Journal of Obstetrics and Gynaecology

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Prenatal diagnosis and prognosis assessment of

congenital choledochal cyst in 21 cases

R. Weng, W. Hu, S. Cai, X. Guo & Q. Luo

To cite this article: R. Weng, W. Hu, S. Cai, X. Guo & Q. Luo (2015): Prenatal diagnosis and
prognosis assessment of congenital choledochal cyst in 21 cases, Journal of Obstetrics and
Gynaecology, DOI: 10.3109/01443615.2015.1050648

To link to this article: http://dx.doi.org/10.3109/01443615.2015.1050648

Published online: 14 Oct 2015.

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 Journal of Obstetrics and Gynaecology, 2015; Early Online: 1–4
© 2015 Taylor & Francis Group, LLC
ISSN 0144-3615 print/ISSN 1364-6893 online
DOI: 10.3109/01443615.2015.1050648
ORIGINAL ARTICLE
Prenatal diagnosis and prognosis assessment of congenital choledochal
cyst in 21 cases
R. Weng, W. Hu, S. Cai, X. Guo & Q. Luo
Department of Obstetrics, Women’s Hospital, Zhejiang University School of Medicine, Hangzhou, China
To investigate the clinical significance of prenatal diagnosis and
Downloaded by [University of California, San Diego] at 01:04 26 November 2015
prognosis evaluation of congenital choledochal cyst (CCC), we
reviewed CCC cases of diagnosed antenatally in our hospital
from 2007 to 2013, summarised and analysed prenatal sono-
graphic features and clinical outcomes, and followed these cases
up to six months after birth. We found that induced labour was
conducted in 7 cases, and term labour progressed smoothly in
14 cases among the 21 cases. Operations were completed within
3 months after birth and all the operation cases received a good
prognosis. We suggest that CCC is one kind of non-lethal con-
genital malformation which can be treated after birth. Prenatal
diagnosis is important for its treatment after birth, because early
surgery after birth is associated with good treatment outcomes
and prognosis.
Keywords: Congenital choledochal cyst, foetus, prenatal diagnosis,
ultrasound, congenital malformation, prognosis
Introduction
Congenital choledochal cyst (CCC), also known as ‘congenital
biliary dilatation’, is a relatively rare disease of the biliary system.
CCC is rare in the West (1/100000–1/150000), but the incidence
is significantly higher in Asian countries such as China and
Japan (1/10000) (Liu et al. 2007). The disease can occur at any
age, but it is often found in childhood as a result of abdominal
pain, jaundice, abdominal mass and other symptoms. Women are
significantly more affected than men (3–4:1). For a long time, the
disease was considered a disease of childhood. However, CCC
has often been found in foetuses with the advances in prenatal
ultrasound techniques.
CCC is a digestive disease, which had been paid more attention
to by paediatrics, surgeons and radiologists. Due to the presence
of the incomplete obstruction of biliary and reflux of pancreatic
juice in that disease, secondary cholangitis, biliary calculi, bil-
iary cirrhosis, portal hypertension and cystic cancer can occur
in these patients. Most cases of CCC are diagnosed before the
age of ten years, and significant clinical symptoms occur in most
patients (Shi et al. 2001). At this point, even though operation
can be conducted, it is difficult to produce good outcomes. Diao
et al. (Diao et al. 2014) provides convincing data from a prospec-
tive randomised trial of a fairly large number of neonates, which
shows that early surgical intervention is advocated for the pre-
Correspondence: Qiong Luo, Department of Obstetrics, Women’s Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310006, China.
E-mail: luoqionglq@hotmail.com
vention of deterioration of liver function, coagulopathy and other
severe complications.
Fortunately, with the development of modern ultrasound
technology, diagnosis of CCC has gradually turned from after
birth to prenatal. The first case of prenatally diagnosis of CCC
occurred in 1980 (Dewbury et al. 1980; Shi et al. 2001; Liu et al.
2007). There had been no cases of prenatal diagnosis in our hos-
pital before 2007. However, prenatally diagnosed cases increased
gradually from 2007 to 2013. Prenatal diagnosis is favourable for
optimal management and selection of operation time. In particular,
prenatal diagnosis can help for timely intervention before the occur-
rence of the clinical symptoms. Therefore, prenatal diagnosis of CCC
has very important clinical significance for early treatment and good
prognosis of that disease. However, up to now, prenatal ultrasound
diagnosis of CCC was reported less commonly both in China and
other countries. Some obstetricians lack knowledge of this dis-
ease, the awareness on the prognosis in particular, and are unable
to provide professional advice. Some pregnant women terminate
the pregnancy because of this. The aim of the current study was to
analyse the ultrasound characteristics, prenatal diagnosis results and
prognosis of 21 cases of CCC prenatal diagnosed in our hospital and
recorded the follow-up results after birth. Our study may provide
important reference for the clinical management of CCC.
Materials and methods
We reviewed the cases for prenatal diagnosis in our hospital
from 2007 to 2013, and analysed the prenatal ultrasound features
and clinical outcomes of 21 cases of CCC. Our present research
was approved by the institutional review board of Zhejiang
University. From 2007 to 2013, our hospital had established a
multi-disciplinary expert consultation system for difficult cases,
by formation of a series of management such as the abnormal
foetus outpatient consultation, registration filing, consultation
discussions, proposed feedback, tracking guide and so on. Office
of prenatal diagnosis is in charge of coordinating the large con-
sultation by the permanent staff of foetal medicine expert group,
and related discipline experts of the discussed cases. During con-
sultation, detailed medical records were shown and radiographic
images were analysed; a diagnosis was made or additional means
for diagnosis were suggested, in order to make the most appropri-
ate medical treatment recommendations. Prenatal diagnosis of
21 cases of congenital bile duct cysts was selected from a total of
8989 cases of prenatal diagnosis and the outcomes of birth were
followed up in all CCC cases.
 2 R. Weng et al.
A retrospective analysis of the 21 cases of prenatal diagnosis of
CCC was conducted. Prenatal diagnosis of CCC mainly depended
on ultrasonography with specific images. Cystic mass in foetal
hepatic portal, not being connected with the stomach bubble,
observed by ultrasound was considered as the main basis for the
diagnosis. Especially, an observation of the communication of the
cystic mass to the intrahepatic bile ducts or (and) gallbladder was
considered as a fundamental basis for diagnosis. Cyst connected
to proximal hepatic duct, and mostly, cystic dilatation of com-
mon bile duct was shown obviously with normal intrahepatic bile
duct diameter. Repeated ultrasound imaging at 32, 36, 37, 38 and
40 weeks’ gestation during the course of the pregnancy is recom-
mended to confirm the presence of the cyst and possible changes
of the cyst size (Lee & Kim, 2008). We summarised the imaging
features of prenatal ultrasound, including cyst size, with or with-
out malformations, etc., and the treatment and prognosis in all of
the patients. Especially, we focussed on the diagnostic compliance
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and health status of those infants treated by surgery after birth.
All the data were presented as mean ⫾ standard deviation
(SD), and the two groups were compared with Student’s t test.
The SPSS15.0 (Statistical Analysis System, Chicago, IL, USA) was
used for data analysis. P ⬍ 0.05 was considered to be statistically
significant.
Results
All detailed information of the 21 CCC cases is shown in Table I.
In these 21 CCC cases, the minimum age of the mother was 22,
the maximum age was 39 and mean age was 27.67 ⫾ 3.99 years.
There were 19 cases of primipara and 2 cases of multipara. The
earliest gestational week of diagnosis was 17 weeks and the latest
was 39 ⫹ weeks, average: 27.95 ⫾ 4.73 weeks. CCC is in 0.23% of
all the prenatal diagnosis cases in our hospital. For most cases in
our hospital, prenatal diagnoses are from outside the hospital; the
actual incidence of CCC could not be calculated. Prenatal screen-
ing of trisomy 21 and 18 in all 21 cases showed low risk.
Prenatal ultrasound found the disease at earliest in 17 weeks’
and latest in 39 ⫹ weeks’, average 28 weeks’ gestation (the third
ultrasound of prenatal examination). Before the disease was
found, there was no obvious abnormality by prenatal ultrasound
examination. The ultrasound section was mainly transverse.
A cystic dark area located in the hepatic portal region in the right
upper quadrant, or located in the region between the lower edge
of liver and gallbladder was seen on ultrasound. The configura-
tion was mainly oval with cystic duct-like structures. The cyst wall
was thin, and cyst fluid was clear, with cyst size from 1.4 cm to
3.0 cm, and gradually increased with the gestational age (Figure 1),
without blood within the cyst and surrounding abundant blood
flow, concomitant with portal veins. A typical prenatal ultrasound
photograph of CCC in the present study is shown in Figure 2.
There were no other commonly associated ultrasound findings.
There were no ultrasound tips with other abnormalities found in
these 21 cases of CCC in the present study.
The 21 cases of CCC were jointly consulted by paediatric sur-
geons, ultrasound experts and obstetricians. Mainly according to
the B ultrasound findings, prenatal diagnosis reports of 19 cases
were issued (90.48%). Another case was informed clearly to be
able to continue pregnancy with follow-up, according to assess-
ment of the prognosis of the other 19 patients. The information
of those 19 cases showed that the disease could be cured and the
prognosis was relatively good. In addition, because another case
of CCC was present along with other abnormalities such as foe-
tal oedema, polyhydramnios, absence of foetal umbilical artery
diastolic flow and advanced age of 39 years of the mother, it was
taken into consideration by the joint consultation that the foetus
might get poor prognosis, and induced labour was suggested in
that case. Induced labour was selected in 7 cases (33.33%) because
the mothers feared the prognosis of CCC or did not want to bear
the risk of operation after birth. Although the prognosis of the
disease was fully informed by us, they still persisted in abortion
and refused autopsy after abortion. Another 14 cases continued
pregnancy to term, and the gender ratio is male/female 1:6, and
the mode of delivery was vaginal/caesarean section 1:0.2. The
minimum foetal birth weight was 2.80 kg, maximum 4.40 kg and
average 3.49 ⫾ 0.39 kg.
All of the 14 patients underwent operation (7–95 days after
birth), average: 40.36 ⫾ 26.29 days. Among all the cases, jaundice
respectively with clay-colored stools, vomiting, continued cry-
ing and other symptoms occurred in 6 cases and operation was
arranged within one week after these symptoms occurred. The
other 8 cases underwent operation asymptomatically and single
operation was performed in all 14 patients. The main operation
mode was abdominal (11 cases) or laparoscopic (3 cases) excision
of choledochal cyst and Roux-en-Y liver and jejunum anasto-
mosis. Postoperative recovery was good and the incision healed
well in all cases. In follow-up at 6 months postpartum, digestive
function of these infants were normal without distinction
from normal infants. Liver function and bilirubin index were
monitored, showing no abnormal increase. There was no obvious
abnormal growth and development in all infants.
Discussion
In 1973, Professor Todani from Japan classified choledochal cyst
into five types (Todani et al. 1977). Type I is most common in
clinic, accounting for 48%–85%, and is also divided into type
IA, cystic dilatation of the common bile duct; IB type, segmental
extrahepatic biliary dilatation and IC type, diffuse extrahepatic
bile duct dilatation. Type II is rare, congenital diverticulum of
the lateral wall of the common bile duct, accounting for 2%-3%.
Type III is cyst in the end of the common bile duct, accounting for
1.4%-5.6%. Type IV A/B type is multiple cysts intrahepatic and/or
extrahepatic bile duct, accounting for 18%-20%. Type V Caroli’s
disease, is intrahepatic bile duct cyst and rare. This classification is
still currently in use. The aetiology of CCC is not currently known.
Mainstream theories suggest that the occurrence of CCC might
be related to abnormal development of the confluence of bile
and pancreatic duct, distal obstruction of common bile duct, and
abnormal development of extrahepatic bile duct and pancreas in
embryonic stage. Our results indicated that the growth and devel-
opment of foetuses with CCC do not show obvious abnormalities,
and that the pregnancy can continue in term with vaginal deliv-
ery. The birth weight of these CCC babies was also in the normal
range. In addition, we found that, although the CCC maximum
diameter increased with the progress of pregnancy, CCC would
not cause any abnormalities of either pregnant women or foetus.
Mostly, CCC was found to be the single abnormality in foetus,
and there is no relevant literature reporting that other malforma-
tions may happen with the CCC.
Early diagnosis, especially the prenatal diagnosis, has a very
important clinical significance in treatment and good prognosis
of CCC. At present, the obstetrician has poor cognition of that
disease, and could not provide good advice. According to the
results of our present study, the prognosis is good for foetuses
without other abnormalities who had the operation as a newborn.
So we suggest that, once a careful prenatal diagnosis consultation
is conducted and CCC is highly suspected, induced labour should
not be conducted blindly.
 Prenatal diagnosis and prognosis assessment of CCC 3
Table I. Prenatal diagnosis of 21 CCC cases and prognosis evaluation (2007–2013).

Maximum Gestational age Follow-up and

Age of Gravidity/ Pregnant weeks diameter of Consultation and mode of Gender of Birth prognosis
mother parity at diagnosis CCC results delivery baby weight(g) Treatment evaluation

27 1/0 31 1.8 cm Prenatal diagnosis Full-term normal Female 3100 Operation Good
report issued delivery 7 days
after birth
28 3/1 26⫹ 2.6 cm Prenatal diagnosis Full-term Female 3450 Operation Good
report issued caesarean 50 days
section after birth
28 2/0 28⫹ 3.0 cm Prenatal diagnosis Full-term normal Female 3500 Operation Good
report issued delivery 45 days
after birth
23 1/0 28⫹ 3.0 cm Prenatal diagnosis Full-term Female 3700 Operation Good
report issued caesarean 35 days
section after birth
28 2/0 39⫹ 3.9 cm Prenatal diagnosis Full-term normal Female 3250 Operation Good
report issued delivery 30 days
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after birth
26 2/0 30⫹ 3.1 cm Prenatal diagnosis Full-term normal Female 3400 Operation Good
report issued delivery 92 days
after birth
30 5/1 28⫹ 2.0 cm Prenatal diagnosis Full-term normal Male 3350 Operation Good
report issued delivery 30 days
after birth
26 2/0 25 2.5 cm Prenatal diagnosis Full-term normal Female 3450 Operation Good
report issued delivery 31 days
after birth
23 1/0 30 2.3 cm Prenatal diagnosis Full-term normal Male 3450 Operation Good
report issued delivery 11 days
after birth
22 1/0 26 1.8 cm Prenatal diagnosis Full-term normal Female 4400 Operation Good
report issued delivery 95 days
after birth
27 3/0 35⫹ 3.0 cm Prenatal diagnosis Full-term normal Female 3400 Operation Good
report issued delivery 53 days
after birth
27 1/0 25 2.1 cm Follow-up Full-term normal Female 3500 Operation Good
Recommend delivery 45 days
after birth
29 3/0 25 1.8 cm Prenatal diagnosis Full-term normal Female 4100 Operation Good
report issued delivery 30 days
after birth
30 5/1 33 3.0 cm Prenatal diagnosis Full-term normal Female 2800 Operation Good
report issued delivery 9 months
after birth
35 1/0 24⫹ 1.8 cm Prenatal diagnosis Induced labour No autopsy
report issued
27 1/0 26⫹ 1.4 cm Prenatal diagnosis Induced labour No autopsy
report issued
25 1/0 17⫹ 1.4 cm Prenatal diagnosis Induced labour No autopsy
report issued
31 2/1 31 3.3 cm Prenatal diagnosis Induced labour No autopsy
report issued
39 3/1 26 2.0 cm Termination of Induced labour No autopsy
pregnancy
suggested
27 1/0 23 1.8 cm Prenatal diagnosis Induced labour No autopsy
report issued
23 1/0 32⫹ 2.5 cm Prenatal diagnosis Induced labour No autopsy
report issued

At present, ultrasound has been widely used in the diagnosis of
CCC. It is reported that CCC could be found earliest at 15 weeks’
gestation, and the earliest in our hospital was at 17 weeks’ gesta-
tion. The differential diagnosis includes simple cyst of the liver,
biliary atresia, omental and mesenteric cyst, ovarian cysts, adre-
nal cysts, renal cysts, dilated intestinal loops and hydronephrosis.
Congenital biliary atresia (BA) must be differentiated because it
requires immediate surgery in the newborn (Clifton et al. 2006).
However, CCC and BA are sometimes difficult to differentiate.
Nuclear magnetic resonance imaging (MRI) may contribute to
the further identification. Ultrasound-guided aspiration of the
choledochal cyst in the newborn may be helpful for the diagnosis
(Bianchi et al. 2011).
 4 R. Weng et al.
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Figure 1. CCC maximum diameter in the second and third trimester of
pregnancy. CCC maximum diameter is significantly larger in the third
trimester of pregnancy (28–42 weeks’ gestation) than that in the second
trimester of pregnancy (13–27 weeks’ gestation) (P ⬍ 0.01). Data were
presented in mean with SD and compared with Student’s t-test.
Recently, the outcome has been positive with surgery as soon
as possible after birth. The main purpose of treatment is resection
of the cyst, reconstruction of correct digestive tract bile flow path,
and prevention of CCC complications such as obstructive jaundice,
repeated attack of cholangitis and pancreatitis. Most scholars rec-
ommend Roux-en-Y hepatic jejunostomy. It is reported that the
operation success rate was 92%, and the occurrence of operation
complications was 7%. However, without treatment, the CCC
patients have a 10–15% chance for the development of cholan-
giocarcinoma (Shimotakahara et al. 2005). Nicholl et al. (Nicholl
et al. 2004) reported that there was a direct association between
age and cancer risk in CCC patients: 0% in 0–30 years of age, 19%
in 31–50 years of age and 50% in 51–70 years of age. The clinical
Figure 2. A typical prenatal ultrasound photograph of CCC. The white
arrow in the photograph refers to the CCC and the black arrow refers to the
gallbladder. The cystic dark area was located in region between the lower edge
of liver and gallbladder, partly trafficked with gallbladder. The configuration
was cystic duct-like structures with thin cyst wall and clear cyst fluid.
value of early operation is obvious. Previously, some researchers
suggested that too early operation may have technical difficulties
and increase the risk of anaesthesia. Tsai et al. investigated the
relationship between the patient’s age and the operation treatment
results (Tsai et al. 2008) by comparing three groups of patients:
⬍ 1 years old (group I, n ⫽ 26), 1 years old (group 2, n ⫽ 48)
and over 16 years old (Group 3, n ⫽ 33). The results showed
that operation complications and severe liver fibrosis symptoms
of patients in group I was significantly reduced compared with
those in groups II and III. Therefore, early treatment, that is, treat-
ment in infancy is recommended. Germani et al. (Germani et al.
2011) recommended that the operation should be conducted only
at least 3 months after birth, because with the development of
anatomical structure of the CCC babies, the success rate of opera-
tion can increase; however, operation is performed for infants
younger than 3 months just in cases where severe symptoms occur.
A series of reports in recent years agreed that operation time
for CCC should be better as early as possible, especially before
serious clinical symptoms appear in the CCC children. Patients
with no local inflammation, adhesion, and good basic condition
can tolerate the operation well and postoperative complications
are relatively less. Delayed operation may lead to a series of events,
such as the liver cell damage, fibrous tissue hyperplasia of micro
bile duct and liver cirrhosis (O’Neill et al. 2006). All patients in
our present study underwent the operation within 3 months after
birth. Compared with the reports in other countries, no cases of
serious operation complication and operation failure occurred,
which might be related to the large case number and the rich
operation experience.
Declaration of interest: The authors report no declarations of
interest.
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