ORI GI NAL ARTI CLE

Triangular Cord Sign in Detection of Biliary Atresia:

Is It a Valuable Sign?
Mohammad Hadi Imanieh Seyed Mohsen Dehghani Mohammad Hadi Bagheri
Vahid Emad Mahmood Haghighat Mozhgan Zahmatkeshan
Hamid Reza Forutan Ali Reza Rasekhi Farshid Gheisari
Received: 10 October 2008 / Accepted: 12 January 2009 / Published online: 20 February 2009
Springer Science+Business Media, LLC 2009
Abstract Background Early detection of biliary atresia
(BA) has a vital role in prevention of liver cirrhosis in these
patients. There are some evidences that triangular cord
(TC) sign, i.e., triangular structure located cranial to the
portal vein bifurcation on ultrasonographic examination, is
suggestive of BA in suspected cases. The aim of this study
is to evaluate and compare the sensitivity, specicity, and
accuracy of TC sign with other methods of diagnosis such
as hepatobiliary scan. Methods Fifty-eight infants referred
to pediatric gastroenterology ward with diagnosis of
infantile cholestasis from March 2004 to March 2008 were
evaluated to nd the cause of cholestasis. Diagnosis was
made by means of history, clinical examination, hepatob-
iliary scan, and liver biopsy. Ultrasonographic examination
was focused on presence of TC sign in patients. If the
diagnosis was in favor of BA, patient was sent for direct
cholangiography as a gold-standard test for conrmation of
the diagnosis. The sensitivity, specicity, and accuracy of
the tests were compared with golden standard. Results
Among 58 infants with infantile cholestasis, BA was
diagnosed and conrmed in 10 infants (17.2%). Hepatob-
iliary scintigraphy had 80% sensitivity, 72.9% specicity,
and 74.1% accuracy. TC sign had 70% sensitivity, 95.8%
specicity, and 91.3% accuracy. Conclusion TC sign is
more accurate than hepatobiliary scan and has acceptable
sensitivity and specicity for diagnosis of BA.
Keywords Biliary atresia Triangular cord sign
Accuracy
Introduction
Early detection of infantile cholestasis is the most impor-
tant factor for determination of prognosis, especially in
biliary atresia (BA) [1]. In 70% of cases, differentiation
between neonatal hepatitis and BA is a working diagnosis
[2]. The diagnosis of BA in infants is based on hepatobil-
iary scintigraphy, sonography, percutaneous liver biopsy,
and intraoperative cholangiography.
Stable general condition of BA patients is one of the
factors that delay referral to gastroenterologist.
Another factor is invasiveness of diagnostic methods,
which are also time consuming and costly.
In 1996, Choi et al. [1] reported a new sonographic
nding in BA patients, called triangular cord (TC) sign. It
is a triangular structure, composed of brous tissue located
cranial to the portal vein bifurcation. Although some
studies reported 100% accuracy of TC sign in detection of
BA [3], most studies report accuracy of 7090% for this
sign [4].
M. H. Imanieh S. M. Dehghani (&) V. Emad M. Haghighat
Gastroenterohepatology Research Center, Nemazee Hospital,
Shiraz University of Medical Sciences, Shiraz 71937-11351, Iran
e-mail: dehghanism@sums.ac.ir
M. H. Imanieh S. M. Dehghani M. Haghighat
M. Zahmatkeshan
Department of Pediatric Gastroenterology, Nemazee Hospital,
Shiraz University of Medical Sciences, Shiraz, Iran
M. H. Bagheri A. R. Rasekhi
Department of Radiology, Nemazee Hospital,
Shiraz University of Medical Sciences, Shiraz, Iran
H. R. Forutan
Department of Pediatric Surgery, Nemazee Hospital,
Shiraz University of Medical Sciences, Shiraz, Iran
F. Gheisari
Department of Nuclear Medicine, Nemazee Hospital,
Shiraz University of Medical Sciences, Shiraz, Iran
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Dig Dis Sci (2010) 55:172175
DOI 10.1007/s10620-009-0718-3
In this study we investigate the accuracy of TC sign
compared with hepatobiliary scintigraphy, liver biopsy,
and cholangiography in diagnosis of BA.
Patients and Methods
Participants in this study were infants referred to pediatric
gastroenterology ward afliated to Shiraz University of
Medical Sciences with diagnosis of infantile cholestasis
from March 2004 to March 2008. After preliminary
examination and blood tests, liver and biliary system
sonography was performed after at least 4 h fasting using
General Electric Logic 7 equipped with 7-MHz linear
transducer for all patients. The ultrasonographic examina-
tion focused on presence or absence of TC sign at the porta
hepatis as mentioned above. Hepatobiliary scintigraphy
was also performed for all patients. Percutaneous liver
biopsy was the next step of diagnosis and differentiating
between neonatal hepatitis and BA. The diagnosis of BA in
liver biopsy was suggested by presence of bile duct pro-
liferation, bile rosette, or other features suggestive of
extrahepatic obstruction. If the diagnosis was in favor of
BA, patient was sent for direct cholangiography for con-
rmation of the diagnosis. Kasai operation was done for
BA patients.
Presence of TC sign was investigated in patients con-
sidering the denite diagnosis by means of liver biopsy and
cholangiography.
Results
Of 58 patients recruited for the study, 25 patients were
female (43.1%) and 33 patients were male (56.9%) with
mean age of 46 14 days (range 30120 days). The
beginning onset of the symptoms in infants ranged from 1
to 120 days old (mean 32.4 33.5 days).
Among 58 infants with infantile cholestasis, BA was
diagnosed and conrmed in 10 infants (17.2%). Other
causes of cholestasis are shown in Table 1.
From ten infants with denite diagnosis of BA, hepa-
tobiliary scintigraphy was positive in eight patients
(sensitivity = 80%).
Thirteen patients had positive hepatobiliary scan for BA
but further workups conrmed other diagnoses [specic-
ity = 72.9%, positive predictive value (PPV) = 38.1%,
negative predictive value (NPV) = 94.5%] (Table 2).
From ten patients with conrmed BA, seven patients
(70%) had TC sign in their ultrasonographic examination
(sensitivity = 70%).
Two patients had TC sign in the ultrasonographic
examination but further workups revealed the diagnosis of
neonatal hepatitis (specicity = 95.8%).
Presence of TC sign in ultrasonographic examination
was signicantly correlated with diagnosis of BA in
infants, with PPV of 77.8% and NPV of 93.9%.
(P = 0.0002) (Table 3).
In the validity study, the percentage agreement between
ultrasonographic nding of TC sign and gold standard
(accuracy of the test) was 91.3%. The calculated accuracy
for hepatobiliary scan was 74.2%. Combination of hepa-
tobiliary scan and TC sign in ultrasonography showed that
nine of ten conrmed BA patients had at least one positive
result for these diagnostic methods (sensitivity 90%). The
number of false-positive and false-negative results of
combination diagnosis was 12 and 1, respectively (speci-
city = 79.9%, PPV = 40.9%, NPV = 97.2%).
Table 1 Final diagnosis of patients referred for infantile cholestasis
Idiopathic
neonatal
hepatitis
Biliary
atresia
Progressive
familial
intrahepatic
cholestasis
Biliary
sludge
Cystic
brosis
Galactosemia Cytomegalovirus
hepatitis
Glycogen
storage
disease
Alagille
syndrome
Niemann
Pick
disease
23
(39.6%)
10
(17.2%)
7
(12.1%)
4
(6.9%)
3
(5.2%)
3
(5.2%)
2
(3.4%)
2
(3.4%)
2
(3.4%)
2
(3.4%)
Table 3 Comparison of the results of ultrasonography and
cholangiography
Positive
TC sign
Negative
TC sign
Cholangiogram in favor of BA 7 3
Cholangiogram against BA 2 46
Table 2 Comparison of the results of hepatobiliary scintigraphy and
cholangiography
Hepatobiliary
scan in favor
of BA
Hepatobiliary
scan against
BA
Cholangiogram in favor of BA 8 2
Cholangiogram against BA 13 35
Dig Dis Sci (2010) 55:172175 173
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Discussion
Immediate treatment of BA has vital role in prevention of
liver cirrhosis. Accurate and early diagnosis is important
for early treatment.
Invasive and expensive methods of diagnosis of BA are
two major factors that delay the process of diagnosis in
golden time.
Liver biopsy is a valuable procedure to differentiate
neonatal hepatitis from BA [5]. Although liver biopsy is
safe, it is still an invasive procedure and needs hemody-
namic and coagulation system stability. Impaired liver
function in patients sometimes leads to coagulopathies that
delay the process of liver biopsy. It will be advantageous
for patients to nd a noninvasive method to replace liver
biopsy for diagnosis of BA.
In the study, performed in our center between 2003 and
2006, sensitivity, specicity, PPV, and NPV of different
diagnostic methods was evaluated and compared with
direct cholangiography as the gold-standard method for
diagnosis of BA [5]. Ultrasonography, which is noninva-
sive, nonionizing, less expensive, and produces real-time
images, is still a preferable tool for evaluation of hepa-
tobiliary tree, especially in infants and neonates who need
sedation for magnetic resonance imaging. Reported accu-
racies of the hepatobiliary scintigraphy and ultraso-
nography (not on the basis of TC sign) for detection of BA
were 58.5% and 69.2%, respectively [5].
After reports of Choi et al. [1] and Park et al. [68]
about the sensitivity and specicity of TC sign in diagnosis
of BA, it became a major interest to evaluate and stan-
dardize the accuracy of this sign in diagnosis of BA.
Our research showed 70% sensitivity and 95.8% speci-
city of TC sign in diagnosis of BA.
The specicity of TC sign (95.8%) is much higher
compared with hepatobiliary scintigraphy (72.9%). The
PPV of TC sign is two times higher than hepatobiliary
scintigraphy (77.8% versus 38.1%). To improve the accu-
racy of ultrasonographic imaging in the diagnosis of BA,
Park et al. [6] proposed combining TC sign with gallbladder
imaging. Positive TC sign coupled with abnormal gall-
bladder length improves PPV value to 100% in the
diagnosis of BA. Recent study showed PPV and NPV in the
diagnosis of BA of 98 and 100%, respectively, if positive
TC sign was combined with either abnormal size or
abnormal contractility of the gallbladder [9]. Our study
showed that TC sign has much higher accuracy compared
with hepatobiliary scan. Statistical analysis showed agree-
ment of 73.6% between TC sign and hepatobiliary scan.
Also Wongsawasdi et al. [10] suggested that TC sign is a
noninvasive and easily available test when combined with
acholic stool and gammaglutamyl transpeptidase level.
It seems that positive TC sign in ultrasonography is
accurate enough to bypass liver biopsy and to candidate
patient for intraoperative cholangiography, but in case of
clinical suspicion of BA and negative TC sign, liver biopsy
is essential to conrm the diagnosis. The suggested algo-
rithm for evaluation of suspicious infant to have BA is
shown in Fig. 1.
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174 Dig Dis Sci (2010) 55:172175
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