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

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.)

Biliary abnormalities in children are uncommon, and the spectrum of biliary disorders is broader than in adult patients. Unlike in adults, biliary disorders in children are rarely neoplastic and are more commonly rhabdomyosarcoma rather than cholangiocarcinoma. Pediatric biliary disorders may be embryologic or congenital, such as anatomic gallbladder anomalies, anomalous pancreaticobiliary tracts, various cholestatic processes, congenital cystic lesions, or genetic conditions. They may also be benign, such as biliary filling anomalies, biliary motility disorders, and biliary inflammatory and infectious disorders. Distinguishing these entities with a single imaging modality is challenging. US is the primary imaging modality for initial evaluation of biliary abnormalities in children, due to its wide availability, lack of ionizing radiation, and low cost and because it requires no sedation. Other examinations such as MRI, CT, and nuclear medicine examinations may provide anatomic and functional information to narrow the diagnosis further. Hepatobiliary-specific contrast material with MRI can provide better assessment of biliary anatomy on delayed images than can traditional MRI contrast material. MR cholangiopancreatography (MRCP) allows visualization of the intra- and extrahepatic biliary ducts, which may not be possible with endoscopic retrograde cholangiopancreatography (ERCP). Suspected biliary atresia requires multiple modalities for diagnosis and timely treatment. Determining the type of choledochal cyst calls for a combination of initial US and MRCP. Many benign and malignant biliary masses require biopsy for definitive diagnosis. Knowledge of the imaging appearances of different pediatric biliary abnormalities is necessary for appropriate imaging workup, providing a diagnosis or differential diagnosis, and guiding appropriate management. © RSNA, 2024 Test Your Knowledge questions for this article are available in the supplemental material.

Congenital biliary dilatation (CBD) is a congenital malformation of focal dilatation of the extrahepatic bile ducts, including the common bile duct, and is often associated with pancreaticobiliary maljunction (PBM). In this article, we report a CBD case that presented with focal dilation of the common bile duct without PBM (Todani's classification type Ib). The patient was a 32-year-old man who visited a doctor with a chief complaint of abdominal distension. Computed tomography revealed cystic dilatation of the common bile duct, and the patient was referred to our institution. Magnetic resonance cholangiopancreatography showed cystic dilatation of the common bile duct with a maximum diameter of 7 cm; however, evaluating the presence of PBM was challenging. Endoscopic ultrasonography showed small gallstones and debris in the dilated common bile duct and no thickening of the gallbladder wall. Endoscopic retrograde cholangiopancreatography revealed no PBM or markedly elevated bile amylase levels. Based on these findings, the patient was diagnosed with Todani Type Ib CBD. Since this patient did not have pancreatobiliary reflux, it was unclear whether the risk of developing biliary tract cancer was high, and since the treatment was highly invasive, the decision was to follow up without surgical treatment., (© 2023. Japanese Society of Gastroenterology.)

Signal transducer and activator of transcription 3 (STAT3) have been highlighted in cancer regulation. Its roles in Cholangiocarcinoma (CCA) arising from the choledochal cyst (CC) were unclear. Here, we attempted to elucidate the roles of STAT3 in CCA-CC and explore its mechanism. A total of 20 patients with CCA arising from CC, that underwent CC excision in the infant stage were included. The expressions of STAT3, miR200c and c-Myb in clinical samples were assessed by RT-qPCR and/or western blot. Their expression correlations in tumor tissues were evaluated by Pearson correlation analysis. Their roles in CCA cell migration and invasion were investigated by gene silence using siRNA or miRNA inhibitor mediated approach and MEK activator. The expression levels of EMT, metastasis and MEK/ERK pathway-related proteins were checked by western blot. The high expressions of STAT3 and c-Myb, and low expression of miR200c were detected in CCA samples. We defined the transcription inhibition of STAT3 in miR200c expression and the negative correlation between miR200c and c-Myb expression. Silence of STAT3 increased miR200c expression and retarded the migration and invasion of CCA cells, accompanied by decreased levels of Vimentin, N-cadherin, MMP2 and MMP9, and elevated expression of E-cadherin, resulting in inactivating MEK/ERK pathway. MiR200c inhibitor reversed the changes induced by STAT3 silence, which was restored by si-c-Myb. MEK activator significantly reversed the inactivation of the MEK/ERK pathway induced by si-STAT3+miR200c inhibitor+si-c-Myb. In summary, the silence of STAT3 suppressed metastasis and progression of CCA cells by regulating miR200c through the c-Myb mediated MEK/ERK pathway, suggesting STAT3 is the effective target for CCA arising from CC.

Purpose: Complicated choledochal cysts (CDC) have a variable presentation, and their management differs from an uncomplicated CDC. They are infrequently reported. We present our 15 years of experience in the management of complicated CDC., Methodology: We reviewed the data of patients with CDCs managed at a tertiary level center from 2005 to 2020 from a prospectively maintained database., Results: Of 215 patients with CDC, 123 patients presented with complicated CDC. The median age of complicated CDC was 31 years with a female preponderance (62.6%). The most common type of CDC associated with complications was type I (69.1%), followed by type IVA (29.3%). The Complicated CDC was presented as cholangitis with or without cystolithiasis (n = 45), cystolithiasis and hepatolithiasis(n = 44), malignancy(n = 10), complications associated with incomplete cyst excision (n = 10), acute pancreatitis (n = 8), chronic pancreatitis(n = 8), portal hypertension (n = 6), spontaneous rupture (n = 4), gastric outlet obstruction (n = 1). These patients were managed as a one-stage approach (52.03%) and a two-stage approach (47.96%). On univariate and multivariate analysis, increasing age, prolonged duration of symptoms, and presence of abnormal pancreaticobiliary ductal junction (APBDJ) were significantly associated with complicated CDC., Conclusion: The management of complicated CDC varied depending on the associated pathology, many of them required a staged approach. Increasing age, prolonged duration of symptoms, and presence of APBDJ were significantly associated with complicated CDC., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

The literature on liver cysts is highly conflicting, mostly owing to definitional variations. Two hundred and fifty-eight ≥1 cm cysts evaluated pathologically using updated criteria were classifiable as: I. Ductal plate malformation related (63%); that is, cystic bile duct hamartoma or not otherwise specified-type benign biliary cyst (35 with polycystic liver disease). These were female predominant (F/M=2.4), large (10 cm), often multifocal with degenerative/inflammatory changes and frequently misclassified as "hepatobiliary cystadenoma." II. Neoplastic (13%); 27 (10.5%) had ovarian-type stroma (OTS) and qualified as mucinous cystic neoplasm (MCN) per World Health Organization (WHO). These were female, solitary, mean age 52, mean size 11 cm, and 2 were associated with carcinoma (1 in situ and 1 microinvasive). There were 3 intraductal papillary neoplasms, 1 intraductal oncocytic papillary neoplasm, 1 cystic cholangiocarcinoma, and 2 cystic metastasis. III. Infectious/inflammatory (12%). These included 23 hydatid cysts (including 2 Echinococcus alveolaris both misdiagnosed preoperatively as cancer), nonspecific inflammatory cysts (abscesses, inflammatory cysts: 3.4%). IV. Congenital (7%). Mostly small (<3 cm); choledochal cyst (5%), foregut cyst (2%). V. Miscellaneous (4%). In conclusion, hepatic cysts occur predominantly in women (3/1), are mostly (90%) non-neoplastic, and seldom (<2%) malignant. Cystic bile duct hamartomas and their relative not otherwise specified-type benign biliary cysts are frequently multifocal and often misdiagnosed as "cystadenoma/carcinoma." Defined by OTS, MCNs (the true "hepatobiliary cystadenoma/carcinoma") are solitary, constitute only 10.5% of hepatic cysts, and have a significantly different profile than the impression in the literature in that essentially all are perimenopausal females, and rarely associated with carcinoma (7%). Since MCNs can only be diagnosed by demonstration of OTS through complete microscopic examination, it is advisable to avoid the term "cystadenoma/cystadenocarcinoma" solely based on radiologic examination, and the following simplified terminology would be preferable in preoperative evaluation to avoid conflicts with the final pathologic diagnosis: (1) noncomplex (favor benign), (2) complex (in 3 subsets, as favor benign, cannot rule out malignancy, or favor malignancy), (3) malignant features., Competing Interests: Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

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.

Backgrounds and Aims: CD56 immunostain is used as an adjunct to aid in the preoperative diagnosis of biliary atresia (BA) by liver biopsy. We aimed to study the expression of CD56 in different pediatric cholestatic diseases thereby evaluating its utility in the diagnosis of BA., Methods: We performed immunohistochemistry for CD56 on 35 cases of pediatric cholestatic diseases and five age-matched controls. CD56 expression was assessed by a multiplication score (percentage positivity x intensity) in the biliary epithelium., Results: The multiplication score between BA and choledochal cyst was not significantly different. High scores were also encountered in other cholestatic disorders. The score showed a significant negative association with serum albumin and a significant positive correlation with the serum ALT level. Very significant positive correlation between the score and portal fibrosis was obtained., Conclusion: CD56 expression is an infidel marker for the histological diagnosis of BA and rather provides a clue to the disease status in pediatric cholestatic diseases.

Choledochal cyst (CC) is a cystic disease predominantly involving the extrahepatic biliary tree. Biliary atresia (BA), another disorder of the extrahepatic biliary tree, is sometimes considered to be in the same spectrum as pediatric CC. Recently, the absence and/or the structural abnormality of the primary cilia in the intrahepatic cholangiocytes have been implicated in the pathogenesis of BA. We aimed to evaluate the expression of primary ciliary proteins in the intrahepatic cholangiocytes in cases of pediatric CC and compare it with normal control and BA. We performed immunohistochemistry for primary ciliary proteins (acetylated-α-tubulin and double-cortin domain containing 2) on the liver biopsies of control liver (n=5), pediatric CC (n=13), and BA (n=14). We also compared the expression with various clinical, biochemical, histopathologic (portal fibroinflammation and ductal plate malformation), and immunohistochemical (proliferative index) data. There was significant loss of primary cilia from the intrahepatic cholangiocytes in cases of CC and BA as compared with the normal control by both immunostains (CC: P=0.003 and 0.001, respectively; BA: P=0.001 and 0.001, respectively). There was no significant difference between the CC and BA in terms of ciliary protein loss. The loss of the ciliary proteins occurred irrespective of the proliferative (MIB-1 labeling) index, portal fibroinflammation, or ductal plate malformation. The loss of cilia did not correlate with the clinical follow-up in cases of pediatric CC. The loss of primary cilia from the intrahepatic cholangiocytes may be crucial in the etiopathogenesis of pediatric CC., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Choledochal cyst (CC) is believed to be a mostly Asian disorder. As a clinically defined entity, its pathologic correlates are poorly characterized. Eighty-four resected CCs from the West were reanalyzed. After applying established Japanese criteria, 9/66 with available imaging were disqualified and 10/39 with preoperative cyst typing had to be recategorized. None had been diagnosed with, or evaluated for, pancreatobiliary maljunction, but on retrospective analysis of radiologic images, 12/66 were found to have pancreatobiliary maljunction. The clinical findings were: F/M=5.7; mean age, 48; most (77%) presented with abdominal pain; mean size, 2.9 cm; choledocholithiasis 11%. Gross/histologic examination revealed 3 distinct pathology-based categories: (I) Cystic dilatation of native ducts (81%). (II) Double bile duct (13%), almost all of which were found in women (10/11); all were diagnosed by pathologic examination, and not preoperative diagnosis. (III) Gastrointestinal (GI) duplication type (6%). Microscopic findings of the entire cohort included mucosal-predominant lymphoplasmacytic inflammation (50%), follicular cholangitis (7%), mucosal hyperplasia (43%; 13% with papillae), intestinal metaplasia (10%), BilIN-like hyperplasia (17%), erosion/ulceration (13%), and severe dysplasia-mimicking atypia including "detachment atypia" and micropapillary degeneration (11%). Carcinomatous changes were seen in 14 cases (17%) (high-grade dysplasia/carcinoma in situ in 7, intraductal papillary neoplasm 1, and invasive carcinoma 6); and 13/14 of these occurred in pathologic category I, all with cyst size >1 cm. In conclusion, diagnostic imaging guidelines used in Asia are not routinely used (but should be adopted) in the West. Pathologically, cases designated as CC are classifiable in 3 groups: category 1 (dilated native duct type), more prone to carcinomatous change; category 2, double-duct phenomenon (all but 1 being female in this study); and category 3, GI-type duplication. Overall, 17% of CCs show carcinomatous change (50% of them invasive). CC specimens should be carefully examined with this classification and submitted entirely for assessment of at-risk mucosa and cancerous transformation., Competing Interests: Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Introduction:  In patients with choledochal cysts (CDC), a hyperplasia-dysplasia-carcinoma sequence can lead to biliary tract malignancy. The limited data available suggest that the risk decreases considerably after excision in childhood. We analyzed samples of resected CDC from pediatric patients histologically and performed mutational analysis of the proto-oncogenes KRAS and BRAF as early markers of malignant alteration in cholangiocytes., Materials and Methods:  After institutional review board approval, patients undergoing resection for CDC in our center from 2011 to 2019 were retrospectively identified. Histopathological reports were searched for inflammation and endothelial alteration. Cases with sufficient tissue specimen were tested for KRAS codon 12/13 and BRAF codon 600 mutations by pyrosequencing., Results:  In total, 42 patients underwent resection for choledochal cyst in the study period. Median age at surgery was 2.4 years (range = 18 days-18 years). Histopathological analysis showed no malignancy, but various degrees of inflammation or fibrosis in approximately 50% of the patients and in all age groups. Sufficient tissue for mutation analysis was available for 22 cases, all of which tested negative for KRAS or BRAF mutation., Conclusion:  In our series, chronic inflammatory changes were frequently present in CDC of infants and children. However, the lack of KRAS and BRAF mutations suggests that no malignant changes have been initiated in this group of European patients undergoing early resection., Competing Interests: None declared., (Thieme. All rights reserved.)

Rationale: Biliary cysts (BC) are rare dilatations of various parts of a biliary tract. They account for approximately 1% of all benign biliary diseases. Total cyst excision and Roux-Y hepaticojejunostomy is the treatment method of choice in most patients. In this paper, a novel surgical treatment with the use of internal biliary and pancreatic catheters was presented., Patient Concerns: A 21-years-old woman with a giant choledochal cyst of Todani IA type presenting with abdominal pain, nausea, fever and palpable abdominal mass. It had been previously drained as a misdiagnosed pancreatic cyst in another hospital., Diagnosis: A very high amylase level (107140,0 U/l) in drain fluid was noted in laboratory tests. Endoscopic retrograde cholangiopancreatography revealed a biliary cyst located in the distal common bile duct and a pancreaticobiliary anomaly was suggested. A cholangiography per catheter inserted to the biliary cyst showed a large round contrast-filled cyst. A cholangiography following cyst decompression revealed a very long, tortuous bile duct entering the duodenum., Interventions: Cholecystectomy, cyst resection, Roux-Y hepaticojejunostomy, and implantation of catheters into pancreatic and bile duct were performed. The postoperative course was uneventful and she was discharged on 12th day without any complications. Histopathology revealed a cyst wall partially lined with biliary-type and mucinous epithelium, with dysplasia ranging from low to high grade (biliary intraepithelial neoplasia, high grade), without invasion., Outcomes: The biliary and pancreatic catheters were removed during endoscopic retrograde cholangiopancreatography 8 weeks following surgery without any complications. Fourteen months later, the patient reported good health., Lessons: Diagnosis of the abdominal cyst should be very precise in order to avoid misdiagnosis and inadequate management. The early diagnosis and proper treatment of BC are needed in order to avoid serious complications. The cholangiocarcinoma is the most dangerous potential complication of BC due to dysplasia within the cyst wall as in our young female patient., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Objectives: Histopathologic characteristics of choledochal cysts and their clinical implications have not been previously comprehensively studied., Methods: Smooth muscle distribution patterns and other histologic findings (inflammation, metaplasia, dysplasia, and heterotopia) in 233 surgically resected choledochal cysts were evaluated., Results: Mean patient age was 23.3 ± 19.8 years, with male:female ratio of 0.3. Most cases were Todani type I (175 cases, 75.1%) or IVa (56 cases, 24.1%). Choledochal cysts with thin scattered/no muscle fiber (175 cases, 75.1%) were the predominant pattern and were associated with more frequent postoperative biliary stricture (P = .031), less frequent pyloric metaplasia (P = .016), and mucosal smooth muscle aggregates (P < .001) compared to cysts with thick muscle bundles. Severe chronic cholangitis (P = .049), pyloric metaplasia (P = .019), mucosal smooth muscle aggregates (P < .001), biliary intraepithelial neoplasia (P = .021), and associated bile duct (P = .021) and gallbladder carcinomas (P = .03) were more common in adults (age >20 years vs ≤20 years), suggesting that chronic irritation in association with developmental anomalies involves tumorigenesis from choledochal cysts., Conclusion: Smooth muscle distribution pattern of choledochal cyst may predict postoperative complication, raising clinical implications of smooth muscle patterns in postoperative management of choledochal cysts., (© American Society for Clinical Pathology, 2020. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background: Choledochal cysts are congenital dilations of the biliary tree. Complete cyst excision and biliary-enteric reconstruction have been the standard operations. In our center, more than 95% of choledochal cyst excision is now performed laparoscopically. Majority of current studies describe laparoscopic-assisted reconstruction using Roux-en-Y hepaticojejunostomy (HJ). However, only a few have studied laparoscopic hepaticoduodenostomy (HD) as an alternative method of biliary-enteric reconstruction. In this study, we focused on comparing longer-term outcomes between laparoscopic HJ and HD reconstruction following choledochal cyst excision., Methods: We performed retrospective analysis of 54 children who had undergone laparoscopic choledochal cyst excision and biliary-enteric reconstruction between October 2004 and April 2018. Short-term outcomes including operative time, complications such as anastomotic leakage and bleeding, and hospital stays were included. Long-term outcomes including contrast reflux into biliary tree, cholangitis, anastomotic strictures, and need of reoperation were analyzed., Results: Of the 54 patients, 21 of them underwent laparoscopic HD and 33 underwent laparoscopic Roux-en-Y HJ anastomosis reconstruction. There were no significant differences in gestation, gender, age at operation, antenatal diagnosis, and Todani type of choledochal cyst between HD and HJ group. Operative time was significantly shortened in HD group (p = 0.001). Median time to enteral feeding was 3 days in both groups. Median intensive care unit (p = 0.001) and hospital stay (p = 0.019) were significantly shorter in HD group. There was no perioperative mortality. There was no significant difference in anastomotic leakage requiring reoperation (p = 0.743). There were no significant differences in long-term outcomes including anastomotic stricture (p = 0.097), cholangitis (p = 0.061), symptoms of recurrent abdominal pain or gastritis (p = 0.071), or need of reoperation (p = 0.326). All patients had normal postoperative serum bilirubin level., Conclusions: Laparoscopic excision of choledochal cyst with HD reconstruction is safe and feasible with better short-term outcomes and comparable long-term outcomes compared to Roux-en-Y HJ reconstruction.

Few reports describe oxysterols in healthy children or in children with liver disease. We aimed to determine whether developmental changes in urinary and serum oxysterols occur during childhood, and to assess whether oxysterols might be biomarkers for pediatric liver disease. Healthy children enrolled as subjects (36 and 35 for urine and serum analysis, respectively) included neonates, infants, preschoolers, and school-age children, studied along with 14 healthy adults and 8 children with liver disease. We quantitated 7 oxysterols including 4β-, 20(S)-, 22(S)-, 22(R)-, 24(S)-, 25-, and 27-hydroxycholesterol using liquid chromatography/electrospray ionization-tandem mass spectrometry. Urinary total oxysterols were significantly greater in neonates than in infants (P < 0.05), preschoolers (P < 0.001), school-age children (P < 0.001), or adults (P < 0.001), declining with age. Serum total oxysterols in neonates were significantly lower than in infants (P < 0.05), preschoolers (P < 0.001), school-age children (P < 0.05), or adults (P < 0.01). Compared with healthy children, total oxysterols and 24(S)-hydroxycholesterol in liver disease were significantly increased in both urine (P < 0.001 and P < 0.001, respectively) and serum (P < 0.001 and P < 0.05, respectively). Oxysterols in liver disease, particularly 24(S)-hydroxycholesterol, were greater in urine than serum. Oxysterols change developmentally and might serve as a biomarker for pediatric liver disease. To our knowledge, this is the first such report.

Background: The presence of cartilage in extra hepatic biliary tree is an unusual finding. An isolated presence of the cartilage is possibly heterotopic or occurs as a metaplastic response to the inflammatory insult. Material and methods: We had examined the liver biopsy and the resected specimen of a biliary atresia (BA) after Kasai procedure. Results: There was hyaline cartilage around the common hepatic and common bile duct in a 3-months-old male infant with distal obstructive cholangiopathy on liver biopsy and had positive serum IgM for cytomegalovirus (CMV). Similar findings could not be documented in the pericholedochal tissue of any of the 25 other pediatric cases operated for BA or choledochal cyst and three neonatal autopsies performed for liver-related deaths. Conclusion: Peri-bile duct cartilage is a unique finding and could represent an unusual form of heterotopia or connective tissue metaplasia.

An isolated dilatation of the cystic duct (type VI choledochal cyst (CDC)) is extremely rare with only 21 cases reported in the world literature until now. There is only one case of in situ gall bladder cancer (GBC) reported in association with type VI CDC in the literature. Here we are reporting a case of type VI CDC with papillary GBC., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: Both cystic biliary atresia and choledochal cyst present as infantile obstructive cholangiopathy. Methods : We detail an infant with congenital biliary dilatation and obstructive cholangiopathy where clinicoradiological features (antenatally imaged subhepatic cyst, early onset jaundice, intrahepatic dilated biliary radicals) suggested a choledochal cyst but operative findings (lack of a distal communication of cyst with duodenum) and histomorphological features (cicatricial collagen and myofibroblastic hyperplasia in the cyst wall; ductal plate malformation, ductular cholestasis and strong expression of CD56 in the liver) were those associated with biliary atresia. Conclusion : The observations support the contention that BA and CC may be interim entities in a continuum of manifestation of the same pathology.

Choledochal cysts (CCs) are rare bile duct dilatations, intra-and/or extrahepatic, and have higher prevalence in the Asian population compared to Western populations. Most of the current literature on CC disease originates from Asia where these entities are most prevalent. They are thought to arise from an anomalous pancreaticobiliary junction, which are congenital anomalies between pancreatic and bile ducts. Some similarities in presentation between Eastern and Western patients exist such as female predominance, however, contemporary studies suggest that Asian patients may be more symptomatic on presentation. Even though CC disease presents with an increased malignant risk reported to be more than 10% after the second decade of life in Asian patients, this risk may be overstated in Western populations. Despite this difference in cancer risk, management guidelines for all patients with CC are based predominantly on observations reported from Asia where it is recommended that all CCs should be excised out of concern for the presence or development of biliary tract cancer., Competing Interests: Conflict-of-interest statement: There is no conflict of interest associated with any of the senior author or other coauthors who contributed their efforts in this manuscript.

Background: Complete cyst excision with Roux-en-Y hepaticojejunostomy is the standard procedure for choledochal cysts (CCs). In recent years, neonates have been increasingly diagnosed with CCs prenatally. Earlier treatment has been recommended to avoid complications. For type IVa malformation without extensive intrahepatic bile duct dilatation, laparoscopic hepaticojejunostomy is technically challenging, and anastomotic stricture is a concern. Therefore, we propose laparoscopic synthetical techniques-laparoscopic excision of cyst and ductoplasty plus widened portoenterostomy to avoid stricture in CCs with a narrow hilar duct., Methods: An anastomosis was created around the transected end of the common bile duct in 12 minipigs (Group A), and another 12 minipigs (Group B) received conventional cholangiojejunostomy. Anastomotic diameter measurements and cholangiography were conducted at different times. Histological findings of inflammation and scarring were compared. The expression levels of TGF-β1 and type I collagen were detected by real-time quantitative PCR. Between January 2012 and January 2016, laparoscopic excision of cyst and ductoplasty plus widened portoenterostomy were performed on 29 children with confirmed CCs with a narrow portal bile duct who were followed up for 12-48 months., Results: Group A survived well without obstruction. Slight inflammation and fibrotic tissue were confined to the bile duct periphery. In Group B, five pigs developed stricture. Severe inflammation and diffuse fibrosis affected the whole layer of the anastomosis. Fibrotic biomarkers were significantly higher postoperatively in Group B. Clinically, 29 patients exhibited satisfactory outcomes. No anastomotic stricture has been observed to date., Conclusions: Laparoscopic synthetical techniques may be a superior option to prevent anastomotic stricture in treating CCs with a narrow portal bile duct.

Background: Undifferentiated carcinoma with osteoclast-like giant cells (UC-OGC) in distal common bile duct (CBD) is a rare entity., Case Report: This case report describes a 45-year-old male with a history of a choledochal cyst status post partial excision and cholecystectomy who presented with a mass in the remaining distal/intrapancreatic common bile duct. It was initially mistaken for post-surgery hematoma; however, the rapid growth raised concern for malignancy, and prompted a pancreaticoduodenectomy (Whipple) procedure. Macroscopic examination revealed a 5.5 cm polypoid mass grossly confined in the lumen of the distal CBD. Histology was consistent with UC-OGC, with minimal invasion into the polyp stalk and adjacent CBD wall. Immunohistochemistry demonstrated co-expression of CK7 and p40, normal/wild-type p53, and retained SMAD4 expression in tumor cells. Next-generation sequencing detected mutations at p.Q61H (c.183A>C) of KRAS and p.E545K (c.1633G>A) of PIK3CA, keeping in line with similarity to conventional cholangiocarcinoma. The patient remained disease-free after two years of follow-up without chemotherapy., Conclusion: To our knowledge, this is the first case report of UC-OGC presented as a polypoid mass in the distal CBD. It highlights the complex dynamism and controversial pathogenesis of this unique entity, which should be made aware to avoid diagnostic pitfalls., (Copyright© 2019, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Background: The aim of the present study was to investigate the characteristics, management, and outcomes of congenital biliary dilatation (CBD) in neonates and infants (<1 year old) in a single institution over the past 20 years., Methods: From 1997 to 2016, 21 patients <1 year old underwent definitive surgery for CBD. Open surgery (OS) was performed between 1997 and 2008, and laparoscopic surgery (LS) has been performed since 2009., Results: The bile duct showed cystic dilatation in all patients. Sixteen (76.2%) of the 21 patients were diagnosed prenatally, and the incidence increased with time (OS 63.6%, LS 90%). Fourteen patients (66.7%) were symptomatic before surgery, with jaundice in 11 (52.4%), acholic stool in seven (33.3%), and vomiting in three (14.3%). There were no significant differences in operation time and blood loss, but the postoperative fasting period and hospital stay were significantly shorter in the LS group (P < 0.05). There were no intraoperative complications, but there was one postoperative early complication in one LS group patient, who had bile leakage and was treated with redo hepaticojejunostomy., Conclusion: The incidences of prenatally diagnosed and asymptomatic patients increased with time. Although longer follow-up is needed, LS for CBD could be safely performed even in neonates and early infants., (© 2018 Japanese Society of Hepato-Biliary-Pancreatic Surgery.)

Background: Multiple gallbladders (MG) are a rare malformation, with no clear data on its clinical impact, therapeutic indications or risk for malignancy., Methods: A systematic review of all published literature between 1990 and 2017 was performed using the PRISMA guidelines., Results: Data of 181 patients extracted from 153 studies were reviewed. MG were diagnosed during the treatment of a gallstone-related disease in 83% of patients, of which 13% had previous cholecystectomy and had a recurrence of biliary stone disease. The sensitivity of ultrasound scan was 66%, and that of magnetic resonance imaging cholangio-pancreatography, 97%. The cystic duct was common to both gallbladders (type1) in 43% and separated (type 2) in 50% of patients. In the latter case, there was no way to differentiate preoperatively an accessory gallbladder from a Todani II bile duct cyst. Cholecystectomy was performed in 129 patients by laparotomy (43%) or laparoscopy (56%). MG was undiagnosed before surgery in 24% of the patients. The postoperative biliary leakage rate was 0.7%. In two patients, gallbladder cancers were detected., Conclusion: MG are difficult to diagnose and share a common natural history with single gallbladders, without evidence of increased risk for malignancy. Excision of both gallbladders is indicated in symptomatic stone disease. However, prophylactic cholecystectomy must be considered for type 2 MG, since it cannot be preoperatively differentiated from a Todani II bile duct cyst, which is associated with a risk of malignant transformation., (Copyright © 2018 International Hepato-Pancreato-Biliary Association Inc. Published by Elsevier Ltd. All rights reserved.)

Aim: To analyze the expression and function of the Notch signaling target gene Hes1 in a rhesus rotavirus-induced mouse biliary atresia model., Methods: The morphologies of biliary epithelial cells in biliary atresia patients and in a mouse model were examined by immunohistochemical staining. Then, the differential expression of Notch signaling pathway-related molecules was investigated. Further, the effects of the siRNA-mediated inhibition of Hes1 expression were examined using a biliary epithelial cell 3D culture system., Results: Both immature (EpCAM + ) and mature (CK19 + ) biliary epithelial cells were detected in the livers of biliary atresia patients without a ductile structure and in the mouse model with a distorted bile duct structure. The hepatic expression of transcripts for most Notch signaling molecules were significantly reduced on day 7 but recovered to normal levels by day 14, except for the target molecule Hes1, which still exhibited lower mRNA and protein levels. Expression of the Hes1 transcriptional co-regulator, RBP-Jκ was also reduced. A 3D gel culture system promoted the maturation of immature biliary epithelial cells, with increased expression of CK19 + cells and the formation of a duct-like structure. The administration of Hes1 siRNA blocked this process. As a result, the cells remained in an immature state, and no duct-like structure was observed., Conclusion: Our data indicated that Hes1 might contribute to the maturation and the cellular structure organization of biliary epithelial cells, which provides new insight into understanding the pathology of biliary atresia., Competing Interests: Conflict-of-interest statement: The authors declare that there is no conflict of interest related to this study.

Biliary cystic disease is a rare entity. Twenty-five per cent of cases are diagnosed during adulthood and only a few reports have described this condition during pregnancy, where it represents a therapeutic challenge for both obstetricians and surgeons with regard to the risks it entails for the patient and the fetus.Definitive management is surgical resection, as cysts may progress to malignancy if untreated. During pregnancy, resection is generally deferred to after delivery, especially in the context of suspected cholangitis.A 19-year-old young woman with no previous prenatal control, presented to the emergency department on her 32nd week of gestation with abdominal pain and jaundice. A giant Todani I biliary cyst was observed on imaging along with dilation of the proximal biliary tree suggesting acute cholangitis. Fetal compromise prompted immediate delivery after which percutaneous biliary drainage was performed. Following recovery, the cyst was surgically resected., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Background: The option of either single- or two-staged cyst excision has been proposed for perforated choledochal cysts (CCs), but which of the two methods is more effective remains controversial. We examined the complications and short-term outcomes of single-stage excision of perforated and non-perforated CCs., Methods: The medical records of patients treated for CCs from 2003 to 2016 were retrospectively reviewed. Outcomes were compared between patients with perforated CCs (Group A) and non-perforated CCs (Group B). The operative time, intraoperative bleeding, length of stay, and postoperative complications were analyzed., Results: Group A comprised 6 patients (2 males, 4 females; mean age, 29months), and Group B comprised 26 patients (2 males, 24 females; mean age, 41months). All patients underwent single-stage complete excision with Roux-en-Y hepaticojejunostomy. There were no significant differences in the operative time, bleeding, and/or length of stay. There were no operative deaths or complications such as anastomosis leakage or postoperative cholangitis, but a pancreatic fistula developed in one patient in Group A and two in Group B., Conclusion: Single-stage excision for a perforated CC is feasible if the patient's condition is stable., Levels of Evidence: Treatment Study, LEVELIII., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective To investigate the effectiveness of intrapancreatic choledochal cyst excision in treating type I choledochal cyst, and increase understanding of the need for thorough surgical management of the disease. Methods Primary and secondary (including multiple) surgical cases, treated between 2005 and 2015, were retrospectively analysed, and follow-up data of post-treatment effectiveness to date were reviewed. Differences in curative effects were compared between whole and partial excision of the choledochal cyst. Results Out of 350 cases, patients with whole excision of the choledochal cyst ( n = 272) experienced no associated symptoms in the long-term (3/272 [1.1%] experienced stomach ache or fever). Patients with partial resection of the choledochal cyst ( n = 78) developed associated symptoms, including new cyst, calculus of the bile duct (51/78 [65.4%]), and carcinogenesis (11/78 [14.1%]) in the residual intrapancreatic biliary duct. Post-treatment clinical manifestations were significantly different between patients with partial resection versus whole excision of the choledochal cyst ( P<0.05). Conclusion Surgical re-excision should be considered in patients with a residual intrapancreatic portion of the choledochal cyst due to prior incomplete surgery, regardless of clinical symptoms.

Isolated choledochal cysts involving the cystic duct are rare. We present a case of a choledochal cyst involving only the proximal cystic duct, and discuss the taxonomic and therapeutic challenges. There is a need for a clearly defined classification system for these cysts as they may be categorised as either type II or type VI cysts. The optimal treatment remains debatable, with some authors recommending a bilioenteric reconstruction owing to the wide cystic duct-bile duct junction. However, we suggest that a cholecystectomy should be performed with examination of the specimen and frozen section in case of any abnormality rather than upfront bile duct excision. In addition, given the rarity of this condition and the paucity of long-term data, we recommend meticulous follow-up for development of any malignancy.

Choledochal cyst is a cystic dilation of the biliary tree that can increase the risk of malignancy in bile ducts and the gallbladder. These are usually lined by bile duct epithelium, which may undergo intestinal and squamous metaplasia. This is the first report of clinically diagnosed type II choledochal cyst that is entirely lined by metaplastic stratified squamous epithelium, unlike most other cysts, which are histologically lined by bile duct epithelium. This observation can potentially explain the underlying pathogenic mechanism of rare reports of squamous cell carcinomas arising in bile duct systems.

Introduction: Heterotopic pancreas (HP) has rarely been identified in the wall of choledochal cyst (CC)., Methods: Retrospectively we screened 200 excised specimens of CC received at our Institute over a period of last eight years and looked for presence of HP rests in them. All the specimens were processed in their entirety., Result: HP was identified in the wall of 13 (6.5%) CCs, out of which 11 were Heinrich Type 2, and two were Heinrich Type 1. In half of the cases peribiliary mucous glands were observed intermingled with the HP rests. Features of chronic fibrosing pancreatitis were identified in these rests, with ulceration of overlying cyst lining., Conclusions: HP rests in the wall of CC though rare; their coexistence with peribiliary glands may possibly indicate their common embryonic origin. As a common site of inflammation, HP rest may be one of the common causes of CC., (Copyright © 2017 Elsevier GmbH. All rights reserved.)

Background: The closure of a cystic duct during cholecystectomy by means of radiofrequency is still controversial. We report our preliminary experimental results with the use of LigaSure™ on common bile duct in rats., Methods: Thirty Wistar rats weighing 70 to 120 g were employed for this study. The animals were all anesthetized with intraperitoneal ketamine and then divided into three groups. The first group (10 rats, Group C) underwent only laparotomy and isolation of the common bile duct. The second (10 rats, Group M) underwent laparotomy and closure of the common bile duct (CBD) with monopolar coagulation. The third group (10 rats, Group L) underwent laparotomy and sealing of the common bile duct with two application of LigaSureTM. Afterwards, all rats were kept in comfortable cages and were administered dibenzamine for five days. They were all sacrificed on day 20. Through a laparotomy, the liver and bile duct were removed for histological examination. Blood samples were obtained to dose bilirubin, amylase and transaminase levels., Results: Mortality rate was 0 in the control group (C), 3/10 rats in group M and 0 in group L. In group L, the macroscopic examination showed a large choledochocele (3-3.5 × 1.5 cm) with few adhesions. At the histological examination there was optimal sealing of the common bile duct in 9/10 rats. In group M, 2/10 rats had liver abscesses, 3/10 rats had choledochocele and 5/10 rats, biliary peritonitis. There was intense tissue inflammation and the dissection was difficult. Analyses of blood samples showed an increase in total bilirubin, aspartate aminotransferase (AST) and alanine aminotransferase (ALT) in groups M and L., Conclusions: The preliminary results of our study confirm that radiofrequency can be safely used for the closure of the common bile duct. The choledochocele obtained with this technique could represent a good experimental model. These results could be a further step for using the LigaSureTM in clipless cholecystectomy.

An 18-year-old man presented with spontaneous severe epigastric pain, progressing and radiating to back since 3 days. It was associated with epigastric tenderness, bilious vomiting and jaundice. He had been intermittently experiencing these symptoms for the last 1 year. No known comorbid. Ultrasound showed a poorly visualised heterogeneous focus at porta hepatis; considering poor visualisation, this might represent an enlarged calcified lymph node or cystic duct calculus causing extrinsic compression or a large sludge ball within the common bile duct (CBD), leading to dilatation of common hepatic duct and intrahepatic biliary system. Subsequent magnetic resonance cholangiopancreatography revealed a focal saccular dilatation of middle part of CBD, a type I-B choledochal cyst, large heterogeneous focus seen within it representing choledochocystolithiasis. Later, CT was performed for further characterisation of surrounding anatomy and pathology, which confused the appearance of choledochocystolithiasis for Mirizzi syndrome. Later, surgery and histopathology confirmed type I-B choledochocystolithiasis and chronic cholecystitis., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Todani proposed the most accepted classification for cystic lesions of the bile duct, including 5 types. Serena described another one, type VI, which includes cystic lesions of the isolated cystic duct, of which there are less than twenty reported cases. They differ from type II in terms of the distal cystic caliber, which is normal in type VI, and in the diagnosis which is performed intra-operatively in most of the cases. Neoplastic degeneration has an incidence of 10 to 30% in all these lesions, which is the reason why the most widely accepted treatment is cholecystectomy with resection of the cystic duct cyst dilatation and preservation of the main bile duct by a laparoscopic approach.

Background and Aims: Cholangiopancreatoscopy for evaluating pancreaticobiliary pathology is currently limited by suboptimal optics. The aim of this study was to characterize the operating characteristics of per-oral video cholangiopancreatoscopy with narrow-band imaging (POVCP) findings in indeterminate pancreaticobiliary disease and to describe their association with neoplasia., Methods: Data from consecutive patients undergoing POVCP for the evaluation of indeterminate pancreaticobiliary disease at a single tertiary care center were analyzed. Two experienced investigators had previously agreed on POVCP findings and terminology that were documented in endoscopy reports. Endoscopic procedural data from POVCPs performed between January 2006 and April 2015 and clinical data were abstracted from the endoscopic database and electronic medical records. Study endpoints included tissue-proven neoplasia or benign disease with ≥1 year of follow-up., Results: A total of 109 patients were identified; 13 were excluded because of the presence of stone disease, known pancreaticobiliary malignancy, or presumed benign disease with ≤1 year of follow-up. Most patients (85%) underwent POVCP for biliary disease and 15% underwent POVCP for a pancreatic cause. Tortuous and dilated vessels (P < .001), infiltrative stricture (P < .001), polypoid mass (P = .003), and the presence of fish-egg lesions (P = .04) were found to be significantly associated with neoplasia. The overall POVCP impression had a high sensitivity (85%) and negative predictive value (89%) in assessing for the presence of neoplasia., Conclusions: Per-oral video cholangiopancreatoscopy is effective in the evaluation of indeterminate pancreaticobiliary disease. Tortuous and dilated vessels, infiltrative stricture, polypoid mass, and the presence of fish-egg lesions are significantly associated with neoplasia., (Copyright © 2017. Published by Elsevier Inc.)

Cystic biliary atresia (CBA), a rare cystic expansion of atretic extrahepatic bile ducts in young infants, overlaps in age at presentation and imaging features with early choledochal cysts (CC). Treatment and prognosis differ; histologic differences are unsettled. We compared 10 patients with CBA, 1975 to 2015, to an age-similar cohort of 13 infants, and to older patients who had surgery for CC. Operative details, imaging, and clinical courses were correlated to pathologic specimens. Immunostains for smooth muscle actin and myosin heavy chain were used to evaluate cyst walls and atretic segments. CBA cysts typically lacked epithelium and inflammation; cyst walls had an inner, dense cicatricial layer associated with myofibroblastic (MF) hyperplasia that often delaminated producing a grossly visible inner cyst wall. Seven proximal biliary remnants in CBA featured circumferential peribiliary MF hyperplasia/fibrosis with little or no inflammation, similar to isolated BA. Extrahepatic atresia was usually both proximal and distal to the cyst. Features in 10/13 CC from infants and 8/8 CC in older patients had mostly preserved uninjured epithelium and no subepithelial cicatrix. Mural smooth muscle (absent in CBA) was present to some extent in CC at all ages. Unexpectedly, focal MF hyperplasia and laminar sclerosis was present in a few CC in infants, resembling CBA. CBA and infant CC are distinct histologic entities that occasionally overlap. CBA bile duct injury mimics non-CBA. Cystification is an aberrant manifestation of stromal proliferation in BA. The current management approach assuming CBA and CC in infants are 2 separate disease processes is supported but caution is advised.

Purpose: There is a predisposition to the development of malignancy in congenital choledochal malformation (CCM) although the degree of risk is unknown. We investigated the role of CA19-9 in bile and the MIB-1 (Ki-67) epithelial proliferation index as markers of an at risk choledochal epithelium at the time of definitive surgery., Methods: Bile collected at surgery was analyzed for levels of amylase (as a surrogate of pancreatic reflux) and CA19-9. Immunohistochemical staining for CA19-9 and MIB-1 index (expressed as %) was performed on resected specimens. Data are quoted as median (IQR) and differences assessed using non-parametric statistics. A P value of 0.05 was regarded as significant., Results: Our study group consisted of 78 children with CCM (Type 1 fusiform, n=34; Type 1 cystic, n=30 and Type 4, n=14). Median bile CA19-9 was 159,400 (6-1×10(6)) kU/L. There was no correlation with bile amylase (P=0.49) or biliary pressure (P=0.17) but modest correlation with bilirubin (rs=0.24; P=0.02). In contrast, bile amylase was correlated with plasma γ-glutamyl transpeptidase (P=0.02), alkaline phosphatase (P=0.05) and aspartate aminotransferase (P=0.02); and inversely correlated with biliary pressure (rs=-0.38; P<0.0008). Epithelial expression of CA19-9 and MIB-1 was assessed in 43 specimens. CA19-9 was diffusely expressed on all choledochal epithelium. MIB-1 expression was divided into: high expression (>40%) n=3; moderate (20-40%) n=5, low (6-20%) n=7 and very low (≤5%) n=28. There was no correlation with choledochal pressure (P=0.87), CA19-9 (P=0.51) or bile amylase (P=0.55)., Conclusion: Biliary CA19-9 levels were grossly (and unexpectedly) raised in choledochal malformation and appear to arise from biliary rather than pancreatic epithelium. MIB-1 confirms that a small proportion (19%) has marked epithelial proliferation but no clinical correlates could be identified., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Intracholecystic papillary-tubular neoplasms are rare precursor lesions of gallbladder cancer. They were proposed as a separate pathologic entity in 2012 by Adsay et al. for the unification of a variety of mass-forming precursor lesions including papillary adenomas, tubulopapillary adenomas, intestinal adenomas, and others. They are considered homologous to intrapapillary mucinous neoplasms of the pancreas and intrabiliary papillary neoplasms of the common bile duct. In contrast with the commoner flat-type precursor gallbladder cancer lesions, they follow a more indolent clinical course and probably different genetic pathways to carcinogenesis. They are largely uninvestigated with only a handful of studies providing biological and clinical information. Choledochal cysts are dilation of the common bile duct. Diagnosis is usually established during childhood, and only a minority of patients are diagnosed at adulthood. They are of major clinical importance as they are known predisposing factors for biliary carcinogenesis., Case Presentation: The current report describes a patient with a simultaneous diagnosis of choledochal cyst and intracholecystic papillary-tubular neoplasm. The patient underwent excision of the extrahepatic biliary tree for a Todani I choledochal cyst, and histological examination of the specimen revealed an intracholecystic papillary-tubular neoplasm of the gallbladder. Authors describe diagnostic and clinical course of the patient alongside clinical and biological characteristics of these rare lesions., Conclusions: To the best of our knowledge, this is the first report of a patient with a simultaneous diagnosis of choledochal cyst and intracholecystic papillary-tubular neoplasm. Those rare lesions shed light on different forms of gallbladder cancer carcinogenesis and its relationship with choledochal cysts and cholestasis.

The aim of the current study was to investigate survivin expression in congenital choledochal cysts (CCCs), and its associations with clinical parameters of CCCs. In total, 121 children with CCCs were included in this study as the case group, and their cysts were staged according to the Todani classification system. Additionally, 49 normal gallbladder specimens from healthy children were included as the control group. Survivin detection was conducted using immunohistochemical staining. Associations between positive survivin expression and clinical parameters of CCCs were then analyzed. Positive survivin expression was observed in the cytoplasm, and was seen as granular with yellow or dark brown staining. In the case group, positive survivin expression was detected in most tissues. Specifically, compared to that of normal tissues, the cystic-shaped and fusiform-shaped CCC tissues had significantly higher positive survivin expression rates (all P < 0.05). Importantly, positive survivin expression was also shown to be significantly associated with gender and histological type (both P < 0.05). In conclusion, increased survivin expression was observed in CCC tissues, and was correlated with certain clinical parameters of CCCs, suggesting a possible prognostic value of survivin for CCC progression.

Background: The aim of this study was to assess factors for delineating the pancreaticobiliary junction in the presence of pediatric congenital choledochal cysts (CCC) using Magnetic resonance cholangiopancreatography (MRCP)., Methods: Retrospective review of medical records for 48 patients with CCC was conducted, including demographics, biliary amylase and MRCP findings if available. With univariate and multivariate logistic regression, we measured significant factors affecting pancreaticobiliary maljunction(PBM) diagnoses by MRCP., Results: Of the subjects enrolled with CCC. Twenty-eight cases had PBM according to MRCP. Univariate analysis confirmed that age, cyst diameter > 30 mm and cysts that descended to the introitus pelvis affected junctional delineation and detection of PBM (P<0.05). Stepwise logistic regression analysis confirmed large cysts in the introitus pelvis predicted pancreaticobiliary junctional delineation in MRCP and these data agreed with the literature. A correlation between cyst diameter and the length of the common channel was found as was cyst diameter and biliary amylase although there were no significant differences between them., Conclusions: Age, cyst diameter >30 mm and descending cysts into the introitus pelvis affected junctional delineation of the pancreatic and bile duct in PBM with MRCP. Large cyst descension into the introitus pelvis was an independent factors affecting PBM detection.

Background and Aims: Interstitial cells of Cajal (ICC) have been shown to be present in the extrahepatic biliary tract of animals and humans. However, ICC distribution in choledochal cysts (CC) has not been investigated. A study was conducted to investigate the distribution of ICC in the extrahepatic biliary tract, including CC, in pediatric human specimens., Method: The specimens were divided into two main groups as gallbladders and common bile ducts. Gallbladders were obtained from the cholelithiasis, CC operations and autopsies. Common bile ducts were obtained from autopsies. Tissues were stained using c-kit immunohistochemical staining. ICC were assessed semi-quantitatively by applying morphological criteria and were counted as the number of cells/0.24 mm(2) in each area under light microscopy., Results: A total of 35 gallbladders and 14 CC were obtained from operations. Ten gallbladders plus common bile ducts were obtained from autopsies. The mean numbers of ICC in the gallbladders of cholelithiasis and the gallbladders of CC were 12.2 ± 4.9 and 5.3 ± 1.2, respectively (p = 0.003). The mean numbers of ICC in the common bile ducts and CC were 9.8 ± 2.9 and 3.4 ± 1.4, respectively (p = 0.001)., Conclusion: The scarcity of ICC in the extrahepatic biliary tract may be responsible for the etiopathogenesis of the CC.

Introduction: Cysts of the bile duct or choledochal cysts are rare diseases in our area. The aetiology is unknown, with the most accepted hypothesis being a pancreatobiliary maljunction anomaly., Objective: To analyse the clinical data, diagnosis and treatment of a number of patients with choledochal cyst, as well as presenting an update on this condition., Method: A retrospective descriptive study was performed on paediatric patients diagnosed with choledochal cyst in the last 20 years in a tertiary hospital., Case Reports: A total of 4 choledochal cyst cases in childhood, predominantly female, are pre- sented. The most frequent reason for consultation was vomiting, and presenting with jaundice and choluria in all cases. Patients with choledochal cyst were classified as type I in 3 cases, and one case of type IVa. In all cases surgical treatment was performed; any patient had complications to date., Conclusions: Cysts of the bile ducts have a low prevalence. The treatment of choice is surgical, requiring close monitoring due to the risk of cholangiocarcinoma., (Copyright © 2015 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.)