Skip to main content

Advertisement

SpringerLink
Log in

Long-term outcome for children undergoing open hepatico-jejunostomy for choledochal malformations: a 43-year single-center experience

  • Original Article
  • Published:
Pediatric Surgery International Aims and scope Submit manuscript

Abstract

Purpose

To report on our 43-year single-center experience with children operated on for Choledochal Malformations (CMs), focusing on long-term results and Quality of life (QoL).

Materials and methods

All consecutive pediatric patients with CMs who underwent surgical treatment at our center between October 1980 and December 2022 were enrolled in this retrospective study. We focused on long-term postoperative complications (POCs), considered to be complications arising at least 5 years after surgery. We analyzed QoL status once patients reached adulthood, comparing the results with a control group of the same age and sex.

Results

One hundred and thirteen patients underwent open excision of CMs with a Roux-en-Y hepaticojejunostomy (HJ). The median follow-up was 8.95 years (IQR: 3.74–24.41). Major long-term POCs occurred in six patients (8.9%), with a median presentation of 11 years after surgery. The oldest patient is currently 51. No cases of biliary malignancy were detected. The QoL of our patients was comparable with the control group.

Conclusion

Our experience suggests that open complete excision of CMs with HJ achieves excellent results in terms of long-term postoperative outcomes. However, since the most severe complications can occur many years after surgery, international cooperation is advisable to define a precise transitional care follow-up protocol.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

Data availability

Data from this research may be available if requested to contribute to the development of an international register.

Abbreviations

CMs:

Choledochal malformations

QoL:

Quality of life

POCs:

Postoperative complications

HJ:

Hepaticojejunostomy

RHJ:

Roux-en-Y hepaticojejunal anastomosis

PBM:

Pancreatico-biliary maljunction

LBPCC:

Long bilio-pancreatic common channel

IHBD:

Intrahepatic bile duct

HD:

Hepatico-duodenostomy

US:

Ultrasound

CT:

Computed tomography scan

MRCP:

Magnetic resonance cholangio-pancreatography

ERCP:

Endoscopic retrograde cholangio-pancreatography

UDCA:

Ursodeoxycholic acid

CBD:

Common bile duct

CHD:

Common hepatic duct

IEE:

Intraoperative endoscopic evaluation

ASBO:

Adhesive small bowel obstruction

CG:

Control group

DGBR:

Duodenogastric bile reflux

References

  1. Soares K, Arnaoutakis D, Kamel I et al (2014) Choledochal cysts: presentation, clinical differentiation, and management. J Am Coll Surg 219(6):1167–1180

    Article  PubMed  PubMed Central  Google Scholar 

  2. Parolini F, Davenport M (2018) Choledochal malformation: terminology and aetiology. Ped Surg Int 34(1):117–118

    Article  Google Scholar 

  3. Atkinson J, Davenport M (2014) Controversies in choledochal malformation. S Afr Med J 104(11 Pt 2):816–819

    Article  CAS  PubMed  Google Scholar 

  4. Ishibashi H, Shimada M, Kamisawa T et al (2017) (JSCBD) Japanese clinical practice guidelines for congenital biliary dilatation. J Hepatobiliary Pancreatic Sci 24(1):1–16

    Article  Google Scholar 

  5. Hukkinen M, Koivusalo A, Lindahl H et al (2014) Increasing occurrence of choledochal malformations in children: a single-center 37-year experience from Finland. Scand J Gastroenterol 49(10):1255–1260

    Article  PubMed  Google Scholar 

  6. Parolini F, Davenport M (2018) Choledochal malformation and pancreaticobiliary maljunction: a European perspective. Springer Nature, Singapore Ltd, pp 71–79

    Google Scholar 

  7. van den Eijnden M, Kleine R, de Blaauw I et al (2017) Choledochal malformation in children: lessons learned from a dutch national study. World J Surg 41(10):2631–2637

    Article  PubMed  PubMed Central  Google Scholar 

  8. Madadi-Sanjani O, Wirth T, Kuebler J et al (2019) Choledochal cyst and malignancy: a plea for lifelong follow-up. Eur J Pediatr Surg 29(2):143–149

    Article  PubMed  Google Scholar 

  9. Makin E, Davenport M (2012) Understanding choledochal malformation. Arch Dis Child 97(1):69–72

    Article  PubMed  Google Scholar 

  10. Koga H, Murakami H, Ochi T et al (2019) Comparison of robotic versus laparoscopic hepaticojejunostomy for choledochal cyst in children: a first report. Pediatr Surg Int 35:1421–1425

    Article  PubMed  Google Scholar 

  11. Liem N, Pham H, le Dung A et al (2012) Early and intermediate outcomes of laparoscopic surgery for choledochal cysts with 400 patients. J Laparoendosc Adv Surg Tech A 22(6):599–603

    Article  PubMed  Google Scholar 

  12. Yeung F, Fung A, Chung P et al (2020) Short-term and long-term outcomes after Roux-en-Y hepaticojejunostomy versus hepaticoduodenostomy following laparoscopic excision of choledochal cyst in children. Surg Endosc 34(5):2172–2177

    Article  PubMed  Google Scholar 

  13. Todani T, Watanabe Y, Narusue M et al (1977) Congenital bile duct cysts: classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 134:263–269

    Article  CAS  PubMed  Google Scholar 

  14. Wilde J, Calinescu A, Wildhaber B (2018) Perioperative complications in neonatal surgery: biliary atresia and choledochal malformations. Eur J Pediatr Surg 28(2):156–162

    Article  PubMed  Google Scholar 

  15. Abu-El-Haija MJ, Kumar S, Quiros J et al (2018) Management of acute pancreatitis in the pediatric population: a clinical report from the North American society for pediatric gastroenterology. Hepatol Nutr Pancreas Committee Ped Gastroenterol Nutr 66(1):159–176

    Google Scholar 

  16. Dindo D, Demartines N, Clavien P (2004) Classification of surgical complications: a new proposal with evaluation in a cohort of 6336 patients and results of a survey. Ann Surg 240(2):205–213

    Article  PubMed  PubMed Central  Google Scholar 

  17. Whoqol Group (1998) Development of the world health organization WHOQOLBREF quality of life assessment. Psychol Med 28(3):551–558

    Article  Google Scholar 

  18. Lilly JR (1978) Total excision of choledochal cyst. Surg Gynecol Obstet 146(2):254–256

    CAS  PubMed  Google Scholar 

  19. Takeshita N, Ota T, Yamamoto M (2011) Forty-year experience with flow-diversion surgery for patients with congenital choledochal cysts with pancreaticobiliary maljunction at a single institution. Ann Surg 254(6):1050–1053

    Article  PubMed  Google Scholar 

  20. Mukai M, Kaji T, Masuya R et al (2018) Long-term outcomes of surgery for choledochal cysts: a single-institution study focusing on follow-up and late complications. Surg Today 48(9):835–840

    Article  PubMed  Google Scholar 

  21. Zheng Q, Zhang S, Ge L et al (2020) Investigation into multi-centre diagnosis and treatment strategies of biliary atresia in mainland China. Pediatr Surg Int 36(7):827–833

    Article  PubMed  Google Scholar 

  22. Yamataka A, Kobayashi H, Shimotakahara A et al (2003) Recommendations for preventing complications related to Roux-en-Y hepatico-jejunostomy performed during excision of choledochal cyst in children. J Pediatr Surg 38(12):1830–1832

    Article  PubMed  Google Scholar 

  23. Todani T, Watanabe Y, Urushihara N et al (1995) Biliary complications after excisional procedure for choledochal cyst. J Pediatr Surg 30:478–481

    Article  CAS  PubMed  Google Scholar 

  24. Ando H, Ito T, Kaneko K et al (1995) ongenital stenosis of the intrahepatic bile duct associated with choledochal cysts. Am Coll Surg 181(5):426–430

    CAS  Google Scholar 

  25. Uno K, Tsuchida Y, Kawarasaki H (1996) Development of intrahepatic cholelithiasis long after primary excision of choledochal cysts. J Am Coll Surg 183(6):583–588

    CAS  PubMed  Google Scholar 

  26. Todani T, Watanabe Y, Toki A, et al (2002) Hilar duct carcinoma developed after cyst excision followed by hepaticoduodenostomy. In: Koyanagi Y, Aoki T (eds) Pancreaticobiliary maljunction, Tokyo, Igaku tosho shuppan, pp. 17–21

  27. Singham J, Yoshida E, Scudamore C (2010) Choledochal cysts. Part 3 of 3: management. Can J Surg 53(1):51–56

    PubMed  PubMed Central  Google Scholar 

  28. Ariake K, Unno M, Yoshida H (2020) Risk factors and characteristics of young patients with the biliary tract carcinoma: results of a project study for biliary surgery by the Japanese Society of Hepato-Biliary-Pancreatic Surgery. J Hepatobiliary Pancreat Sci 27(9):571–580

    Article  PubMed  PubMed Central  Google Scholar 

  29. Tanaka H, Sasaki H, Wada M et al (2015) Postnatal management of prenatally diagnosed biliary cystic malformation. J Pediatr Surg 50(4):507–510

    Article  PubMed  Google Scholar 

  30. Okada T, Sasaki F, Ueki S et al (2004) Postnatal management for prenatally diagnosed choledochal cysts. J Pediatr Surg 39(7):1055–1058

    Article  PubMed  Google Scholar 

  31. Chen C (2003) Clinical and operative findings of choledochal cysts in neonates and infants differ from those in older children. Asian J Surg 26(4):213–217

    Article  PubMed  Google Scholar 

  32. Narayanan S, Chen Y, Narasimhan K et al (2013) Hepaticoduodenostomy versus hepaticojejunostomy after resection of choledochalcyst: a systematic review and meta-analysis. J Pediatr Surg 48(11):2336–2342

    Article  PubMed  Google Scholar 

  33. Stringer M (2017) Laparoscopic management of choledochal cysts: is a keyhole view missing the big picture? Pediatr Surg Int 33(6):651–655

    Article  PubMed  Google Scholar 

  34. Shimotakahara A, Yamataka A, Yanai T et al (2005) Roux-en-Y hepaticojejunostomy or hepaticoduodenostomy for biliary reconstruction during the surgical treatment of choledochal cyst: which is better? Pediatr Surg Int 21(1):5–7

    Article  PubMed  Google Scholar 

  35. Hamada Y, Hamada H, Shirai T et al (2017) Duodenogastric regurgitation in hepaticoduodenostomy after excision of congenital biliary dilatation (choledochal cyst). J Pediatr Surg 52(10):1621–1624

    Article  PubMed  Google Scholar 

  36. Tan H, Shankar K, Ford W (2003) Laparoscopic resection of type I choledochal cyst. Surg Endosc 17(9):1495

    Article  CAS  PubMed  Google Scholar 

  37. Takada K, Hamada Y, Watanabe K et al (2005) Duodenogastric reflux following biliary reconstruction after excision of choledochal cyst. Pediatr Surg Int 21:1–4

    Article  CAS  PubMed  Google Scholar 

  38. Ten Hove A, de Meijer V, Hulscher J et al (2018) Meta-analysis of risk of developing malignancy in congenital choledochal malformation. Br J Surg 105(5):482–490

    Article  PubMed  Google Scholar 

  39. He X, Wang L, Liu W et al (2014) The risk of carcinogenesis in congenital choledochal cyst patients: an analysis of 214 cases. Ann Hepatol 13(6):819–826

    Article  PubMed  Google Scholar 

  40. Todani T, Watanabe Y, Urushihara N et al (1994) Choledochal cyst, pancreatobiliary malunion, and cancer. J Hepatobiliary Pancreat Surg 1:247–251

    Article  Google Scholar 

  41. Watanabe Y, Toki A, Todani T (1999) Bile duct cancer developed after cyst excision for choledochal cyst. J Hepatobiliary Pancreat Surg 6:207–212

    Article  CAS  PubMed  Google Scholar 

  42. Ono S, Fumino S, Shimadera S et al (2010) Long-term outcomes after hepaticojejunostomy for choledochal cyst: a 10- to 27-year follow-up. J Pediatr Surg 45:376–378

    Article  PubMed  Google Scholar 

  43. Tajiri T, Tate G, Inagaki T et al (2004) Mucinous cystadenoma of the pancreas 17 years after excision of gallbladder because of a choledochal cyst. J Gastroenterol 39(02):181–187

    Article  PubMed  Google Scholar 

  44. Ng D, Chiow A, Poh W et al (2016) 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 2(1):60

    Article  PubMed  PubMed Central  Google Scholar 

  45. Otani T, Shioiri T, Mishima H et al (2005) Bile duct schwannoma developed in the remnant choledochal cyst-a case associated with total agenesis of the dorsal pancreas. Dig Liver Dis 37(09):705–708

    Article  CAS  PubMed  Google Scholar 

  46. Baba T, Mukai M, Masuya R et al (2019) The long-term health-related quality of life in patients operated for choledochal cyst. Pediatr Surg Int 35(5):539–546

    Article  PubMed  Google Scholar 

  47. Shen H, Xu M, Zhu H et al (2015) Laparoscopic versus open surgery in children with choledochal cysts: a meta-analysis. Pediatr Surg Int 31:529–534

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

We would like to express our gratitude to Professor Yamataka Atsuyuki (Full Professor of Pediatric Surgey. Juntendo University; Tokyo) and Professor Nicola De Angelis (Full Professor of General Surgey. University Paris Cite; Paris) for their suggestion in reviewing this study.

Author information

Authors and Affiliations

  1. Department of Pediatric Surgery, “Spedali Civili” Children’s Hospital, Brescia, Italy

    M. V. Stern, G. Boroni, F. Parolini, F. Torri & D. Alberti

  2. European Reference Network for Hepatological Diseases (ERN RARE-LIVER), Brescia, Italy

    G. Boroni, F. Parolini & D. Alberti

  3. Department of Molecular and Translational Medicine, Unit of Biostatistics and Bioinformatics, University of Brescia, Brescia, Italy

    S. Calza

  4. Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy

    D. Alberti

Authors
  1. M. V. Stern
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. G. Boroni
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. F. Parolini
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. F. Torri
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. S. Calza
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. D. Alberti
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

MVS and DA conceptualized and designed the study. GB, FP, FT, MVS and DA were involved in medical and surgical care of the patients. GB and DA prepared figures and video. MVS collected the clinical data. MVS, GB and FP contacted and retrieved adult patient data. SC edited the statistic of the study. All authors contributed equally to preparation of the manuscript, reviewed and approved the final manuscript as submitted.

Corresponding author

Correspondence to M. V. Stern.

Ethics declarations

Competing interests

The authors declare no competing interests.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Supplementary Information

Below is the link to the electronic supplementary material.

Supplementary file1 (MP4 7497 KB)

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Stern, M.V., Boroni, G., Parolini, F. et al. Long-term outcome for children undergoing open hepatico-jejunostomy for choledochal malformations: a 43-year single-center experience. Pediatr Surg Int 40, 36 (2024). https://doi.org/10.1007/s00383-023-05622-8

Download citation

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1007/s00383-023-05622-8

Keywords

Search

Navigation