{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T10:09:56Z","timestamp":1790244596718,"version":"4.1.0"},"reference-count":39,"publisher":"MDPI AG","issue":"19","license":[{"start":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T00:00:00Z","timestamp":1790208000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. Clin. Med.","JCM"],"abstract":"<jats:p>Background: Endoscopic retrograde cholangiopancreatography is an established therapeutic modality for pancreaticobiliary disease. Congenital anatomical anomalies, including situs inversus totalis and rare hepatobiliary malformations, substantially increase the technical complexity of endoscopic retrograde cholangiopancreatography and are seldom encountered in clinical practice. Published experience is limited predominantly to individual case reports and smaller case series. Methods: We present a report of three patients who underwent endoscopic retrograde cholangiopancreatography: two with situs inversus totalis and choledocholithiasis complicated by acute cholangitis, and one with a rare congenital anomaly consisting of a completely separated right hepatic lobe combined with choledocholithiasis. Results: Successful endoscopic retrograde cholangiopancreatography was achieved in all three patients. In both situs inversus totalis cases, biliary cannulation and stone clearance were accomplished using modified endoscopic techniques, including endoscope rotation and positional adjustments, without procedural complications. In the patient with the separated hepatic lobe, endoscopic retrograde cholangiopancreatography allowed common bile duct stone clearance; however, an attempted mechanical lithotripsy of a gallbladder calculus resulted in basket entrapment, necessitating surgical treatment. Conclusions: Endoscopic retrograde cholangiopancreatography is feasible and effective in patients with congenital hepatobiliary anomalies, but demands meticulous pre-procedural planning, thorough anatomical understanding, and technical versatility. A cautious step-by-step approach and readiness to adapt technique are essential to optimise outcomes and minimise risk.<\/jats:p>","DOI":"10.3390\/jcm15197419","type":"journal-article","created":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T09:07:54Z","timestamp":1790240874000},"page":"7419","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Managing Choledocholithiasis in Rare Congenital Hepatobiliary Anomalies: A Report of Three Cases"],"prefix":"10.3390","volume":"15","author":[{"given":"Andreja","family":"Ocepek","sequence":"first","affiliation":[{"name":"Department of Gastroenterology, University Division of Internal Medicine, University Medical Centre Maribor, Ljubljanska 5, 2000 Maribor, Slovenia"},{"name":"Medical Faculty, University of Maribor, Taborska 8, 2000 Maribor, Slovenia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luka","family":"Strni\u0161a","sequence":"additional","affiliation":[{"name":"Clinical Department of Gastroenterology, Division of Internal Medicine, University Medical Centre Ljubljana, Japljeva 2, 1000 Ljubljana, Slovenia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabina","family":"Vadnjal \u0110onlagi\u0107","sequence":"additional","affiliation":[{"name":"Department of Radiology, University Medical Centre Maribor, Ljubljanska 5, 2000 Maribor, Slovenia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-2017-7927","authenticated-orcid":false,"given":"Darinka","family":"Purg","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, University Division of Internal Medicine, University Medical Centre Maribor, Ljubljanska 5, 2000 Maribor, Slovenia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3751-2619","authenticated-orcid":false,"given":"Sara","family":"Nikoli\u0107","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, University Division of Internal Medicine, University Medical Centre Maribor, Ljubljanska 5, 2000 Maribor, Slovenia"},{"name":"Medical Faculty, University of Maribor, Taborska 8, 2000 Maribor, Slovenia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,9,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"55","DOI":"10.32074\/1591-951X-709","article-title":"Congenital anomalies of the gastrointestinal tract: The liver, extrahepatic biliary tree and pancreas","volume":"114","author":"Ludwig","year":"2022","journal-title":"Pathologica"},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"TA, H., and Vagli, P. (2008). Congenital Malformations, Liver and Biliary Tract. Encyclopedia of Diagnostic Imaging, Springer.","DOI":"10.1007\/978-3-540-35280-8_573"},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Eitler, K., Mathe, Z., Papp, V., Zalatnai, A., Bibok, A., Deak, P.A., Kobori, L., and Telkes, G. (2021). Double rarity: Malignant masquerade biliary stricture in a situs inversus totalis patient. BMC Surg., 21.","DOI":"10.1186\/s12893-021-01155-w"},{"key":"ref_4","first-page":"e87427","article-title":"Situs Inversus Totalis: Challenges and Anatomical Considerations in Endoscopic Retrograde Cholangiopancreatography","volume":"17","author":"Aujla","year":"2025","journal-title":"Cureus"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1002\/jhbp.512","article-title":"Tokyo Guidelines 2018: Diagnostic criteria and severity grading of acute cholangitis (with videos)","volume":"25","author":"Kiriyama","year":"2018","journal-title":"J. Hepato-Biliary-Pancreat. Sci."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1055\/a-1075-4080","article-title":"ERCP-related adverse events: European Society of Gastrointestinal Endoscopy (ESGE) Guideline","volume":"52","author":"Dumonceau","year":"2020","journal-title":"Endoscopy"},{"key":"ref_7","doi-asserted-by":"crossref","unstructured":"Ding, B., Wang, J., Wei, X., Du, Y., Xia, L., Sun, C., Han, K., Yang, X., Guo, X., and Pan, Y. (2022). Efficacy and safety of ERCP in patients with situs inversus totalis: Multicenter case series and literature review. BMC Gastroenterol., 22.","DOI":"10.1186\/s12876-022-02593-3"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"100555","DOI":"10.1016\/j.gastha.2024.09.010","article-title":"Successful Endoscopic Retrograde Cholangiopancreatography for Management of Choledocholithiasis in a Patient with Situs Inversus Totalis: A Case Report and Literature Review","volume":"4","author":"Alrufayi","year":"2025","journal-title":"Gastro Hep Adv."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"256","DOI":"10.22543\/2392-7674.1446","article-title":"ERCP extraction of stones in situs inversus patients; state-of-the-art techniques","volume":"11","author":"Gunsahin","year":"2024","journal-title":"J. Mind Med. Sci."},{"key":"ref_10","first-page":"5","article-title":"Coledocolitiasis primaria en situs inversus total","volume":"43","year":"2021","journal-title":"Cir. Gen."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"106873","DOI":"10.1016\/j.ijscr.2022.106873","article-title":"Successful endoscopic drainage of malignant obstructive jaundice in patients with situs inversus totalis: Two cases report","volume":"93","author":"Gao","year":"2022","journal-title":"Int. J. Surg. Case Rep."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1007\/s12328-020-01198-1","article-title":"Successful removal of multiple bile duct stones using a papillary large balloon dilation in a very elderly woman with situs inversus totalis","volume":"14","author":"Shimoda","year":"2021","journal-title":"Clin. J. Gastroenterol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1016\/j.dld.2020.09.019","article-title":"Successful endoscopic retrograde cholangiopancreatography and bilateral stenting in a patient with situs inversus totalis","volume":"54","author":"Oh","year":"2022","journal-title":"Dig. Liver Dis."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"106347","DOI":"10.4253\/wjge.v17.i6.106347","article-title":"Endoscopic retrograde cholangiopancreatography treatment of cholangitis stone in a patient with total situs inversus: A case report","volume":"17","author":"Gong","year":"2025","journal-title":"World J. Gastrointest. Endosc."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"362","DOI":"10.1016\/j.vgie.2021.05.002","article-title":"A rotary papillotome plus adjustment of the monitor position to the foot side helps facilitate ERCP in a patient with situs inversus totalis","volume":"6","author":"Zennyoji","year":"2021","journal-title":"VideoGIE"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"e11","DOI":"10.1053\/j.gastro.2019.11.022","article-title":"A Challenging Abdominal Pain","volume":"158","author":"Rocha","year":"2020","journal-title":"Gastroenterology"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1159\/000119713","article-title":"ERCP in Total Situs Viscerum Inversus","volume":"2","author":"Fiocca","year":"2008","journal-title":"Case Rep. Gastroenterol."},{"key":"ref_18","first-page":"488","article-title":"A review on biodegradable biliary stents: Materials and future trends","volume":"17","author":"Song","year":"2022","journal-title":"Bioact. Mater."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"108391","DOI":"10.12998\/wjcc.v13.i27.108391","article-title":"Bioresorbable biliary stents: A step forward","volume":"13","author":"Zhang","year":"2025","journal-title":"World J. Clin. Cases"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1007\/BF01784645","article-title":"A general review of anomalies of hepatic morphology and their clinical implications","volume":"7","author":"Champetier","year":"1985","journal-title":"Anat. Clin."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1016\/j.jviscsurg.2014.09.013","article-title":"Accessory liver lobes: Anatomical description and clinical implications","volume":"151","author":"Glenisson","year":"2014","journal-title":"J. Visc. Surg."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"e430","DOI":"10.1097\/MD.0000000000000430","article-title":"Riedel\u2019s lobe of the liver: A case report","volume":"94","author":"Savopoulos","year":"2015","journal-title":"Medicine"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1055\/a-1020-3932","article-title":"Endoscopic ultrasound-guided gallbladder drainage, transpapillary drainage, or percutaneous drainage in high risk acute cholecystitis patients: A systematic review and comparative meta-analysis","volume":"52","author":"Mohan","year":"2020","journal-title":"Endoscopy"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"411","DOI":"10.5946\/ce.2015.48.5.411","article-title":"Endoscopic Gallbladder Drainage for Acute Cholecystitis","volume":"48","author":"Widmer","year":"2015","journal-title":"Clin. Endosc."},{"key":"ref_25","doi-asserted-by":"crossref","unstructured":"Cao, J., Ding, X., Wu, H., Shen, Y., Zheng, R., Peng, C., Wang, L., and Zou, X. (2019). Classification of the cystic duct patterns and endoscopic transpapillary cannulation of the gallbladder to prevent post-ERCP cholecystitis. BMC Gastroenterol., 19.","DOI":"10.1186\/s12876-019-1053-6"},{"key":"ref_26","doi-asserted-by":"crossref","unstructured":"Zhang, Y., Zhang, S., Li, F., Jing, X., Liu, Q., and Yu, M. (2026). ERCP management of cystic duct stones with a rare anatomic variant in octogenarian: A case report. Front. Surg., 13.","DOI":"10.3389\/fsurg.2026.1801270"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"172","DOI":"10.5009\/gnl.2012.6.2.172","article-title":"Epidemiology of gallbladder disease: Cholelithiasis and cancer","volume":"6","author":"Stinton","year":"2012","journal-title":"Gut Liver"},{"key":"ref_28","doi-asserted-by":"crossref","unstructured":"ASGE Technology Committee, Adler, D.G., Conway, J.D., Farraye, F.A., Kantsevoy, S.V., Kaul, V., Kethu, S.R., Kwon, R.S., Mamula, P., and Pedrosa, M.C. (2009). Biliary and pancreatic stone extraction devices. Gastrointest. Endosc., 70, 603\u2013609.","DOI":"10.1016\/j.gie.2009.06.015"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"114936","DOI":"10.3748\/wjg.114936","article-title":"Technical overview of difficult biliary cannulation in endoscopic retrograde cholangiopancreatography: A guide for the endoscopist","volume":"32","author":"Mishra","year":"2026","journal-title":"World J. Gastroenterol."},{"key":"ref_30","first-page":"64","article-title":"Difficult Biliary Cannulation in Endoscopic Retrograde Cholangiopancreatography: Definitions, Risk Factors, and Implications","volume":"9","author":"Fung","year":"2021","journal-title":"Eur. Med. J. Hepatol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"E671","DOI":"10.1055\/a-2358-1090","article-title":"Integrated cholangioscopy-assisted lithotripsy and a novel basket approach for managing complex cystic duct confluence stones","volume":"56","author":"Kuraishi","year":"2024","journal-title":"Endoscopy"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1016\/j.vgie.2020.03.009","article-title":"ERCP-directed electrohydraulic lithotripsy for treatment of cystic duct and remnant gallbladder stones","volume":"5","author":"Marya","year":"2020","journal-title":"VideoGIE"},{"key":"ref_33","doi-asserted-by":"crossref","unstructured":"Yang, Z., Hong, J., Zhu, L., Zhang, C., Zhou, X., Li, G., Zhu, Y., Liu, Z., Zhou, X., and Chen, Y. (2022). The clinical effect of a strategy called transcystic gallbladder-preserving cholecystolithotomy based on endoscopic retrograde cholangiopancreatography for cholecystolithiasis: A retrospective study from a single center. Front. Surg., 9.","DOI":"10.3389\/fsurg.2022.1021395"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1016\/j.vgie.2020.03.005","article-title":"Efficacy and safety of endoscopic laser lithotripsy and lithotomy through the lu-men-apposing metal stent for giant gallbladder stones","volume":"5","author":"Wang","year":"2020","journal-title":"VideoGIE"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"322","DOI":"10.14701\/ahbps.22-138","article-title":"A rare case report of Mirizzi syndrome type III treatment algorithm in situs inversus totalis, large ventricular septal defect and transposition of great arteries in a young diabetic patient","volume":"27","author":"Badipati","year":"2023","journal-title":"Ann. Hepato-Biliary-Pancreat. Surg."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"736","DOI":"10.12998\/wjcc.v9.i3.736","article-title":"Cystic duct dilation through endoscopic retrograde cholangiopancreatography for treatment of gallstones and choledocholithiasis: Six case reports and review of literature","volume":"9","author":"He","year":"2021","journal-title":"World J. Clin. Cases"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"S144","DOI":"10.1055\/s-0046-1820881","article-title":"3D visualization enhances cannulation performance in ERCP simulation among novice trainees\u2014Simulator study","volume":"58","author":"Finta","year":"2026","journal-title":"Endoscopy"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"808","DOI":"10.1097\/JS9.0000000000003302","article-title":"3D-assisted ERCP enhances cannulation efficiency in malignant biliary obstruction: A prospective two-center study","volume":"112","author":"Fan","year":"2026","journal-title":"Int. J. Surg."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"681","DOI":"10.5152\/tjg.2024.24149","article-title":"Endoscopic management of benign gallbladder diseases","volume":"35","author":"Ji","year":"2024","journal-title":"Turk. J. Gastroenterol."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/19\/7419\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T09:36:50Z","timestamp":1790242610000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/19\/7419"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,24]]},"references-count":39,"journal-issue":{"issue":"19","published-online":{"date-parts":[[2026,10]]}},"alternative-id":["jcm15197419"],"URL":"https:\/\/doi.org\/10.3390\/jcm15197419","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,24]]}}}