of the choledoch in PBM patients and verified the robustness through cross-validation performed five times. This study provided an important basis for classification of inflammation severity of the choledoch in PBM patients., Advances in Knowledge: We combined the UNet++ network and the ResNeSt network to achieve automatic classification of chronic inflammation of the choledoch in PBM. These results provided an important basis for classification of choledochal inflammation in PBM and for surgical therapy.

Sixty year old female with hypertension and crampy abdominal pain episodes. Admitted to hospital (September-2020) by obstructive jaundice. MRCP: biliary dilation due to Todani Ic (fusiform) choledocal cyst (CC), distal sludge. ERCP: normal mucosa prominent papilla; biliary dilation compatible with CC; choledocholithiasis; 8-mm CHD filling defect. Sphincterotomy, removal of stones/sludge, brush-cytology of the filling defect (pathology: atypias). US: dilation resolution (CBD: 6.5 mm).

Background: Infants prenatally suspected of having a choledochal cyst (CDC) typically undergo ultrasound imaging shortly after birth. This study sought to evaluate features on the initial postnatal ultrasound (IPU) that could identify newborns at risk for early complications., Methods: Following IRB approval, patients from four US fetal centers with prenatal suspicion for CDC and postnatal imaging from 2000 to 2017 were reviewed. Imaging and clinical courses were assessed., Results: Forty-two patients had prenatal ultrasounds suspicious for CDC. Nineteen (45.2%) were excluded due to diagnostic revision ( n  = 9), cyst resolution ( n  = 5), lack of IPU measurements ( n  = 3), or lack of follow-up ( n  = 2). The 23 remaining patients were included in the study. Of these, five (21.7%) developed symptoms at a median age of 16.5 days (IQR 16-19 days), and 18 (78.3%) remained asymptomatic throughout the first year after birth. Five patients (21.7%) had cysts ≥ 4.5 cm on IPU (Symptomatic: n  = 3; Asymptomatic: n  = 2). Eighteen patients (78.3%) had cysts < 4.5 cm on IPU (Symptomatic: n  = 2; Asymptomatic: n  = 16). An IPU cyst size ≥ 4.5 cm was associated with neonatal symptom manifestation ( p  = 0.048), with 88.9% specificity (95% CI 65.3-98.6%) and 60% sensitivity (95% CI 14.7-94.7%)., Conclusions: In newborns with prenatally diagnosed CDC, a cyst size ≥ 4.5 cm on IPU is associated with symptom development during the first month after birth and therefore early cyst excision is recommended.

Background: The present study was aimed to update the classification of hepatobiliary malformations and study the clinicopathological profile of pediatric choledochal cyst (CDC) and pediatric giant choledochal cyst (GCC) patients undergone surgery., Methods: We have retrospectively analysed the data of 57 consecutive cases of CDCs in paediatric patients from a time period spanning from 2016 to 2020., Results: Revised classification of hepatobiliary malformations was proposed and these were divided into 2 broad headings, choledochal (congenital and acquired) and extra-choledochal spectrum. 57 pediatric patients were diagnosed as having CDC with average age 4.615 years and female to male ratio of 3.7:1. We have also reported one of the largest GCC measuring 23 × 10 × 9 cm size. The classical triad was known to be more common and seen in 60% GCCs as opposed to 14.5% in CDCs. Values of serum bilirubin, SGOT, SGPT, PT/INR were elevated in CDC series and normal in GCC patients. 55 patients (96.5%) underwent cyst excision and Roux-en-Y hepaticojejunostomy. Mortality was seen in 7.01% patients (n = 4) following surgery., Conclusion: Simplified and broader classification system for CDCs has been proposed. Clinical studies found that GCC differs considerably from classical CDCs., (Copyright © 2021 International Hepato-Pancreato-Biliary Association Inc. Published by Elsevier Ltd. All rights reserved.)

Cystic biliary atresia (BA) is a rare but an important type of BA. An antenatally detected cystic lesion at the porta hepatis raises the suspicion of cystic BA. It is very important to differentiate this from choledochal cyst in infants with cholestasis and cystic lesions. This case report outlines the clinical presentation and radiological findings of an infant who had an antenatally detected intra-abdominal cystic mass, thought to be a choledochal cyst., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To compare the clinical and radiologic findings between perforated and non-perforated choledochal cysts in children., Materials and Methods: Fourteen patients (mean age ± standard deviation, 1.7 ± 1.2 years) with perforated choledochal cysts (perforated group) and 204 patients (3.6 ± 3.8 years) with non-perforated choledochal cysts (non-perforated group) were included between 2000 and 2019. All patients underwent choledochal cyst excision after ultrasound, CT, or MR cholangiopancreatography. Relevant data including demographics, clinical symptoms, laboratory findings, imaging findings, and outcomes were analyzed. Statistical differences were compared using the Mann-Whitney U test and Fisher's exact test., Results: Choledochal cyst perforation occurred only in children under the age of 4 years. Acute symptoms, including fever ( p < 0.001), were more common in the perforated group than in the non-perforated group. High levels of white blood cells ( p = 0.004), C-reactive protein ( p < 0.001), and serum amylase ( p = 0.002), and low levels of albumin ( p < 0.001) were significantly associated with the perforated group. All 14 patients with perforated choledochal cysts had ascites, whereas only 16% (33/204) of patients in the non-perforated group had ascites ( p < 0.001). In the subgroup of patients who had ascites, a large amount of ascites ( p = 0.001), increase in the amount of ascites in a short time ( p < 0.001), complex ascites ( p < 0.001), and perihepatic pseudocysts ( p < 0.001) were more common in the perforated group than in the non-perforated group., Conclusion: Children with perforated choledochal cysts have characteristic clinical and radiologic findings compared to those with non-perforated choledochal cysts. In young children with choledochal cysts, perforation should be differentiated in cases with acute symptoms, laboratory abnormalities, and characteristic ascites findings., Competing Interests: So-Young Yoo who is on the editorial board of the Korean Journal of Radiology was not involved in the editorial evaluation or decision to publish this article. All remaining authors have declared no conflicts of interest., (Copyright © 2022 The Korean Society of Radiology.)

Aim: The safety and success of laparoscopic choledochal cyst surgery (LapCC) depends upon two critical elements during the hepaticojejunostomy anastomosis (HJA). These may be termed the Difficulty of Differentiation (DOD) and the Difficulty of Suturing (DOS). The type of imaging system (2 or 4 K) used may influence either of these. We compared outcomes of LapCC using 2 or 4 K imaging systems., Methods: LapCC were performed at a single institution by the same team using a 2 K system (2009-2018; n = 26) and a 4 K system (2018-2019; n = 11) were compared. 4 K cases were chosen to match 2 K cases to minimize bias. Five independent senior pediatric surgeons scored DOD and DOS blindly from intraoperative video recordings of LapCC using a subjective 5-point scale (5: impossible, 4: difficult, 3: tedious, 2: slow, and 1: easy) and rated their over all impression as +1 if 4 K was better, 0 if they were the same, and -1 if 4 K was worse. Total HJA anastomosis time (TAT) and TAT/suture were also calculated., Results: LapCC was performed in 37 age/weight/HJA diameter matched children. Scores for DOD (p<0.001) were lower with 4 K with less variance, although there was no difference in DOS (p = 0.08). Operative time (p = 0.03) and duration of hospitalization (p < 0.001) were significantly shorter with 4 K. 4 K was rated +1 unanimously. There was no difference in TAT (p = 0.17) and TAT/suture (p = 0.22). There was one HJA leak with 2 K (3.8%) and no complications with 4 K., Conclusions: Improved resolution with 4 K improved the progress of surgery as reflected by shorter operative time and duration of hospitalization, enhancing the performance of LapCC in children., Level of Evidence: III., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Competing Interests: The authors declare that they have no conflict of interest.

Purpose: It is difficult to distinguish cystic biliary atresia (CBA) from choledochal cyst (CC) before intraoperative cholangiography in neonates or young infants because of the similar ultrasonographic patterns and clinical manifestations. This study is to investigate the difference of clinical parameters between CBA and CC., Methods: 96 patients with cyst at hepatic hilum whose ages were less than 120 days during the period from Jan'2013 to Nov'2015 were retrospectively studied, they were divided into CBA group and CC group by intraoperative cholangiography, there were 29 cases of CBA and 67 cases of CC. It was compared and analyzed on laboratory data, preoperative ultrasonographic features, intraoperative cholangiography and histopathological results between the two groups. Data were quoted as means ± standard deviation (SD) or median (range), Student's t test, non-parametric test and χ2 test were used as appropriate. P < 0.05 was considered as significant statistical difference., Results: CBA group and CC group were comparable for the operative age (51.76 ± 23.99 days vs. 50.03 ± 26.38 days, P = 0.76), weight (4.60 ± 0.75 kg vs. 4.44 ± 1.03 kg, P = 0.46) and sex proportion (M/F: 9/20 vs. 20/47, P = 0.91). there were significant differences (CBA vs. CC) in ALT [59(3-375) vs. 30 (6-247) IU/L, P < 0.001], AST [140(30-694) vs. 44(18-410) IU/L, P < 0.001], T-Bil [190 (107-326) vs.87(5-310) μmol/L, P < 0.001], D-Bil [85 (31-174) vs.14(1-122) μmol/L, P < 0.001] and TBA [112 (23-269) vs.9(1-337) μmol/L, P < 0.001]. There was insignificant statistical difference in GGT [332(112-2154) vs. 226(21-1810) IU/L, P = 0.099] between CBA group and CC group. The cyst size in CBA group was obviously smaller than CC group (the maximum longitudinal diameter: 2.52 ± 1.16 cm vs. 4.71 ± 2.23 cm, P < 0.001; the maximum transverse diameter: 1.64 ± 0.75 cm vs. 3.41 ± 1.79 cm, P < 0.001). Significant differences presented in preoperative ultrasonographic features and histopathological results between the two groups., Conclusion: CBA should be suspected if ultrasonography indicates small cyst with characteristic manifestations of BA and elevated laboratory data. GGT was an insensitive indicator in distinguishing CBA from CC. Cholangiography should be done for the suspected CBA patients as soon as possible to confirm the diagnosis and have surgical intervention timely., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)