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Ray S, Saha H, Sen S, Das S, Lahiri S, Das S, Mandal TS, Biswas J, Khamrui S. Presentation and outcomes of surgery for choledochal cyst in children and adults: an experience of 329 cases. Updates Surg 2025; 77:459-470. [PMID: 40035920 DOI: 10.1007/s13304-025-02138-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/24/2024] [Accepted: 02/24/2025] [Indexed: 03/06/2025]
Abstract
The purpose of this study is to report our experience in patients with choledochal cyst (CDC) and to evaluate whether there is any difference in the clinical presentation, cyst -related complications, and long-term biliary outcomes after surgery among children and adults. All the patients who underwent CDC excision between January 2008 and December 2023 were retrospectively reviewed. The patients were divided into two groups: children (< 18 years) and adults (≥ 18 years). There were 329 patients with CDC excision (77.5% were female, adult/children = 251/78). Abdominal pain was the predominant presenting symptom in both groups. Abdominal lump (10.3% vs 3.2%; p = 0.011) and the triad of CDC (9% vs 2.4%; p = 0.009) were more common in children. But, the preoperative biliary intervention (37.8% vs 15.4%; p = < 0.001), previous biliary surgery (32.7% vs 3.8%; p = < 0.001), and prevalence of gallstones (36.7% vs 3.8%; p = < 0.001), cystolithiasis (56.6% vs 38.5%; p = 0.005), and intrahepatic stones (7.6% vs 1.3%; p = 0.054) were more common in adults. Most patients were presented with type I CDC (71.4%). Extrahepatic bile duct excision with Roux en-Y hepaticojejunostomy was performed in all the patients. Although overall postoperative complications were comparable between adults and children (31.1% vs 26.9%; p = 0.485), wound infection was more common in adults (19.9% vs 6.4%; p = 0.005) and bile leak was more common in children (12.8% vs 5.2%; p = 0.021). There was no operative mortality. Over a mean follow-up of 59 (40) months, 2 patients (0.78%) developed malignancy. Excellent or good Long-term biliary outcome was significantly better in children than adults (98.1% vs 90.5%; p = 0.010). Independent predictors for unsatisfactory biliary outcomes were type IVA cysts, postoperative bile leak, adult patients, and longer duration of follow-up. In conclusion, presentation and cyst-related complications differ between adults and children. Surgery offers excellent results in the vast majority of patients. Long-term biliary complications were more in adults. Long-term follow-up is recommended for the possibility of future development of hepato-biliary-pancreatic malignancy and delayed biliary complications.
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Affiliation(s)
- Sukanta Ray
- Division of Surgical Gastroenterology, School of Digestive and Liver Diseases, Institute of Postgraduate Medical Education and Research, 244 A. J. C. Bose Road, Kolkata, West Bengal, 700020, India.
| | - Hemabha Saha
- Division of Surgical Gastroenterology, School of Digestive and Liver Diseases, Institute of Postgraduate Medical Education and Research, 244 A. J. C. Bose Road, Kolkata, West Bengal, 700020, India
| | - Swapnil Sen
- Division of Surgical Gastroenterology, School of Digestive and Liver Diseases, Institute of Postgraduate Medical Education and Research, 244 A. J. C. Bose Road, Kolkata, West Bengal, 700020, India
| | - Suman Das
- Division of Surgical Gastroenterology, School of Digestive and Liver Diseases, Institute of Postgraduate Medical Education and Research, 244 A. J. C. Bose Road, Kolkata, West Bengal, 700020, India
| | - Somdatta Lahiri
- Division of Surgical Gastroenterology, School of Digestive and Liver Diseases, Institute of Postgraduate Medical Education and Research, 244 A. J. C. Bose Road, Kolkata, West Bengal, 700020, India
| | - Somak Das
- Division of Surgical Gastroenterology, School of Digestive and Liver Diseases, Institute of Postgraduate Medical Education and Research, 244 A. J. C. Bose Road, Kolkata, West Bengal, 700020, India
| | - Tuhin Subhra Mandal
- Division of Surgical Gastroenterology, School of Digestive and Liver Diseases, Institute of Postgraduate Medical Education and Research, 244 A. J. C. Bose Road, Kolkata, West Bengal, 700020, India
| | - Jayanta Biswas
- Division of Surgical Gastroenterology, School of Digestive and Liver Diseases, Institute of Postgraduate Medical Education and Research, 244 A. J. C. Bose Road, Kolkata, West Bengal, 700020, India
| | - Sujan Khamrui
- Division of Surgical Gastroenterology, School of Digestive and Liver Diseases, Institute of Postgraduate Medical Education and Research, 244 A. J. C. Bose Road, Kolkata, West Bengal, 700020, India
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Mao HM, Zhang JJ, Zhu B, Guo WL. A novel deep learning radiopathomics model for predicting carcinogenesis promotor cyclooxygenase-2 expression in common bile duct in children with pancreaticobiliary maljunction: a multicenter study. Insights Imaging 2025; 16:74. [PMID: 40146354 PMCID: PMC11950503 DOI: 10.1186/s13244-025-01951-5] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/23/2025] [Accepted: 03/09/2025] [Indexed: 03/28/2025] Open
Abstract
OBJECTIVES To develop and validate a deep learning radiopathomics model (DLRPM) integrating radiological and pathological imaging data to predict biliary cyclooxygenase-2 (COX-2) expression in children with pancreaticobiliary maljunction (PBM), and to compare its performance with single-modality radiomics, deep learning radiomics (DLR), and pathomics models. METHODS This retrospective study included 219 PBM patients, divided into a training set (n = 104; median age, 2.8 years, 75.0% females) and internal test set (n = 71; median age, 2.2 years, 83.1% females) from center I, and an external test set (n = 44; median age, 3.4 years, 65.9% females) from center II. Biliary COX-2 expression was detected using immunohistochemistry. Radiomics, DLR, and pathomics features were extracted from portal venous-phase CT images and H&E-stained histopathological slides, respectively, to build individual single-modality models. These were then integrated to develop the DLRPM, combining three predictive signatures. Model performance was evaluated using AUC, net reclassification index (NRI, for assessing improvement in correct classification) and integrated discrimination improvement (IDI). RESULTS The DLRPM demonstrated the highest performance, with AUCs of 0.851 (95% CI, 0.759-0.942) in internal test set and 0.841 (95% CI, 0.721-0.960) in external test set. In comparison, AUCs for the radiomics, DLR, and pathomics models were 0.532-0.602, 0.658-0.660, and 0.787-0.805, respectively. The DLRPM significantly outperformed three single-modality models, as demonstrated by the NRI and IDI tests (all p < 0.05). CONCLUSION The multimodal DLRPM could accurately and robustly predict COX-2 expression, facilitating risk stratification and personalized postoperative management in PBM. However, prospective multicenter studies with larger cohorts are needed to further validate its generalizability. CRITICAL RELEVANCE STATEMENT Our proposed deep learning radiopathomics model, integrating CT and histopathological images, provides a novel and cost-effective approach to accurately predict biliary cyclooxygenase-2 expression, potentially advancing individualized risk stratification and improving long-term outcomes for pediatric patients with pancreaticobiliary maljunction. KEY POINTS Predicting biliary COX-2 expression in pancreaticobiliary maljunction (PBM) is critical but challenging. A deep learning radiopathomics model achieved high predictive accuracy for COX-2. The model supports patient stratification and personalized postoperative management in PBM.
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Affiliation(s)
- Hui-Min Mao
- Department of Radiology, Children's Hospital of Soochow University, Suzhou, China
| | - Jian-Jun Zhang
- Department of Neonatal Surgery, Xuzhou Children's Hospital, Xuzhou, China
| | - Bin Zhu
- Department of Interventional Therapy, Xuzhou Children's Hospital, Xuzhou, China
| | - Wan-Liang Guo
- Department of Radiology, Children's Hospital of Soochow University, Suzhou, China.
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Nigam A, Bloomfield GC, Boumezrag M, Ali SM, Kwon D, Jha RC, Fishbein TM, Radkani P, Winslow ER. Impact of prior cholecystectomy on diagnosis and outcomes of choledochal cyst resection in adults. Am J Surg 2025; 240:116088. [PMID: 39577120 DOI: 10.1016/j.amjsurg.2024.116088] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/24/2024] [Revised: 10/04/2024] [Accepted: 11/11/2024] [Indexed: 11/24/2024]
Abstract
INTRODUCTION The diagnosis of choledochal cysts in the adult population is complicated by the expected physiologic dilation of the common bile duct after cholecystectomy. We aimed to compare patients who underwent choledochal cyst resection based on cholecystectomy status. METHODS A retrospective analysis was conducted of patients who underwent choledochal cyst resection between 1/1/1998-12/31/2021. Patients were categorized based on whether they had undergone cholecystectomy prior to choledochal cyst diagnosis. Preoperative imaging characteristics, pathology findings, and outcomes were evaluated. RESULTS Amongst 119 patients who underwent excision, 58 (46 %) had and 69 (54 %) had not undergone prior cholecystectomy. Preoperative imaging demonstrated no difference in biliary tract diameter although a greater proportion of patients with a gallbladder in place had an anomalous pancreaticobiliary junction (55 % v 33 %, p < 0.05). Biliary malignancy was observed in a greater proportion of patients with prior cholecystectomy although this was not statistically significant (5 % v 3 %; p = 0.9). Rates of post-operative complications were statistically similar between patient cohorts. DISCUSSION Radiographic and clinical features were similar among patients who had and had not undergone cholecystectomy. Choledochal cyst patients should be managed uniformly regardless of cholecystectomy status.
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Affiliation(s)
- Aradhya Nigam
- Department of Surgery, Medstar Georgetown University Hospital, Washington, DC, USA
| | | | - Maryam Boumezrag
- Department of Diagnostic Radiology, Medstar Georgetown University Hospital, Washington, DC, USA
| | - Salima Mansoor Ali
- Department of Clinical and Laboratory Pathology, Medstar Georgetown University Hospital, Washington, DC, USA
| | - DongHyang Kwon
- Department of Clinical and Laboratory Pathology, Medstar Georgetown University Hospital, Washington, DC, USA
| | - Reena C Jha
- Department of Diagnostic Radiology, Medstar Georgetown University Hospital, Washington, DC, USA
| | - Thomas M Fishbein
- Department of Surgery, Medstar Georgetown University Hospital, Washington, DC, USA
| | - Pejman Radkani
- Department of Surgery, Medstar Georgetown University Hospital, Washington, DC, USA
| | - Emily R Winslow
- University of Wisconsin School of Medicine and Public Health, Madison, WI, USA.
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Konstantinoff KS, Ludwig DR, Sharbidre K, Arif-Tiwari H, Itani M. Gallbladder and biliary pathology: lessons learned from multidisciplinary conference. Abdom Radiol (NY) 2025; 50:916-935. [PMID: 39259353 DOI: 10.1007/s00261-024-04533-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/28/2024] [Revised: 08/12/2024] [Accepted: 08/14/2024] [Indexed: 09/13/2024]
Abstract
The radiologic diagnosis of biliary disease can be challenging due to atypical or delayed presentation, rare or less common entities, and imaging overlap of benign and malignant processes. Establishing a specific diagnosis, when possible, is important to avoid progression of infections to sepsis and multiorgan failure, and for appropriate staging and management in cases of malignancy. Gallstones are the most common biliary disease, and along with stone-related complications, including cholecystitis and choledocholithiasis, constitute the majority of acute biliary pathology. Late and atypical manifestations of acute cholecystitis demonstrate imaging overlap with primary gallbladder cholangiocarcinoma, especially with cases of exuberant inflammatory reaction such as xanthogranulomatous cholecystitis. Additional challenging scenarios related to gallbladder disease, that may be benign or malignant, include adjacent fistulas and lymphadenopathy. Dropped gallstones, especially in atypical locations, may be misdiagnosed as neoplastic. Recurrent cholecystitis after cholecystectomy, whether related to subtotal cholecystectomy or to stumpitis, is another entity that can be confusing to the radiologist with a documented history of cholecystectomy. Inflammatory and autoimmune conditions, such as pseudotumors and IgG4 disease, are a less common but not infrequent cause of diagnostic dilemma. Furthermore, biliary strictures and hepatobiliary cystic lesions can be benign or malignant and could constitute a diagnostic and management challenge. The goal of this manuscript is to present the lessons learned from multidisciplinary conferences on the above entities and suggest tips and pearls to maximize the value of radiologists' contribution to patient management.
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Affiliation(s)
- Katerina S Konstantinoff
- Mallinckrodt Institute of Radiology, Washington University in St. Louis, 510 S. Kingshighway Blvd, St., St. Louis, MO, 63110, USA
| | - Daniel R Ludwig
- Mallinckrodt Institute of Radiology, Washington University in St. Louis, 510 S. Kingshighway Blvd, St., St. Louis, MO, 63110, USA
| | - Kedar Sharbidre
- Department of Radiology, University of Alabama in Birmingham, 500 22nd Street South, Birmingham, AL, 35233, USA
| | - Hina Arif-Tiwari
- Department of Medical Imaging, University of Arizona College of Medicine, 1501 N. Campbell, Tucson, AZ, 85724, USA
| | - Malak Itani
- Mallinckrodt Institute of Radiology, Washington University in St. Louis, 510 S. Kingshighway Blvd, St., St. Louis, MO, 63110, USA.
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Tao M, Wang X, Han J, Cao L, Li J, Zheng S. A new classification and laparoscopic treatment of extrahepatic choledochal cyst. Clin Res Hepatol Gastroenterol 2024; 48:102413. [PMID: 38960124 DOI: 10.1016/j.clinre.2024.102413] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/22/2024] [Revised: 06/19/2024] [Accepted: 07/01/2024] [Indexed: 07/05/2024]
Abstract
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.
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Affiliation(s)
- Meng Tao
- Department of Hepatobiliary Surgery, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, 510250, PR China
| | - Xiaojun Wang
- Institute of Hepatobiliary Surgery, First Affiliated Hospital, Army Medical University, Chongqing, 400038, PR China
| | - Jing Han
- Cancer Hospital of Zhengzhou University & Henan Cancer Hospital, Zhengzhou, 450008, PR China
| | - Li Cao
- Institute of Hepatobiliary Surgery, First Affiliated Hospital, Army Medical University, Chongqing, 400038, PR China
| | - Jianwei Li
- Institute of Hepatobiliary Surgery, First Affiliated Hospital, Army Medical University, Chongqing, 400038, PR China
| | - Shuguo Zheng
- Institute of Hepatobiliary Surgery, First Affiliated Hospital, Army Medical University, Chongqing, 400038, PR China.
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Abo-Namous R, Kuebler JF, Potthoff A, Madadi-Sanjani O, Uecker M, Dingemann J, Petersen C, Ure B, Schukfeh N. Lost in transition? Loss of follow-up and quality of life in adults after resection of choledochal malformation in childhood. Innov Surg Sci 2024; 9:93-98. [PMID: 39100717 PMCID: PMC11293947 DOI: 10.1515/iss-2023-0061] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/10/2023] [Accepted: 05/21/2024] [Indexed: 08/06/2024] Open
Abstract
Objectives Choledochal malformation (CM) is a rare disease that can lead to malignancy and potential long-term sequelae despite surgical resection. There is no long-term follow-up data on patients after CM resection in Germany. We aimed to determine the long-term outcome of our patients with a duration of follow-up >10 years and focused on long-term sequelae and health-related quality of life (HRQOL). Methods All patients who had undergone CM-resection in our department from 01/1978 to 06/2009 were contacted. Patients were interviewed about postoperative complications and their present medical attendance. HRQOL was determined using Pediatric Quality of Life Inventory 4.0 (PedsQL), version for adults. The PedsQL scales the HRQOL from 0 to 100, with higher scores indicating a better HRQOL. Scores were compared to those published for a healthy population. Results Out of 56 patients who were contacted, 23 (41 %) participated. The median age at time of surgery was 3.1 years (6 days-16.1 years) and at time of the survey 24.3 years (11.1-53.8 years). Eighteen patients (78 %) had ceased their gastroenterologic follow-up at a median time of 4.3 years after surgery. Five (22 %) were still in gastroenterologic follow-up, two of these had an uneventful clinical course, and three (13 %) had ongoing complications attributed to the CM. One of these had undergone hemihepatectomy 34 years postoperatively due to bile duct stenosis, one had undergone removal of bile duct stones 14 years postoperatively, and one suffered from portal vein thrombosis with esophageal and jejunal varices. There was no mortality in our series. Median total HRQOL score was 89. There was no significant difference in the median total health, physical health, and psychosocial health scores of our patients in comparison to the healthy population. Conclusions We confirmed that the majority of patients after CM resection are lost to follow-up. Those who answered our questionnaire showed a good HRQOL. Given the high rate of severe long-term complications and the life-long risk of malignancy, we recommend a transition program for all patients.
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Affiliation(s)
- Reem Abo-Namous
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
| | - Joachim F. Kuebler
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
| | - Andrej Potthoff
- Department of Gastroenterology, Hannover Medical School, Hannover, Germany
| | | | - Marie Uecker
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
| | - Jens Dingemann
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
| | - Claus Petersen
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
| | - Benno Ure
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
| | - Nagoud Schukfeh
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
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Bloomfield GC, Nigam A, Calvo IG, Dorris CS, Fishbein TM, Radkani P, Winslow ER. Characteristics and malignancy rates of adult patients diagnosed with choledochal cyst in the West: a systematic review. J Gastrointest Surg 2024; 28:77-87. [PMID: 38353080 DOI: 10.1016/j.gassur.2023.11.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/03/2023] [Revised: 10/14/2023] [Accepted: 10/25/2023] [Indexed: 02/16/2024]
Abstract
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.
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Affiliation(s)
- Grace C Bloomfield
- Georgetown University School of Medicine, Washington, District of Columbia, United States
| | - Aradhya Nigam
- Department of Surgery, Medstar Georgetown University Hospital, Washington, District of Columbia, United States
| | - Inochi Gonzalez Calvo
- Georgetown University School of Medicine, Washington, District of Columbia, United States
| | - C Scott Dorris
- Dahlgren Memorial Library, Georgetown University Medical Center, Washington, District of Columbia, United States
| | - Thomas M Fishbein
- MedStar Georgetown Transplant Institute, Medstar Georgetown University Hospital, Washington, District of Columbia, United States
| | - Pejman Radkani
- MedStar Georgetown Transplant Institute, Medstar Georgetown University Hospital, Washington, District of Columbia, United States
| | - Emily R Winslow
- University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin.
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Durgun AV, Ergün S, Turgut BC, Şimşek O, Velidedeoglu M, Sarıbeyoğlu K, Pekmezci S. Biliary cysts in adults: Cerrahpaşa experience. Turk J Surg 2023; 39:315-320. [PMID: 38694518 PMCID: PMC11057935 DOI: 10.47717/turkjsurg.2023.6285] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/30/2023] [Accepted: 12/16/2023] [Indexed: 05/04/2024]
Abstract
Objectives Biliary cysts are biliary duct dilatations, with 20% of the cysts being diagnosed in adulthood. Abdominal pain, jaundice and palpable abdominal mass are defined as the classical triad. However, nausea, vomiting, fever, itching and weight loss are frequent complaints. There are several treatment options depending on the type of the cyst. This study aimed to share our experience with biliary cysts and contribute to the literature on this subject. Material and Methods Thirty patients, who received treatment for biliary cyst from January 1981 to December 2018 at our clinic, were studied retrospectively. The patients were analyzed based on age, sex, type of the cyst, diagnosis and treatment methods, post-op follow up and complications. Results Twenty-seven of the patients were females, and three were males. The patients were aged between 16 and 76 years, and the median age was 41.9 years. All patients presented with abdominal pain, which was accompanied by cholangitis in nine patients, nausea and vomiting in four patients, dyspepsia in three patients and palpable mass in one patient. According to the Todani classification, biliary cyst findings were consistent with Type I in 23 patients, Type V in three patients, Type IV in two patients, Type II in one patient and Type III in one patient. Conclusion Diagnosis and treatment are complex in biliary cysts due to anatomical proximity and variations. Therefore, it would be beneficial to refer them to referral centers. Choice of treatment should be based on the type of the cyst.
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Affiliation(s)
- Ali Vedat Durgun
- Department of General Surgery, İstanbul University-Cerrahpaşa Cerrahpaşa Faculty of Medicine, İstanbul, Türkiye
| | - Sefa Ergün
- Department of General Surgery, İstanbul University-Cerrahpaşa Cerrahpaşa Faculty of Medicine, İstanbul, Türkiye
| | - Başar Can Turgut
- Department of General Surgery, İstanbul University-Cerrahpaşa Cerrahpaşa Faculty of Medicine, İstanbul, Türkiye
| | - Osman Şimşek
- Department of General Surgery, İstanbul University-Cerrahpaşa Cerrahpaşa Faculty of Medicine, İstanbul, Türkiye
| | - Mehmet Velidedeoglu
- Department of General Surgery, İstanbul University-Cerrahpaşa Cerrahpaşa Faculty of Medicine, İstanbul, Türkiye
| | - Kaya Sarıbeyoğlu
- Department of General Surgery, Charite University, Berlin, Germany
| | - Salih Pekmezci
- Department of General Surgery, İstanbul University-Cerrahpaşa Cerrahpaşa Faculty of Medicine, İstanbul, Türkiye
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Nikai K, Koga H, Suda K, Miyahara K, Lane GJ, Arakawa A, Fukumura Y, Saiura A, Hayashi Y, Nagakawa Y, Okazaki T, Takishita C, Yanai T, Yamataka A. Prospective evaluation of common hepatic duct histopathology at the time of choledochal cyst excision ranging from children to adults. Pediatr Surg Int 2023; 40:15. [PMID: 38032513 DOI: 10.1007/s00383-023-05589-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 10/31/2023] [Indexed: 12/01/2023]
Abstract
PURPOSE To evaluate common hepatic duct just distal to the HE anastomosis (d-CHD) prospectively for mucosal damage, inflammation, fibrosis, dysplasia, carcinoma in situ, malignant transformation, effects of serum amylase, and symptoms at presentation in CC cases ranging from children to adults. METHODS Cross-sections of d-CHD obtained at cyst excision 2018-2023 from 65 CC patients; 40 children (< 15 years old), 25 adults (≥ 15) were examined with hematoxylin and eosin, Ki-67, S100P, IMP3, p53, and Masson's trichrome to determine an inflammation score (IS), fibrosis score (FS), and damaged mucosa rate (DMR; damaged mucosa expressed as a percentage of the internal circumference). RESULTS Mean age at cyst excision ("age") was 18.2 years (range: 3 months-74 years). Significant inverse correlations were found for age and DMR (p = 0.002), age and IS (p = 0.011), and age and Ki-67 (p = 0.01). FS did not correlate with age (p = 0.32) despite significantly increased IS in children. Dysplasia was identified in a 4-month-old girl with cystic CC. Serum amylase was elevated in high DMR subjects. CONCLUSIONS High DMR, high IS, and evidence of dysplasia in pediatric CC suggest children are at risk for serious sequelae best managed by precise histopathology, protocolized follow-up, and awareness that premalignant histopathology can arise in infancy.
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Affiliation(s)
- Koki Nikai
- Department of Pediatric Surgery, Juntendo Urayasu Hospital, Chiba, Japan
| | - Hiroyuki Koga
- Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan.
| | - Kazuto Suda
- Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan
| | - Katsumi Miyahara
- Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan
| | - Geoffrey J Lane
- Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan
| | - Atsushi Arakawa
- Department of Human Pathology, Juntendo University School of Medicine, Tokyo, Japan
| | - Yuki Fukumura
- Department of Human Pathology, Juntendo University School of Medicine, Tokyo, Japan
| | - Akio Saiura
- Department of Hepatobiliary-Pancreatic Surgery, Juntendo University School of Medicine, Tokyo, Japan
| | - Yutaka Hayashi
- Department of Gastrointestinal and Pediatric Surgery, Tokyo Medical University, Tokyo, Japan
| | - Yuichi Nagakawa
- Department of Gastrointestinal and Pediatric Surgery, Tokyo Medical University, Tokyo, Japan
| | - Tadaharu Okazaki
- Department of Pediatric Surgery, Juntendo Urayasu Hospital, Chiba, Japan
| | - Chie Takishita
- Department of Gastrointestinal and Pediatric Surgery, Tokyo Medical University, Tokyo, Japan
- Department of Surgery, Toda Chuo General Hospital, Saitama, Japan
| | - Toshihiro Yanai
- Department of Pediatric Surgery, Ibaraki Children's Hospital, Ibaraki, Japan
| | - Atsuyuki Yamataka
- Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan
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Abstract
Choledochal cyst (CC) or congenital biliary dilatation, has a skewed distribution with hereditary features that is far more common in East Asian females. CC is usually associated with pancreaticobiliary malunion (PBMU) forming a common channel. CC requires early definitive diagnosis, since there is a risk for malignancy occurring in the CC and/or intrahepatic bile ducts (IHBD). Complete CC excision and Roux-en-Y hepaticoenterostomy is required and can be performed by open or minimally invasive surgery with hepatojejunostomy the recommended procedure of choice. Principles of open surgical intervention form the basis of minimally invasive management with laparoscopy and robotic assistance. Current surgical management is associated with fewer early and late complications, such as hepaticoenterostomy anastomotic leakage, cholangitis, anastomosis stricture, and cholangiocarcinoma. Specific features of CC management at Juntendo include: intraoperative endoscopy of the common channel and IHBD for inspecting and clearing debris to significantly reduce post-operative pancreatitis or stone formation; near infra-red fluorescence with indocyanine green for visualizing tissue planes especially during minimally invasive surgery for CC; and a classification system for CC based on PBMU that overcomes inconsistencies between existing classification systems and clinical presentation.
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Affiliation(s)
- Joel Cazares
- Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan
| | - Hiroyuki Koga
- Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan
| | - Atsuyuki Yamataka
- Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan.
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Pakkala AK, Nekarakanti PK, Nagari B, Bansal AK, Thumma V, Gunturi SV. An audit of Complicated Choledochal Cysts- 15-years' experience at a tertiary care center. Langenbecks Arch Surg 2023; 408:212. [PMID: 37247085 DOI: 10.1007/s00423-023-02952-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/21/2022] [Accepted: 05/21/2023] [Indexed: 05/30/2023]
Abstract
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.
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Affiliation(s)
- Amith Kumar Pakkala
- Department of Surgical Gastroenterology, Nizam's Institute of Medical Sciences, Hyderabad, India
| | - Phani Kumar Nekarakanti
- Department of Surgical Gastroenterology, Nizam's Institute of Medical Sciences, Hyderabad, India
| | - Bheerappa Nagari
- Department of Surgical Gastroenterology, Nizam's Institute of Medical Sciences, Hyderabad, India.
| | - Ashish Kumar Bansal
- Department of Surgical Gastroenterology, Nizam's Institute of Medical Sciences, Hyderabad, India
| | - VenuMadhav Thumma
- Department of Surgical Gastroenterology, Nizam's Institute of Medical Sciences, Hyderabad, India
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12
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Miron A, Popa LG, Toma EA, Calu V, Parvuletu RF, Enciu O. The Curious Case of the Choledochal Cyst—Revisiting the Todani Classification: Case Report and Review of the Literature. Diagnostics (Basel) 2023; 13:diagnostics13061059. [PMID: 36980367 PMCID: PMC10047054 DOI: 10.3390/diagnostics13061059] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/14/2023] [Revised: 03/03/2023] [Accepted: 03/07/2023] [Indexed: 03/14/2023] Open
Abstract
Choledochal cysts (CCs) are rare occurrences presenting as dilatations of biliary structures, which can present as single or multiple dilatations and can appear as both intra- and extrahepatic anomalies. The most widespread classification of CCs is the Todani classification, but there have been numerous reports of cysts that do not fall into any of the types described. We present such a case—a male patient 36 years of age who underwent preoperative CT, MRCP, and ERCP, which mistakenly indicated a type II Todani CC, and intraoperatively was found to be located at the confluence of the hepatic ducts and encompassed the origin of the common bile duct. Complete resection of the cyst and the proximal segment of the common bile duct was performed, and reconstruction was carried out by Roux-en-Y double-tutorized hepaticojejunostomy. Considering the risk of malignant transformation, the frequent preoperative misdiagnosis, as well as the technically challenging surgery required in such cases, we advocate for a revision of the classification and raise awareness of the need for guidelines regarding the proper short-term and long-term management of this disease to ensure adequate quality of life and disease-free survival for patients.
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Affiliation(s)
- Adrian Miron
- Faculty of Medicine, Carol Davila University of Medicine and Pharmacy, 050474 Bucharest, Romania (O.E.)
- Elias Emergency University Hospital, 011461 Bucharest, Romania
| | - Liliana Gabriela Popa
- Faculty of Medicine, Carol Davila University of Medicine and Pharmacy, 050474 Bucharest, Romania (O.E.)
- Elias Emergency University Hospital, 011461 Bucharest, Romania
| | - Elena Adelina Toma
- Faculty of Medicine, Carol Davila University of Medicine and Pharmacy, 050474 Bucharest, Romania (O.E.)
- Elias Emergency University Hospital, 011461 Bucharest, Romania
- Correspondence: ; Tel.: +40-213161600
| | - Valentin Calu
- Faculty of Medicine, Carol Davila University of Medicine and Pharmacy, 050474 Bucharest, Romania (O.E.)
- Elias Emergency University Hospital, 011461 Bucharest, Romania
| | | | - Octavian Enciu
- Faculty of Medicine, Carol Davila University of Medicine and Pharmacy, 050474 Bucharest, Romania (O.E.)
- Elias Emergency University Hospital, 011461 Bucharest, Romania
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13
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Farooq MA, Khan SA, Malik MI. Choledochal Cyst in Children, presentation and outcome - 10 years' experience from a tertiary care center in Pakistan. Pak J Med Sci 2023; 39:456-459. [PMID: 36950417 PMCID: PMC10025707 DOI: 10.12669/pjms.39.2.6196] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/10/2022] [Revised: 11/01/2022] [Accepted: 12/16/2022] [Indexed: 01/27/2023] Open
Abstract
Objectives This study was done to compare the clinical features, laboratory findings and surgical outcomes of pediatric patients with choledochal cysts. Methods Retrospective review of the hospital records of all pediatric patients admitted with choledochal cysts from 2011 to 2021 were collected and analyzed. Patients were divided into two groups; infant (less than one year age) and pediatric (1 to 16 years) for statistical comparison of two groups. Result The study included 34 children, 9 (26.5%) were infant (<1 year) and 25 (73.5%) were more than one year old. Mean age at diagnosis was 15 months with age ranging from 14 days to 16 years. Females were 19 (55.9%) and males were 15 (44.1%). Type-I choledochal cyst was the most common (73.5%), presentation followed by Type-IVA (26.5%) in our patients. Patients from infant group presented with jaundice in 7 (77.7%), and clay-colored stool in 3 (33.3%) as the most common clinical features, while abdominal pain (88%), vomiting (72%), fever (32%) and pancreatitis (32%) were the frequent presentations among older age group. Post-surgical complications of excision of choledochal cyst were observed in 4 (11.7%) patients. Conclusion Choledochal cysts have variable presentations depending upon age of the patients. Complete surgical excision of choledochal cyst is the treatment modality of choice and timely surgical management can prevent complications.
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Affiliation(s)
- Muhammad Arslan Farooq
- Dr. Muhammad Arslan Farooq, MBBS, FCPS (Pediatrics). Senior Registrar, Pediatrics, Shifa Tameer-e-Millat University (Shifa College of Medicine)/ Shifa International Hospital, Islamabad, Pakistan
| | - Sabeen Abid Khan
- Dr. Sabeen Abid Khan, MBBS, FCPS (Pediatrics). Associate Professor, Pediatrics, Shifa Tameer-e-Millat University (Shifa College of Medicine)/ Shifa International Hospital, Islamabad, Pakistan
| | - Munir Iqbal Malik
- Prof. Dr. Munir Iqbal Malik, MBBS, MD. Diplomat American Board of Pediatrics, Professor of Pediatrics, Shifa Tameer-e-Millat University (Shifa College of Medicine)/ Shifa International Hospital, Islamabad, Pakistan
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14
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Gao R, Ke M, Shi J, Zhang Y, Zou J, Diao M, Li L. Establishment and validation of a predictive nomogram for the risk of premalignant lesions in children with choledochal cyst. Front Pediatr 2023; 11:1108788. [PMID: 36816382 PMCID: PMC9936067 DOI: 10.3389/fped.2023.1108788] [Citation(s) in RCA: 5] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/26/2022] [Accepted: 01/16/2023] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Choledochal cyst (CDC) increases the risk (2.5%-30%) of malignancy. Metaplasia and dysplasia have been recognized as premalignant lesions among CDCs. This study aimed to evaluate the risk factors of metaplasia and dysplasia in CDC children. METHODS Two hundred and ten CDC children who underwent cyst excision and Roux-en-Y hepaticojejunostomy at our institution between July 2020 and November 2021 were included and randomly divided into the training set and validation set. Univariate and multivariate logistic regression analysis were used to identify independent risk factors of premalignant lesions in the training set and build a predictive nomogram. The performance and discriminatory abilities of the nomogram were further assessed and validated in the validation set. RESULTS Of the 210 CDC children, 78 (37.1%) patients developed premalignant lesions. Age (OR, 1.011, 95%CI, 1.000-1.022, P = 0.046), symptoms duration (OR, 1.021, 95%CI, 1.001-1.042, P = 0.036), cyst diameter (OR, 1.737, 95%CI, 1.328-2.273, P < 0.001), recurrent attacks of biliary pancreatitis (OR, 3.653, 95%CI, 1.205-11.076, P = 0.022), and biliary operation history (OR, 5.860, 95%CI, 1.268-27.084, P = 0.024) were identified as independent risk factors. Based on these predictors, a predictive nomogram was generated. The AUC of the nomogram was 0.873 in the training set and 0.793 in the validation set, indicating that it was robust and well calibrated. CONCLUSIONS A novel nomogram to the individualized risk of premalignant lesions in CDC children was successfully built, on the basis of age, symptoms duration, cyst diameter, recurrent attacks of biliary pancreatitis, and biliary operation history. This nomogram, combined with the final pathological results, can help clinicians to develop more efficient follow-up strategies for the high-risk children with CDC.
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Affiliation(s)
- Ruyue Gao
- Department of Pediatric Surgery, Children's Hospital Capital Institute of Pediatrics, Beijing, China.,Graduate School of Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - Meng Ke
- Department of Pediatric Surgery, Children's Hospital Capital Institute of Pediatrics, Beijing, China.,Graduate School of Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - Jie Shi
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yandong Zhang
- Department of Pathology, Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China
| | - Jizhen Zou
- Department of Pathology, Children's Hospital Capital Institute of Pediatrics, Beijing, China
| | - Mei Diao
- Department of Pediatric Surgery, Children's Hospital Capital Institute of Pediatrics, Beijing, China
| | - Long Li
- Department of Pediatric Surgery, Children's Hospital Capital Institute of Pediatrics, Beijing, China.,Department of Pediatric Surgery, Beijing Tsinghua Changgung Hospital, Beijing, China.,Research Unit of Minimally Invasive Pediatric Surgery on Diagnosis and Treatment (2021RU015), Chinese Academy of Medical Sciences, Beijing, China
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15
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Ishii J, Miyano G, Takahashi T, Ochi T, Miyake Y, Koga H, Seo S, Lane GJ, Fukumoto K, Arakawa A, Yamataka A. Oncologic safety of Carrel patch hepaticojejunostomy for treating cystic-type choledochal cyst in children based on 20-plus years follow-up. Pediatr Surg Int 2022; 39:65. [PMID: 36574035 DOI: 10.1007/s00383-022-05339-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 12/07/2022] [Indexed: 12/28/2022]
Abstract
AIM During choledochal cyst (CC) excision, the hepaticojejunostomy anastomosis (HJA) can be performed conventionally (CHJA) or with a Carrel patch (CPA). CPA can increase CHD diameter to 10-13 mm, preventing anastomotic stenosis and intrahepatic bile duct (IHBD) stones but may be at risk for malignant transformation. METHODS The medical records of 83 cystic-type CC with CHD ≤ 9 mm followed up for at least 20 years were reviewed retrospectively. Available excised CC specimens (70/83) were re-examined blindly for pre-malignant changes. A questionnaire about suturing narrow lumens was conducted. RESULTS All 83 had pancreaticobiliary maljunction. Group data were similar. Anastomoses were CPA (n = 43) and CHJA (n = 40). Mean diameter for CPA was 11.4 mm (range: 10-13 mm); for CHJA was 7.4 mm (range: 5-9 mm). Mean follow-up was 27.7 years (range: 20-42). Postoperative anastomotic stenoses were less after CPA: 1/43 (2.3%) versus 5/40 (12.5%) (p = 0.10), but CHJA had significantly more postoperative IHBD stones: 0% versus 4/40 (10.0%) (p < 0.05). All IHBD stone patients had anastomotic stenosis. Excised specimens showed no pre-malignant cytology. Lumen diameter ≤ 9 mm was considered challenging by 10/10 surgical trainees and ≤ 7 mm by 16/22 pediatric surgeons. CONCLUSIONS CPA appears to be oncologically safe because of the absence of malignant transformation for at least 20 years.
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Affiliation(s)
- Junya Ishii
- Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan
| | - Go Miyano
- Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan.
| | - Toshiaki Takahashi
- Department of Pediatric Surgery, Shizuoka Children's Hospital, Shizuoka, Japan
| | - Takanori Ochi
- Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan
| | - Yuichiro Miyake
- Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan
| | - Hiroyuki Koga
- Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan
| | - Shogo Seo
- Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan
| | - Geoffrey J Lane
- Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan
| | - Koji Fukumoto
- Department of Pediatric Surgery, Shizuoka Children's Hospital, Shizuoka, Japan
| | - Atsushi Arakawa
- Department of Human Pathology, Juntendo University School of Medicine, Tokyo, Japan
| | - Atsuyuki Yamataka
- Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan
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16
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Treatment and follow-up of late onset intra hepatic bile duct stones in congenital biliary dilatation. Pediatr Surg Int 2022; 39:20. [PMID: 36449153 DOI: 10.1007/s00383-022-05321-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 11/23/2022] [Indexed: 12/05/2022]
Abstract
PURPOSE The postoperative course after surgery for congenital biliary dilatation (CBD) has some complications. Intrahepatic bile duct (IHBD) stones were known as a late complication. We report on the treatment and long-term follow-up of postoperative IHBD stones in our department. METHODS Patients who underwent CBD surgery at age 15 years or younger in our department were identified. Those followed up for 5 years or more were enrolled. Annual blood chemistry tests and abdominal ultrasonography were performed. Each patient's surgical procedure, IHBD stone diagnosis, treatments, and outcomes were retrospectively assessed. RESULTS Fifty-one patients were analyzed. The median age at the last visit was 24 years (range 7-45 years), and the median age at CBD surgery was 3 years. Eight patients (16%) developed late-onset IHBD stones. The median age at onset was 25 years, and the median duration after surgery was 20 years. The initial treatment was double-balloon enteroscopy (DBE) in 4 cases, which resulted in stone removal in 3 of the 4 patients (75%). CONCLUSION Since CBD may cause late-onset IHBD stones, continuous follow-up is required even in adulthood. In this study, DBE was effective and minimally invasive, and it is recommended as the initial treatment.
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17
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Yaghi M, Jaafar R, Kanso M, Khalife M, Faraj W. Choledochal anomalies in adults: A 20-year single-center retrospective cohort experience in the Middle East. Arab J Gastroenterol 2022; 23:235-240. [PMID: 36371373 DOI: 10.1016/j.ajg.2022.10.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/04/2021] [Revised: 03/31/2022] [Accepted: 10/14/2022] [Indexed: 11/11/2022]
Abstract
BACKGROUND AND STUDY AIMS Choledochal cysts are rare congenital cystic dilatations of the bile ducts that occur in fewer than 1% of individuals. The disease is common in East Asia, and most of the literature concerns those populations, but some data about Western populations have been published recently. Long-term reports about the disease in Middle Eastern populations, however, are currently lacking. We report a single-center 20-year experience in diagnosing and managing choledochal anomalies. PATIENTS AND METHODS Participants were adult patients in whom choledochal cysts were diagnosed over a 20-year (2000-2019) period at a single tertiary academic care center. Clinical data, including radiologic imaging findings, were retrieved from the patients' medical records. To describe the baseline characteristics of the population, we calculated descriptive statistics. RESULTS Choledochal anomalies were diagnosed in 19 adult patients, whose median age was 30 years (interquartile range [IQR], 23-67 years). Of the choledochal cysts 13 (68.4%) were classified as Todani type I, 4 (21.1%) as Todani type IV, and 3 (15.8%) as Todani type V (Caroli's disease). No patient had underlying chronic liver disease, and liver synthetic function was preserved in all. Eighteen patients (94.7%) underwent surgery: cyst excision with Roux-en-Y hepaticojejunostomy in 17 and liver transplantation in 1. All 18 survived surgery, and the median postoperative hospital stay was 11 days (IQR, 5-34 days). All 18 were alive 90 days after surgery, and the median follow-up period was 40 months (IQR, 12-140 months). Seven patients (36.8%) developed postoperative surgical complications; 2 patients required rehospitalization, and 1 required reoperation. CONCLUSION This description of adults with choledochal cysts is the latest long-term report about this disease in the Middle East. In our 20-year experience, the disease characteristics in our patients were moderately consistent with those described previously.
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Affiliation(s)
- Marita Yaghi
- Liver Transplantation and Hepatopancreaticobiliary Surgery, Department of General Surgery, American University of Beirut, Lebanon
| | - Rola Jaafar
- Liver Transplantation and Hepatopancreaticobiliary Surgery, Department of General Surgery, American University of Beirut, Lebanon
| | - Mariam Kanso
- Liver Transplantation and Hepatopancreaticobiliary Surgery, Department of General Surgery, American University of Beirut, Lebanon
| | - Mohamed Khalife
- Liver Transplantation and Hepatopancreaticobiliary Surgery, Department of General Surgery, American University of Beirut, Lebanon
| | - Walid Faraj
- Liver Transplantation and Hepatopancreaticobiliary Surgery, Department of General Surgery, American University of Beirut, Lebanon.
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Cai Q, Yu SZ, Yu ZY. Progress in research of pancreaticobiliary maljunction and biliary dilatation. Shijie Huaren Xiaohua Zazhi 2022; 30:498-503. [DOI: 10.11569/wcjd.v30.i11.498] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Pancreaticobiliary maljunction (PBM) is a congenital anomaly that is defined as a junction of the pancreatic and bile ducts located outside the duodenal wall during the embryonic stage, resulting in bile and pancreatic juice reflux and corresponding clinical symptoms. PBM is a high risk factor for cholangiocarcinoma. PBM can occur with or without biliary dilatation (BD). Early diagnosis of PBM can prevent complications. Ultrasonography, magnetic resonance imaging, multi-slice spiral computed tomography, endoscopic ultrasonography, endoscopic retrograde cholangiopancreatography, and cholangiopancreatography are important modalities for diagnosis of this disease. The standard surgical procedure for PBM with BD is dilated cholangiectomy and choledochoenteroreconstruction. Cholecystectomy should be performed as early as possible for PBM without BD.
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Affiliation(s)
- Qiang Cai
- Department of Hepatobiliary Surgery, Affiliated Hospital of Yunnan University, Kunming 650021, Yunnan Province, China
| | - Shi-Zhe Yu
- Department of Hepatobiliary Surgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, Henan Province, China
| | - Zhi-Yong Yu
- Department of Hepatobiliary Surgery, Affiliated Hospital of Yunnan University, Kunming 650021, Yunnan Province, China
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19
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Koea J, O'Grady M, Agraval J, Srinivasa S. Defining an optimal surveillance strategy for patients following choledochal cyst resection: results of a systematic review. ANZ J Surg 2022; 92:1356-1364. [PMID: 35579057 DOI: 10.1111/ans.17775] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/04/2021] [Revised: 04/26/2022] [Accepted: 05/03/2022] [Indexed: 11/29/2022]
Abstract
BACKGROUND Choledochal cysts should be treated with complete surgical resection, or, rarely liver transplantation. Treated patients can remain at risk of developing metachronous cholangiocarcinomas and lifelong follow up is indicated. However, there is no agreement on what constitutes an acceptable follow up strategy. This review was undertaken to develop an evidenced based surveillance strategy. METHODS A systematic review of MEDLINE, EMBASE, PubMed, Web of Science, and Google Scholar was undertaken for reports (published up to 10 September 2021) describing late biliary complications and development of metachronous cholangiocarcinoma following choledochal cyst resection. RESULTS Twenty-five publications described 74 metachronous cholangiocarcinomas occurring in 3911 patients (overall incidence 2%). Cancers developed commonly at the hepatic hilus and were diagnosed after a median interval of 92 months (range 9-249 months) after the initial resection. While reporting is incomplete, the majority of cholangiocarcinomas developed following resection of type I and type IV cysts with few metachronous cancers recorded after treatment of type II or III cysts. Peak age range for presentation with metachronous cholangiocarcinoma is in the twenties following cyst resection in childhood suggesting that patients are at greatest risk for metachronous tumour development for up to 20 years (240 months). CONCLUSION A surveillance strategy is proposed for patients treated primarily for cyst types I and IV and unresected type V using annual liver function tests, Ca 19-9 measurement and biannual ultrasound assessment for 20 years post cyst resection, with biannual liver function testing, Ca 19-9 measurement and three yearly ultrasound assessment thereafter.
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Affiliation(s)
- Jonathan Koea
- Department of Surgery, North Shore Hospital, Auckland, New Zealand
| | - Michael O'Grady
- Department of Surgery, Whanganui Hospital, Whanganui, New Zealand
| | - Jash Agraval
- Department of Radiology, North Shore Hospital, Auckland, New Zealand
| | - Sanket Srinivasa
- Department of Surgery, North Shore Hospital, Auckland, New Zealand
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20
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Chen S, Lin Y, Xu D, Lin J, Zeng Y, Li L. Da vinci robotic-assisted treatment of pediatric choledochal cyst. Front Pediatr 2022; 10:1044309. [PMID: 36440346 PMCID: PMC9683341 DOI: 10.3389/fped.2022.1044309] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/14/2022] [Accepted: 10/11/2022] [Indexed: 11/07/2022] Open
Abstract
OBJECTIVE To evaluate the advantages and disadvantages of da Vinci robot and laparoscopy in treating pediatric choledochal cysts. METHODS We retrospectively analyzed clinical data from forty-two children diagnosed with choledochal cysts in our hospital from January 2018 to January 2021. Twenty children underwent da Vinci robotic surgery, and the others underwent traditional laparoscopy. We compared differences in general information and preoperative, intraoperative, and postoperative differences between the two surgical groups. RESULTS There was no statistically significant difference in age, gender, weight, type, maximum cyst diameter, preoperative C-reactive protein (CRP) value, postoperative complication rate, and postoperative pain score between the two surgical groups (P > 0.05). The average age of the robot-assisted group was 3.62 ± 0.71 years old (range = 1-12 years). There were nineteen cases of Todani type I, one patients of other types, and the maximum cyst diameter was 35.45 ± 9.32 mm (range = 12-56 mm). In the laparoscopic group, the average age was 3.08 ± 0.82 years old (range = 3-10 years). Twenty-one patients had Todani type I, and one had other types. The maximum cyst diameter was 31.94 ± 8.64 mm (range = 10-82 mm). The robot-assisted group had better abdominal drainage, postoperative CRP value, fasting time, and discharge time than the laparoscopic group (P < 0.05). CONCLUSION Compared with laparoscopy, the da Vinci system has the advantages of less tissue damage, faster recovery, and better healing in the treatment of children with congenital choledochal cysts. With technological advancements and an increased number of experienced surgeons, robotic surgery may become a new trend in surgery.
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Affiliation(s)
- Shan Chen
- Clinical Laboratory Department, Fuzhou Second Hospital Affiliated to Xiamen University, Fuzhou, China
| | - Yang Lin
- Department of Pediatric Surgery, Shengli Clinical Medical College, Fujian Medical University, Fuzhou, China
| | - Di Xu
- Department of Pediatric Surgery, Shengli Clinical Medical College, Fujian Medical University, Fuzhou, China
| | - Jianli Lin
- Laboratory Provincial Clinical Medical College, Fujian Medical University, Fuzhou, China
| | - Yunlong Zeng
- Department of Pediatric Surgery, Shengli Clinical Medical College, Fujian Medical University, Fuzhou, China
| | - Lizhi Li
- Department of Pediatric Surgery, Shengli Clinical Medical College, Fujian Medical University, Fuzhou, China
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Ranucci G, Della Corte C, Alberti D, Bondioni MP, Boroni G, Calvo PL, Cananzi M, Candusso M, Clemente MG, D'Antiga L, Degrassi I, De Ville De Goyet J, Di Dato F, Di Giorgio A, Vici CD, Ferrari F, Francalanci P, Fuoti M, Fusaro F, Gaio P, Grimaldi C, Iascone M, Indolfi G, Iorio R, Maggiore G, Mandato C, Matarazzo L, Monti L, Mosca F, Nebbia G, Nuti F, Paolella G, Pinon M, Roggero P, Sciveres M, Serranti D, Spada M, Vajro P, Nicastro E. Diagnostic approach to neonatal and infantile cholestasis: A position paper by the SIGENP liver disease working group. Dig Liver Dis 2022; 54:40-53. [PMID: 34688573 DOI: 10.1016/j.dld.2021.09.011] [Citation(s) in RCA: 13] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/03/2021] [Revised: 08/23/2021] [Accepted: 09/12/2021] [Indexed: 12/11/2022]
Abstract
Neonatal and infantile cholestasis (NIC) can represent the onset of a surgically correctable disease and of a genetic or metabolic disorder worthy of medical treatment. Timely recognition of NIC and identification of the underlying etiology are paramount to improve outcomes. Upon invitation by the Italian National Institute of Health (ISS), an expert working grouped was formed to formulate evidence-based positions on current knowledge about the diagnosis of NIC. A systematic literature search was conducted to collect evidence about epidemiology, etiology, clinical aspects and accuracy of available diagnostic tests in NIC. Evidence was scored using the GRADE system. All recommendations were approved by a panel of experts upon agreement of at least 75% of the members. The final document was approved by all the panel components. This position document summarizes the collected statements and defines the best-evidence diagnostic approach to cholestasis in the first year of life.
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Affiliation(s)
- Giusy Ranucci
- Department of Pediatrics, Santobono-Pausilipon Children's Hospital AORN, Naples, Italy
| | - Claudia Della Corte
- Hepatology, Gastroenterology, Nutrition and Liver transplantation Unit, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
| | | | - Maria Pia Bondioni
- Pediatric Radiology, University of Brescia, ASST Spedali Civili di Brescia, Brescia, Italy
| | | | - Pier Luigi Calvo
- Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital, Azienda Ospedaliera-Universitaria Città della Salute e della Scienza, Turin, Italy
| | - Mara Cananzi
- Unit of Gastroenterology, Digestive Endoscopy, Hepatology and Care of Children with Liver Transplantation, University Hospital of Padova, Padova, Italy
| | - Manila Candusso
- Hepatology, Gastroenterology, Nutrition and Liver transplantation Unit, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
| | - Maria Grazia Clemente
- Department of Medical, Surgical and Experimental Sciences, University of Sassari, Sassari, Italy
| | - Lorenzo D'Antiga
- Hepatology, Gastroenterology and Transplantation Unit, Hospital Papa Giovanni XXIII, Bergamo, Italy
| | - Irene Degrassi
- Service of Paediatric Hepatology, Department of Paediatrics, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
| | - Jean De Ville De Goyet
- Pediatric Department for the Treatment and Study of Abdominal Diseases and Abdominal Transplantation, ISMETT UPMC, Palermo, Italy
| | - Fabiola Di Dato
- Department di Translational Medical Science, School of Medicine and Surgery, University of Naples Federico II, Naples, Italy
| | - Angelo Di Giorgio
- Hepatology, Gastroenterology and Transplantation Unit, Hospital Papa Giovanni XXIII, Bergamo, Italy
| | - Carlo Dionisi Vici
- Division of Metabolism and Metabolic Diseases Research Unit, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
| | | | - Paola Francalanci
- Department of Pathology, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
| | - Maurizio Fuoti
- Pediatric Gastroenterology and Endoscopy Unit Children's Hospital, ASST Spedali Civili di Brescia, Brescia, Italy
| | - Fabio Fusaro
- Department of Medical and Surgical Neonatology, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
| | - Paola Gaio
- Unit of Gastroenterology, Digestive Endoscopy, Hepatology and Care of Children with Liver Transplantation, University Hospital of Padova, Padova, Italy
| | - Chiara Grimaldi
- Division of Abdominal Transplantation and Hepatobiliopancreatic Surgery, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
| | - Maria Iascone
- Medical Genetics Laboratory, Hospital Papa Giovanni XXIII, Bergamo, Italy
| | - Giuseppe Indolfi
- Department of Pediatrics, Meyer Children's University Hospital, Florence, Italy
| | - Raffaele Iorio
- Department di Translational Medical Science, School of Medicine and Surgery, University of Naples Federico II, Naples, Italy
| | - Giuseppe Maggiore
- Hepatology, Gastroenterology, Nutrition and Liver transplantation Unit, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
| | - Claudia Mandato
- Department of Pediatrics, Santobono-Pausilipon Children's Hospital AORN, Naples, Italy
| | | | - Lidia Monti
- Department of Radiology, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
| | - Fabio Mosca
- Neonatal Intensive Care Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
| | - Gabriella Nebbia
- Service of Paediatric Hepatology, Department of Paediatrics, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
| | - Federica Nuti
- Service of Paediatric Hepatology, Department of Paediatrics, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
| | - Giulia Paolella
- Service of Paediatric Hepatology, Department of Paediatrics, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
| | - Michele Pinon
- Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital, Azienda Ospedaliera-Universitaria Città della Salute e della Scienza, Turin, Italy
| | - Paola Roggero
- Neonatal Intensive Care Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
| | - Marco Sciveres
- Pediatric Hepatology and Liver Transplantation, ISMETT UPMC, Palermo, Italy
| | - Daniele Serranti
- Department of Pediatrics, Meyer Children's University Hospital, Florence, Italy
| | - Marco Spada
- Division of Abdominal Transplantation and Hepatobiliopancreatic Surgery, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
| | - Pietro Vajro
- Department of Medicine, Surgery and Dentistry, "Scuola Medica Salernitana" University of Salerno, Baronissi, Italy
| | - Emanuele Nicastro
- Hepatology, Gastroenterology and Transplantation Unit, Hospital Papa Giovanni XXIII, Bergamo, Italy
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Han WS, Kim H, Sohn HJ, Lee M, Kang YH, Kim HS, Han Y, Kang JS, Kwon W, Jang JY. Clinical characteristics of patients with malignancy and long-term outcomes of surgical treatment of patients with choledochal cyst. Ann Surg Treat Res 2021; 101:332-339. [PMID: 34934760 PMCID: PMC8651987 DOI: 10.4174/astr.2021.101.6.332] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/28/2021] [Revised: 10/12/2021] [Accepted: 10/26/2021] [Indexed: 11/30/2022] Open
Abstract
Purpose There are few reports of postoperative long-term malignant risk or postoperative sequelae after surgery for choledochal cysts (CCs). This study aimed to analyze the clinical characteristics of patients with malignancy and the long-term results of operated CC. Methods The patients who underwent surgical treatments for CC between 2003 and 2020 at Seoul National University Hospital were enrolled. Clinicopathologic factors and pre-/postoperative computed tomography or magnetic resonance imaging were reviewed. Results Of the 153 patients, Todani classification Ic (36.6%), C-P type (43.8%) anomalous pancreaticobiliary duct union were the most common type respectively. Fourteen patients (9.2%) had biliary tract cancer and a comparison of patients with and without malignancy showed that the diameter of cyst was significantly lower in malignant patients and malignancy was observed to be significantly higher in P-C type. The incidence of long-term complications was 9.8%, and the median time interval was 30 months. The 2 most common complications were cholangitis and stricture (60.0%). There was one case of new cancer near the intrapancreatic remnant bile duct. Conclusion Of the resected CCs, 9.2% had a combined malignancy on the biliary tracts. Long-term complications such as cholangitis, anastomotic stricture, and new cancers may occur. Therefore, continuous surveillance is required.
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Affiliation(s)
- Wung Sun Han
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, Seoul National University College of Medicine, Seoul, Korea
| | - Hongbeom Kim
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, Seoul National University College of Medicine, Seoul, Korea
| | - Hee Ju Sohn
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, Seoul National University College of Medicine, Seoul, Korea
| | - Mirang Lee
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, Seoul National University College of Medicine, Seoul, Korea
| | - Yoon Hyung Kang
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, Seoul National University College of Medicine, Seoul, Korea
| | - Hyeong Seok Kim
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, Seoul National University College of Medicine, Seoul, Korea
| | - Youngmin Han
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, Seoul National University College of Medicine, Seoul, Korea
| | - Jae-Seung Kang
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, Seoul National University College of Medicine, Seoul, Korea
| | - Wooil Kwon
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, Seoul National University College of Medicine, Seoul, Korea
| | - Jin-Young Jang
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, Seoul National University College of Medicine, Seoul, Korea
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Wu X, Li BL, Zheng CJ, He XD. Risk factors for preoperative carcinogenesis of bile duct cysts in adults. World J Clin Cases 2021; 9:6278-6286. [PMID: 34434994 PMCID: PMC8362564 DOI: 10.12998/wjcc.v9.i22.6278] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/11/2021] [Revised: 05/06/2021] [Accepted: 05/19/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Bile duct cyst (BDC) is a rare congenital bile duct malformation. The incidence of bile duct malignancy in BDC patients is markedly higher than that in the general population. However, few studies have been conducted on the risk factors for preoperative carcinogenesis in BDC patients. AIM To analyze the risk factors associated with preoperative carcinogenesis in BDC patients. METHODS The medical records of BDC patients treated at our hospital between January 2012 and December 2018 were retrospectively reviewed. We constructed a database and compared the characteristics of BDC patients with dysplasia and carcinoma against those with benign cysts. The risk factors for preoperative carcinogenesis were identified using univariate and multivariate analyses. RESULTS The cohort comprised 109 BDC patients. Ten patients had preoperative dysplasia or adenocarcinoma. Univariate and multivariate analyses showed that gallbladder wall thickness > 0.3 cm [odds ratio (OR), 6.551; 95% confidence interval (CI), 1.351 to 31.763; P = 0.020] and Todani type IV (OR, 7.675; 95%CI, 1.584 to 37.192; P = 0.011) were independent factors associated with preoperative carcinogenesis. CONCLUSION BDC is a premalignant condition. Our findings show that gallbladder wall thickness > 0.3 cm and Todani type IV are independent risk factors for preoperative carcinogenesis of BDC. They are therefore useful for deciding on the appropriate treatment strategy, especially in asymptomatic patients.
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Affiliation(s)
- Xin Wu
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
| | - Bing-Lu Li
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
| | - Chao-Ji Zheng
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
| | - Xiao-Dong He
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
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Abstract
OBJECTIVE The aim of the study was to assess long-term morbidity in children operated for choledochal malformation (CM) by relating clinical complications to liver histopathology, follow-up imaging, liver stiffness, and biochemistry. METHODS A single-center retrospective follow-up study including all CM patients (n = 55, 71% girls) treated during 1976 to 2018 was performed. Mann-Whitney U test and Spearman rank correlation were used for statistical analyses. RESULTS During median follow-up of 5.8 (interquartile range, 2.5-12) years, 1 patient was lost to follow-up whereas all survived. Intraoperative liver biopsies showed fibrosis in 32%, and patients with Metavir stage ≥2 were younger at surgery (0.36 [0.11-1.9] vs 3.8 [0.72-10.5] years, P = 0.024) than those without fibrosis. Overall, 21% had long-term complications including cholangitis in 9 (>2 episodes in 5) patients, anastomotic stricture in 2 referred patients and adhesive volvulus or hepatocellular carcinoma in 1 each. Anastomotic strictures were successfully managed nonoperatively and hepatocellular carcinoma with thermoablation. In postoperative magnetic resonance cholangiography (MRCP) performed 6.4 (3.6-16) years after hepaticojejunostomy, diameters of both main intrahepatic ducts had decreased significantly to 3.0 (2.5-3.5) mm (P = 0.0001) but a distal cyst stump was remaining in 30% with a length of 6.0 (4.0-20) mm that associated with operation age (r = 0.71, P = 0.015) and fusiform CM type. Follow-up ultrasound revealed mild dilation of intrahepatic bile ducts in 6.3% and mildly to moderately elevated liver biochemistry in 23%, and liver stiffness (>7 kPa) in 22%. CONCLUSIONS Whilst cholangitis was the most common postoperative problem, individual patients experienced other more significant complications and one quarter of patients showed evidence of underlying liver dysfunction.
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25
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Razumovsky AY, Shubin NV. [Surgical treatment of common bile duct malformations in children]. Khirurgiia (Mosk) 2021:92-97. [PMID: 33759476 DOI: 10.17116/hirurgia202104192] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
The review is devoted to the current classifications of common bile duct malformations, authors' opinion regarding surgical approach and reconstruction of the bile ducts. The authors describe the modern modifications of bile duct reconstructions and estimate their effectiveness. PubMed database, Google Scholar and National Scientific Electronic Library eLIBRARY.ru databases were used. We analyzed the complications in children depending on various reconstructions, anatomical form, timing of reconstructive surgery and early diagnosis of this pathology.
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Affiliation(s)
- A Yu Razumovsky
- Pirogov Russian National Research Medical University, Moscow, Russia.,Filatov Children's City Clinical Hospital, Moscow, Russia
| | - N V Shubin
- Pirogov Russian National Research Medical University, Moscow, Russia.,Filatov Children's City Clinical Hospital, Moscow, Russia
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26
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Uecker M, Lehmann U, Braubach P, Schukfeh N, Madadi-Sanjani O, Ure BM, Petersen C, Kuebler JF. Choledochal Cysts Resected during Childhood Show No Mutations of KRAS and BRAF as Early Markers of Malignancy in Cholangiocytes. Eur J Pediatr Surg 2021; 31:20-24. [PMID: 32820496 DOI: 10.1055/s-0040-1715610] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/25/2022]
Abstract
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.
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Affiliation(s)
- Marie Uecker
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
| | - Ulrich Lehmann
- Department of Pathology, Hannover Medical School, Hannover, Germany
| | - Peter Braubach
- Department of Pathology, Hannover Medical School, Hannover, Germany
| | - Nagoud Schukfeh
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
| | | | - Benno M Ure
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
| | - Claus Petersen
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
| | - Joachim F Kuebler
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
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Ravichandran A, Pichaimuthu A, Arcot R. Laparoscopic excision of a Type II choledochal cyst with cystolithiasis. J Minim Access Surg 2021; 17:259-261. [PMID: 33723185 PMCID: PMC8083736 DOI: 10.4103/jmas.jmas_181_20] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Choledochal cysts can present with abdominal pain, jaundice and stones.MRI is the standard imaging tool and the type of biliary enteric anastomosis depends on the cyst type.
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Affiliation(s)
- Arihanth Ravichandran
- Department of General Surgery, Saveetha Institute of Medical and Technical Sciences, Saveetha Medical College, Chennai, Tamil Nadu, India
| | - Anbalagan Pichaimuthu
- Department of General Surgery, Saveetha Institute of Medical and Technical Sciences, Saveetha Medical College, Chennai, Tamil Nadu, India
| | - Rekha Arcot
- Department of General Surgery, Saveetha Institute of Medical and Technical Sciences, Saveetha Medical College, Chennai, Tamil Nadu, India
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De novo somatic mutations and KRAS amplification are associated with cholangiocarcinoma in a patient with a history of choledochal cyst. J Pediatr Surg 2020; 55:2657-2661. [PMID: 32295706 PMCID: PMC7942710 DOI: 10.1016/j.jpedsurg.2020.03.008] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/10/2019] [Revised: 02/26/2020] [Accepted: 03/02/2020] [Indexed: 02/07/2023]
Abstract
BACKGROUND/PURPOSE Choledochal cysts are congenital dilations of the bile ducts, and are associated with an increased risk of malignant transformation. The purpose of this study is to report the outcomes of a large series of patients with choledochal cysts and to highlight our analysis of one patient who developed malignancy after cyst resection. METHODS We conducted a retrospective review of patients <18 years of age with a choledochal cyst who underwent surgical resection between 1995 and 2018. Molecular testing of resected choledochal cyst specimens using the UCSF500 gene panel was performed on three patients including a 3-month-old boy and a 7-year-old girl who have remained cancer-free, and a 16-year-old girl who subsequently developed cholangiocarcinoma less than two years after resection. RESULTS One patient of the 48 included in our study developed cholangiocarcinoma after choledochal cyst resection. We observed de novo somatic mutations in TP53 and RBM10, and KRAS amplification in this patient's tumor. CONCLUSIONS In our series, the rate of malignancy after choledochal cyst resection was low. One patient developed de novo mutations in the remnant bile ducts after cyst resection. While it is a rare occurrence, the risk of malignancy following cyst resection supports the need for lifelong surveillance. LEVEL OF EVIDENCE IV.
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Omata K, Ishimaru T, Nakazawa A, Mitani Y, Mori M, Akamatsu N, Koh K, Kawashima H, Hasegawa K. Intrahepatic cholangiocarcinoma developing seven years after resection of congenital biliary dilatation. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2020; 63:101693. [DOI: 10.1016/j.epsc.2020.101693] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/23/2022] Open
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Kronfli R, Davenport M. Insights into the pathophysiology and classification of type 4 choledochal malformation. J Pediatr Surg 2020; 55:2642-2646. [PMID: 32561173 DOI: 10.1016/j.jpedsurg.2020.05.017] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/01/2019] [Revised: 05/07/2020] [Accepted: 05/11/2020] [Indexed: 11/26/2022]
Abstract
INTRODUCTION Type 4 choledochal malformations (CMs) can be defined as extra- and intrahepatic biliary dilatation. They are of uncertain etiology but make up about 20% of most series. The aim of this study was to investigate the pathophysiology and their natural history following surgical intervention. METHODS Ambispective review of a single-center series of type 4 CM between 1996 and 2017. Perioperative imaging, intraoperative pressure monitoring, bile amylase, and long-term follow-up data were analyzed based on extrahepatic morphology [(cystic (C), fusiform (F)]. Data were expressed as median (range). Statistical analysis was performed with nonparametric tests. P < 0.05 was considered significant. RESULTS 37 children had type 4 CM in the study period [age at surgery 4 (0.2-16) years] and could be further subdivided into cystic (4C) (n = 22) or fusiform (4F) (n = 15). There was significantly greater dilatation of the extrahepatic component in the cystic group [30 (11-94) versus 15 (8-90) mm; P = 0.0002] though there was no difference in left duct diameter [cystic 7.5 (0-17) mm versus fusiform 7.5 (3-16) mm; P = 0.86]. There was a trend to higher choledochal pressure in the cystic group [19 (4-40) versus 9 (6-25) mmHg; P = 0.09] and those in the fusiform group had higher bile amylase [8650 (3-890,000) versus 592 (1-123,000) IU/L; P = 0.01] and were older [4.1 (0.92-16.43) versus 2.4 (0.15-15.48) years; P = 0.03]. Children with type 4 CM were then separated simply on the basis of bile amylase into LOW (characterized by high pressure, cystic morphology and young age at surgery) and HIGH bile amylase (low pressure, fusiform morphology and older at time of surgery). CONCLUSIONS We propose further division of type 4 CM into 4C and 4F on clinical and pathophysiological grounds. LEVEL OF EVIDENCE II (prospective cohort).
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Affiliation(s)
- Rania Kronfli
- Department of Paediatric Surgery, Kings College Hospital, London SE5 9RS.
| | - Mark Davenport
- Department of Paediatric Surgery, Kings College Hospital, London SE5 9RS.
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31
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Wang X, Gao K, Yan C, Guo C. Short- and intermediate-term evaluation of the initial definitive operation for perforated choledochal cysts compared to two-stage management. Eur J Trauma Emerg Surg 2020; 48:1129-1135. [PMID: 33245366 DOI: 10.1007/s00068-020-01553-4] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/12/2020] [Accepted: 11/10/2020] [Indexed: 01/20/2023]
Abstract
BACKGROUND Recently, single definitive hepaticojejunostomy has been proposed to replace two-stage procedures, including initial urgent biliary drainage and final biliary reconstruction, with a reduced hospitalization time and corresponding overall hospitalization cost. We aimed to investigate the safety and efficacy of the initial definitive operation compared with the two-stage approach. METHODS The medical records of patients with perforated CDCs managed between 2010 and 2017 were retrospectively reviewed. The qualified samples were divided according to a single definitive operation or two-stage approach. We conducted a comparison of the clinical characteristics, including surgical parameters, length of stay, and short- and intermediate-term complications. RESULTS A total of 117 patients with perforated CDCs were reviewed, with 48 cases of single-stage management and 69 cases of two-stage management. No differences in the baseline characteristics between the two groups were found, such as age, sex distribution, ultrasound presentation, or laboratory findings. The initial definitive operation was associated with a lower total duration of drainage, including abdominal drainage and biliary drainage (p < 0.001), and rate of unplanned readmission (OR, 0.056; 95% CI 0.007-0.435; p < 0.001) than those who underwent two-stage management. Furthermore, the initial definitive operation significantly reduced the postoperative hospital stay (p < 0.001), and the overall hospitalization cost was accordingly reduced compared with two-stage management (p < 0.001). CONCLUSIONS The initial single definitive operation was safe and effective for most of the patients with perforated CDCs when expertise was available. Therefore, a single definitive operation should be considered the treatment of choice for perforated CDCs.
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Affiliation(s)
- Xu Wang
- Department of Pediatric General Surgery, Children's Hospital, Chongqing Medical University, Chongqing, 400014, People's Republic of China
| | - Kai Gao
- Department of Pediatric General Surgery, Children's Hospital, Chongqing Medical University, Chongqing, 400014, People's Republic of China
| | - Chengwei Yan
- Department of Pediatric General Surgery, Chongqing Three Gorges Central Hospital, Chongqing, 400014, People's Republic of China
| | - Chunbao Guo
- Department of Ultrasound, The Affiliated Hospital of Medical College, Qingdao University, Qingdao, Chongqing, People's Republic of China. .,Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Center for Child Health and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, Chongqing Engineering Research Center of Stem Cell Therapy, Children's Hospital of Chongqing Medical University, Chongqing, 400014, People's Republic of China. .,Department of Pediatric General Surgery and Liver Transplantation, Children's Hospital of Chongqing Medical University, 136 Zhongshan 2nd Rd., Chongqing, 400014, People's Republic of China.
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32
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Guan H, Chen R, Li D, Hoosen R, Xie S, Chen C, Jin S. Potential Risk Factors and Prognostic Evaluation of Malignant Changes Following Congenital Choledochal Cyst: a Retrospective Analysis. Indian J Surg 2020. [DOI: 10.1007/s12262-020-02138-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023] Open
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Ha S, Hwang S, Ryu LN. Development of intrahepatic cholangiocarcinoma at the remnant intrahepatic cyst portion 10 years after resection of type IV choledochal cyst. Ann Hepatobiliary Pancreat Surg 2020; 24:366-372. [PMID: 32843607 PMCID: PMC7452807 DOI: 10.14701/ahbps.2020.24.3.366] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/08/2020] [Revised: 06/10/2020] [Accepted: 06/11/2020] [Indexed: 11/27/2022] Open
Abstract
Complete resection of Todani type IV choledochal cyst (CC) is not possible, because the intrahepatic portion is not resectable. We present a case of intrahepatic cholangiocarcinoma that arose from the remnant CC portion that was located within the liver 10 years after resection. A 59-year-old female patient had undergone resection of type IV CC 10 years ago, leaving large remnant portions of CC at the liver and pancreas. Two and four years after resection of the extrahepatic CC, cholangitis with intrahepatic stones developed hence these episodes were treated with percutaneous transhepatic cholangioscopy. Ten years after the first operation, intrahepatic stones and a new mass were identified in follow-up imaging studies. Because the mass was identified as adenocarcinoma on biopsy, we performed left hepatectomy with redo hepaticojejunostomy. Pathologic examination showed a 4.5-cm-sized moderately differentiated adenocarcinoma arising from the remnant CC with lymph node metastasis. The patient recovered uneventfully and is currently undergoing adjuvant chemotherapy. Our case indicates that the remnant intrahepatic CC can undergo malignant transformation long after resection of CC. Since the intrahepatic CC portion in type IV CC is usually unresectable, wide hepaticojejunostomy and life-long observation with regular imaging study follow-up are highly recommended for prevention and early detection of malignant transformation.
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Affiliation(s)
- Suhyeon Ha
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Shin Hwang
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Lee Na Ryu
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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Abstract
To investigate risk factors for extrahepatic cholangiocarcinoma (ECC) in China. A total of 200 ECC cases and 200 matched control were included in the study. The presence of cigarette smoking, alcohol drinking, choledocholithiasis, primary sclerosing cholangitis, liver fluke infection (Clonorchis sinensis), diabetes mellitus, was investigated through clinical records. Blood from all cases was tested for hepatitis B surface antigen. Odds ratios (OR) and 95% confidence intervals (CIs) were estimated using conditional logistic regression. Compared with controls, ECC patients had a higher prevalence of cigarette smoking (61.0 vs. 47.0%, P=0.007), alcohol drinking (17.5 vs. 3.5%, P<0.000), and choledocholithiasis (6.0 vs. 1.0%, P=0.011). By multivariate analysis, the significant risk factors for the development of ECC were current smoking (OR=1.90, 95% CI=1.08-3.34), heavy alcohol drinking (OR=2.08, 95% CI=1.39-3.13), and choledocholithiasis (OR=6.68, 95% CI=1.48-30.27). The prevalence of hepatitis B virus infection, diabetes mellitus were not significantly different between cases and controls. These findings suggest that smoking, alcohol drinking, and choledocholithiasis are positive risk factors for the development of ECC in China.
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Choi JU, Hwang S, Chung YK. Management of intractable pancreatic leak from iatrogenic pancreatic duct injury following resection of choledochal cyst in an adult patient. Ann Hepatobiliary Pancreat Surg 2020; 24:228-233. [PMID: 32457272 PMCID: PMC7271105 DOI: 10.14701/ahbps.2020.24.2.228] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/09/2020] [Revised: 03/13/2020] [Accepted: 03/15/2020] [Indexed: 11/30/2022] Open
Abstract
Iatrogenic pancreatic duct injury can occur during resection of the choledochal cyst (CC). We herein present a case of postoperative pancreatic fistula (POPF) developed after resection of the CC in an adult patient with variant anomalous union of pancreatobiliary duct. The 55-year-old female patient underwent surgery after the diagnosis of CC-associated gallbladder cancer. During surgery, the CC mass was accidentally pulled out, by which the intrapancreatic CC portion was torn out from the main pancreatic duct. Since the pancreatic duct stump was not identified due to its small size, repair was not possible. The excavated defect at the pancreas head was closed securely combined with insertion of multiple drains. Postoperative POPF and peripancreatic fluid collection developed and the patient had to be fasted for 4 weeks. She was first discharged at 6 weeks after surgery. At 10 weeks, she was readmitted due to progression of peripancreatic fluid collection, which was controlled by percutaneous drain insertion. At 6 months, she was readmitted again due to repeated progression of peripancreatic fluid collection, which were controlled by endoscopic transmural duodenocystostomy. It took 8 months to resolve the pancreatic duct injury-associated pancreatitis. The experience in this case suggests that iatrogenic pancreatic duct injury during resection of CC can induce catastrophic complications, thus special attention should be paid to prevent pancreatic duct injury.
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Affiliation(s)
- Jin Uk Choi
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Shin Hwang
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Yong-Kyu Chung
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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Cochran ED, Lazow SP, Kim AG, Burkhalter LS, Frost NW, Stitelman D, Davis J, Santiago-Munoz P, Buchmiller TL, Perrone EE, Schindel DT. The in-utero diagnosis of choledochal cyst: can postnatal imaging predict benefit from early surgical intervention? J Matern Fetal Neonatal Med 2020; 35:1070-1074. [PMID: 32188329 DOI: 10.1080/14767058.2020.1742320] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/24/2022]
Abstract
Background: Infants prenatally suspected of having a choledochal cyst (CDC) typically undergo ultrasound imaging shortly after birth. This study sought to evaluate features on the initial postnatal ultrasound (IPU) that could identify newborns at risk for early complications.Methods: Following IRB approval, patients from four US fetal centers with prenatal suspicion for CDC and postnatal imaging from 2000 to 2017 were reviewed. Imaging and clinical courses were assessed.Results: Forty-two patients had prenatal ultrasounds suspicious for CDC. Nineteen (45.2%) were excluded due to diagnostic revision (n = 9), cyst resolution (n = 5), lack of IPU measurements (n = 3), or lack of follow-up (n = 2). The 23 remaining patients were included in the study. Of these, five (21.7%) developed symptoms at a median age of 16.5 days (IQR 16-19 days), and 18 (78.3%) remained asymptomatic throughout the first year after birth. Five patients (21.7%) had cysts ≥ 4.5 cm on IPU (Symptomatic: n = 3; Asymptomatic: n = 2). Eighteen patients (78.3%) had cysts < 4.5 cm on IPU (Symptomatic: n = 2; Asymptomatic: n = 16). An IPU cyst size ≥ 4.5 cm was associated with neonatal symptom manifestation (p = 0.048), with 88.9% specificity (95% CI 65.3-98.6%) and 60% sensitivity (95% CI 14.7-94.7%).Conclusions: In newborns with prenatally diagnosed CDC, a cyst size ≥ 4.5 cm on IPU is associated with symptom development during the first month after birth and therefore early cyst excision is recommended.
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Affiliation(s)
- Elizabeth D Cochran
- Children's Health, Pediatric Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA
| | - Stefanie P Lazow
- Pediatric Surgery, Harvard University Medical Center/Boston Children's Hospital, Boston, MA, USA
| | - Aimee G Kim
- Pediatric Surgery, CS Mott's Children's Hospital, University of Michigan Medical Center, Ann Arbor, MI, USA
| | - Lorrie S Burkhalter
- Pediatric Surgery, Harvard University Medical Center/Boston Children's Hospital, Boston, MA, USA
| | - Natalie W Frost
- Children's Health, Neonatology, University of Texas Southwestern Medical Center, Dallas, TX, USA
| | - David Stitelman
- Pediatric Surgery, Yale University Medical Center, New Haven, CT, USA
| | - James Davis
- Children's Health, Pediatric Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA
| | | | - Terry L Buchmiller
- Pediatric Surgery, Harvard University Medical Center/Boston Children's Hospital, Boston, MA, USA
| | - Erin E Perrone
- Pediatric Surgery, CS Mott's Children's Hospital, University of Michigan Medical Center, Ann Arbor, MI, USA
| | - David T Schindel
- Children's Health, Pediatric Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA
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Di Serafino M, Gioioso M, Severino R, Esposito F, Vezzali N, Ferro F, Pelliccia P, Caprio MG, Iorio R, Vallone G. Ultrasound findings in paediatric cholestasis: how to image the patient and what to look for. J Ultrasound 2020; 23:1-12. [PMID: 30756259 PMCID: PMC7010886 DOI: 10.1007/s40477-019-00362-9] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/14/2018] [Accepted: 01/24/2019] [Indexed: 02/07/2023] Open
Abstract
Paediatric biliary tract and gallbladder diseases include a variety of entities with a wide range of clinical presentations. Cholestasis represents an impaired secretion of bilirubin by hepatocytes, manifesting with high blood levels of conjugated bilirubin and jaundice. Various causes may be involved, which can be recognised analysing blood tests and hepatobiliary imaging, while sometimes liver biopsy or surgery may be necessary. High-resolution real-time ultrasonography is an important tool for differentiation of obstructive and non-obstructive causes of jaundice in infants and children. In this paper, we briefly review the normal anatomy and the ultrasound aspects of main pathologies affecting gallbladder and biliary tree in neonatal and paediatric age.
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Affiliation(s)
- Marco Di Serafino
- Department of Radiology, "Antonio Cardarelli" Hospital, Antonio Cardarelli st 9, 80131, Naples, Italy.
| | - Matilde Gioioso
- Department of Radiology, "San Carlo Regional Hospital", Potenza, Italy
| | - Rosa Severino
- Department of Radiology, "San Carlo Regional Hospital", Potenza, Italy
| | - Francesco Esposito
- Department of Radiology, "Santobono-Pausilipon" Children Hospital, Naples, Italy
| | - Norberto Vezzali
- Department of Radiology, "Regional Hospital of Bolzano", Bolzano, Italy
| | - Federica Ferro
- Department of Radiology, "Regional Hospital of Bolzano", Bolzano, Italy
| | | | - Maria Grazia Caprio
- Institute of Biostructure and Bioimaging IBB, Italian National Research Council CNR, Rome, Italy
| | - Raffaele Iorio
- Department of Paediatrics, "Federico II" University Hospital, Naples, Italy
| | - Gianfranco Vallone
- Department of Radiology, "Federico II" University Hospital, Naples, Italy
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Mehrotra S, Lalwani S, Nundy S. Management Strategies for Patients with Hilar Cholangiocarcinomas: Challenges and Solutions. Hepat Med 2020; 12:1-13. [PMID: 32158282 PMCID: PMC6986165 DOI: 10.2147/hmer.s223022] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/12/2019] [Accepted: 01/09/2020] [Indexed: 12/22/2022] Open
Abstract
Advances in imaging, pathology and therapy have resulted in major improvements in the management of cholangiocarcinomas; the mortality has come down and with it there has been an improved 5-year survival. Surgical resection remains the treatment of choice and reports from high volume centres have shown an increase in resectability rates, R0 resection, a decrease in mortality and an improvement in 5-year survival; however, the operative morbidity remains high, pointing towards the complexity of the management of these difficult lesions. Complete excision is also often limited by the locally advanced nature of the disease at the time of diagnosis and a proportion of patients who were earlier deemed resectable on imaging are found to have unresectable disease at the time of operation. Neoadjuvant therapy has had only a limited impact on survival. Liver transplantation is also an option in a few patients following strict criteria for selection. Since the large majority of patients are only diagnosed at the late stages of the disease palliation (endoscopic or surgical) is an important part of treatment. Portal vein embolisation and pre-operative biliary drainage have had a major impact on outcomes. Major liver resection with caudate lobe removal remains the standard operation and procedures like routine vascular resection and liver transplant should only be carried out in experienced centres. Improvements in both neo as well as adjuvant therapy may lead to a standardized protocol in the future, as well as an improvement in survival.
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Affiliation(s)
- Siddharth Mehrotra
- Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, New Delhi, India
| | - Shailendra Lalwani
- Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, New Delhi, India
| | - Samiran Nundy
- Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, New Delhi, India
- Correspondence: Samiran Nundy Email
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Hukkinen M, Björnland K, Gatzinsky V, Iber T, Johansen LS, Qvist N, Stenström P, Svensson JF, Pakarinen MP. Management strategies and treatment results of pediatric choledochal malformations in the Nordic countries. HPB (Oxford) 2020; 22:161-168. [PMID: 31371244 DOI: 10.1016/j.hpb.2019.07.001] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/19/2019] [Revised: 04/28/2019] [Accepted: 07/01/2019] [Indexed: 12/12/2022]
Abstract
BACKGROUND Incidence and long-term outcomes of choledochal malformations (CMs) in children remain unclear. METHODS Clinical characteristics, operative details, complications, and follow-up data were collected from eight pediatric surgical centers in Sweden, Norway, Denmark, and Finland, which also answered a questionnaire addressing management practices. RESULTS During 2000-2017, 126 pediatric CMs were diagnosed, corresponding an incidence of 1:37,400. Diagnostic, treatment, and follow-up practices varied markedly. Of patients with complete clinical data (n = 119), 85% and 11% had type I and IV CMs and were managed by open hepaticojejunostomy at median age of 2.5 (interquartile range 0.46-5.8) years. Associated malformations were more common in fusiform and type IV (23%) than cystic CMs (8%, p = 0.043). Pancreaticobiliary maljunction was more frequently confirmed in patients presenting with pancreatitis (26% vs. 7%, p = 0.005) and with fusiform CMs (56% vs. 25%, p = 0.001). Cholangitis/pancreatitis episodes, occurring in 12% during postoperative follow-up of 4.0 (2.0-7.9) years, associated with longer surveillance (OR 1.32, 95% CI 1.13-1.54, p < 0.001). However, only two thirds of centers continued follow-up until adulthood. No malignancies were reported. CONCLUSIONS CM incidence was higher than traditionally reported among Western populations. Although open hepaticojejunostomy carries good short-term outcomes, long-term morbidity is noteworthy. Standardized evidence-based management strategies and long-term follow-up are encouraged.
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Affiliation(s)
- Maria Hukkinen
- Pediatric Liver and Gut Research Group and Section of Pediatric Surgery, Helsinki University Children's Hospital, Helsinki, Finland
| | - Kristin Björnland
- Section of Pediatric Surgery, Oslo University Hospital, University of Oslo, Oslo, Norway
| | - Vladimir Gatzinsky
- Section of Pediatric Surgery, Queen Silvia Children's Hospital, Gothenburg, Sweden
| | - Tarja Iber
- Section of Pediatric Surgery, Tampere University Hospital, Tampere, Finland
| | | | - Niels Qvist
- Surgical Department A, Odense University Hospital, Odense, Denmark
| | - Pernilla Stenström
- Section of Pediatric Surgery, Skåne University Hospital, Lund University
| | - Jan F Svensson
- Section of Pediatric Surgery, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden
| | - Mikko P Pakarinen
- Pediatric Liver and Gut Research Group and Section of Pediatric Surgery, Helsinki University Children's Hospital, Helsinki, Finland.
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Tannuri ACA, Hara LADA, Paganoti GDF, Andrade WDC, Tannuri U. Choledochal cysts in children: How to Diagnose and Operate on. Clinics (Sao Paulo) 2020; 75:e1539. [PMID: 32215454 PMCID: PMC7074585 DOI: 10.6061/clinics/2020/e1539] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/29/2019] [Accepted: 12/18/2019] [Indexed: 11/18/2022] Open
Abstract
OBJECTIVE To identify the best mode for diagnosing and treating the patients with choledochal cysts. METHODS A retrospective study was performed with medical records of patients diagnosed with choledochal cysts from January 1994 to December 2017. In all cases, the diagnosis was based on ultrasound examination. All the patients underwent cyst resection and were divided in two groups: bile enteric anastomosis in the high portion of the common hepatic duct or in the dilated lower portion. RESULTS Eighty-one cases were studied. The age of presentation was 4 y 2 mo ± 4 y 1 mo, and the age for the surgical treatment was 5 y 5 mo ± 4 y 6 mo. In 61 cases, US was the only image examination performed. There were 67 cases of Todani type I (82.7%), 13 cases of type IV (16.0%) and one case of type III (1.2%). Nine patients (29.0%) in the first period and 2 patients (4.0%) in the second period presented with postoperative complications (p=0.016). CONCLUSION In patients with choledochal cysts, US is the only necessary diagnostic imaging. Performing the bile enteric anastomosis in the lower portion of the common hepatic duct is safer and has a lower risk of complications.
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Affiliation(s)
- Ana Cristina Aoun Tannuri
- Divisao de Cirurgia Pediatrica, Unidade Pediatrica de Transplante de Figado e Laboratorio de Pesquisa em Cirurgia Pediatrica (LIM 30), Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Sao Paulo, SP, BR
| | - Lucas Arjona de Andrade Hara
- Divisao de Cirurgia Pediatrica, Unidade Pediatrica de Transplante de Figado e Laboratorio de Pesquisa em Cirurgia Pediatrica (LIM 30), Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Sao Paulo, SP, BR
| | - Guilherme de Freitas Paganoti
- Divisao de Cirurgia Pediatrica, Unidade Pediatrica de Transplante de Figado e Laboratorio de Pesquisa em Cirurgia Pediatrica (LIM 30), Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Sao Paulo, SP, BR
| | - Wagner de Castro Andrade
- Divisao de Cirurgia Pediatrica, Unidade Pediatrica de Transplante de Figado e Laboratorio de Pesquisa em Cirurgia Pediatrica (LIM 30), Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Sao Paulo, SP, BR
| | - Uenis Tannuri
- Divisao de Cirurgia Pediatrica, Unidade Pediatrica de Transplante de Figado e Laboratorio de Pesquisa em Cirurgia Pediatrica (LIM 30), Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Sao Paulo, SP, BR
- Corresponding author. E-mail:
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Ataka R, Ito T, Masui T, Seo S, Ishii T, Ogiso S, Yagi S, Taura K, Uemoto S. Cholangiocarcinoma after flow diversion surgery for congenital biliary dilatation: A case report and review of literature. World J Hepatol 2019; 11:743-751. [PMID: 31772721 PMCID: PMC6856020 DOI: 10.4254/wjh.v11.i11.743] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/18/2019] [Revised: 08/19/2019] [Accepted: 10/02/2019] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Pancreaticobiliary maljunction (PBM) can be classified into two categories, PBM with congenital biliary dilatation (CBD) or PBM without biliary dilatation, and the management of PBM is often controversial. The treatment for PBM with CBD is prophylactic flow diversion surgery, and some authors have reported that the incidence of cancer after extrahepatic bile duct excision is less than 1%. A very rare case of intrahepatic cholangiocarcinoma 6 years after flow diversion surgery for PBM with CBD is reported.
CASE SUMMARY A 30-year-old man was diagnosed as having PBM with CBD, Todani classification type IVA, because of abnormal liver enzyme profiles. He underwent flow diversion surgery and cholecystectomy, and the specimen showed adenocarcinoma foci, pT1, pStage IA. Five and a half years passed without any recurrence of bile duct cancer. However, 6 years after his operation, computed tomography showed a gradually growing nodule in the bile duct. Fluorodeoxyglucose positron emission tomography showed high uptake, and magnetic resonance imaging showed restricted diffusion signals. On double balloon enteroscopy, the nodule at the posterior bile duct-jejunum anastomosis was directly visualized, and its biopsy specimen showed adenocarcinoma. The patient underwent right lobectomy and biliary reconstruction. The pathological diagnosis was intraductal papillary neoplasm with high-grade intraepithelial neoplasia, pTis, pN0, pStage 0. The patient’s postoperative course was uneventful, and he has had no recurrence up to the present time.
CONCLUSION This case suggests the necessity of careful observation after flow diversion surgery, especially when PBM with CBD is detected in adulthood.
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Affiliation(s)
- Ryo Ataka
- Department of Hepato-Biliary-Pancreatic Surgery and Transplantation, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan
| | - Takashi Ito
- Department of Hepato-Biliary-Pancreatic Surgery and Transplantation, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan
| | - Toshihiko Masui
- Department of Hepato-Biliary-Pancreatic Surgery and Transplantation, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan
| | - Satoru Seo
- Department of Hepato-Biliary-Pancreatic Surgery and Transplantation, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan
| | - Takamichi Ishii
- Department of Hepato-Biliary-Pancreatic Surgery and Transplantation, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan
| | - Satoshi Ogiso
- Department of Hepato-Biliary-Pancreatic Surgery and Transplantation, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan
| | - Shintaro Yagi
- Department of Hepato-Biliary-Pancreatic Surgery and Transplantation, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan
| | - Kojiro Taura
- Department of Hepato-Biliary-Pancreatic Surgery and Transplantation, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan
| | - Shinji Uemoto
- Department of Hepato-Biliary-Pancreatic Surgery and Transplantation, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan
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Baison GN, Bonds MM, Helton WS, Kozarek RA. Choledochal cysts: Similarities and differences between Asian and Western countries. World J Gastroenterol 2019; 25:3334-3343. [PMID: 31341359 PMCID: PMC6639560 DOI: 10.3748/wjg.v25.i26.3334] [Citation(s) in RCA: 35] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/20/2019] [Revised: 05/05/2019] [Accepted: 05/31/2019] [Indexed: 02/06/2023] Open
Abstract
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.
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Affiliation(s)
- George N Baison
- Digestive Disease Institute, Virginia Mason Medical Center, Seattle, WA 98101, United States
| | - Morgan M Bonds
- Digestive Disease Institute, Virginia Mason Medical Center, Seattle, WA 98101, United States
| | - William S Helton
- Digestive Disease Institute, Virginia Mason Medical Center, Seattle, WA 98101, United States
| | - Richard A Kozarek
- Digestive Disease Institute, Virginia Mason Medical Center, Seattle, WA 98101, United States
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Oh SY, Kwon JH, Hwang S. Development of adenocarcinoma at the remnant intrapancreatic cyst 16 years after resection of the choledochal cyst. Ann Hepatobiliary Pancreat Surg 2019; 23:192-196. [PMID: 31225424 PMCID: PMC6558127 DOI: 10.14701/ahbps.2019.23.2.192] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/11/2019] [Revised: 04/15/2019] [Accepted: 04/20/2019] [Indexed: 12/18/2022] Open
Abstract
One of the main reasons to resect choledochal cyst (CC) is to prevent malignant transformation. After resection of CC, the remnant intrapancreatic bile duct is no longer exposed to the enzymatic insult from pancreatic juice activated by the bile. Thus theoretically, the risk of malignant transformation disappears. We present a case showing development of adenocarcinoma from the remnant CC 16 years after resection of CC. A 43 year-old female patient underwent resection of type I CC 16 years ago, leaving a small remnant portion of CC as it was located deep within the pancreas close to the anomalous union of the pancreatobiliary duct. Four years later, intrahepatic ducts were dilated due to anastomotic stricture of the hepaticojejunostomy, which was resolved after repeated balloon dilatation. On follow-up examination at postoperative 16 years, a mass within the pancreas head was identified. As the mass was identified as adenocarcinoma on biopsy, pylorus-preserving pancreatoduodenectomy was performed. Pathologic examination showed a 3.5 cm-sized moderately differentiated adenocarcinoma arising from the remnant CC, with no lymph node metastasis. The patient recovered uneventfully, and is currently undergoing adjuvant chemotherapy. This case indicates that remnant CC can undergo malignant transformation. Therefore, complete resection of CC coupled with life-long follow-up is emphasized.
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Affiliation(s)
- Soo Young Oh
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Jae Hyun Kwon
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Shin Hwang
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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Aspelund G, Mahdi EM, Rothstein DH, Wakeman DS. Transitional care for patients with surgical pediatric hepatobiliary disease: Choledochal cysts and biliary atresia. J Gastroenterol Hepatol 2019; 34:966-974. [PMID: 30552863 DOI: 10.1111/jgh.14575] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/09/2018] [Revised: 11/13/2018] [Accepted: 12/09/2018] [Indexed: 12/14/2022]
Abstract
Choledochal cysts (CDCs) and biliary atresia (BA) are rare pediatric hepatobiliary anomalies that require surgical intervention due to increased risk of malignancy and liver failure, respectively. The underlying disease and operative procedures place patients at risk for long-term complications, which may continue to affect them into adulthood. Lack of a transitional care model in the health-care system potentiates the challenges they will face following aging out of their pediatric providers' care. We sought to elucidate the long-term complications and challenges patients with CDCs and BA face, review the current literature regarding transitioning care, and propose guidelines aiding adult providers in continued care and surveillance of these patients. A literature review was performed to assess short-term and long-term complications after surgery and the current standards for transitioning care in patients with a history of CDCs and BA. While transitional programs exist for patients with other gastrointestinal diseases, there are few that focus on CDCs or BA. Generally, authors encourage medical record transmission from pediatric to adult providers, ensuring accuracy of information and compliance with treatment plans. Patients with CDCs are at risk for developing biliary malignancies, cholangitis, and anastomotic strictures after resection. Patients with BA develop progressive liver failure, necessitating transplantation. There are no consensus guidelines regarding timing of follow up for these patients. Based on the best available evidence, we propose a schema for long-term surveillance.
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Affiliation(s)
- Gudrun Aspelund
- Department of Pediatric Surgery, Maria Fareri Children's Hospital, Valhalla, New York, USA
| | - Elaa M Mahdi
- Department of Surgery, School of Medicine and Dentistry, University of Rochester, Rochester, New York, USA
| | - David H Rothstein
- Department of Pediatric Surgery, School of Medicine and Biomedical Sciences, State University of New York at Buffalo, Buffalo, New York, USA
| | - Derek S Wakeman
- Department of Surgery, School of Medicine and Dentistry, University of Rochester, Rochester, New York, USA
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Chang X, Zhang X, Xiong M, Yang L, Li S, Cao G, Zhou Y, Yang D, Tang ST. Laparoscopic-assisted cyst excision and ductoplasty plus widened portoenterostomy for choledochal cysts with a narrow portal bile duct. Surg Endosc 2019; 33:1998-2007. [PMID: 30604269 PMCID: PMC6505504 DOI: 10.1007/s00464-018-06635-4] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/06/2018] [Accepted: 12/19/2018] [Indexed: 12/24/2022]
Abstract
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.
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Affiliation(s)
- Xiaopan Chang
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China
| | - Xi Zhang
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China
| | - Meng Xiong
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China
| | - Li Yang
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China
| | - Shuai Li
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China
| | - Guoqing Cao
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China
| | - Ying Zhou
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China
| | - Dehua Yang
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China
| | - Shao-Tao Tang
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China.
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Baba T, Mukai M, Masuya R, Kawano M, Yano K, Murakami M, Onishi S, Yamada K, Yamada W, Kawano T, Machigashira S, Nakame K, Kaji T, Ieiri S. The long-term health-related quality of life in patients operated for choledochal cyst. Pediatr Surg Int 2019; 35:539-546. [PMID: 30762105 DOI: 10.1007/s00383-019-04456-7] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 02/08/2019] [Indexed: 01/21/2023]
Abstract
PURPOSE Late postoperative complications in choledochal cyst (CC) patients are severe problems that affect the quality of life (QOL). We evaluated the postoperative complications and health-related QOL (HRQOL) of CC patients ≥ 18 years of age. METHODS From April 1984 to January 2018, 114 CC patients underwent definitive surgery at our institution. Seventy-nine patients reached ≥ 18 years of age. The HRQOL was assessed using the Japanese version of the SF-36v2. Eight health domain (physical functioning, role-physical, body pain, general health, role-emotional, vitality, mental health, and social functioning) scores and three component summary [physical component summary, mental component summary (MCS), and role-social component summary] scores were compared between the patients with and without complications (C [+] vs. C [-]). RESULTS Thirty-five patients answered the questionnaires. Twelve patients had postoperative complications. Among the eight domains, the score of general health was significantly lower in C [+] patients than in C [-] patients (p = 0.0488). Among the three component scores, the MCS score in C [+] patients was lower than in C [-] patients without significance (p = 0.0953). CONCLUSIONS The HRQOL of CC patients ≥ 18 years of age was mostly acceptable. However, postoperative complications can impair the sense of well-being and affect the mental health.
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Affiliation(s)
- Tokuro Baba
- Department of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan
| | - Motoi Mukai
- Department of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan
| | - Ryuta Masuya
- Department of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan
| | - Masato Kawano
- Department of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan
| | - Keisuke Yano
- Department of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan
| | - Masakazu Murakami
- Department of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan
| | - Shun Onishi
- Department of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan
| | - Koji Yamada
- Department of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan
| | - Waka Yamada
- Department of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan
| | - Takafumi Kawano
- Department of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan
| | - Seiro Machigashira
- Department of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan
| | - Kazuhiko Nakame
- Department of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan
| | - Tatsuru Kaji
- Department of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan
| | - Satoshi Ieiri
- Department of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan.
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Aota T, Kubo S, Takemura S, Tanaka S, Amano R, Kimura K, Yamazoe S, Shinkawa H, Ohira G, Shibata T, Horiike M. Long-term outcomes after biliary diversion operation for pancreaticobiliary maljunction in adult patients. Ann Gastroenterol Surg 2019; 3:217-223. [PMID: 30923792 PMCID: PMC6422791 DOI: 10.1002/ags3.12239] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/19/2018] [Revised: 01/15/2019] [Accepted: 01/16/2019] [Indexed: 12/13/2022] Open
Abstract
AIM Pancreaticobiliary maljunction (PBM) with or without congenital biliary dilatation (CBD) is a risk factor for biliary tract cancer. We investigated long-term outcomes after biliary diversion operation with special reference to types of CBD. METHODS Subjects comprised 40 adult patients who underwent biliary diversion operation for PBM without biliary tract cancer. Group A comprised 20 patients with type Ia or Ic CBD, or non-dilated bile ducts, while group B comprised 20 patients with type IV-A CBD. The clinical findings and postoperative outcomes were compared between groups. RESULTS Of 40 patients, nine patients suffered from repeated cholangitis and eight of these nine patients suffered from hepatolithiasis after biliary diversion operation. Biliary tract cancer or pancreatic cancer was detected in four patients at 3 years and 2 months to 24 years after the operation. In three of these four patients, the serum concentration of carbohydrate antigen 19-9 increased before detection of carcinoma. One patient died of hepatic failure due to repeated cholangitis. The proportions of patients with repeated cholangitis, hepatolithiasis, and re-operation, and patients who died of biliary tract cancer, pancreatic cancer, or hepatic failure, were significantly higher in group B than in group A. The survival rate was significantly worse in group B than in group A. CONCLUSIONS Careful long-term follow-up with measurement of serum tumor markers is necessary after biliary diversion operation for PBM, especially in patients with type IV-A CBD or repeated cholangitis.
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Affiliation(s)
- Takanori Aota
- Department of Hepato‐Biliary‐Pancreatic SurgeryOsaka City University Graduate School of MedicineOsakaJapan
| | - Shoji Kubo
- Department of Hepato‐Biliary‐Pancreatic SurgeryOsaka City University Graduate School of MedicineOsakaJapan
| | - Shigekazu Takemura
- Department of Hepato‐Biliary‐Pancreatic SurgeryOsaka City University Graduate School of MedicineOsakaJapan
| | - Shogo Tanaka
- Department of Hepato‐Biliary‐Pancreatic SurgeryOsaka City University Graduate School of MedicineOsakaJapan
| | - Ryosuke Amano
- Department of Hepato‐Biliary‐Pancreatic SurgeryOsaka City University Graduate School of MedicineOsakaJapan
| | - Kenjiro Kimura
- Department of Hepato‐Biliary‐Pancreatic SurgeryOsaka City University Graduate School of MedicineOsakaJapan
| | - Sadaaki Yamazoe
- Department of Hepato‐Biliary‐Pancreatic SurgeryOsaka City University Graduate School of MedicineOsakaJapan
| | - Hiroji Shinkawa
- Department of Hepato‐Biliary‐Pancreatic SurgeryOsaka City University Graduate School of MedicineOsakaJapan
| | - Go Ohira
- Department of Hepato‐Biliary‐Pancreatic SurgeryOsaka City University Graduate School of MedicineOsakaJapan
| | - Toshihiko Shibata
- Department of Hepato‐Biliary‐Pancreatic SurgeryOsaka City University Graduate School of MedicineOsakaJapan
| | - Masaki Horiike
- Department of Pediatric SurgeryOsaka City University Graduate School of MedicineOsakaJapan
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Abstract
Although the most common presentation of biliary disorder in North America is secondary to gallstone disease, an awareness of benign biliary cystic neoplasms is important because of the risk of malignant transformation. The incidence of premalignant cystic neoplasms of the bile duct is not well characterized and they often are detected incidentally for suspicion of other abdominal disorders. This article describes the 4 most common premalignant biliary cystic neoplasms: biliary mucinous cystic neoplasms, intraductal papillary mucinous neoplasms of the bile duct, intraductal tubular papillary neoplasms of the bile duct, and choledochal cysts.
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Affiliation(s)
- Zaheer S Kanji
- Section of General, Thoracic and Vascular Surgery, Virginia Mason Medical Center, 1100 Ninth Avenue, Seattle, WA 98101, USA; Division of General Surgery, Department of Surgery, Royal Inland Hospital, University of British Columbia, 311 Columbia Street, Kamloops, British Columbia V2C 2T1, Canada
| | - Flavio G Rocha
- Section of General, Thoracic and Vascular Surgery, Virginia Mason Medical Center, 1100 Ninth Avenue, Seattle, WA 98101, USA.
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49
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Use of 18F-FDG PET/CT for the Evaluation of Carcinoma Arising From Choledochal Cysts. Clin Nucl Med 2019; 44:e51-e53. [DOI: 10.1097/rlu.0000000000002342] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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50
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Kamisawa T, Kaneko K, Itoi T, Ando H. Pancreaticobiliary maljunction and congenital biliary dilatation. Lancet Gastroenterol Hepatol 2018; 2:610-618. [PMID: 28691687 DOI: 10.1016/s2468-1253(17)30002-x] [Citation(s) in RCA: 78] [Impact Index Per Article: 11.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/17/2016] [Revised: 12/27/2016] [Accepted: 01/03/2017] [Indexed: 02/07/2023]
Abstract
Pancreaticobiliary maljunction is a congenital malformation in which the pancreatic and bile ducts join outside the duodenal wall, usually forming a long common channel. Because the action of the sphincter of Oddi does not regulate the function of the pancreaticobiliary junction in patients with pancreaticobiliary maljunction, two-way regurgitation occurs. Reflux of pancreatic juice into the biliary tract is associated with a high incidence of biliary cancer. Biliary carcinogenesis in patients with pancreaticobiliary maljunction is thought to follow the hyperplasia, dysplasia, then carcinoma sequence due to chronic inflammation caused by pancreatobiliary reflux. Pancreaticobiliary maljunction is diagnosed when an abnormally long common channel is evident on imaging studies. Congenital biliary dilatation involves both local dilatation of the extrahepatic bile duct, including the common bile duct, and pancreaticobiliary maljunction. Extrahepatic bile duct resection is the standard surgery for congenital biliary dilatation. However, complete excision of the intrapancreatic bile duct and removal of stenoses of the hepatic ducts are necessary to prevent serious complications after surgery.
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Affiliation(s)
- Terumi Kamisawa
- Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan.
| | - Kenitiro Kaneko
- Division of Pediatric Surgery, Department of Surgery, Aichi Medical University, Nagakute, Japan
| | - Takao Itoi
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Tokyo, Japan
| | - Hisami Ando
- Department of Pediatric Surgery, Aichi Prefectural Colony, Kasugai, Japan
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