Risk Factors for Postoperative Stone Formation in Pediatric Choledochal Cysts: A Study of 457 Cases

doi:10.21203/rs.3.rs-4711435/v1

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Risk Factors for Postoperative Stone Formation in Pediatric Choledochal Cysts: A Study of 457 Cases

https://doi.org/10.21203/rs.3.rs-4711435/v1

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Background: Postoperative bile duct stones, including intrahepatic bile duct (IHD) stones and remnant distal common bile duct (remnant intrapancreatic common bile duct, RIPD) or common channel duct (CCD) stones, cause long-term complications following choledochal cyst excision. We aimed to retrospectively review occurrence, associated factors, and treatment of postoperative bile duct stones.

Methods: Records of 457 pediatric patients who underwent choledochal cyst excision at Asan Medical Center (1992-2021) were retrospectively reviewed. Data on cholelithiasis, operation, and outcomes were analyzed.

Results: Overall, 457 pediatric patients underwent choledochal cyst excision, with 21 developing intrahepatic duct (IHD) stones, primarily associated with Todani type IVa cysts, especially Tsuchida types 2 or 3, which are often linked to IHD dilation and upstream stenosis. Patients with RIPD/CCD stones were all identified with complicated Komi types, and the impact of pancreas divisum itself was unclear. Early surgery even before six months of age is associated with improved prognosis regarding the occurrence of IHD stones, but not related with RIPD/CCD stones. Early surgery even before six months of age is associated with improved prognosis regarding the occurrence of IHD stones, but not related with RIPD/CCD stones.

Conclusions: This is the largest retrospective study comprising 21 IHD and 18 RIPD/CCD stones from 457 pediatric patients with choledochal cysts and including 21 cases of IHD stones and 18 cases of RIPD/CCD stones as well as their clinical and treatment outcomes to date in this aspect. Our findings suggest that a more systematic and long-term follow-up is needed for patients with choledochal cysts CC postoperatively.

Health sciences/Diseases/Gastrointestinal diseases

Health sciences/Diseases

Health sciences/Pathogenesis

bile duct stone

choledochal cyst

intrahepatic duct stone

intrapancreatic common bile duct stone

pediatric

A choledochal cyst (CC) refers to an abnormal dilation of the biliary tract, affecting both intra- and extrahepatic bile ducts. CCs are highly prevalent in Asian populations, with an estimated incidence of approximately 1 in 1,000 individuals.¹ The Todani classification, based on morphology, categorizes CCs into five types, of which type I is the most frequent (75–85%).^{2, 3}

Distinguishing between Todani type IVa and type I accompanied by intrahepatic bile duct (IHD) dilatation is difficult but crucial. In patients with type I CC, obstruction of the distal bile duct due to protein plugs, can lead to subsequent IHD dilatation, which typically resolves after at least 1 year.

Although the etiology of CC formation is unclear, congenital weakness in the bile duct wall, genetic predisposition, bile duct obstruction, abnormal pressure, structural changes, and chronic inflammation may be involved.^{1, 4} Treatment involves resection of the dilated bile ducts and hepaticojejunostomy. With the advent of minimally invasive surgery, most procedures are now performed laparoscopically or robotically.

However, rare, long-term complications, such as cholangitis, pancreatitis, lithiasis, biliary tract malignancy, cholangiocarcinoma (CCA), and pancreatic carcinoma, may occur.³ Specifically, bile duct stenosis and subsequent cholangitis or pancreatitis are associated with progressive duct stricture and carcinogenesis.^{5, 6} During the process described above, stones can occur in the IHD, the remnant intrapancreatic common bile duct (RIPD), and the common channel duct (CCD). IHD stones are the most common late complication of CC surgery, occurring in 2.7–11.0% of the patients and attributed to incomplete excision, anastomotic stricture, residual debris, etc.⁷ Todani type IVa and primary IHD strictures near the hilum are risk factors for IHD stricture and bile stasis.

Despite complete surgical excision of CCs, CCA has been reported in several studies, with an incidence of 0.7–5.4%.⁸ IHD stones are believed to increase the risk of carcinogenesis, possibly due to chronic inflammation-induced dysplasia occurring concurrently with stone formation. Furthermore, anomalous pancreaticobiliary ductal union (APBDU) and pancreatic divisum are risk factors for RIPD/CCD, pancreatitis, and pancreatic duct stricture.⁹ However, other factors contributing to these complications are largely unknown.

Studies on a small number of patients from single institutions with 10–20 years of follow-up revealed that long-term complications are relatively rare.¹⁰ In our study, we aimed to investigate the incidence and characteristics of postoperative bile duct stones following conventional surgical procedures.

Patients

This retrospective study was conducted for 455 pediatric patients who had CC excision and Roux-en-Y hepaticojejunostomy (HJ) procedures at Asan Medical Center between 1995 and 2021 (Fig. 1). The specific characteristics of IHD and RIPD/CCD stones in imaging studies were presented in Supplementary Tables 1 and 2. Two patients who had surgery at other centers but were transferred for postoperative choledocholithiasis management were included. Thus, 457 patients were included in the study. IHD and RIPD/CCD stones were diagnosed in 21 and 18 patients, respectively. Initial classification was performed based on the interpretation by a pediatric radiologist with > 5 years of experience in pediatric diagnostic imaging. Patients diagnosed with stones were reevaluated, classifying them according to the Todani classification and Komi classification.^{3, 9} Image analysis was performed on MR images, including magnetic resonance cholangiopancreatography (MRCP).¹¹ Briefly, MRCP includes the following sequences: precontrast axial T1 in- and out-of-phase, axial T2, axial single-shot fat-saturated T2, axial diffusion-weighted imaging, coronal single-shot fat-saturated T2, 3D coronal turbo spin echo with maximal intensity projection image, 2D T2 with thick-slab imaging, and 3D T1-weighted gradient echo sequences in the hepatic arterial/portal venous/delayed phase. The classification method proposed by Tsuchida in 2002 was also incorporated. Comprehensive data, including demographic information, surgical procedures, and subsequent follow-ups of each patient, were collected in addition to anatomical classification. The follow-up period was determined based on the date of the most recent outpatient visit. Complications were assessed using the Clavien–Dindo classification system. This study adhered to the principles of the Declaration of Helsinki and was approved by the Institutional Review Board of Asan Medical Center (approval No: 2023 − 0840). Informed consent was waived due to the retrospective nature of the study.

Surgical procedure

Open or minimally invasive approach was applied based on the surgeon’s preference and timing. Right subcostal incision was used as an open approach, and a three- or four-port system was utilized as minimally invasive approach. Once CC was identified during exploration, both the cystic duct and artery were found and tied off before cyst removal, serving as landmarks for dissection.

In some cases, the cyst was cut horizontally to prevent artery damage to confirm the hepatic artery during dissection. Proximal resection was done at the level of the normal common bile duct to ensure complete cyst removal. Distally, the bile duct was dissected toward the inside of the pancreatic head. Upon confirming the normal duct, it was cut and tied off using a Hem-o-lock clip.

An end-to-side jejunojejunal (JJ) anastomosis was made approximately 40 cm distal to the Treitz ligament, leaving a 40 cm length of jejunum between the JJ and HJ anastomoses. The HJ anastomosis was formed in a retrocolic manner. After ensuring no leaks at the HJ anastomosis site, a drain was placed in the posterior HJ, and the operation ended with skin closure.

Statistical analysis

All statistical analyses were conducted using SPSS version 27.0 for Windows (IBM Corp., Armonk, NY, USA), and Student’s t-test was used to compare the means between groups. Regression analysis was employed to test for risk factors. Statistical significance was set at a p-value of < 0.05.

IHD stones were identified in 21 patients (Fig. 1). In four patients, stones developed due to HJ stricture, and 17 (81%) were diagnosed with Todani type IVa-associated IHD stone related to IHD dilatation. Among them, > 50% (n = 9) were classified as Tsuchida type 3, suggesting a proximal IHD stenosis at the bifurcation level. Among the eight patients who had surgical treatment for HJ stricture without stone formation, one was confirmed Todani type IVa. Of the patients with RIPD/CCD stones, 16 could be analyzed with MRI, with nine having concomitant pancreas divisum of Komi type 3C. The next most common type was Komi type 1.

Detailed descriptions of the 21 patients with IHD stones are provided in Table 1. Initial symptoms of stone occurrence were cholangitis in 9 (42.9%) patients and incidental findings on follow-up ultrasound or MR in 8 (38.1%) patients. CC excision was performed after the age of 1 year in 19 (90.5%) patients, and stones were diagnosed at 17.4 ± 6.2 years of age and 122.2 ± 70.6 months after surgery. Figure 2 illustrates the occurrence of IHD stone in the right hepatic duct (n = 9), left hepatic duct (n = 8), and bilateral IHD (n = 3). In one patient with common hepatic duct stones, residual CC was identified, and remnant cyst excision was performed. Treatment for IHD stones primarily involved percutaneous transhepatic cholangioscopy (PTCS, n = 10, 47.6%) and surgical removal (n = 5, 23.8%). In three patients, stone removal under endoscopic retrograde cholangiopancreatography (ERCP) (Fig. 3) was attempted. A pediatric colonoscopy was used to directly access the HJ along the R-en-Y-limb in one patient, and endoscopic ultrasound-guided hepaticogastro/jejunostomy was performed in two for stone removal and stent insertion (Fig. 4). All the patients showed favorable outcomes without complications. With a follow-up period of 41.8 ± 47.9 months from stone diagnosis, five patients are preparing for further treatment due to recurrence or recent diagnosis (Table 1). No CCA associated with IHD stones has been diagnosed in our study population.

Table 1

**Characteristics of patients with intrahepatic bile duct (IHD) stones (**n **= 21).**
	N = 21
Male:female	5:16
Clinical presentation at diagnosis of IHD stones
Cholangitis (abdominal pain and fever)	9 (43.0%)
Asymptomatic	8 (38.1%)
Abdominal pain	3 (14.3%)
Liver abscess	1 (4.8%)
Age at choledochal cyst excision (months)
Neonate (age ≤ 30 days)	2 (9.5%)
Infants (age ≤ 1 year)	0
Children (age ≥ 1 year)	19 (90.5%)
Preoperative intervention	8 (38.1%, ERCP (n = 7), PTBD (n = 1))
Age at choledocholithiasis diagnosis (years, range)	17.4 ± 6.2 (4.5–33.6)
Time from operation to stone occurrence (months, range)	122.2 ± 70.6 (18.6–277.1)
Follow-up duration (months, range)	41.8 ± 47.9 (2.2–155.9)
Outcome
Symptoms: Persisting/resolved/no symptom	5 (one with mild symptoms)/15/1 (23.8%/71.4%/4.8%)
Residual stone: large stones/small stones/no residual stone	3/9/7
Follow-up loss	1 (4.8%)
Plan
Observation (small stones > clear)	4 (19.0%)
Regular follow-up (clear > small stones)	8 (38.1%)
Medical treatment	1 (4.8%)
Planning PTCS	4 (19.0%)
Planning endoscopic HJ	2 (9.5%)
* IHD = intrahepatic bile duct; PTCS = percutaneous transhepatic cholangioscopy; HJ = hepaticojejunostomy.

Majority of the 18 patients with RIPD/CCD stones experienced pancreatitis (n = 13, 72.2%, Table II). Stones were observed in 45% of patients showed postoperative dilatation of RIPD/CCD (18/40). CC excision was performed after the age of 1 year in all patients, and stones occurred at the age of 10.5 ± 5.4 years and 65.6 ± 72.3 months after surgery. RIPD/CCD stones were found in various parts of the common channel and residual common bile duct in the pancreas head. In case of pancreas divisum, stones developed in both dorsal and ventral ducts. ERCP was performed in 14 (77.8%) patients, and for three symptom-free (16.7%) patients, observation was performed. Repeated ERCP procedures were required for most patients, and endoscopic retrograde pancreatic drainage insertion was done in four patients (Fig. 5). All patients showed resolution of symptoms during 72.7 ± 56.3 months of follow-up after stone diagnosis, although ERCP was under consideration in case of symptom recurrence for two patients (Table 2). There were differences in the timing and age at the occurrence of RIPD/CCD stones and IHD stones after surgery, with IHD stones occurring relatively later (Table 3). Subgroup analysis was conducted to identify and evaluate factors associated with stone formation (Table 4). IHD and RIPD/CCD stones occurred less frequently in patients operated on during infancy. For IHD stones, the age at occurrence was significantly higher in patients with stones than without stones (p < 0.01). Linear regression analysis revealed that age at surgery significantly influenced the occurrence of IHD stones (p < 0.001).

Table 2

Characteristics of patients with stone in remnant intrapancreatic common bile duct/common channel duct (RIPD/CCD) (n = 18).
	N = 18
Male:female	4: 14
Clinical presentation at diagnosis of RIPD/CCD stones
Pancreatitis	13 (72.2%)
Asymptomatic	5 (27.8%)
Age at choledochal cyst excision (months)
Neonate (age ≤ 30 days)	0
Infants (age ≤ 1 year)	0
Children (age ≥ 1 year)	18 (100.0%)
Symptoms at the time of choledochal cyst diagnosis
Biliary obstruction	4 (22.2%)
Pancreatitis	5 (27.8%)
Both	9 (50.5%)
Preoperative intervention	11 (61.1%)
Age at choledocholithiasis diagnosis (years)	10.5 ± 5.4 (3.4–18.8)
Time from operation to stone occurrence (months)	65.6 ± 72.3 (0.4-210.3)
Follow-up duration (months)	72.7 ± 56.3 (4.4-212.9)
Outcome
Symptoms: Persisting/no symptom	0/18 (0%/100.0%)
Residual stone: Residual stone/no residual stone/	4/14 (22.2%/77.8%)
Follow-up loss	1 (5.6%)
Plan
Regular follow-up	16 (88.9%)
Further ERCP	2 (11.1%, ERPD insertion in 1 patient)
* RIPD: remnant intrapancreatic common bile duct; CCD: common channel duct; ERCP: Endoscopic retrograde cholangiopancreatography. ERPD: endoscopic retrograde pancreatic drainage

Table 3

The difference in occurrence patterns between RIPD/CCD stones and IHD stones.
	The time between the operation and diagnosis (months)	Age at diagnosis (years)
IHD stone (n = 21)	122.2 ± 70.6	17.45 ± 6.2
RIPD/CCD stone (n = 18)	65.6 ± 72.3	10.5 ± 5.4
p-value	0.018^*	0.001^*
* RIPD: remnant intrapancreatic common bile duct; CCD: common channel duct; IHD: intrahepatic bile duct

Table 4

Factors associated with stone formation: subgroup analysis.
	IHD stone			RIPD/CCD stone
	Yes	No	p-value	Yes	No	p-value
Gender			0.533			0.952
Male	6 (28.6%)	99 (22.7%)		4 (3.8%)	100 (96.2%)
Female	15 (71.4%)	337 (77.3%)		14 (4.0%)	338 (96.0%)
Neonate	2 (9.5%)	57 (13.1%)	0.636	0 (0.0%)	59 (13.4%)	0.096
Infant	2 (9.5%)	132 (30.3%)	0.041^*	1 (5.6%)	133 (30.3%)	0.024^*
OP age (days)	2890.76 ± 1872.23	1315.08 ± 1401.21	0.001^*	1866.11 ± 1514.23	1356.89 ± 1457.66	0.157
* RIPD: remnant intrapancreatic common bile duct; CCD: common channel duct; IHD: intrahepatic bile duct. OP: operation

Bile duct stones occurring after CC excision serve as indicators of chronic inflammation in the biliary tract and perpetuate conditions such as cholangitis or pancreatitis. Ultimately, they can worsen ductal stricture and elevate cancer risk. Therefore, long-term follow-up is required in patients diagnosed them. IHD stones emerge as the most common late complication following CC excision, significantly increasing after > 10 years postoperatively. Takimoto reported that IHD stones developed in 1.7% of patients who underwent CC excision in 2009 but confessed that incidence may increase in their recent report.⁸ In our study, stones were diagnosed 122.2 ± 70.6 months after surgery, and the patient age was 17.4 ± 6.2 years (Table 1). This aligns with previous reports. Ono et al. reported cases of a 14-year-old girl and a 26-year-old man succumbing to cholangiocarcinoma 2 and 26 years after CC excision, respectively, in 2009.^{10, 12} Nishiyama et al. reported a case of cholangiocarcinoma 33 years following complete excision of CC, highlighting the necessity of > 30 years of long-term follow-up.¹³ Nonetheless, considering Ono’s report of a 96% overall survival rate, early diagnosis and treatment of CC can yield favorable outcomes.¹⁰

In 2022, Takimoto et al. reported that postoperative IHD stone occurred in 10.2% of patients. In our study, IHD stones were identified in 4.6% of patients. Takimoto observed that patients who developed IHD stones were significantly younger at surgery, with type IVa being significantly higher in this group. In our study, type IVa was present in 17 (81.0%) patients. When analyzing patients with clearly identified types of choledochal cysts (n = 332), it was significantly higher than the 34 patients (10.9%) without IHD stones (p < 0.001). According to Kemmotsu et al. and Tsuchida et al., anatomical features, such as stricture at the hepatic duct bifurcation site, are associated with the formation of IHD stones.^14–16 Based on this data, Urushihara et al. and Nakagawa et al. suggested preventive hilar ductoplasty, citing promising results and feasibility.^{17, 18} After securing the long-term outcomes of plasty and the complications associated with it, such as postoperative leaks and re-stenosis at the plasty site, it may be necessary to introduce this procedure in the future. However, it is known that in patients with Type IVa before surgery, intrahepatic duct (IHD) dilatation resolves within one year after surgery for choledochal cysts. Considering this, it is also necessary to establish evidence regarding which patients should be considered candidates for plasty. Based on this, it is also considered necessary to establish criteria for determining the optimal timing for performing ductoplasty to maximize patient outcomes. Although conventional R-en-Y anastomosis was consistently performed in our center, we tried to minimize damage to original structure of the common hepatic duct bifurcation and lymphovascular structures during dissection around the duct and cyst in order to prevent anastomotic stricture and postoperative structural deformation of the main hepatic duct.

Age at surgery was significantly lower in patients who did not develop IHD stones compared with those who did. Subgroup analysis between patients with and without stone formation revealed that IHD stone occurrence was lower in patients who received CC excision (p < 0.05). Patients who developed IHD stones were significantly older at surgery (p < 0.001), and linear regression analysis confirmed an association between age at surgery and occurrence of IHD stones (p < 0.001). Considering the results of our study, we hypothesize that diagnosis and operation at a younger age would be advantageous for prognoses related to IHD stones. Although early diagnosis and surgery have a positive effect on prognosis, waiting until six months of age for surgery is suggested considering the technical aspects of surgery, such as HJ stricture. Ryu et al. reported the outcomes of 43 neonates who underwent CC excision via open and laparoscopic approaches and were followed for an average of 37 months.¹⁹ Except for cholangitis and ileus in three patients who underwent open surgery, no known complications occurred in all patients. This demonstrated the technical feasibility of CC excision in neonates, emphasizing the need for consideration of prompt surgery after diagnosis.

In terms of treatment, oral ursodeoxycholic acid (UDCA) may be beneficial for asymptomatic small-sized stones.⁸ Takimoto reported the resolution of IHD stones in two patients included in the study, with unchanging in three patients. Among patients diagnosed with IHD stones, 16 (76.2%) were taking UDCA. At our center, we suggest UDCA supplementation not only for patients with events related to stones or asymptomatic small stones but also for adolescents at high risk of stone formation, as indicated by follow-up imaging showing IHD dilatation or pneumobilia. Senyuz reported the use of extracorporeal shock wave lithotripsy for stone removal. However, PTCS is the most longstanding therapeutic approach.²⁰ In our study, PTCS was performed in 11 patients. ERCP for stone removal has been recently introduced, in addition to the approach reported by Takimoto, involving accessing the hepaticojejunostomy through the Y-limb, we performed hepaticogastro/jejunostomy via direct access by puncturing through the stomach and duodenal wall under endoscopic ultrasound guide. Stone removal and balloon dilatation was performed via stomy and a stent was maintained for repetitive procedures.⁷ Two patients have exhibited favorable outcomes without complications and were satisfied with the aesthetic aspect of the procedure. Considering that patients developing stones are adolescents in their 10s to 20s, endoscopic ultrasound-guided stone removal would be a promising modality if technical barriers are overcome. However, this approach is challenging, therefore, only a few gastroenterologists from our center can perform it. Hepatectomy can be considered in cases of recurrent IHD stones or severe ductal stenosis. At our center, left hepatectomy was performed on two patients, with no recurrence of stones and favorable outcomes.

No CCA case was identified in our study population. Mukai’s 2018 report, which conducted a 20-year follow-up, also did not identify any case of biliary carcinoma in pediatric patients.²¹ Considering that stone occurrence increases after at least 10 years and CCA is reported to occur after 20 years from the operation, a longer-term follow-up seems necessary for patients who have experienced or are at risk of stone formation.

RIPD/CCD stones are poorly understood, with factors such as APBDU and pancreas divisum being considered influential, however, independent reports from patients with CC are limited.^{1, 9} Komi asserted that even after radical operation, in patients with complicated types, such as type Ib, IIb, and IIIc3, chronic pancreatitis can progress due to protein plugs or pancreatic calculus in the dilated duct.^{1, 9} Except two patients, whose MRCP was not available, we could identify the type of APBDU in the rest of patients. Two patients were included in Komi type 1A and the rest in complicated type. Among them, Komi type IIIC accompanying pancreas divisum was identified in nine patients. Typically, pancreas divisum is believed to be associated with pancreatitis. However, Terui reported that pancreas divisum is found in 1.4% of APBDU cases and does not always lead to pancreatitis.²² In patients with pancreas divisum, if the minor duct serves as the main drainage pathway, sludge in the common channel may have a lower likelihood of inducing pancreatitis, whereas if the major duct is involved, the risk of pancreatitis may increase. Moreover, given that most cases of pancreas divisum associated with CC are incomplete type, this introduces additional variables to consider.^{23, 24} Therefore, it seems difficult to establish a direct association between pancreas divisum and increased incidence of pancreatitis. Thus, prospective studies conducting subgroup analysis based on presence of stones is warranted to elucidate the relationship with RIPD/CCD stones. In our study, stones occurred at various sites, including the common channel, major duct, and minor duct. All patients exhibited obstructive symptoms at CC diagnosis, and 11 had preoperative ERCP, suggesting the need to consider the contribution of preoperative obstructive lesions and ERCP to long-term stone formation in RIPD/CCD.²⁵ Stones occurred at 65.6 ± 72.3 months postoperatively, significantly earlier than IHD stones (Table 3). Subgroup analysis and linear regression results (p = 0.05) revealed no association between age at surgery and stone (Table IV). Considering previous reports of biliary epithelial hyperplasia in CC excision specimens, early surgery might reduce inflammation in the biliary system, potentially decreasing IHD stone occurrence, especially in younger patients.^26–28 Treatment primarily involved ERCP in all patients except three asymptomatic patients with favorable outcomes. One patient underwent pylorus-preserving pancreaticoduodenectomy and later required lateral pancreaticojejunostomy due to recurrent stones and resultant severe pancreatic duct stricture. Although many patients had repeated ERCP, none showed dilatation of the main pancreatic duct.

The retrospective nature of this study posed limitations in comparing imaging results between the stone occurrence and non-occurrence groups. Most patients with stones got imaging tests since symptom onset, with subsequent tight follow-up. Conversely, asymptomatic patients were primarily monitored with ultrasonography, leading to an insufficient retrospective comparison of anatomy. Additionally, early MRCP images lacked precision, hindering interpretation and direct comparison of anatomical features, potentially influencing stone formation. A prospective study, including MRCP follow-up, is necessary to analyze whether Tsuchida type 2 or 3 persists after surgery and to determine the characteristics of patients in whom changes in RPID/CCD occur in cases of pancreas divisum and complicated ABPDU types.

In previous reports, postoperative stones were included in postoperative complications, and the significance of anatomical variation was only meaningfully reported in a few studies. We analyzed the impact of anatomical variation separately from postoperative complications and to confirm its clinical significance in a large cohort of pediatric patients. Considering the average age of IHD stone occurrence, it is necessary to establish a follow-up plan that extends into adolescence and beyond. For patients with Todani type IVa who exhibit pre/postoperative Tsuchida type 2 or 3 findings, it is necessary to consider a tighter follow-up regarding stone occurrence or surgical options such as ductoplasty. Detecting complicated APBDU type and pancreas divisum, may be crucial for predicting the occurrence of RIPD/CCD stones and guiding therapeutic approaches. This is necessary to prevent repetitive pancreatitis and potential damage to the pancreatic duct through timely intervention. Considering the evidence of carcinogenesis reported in some studies, our findings suggest that a more systematic and long-term follow-up is needed for patients with choledochal cysts (CC) than previously recommended.

APBDU

anomalous pancreaticobiliary ductal union

IHD

intrahepatic duct

RIPD

remnant intrapancreatic common bile duct

CCA

cholangiocarcinoma

CCD

common channel duct

choledochal cyst

hepaticojejunal

Jejunojejunal

MRCP

magnetic resonance cholangiopancreatography

SPSS

statistical package for the social sciences

ultrasonography

Competing interests

The authors declare no competing interests.

Consent statement

The study was conducted according to the guidelines of the Declaration of Helsinki and approved by the Ethics Committee of Asan Medical Center (2023 − 0840). Permission was waived by committee due to the retrospective nature of our study. This study did not involve human intervention.

Funding

This research was conducted in the absence of any commercial or financial fundings.

Author Contribution

These authors contributed equally: Sujin Gang, MD and Pyeong Hwa Kim, MD, PhDConceptualization: S.G., J-M.N.Formal Analysis: S.G.,PH.K.Investigation: S.G.Methodology: S.G.Project Administration: S.G., J-M.N.Writing – Original Draft: S.G.Writing – Review & Editing: All authors.

Acknowledgement

Not applicable.

Data Availability

The datasets generated and/or analyzed in the current study are available from the corresponding authors with permission from the Institutional Review Board of Asan Medical Center.

Park SW, Koh H, Oh JT, Han SJ, Kim S (2014) Relationship between anomalous pancreaticobiliary ductal union and pathologic inflammation of bile duct in choledochal cyst. Pediatr Gastroenterol Hepatol Nutr 17:170–177. https://doi.org/10.5223/pghn.2014.17.3.170
Alonso-Lej F, Rever WB, Pessagno DJ (1959) Congenital choledochal cyst, with a report of 2, and an analysis of 94, cases. Int Abstr Surg 108:1–30.
Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K (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. https://doi.org/10.1016/0002-9610(77)90359-2
Kimura K, Ohto M, Saisho H, Unozawa T, Tsuchiya Y, Morita M, Ebara M, Matsutani S, Okuda K (1985) Association of gallbladder carcinoma and anomalous pancreaticobiliary ductal union. Gastroenterology 89:1258–1265. https://doi.org/10.1016/0016-5085(85)90641-9
Ando H, Ito T, Kaneko K, Seo T (1995) Congenital stenosis of the intrahepatic bile duct associated with choledochal cysts. J Am Coll Surg 181:426–430.
Uno K, Tsuchida Y, Kawarasaki H, Ohmiya H, Honna T (1996) Development of intrahepatic cholelithiasis long after primary excision of choledochal cysts. J Am Coll Surg 183:583–588.
Shirota C, Kawashima H, Tainaka T, Sumida W, Yokota K, Makita S, Amano H, Takimoto A, Hinoki A, Uchida H (2021) Double-balloon endoscopic retrograde cholangiography can make a reliable diagnosis and good prognosis for postoperative complications of congenital biliary dilatation. Sci Rep 11:11052. https://doi.org/10.1038/s41598-021-90550-7
Takimoto A, Fumino S, Iguchi M, Takemoto M, Takayama S, Kim K, Higashi M, Aoi S (2022) Current treatment strategies for postoperative intrahepatic bile duct stones in congenital biliary dilatation: a single center retrospective study. BMC Pediatr 22:695. https://doi.org/10.1186/s12887-022-03759-4
Komi N, Takehara H, Kunitomo K, Miyoshi Y, Yagi T (1992) Does the type of anomalous arrangement of pancreaticobiliary ducts influence the surgery and prognosis of choledochal cyst? J Pediatr Surg 27:728–731. https://doi.org/10.1016/s0022-3468(05)80102-2
Ono S, Fumino S, Shimadera S, Iwai N (2010) Long-term outcomes after hepaticojejunostomy for choledochal cyst: a 10- to 27-year follow-up. J Pediatr Surg 45:376–378. https://doi.org/10.1016/j.jpedsurg.2009.10.078
Sugiyama M, Baba M, Atomi Y, Hanaoka H, Mizutani Y, Hachiya J (1998) Diagnosis of anomalous pancreaticobiliary junction: value of magnetic resonance cholangiopancreatography. Surgery 123:391–397. https://doi.org/10.1016/S0039-6060(98)70159-X
Ono S, Sakai K, Kimura O, Iwai N (2008) Development of bile duct cancer in a 26-year-old man after resection of infantile choledochal cyst. J Pediatr Surg 43:E17-E19. https://doi.org/10.1016/j.jpedsurg.2008.01.073
Nishiyama R, Shinoda M, Tanabe M, Masugi Y, Ueno A, Hibi T, Takano K, Fujisaki H, Kitago M, Itano O, Kawachi S, Aiura K, Tanimoto A, Sakamaoto M, Kitagawa Y (2011) Intrahepatic cholangiocarcinoma arising 33 years after excision of a choledochal cyst: report of a case. Int Surg 96:320–325. https://doi.org/10.9738/cc82.1
Yamataka A, Ohshiro K, Okada Y, Hosoda Y, Fujiwara T, Kohno S, Sunagawa M, Futagawa S, Sakakibara N, Miyano T (1997) Complications after cyst excision with hepaticoenterostomy for choledochal cysts and their surgical management in children versus adults. J Pediatr Surg 32:1097–1102. https://doi.org/10.1016/s0022-3468(97)90407-3
Tsuchida Y, Takahashi A, Suzuki N, Kuroiwa M, Murai H, Toki F, Kawarasaki H, Hashizume K, Honna T (2002) Development of intrahepatic biliary stones after excision of choledochal cysts. J Pediatr Surg 37:165–167. https://doi.org/10.1053/jpsu.2002.30243
Kemmotsu H, Mouri T, Muraji T (2009) Congenital stenosis of the hepatic duct at the porta hepatis in children with choledochal cyst. J Pediatr Surg 44:512–516. https://doi.org/10.1016/j.jpedsurg.2008.06.011
Nakagawa Y, Uchida H, Hinoki A, Shirota C, Tainaka T, Sumida W, Makita S, Yasui A, Takimoto A, Guo Y (2023) Laparoscopic bile duct plasty for hilar bile duct stenosis (HBDS) in patients with congenital biliary dilatation: diagnosis of HBDS by preoperative MRCP and laparoscopic strategy to relieve HBDS. J Hepatobiliary Pancreat Sci 30:473–481. https://doi.org/10.1002/jhbp.1235
Urushihara N, Fukumoto K, Fukuzawa H, Mitsunaga M, Watanabe K, Aoba T, Yamoto M, Miyake H (2012) Long-term outcomes after excision of choledochal cysts in a single institution: operative procedures and late complications. J Pediatr Surg 47:2169–2174. https://doi.org/10.1016/j.jpedsurg.2012.09.001
Ryu HS, Lee JY, Kim DY, Kim SC, Namgoong JM (2019) Minimally-invasive neonatal surgery: laparoscopic excision of choledochal cysts in neonates. Ann Surg Treat Res 97:21–26. https://doi.org/10.4174/astr.2019.97.1.21
Şenyüz OF, Gülşen F, Gökhan O, Emre Ş, Eroğlu E (2015) Effectiveness of extracorporeal shock wave lithotripsy on intrahepatic biliary calculi developing after choledochal cyst surgery: a case report. Turk J Gastroenterol 26:274–276. https://doi.org/10.5152/tjg.2015.0045
Mukai M, Kaji T, Masuya R, Yamada K, Sugita K, Moriguchi T, Onishi S, Yamada W, Kawano T, Machigashira S, Nakame K, Takamatsu H, Ieiri S (2018) Long-term outcomes of surgery for choledochal cysts: a single-institution study focusing on follow-up and late complications. Surg Today 48:835–840. https://doi.org/10.1007/s00595-018-1660-9
Terui K, Hishiki T, Saito T, Sato Y, Takenouchi A, Saito E, Ono S, Kamata T, Yoshida H (2010) Pancreas divisum in pancreaticobiliary maljunction in children. Pediatr Surg Int 26:419–422. https://doi.org/10.1007/s00383-010-2559-8
Kamisawa T, Tu Y, Egawa N, Tsuruta K, Okamoto A, Matsukawa M (2008) Pancreas divisum in pancreaticobiliary maljunction. Hepato-Gastroenterology 55:249–253
Petrasek J, Hucl T, Spicak J (2008) Pancreaticobiliary malunion and incomplete pancreas divisum: an unusual cause of common bile duct obstruction. Adv Med Sci 53:6–10. https://doi.org/10.2478/v10039-008-0002-3
Kopáčová M, Bureš J, Rejchrt S, Vávrová J, Bártová J, Soukup T, Tomš J, Tachecí I (2016) Risk factors of acute pancreatitis in oral double balloon enteroscopy. Acta Medica (Hradec Králové) 59:84–90. https://doi.org/10.14712/18059694.2016.95
Chen WJ, Wolff E, Varma CR, Shoela R (2022) Biliary intraepithelial neoplasia with gallbladder adenoma and cirrhosis: a case report. Cureus 14:e27780. https://doi.org/10.7759/cureus.27780
Gang S, il Lee Y, Ha S, Kwon H, Park J, Namgoong JM (2023) Biliary intraepithelial neoplasia (BilIN) diagnosed from choledochal cyst in 7-year-old girl with underlying anomalous pancreaticobiliary ductal union (APBDU). Adv Pediatr Surg 29:72–77. https://doi.org/10.13029/aps.2023.29.2.72
Iwai N, Fumino S, Tsuda T, Ono S, Kimura O, Deguchi E (2004) Surgical treatment for anomalous arrangement of the pancreaticobiliary duct with nondilatation of the common bile duct. J Pediatr Surg 39:1794–1796. https://doi.org/10.1016/j.jpedsurg.2004.08.010

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Risk Factors for Postoperative Stone Formation in Pediatric Choledochal Cysts: A Study of 457 Cases

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