Journal of Pediatric Surgery (2009) 44, 2307–2311

www.elsevier.com/locate/jpedsurg

Management of choledochal cyst: 30 years of experience

and results in a single center
Wong-Hoi She, H.Y. Chung, L.C.L. Lan, Kenneth K.Y. Wong ⁎, H. Saing, P.K.H. Tam
Department of Surgery, University of Hong Kong Medical Centre, Queen Mary Hospital, Hong Kong

Received 25 July 2009; accepted 31 July 2009

Key words:
Abstract
Choledochal cyst;
Background: Choledochal cyst is usually diagnosed in childhood. Early treatment can prevent further
Todani classification;
complication. We report on our series of patients over the past 30 years.
Surgical management
Methods: A retrospective study was performed on all pediatric patients who presented with choledochal
cyst from January 1978 to December 2008. The main outcome measures recorded were the clinical
presentation, management, and long-term outcome of the patients.
Results: Eighty-three patients presented to us during the caption period with a mean age at diagnosis of
45 months (0 month to 16 years). Diagnoses were made antenatally in 15 patients. The most common
symptoms were abdominal pain (n = 39) and jaundice (n = 35). Seventy-five patients had surgery, in
which 72 patients had resection of the cyst and Roux-en-Y hepaticojejunostomy. Ten were performed
by laparoscopic means. We categorized the cysts based on the Todani classification. There was no
mortality. No malignant change was documented. For those 4 who had Caroli disease, 2 underwent liver
transplantation and 2 had hepatectomy. Overall early complication rate was 5.3% (4/75).
Conclusions: Complete excision of cyst with Roux-en-Y hepaticojejunostomy is the treatment of choice,
and the late result is good. Laparoscopic surgery is feasible. Long-term follow-up is necessary. There is no
evidence to suggest that some type IV cysts are the result of disease progression from type I cysts.
© 2009 Elsevier Inc. All rights reserved.

Choledochal cyst is the congenital cystic dilatation of
the extrahepatic and/or intrahepatic biliary tree with a
higher prevalence in Asia [1]. The diagnosis is usually
made in the first few years of life; especially more and
more patients are diagnosed antenatally in recent years. As
Presented at the 42nd Annual Meeting of the Pacific Association of
Pediatric Surgeons, Hong Kong, China, May 10-14, 2009.
⁎ Corresponding author. Division of Paediatric Surgery, Department of
Surgery, The University of Hong Kong, Queen Mary Hospital, Pokfulam
Road, Hong Kong SAR. Tel.: +852 2855 4850; fax: +852 2817 3155.
E-mail address: kkywong@hku.hk (K.K.Y. Wong).
there is an increased risk in developing cholangiocarci-
noma, recognition and proper management of choledochal
cyst disease are important.
Choledochal cysts are generally classified using the
Todani modification of the Alonso-Lej classification [2]. In
this study, we present our experience of the management of
children with choledochal cysts over a 30-year period. As the
underlying etiology of various subtypes of cysts remains
unknown, the current classification may potentially be
describing overlapping subtypes. We have attempted to ask
if type IV choledochal cysts could be part of the disease
progression of type I cysts.

0022-3468/$ – see front matter © 2009 Elsevier Inc. All rights reserved.
doi:10.1016/j.jpedsurg.2009.07.071
2308 W.-H. She et al.

1. Materials and methods Table 2 Comparison between open and laparoscopic

procedures for choledochal cysts
A retrospective study was performed on all patients who Open Laparoscopic
presented with choledochal cysts from January 1978 to (65) (10)
December 2008 after obtaining approval from the institu-
Early complications 4 (5.3%)
tional review board of the hospital. Clinical details were
Bleeding at HJ 0 1 1
derived from patients' medical records. Demographic and
Wound dehiscence 2 0 2
clinical data, including both operative and radiologic Chylous ascites 1 0 1
findings, were recorded on a standardized form. Antenatal Late complications 12 (16%)
histories were recorded. The choledochal cysts were Residual cyst 3 1 4
classified into 5 types according to the Todani modification Biliary stones 3 0 3
of the Alonoso-Lej classification [2]. Operation was HJ stricture 1 1 2
performed by cyst excision and hepaticojejunostomy (EX- Cholangitis 1 0 1
HJ) using a 40-cm Roux limb without additional antireflux Pancreatitis 1 0 1
procedure for those patients who had extrahepatic cysts; Adhesive intestinal 1 0 1
hepatectomy for unilateral liver involvement and transplan- obstruction
Total no. of patients (%) 13 (20) 3 (30) 16 (21.3)
tation for Caroli disease. Early and late postoperative
complications were also noted. To examine the possible P N .05, no difference in terms of complication in both surgical groups.
correlation between type I and IV choledochal cysts, we
compared the age of patients when the diagnosis were first months (SE ± 64 months). The most common presenting
made. Statistical analysis was performed with the help of symptoms were abdominal pain (n = 39), jaundice (n = 35),
SPSS v.16 (SPSS, Chicago, Ill) using appropriate tests and vomiting (n = 26). The classic triad of abdominal pain,
including Mann-Whitney test. P values of less than .05 were jaundice, and palpable right upper quadrant mass was only
considered to indicate statistical significance. identified in 2 patients (Table 1). There were 75 patients who
had received definitive surgery. One patient is currently
awaiting surgery, one patient is being followed up in our
clinic but had surgery in another hospital, one decided to
2. Results have operation elsewhere, and the parents of the remaining 5
children refused surgery all together.
A total of 83 patients (60 females, 23 males, ratio 2.6:1) No malignant change was observed or documented in any
presented to our center during the study period. The mean of our patients either before or after EX-HJ, even after 30
age at diagnosis was 45 months old (range between 0 month years. For the 4 patients who had Caroli disease, 2 underwent
to 16 years old). The median follow-up period was 62 liver transplantation, 1 had right trisectionectomy, and 1 had
left hepatectomy 6 years after initial cyst excision procedure.
There was no early or late mortality postoperatively. Based
Table 1 Demographic data of patients with choledochal cysts on the Todani modification of the Alonso-Lej classification,
Present study we identified 53 patients with type I (67.9%), 4 with type II
Total number 83 (5.1%), 2 with type III (2.6%), 15 with type IV (19.2%), and
Female/male ratio 2.6:1 4 with type V (5.1%) choledochal cyst, whereas the others
Clinical presentation (%) could not be ascertained because of incomplete records. All
Jaundice 35 (42) patients have been followed up in our clinic with regular liver
Pain 39 (47) function test and ultrasonography for assessment.
Mass 18 (22) For those 72 patients who had EX-HJ in our center, 10
Complete triad 2 (2) were performed laparoscopically. Five patients (50%)
Vomiting 26 (31) required open conversion. Of these, 1 was because of
Cholangitis 10 (12) intrahepatic involvement of the cyst, 1 was because of distal
Pancreatitis 18 (22)
Antenatal diagnosis by ultrasound (%) 15 (18)
Anatomical classification (%)
Type I 53 (68) Table 3 Complications between antenatal and postnatal
Type II 4 (5) groups
Type III 2 (3)
Nil Early Late Total
Type IV 15 (19)
Type V 4 (5) Postnatal group (%) 47 (75.8) 4 (6.5) 11 (17.7) 62
Refuse operation (n) 5 Antenatal group (%) 12 (92.3) 0 (0) 1 (7.7) 13
Pending operation (n) 1 Total no. of patients (%) 59 (78.7) 4 (5.3) 12 (16) 75
Management of choledochal cyst 2309

Table 4 Comparison and statistical values for type I and type IV cysts
Type Statistic SE
Age at diagnosis (mo) I Mean 41.3 6.81
95% CI Lower bound 27.6
Upper bound 54.9
Median 25.0
Variance 2460
SD 49.6
Interquartile range 59.0
Skewness 1.34 0.33
IV Mean 54.4 12.6
95% confidence interval Lower bound 27.5
Upper bound 81.3
Median 48.0
Variance 2360
SD 48.6
Interquartile range 71.0
Skewness 0.99 0.58
P = .197.

extension of the cyst to posterior pancreas, 2 were because of
difficult dissection, and 1 was because of tearing of the
common hepatic duct during performing HJ. One early
postoperative complication (1/10, 10%) seen was the
bleeding at the HJ site, whereas 2 late complications (2/10,
20%) were found because of HJ stricture and residual cyst
after laparoscopic procedure. The remaining patients,
including those who had Caroli disease, had operation
performed in open manner. Early complications (3/65, 4.6%)
included postoperative wound disruptions in 2 patients and
chylous ascites in 1. Late complications (10/65, 15.4%)
included residual cyst in 3, presence of biliary stones in 3, HJ
stricture in 1, cholangitis in 1, pancreatitis in 1, and adhesive
intestinal obstruction in 1. Overall early complication rate is
5.3% (4/75) and late complication rate is 16% (12/75).
Overall complications rate including early and late compli-
cation is 21.3% (16/75). There is no difference in terms of
complication rate in our patients who underwent open or
laparoscopic surgery (P = .472) (Table 2).
As described in our previous study [3], more patients with
choledochal cysts are now diagnosed antenatally. Indeed, 15
of our patients were diagnosed to have choledochal cysts by
antenatal ultrasound, and 2 of these were found as early as 18
weeks of gestation. Thirteen patients had undergone
operation and the remaining 2 are still waiting for operation.
The mean age of operation for antenatally diagnosed patients
was 5 months old (SE ± 4 months), with the youngest done at
1 month for obstructive jaundice and oldest at 11 months.
Two patients had laparoscopic cyst excision. One patient
required open conversion for HJ anastomosis because of
tearing of the common hepatic duct during the last suture.
The median follow-up period was 59 months (SE ± 39

Fig. 1 Frequency of type I and IV cyst diagnosis at different ages.

2310
months). There was no early complication but one late
complication was seen (7.7%, 1/13) (Table 3).
The most common types of the choledochal cysts are type
I and IV cysts, accounting for 85% (68/78) of our patients.
As type I is the cystic dilatation of the common bile duct,
whereas type IV is the dilatation extending to the left and
right hepatic ducts, we postulated whether it would be
possible that the occurrence of some type IV cysts might be a
result of chronic inflammation and obstruction of distal bile
ducts from cases of type I cysts. In other words, some
patients who were found to have type IV at presentation
might already have had asymptomatic type I cysts for
sometime before diagnosis of choledochal cyst had been
made. If this were the case, then the proportion of type IV
cysts would rise in older patients. Here, we compared the
medians of type I and IV cysts. The median of type I was 25
months (interquartile range, 59 months), whereas the median
of type IV was 48 months (interquartile range, 71 months),
with P = .197. Although it would appear that type I cyst
aroused earlier in age, because of small sample size in the
type IV population, we could not confirm our original
postulation at this stage (Table 4 and Fig. 1).
3. Discussion
Choledochal cyst is a congenital anomaly usually found
in pediatric population. It is estimated to occur in 1 in 5000
live births, with a higher frequency in Asians [4,5]. Common
symptoms include abdominal pain and vomiting, but these
are nonspecific, and therefore the condition is usually
difficult to diagnose during infancy. On the other hand,
jaundice is a more specific symptom and the diagnosis is
usually made early if the jaundice is prolonged during
neonatal period or related to the deranged liver function test
because of cholestasis or cholangitis. Presence of the triad of
symptoms is the classic description of choledochal cyst but is
rare in our study.
Laparoscopic surgery is feasible in selected patients and is
safe, but it has a high technical demand. The short-term
complications of our patients are generally good, with an
early complication rate of 5.3%, which is comparable to
other series [6]. Recurrent strictures and intrahepatic stone
formations are well-known and well-described long-term
complications, with the risks being forever present [7]. Our
long-term complication rate is 16%. There has been no
malignancy documented so far in any of our patients.
As the antenatal ultrasonography is getting more popular
and easily available in this era, more and more of the
diagnoses are made antenatally. This enables us to have a
better communication with parents. The parents are likely to
have better acceptance and be more prepared to agree for
early surgical intervention for the patients. The common bile
duct pathology can be detected antenatally as early as at 15
weeks of gestation [8]. All the patients diagnosed antenatally
W.-H. She et al.
were asymptomatic before operation. Early surgery greatly
decreases the occurrence of disease-related complications,
for example, acute cholangitis, acute pancreatitis, early
formation of biliary stone disease, biliary cirrhosis, liver
cirrhosis, and malignancy. Although the postoperative
complication rates in the antenatal and postnatal group did
not show any statistically significance possibly because of
the small number [3], we have experienced easier dissection
during the surgical procedure in the antenatally diagnosed
patients as a result of decreased periductal inflammation.
Type I and type IV choledochal cysts are 2 distinct entities
according to the Todani classification. Type I cyst has no
intrahepatic component and the common hepatic duct
proximal to the cyst is usually normal, whereas type IV
disease contains multiple cysts, with involvement of the
intrahepatic bile ducts [9]. In our series, type I cyst occurred
more frequently in those patients who were diagnosed earlier
in their lives, whereas type IV cyst was usually found in
older children. The usual presenting symptoms for older
children are the complications of the choledochal cysts, that
is, stone formation and acute cholangitis. Because of
repeated cholangitis, there could be bile duct stricture
formation. It would be possible to deduce that dilatation of
the proximal segment takes time to develop and is a gradual
process. Hence, the diagnosis of type IV choledochal cyst
could well be a result of chronic complications of type I
choledochal cyst. The chronic inflammation might result in
further stone and stricture formation. The end result would be
the dilatation of the more proximal segments and therefore
the presence of multiple cysts, that is, “type IV” choledochal
cysts. However, our current study does not support this
hypothesis, and it would appear that type I and type IV cysts
are 2 separate entities.
In conclusion, choledochal cyst is a rare disease and is
commonly present in childhood. Antenatal diagnosis by
means of routine ultrasound screening is possible and allows
earlier surgical intervention, which may prevent later
complications. Laparoscopic surgery is feasible but is
technically demanding. Long-term follow-up is necessary
to identify those who are at risk to have complications.
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