Classification of choledochal cyst with MR

cholangiopancreatography in children and infants: special

reference to type Ic and type IVa cyst

Poster No.: C-1333

Congress: ECR 2011
Type: Educational Exhibit
Authors: A. S. Littooij, M. V. Fortier; Singapore/SG
Keywords: Pediatric, Abdomen, Biliary Tract / Gallbladder, MR
DOI: 10.1594/ecr2011/C-1333

Any information contained in this pdf file is automatically generated from digital material
submitted to EPOS by third parties in the form of scientific presentations. References
to any names, marks, products, or services of third parties or hypertext links to third-
party sites or information are provided solely as a convenience to you and do not in
any way constitute or imply ECR's endorsement, sponsorship or recommendation of the
third party, information, product or service. ECR is not responsible for the content of
these pages and does not make any representations regarding the content or accuracy
of material in this file.
As per copyright regulations, any unauthorised use of the material or parts thereof as
well as commercial reproduction or multiple distribution by any traditional or electronically
based reproduction/publication method ist strictly prohibited.
You agree to defend, indemnify, and hold ECR harmless from and against any and all
claims, damages, costs, and expenses, including attorneys' fees, arising from or related
to your use of these pages.
Please note: Links to movies, ppt slideshows and any other multimedia files are not
available in the pdf version of presentations.
www.myESR.org

Page 1 of 15
Learning objectives

In this poster, we illustrate the different types of choledochal cysts seen in a pediatric
population and outline the importance of differentiating between dilatation of the intra-
hepatic ducts due to either obstruction or extention of choledochal cyst. The diagnostic
features that can help in making the accurate diagnosis will be described.

Background

Choledochal cysts are congenital dilatations of the biliary tree usually found in infancy or
childhood. The incidence is much higher in Asia (Fig 1) and is approximately three times
more frequent in females. Only 20% of the cases will present with the classical triad of
abdominal pain, palpable mass and jaundice.

Choledochal cysts are usually classified according to Todani into five types (Fig 2): Type
I, occuring up to 80-90% of the cases, consists of dilatation of the extrahepatic bile
ducts. This dilatation may be cystic (Type 1a), focal (Type Ib) or fusiform (Type 1c). Type
II describes a diverticulum in the CBD. Type III, also called choldochocele, is a focal
dilatation of the distal common bile duct in the papillary region into which the pancreatic
ducts drain. Type IVa shows multiple dilatations in the extra and intrahepatic bile ducts,
whereas type IVb represents multiple dilatations in only the extrahepatic bile duct. Type
V is synonymous with Caroli disease.

The choledochal cysts seen in the pediatric population are type I and IVa cysts. These
cysts are highly associated with an abnormal junction of the common bile duct with
the pancreatic duct and this is generally accepted as the cause of it. The abnormal
anomalous pancreaticobiliary union (APBU) is characterized by fusion of pancreatic duct
and common bile duct outside the duodenal wall, with formation of a long common
channel, usually more than 15mm (Fig 3 and 4). This APBU allows reflux of pancreatic
enzymes into the biliary tract causing cholangitis with subsequent dilatation of the bile
duct.

The standard classification system for choledochal cysts is based on the

cholangiographic morphology, location and number of extrahepatic and intrahepatic bile
duct dilatations. This classification is confusing because it includes distinct disease
entities such as Caroli's disease, diverticulum and choledochocele. Furthermore, the
distinction between type I and IVa is arbitrary for the intrahepatic ducts are seldom
completely normal, suggesting they are variations of the same disease entity and defining
is arbitrary.

Page 2 of 15
Complications of untreated choledochal cysts of varying severity are reported ranging
from lithiasis and infection to cyst rupture (Fig 5), biliary cirrosis and cholangiocarcinoma.

The initial imaging modality of choice is ultrasound often followed by MR

cholangiopancreatography (MRCP). MRCP provides accurate preoperative evaluation
to plan for total cyst resection. Resection and Roux-en-Y hepaticojejunostomy (Fig 6) is
the management of choice. With this only the extrahepatic cyst is removed. In type IVa
cyst, the intrahepatic disease remains and is prone to bile stasis, stone/sludge formation
and cholangiocarcinoma. These risks are alleviated by wide hilar anastomosis. Localized
intrahepatic disease may be treated by segmental hepatic lobectomy.

Images for this section:

Fig. 1: Table 1

Page 3 of 15
Fig. 2: Choledochal cysts - Todani's Classification

Page 4 of 15
Page 5 of 15
Fig. 3: The pancreaticobiliary union

Page 6 of 15
Page 7 of 15
Fig. 4: 6 year old girl presented with abdominal pain. MRCP shows choledochal cyst
with intrahepatic extention and minor strictures (red arrow) in the hepatic hilum. Common
channel with stones (yellow arrow) is identified.

Fig. 5: 7 year old boy presented with abdominal pain and fever. MRCP (a-c) shows
choledochal cyst with obstruction (red arrow). Periportal edema and gallbladder wall
thickening (yellow arrow) suggest cholangitis and cholecystitis. US (d-f) 2 days later
revealed decrease in dilatation of the biliary tract with # ascites, representing cyst rupture.
The cholangiogram (g) after conservative treatment shows type IVa cyst without contrast
lekkage.

Page 8 of 15
Fig. 6: Treatment of choledochal cysts: complete resection and hepaticojejunostomy

Page 9 of 15
Imaging findings OR Procedure details

Generally, the type Ic cysts are defined as fusiform dilatation of the common bile duct and
common hepatic duct without involvement of the intrahepatic bile ducts (Fig 1). When
there is associated obstruction due to calculi and/or sludge, dilatation of the intra-hepatic
ducts is often seen (Fig 2). This can lead to confusion with type IVa cysts that show
multiple cystic dilatations of extra- and intra-hepatic bile ducts due to strictures (Fig 3).
To differentiate between type Ic and type IVa cyst it is important to demonstrate the
obstructing calculus, looking for signs of obstruction, such as dilatation of the pancreatic
duct and hydrops of the gallbladder as well as to correlate findings with biochemical
markers (Fig 2). Identifying a ductal stricture can have important implications. Such a
stricture is characteristic of type IVa cysts (Fig 4) and determines surgical management,
especially when located around the hepatic hilum, because this stricture can be corrected
by portal dissection of the liver to obtain a large anastomosis. Identifying such as stricture
is therefore more important than classifying the cyst correctly as type Ic or IVa.

Images for this section:

Fig. 1: Type 1 choledochal cyst

Page 10 of 15
Fig. 2: Type IVa or Type Ic Choledochal cyst? 2 ½ year old boy presenting with jaundice.
US (a,b) and MRCP (c,d) show choledochal cyst with intrahepatic dilatation & obstruction.
Intra-operative cholangiogram (e) after stone removal, revealed decrease in intrahepatic
dilatation. Diagnosis?

Page 11 of 15
Page 12 of 15
Fig. 3: 6 year old girl presented with abdominal pain. MRCP shows choledochal cyst
with intrahepatic extention and minor strictures (red arrow) in the hepatic hilum. Common
channel with stones (yellow arrow) is identified

Fig. 4: 3 years old girl, presented with abdominal pain and vomiting MRCP shows
choledochal cyst with intrahepatic extention and strictures

Page 13 of 15
Conclusion

Choledochal cysts are rare congenital dilatations of the biliary tree. They require
preoperative evaluation to plan for total cyst resection, generally by means of MRCP.
The most common used classification is devised by Todani et al. We believe that this
classification system is confusing as it includes distinct disease entities with different
etiology, prognosis and therapy. Futhermore, differentiation between the congenital
choledochal cysts seen in the pediatric population is arbitrary as the intrahepatic bile
ducts are seldom completely normal. Therefore, differentiation between intrahepatic
extention due to obstruction or extention of the choledochal cyst is challenging. Detecting
a ductal stricture in the hepatic hilum is most important, because this stricture changes
operative management.

Personal Information

Annemieke S. Littooij

Department of Diagnostic Imaging

KK Women's and Children's Hospital Singapore

100 Bukit Timah Road

229899 Singapore

alittooij@hotmail.com

Marielle V. Fortier

Department of Diagnostic Imaging

KK Women's and Children's Hospital Singapore

100 Bukit Timah Road

229899 Singapore

Marielle.Fortier@kkh.com.sg

Page 14 of 15
References

- Todani, Watanabe Y, Narusue M, Tabuchi K, Oka jima K. Congenital bile duct cysts:
classifiaction, operative procedures and review of thirty-seven cases including cancer
arising form choledochal cyst. J. Surg 1977;147:672-676

- Mortele KJ, Rocha TC, Streetr JL, Taylor AJ. Multimodality imaging of pancreatic and
biliary congenital anomalies. Radiographics 2006;26:715-731

- Visser BC, Suh I, Way LW, Kang SM. Congenital choledochal cysts in adults. Arch Surg
2004; 139 (8):855-860

- Kim MJ, Han SJ, Kim JH, Oh JT, Chung KS, Yoo HS. Using MR
Cholangiopancreatography to reveal anomalous pancreaticobiliary ductal union in infants
and children with choledochal cysts. AJR 2002; 179: 209-214

- Chavhan GB, Babyn PS, Manson D, Idarsson LV. Pediatric

MR Cholangiopancreatography:Principles, technique,and clinical applications.
Radiographics 2008; 28: 1951-1962

Page 15 of 15