Portal biliopathy: MR cholangiopancreatography features

e-Poster: C-010

Congress: ECR 2008

Type: Scientific Exhibit

Topic: Abdominal and Gastrointestinal / Abdominal Viscera (Solid Organs) / Biliary Tract

Authors: E. Özkavukcu, A. Erden, I. Erden; Ankara/TR

Keywords: Magnetic resonance cholangiopancreatography, Portal biliopathy

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1. Purpose

Portal biliopathy is a recent term defining the gall bladder, and biliary ductal abnormalities seen in
extrahepatic portal venous obstruction 1,2. MR cholangiopancreatography (MRCP) is a non-invasive
and highly effective technique which provides adequate information about portal biliopathy. The aim
of this study was to investigate the imaging features of portal biliopathy with emphasis on MRCP.

2. Methods and Materials

Patients

A total number of 16 patients (9 male, 7 female) with portal vein thrombosis (PVT) were included in
this study. All patients had undergone MRCP (n=16) studies between August 2004 and September
2007. Patients` ages were between 21-60 years with a mean of 39.3 years. All patients had chronic
stage PVT and portal cavernoma proven by MR portography, dynamic contrast enhanced CT or
dynamic contrast enhanced MRI of the liver. Six patients had hematological disorders, two patients
had idiopatic PVT, one patient had hydatid cysts of the liver, and one had multiple liver abcesses. Rest
of the patients had chronic liver diseases, mostly of viral origin. Patients with other causes of biliary
abnormality, such as primary sclerosing cholangitis etc., or tumors causing biliary obstruction like
hepatocellular, cholangiocellular, or pancreatic carcinomas were not included in the study. Because of
the retrospective nature of the study informed patient consent was not required.

Technique

All the MRI studies were performed with a 1 Tesla MR system using a phased-array torso coil. MRCP
studies were consisted of radially oriented thick slabs of heavily T2- weighted
cholangiopancreatography images using “single shot fast spin-echo” (SSFSE) sequence (TR = 5834
msec, TE = 1146 msec, band width = 25 kHz, FOV = 27 cm, slice thickness= 40 mm, spacing= 0, image
matrix=256x256, NEX=0.94) and axial fast spin echo T2-weighted MR images with respiratory
triggering (TR= 5217 msec, TE = 102 msec, band width = 41.67 kHz, FOV= 34 cm, slice thickness= 7.5
mm, spacing= 1.5 mm, image matrix = 320x224, NEX = 3) involving the liver, and the biliary system.

Image evaluation

Two radiologists evaluated all examinations that were archived digitally on a work-station, together,
retrospectively. All disagreements were resolved by consensus. MRCP images were analyzed for the
presence of biliary stenosis (including the quantity [whether solitary or multiple] and location of the
stenosis), upstream (prestenotic) dilatation, wavy appearance of the bile ducts, angulation or
displacement of the common bile duct (CBD), and choledocholithiasis. Finally, categorization of the
patients was made, based on the MRCP appearances of the biliary tree, using the classification system
suggested by Chandra et al.1 (Type I: involvement of extrahepatic bile duct; type II: involvement of
intrahepatic bile ducts only; type IIIa: involvement of extrahepatic bile duct and unilateral intrahepatic
bile duct [left or right]; type IIIb: involvement of extrahepatic bile duct and bilateral intrahepatic ducts).

3. Results

All of the patients had at least one sign of portal biliopathy (biliary stenosis, upstream dilatation, wavy
appearance of the biliary tree, or angulation of the CBD) on MRCP studies (Figs. 1-5).

Only one patient didn’t have stenosis of the biliary system. Rest of the patients (n=15; 93.7%) had
either solitary, but mostly multiple biliary stenosis (n=5; 33.3%, n=10; 66.6% respectively). Common
hepatic duct or CBD were invariably involved in all cases that had stenosis. Among them, 7 patients
also had stenosis of the right or left hepatic bile ducts.

Upstream or prestenotic dilatation was observed in 11 (n=11, 68.7%) patients.

Most of the patients had (n=14; 87.5%) mural wavy appearance of the biliary tree. Only 2 patients did
not show such abnormalities.

None of the patients had choledocholithiasis.

On MRCP images, 12 patients (n=12, 75%) showed angulation or displacement of the CBD due to
compression of the portal cavernoma.

According to the classification system suggested by Chandra et al., 6 patients (n=6, 37.5%) had type I,
6 patients (n=6, 37.5%) had type IIIa, and 4 patients had type IIIb (n=4, 25%) changes of the biliary tree.

The portal biliopathy findings and their frequencies on MRCP are given in the Table.

4. Conclusions

The rate of portal biliopathy in patients with extrahepatic portal venous obstruction is reported to be
70-100% in the previous literature 1-6. Supporting the relevant literature, all patients had signs of
portal biliopathy on both MRCP and MR portography examinations in our study (100%).

In their study including the MRCP examinations of portal cavernoma patients coupled with MR
portography, Condat et al.4, found out that the most common MRCP findings were biliary stenosis,
upstream dilatation, angulation, and bile duct parietal irregularities (this finding may correspond to
the “wavy appearance” in our study), respectively. Although “wavy appearance” of the bile ducts is
found more common in our study, percentages of the other MRCP findings were comparable.

As mentioned before, although most patients are asymptomatic 1,3,4, portal biliopathy can cause
obstructive jaundice, or cholangitis and may contribute to the development of choledocholithiasis 1.
Clinical problems or the biochemical parameters of cholestasis were not taken into consideration in
our study. Yet, like the low rates of choledocholithiasis reported in the previous literature 2,4, none of
our patients had choledocholithiasis on MRCP examinations.

According to Chandra’s classification system1, most patients had either type I, or type IIIa biliary
changes (totally 75% of the patients) in our study, whereas, rest of the patients (25%) had type IIIb
changes. Thus, common hepatic duct or CBD were involved in all cases that had biliary stenosis. These
rates are suggestive of the previous finding (by Condat et al.) that, the CBDs or extrahepatic bile ducts
are mostly affected, in terms of biliary stenosis 4.

Because endoscopic retrograde cholangiography (ERC) is an invasive procedure and abdominal

ultrasonography may not give a definite outline of the biliary ductal anomalies, MRCP has been found
to be helpful in the diagnosis of several biliary tract abnormalities 1,2,4,5. Results show that the MRCP
findings are similar to ERC4, yet it can overestimate the severity of the biliary stenosis in portal
biliopathy 5. Still, ERC should be performed only if endoscopic treatment (such as dilatation or
stenting) is planned 7.

In conclusion, portal biliopathy is very common in patients with portal cavernomas. MRCP, particularly
when coupled with MR portography is a highly effective, and non-invasive method in showing the
features of portal biliopathy. In this study, features of portal biliopathy on MRCP in order to their
frequencies were as follows: biliary stenosis, wavy appearance of the bile ducts, angulation of the CBD,
and upstream (prestenotic) dilatation.

5. References

1. Chandra R, Kapoor D, Tharakan A, Chaudhary A, Sarin SK. Portal biliopathy. J Gastroenterol

Hepatol 2001;16(10):1086-92.
2. Shin SM, Kim S, Lee JW, et al. Biliary abnormalities associated with portal biliopathy: evaluation
on MR cholangiography. AJR Am J Roentgenol 2007;188(4):341-7.
3. Khuroo MS, Yattoo GN, Zargar SA, et al. Biliary abnormalities associated with extrahepatic portal
venous obstruction. Hepatology 1993;17(5):807-13.
4. Condat B, Vilgrain V, Asselah T, et al. Portal cavernoma-associated cholangiopathy: a clinical and
MR cholangiography coupled with MR portography imaging study. Hepatology
2003;37(6):1302-8.
5. Chevallier P, Denys A, Novellas S, Schmidt S, Schnyder P, Bruneton JN. Magnetic resonance
cholangiography features of biliary abnormalities due to cavernous transformation of the portal
vein. Clin Imaging 2006;30(3):190-4.
6. Dhiman RK, Puri P, Chawla Y, et al. Biliary changes in extrahepatic portal venous obstruction:
compression by collaterals or ischemic? Gastrointest Endosc 1999;50(5):646-52.
7. Perlemuter G, Béjanin H, Fritsch J, et al. Biliary obstruction caused by portal cavernoma: a study
of 8 cases. J Hepatol 1996;25(1):58-63.
6. Mediafiles

Figure 1

MRCP image of a 28-year-old female patient with portal cavernoma due to hematological disorder.
Multiple biliary stenoses involving the common bile duct and both hepatic ducts are seen. Thus,
this was classified as type IIIb change according to Chandra. Wavy appearance of the biliary tree,
prestenotic dilatations and angulation of the common bile duct were also noted.
Table
figure 2

MRCP image of a 49-year-old male patient with portal cavernoma due to hematological disorder.
Biliary stenoses (arrows) involving the common bile duct and left hepatic duct are seen (type IIIa
change according to Chandra). Prestenotic dilatation (arrowhead) was also noted on the left side.
figure 3

MRCP image of a 40-year-old male patient with portal cavernoma, and cirrhosis due to chronic viral
hepatitis. Severe and multiple biliary stenoses (arrows) involving the common bile duct and right
hepatic duct are seen. Prominent dilatation of the intrahepatic bile ducts (prestenotic dilatations)
and wavy appearance of the biliary tree are also evident.
figure 4

MRCP image (a) and sequential contrast enhanced CT images (b, c) of a 35-year-old female patient
with portal cavernoma and hydatid cysts of the liver. On MRCP image, wavy appearance of the
biliary tree, and prominent stenoses (white arrowheads, a) involving the common bile duct (type I
change according to Chandra) due to the compression by the choledochal varices are noted.
Choledochal varices (black arrows and arrowheads in b, c) compressing the common bile duct and
gall bladder varices (white arrow, c) can be seen on CT images.
figure 5

MRCP images (a, b), coronal source image obtained at portal phase of MR angiography (c), and
axial postcontrast FSPGR MR image (d) of a 24-year-old male patient with portal cavernoma due to
hematological disorder. On the initial MRCP image (a), multiple stenoses (white arrows) at the
common bile duct (CBD), correlating well with the MR angiography findings are seen. The second
MRCP study (b) was performed at 8th month follow-up, following stenting of the CBD (white
arrowheads). Although most patients are asymptomatic, this patient was suffering from
progressive cholestasis due to portal biliopathy. The progress in the intrahepatic bile duct
dilatation can easily be noticed on the second MRCP study (b), despite the CBD stent. Contrast
enhanced MR images (c, d) are showing the choledochal varices (black arrows) surrounding the
common bile duct (black arrowheads).