A new classification of congenital biliary dilatation—HUAXI CBD classification_CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Authors：

PU Siyu ^1,2 ,  JIN Shuguang ^1,2 , YAN Lünan ² , AI Chengbo ^1,2

1. Department of Pediatric Surgery, West China Hospital, Sichuan University, Chengdu 610041, P. R. China;
2. Center of Liver Transplantation, West China Hospital, Sichuan University, Chengdu 610041, P. R. China;

Corresponding?author：

JIN Shuguang, Email: shgjin2003@aliyun.com

Keywords：

biliary dilatation; classification; imaging; diagnosis; treatment

DOI：

10.7507/1007-9424.202209068

Video：

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Objective To intend to propose a new clinical classification of congenital biliary dilatation (CBD) which is more concise and suitable for diagnosis and treatment, and explore feasibility and reliability of the new classification. Methods Based on the preoperative imaging data of patients with CBD admitted to the Department of Pediatric Surgery of West China Hospital of Sichuan University, from January 2015 to December 2018, a new classification of CBD was accomplished according to the site of bile duct dilatation lesions, which was named HUAXI CBD classification. The CBD was classified into 4 types: type Ⅰ (distal extra-hepatic bile duct dilatation), type Ⅱ (distal extra-hepatic combined with right and left primary hepatic bile duct dilatation), type Ⅲ (extra-hepatic combined with secondary and above hepatic bile duct dilatation), and type Ⅳ (intra-hepatic bile duct dilatation). Meanwhile, the feasibility and reliability of the HUAXI CBD classification were analyzed. Results A total of 300 patients with CBD were included in this study. According to the HUAXI CBD classification method, 240 cases were type Ⅰ, 48 cases were type Ⅱ, 10 cases were type Ⅲ, and 2 cases were type Ⅳ. For type Ⅰ, 236 patients underwent cholecystectomy and Roux-en-Y hepaticojejunostomy and 4 cases were treated non-operatively; For type Ⅱ, 48 patients underwent cholecystectomy, central hepatic duct reconstruction and Roux-en-Y hepaticojejunostomy; For type Ⅲ, 9 patients underwent cholecystectomy and Roux-en-Y hepaticojejunostomy, 5 of which had intrahepatic dilatation confined to part of the liver lobe and underwent partial hepatectomy with intra-hepaticojejunostomy. One case was treated with percutaneous transhepatic cholangial drainage (PTCD) only; two type Ⅳ patients were treated with PTCD only. Patients with type Ⅰ had a normal hepatobiliary function after surgery and a good prognosis; patients with type Ⅱ had good recovery of liver function in 37 cases (77.1%), but 11 cases (22.9%) had postoperative complications during follow-up; patients with type Ⅲ and Ⅳ had high incidence of cholangitis (6/10, 2/2, respectively) and choledocholithiasis (5/10, 2/2, respectively) after surgery, especially those with diffuse intrahepatic dilatation having a poor prognosis, eventually developing cirrhosis and necessitating liver transplantation. Conclusion The HUAXI CBD classification is consistent with treatment principles, concise and easy to remember, and more suitable for pediatric clinical application, which can effectively assist in the selection of clinical treatment strategies for children with BD.

Citation： PU Siyu, JIN Shuguang, YAN Lünan, AI Chengbo. A new classification of congenital biliary dilatation—HUAXI CBD classification. CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY, 2023, 30(1): 23-29. doi: 10.7507/1007-9424.202209068 Copy

1.	Hamada Y, Ando H, Kamisawa T, et al. Diagnostic criteria for congenital biliary dilatation 2015. J Hepatobiliary Pancreat Sci, 2016, 23(6): 342-346.
2.	Khan S , Nagorney DM. Bile duct cysts in adults // Jarnagin WR, Blumgart LH. Blumgarts Surgery of the Liver Pancreas & Biliary Trac. The fifth edtion. Elsevier, 2012.
3.	Yoon JH. Magnetic resonance cholangiopancreatography diagnosis of choledochal cyst involving the cystic duct: report of three cases. Br J Radiol, 2011, 84(997): e18-e22. doi: 10.1259/bjr/77844300.
4.	Popova-Jovanovska R, Genadieva-Dimitrova M, Trajkovska M, et al. Choledochal cysts: diagnosis and treatment. Prilozi, 2012, 33(1): 49-63.
5.	Todani T, Watanabe Y, Narusue M, et al. Congenital bile duct cysts: classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg, 1977, 134(2): 263-269.
6.	Alonso-Lej F, Rever W, Pessagno D. A study of the congenital choledochal cyst with a report of two patients and an analysis of 94 cases. Surg Gynec Obstet, 1959, 108(1): 1-30.
7.	Visser BC, Suh I, Way LW, et al. Congenital choledochal cysts in adults. Arch Surg, 2004, 139(8): 855-860.
8.	Gadelhak N, Shehta A, Hamed H. Diagnosis and management of choledochal cyst: 20 years of single center experience. World J Gastroenterol, 2014, 20(22): 7061-7066.
9.	Nakanuma Y, Harada K, Sato Y, et al. Recent progress in the etiopathogenesis of pediatric biliary disease, particularly Caroli's disease with congenital hepatic fibrosis and biliary atresia. Histol Histopathol, 2010, 25(2): 223-235.
10.	Todani T. Congenital choledochal dilatation: classification, clinical features, and long-term results. J Hepato-Biliary-Pancreatic Surg, 1997, 4(3): 276-282.
11.	Todani T, Watanabe Y, Toki A, et al. Classification of congenital biliary cystic disease: special reference to type Ⅰc and ⅣA cysts with primary ductal stricture. J Hepatobiliary Pancreat Surg, 2003, 10(5): 340-344.
12.	Lenriot JP, Gigot JF, Ségol P, et al. Bile duct cysts in adults: a multi-institutional retrospective study. French Associations for Surgical Research. Ann Surg, 1998, 228(2): 159-166.
13.	Wiseman K, Buczkowski AK, Chung SW, et al. Epidemiology, presentation, diagnosis, and outcomes of choledochal cysts in adults in an urban environment. Am J Surg, 2005, 189(5): 527-531.
14.	Ziegler KM, Pitt HA, Zyromski NJ, et al. Choledochoceles: are they choledochal cysts? Ann Surg, 2010, 252(4): 683-690.
15.	董家鴻, 鄭秀海, 夏紅天, 等. 膽管囊狀擴張癥: 新的臨床分型與治療策略. 中華消化外科雜志, 2013, 12(5): 370-377.
16.	Huang CS, Huang CC, Chen DF. Choledochal cysts: differences between pediatric and adult patients. J Gastrointest Surg, 2010, 14(7): 1105-1110.
17.	Singham J, Schaeffer D, Yoshida E, et al. Choledochal cysts: analysis of disease pattern and optimal treatment in adult and paediatric patients. HPB (Oxford), 2007, 9(5): 383-387.
18.	Katabathina VS, Kapalczynski W, Dasyam AK, et al. Adult choledochal cysts: current update on classification, pathogenesis, and cross-sectional imaging findings. Abdom Imaging, 2015, 40(6): 1971-1981.
19.	中華醫學會外科學分會膽道外科學組. 膽管擴張癥診斷與治療指南(2017 版). 中華消化外科雜志, 2017, 16(8): 767-774.
20.	Sacher VY, Davis JS, Sleeman D, et al. Role of magnetic resonance cholangiopancreatography in diagnosing choledochal cysts: case series and review. World J Radiol, 2013, 5(8): 304-312.
21.	Kamisawa T, Tu Y, Egawa N, et al. MRCP of congenital pancreaticobiliary malformation. Abdom Imaging, 2007, 32(1): 129-133.
22.	Chen YK, Pleskow DK. SpyGlass single-operator peroral cholangiopancreatoscopy system for the diagnosis and therapy of bile-duct disorders: a clinical feasibility study (with video). Gastrointest Endosc, 2007, 65(6): 832-841.
23.	Saito T, Terui K, Mitsunaga T, et al. Role of pediatric endoscopic retrograde cholangiopancreatography in an era stressing less-invasive imaging modalities. J Pediatr Gastroenterol Nutr, 2014, 59(2): 204-209.
24.	Yoo KS, Lehman GA. Endoscopic management of biliary ductal stones. Gastroenterol Clin North Am, 2010, 39(2): 209-227.
25.	Stavropoulos S, Larghi A, Verna E, et al. Intraductal ultrasound for the evaluation of patients with biliary strictures and no abdominal mass on computed tomography. Endoscopy, 2005, 37(8): 715-721.
26.	Farrell RJ, Agarwal B, Brandwein SL, et al. Intraductal US is a useful adjunct to ERCP for distinguishing malignant from benign biliary strictures. Gastrointest Endosc, 2002, 56(5): 681-687.
27.	Jan YY, Chen HM, Chen MF. Malignancy in choledochal cysts. Hepatogastroenterology, 2000, 47(32): 337-340.
28.	Roskams T, Desmet V. Embryology of extra- and intrahepatic bile ducts, the ductal plate. Anat Rec (Hoboken), 2008, 291(6): 628-635.
29.	Sato Y, Ren XS, Nakanuma Y. Caroli’s disease: current knowledge of its biliary pathogenesis obtained from an orthologous rat model. Int J Hepatol, 2012, 2012: 107945. doi: 10.1155/2012/107945.
30.	Drenth JP, Chrispijn M, Bergmann C. Congenital fibrocystic liver diseases. Best Pract Res Clin Gastroenterol, 2010, 24(5): 573-584.
31.	Ponsky LE, Geisinger MA, Ponsky JL, et al. Contemporary “urologic” intervention in the pancreaticobiliary tree. Urology, 2001, 57(1): 21-25.
32.	Amano H, Shirota C, Tainaka T, et al. Late postoperative complications of congenital biliary dilatation in pediatric patients: a single-center experience of managing complications for over 20 years. Surg Today, 2021, 51(9): 1488-1495.
33.	Koshinaga T, Inoue M, Ohashi K, et al. Persistent biliary dilatation and stenosis in postoperative congenital choledochal cyst. J Hepatobiliary Pancreat Sci, 2011, 18(1): 47-52.

1. Hamada Y, Ando H, Kamisawa T, et al. Diagnostic criteria for congenital biliary dilatation 2015. J Hepatobiliary Pancreat Sci, 2016, 23(6): 342-346.
2. Khan S , Nagorney DM. Bile duct cysts in adults // Jarnagin WR, Blumgart LH. Blumgarts Surgery of the Liver Pancreas & Biliary Trac. The fifth edtion. Elsevier, 2012.
3. Yoon JH. Magnetic resonance cholangiopancreatography diagnosis of choledochal cyst involving the cystic duct: report of three cases. Br J Radiol, 2011, 84(997): e18-e22. doi: 10.1259/bjr/77844300.
4. Popova-Jovanovska R, Genadieva-Dimitrova M, Trajkovska M, et al. Choledochal cysts: diagnosis and treatment. Prilozi, 2012, 33(1): 49-63.
5. Todani T, Watanabe Y, Narusue M, et al. Congenital bile duct cysts: classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg, 1977, 134(2): 263-269.
6. Alonso-Lej F, Rever W, Pessagno D. A study of the congenital choledochal cyst with a report of two patients and an analysis of 94 cases. Surg Gynec Obstet, 1959, 108(1): 1-30.
7. Visser BC, Suh I, Way LW, et al. Congenital choledochal cysts in adults. Arch Surg, 2004, 139(8): 855-860.
8. Gadelhak N, Shehta A, Hamed H. Diagnosis and management of choledochal cyst: 20 years of single center experience. World J Gastroenterol, 2014, 20(22): 7061-7066.
9. Nakanuma Y, Harada K, Sato Y, et al. Recent progress in the etiopathogenesis of pediatric biliary disease, particularly Caroli's disease with congenital hepatic fibrosis and biliary atresia. Histol Histopathol, 2010, 25(2): 223-235.
10. Todani T. Congenital choledochal dilatation: classification, clinical features, and long-term results. J Hepato-Biliary-Pancreatic Surg, 1997, 4(3): 276-282.
11. Todani T, Watanabe Y, Toki A, et al. Classification of congenital biliary cystic disease: special reference to type Ⅰc and ⅣA cysts with primary ductal stricture. J Hepatobiliary Pancreat Surg, 2003, 10(5): 340-344.
12. Lenriot JP, Gigot JF, Ségol P, et al. Bile duct cysts in adults: a multi-institutional retrospective study. French Associations for Surgical Research. Ann Surg, 1998, 228(2): 159-166.
13. Wiseman K, Buczkowski AK, Chung SW, et al. Epidemiology, presentation, diagnosis, and outcomes of choledochal cysts in adults in an urban environment. Am J Surg, 2005, 189(5): 527-531.
14. Ziegler KM, Pitt HA, Zyromski NJ, et al. Choledochoceles: are they choledochal cysts? Ann Surg, 2010, 252(4): 683-690.
15. 董家鴻, 鄭秀海, 夏紅天, 等. 膽管囊狀擴張癥: 新的臨床分型與治療策略. 中華消化外科雜志, 2013, 12(5): 370-377.
16. Huang CS, Huang CC, Chen DF. Choledochal cysts: differences between pediatric and adult patients. J Gastrointest Surg, 2010, 14(7): 1105-1110.
17. Singham J, Schaeffer D, Yoshida E, et al. Choledochal cysts: analysis of disease pattern and optimal treatment in adult and paediatric patients. HPB (Oxford), 2007, 9(5): 383-387.
18. Katabathina VS, Kapalczynski W, Dasyam AK, et al. Adult choledochal cysts: current update on classification, pathogenesis, and cross-sectional imaging findings. Abdom Imaging, 2015, 40(6): 1971-1981.
19. 中華醫學會外科學分會膽道外科學組. 膽管擴張癥診斷與治療指南(2017 版). 中華消化外科雜志, 2017, 16(8): 767-774.
20. Sacher VY, Davis JS, Sleeman D, et al. Role of magnetic resonance cholangiopancreatography in diagnosing choledochal cysts: case series and review. World J Radiol, 2013, 5(8): 304-312.
21. Kamisawa T, Tu Y, Egawa N, et al. MRCP of congenital pancreaticobiliary malformation. Abdom Imaging, 2007, 32(1): 129-133.
22. Chen YK, Pleskow DK. SpyGlass single-operator peroral cholangiopancreatoscopy system for the diagnosis and therapy of bile-duct disorders: a clinical feasibility study (with video). Gastrointest Endosc, 2007, 65(6): 832-841.
23. Saito T, Terui K, Mitsunaga T, et al. Role of pediatric endoscopic retrograde cholangiopancreatography in an era stressing less-invasive imaging modalities. J Pediatr Gastroenterol Nutr, 2014, 59(2): 204-209.
24. Yoo KS, Lehman GA. Endoscopic management of biliary ductal stones. Gastroenterol Clin North Am, 2010, 39(2): 209-227.
25. Stavropoulos S, Larghi A, Verna E, et al. Intraductal ultrasound for the evaluation of patients with biliary strictures and no abdominal mass on computed tomography. Endoscopy, 2005, 37(8): 715-721.
26. Farrell RJ, Agarwal B, Brandwein SL, et al. Intraductal US is a useful adjunct to ERCP for distinguishing malignant from benign biliary strictures. Gastrointest Endosc, 2002, 56(5): 681-687.
27. Jan YY, Chen HM, Chen MF. Malignancy in choledochal cysts. Hepatogastroenterology, 2000, 47(32): 337-340.
28. Roskams T, Desmet V. Embryology of extra- and intrahepatic bile ducts, the ductal plate. Anat Rec (Hoboken), 2008, 291(6): 628-635.
29. Sato Y, Ren XS, Nakanuma Y. Caroli’s disease: current knowledge of its biliary pathogenesis obtained from an orthologous rat model. Int J Hepatol, 2012, 2012: 107945. doi: 10.1155/2012/107945.
30. Drenth JP, Chrispijn M, Bergmann C. Congenital fibrocystic liver diseases. Best Pract Res Clin Gastroenterol, 2010, 24(5): 573-584.
31. Ponsky LE, Geisinger MA, Ponsky JL, et al. Contemporary “urologic” intervention in the pancreaticobiliary tree. Urology, 2001, 57(1): 21-25.
32. Amano H, Shirota C, Tainaka T, et al. Late postoperative complications of congenital biliary dilatation in pediatric patients: a single-center experience of managing complications for over 20 years. Surg Today, 2021, 51(9): 1488-1495.
33. Koshinaga T, Inoue M, Ohashi K, et al. Persistent biliary dilatation and stenosis in postoperative congenital choledochal cyst. J Hepatobiliary Pancreat Sci, 2011, 18(1): 47-52.

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A new classification of congenital biliary dilatation—HUAXI CBD classification

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