Timing of Operation in Children With a Prenatal Diagnosis of Choledochal Cyst：A Single-center Prospective Study
Phase: NA
Conditions: Choledochal Cyst
Sponsor: Guangzhou Women and Children's Medical Center

 Guangzhou Women and Children's Medical Center 
 
 1
 
 
 
 
Timing of operation in children with a prenatal 
diagnosis of choledochal cyst：A single-center 
prospective study 
 
Xisi Guan 
Department of Neonatal Surgery, Guangzhou Women and Children's Medical Center, 
Guangzhou, Guangdong, China 
2022-10-23 
 
 
 
 
 
 
 

 Guangzhou Women and Children's Medical Center 
 
 2
1、Background 
A choledochal cyst (CDC) is a congenital anomaly of the biliary system, which is more 
common in the Asian population [1]. If a CDC is not diagnosed and treated promptly, it 
often leads to a series of serious complications, including cholangitis, cyst rupture, 
cholestatic cirrhosis, and even cholangiocarcinoma. Infants with a postnatal diagnosis 
of CDC often present with symptoms, and to avoid the occurrence of serious 
complications, operative correction should be performed as soon as possible when their 
clinical conditions allow. However, in the current era with the improvement of prenatal 
screening technology, an increasing number of choledochal cysts are diagnosed 
prenatally in the fetus. In developed countries, as many as 15% of choledochal cysts are 
found before birth [2-5]. Some of these children receive intervention when they are 
asymptomatic at an early stage, while some have progressed to CDC-related symptoms 
before operative correction. The timing of operation for children with a prenatal 
diagnosis of CDC remains controversial. Redka et al. believed that infants with an 
antenatal diagnosis of CDC needed to be followed closely with ultrasonography and 
biochemical liver function tests and undergo elective reconstructive surgery at 3 months 
of age [6]. In contrast, Diao et al. performed a prospective cohort study on asymptomatic 
children with a prenatal diagnosis of CDC and divided the children into an early (≤ 1 
month) operative group and a late (> 1 month) operative group. They found that the 
extent of liver fibrosis was greater in the late operative group, and the postoperative 
recovery was statistically significantly delayed in patients with delayed surgery [7]. 
Therefore, the authors recommended that operative correction should be performed in 
the neonatal period. In addition, Japanese clinical guidelines for pancreaticobiliary 
malformations suggest that symptomatic neonates and infants should be operated on as 
soon as possible. Elective operations should be considered for asymptomatic cases at 
approximately 3–6 months of age along with postnatal monitoring of organ functions, 
primarily of the liver, to determine if laboratory symptoms develop to warrant earlier 
intervention [8]. Our previous study [9] showed that it is more advantageous to receive 
surgical treatment in the asymptomatic period for patients with prenatally diagnosed 
CDC. In addition, the age at operation (months) appears to be unrelated to intraoperative 
and postoperative complications, which is distinct from previous studies. More 
interestingly, we found that a specific cyst size (length > 5.2 cm and width > 4.1 cm) 

 Guangzhou Women and Children's Medical Center 
 
 3
suggested that clinical symptoms might appear and that the surgery should be performed 
as soon as clinically safe to proceed.  
2、Objective 
We tried to select the operation time according to the cyst size and evaluate the treatment 
effect. 
3、Study design 
The follow-up process from antenatal to perinatal was established, and the maximum 
diameter of postnatal cyst was used as the standard for the selection of surgical 
opportunity. The curative effect was evaluated by comparing with previous treatment 
schemes. 
（1）Inclusion Criteria： 
· Infants with a prenatal and postnatal diagnosis with CDC 
· Prenatal and postnatal hepatobiliary ultrasound data were complete 
· Age of visit < 3 months 
         Exclusion criteria： 
· Unable to tolerate surgery after birth 
（2）Flow Chart for prenatal diagnosis of choledochal cyst 
 
 
 
 
 
 
 

 Guangzhou Women and Children's Medical Center 
 
 4
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
  
prenatal ultrasonography  
（ages of 22-24 weeks）  
prenatal ultrasonography  
（ages of 28-32 weeks）  
prenatal ultrasonography  
（ages of 36-38 weeks）  
ultrasonography and biochemical tests 
（1 week after birth）  
＞5cm 
＜5cm and DBIL＜20%TBIL 
ultrasonography and biochemical tests 
（2 weeks after birth） 
ultrasonography and biochemical tests 
（1month after birth） 
＜5cm and DBIL＜20%TBIL 
＜5cm and DBIL＜20%TBIL 
ultrasonography and biochemical tests 
（2 months after birth） 
＜5cm and DBIL＜20%TBIL 
ultrasonography and biochemical tests 
（3 months after birth） 
Surgery (laparoscopic-assisted CDC excision and hepaticojejunostomy) 
＞5cm 
＞5cm 
＞5cm 

 Guangzhou Women and Children's Medical Center 
 
 5
Prenatal ultrasonography [10] ： 
（1）visibility of the right hepatic artery (RHA) 
（2）The maximum lumen length and maximum width of the GB and cyst from inner 
wall to inner ,as well as the wall thickness of the GB 
4、Follow-up scheme 
Follow-up scheme during the post-operative period included 1 week post-operative 
day, 1 month, 3-month, 6-month, then every year visits thereafter. 
 Follow-up Patients’follow-up after surgery mainly consisted of ultrasonography 
and biochemical tests (alanine aminotransferase (ALT), aspartate aminotransferase 
(AST), γ-glutamyl transpeptidase (GGT), and direct bilirubin (DBIL)) 
 
5、Outcomes and Efficacy Evaluation 
（1）For the case–control studies, each patient treated with the new protocol was 
matched with two control patients treated with old protocol. Cases and controls were 
matched by all of the following: (1) gestational age at birth (±2 weeks), (2) birth 
weight (±0.5 kg), (3) gender, (4) Age of visit < 3 months, (5)  All patients received 
laparoscopic-assisted CDC excision and hepaticojejunostomy. 
（2）Old protocol [8]： symptomatic neonates and infants should be 
operated on as soon as possible. Elective operations should be considered for 
asymptomatic cases at approximately 3–6 months of age along with postnatal 
monitoring of organ functions, primarily of the liver, to determine if laboratory 
symptoms develop to warrant earlier intervention 
（3）Comparison of baseline material of patients in the two groups 
The median estimated gestational age (EGA) , cyst diameter at the initial time of 
detection of the cyst, gestational age, cesarean rates, mean birth weight, sex ratio, age 
at operation in months, weight in kg at operation, type of CDC. 
（4）Outcomes and Efficacy Evaluation 
·   Comparison of preoperative complications 

 Guangzhou Women and Children's Medical Center 
 
 6
    preoperative symptom rate, cyst rupture, preoperative laboratory results performed 
within 1 week before the operation（ALT,AST，GGT, DBIL, TBIL, DBIL/TBIL） 
· 
Comparison of intraoperative conditions between the 2 groups. 
operative time in minutes, Body weight-corrected blood loss in ml/kg 
· 
Comparison of preoperative complications 
bile leakage、anastomotic stricture、bowel obstruction、pancreatic  
fistula、incision dehiscence 
6、References 
[1] Lipsett P A, Pitt H A . Surgical treatment of choledochal cysts[J]. Journal of 
Hepato-Biliary-Pancreatic Surgery, 2003, 10(5):352-359. 
[2] Lugo-Vicente HL. Prenatally diagnosed choledochal cysts: Observation or early 
surgery?[J]. Journal of Pediatric Surgery, 1995, 30(9):1288. 
[3] Okada T,  Sasaki F,  Ueki S, et al. Postnatal management for prenatally diagnosed 
choledochal cysts[J]. Journal of Pediatric Surgery, 2004, 39(7):1055-1058.  
[4] Tanaka H,  Sasaki H,  Wada M, et al. Postnatal management of prenatally 
diagnosed biliary cystic malformation.[J]. Journal of Pediatric Surgery, 2015, 
50(4):507-510. 
[5] Bancroft J D,  Bucuvalas J C,  Ryckman F C, et al. Antenatal diagnosis of 
choledochal cyst.[J]. Journal of Pediatric Gastroenterology & Nutrition, 1994, 
18(2):142-5. 
[6] Redkar R,  Davenport M,  Howard E R . Antenatal diagnosis of congenital 
anomalies of the biliary tract[J]. Journal of Pediatric Surgery, 1998, 33(5):700. 
[7] Diao M,  Li L, Cheng W . Timing of surgery for prenatally diagnosed 
asymptomatic choledochal cysts: a prospective randomized study[J]. Journal of 
Pediatric Surgery, 2012, 47(3):506-512. 
[8] Kamisawa T, Ando H, Suyama M, et al. Japanese clinical practice guidelines for 
pancreaticobiliary maljunction[J]. J Gastroenterol, 2012,47(7):731-759. 
[9] Xisi Guan #, Junting Li , Zhe Wang , Jixiao Zeng , Wei Zhong , Jiakang Yu. 
Timing of operation in children with a prenatal diagnosis of choledochal cyst: A 
single-center retrospective study. J Hepatobiliary Pancreat Sci.2022 Apr 18. doi: 
10.1002/jhbp.1155 

 Guangzhou Women and Children's Medical Center 
 
 7
[10] Chen L ,  He F ,  Zeng K , et al. Differentiation of cystic biliary atresia and 
choledochal cysts using prenatal ultrasonography.[J]. ULTRASONOGRAPHY, 2021.