Presentation and Management of Choledochal Cysts in Children: The Experience of a Single Institute From a Developing Country Choledochal Cysts in Children
Main Article Content
Children, choledochal cyst, hepaticojejunostomy, biliary ducts malformations
BACKGROUND: Choledochal cysts are rare congenital biliary tract malformations. Presentation is variable based on age at presentation and type of the cyst. Total cyst excision with biliary reconstruction is the treatment of choice.
AIM OF THE STUDY: to present our experience with the presentation and management of these rare malformations with literature review.
MATERIALS AND METHODS: A retrospective analysis of a series of seven patients managed during the period from June 2015 to July 2020 in our institution. Recorded clinical data included sex, age at presentation, mode of presentation, diagnostic modality, cyst type and histopathology, preoperative course, operative procedure, and intraoperative and postoperative complications.
RESULTS: Of the patients enrolled in the study, 5 (71.4%) were female and 2 (28.6%) were male. Age at presentation ranged from 7-36 months. Abdominal pain was the most frequent manifestation and was recorded in 6 (85.7%) patients. The diagnosis was confirmed in all patients with MRCP. Cysts were type I in all (100%) patients. Open total cyst excision with Reux-en-Y hepaticojejunostomy was the standard procedure in all patients. No significant intraoperative vascular or structural injury was recorded. Early postoperative complications were recorded in only 1 (14.3%) patient. All patients achieved good clinical outcome, and none have shown significant findings on long term follow up.
CONCLUSIONS: Choledochal cysts are rare congenital malformations with variable presentation. Total cyst excision with hepaticojujenostomy is a standard therapeutic option and is associated with an excellent outcome and decreased incidence of long term risks.
KEYWORDS: Choledochal cyst, Children, Hepaticojujenostomy
2- Forny DN, Ferrante SM, Da Silveira VG, et al. Choledochal cyst in childhood: review of 30 cases. Rev Col Bras Cir. 2014. 41(5): 331-335.
3- Dabbas N, Davenport M. Congenital choledochal malformation: not just a problem for children. Ann R Coll Surg Engl 2009; 91: 100-5.
4- Silva-Baez H, Coello-Ramírez P, Ixtabalán-Escalante EM, Sotelo-Anaya E, Gallo-Morales M, Cordero-Estrada E, et al. Treatment of choledochal cyst in a pediatric population. A single institution experience of 15-years. Case series. Ann Med Surg. 2016;5:81–5.
5- Holocomb G. W., Murphy J. P. and St. Peter S. D. Holcomb and Ashcraft’s pediatric surgery, 7th edition, 2020
6- Hassan HS. Changing trends in the management of choledochal cysts in children in an Egyptian institution. Ann Pediatr Surg. 2016;12(3):94–99.
7- H.-C. Lee, “Ultrasonography of choledochal cysts in children,” Journal of Medical Ultrasound, vol. 15, no. 3, pp. 191–196, 2007
8- Soares KC, Kim Y, Spolverato G, Maithel S, Bauer TW, Marques H, Sobral M, Knoblich M, Tran T, Aldrighetti L, Jabbour N, Poultsides GA, Gamblin TC, Pawlik TM. Presentation and Clinical Outcomes of Choledochal Cysts in Children and Adults: A Multi-institutional Analysis. JAMA Surg. 2015;150:577–584.
9- Singham J, Schaeffer D, Yoshida E, Scudamore C. Choledochal cysts: analysis of disease pattern and optimal treatment in adult and paediatric patients. HPB (Oxford) 2007;9:383–387.
10- Xie X, Li K, Wang J, Wang C, Xiang B (2020) Comparison of pediatric choledochal cyst excisions with open procedures, laparoscopic procedures and robot-assisted procedures: a retrospective study. SurgEndosc 34(7):3223–3231.
11- Narayanan S.A. , Chen Y. , Narasimham K.L. , Cohen R.C. Hepaticoduodenostomy versus hepaticojejunostomy after resection of choledochal cyst: a systematic review and meta-analysis . J. Pediatr. Surg., 48 (2013), pp. 2336-2342
12- Chan EKW, Lee KH, Wong VHY, Tsui BSY, Wong BS, Pang KK, et al. Laparoscopic management of choledochal cyst in infants and children: a review of current practice. Surg Pract. 2018;22:131-7.