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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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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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Gyawali S, Adhikari G, Shrestha S, Pradhan S, Bhandari RS. Concomitant hilar cholangiocarcinoma with choledochal cyst and cholelithiasis in an asymptomatic patient: A case report. Int J Surg Case Rep 2021; 84:106094. [PMID: 34139420 PMCID: PMC8219770 DOI: 10.1016/j.ijscr.2021.106094] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/19/2021] [Revised: 05/23/2021] [Accepted: 06/05/2021] [Indexed: 10/26/2022] Open
Abstract
INTRODUCTION AND IMPORTANCE Choledochal cyst is an important risk factor for cholangiocarcinoma. Concomitant hilar cholangiocarcinoma with choledochal cyst with cholelithiasis is a rare finding. CASE PRESENTATION A 52-year-old male presented with non-specific complaints of generalized weakness for 3 months. Transabdominal ultrasound showed dilated common bile duct with hyperechoic mass at hilar region; further evaluation with magnetic resonance cholangiopancreatography and contrast-enhanced computer tomography of abdomen and pelvis revealed concomitant intraductal hilar cholangiocarcinoma (Bismuth Corlette type 1) with Choledochal cyst (type IVa) with cholelithiasis. After optimization patient underwent left hepatectomy with common bile duct excision with cholecystectomy with Roux-en-Y hepaticojejunostomy. Histopathological examination confirmed it to be well-differentiated adenocarcinoma, intestinal type at the hilar confluence. CLINICAL DISCUSSION An asymptomatic male patient with the concomitant finding of perihilar cholangiocarcinoma (Bismuth Corlette type I) with choledochal cyst type IVa with cholelithiasis is a rare finding. The patient was managed with surgical excision of the common bile duct with Roux-en-Y hepaticojejunostomy and cholecystectomy. Diagnostic evaluation should be proper not to miss or overlook such a synchronous lesion. CONCLUSION Incidental finding of concomitant perihilar cholangiocarcinoma with the choledochal cyst is rare. Proper evaluation of the patient with CECT abdomen and pelvis and MRCP is necessary for the diagnosis. Proper surgical resection with adequate lymph node removal is important for surgical clearance.
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Affiliation(s)
- Sushil Gyawali
- Department of GI and General Surgery, Tribhuvan University Teaching Hospital, Institute of Medicine, Kathmandu, Nepal.
| | - Gagan Adhikari
- Department of GI and General Surgery, Tribhuvan University Teaching Hospital, Institute of Medicine, Kathmandu, Nepal
| | - Sujan Shrestha
- Department of GI and General Surgery, Tribhuvan University Teaching Hospital, Institute of Medicine, Kathmandu, Nepal
| | - Sumita Pradhan
- Department of GI and General Surgery, Tribhuvan University Teaching Hospital, Institute of Medicine, Kathmandu, Nepal
| | - Ramesh Singh Bhandari
- Department of GI and General Surgery, Tribhuvan University Teaching Hospital, Institute of Medicine, Kathmandu, Nepal
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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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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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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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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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Zhang C, Zhou J, Kou K, Liu S, We F, Wang G. Occurrence of signet-ring cell carcinoma with cholangiocarcinoma 25 years after choledochal cyst excision: A case report. Medicine (Baltimore) 2018; 97:e9956. [PMID: 29465585 PMCID: PMC5841982 DOI: 10.1097/md.0000000000009956] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/12/2022] Open
Abstract
RATIONALE Choledochal cysts are a risk factor for the development of cholangiocarcinoma. Hence, complete surgical excision is the preferred treatment in most cases. However, cholangiocarcinoma still can develop from the remnant biliary system after surgical excision. Signet-ring cell carcinoma is a rare type of cancer of the biliary system, and the occurrence of signet-ring cell carcinoma after surgical excision of choledochal cysts has not been reported in the English literature to date. PATIENT CONCERNS We report a case of a 32-year-old woman who presented with a 1-month history of abdominal pain,obstructive jaundice, itching, and fever. The patient had undergone choledochal cyst excision and Roux-en-Y hepatico-jejunostomy 25 years previously and had now developed signet-ring cell carcinoma along with cholangiocarcinoma at the anastomotic site. DIAGNOSES:: signet-ring cell carcinoma along with cholangiocarcinoma. INTERVENTIONS Interventions included laparotomy with evacuation,blood transfusion,and other adjuvant therapy. OUTCOMES The patient died five months later. LESSONS Surgery is the best treatment for CCCs, and the surgeon should try to remove as much as of the bile duct cyst as possible.
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Kobayashi T, Ohashi T, Sakata J, Miura K, Wakai T. Cholangiocarcinoma Developing from the Remnant Bile Ducts Following Cyst Excision for Congenital Biliary Dilatation. PANCREATICOBILIARY MALJUNCTION AND CONGENITAL BILIARY DILATATION 2018:253-261. [DOI: 10.1007/978-981-10-8654-0_30] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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Ikegame K, Takano A, Watanabe H, Yamamoto A, Miyasaka Y, Furuya K, Nakada H, Sugai H, Yasutome M, Inoue M, Hada M, Nakagomi H, Omata M, Oyama T. Biliary cancer developed after the reparative surgery for congenital choledochal cyst: a case report and review of the literature. Int Cancer Conf J 2017; 6:43-49. [PMID: 31149469 PMCID: PMC6498380 DOI: 10.1007/s13691-016-0270-x] [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: 10/12/2016] [Accepted: 11/06/2016] [Indexed: 11/26/2022] Open
Abstract
The incidence of bile duct cancer developed in the patients with congenital choledochal cysts (CC) with a history of reparative surgery is not well known. We experienced a case developed choleductal cancer 45 years after reparative surgery. She underwent reparative surgery with cyst excision and hepatic bile duct duodenal anastomosis at 1 year of age. She developed the symptoms of jaundice, anorexia, and dull pain in the right upper part of the abdomen at 47 years of age. The carcinoma arose from the dilated proximal bile duct anastomosed with the duodenum. Cholestasis and regurgitation of duodenal fluids seemed to have influenced the development of cancer in this patient. We additionally reviewed seven cohort studies concerning the incidence of biliary carcinoma after surgery for congenital choledocal cysts. The incidence of biliary cancer developed after surgery was 2.2 ± 2.5 (ranged 0-6.5)%. We also reviewed 33 Japanese case reports cited in Japan MEDLINE from 1986 to 2015. Regarding the CC types according to the Totani's classification, 12 were type I and 14 type IVa, 1 was type II and 6 were unknown type. The rate of coexistence of pancreaticobiliary maljunction (PBM) was 92%, (22/24; other 9 cases were not documented), and biliary cancer arose from the proximal stump of the reparative surgery in 68% (17/25) and from the distal stump in 32% (8/25) of cases. These findings suggested that the reflux of pancreatic juice due to PBM only partially explained the carcinogenesis of CCs. Intensive follow-up of such patients throughout their lives is necessary to avoid cancer death even after standard reparative surgery.
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Affiliation(s)
- Kou Ikegame
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Atsushi Takano
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Hideki Watanabe
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Atsushi Yamamoto
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Yoshiaki Miyasaka
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Kazushige Furuya
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Haruka Nakada
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Hidemitsu Sugai
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Michiya Yasutome
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Masayuki Inoue
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Masao Hada
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Hiroshi Nakagomi
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Masao Omata
- Department of Internal Medicine, Yamanashi Prefectural Central Hospital, Kofu, Japan
| | - Toshio Oyama
- Department of Pathology, Yamanashi Prefectural Central Hospital, Kofu, Japan
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Moslim MA, Takahashi H, Seifarth FG, Walsh RM, Morris-Stiff G. Choledochal Cyst Disease in a Western Center: A 30-Year Experience. J Gastrointest Surg 2016; 20:1453-63. [PMID: 27260526 DOI: 10.1007/s11605-016-3181-4] [Citation(s) in RCA: 41] [Impact Index Per Article: 4.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/18/2016] [Accepted: 05/27/2016] [Indexed: 02/07/2023]
Abstract
BACKGROUND The aim of this study was to report a Western experience in the diagnosis and management of choledochal cyst disease. RESULTS Sixty-seven patients were identified including 15 children and 52 adults; 76.1 % were females. The median age at diagnosis was 3 [inter-quartile range (IQR) = 6.0-0.7] years for children, and 46 [IQR = 55.6-34.3] years for adults. Forty-eight patients (72 %) were symptomatic. Types of choledochal cyst included: I (n = 49, 73.1 %), II (n = 1, 1.5 %), IV (n = 9, 13.4 %), and V (n = 8, 12 %). The median diameter of the type I choledochal cyst was 35 [IQR = 47-25] mm. All 48 patients underwent excision of cyst with Roux-en-Y hepaticojejunostomy, and eight underwent resection with hepaticoduodenostomy. Six patients underwent liver resection, and five patients underwent orthotopic liver transplantation. Malignancy was concomitant in five adult patients, being identified on preoperative imaging in three cases; and atypia was seen in three additional patients. Early morbidity included Clavien-Dindo classification grades III (n = 7) and II (n = 5), while long-term complications consisted of Clavien-Dindo grades V (n = 5), IV (n = 2), III (n = 18), and II (n = 1). CONCLUSIONS Presentation and management of choledochal cyst is varied. Malignant transformation is often detected incidentally, and so should be the driving source for resection when a choledochal cyst is diagnosed.
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Affiliation(s)
- Maitham A Moslim
- Department of HPB Surgery, Digestive Disease Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH, 44195, USA.
| | - Hideo Takahashi
- Department of HPB Surgery, Digestive Disease Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH, 44195, USA
| | - Federico G Seifarth
- Department of Pediatric Surgery, Cleveland Clinic Children's Hospital, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH, 44195, USA
| | - R Matthew Walsh
- Department of HPB Surgery, Digestive Disease Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH, 44195, USA
| | - Gareth Morris-Stiff
- Department of HPB Surgery, Digestive Disease Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH, 44195, USA
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Ng DWJ, Chiow AKH, Poh WT, Tan SS. Metachronous cholangiocarcinoma 13 years post resection of choledochal cyst-is long-term follow-up useful?: a case study and review of the literature. Surg Case Rep 2016; 2:60. [PMID: 27307284 PMCID: PMC4909682 DOI: 10.1186/s40792-016-0187-9] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/16/2015] [Accepted: 06/09/2016] [Indexed: 01/08/2023] Open
Abstract
BACKGROUND Patients with congenital choledochal cyst are at risk of the development of hepatobiliary malignancy, with recommended treatment of choledochal cyst being surgical excision. The development of cholangiocarcinoma more than 10 years after excision of choledochal cysts is rare, with less than 21 cases reported in the literature from 1972 to 2014. This is the first reported case of metachronous recurrence after a previously excised adenocarcinoma within a choledochal cyst. CASE PRESENTATION Herein, we review the case of a patient with cholangiocarcinoma arising 13 years post excision of a Todani type 1 choledochal cyst and discuss the theories of carcinogenesis and long-term management of patients with choledochal cysts. The long-term development of a malignancy must be considered in these patients. CONCLUSIONS Reviewing all published cases to date, regular follow-up post resection did not improve on the resectability and long-term survival of these patients. Patients presenting with symptoms did not prejudice against resectability. Despite curative resection, median survival was dismal. Optimal long-term follow-up strategies for these patients remain to be elucidated.
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Affiliation(s)
- Deanna Wan Jie Ng
- Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore
| | - Adrian Kah Heng Chiow
- Hepatopancreatobiliary Unit, Department of General Surgery, Changi General Hospital, Simei Street 3, Singapore, 529889, Singapore.
| | - Wee Teng Poh
- Department of Pathology, Changi General Hospital, Singapore, Singapore
| | - Siong San Tan
- Hepatopancreatobiliary Unit, Department of General Surgery, Changi General Hospital, Simei Street 3, Singapore, 529889, Singapore
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