J Ped Surg Case Reports 4 (2016) 1e3

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Journal of Pediatric Surgery CASE REPORTS

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Prenatal diagnosis of a giant choledochal cyst

Yurema Gonzlez Ruiz*, Paolo Bragagnini Rodriguez, Rafael Leonardo Fernndez Atun,
Natalia lvarez Garca, Alexander Siles Hinojosa, Natalia Gonzlez Martnez-Pardo,
Juan Elas Pollina
Department of Pediatric Surgery, Miguel Servet University Hospital, Paseo Isabel La Catlica s/n, Zaragoza, Aragon, Spain

a r t i c l e i n f o a b s t r a c t

Article history: A choledochal cyst is a rare and usually benign condition that is diagnosed in 80% of cases in early in-
Received 7 October 2015 fancy. Prenatal diagnosis is increasingly identifying the cysts allowing an early surgical treatment with
Received in revised form less risk of complications. We report a case of a newborn with prenatal diagnosis of a giant right upper
1 November 2015
quadrant abdominal cyst. During surgery, an 8 cm choledochal cyst was found. A cholecistectomy, sur-
Accepted 3 November 2015
gical excision of the cyst and formation of a Roux-en-Y hepaticojejunostomy was performed.
2016 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND
license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Key words:
Choledochal cyst
Prenatal diagnosis
Hepaticojejunostomy

A choledochal cyst is a rare congenital cystic dilatation of the
extra or intrahepatic biliary tract or both. It was rst described by
Vater and Ezler in 1723 [1]. They are primarily found in female
gender in young children and are more prevalent in East Asian
populations. A choledochal cyst can be identied in neonates as a
palpable mass, with cholestasis or with jaundice. An early prenatal
diagnosis of the choledochal cyst is essential for an earlier surgical
repair which results in less complications [2].
1. Case report
We present a newborn 40 2 weeks gestational age with ante-
natal diagnosis of a giant abdominal paraduodenal cyst. In a second
trimester ultrasound, a 42  39 mm central cyst, below the stomach
was found. In a third trimester follow-up ultrasound, the cyst was
68.2  67  70.2 mm in diameter, displacing the bladder, kidneys,
liver and gallbladder. The diagnostic options could be a mesenteric
cyst, enteric duplication, small bowel atresia, ovarian cyst or a
choledochal cyst. No other prenatal malformations were observed,
the pregnancy continued uneventfully. At birth, the patient had a
palpable right abdominal mass. Postnatal abdominal ultrasound
showed a cystic lesion with diffuse echoes inside. A magnetic reso-
nance cholangiography showed a 78.1 70.61 79.85 mm diameter
* Corresponding author.
E-mail address: yuremagr@gmail.com (Y. Gonzlez Ruiz).
cyst, which was in contact with the medial and inferior surface of the
liver; dilatation of the central intrahepatic bile duct due to
compression and the cyst seemed to continue with the gallbladder.
These ndings are consistent with a choledochal cyst (Fig. 1). The
patient remained asymptomatic, with mild jaundice. Laboratory
studies revealed a total bilirubin (TB) 4.79 mg/dl, direct bilirubin
(BD) 0.6 mg/dl, alkaline phosphatase 227 U/L, GGT 122 U/L and GPT
12 U/L. The patient was operated on the 11th day of life through a
laparotomy, conrming a choledochal cyst of 8 cm in diameter with
thick walls adhered to the duodenum, pancreas and right paracolic
gutter with the gallbladder draining into the cyst and distal common
bile duct atresia (Fig. 2). The gallbladder was removed, and 150 cc of
bilious content aspirated, cyst excision and Roux-Y hep-
aticojejunostomy was performed. The postoperative course was
favorable, initiating feeds on the fth postoperative day. He was
discharged on the tenth postoperative day, with normal ultrasound
follow-up and normalization of bilirubin levels (BT 0.33 mg/dL and
BD 0.09 mg/dL).
2. Discussion
The choledochal cyst is a congenital cystic dilatation of the bile
ducts which can be intra- or extrahepatic or both. Choledochal cysts
are usually diagnosed in childhood, although in utero and adult
diagnosis is also common [1]. It is a benign disease, four times more
frequent in females than in males [1]. The etiology is unknown, but

2213-5766/ 2016 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
http://dx.doi.org/10.1016/j.epsc.2015.11.001
2 Y. Gonzlez Ruiz et al. / J Ped Surg Case Reports 4 (2016) 1e3
Fig. 1. Magnetic resonance cholangiography showing a 78.1  70.61  79.85 mm
diameter cyst consistent with a choledochal cyst.
in 30e70% of cases, there is an abnormal binding of bile and
pancreatic ducts, allowing pancreatic reux to the biliary tree and
exposing the biliary epithelium to pancreatic enzymes, which could
contribute to the cyst formation [1]. Other etiological hypothesis
include, weakness of the wall of the bile duct, increased intrabiliary
pressure, sphincter of Oddi dysfunction and distal common bile
duct obstruction. This last one is the most likely etiology in our case,
since there was an absence of cyst communication with the duo-
denum, leading to accumulation of bile during pregnancy and a
progressive increase in cyst size up to the enormous size described.
It has been suggested that age-related differences in presenta-
tion is determined by whether there is a reux of activated
pancreatic juice [3]. It may present itself with the classic triad of
abdominal pain, jaundice and right upper quadrant mass and this
Fig. 2. An 8 cm choledochal cyst with the gallbladder draining into the cyst.
triad is most commonly seen in pediatric patients. However, it is
usually asymptomatic or it may be accompanied by complications
such as cholangitis, pancreatitis, cholelithiasis and malignant
transformation. There are distinct differences to the pattern of
presentation in adults and children. Specically, adults are more
likely to present with biliary or pancreatic symptoms and abdom-
inal pain and are more likely to have symptomatic gallstones or
acute cholecystitis. Children are more likely to present with an
abdominal mass and jaundice. Cyst rupture is rare and typically is
seen only in neonates and infants [4,5]. In neonates, biliary brosis
and cirrhosis can occur due to biliary tree obstruction [6].
Prenatal diagnosis is increasingly common because of improved
ultrasound equipment and routine prenatal ultrasound screening. It
is essential since it leads to early surgery with less risk of postnatal
complications such as cholestasis, cholangitis, perforation, biliary
cirrhosis, pancreatitis, portal hypertension, and liver failure [2].
Some studies reported that delayed surgery results in liver brosis
[7]. However, the diagnosis is sometimes difcult, and it is usually
made during the third trimester of pregnancy, especially in cases of
large cysts, as in our case. In some instances, the biliary tract cannot
be differentiated from the cyst due to proximity to other structures.
Ultrasounds performed at an early stage of pregnancy can confuse a
cyst on right upper quadrant with the gallbladder or the umbilical
vein and differential diagnosis must be made with duodenal atresia,
ovarian cyst, hydronephrosis, liver cyst, enteric duplication cyst and
giant meconium pseudocyst [2,8,9]. Also, it is difcult to distinguish
biliary atresia from choledochal cyst in the perinatal period. Pre-
natal differential diagnosis of biliary atresia and choledochal cyst
has been achieved on the basis of chronological changes in cyst size,
cyst patterns, or timing of expression, but no denitive index exists
[7]. The observation that patients with prenatally diagnosed chol-
edochal cysts may, in fact, have biliary atresia warrants cautious
interpretation of prenatal studies with careful family counseling.
Postnatal diagnostic studies should be obtained immediately after
birth, with a low threshold of suspicion for early exploration [10].
The prenatal management of choledochal cysts is conservative.
However, Nasu et al. describe a prenatally diagnosed symptomatic
choledochal cyst in which a percutaneous transhepatic drainage
was performed during pregnancy, later undergoing surgical exci-
sion of the choledochal cyst, cholecystectomy and formation of a
Roux-en-Y hepaticojejunostomy postpartum [11].
There are few cases described in the literature of large chol-
edochal cyst, Rento et al. [12] described a 15 cm diameter cyst at
birth, producing abdominal distension and respiratory
compromise.
The concept of treatment of extrahepatic choledochal cysts has
changed in the past 20 years because of a persistent high risk of
malignancy after drainage procedures [3]. Today, treatment consists
of complete excision of the extrahepatic cyst because of the risk
of malignant transformation [1,3,13] and a bilioenteric
reconstruction through a hepaticoduodenostomy or Roux-en-Y
hepaticojejunostomy, our preferred technique [13]. Simple cyst
excision can be performed depending on the anatomy of the cyst, as
described by Todani, and type III cysts remain an exception to these
guidelines because the risk of carcinoma is considered low, so, these
patients are effectively treated by endoscopic sphincterotomy [3,13].
Management of prenatally diagnosed cystic lesion of the biliary
tree is controversial. Conventionally, surgery is postponed until 3e6
months of age in asymptomatic patients because of the technical
difculties of the surgery and the risk of anesthesia during the
neonatal period [1,8,14]. In symptomatic patients with large cysts
an early surgery is indicated to conrm the diagnosis and avoid
complications such as gallstones, cholangitis, perforation, chole-
stasis, vomiting by compression of the stomach or intestine, and
even respiratory compromise [14]. However, some authors
 Y. Gonzlez Ruiz et al. / J Ped Surg Case Reports 4 (2016) 1e3
recommend excision of choledochal cyst and restoration of bile
ow before 2 weeks of life, even in asymptomatic patients
[6,15e17], in order to avoid the complications previously described
and prevent progressive liver damage leading to liver brosis and
biliary cirrhosis [6].
3. Conclusion
Prenatal diagnosis of choledochal cysts is important because it
can determine the evolution, decreasing the risk of complications if
an early surgical repair is done. Large choledochal cysts have a
higher risk of complications such as perforation and compression of
other organs. So, an early treatment is indicated, regardless if the
patient is asymptomatic.
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