A case series of prenatal hepatic hilar cyst in the presence of a gallbladder - navigating the dilemma between biliary atresia and choledochal cyst.

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.

Choledochal Cyst Diagnosed during Pregnancy and Treated by Postpartum Laparoscopic Choledochal Cyst Excision: Case Report.

We report a rare case of choledochal cyst with acute cholangitis that was diagnosed at 37 weeks' gestation and treated by laparoscopic choledochal resection and biliary reconstruction after delivery. A 31-year-old Japanese primigravida at 37 weeks' gestation presented with right upper quadrant pain. The patient was diagnosed as having acute cholangitis due to a type-Ia choledochal cyst, according to the Todani classification, with pancreaticobiliary maljunction. Acute cholangitis improved with conservative treatment, the fetus was delivered by Cesarean section at 38 weeks' gestation, and the patient was treated by laparoscopic choledochal cyst excision and biliary reconstruction at 47 days postpartum. Total operation time was 579 minutes and intraoperative body fluid loss was 100 mL. The patient is now healthy with normal liver function 7 years after the operation. To ensure good outcomes for the mother and fetus, treatment decisions for choledochal cyst diagnosed during pregnancy must be carefully considered.

Successful Management of a Late Referred Giant Choledochal Cyst Type Ia in 8-Year-old Girl.

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.

RésuméUn kyste cholédochal est une condition rare avec une incidence variable et différentes modalités de présentation selon le contexte et l'âge des patients. Le diagnostic précoce et la résection complète sont les seules options thérapeutiques pour prévenir les complications et les tumeurs malignes. Type IA, La dilatation kystique de l'ensemble du canal extrahépatique est la plus observée et une taille géante (plus de 10 cm de diamètre) peut rarement être atteinte. Ce groupe de patients a souvent des antécédents cliniques de longue durée et une expérience partagée d'accès mal à l'aise aux soins spécialisés. D'énormes dimensions sont Un défi complexe pour une résection complète et le risque de dommages fonctionnels du foie permanents doivent être pris en compte, comme dans le cas actuel.

A new classification and laparoscopic treatment of extrahepatic choledochal cyst.

BACKGROUND: Prior typing methods fail to provide predictive insights into surgical complexities for extrahepatic choledochal cyst (ECC). This study aims to establish a new classification system for ECC through clustering of imaging results. Additionally, it seeks to compare the differences among the identified ECC types and assess the levels of surgical difficulty. METHODS: The imaging data of 124 patients were automatically grouped through a K-means clustering analysis. According to the characteristics of the new grouping, corrections and interventions were carried out to establish a new classification. Demographic data, clinical presentations, surgical parameters, complications, reoperation, and prognostic indicators were analyzed according to different types. Factors contributing to prolonged surgical time were also evaluated. RESULTS: A new classification system of ECC: Type A (upper segment), Type B (middle segment), Type C (lower segment), and Type D (entire bile duct). The incidences of comorbidities (calculus or infection) were significantly different (P = 0.000, P = 0.002). Additionally, variations in the incidence of postoperative biliary stricture were statistically significant (P = 0.046). The operative time was significantly different between groups (P = 0.001). Age, BMI > 30, classification, and the presence of combined stones exhibit a significant association with prolonged operative time (P = 0.002, P = 0.000, P = 0.011, P = 0.011). CONCLUSION: In conclusion, our utilization of machine learning-driven cluster analysis has enabled the creation of a novel extrahepatic biliary dilatation typology. This classification, in conjunction with factors like age, combined stone occurrence, and obesity, significantly influences the complexity of laparoscopic choledochal cyst surgery, offering valuable insights for improved surgical treatment.

Robot-assisted resection of choledochal cyst in neonates.

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.

Surgical aspects of choledochal cyst in children and adults: an experience of 106 cases.

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.

Laparoscopic resection of a giant choledochal cyst: A rare case report.

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.

A nomogram for predicting choledochal cyst with perforation.

BACKGROUND: Choledochal cyst with perforation (CC with perforation) rarely occurs, early diagnosis and timely treatment plan are crucial for the treatment of CC with perforation. This study aims to forecast the occurrence of CC with perforation. METHODS: All 1111 patients were conducted, who underwent surgery for choledochal cyst at our hospital from January 2011 to October 2022. We conducted univariate and multivariate logistic regression analysis to screen for independent predictive factors for predicting CC with perforation, upon which established a nomogram. The predictive performance of the nomogram was evaluated using receiver operating characteristic (ROC) curves, calibration plots, and decision curve analysis (DCA) curves. RESULTS: The age of children with choledochal cyst perforation is mainly concentrated between 1 and 3 years old. Logistic regression analysis indicates that age, alanine aminotransferase, glutamyl transpeptidase, C-reactive protein, vomiting, jaundice, abdominal distension, and diarrhea are associated with predicting the occurrence of choledochal cyst perforation. ROC curves, calibration plots, and DCA curve analysis curves demonstrate that the nomogram has great discriminative ability and calibration, as well as significant clinical utility. CONCLUSION: The age of CC with perforation is mainly concentrated between 1 and 3 years old. A nomogram for predicting the perforation of choledochal cyst was established.

Antenatally Detected Choledochal Malformation: What Are We Waiting For?

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 rS = 0.01, P = 0.44; AST rS = 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.

Robotic Biliary Resection for Type I Choledochal Cyst With Dual Hepaticojejunostomy.

The distal bile duct was isolated and transected with a frozen section examination confirming the absence of malignancy. Attention was then shifted to constructing a 60 cm Roux limb by first identifying and transecting the proximal jejunum 40 cm from the ligamentum of Treitz. A side-to-side stapled jejunojejunostomy anastomosis was completed. The Roux limb was transposed toward the porta hepatis through an antecolic approach.

Preliminary exploration of animal models of congenital choledochal cysts.

BACKGROUND: Various animal models have been used to explore the pathogenesis of choledochal cysts (CCs), but with little convincing results. Current surgical techniques can achieve satisfactory outcomes for treatment of CCs. Consequently, recent studies have focused more on clinical issues rather than basic research. Therefore, we need appropriate animal models to further basic research. AIM: To establish an appropriate animal model that may contribute to the investigation of the pathogenesis of CCs. METHODS: Eighty-four specific pathogen-free female Sprague-Dawley rats were randomly allocated to a surgical group, sham surgical group, or control group. A rat model of CC was established by partial ligation of the bile duct. The reliability of the model was confirmed by measurements of serum biochemical indices, morphology of common bile ducts of the rats as well as molecular biology experiments in rat and human tissues. RESULTS: Dilation classified as mild (diameter, ≥ 1 mm to < 3 mm), moderate (≥ 3 mm to < 10 mm), and severe (≥ 10 mm) was observed in 17, 17, and 2 rats in the surgical group, respectively, while no dilation was observed in the control and sham surgical groups. Serum levels of alanine aminotransferase, aspartate aminotransferase, total bilirubin, direct bilirubin, and total bile acids were significantly elevated in the surgical group as compared to the control group 7 d after surgery, while direct bilirubin, total bilirubin, and gamma-glutamyltransferase were further increased 14 d after surgery. Most of the biochemical indices gradually decreased to normal ranges 28 d after surgery. The protein expression trend of signal transducer and activator of transcription 3 in rat model was consistent with the human CC tissues. CONCLUSION: The model of partial ligation of the bile duct of juvenile rats could morphologically simulate the cystic or fusiform CC, which may contribute to investigating the pathogenesis of CC.

Optimized reusable modular 3D-printed models of choledochal cyst to simulate laparoscopic and robotic bilioenteric anastomosis.

Laparoscopic and robotic surgery is a challenge to the surgeon's hand-eye coordination ability, which requires constant practice. Traditional mentor training is gradually shifting to simulation training based on various models. Laparoscopic and robotic bilioenteric anastomosis is an important and difficult operation in hepatobiliary surgery. We constructed and optimized the reusable modular 3D-printed models of choledochal cyst. The aim of this study was to verify the ability of this optimized model to distinguish between surgeons with different levels of proficiency and the benefits of repeated practice. A total of 12 surgeons with different levels participated in the study. Operation completion time and OSATS score were recorded. The model was validated by Likert scale. Surgeons were shown the steps and contents before performing laparoscopic or robotic bilioenteric anastomosis using the model. Surgeons with different levels of experience showed different levels when performing laparoscopic bilioenteric anastomosis on this model. Repeated training can significantly shorten the time of laparoscopic bilioenteric anastomosis and improve the operation scores of surgeons with different levels of experience. At the same time, preliminary results have shown that the performance of surgeons on the domestic robotic platform was basically consistent with their laparoscopic skills. This model may distinguish surgeons with different levels of experience and may improve surgical skills through repeated practice. It is worth noting that in order to draw more reliable conclusions, more subjects should be collected and more experiments should be done in the future.

The timing of minimally invasive surgery for prenatally diagnosed choledochal cysts.

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.

Clinical implications of pediatric biliary intraepithelial neoplasia diagnosed from a choledochal cyst specimen.

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

Cholangiocarcinoma identified in perforated choledochal cyst in a 3-year-old boy.

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.

[Choledochal cysts: surgical treatment in newborns and infants]. / Kisty kholedokha: osobennosti khirurgicheskogo lecheniya u detei pervykh trekh mesyatsev zhizni.

OBJECTIVE: To improve postoperative outcomes in newborns and infants with choledochal cysts and to determine the indications for surgery. MATERIAL AND METHODS: There were 13 children aged 0-3 months with choledochal cyst who underwent reconstructive surgery between 2019 and 2023. In all children, choledochal cyst was associated with cholestasis. Acholic stool was observed in almost half of the group (n=7). All children underwent cyst resection and Roux-en-Y hepaticoenterostomy. RESULTS: Symptoms of cholestasis regressed in all patients. Mean surgery time was 128±27 min. There were no complications. Enteral feeding was started after 1-2 postoperative days, abdominal drainage was removed after 6.2±1.6 days. Mean length of hospital-stay was 16±3.7 days. Adequate bile outflow is one of the main principles. For this purpose, anastomosis with intact tissues of hepatic duct should be as wide as possible. Roux-en-Y loop should be at least 40-60 cm to prevent postoperative cholangitis. CONCLUSION: Drug-resistant cholestasis syndrome and complicated choledochal cysts (cyst rupture, bile peritonitis) are indications for surgical treatment in newborns and infants. When forming Roux-en-Y hepaticoenterostomy, surgeon should totally excise abnormal tissues of the biliary tract to prevent delayed malignant transformation.

[Laparoscopic excision versus open excision for the treatment of choledochal cysts: a systematic review and meta-analysis]. / Laparoskopicheskoe i otkrytoe khirurgicheskoe lechenie kist kholedokha: sistematicheskii obzor i metaanaliz.

OBJECTIVE: To determine if laparoscopic excision is more effective than open excision in the treatment of choledochal cysts. MATERIAL AND METHODS: A systematic review of randomized clinical trials in 3 databases measuring the efficacy of laparoscopic and open excision of choledochal cysts was performed. The authors considered international and national reports, whose results were analyzed in detail. RESULTS: Mean duration of laparoscopic excision was 51 min, open excision - 35.4 min. Length of hospital-stay after laparoscopic excision ranged between 5 and 74 days, after open excision - between 7 and 146 days. Bile leakage rate was 1-2% and 4%, respectively. Laparoscopic excision was followed by lower complication rate. Morbidity and mortality in laparoscopic excision was 20% and 0%, in open excision - 60% and 3.3%, respectively. CONCLUSION: Laparoscopic excision is more effective than open excision in the treatment of choledochal cysts.

Contemporary trends in choledochal cyst excision: An analysis of the pediatric national surgical quality improvement program.

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

Characteristics and malignancy rates of adult patients diagnosed with choledochal cyst in the West: a systematic review.

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.