Imaging, 22 (2013), 43631920

GASTROINTESTINAL IMAGING

Imaging of the jaundiced child

H E WOODLEY, MRCP, FRCR

Department of Radiology, Leeds Teaching Hospitals Trust, Leeds, UK

Summary
In infants and older children ultrasound remains the initial radiological investigation
and further imaging is dependent upon the sonographic findings.
Advances in MRI technique in the evaluation of the biliary tract in children avoids
more invasive investigations.
Imaging primarily aims to differentiate extrahepatic from intrahepatic causes.
Any neonate with jaundice persisting beyond 2 weeks warrants investigation to
exclude biliary atresia as diagnosis prior to 8-weeks-old achieves the best long term
surgical success.
Gallstones are increasingly prevalent in older children but underlying choledochal
doi: 10.1259/imaging/
cysts should be sought when bile duct stones are found. 43631920
Hepatic and pancreatic masses are a rare but important differential in the
investigation of jaundice and require evaluation by CT or MRI. 2013 The British Institute of
Radiology

Cite this article as: Woodley HE. Imaging of the jaundiced child. Imaging 2013;22:43631920.

Abstract. This article outlines a practical approach to the Imaging techniques

investigation of the jaundiced child and reviews the imaging
modalities and findings that can establish a diagnosis or
narrow the differential. A systematic approach to the work-up
of the jaundiced child prevents unnecessary tests and facilitates
prompt treatment. Imaging of the jaundiced neonate and infant
is reviewed separately to that of the older child and adolescent
owing to different aetiologies in the two age groups.
There are wide and varied differential diagnoses for the cause of
jaundice in a child, which are broadly classified as pre-hepatic,
hepatic and post-hepatic. Imaging does not play a significant
role in elucidating the cause of pre-hepatic (unconjugated
hyperbilirubinaemia); the underlying causes include physio-
logical jaundice, breast milk jaundice, haemolysis and sepsis.
Imaging does, however, play an important role in the
investigation of conjugated hyperbilirubinaemia, which nearly
always reflects hepatic dysfunction. One of the major roles of
imaging is to differentiate intrahepatic from extrahepatic
causes of jaundice although the underlying diagnosis is often
ultimately established by a combination of clinical presentation
and findings, laboratory tests, imaging and histology. Initial
laboratory tests include liver function tests (including
conjugated bilirubin fraction), hepatitis screening, TORCH
(Toxoplasmosis, Other (syphilis, hepatitis, zoster), Rubella,
Cytomegalovirus and Herpes) titres, a sepsis screen (blood,
urine and cerebrospinal fluid (CSF)), metabolic screen (e.g.
alpha1-antitrypsin phenotype testing) and sweat test, and in
older children a toxic screen and autoantibodies. These results
help to determine the most appropriate imaging.
Address correspondence to: Helen Woodley, St. James University
Hospital, Beckett Street, Leeds, UK. LS9 7TF. E-mail: helen.woodley@
leedsth.nhs.uk
Ultrasound
Ultrasound examination remains the imaging inves-
tigation of choice in the initial evaluation of the jaundiced
child. It is a non-invasive, non-ionising imaging modality,
independent of liver function and serves as an important
tool for differentiating between obstructive and non-
obstructive causes of jaundice [1]. Ultrasound evaluation
consists of a systematic review of the right upper quadrant
including liver size and texture, bile ducts, gallbladder,
pancreas, spleen and colour Doppler examination of the
hepatic vessels and collateral vessels. The entire abdomen
and pelvis should be reviewed to exclude ascites, evidence
of neoplasia or lymphadenopathy and a high frequency
linear probe should be available to review the bowel and
gallbladder wall.
The right hepatic lobe should extend no more than 1 cm
below the costal margin in a young infant without pul-
monary hyperinflation and not below the right costal
margin in older infants and children. Normal echotexture
of the hepatic parenchyma in the paediatric liver does not
differ from that of adults; echogenicity is low to medium
and homogeneous with peripheral portal venous vascula-
ture clearly seen. Intrahepatic and extrahepatic bile ducts
should be measured to assess ductal dilatation. The size of
the common bile duct (CBD) increases linearly with age.
The CBD should measure ,1 mm in neonates, ,2 mm in
infants up to a year, ,4 mm in children older than 1 year
and ,7 mm in adolescents [1, 2]. The proximal intrahepatic
ducts can be seen but should not exceed 2 mm. The

imaging.birjournals.org 1 of 14

gallbladder size and wall thickness should be measured.
The normal gallbladder gradually increases in size with
age; 1.53 cm in length in infants ,1 year old and 37 cm
in older children [3]. Gallbladder wall thickness is similar
to adults and should not exceed 3 mm. The pancreatic
size, echotexture and duct should be evaluated. The
pancreas grows substantially in the first year of life with
much slower growth thereafter. The gland is relatively
larger in children compared with adults; the head
measures 12.2 cm, body 0.41 cm and tail 0.81.8 cm
[1]. The mean pancreatic duct diameter should not
exceed 2 mm [4]. Spleen size and texture must be
evaluated. The upper limit of normal is 6 cm in infants
03 months and up to 12 cm in children aged between
12 and 15 years old [5].
Hepatobiliary scintigraphy
Hepatobiliary scintigraphy is used to evaluate pa-
tency and function of the biliary tree. 99Tcm iminodi-
acetic acid (IDA) derivatives that are actively taken up
by hepatocytes and excreted into bile canaliculi are used
for paediatric hepatobiliary scintigraphy. In children
with decreased hepatic uptake, the accuracy of hep-
atobiliary iminodiacetic acid (HIDA) scan is improved
by phenobarbitol induction (5 mg kg21 daily for 3 days)
which enhances excretion of the radiopharmoceutical
into the bile [6]. HIDA is commonly used to investigate
the jaundiced neonate to differentiate biliary atresia
from other causes of neonatal jaundice, but is also
useful to demonstrate biliary leaks in trauma and post-
liver transplantation and assessment of choledochal
cysts.
Computed tomography
CT has limited indications in the investigation of
jaundice in the child. CT may be useful to characterise
a mass lesion or in the staging of malignant disease but
involves a significant radiation dose and intravenous in-
fusion of iodinated contrast agents. MRI has superseded
CT in the evaluation of the biliary tree and liver
parenchyma.
Magnetic resonance imaging
Magnetic resonance cholangiopancreatography (MRCP)
is a well established, non-invasive technique for the in-
vestigation of adults with hepatobiliary disease and is
now increasingly being used in children to evaluate the
biliary tree [7, 8]. The disadvantages are that MRI is not
always readily available, requires general anaesthesia in
most young children and the resolution is still limited in
the neonate. Advances in technique have improved im-
age quality and adaptations for children include coil se-
lection, respiratory compensation techniques, echo time
(TE), echo train length, slice thickness and field of view
[9]. For adults thick slab single shot fast-spin echo (FSE)
(long TE) provides an overview of the biliary system in
coronal and coronal oblique planes and thin slice half-
fourier acquisition single-shot turbo-spin echo (HASTE)
(moderate TE) improves visualisation of fine structures
obtained in coronal oblique and axial planes. MRCP is
useful in the investigation of choledocholithiasis, scle-
rosing cholangitis, pancreaticobiliary malunion and more
2 of 14
H E Woodley
recently has been advocated in the investigation of neo-
natal jaundice [10].
MRI is an excellent modality for characterising mass
lesions within the liver and pancreas [7]. Evaluation of
a mass lesion requires T1 weighted, T2 weighted and
dynamic post-gadolinium enhanced sequences. Prob-
lematic cases may also require liver specific agents.
Direct cholangiography
Endoscopic retrograde cholangiopancreatography
(ERCP) and percutaneous transhepatic cholangiography
(PTC) are technically difficult in children and ERCP is not
possible in patients with biliary enteric anastomoses.
ERCP has recently been advocated for the investigation
of neonatal cholestasis [11], but other non-invasive mo-
dalities remain as first line investigations. The main role
of ERCP occurs when intervention is required but stone
disease and tumours requiring intervention are less
common in children, obviating the need for this invasive
technique. PTC has a role in the treatment of post-
operative stenoses and biliary complications of liver
transplantation and may be used in the treatment of
cholelithiasis of infancy [12].
Investigation of neonatal jaundice
Neonatal jaundice is a common finding in general
paediatrics; 60% of normal full-term infants and 80% of
preterm infants [1] develop physiological jaundice within
the first 2 weeks of life owing to immaturity of the en-
zyme glucuronosyl transferase. However, infants who
are jaundiced beyond the first 2 weeks of life require in-
vestigation to exclude conjugated hyperbilirubinaemia
and underlying liver disease.
The major causes of jaundice in the neonate are listed
in Table 1. Of jaundiced infants with underlying liver
disease, 90% will have either biliary atresia or neonatal
hepatitis. Imaging aims to differentiate intrahepatic cau-
ses of jaundice from extrahepatic causes, in particular
excluding biliary atresia, and ultrasound will be the first
imaging modality of choice. Intrahepatic causes of jaun-
dice often have non-specific imaging findings and the
diagnosis relies more on biochemical laboratory tests
with or without liver biopsy, although imaging may
demonstrate extrahepatic features of these disorders, e.g.
the skeletal abnormalities associated with Alagilles
syndrome.
Biliary atresia
Biliary atresia is the most common cause of neonatal
cholestasis affecting 1 in 16 000 live births in the UK.
Biliary atresia is a congenital obliteration of the extra-
hepatic bile duct although the intrahepatic bile ducts
are often abnormal and irregular. There are three main
types; Type 3 (occlusion at the level of the porta hepatis
with atresia of the whole extrahepatic duct system) is
the most common and accounts for more than 90% of
cases, Type 2 (atresia of the hepatic duct with residual
patency of the right and left hepatic ducts) and Type 1
(atresia of the CBD with proximal patent ducts)
(Figure 1). Types 1 and 2 have a better prognosis. In
Imaging 2013, 22, 43631920
Imaging of the jaundiced child

Table 1. Causes of neonatal jaundice

Extrahepatic Biliary atresia
Choledochal cyst
Bile plug syndrome
Cholelithiasis
Spontaneous perforation of
CHD
Duodenal duplication
Intrahepatic disorders Bile duct paucity
Alagilles
Non-syndromic
Neonatal sclerosing cholangitis
Parenchymal disease
Bylers
Idiopathic neonatal hepatitis
Infection
CMV
Rubella
Herpes simplex
Coxsackie B virus
Echovirus
Congenital syphilis
Toxoplasmosis
Toxic/metabolic
TPN Figure 1. Classification of the main types of biliary atresia.
a1-antitrypsin
Cystic fibrosis
Galactosaemia If ultrasound features suggestive of biliary atresia are
Tyrosinaemia present or the diagnosis cannot be excluded, a HIDA scan
Endocrine should be performed. In biliary atresia there is non-
Hypothyroidism
excretion of bile into the bowel by 24 h (Figure 3). How-
Panhypopituitanism
ever, hepatobiliary scanning is not diagnostic as non-
CHD, common hepatic duct; CMV, cytomegalovirus; TPN, excretion may indicate severe neonatal hepatitis or the
total parental nutrition. presence of interlobular bile duct paucity [22].
Following ultrasound and HIDA scan, if the diagnosis
remains in doubt but biliary atresia remains a diagnostic
Type 3 the gallbladder is small or absent and con- possibility, percutaneous liver biopsy may be considered.
tains clear mucus only. Occasionally a cyst is found MRCP has been advocated to demonstrate the biliary
proximal or distal to the atretic bile duct at the porta structures and diagnose biliary atresia [7, 23] and to
hepatis. Syndromic biliary atresia (biliary atresia demonstrate the triangular cord sign [24] but this tech-
splenic malformation (BASM)) occurs in 1020% of nique requires further evaluation. ERCP has been used in
cases and consists of polysplenia (or rarely, asplenia), the diagnosis of biliary atresia but is technically difficult
interrupted inferior vena cava (IVC), pre-duodenal and rarely performed [11]. Percutaneous cholecysto-
portal vein, intestinal malrotation, situs inversus and cholangiography under ultrasound guidance can also be
cardiac defects. used to assess the biliary tree when a gallbladder is large
Early diagnosis of biliary atresia (,8 weeks old) and enough to perform this procedure safely [25].
prompt surgery improves outcome. Historically a combi- When imaging or biopsy histology is compatible with
nation of tests is usually required to diagnose biliary biliary atresia a Kasai portoenterostomy is performed.
atresia with certainty, although in experienced hands it This is usually preceeded by an operative cholangiogram
can be diagnosed by ultrasound alone [13]. At ultrasound to confirm the diagnosis (Figure 4). Resolution of jaundice
the echogenicity of the liver parenchyma is not usually after surgery is more likely if surgery is performed before
helpful but may be increased, the intrahepatic bile ducts 8 weeks of age when larger ductules are present at the
are not dilated and non-recognition of the CBD is not porta hepatis and in non-syndromic cases of biliary
diagnostic. A number of more specific ultrasound fea- atresia.
tures have been described [14]. These include: (i) the tri-
angular cord sign that represents the fibrotic remnant of
the hepatic duct junction and is seen as an echogenic
Neonatal hepatitis
triangular or tubular structure just cranial to the bi-
furcation of the portal vein at the portal hepatis Neonatal hepatitis, for which there are many un-
(Figure 2a) [1517], (ii) a small or absent gallbladder with derlying diagnoses (Table 1), is the main differential to
irregular walls [18, 19] (Figure 2b) although a small gall- biliary atresia. Often there are no specific ultrasound
bladder may also be seen in neonatal hepatitis, (iii) lack of features, although the liver may be enlarged with in-
gall bladder contractibilty [21] and (iv) increased hepatic creased echogenicity. Usually the biliary system is un-
artery dimension [21]. remarkable with a normal sized gallbladder, although in
Occasionally a cyst is present at the porta hepatis severe hepatocellular dysfunction the gallbladder may be
(Figure 2c) and there may be evidence of BASM. decreased in size owing to decreased volume of bile.

imaging.birjournals.org 3 of 14

(a)
(c)
Figure 2. Ultrasound images of jaundiced 3-week-old infant demonstrate (a) echogenic area anterior and cranial to the portal
bifurcation in keeping with the triangular cord sign; (b) small gallbladder with irregular wall; and (c) a cyst at the porta hepatis, in
keeping with a diagnosis of cystic biliary atresia.
Severe neonatal hepatitis may also show non-excretion of
bile on the HIDA scan and these cases may progress to
liver biopsy to exclude biliary atresia. MRCP may play
a role in this group in the future [8].
Choledochal cyst
Choledochal cysts are cystic malformations of the bil-
iary tree. They can be classified into five types (Figure 5).
The aetiology is thought to be owing to anomalous
pancreaticobiliary ductal union with a long common
channel that allows the reflux of pancreatic juice into the
bile duct.
Choledochal cysts may present incidentally or pre-
natally, but most commonly present with jaundice with
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H E Woodley
(b)
or without abdominal pain and 30% present before
1 year of age [2]. Children may present with secondary
complications (e.g. cholangitis, cholelithiasis, biliary
cirrhosis, spontaneous rupture, pancreatitis and malig-
nant change). The diagnosis is usually made by ultra-
sound where the cystic dilatation of the choledochus is
recognized with or without intrahepatic duct dilatation
(Figure 6). Dilatation of the choledochus may only be
mild and fluctuating and when associated with calculi
can cause diagnostic confusion with choledocholithiasis
with bile duct obstruction. If there is diagnostic un-
certainty or a Type V cyst present HIDA is useful to
confirm the biliary origin of the cyst and exclude other
diagnoses, e.g. simple liver cyst (Figure 7). MRCP
demonstrates the anomalous pancreatobiliary union
(Figure 8) and clarifies anatomy pre-operatively [26].
Imaging 2013, 22, 43631920
Imaging of the jaundiced child
99
Figure 3. A 24 h Tcm hepatic iminodiacetic acid (HIDA)
image in a jaundiced infant with biliary atresia shows no
excretion of tracer into the bowel. A small amount of tracer
is seen within the bladder owing to renal excretion of tracer.
MRCP has been shown to provide equivalent information
to ERCP [27] without the potential complications of
pancreatitis and cholangitis. Manganese-enhanced MR
cholangiography can also be used for differentiating pa-
renchymal lesions from cystic abnormalities communi-
cating with the bile ducts (Type V).
Figure 4. Operative cholangiogram of jaundiced infant with
biliary atresia demonstrates a cyst at the porta hepatis with
no communication with the biliary tract.
imaging.birjournals.org
Treatment is by radical cyst excision and
hepatojejunostomy.
Inspissated bile syndrome
This rare cause of jaundice in neonates is caused by
extrahepatic obstruction owing to sludge or inspissated
bile. Pre-disposing factors include prematurity, in-
fection, dehydration, total parenteral nutrition (TPN),
frusemide, gastrointestinal dysfunction, cystic fibrosis
(CF) and haemolysis. Ultrasound demonstrates di-
latation of the biliary tree with sludge in the bile duct
and gallbladder. Sludge is seen as low-level echoes
without acoustic shadowing (Figure 9). Further imaging
is not usually required.
Bile plug syndrome may reduce spontaneously or
following treatment with ursodeoxycholic acid, al-
though persistence or complications such as cholangitis
and liver abscess can be treated by PTC or cholecystog-
raphy with irrigation of the biliary tree with contrast and
saline [12].
Spontaneous perforation of the bile duct
This occurs at the junction of the cystic and common
hepatic duct with no obvious cause. Ultrasound shows
a complex mass around the bile duct and duodenum with
free intraperitoneal fluid. HIDA shows isotope within the
peritoneal cavity. Treatment is surgical.
Cholelithiasis
Gallstones in infancy are usually asymptomatic and
reduce spontaneously, although they may rarely impact
in the CBD. Incidental gallstones are increasingly being
recognised on antenatal ultrasound. They are mainly
pigment stones that form owing to immaturity of bile
acid production and secretion in association with delayed
oral feed, TPN, dehydration, prematurity and diuretics.
Persistent obstruction and signs of jaundice are indica-
tions for PTC and flushing, ERCP or surgery.
Jaundice in older children
Similar to neonates and infants, the causes of jaundice
in older children are wide and varied, and causes are
broadly classified as intrahepatic and extrahepatic
(Table 2). As with neonates and infants, ultrasound is the
first choice imaging modality to differentiate extrahepatic
from intrahepatic causes of jaundice in older children.
Intrahepatic causes
Intrahepatic causes of jaundice (Table 2) have non-
specific ultrasound findings and appearances depend on
the severity and stage of the disease rather than the
causative agent. In acute hepatitis the liver may be nor-
mal or enlarged with diffuse decrease in parenchymal
echogenicity and bright walled portal tracts (owing to
oedema in the hepatocytes). The gallbladder wall may be
diffusely thickened.
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 H E Woodley

Figure 5. Diagram of the common types of choledochal cyst.

(a) (b)

Figure 6. 3-month-old child presented with jaundice; (a) ultrasound demonstrates a large cyst extending from the porta hepatis
to the head of pancreas, which is confirmed on magnetic resonance cholangiopancreatography; (b) as a type 1 choledochal cyst.

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Imaging of the jaundiced child
(a)
(c)
In chronic hepatitis, ultrasound generally shows in-
creased echogenicity of liver parenchyma with a coarsened
echotexture and decreased visualization of the peripheral
portal venous tracts [28, 29]. The role of other imaging
modalities is limited and ultimately the determination of
the underlying diagnosis requires a multidisciplinary ap-
proach involving clinical assessment, chemistry, haema-
tology, radiology, histopathology and microbiology. Liver
biopsy may be necessary to confirm the diagnosis.
Extrahepatic causes
Cholelithiasis
Cholelithiasis is increasingly being recognised in
paediatric patients over the last two decades [30] al-
though duct stones are unusual [31]. Gallstones are
most common in adolescent girls and in this group are
imaging.birjournals.org
(b)
Figure 7. Images of a type V choledochal cyst in a
5-month-old girl. (a) Longitudinal ultrasound demon-
strates a cyst in the right lobe of the liver; (b) a 99Tcm
hepatic iminodiacetic acid (HIDA) image at 30 min
shows activity within the cyst; (c) operative cholangio-
gram confirms communication with the biliary tract.
associated with obesity, pregnancy or the oral contra-
ceptive pill. Other risk factors include haemolytic dis-
ease (e.g. spherocytosis, sickle cell disease, thalasaemia),
cystic fibrosis, Crohn9s disease or ileal resection, TPN,
drugs (e.g. frusemide), biliary tract obstruction (e.g.
choledochal cyst), sclerosing cholangitis and family
history.
Plain radiography will demonstrate radio-opaque
gallstones more often than in adults (3047%) owing to
the increased percentage of pigment stones [32] but di-
agnosis is made primarily by ultrasound. Gallstones are
seen as echogenic foci with distal acoustic shadowing,
which are mobile within the gallbladder lumen and
usually associated with ductal dilatation when lying
within the CBD. Spontaneous resolution of gallstones
may occur in children, particularly infants, but surgical
treatment is recommended in symptomatic children with
complicated gallstone disease.
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 H E Woodley

(a) (b)

Figure 8. MRI images of a type 1 choledochal cyst in an 8-month-old boy. (a) Magnetic resonance cholangiopancreatography
and (b) post-gadolinium T1 coronal image demonstrates a fusiform choledochal cyst communicating with a dilated pancreatic
duct via a common channel.

Consideration must be given to possible predisposing biliary tree. The disorder may be associated with a vari-
choledochal cysts when duct stones are present. In chil- ety of other diseases, e.g. chronic inflammatory bowel
dren the choledochus may only be mildly dilated and disease, Langerhan cell histiocytosis, immunodeficiency
may be confused with obstructive dilatation secondary disease and autoimmune hepatitis. Cases can be isolated
to a stone. It is important to recognise this entity pre- and neonatal idiopathic sclerosing cholangitis, a distinct
operatively as surgery consists of a cholecystectomy for entity, is also recognised.
simple stones but hepatojejunostomy for a choledochal Imaging aims to establish the diagnosis by demonstrat-
cyst (see choledochal cyst section). Follow-up ultrasound ing the typical findings of strictures in multiple segments of
or MRCP pre-operatively are indicated if there is suspi- the biliary tree with intervening dilated segments. The
cion of an underlying choledochal cyst in cholelithiasis disease may involve both the intrahepatic and extrahepatic
(Figure 10). ducts. Ultrasound may show irregular dilatation of the bile
ducts or thickening of the bile duct walls. MRCP can
demonstrate the typical finding with high specificity [33]
Sclerosing cholangitis (Figure 11), but in negative or inconclusive cases, invasive
Sclerosing cholangitis is a rare cause of chronic progres- imaging, e.g. ERCP or PTC, is required.
sive liver disease characterised by an inflammatory oblit-
erative fibrosis affecting the intrahepatic and extrahepatic
Table 2. Causes of jaundice in the child
Extrahepatic disorders Cholelithiasis
Choledochal cyst
Pancreatic masses
Inflammatory
Neoplastic
Liver trauma
Post-surgical
Benign strictures
Extrahepatic and Sclerosing cholangitis
intrahepatic
bile duct lesions
Intrahepatic disorders Infection
viral hepatitis
Drug-induced hepatitis
Metabolic
a1-antitrypsin deficiency
Cystic fibrosis
Wilsons disease
Glycogen storage disease
Toxic
Progressive intrahepatic
cholestasis
Figure 9. Ultrasound of a premature infant presenting with Alagilles syndrome
jaundice demonstrates sludge within a dilated common bile Autoimmune hepatitis
duct compatible with inspissated bile syndrome.

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Imaging of the jaundiced child
Figure 10. Magnetic resonance cholangiopancreatography
in a 14-year-old girl presenting with jaundice shows multiple
small gallstones and intraductal stones with dilatation of the
common bile duct.
Hepatic masses
Hepatic masses account for 56% of abdominal masses
in children and less than 2% of all paediatric malignancy
[34]. Neoplastic liver disease most commonly presents
with an abdominal mass but can occasionally present
with jaundice. Useful non-radiological findings contrib-
ute greatly in establishing a diagnosis, namely the age of
the child (Table 3), clinical presentation, laboratory
Figure 11. Coronal half-fourier acquisition single-shot
turbo-spin echo magnetic resonance cholangiopancreatog-
raphy image of a 13-year-old boy with abnormal liver
function tests and ulcerative colitis shows irregular di-
latation of the biliary tree and common bile duct compat-
ible with sclerosing cholangitis.
imaging.birjournals.org
findings (alpha-feto protein levels are raised in 8090% of
hepatoblastomas and 50% of hepatocellular carcinoma
(HCC), elevated gonadotrophins may also be seen in
hepatoblastoma and metastastatic choriocarcinoma) and
predisposing conditions (Table 4).
Initial evaluation of a hepatic mass by ultrasound im-
aging is directed towards characterising the lesion and
assessing the extent of the disease within the liver and the
presence of metastatic disease.
Hepatoblastoma, HCC and infantile haemangioendo-
thelioma (IHE) may be solitary or multiple while meta-
static disease is usually multiple. A cystic lesion is more
likely to be benign and the main diagnosis is cystic
mesenchymal hamartoma although neoplastic lesions
such as undifferentiated sarcomas can be necrotic but
have irregular margins, septae and nodules. Calcification
is often present in hepatoblastoma, undifferentiated sar-
coma and IHE and less commonly in HCC. A highly
vascular lesion with increased flow in the hepatic artery
and hepatic veins with tapering of the distal aorta is
typical of IHE. Vascular invasion or thrombosis usually
indicates a malignant process. Biliary duct dilatation is
suggestive of rhabdomyosarcoma of the biliary tree or
cholangiocarcinoma, although it can also be found with
lymphoma.
A diagnosis may be reached if typical ultrasound,
clinical and laboratory findings are found and further
imaging may not be required. If a mass cannot be fully
characterised then CT or MRI will be required and this
will provide further tissue characterisation and detailed
anatomical information. If a lesion is suspected to be
malignant then full staging CT of the chest, abdomen and
pelvis is required to exclude metastatic disease.
Imaging characteristics of hepatoblastoma and HCC
are similar on CT and MRI [35]. On CT pre-contrast the
lesion is low attenuation with early enhancement fol-
lowing contrast. HCC may have a central scar. These
lesions are hypointense relative to normal liver on T1
weighted images, although occasionally isointense or
hyperintense, and hyperintense on T2 weighted images.
Heterogeneity may be because of haemorrhage, necrosis
or focal fat within the lesions. Post-contrast images
demonstrate diffuse heterogenous enhancement with
early washout.
IHE show typical features on CT and MRI following
contrast with early peripheral nodular enhancement and
progressive centripetal enhancement on delayed scans
Table 3. Differential diagnosis of liver neoplasms by age
<3 years >3 years
Hepatoblastoma HCC
Metastases Undifferentiated
(neuroblastoma/Wilms) embryonal sarcoma
Infantile Metastases or lymphoma
haemangioendothioma
(85% occur in infants
,6months)
Cystic mesenchymal FNH
hamartoma
Adenoma
Fibrolamellar HCC
HCC, hepatocellular carcinoma; FNH, focal nodular
hyperplasia.
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 H E Woodley

Table 4. Syndromes and diseases predisposing liver tumours Pancreatic lesions

Hepatoblastoma Beckwith Wiedeman
Hemihypertrophy
Foetal alcohol syndrome
Familial polyposis
Gardners syndrome
Very low birth weight
HCC Viral hepatitis B & C
Glycogen storage disease
type 1 or 3
Tyrosinaemia
Alpha1-antitrypsin deficiency
Haemochromatosis
Spontaneous or surgical
portocaval shunts
HCC, hepatocellular carcinoma.
(Figure 12). These findings will prevent biopsy being
performed in these benign lesions, which have a natu-
ral history of involution by the age of 1218 months
and can be followed up by ultrasound.
Biopsy is necessary when the diagnosis remains prob-
lematic or tissue is required prior to commencing
chemotherapy.
Lesions in the pancreatic head are a rare cause of
jaundice in the child and can be due to inflammatory,
immunological, traumatic and neoplastic disorders. Im-
aging aims to distinguish benign from malignant lesions.
Inflammatory pancreatic masses
Obstructive jaundice is an uncommon complication of
pancreatitis in children although autoimmune pancreati-
tis or idiopathic fibrosing pancreatitis may present with
jaundice [36]. This entity is now recognised as an im-
portant differential for a pancreatic mass in childhood as
conservative treatment is widely advocated [37, 38]. The
natural history is usually of progression from pancreatic
enlargement with compression of the CBD to atrophy. It
is often a diffuse process but may present with focal
changes in the head of the pancreas.
Ultrasound demonstrates a diffusely or focally swollen
and echopoor pancreas with narrowing of both the distal
CBD and pancreatic duct. CT findings include diffuse or
focal enlargement, hypoattenuation of the involved pan-
creas on early post-contrast phase and a capsule-like rim

(a) (b) (c)

(d) (e)

Figure 12. Imaging of a 7-month-old girl with multiple haemangioendothelioma. (a) Ultrasound shows multiple hypoechoic lesions.
(b) MRI shows typical imaging characteristics with high signal on T2 weighted sequences (c) and early peripheral nodular enhancement
on post-gadolinium T1 weighted sequences with gradual centripetal in filling on (d) portal venous and (e) delayed images.

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Imaging of the jaundiced child

(a) (b)

Figure 13. (a) Magnetic resonance cholangiopancreatography image in a 12-year-old boy presenting with jaundice
demonstrates narrowing of the intrapancreatic common bile duct (CBD) and dilatation of the proximal CBD. (b) Delayed post-
gadolinium T1 coronal oblique image demonstrates peripheral enhancement of the pancreas in keeping with autoimmune
pancreatitis.

that shows enhancement on delayed post-contrast radiological appearances are of a focal lesion in the head
images. MRI shows diffuse or focal pancreatic enlarge- of pancreas percutaneous, ultrasound guided biopsy
ment with low signal on T1 weighted images and a cap- should be performed. Follow-up imaging may demon-
sule of low signal on T2 weighted images which shows strate pancreatic atrophy with consequent exocrine and
delayed enhancement on delayed post-gadolinium T1 occasionally endocrine insufficiency.
images. MRCP demonstrates narrowing of the pancreatic
duct and bile duct in the intrapancreatic region with di- Pancreatic tumours
latation of the proximal biliary tracts (Figure 13). ERCP
demonstrates smooth stenosis of the distal CBD. Ob- Pancreatic tumours are rare in children but may
struction may resolve spontaneously although temporary present with obstructive jaundice. Pancreatic tumours
biliary stenting may be required and is now advocated in in children have a different histological spectrum and
preference to more invasive surgical biliary diversion prognosis than in adults [39] and can be divided into
[38]. If there is diagnostic doubt, particularly if the exocrine tumours, endocrine tumours, non-epithelial or
mesenchymal tumours, such as haemangioma, neuro-
fibroma or lymphangioma [4]. The pancreas can also be
involved owing to metastatic diseaselymphoma has
the highest incidence in children [40]or rarely direct
Table 5. Pancreatic masses in children
invasion e.g. neuroblastoma (Table 5). Pancreatic
Epithelial i) Exocrine Ductal Cell ductal carcinoma masses identified on ultrasound require further evalu-
(very rare) ation by CT or MRI to characterise the lesion [4], assess
Acinar Cell pancreatoblastoma the extent of disease and look for other sites of disease
acinar cell carcinoma (Figure 14).
Uncertain solid Pancreatoblastoma is the most common pancreatic
pseudopapillary tumour
neoplasm in young children and may be associated with
ii) Endocrine Functioning Insulinoma
Gastrinoma a raised alpha fetoprotein in up to a third of cases. There
VIPoma is a known association with Beckwith-Wiedemann syn-
Glucagonoma drome. On imaging the tumour is heterogeneous with
Non-functioning central cystic change and is often multiloculated. Calci-
Non-epithelial Lymphoma fications may be seen on CT and there is mild contrast
Sarcoma enhancement. MRI typically demonstrates high signal on
Mesenchymal Lymphangioma T2 weighted sequences and low to intermediate signal on
Haemangiothelioma T1 sequences. Metastatic disease is rare but when it is
Dermoid cyst present prognosis is poor.
Metastatic Lymphoma
Solid pseudopapillary tumour usually presents in
Neuroblastoma
adolescent or young women but is the most common

imaging.birjournals.org 11 of 14
 H E Woodley

(a) (b)

(c) (d)

Figure 14. 9-year-old boy presented with painless jaundice. (a) Magnetic resonance cholangiopancreatography demonstrates
intrahepatic and pancreatic duct dilatation. Axial T2 weighted image; (b) post-gadolinium T1 axial; (c) coronal oblique; (d) images
show a complex cystic and solid mass in the head of pancreas causing intrahepatic and pancreatic duct dilatation. Biopsy
diagnosed an angiosarcoma.

paediatric pancreatic tumour in the Asian population [4].
This tumour is often large at presentation with a prom-
inent fibrous capsule and solid and cystic haemorrhagic
internal component. Ultrasound and CT demonstrate
a well defined large mass with variable cystic compo-
nents, while MRI may demonstrate a T1 low signal rim
representing the fibrous capsule and central high signal
areas consistent with haemorrhagic debris.
Trauma and post-surgical
Jaundice following trauma [42] and surgery will have
an appropriate history and therefore is not discussed in
detail in this article. The causes of jaundice post-
transplantation include rejection, infection, drug toxicity
and biliary complications including anastomotic stric-
tures (Figure 15) or non-anastomotic strictures related to

12 of 14 Imaging 2013, 22, 43631920

Imaging of the jaundiced child
Figure 15. 11-year-old boy presented with jaundice 3
months post-liver transplant. Magnetic resonance cholangio-
pancreatography imaging demonstrates a tight stenosis at
the bile duct anastomosis.
hepatic artery thrombosis, prolonged cold ischaemia time
and ABO incompatibility. Most biliary strictrures occur
within a year of transplantation [43].
Cirrhosis
Cirrhosis is rare in neonates but may occur in children
and occasionally presents with jaundice. The most com-
mon causes of cirrhosis in children are chronic hepatitis,
Figure 16. Algorithm for the investigation of neonatal
jaundice. HIDA, 99Tcm hepatic iminodiacetic acid; MRCP,
magnetic resonance cholangiopancreatography.
imaging.birjournals.org
congenital hepatic fibrosis, biliary atresia, cystic fibrosis,
metabolic disease (Wilsons disease, glycogen storage
disease, tyrosinaemia, galactosaemia, alpha 1-antitrypsin
deficiency), Budd-Chiari syndrome and TPN. Primary
liver disease is often not appreciated on imaging owing to
the superimposition of cirrhosis. On ultrasound the liver
is coarse and nodular often with a shrunken right hepatic
lobe and medial segment of the left lobe and compensa-
tory hypertrophy of the caudate and left lateral lobe.
There are secondary signs of cirrhosis such as ascites and
portal hypertension with splenomegaly and the forma-
tion of varices. Colour Doppler will assess direction of
flow in the portal vein and the presence of varices or
collateral venous channels.
Conclusion
This article describes the imaging findings of the major
causes of jaundice in the neonate and child. The con-
trasting aetiologies in neonates and infants compared
with older children necessitate a different imaging strat-
egy (Figure 16). Ultrasound remains the initial imaging
modality in both these age groups and is used to de-
termine the next investigation.
Ultrasound should be performed promptly in the ne-
onate to exclude biliary atresia and in experienced hands
biliary atresia may be diagnosed by ultrasound alone,
although a combination of tests is often still required.
In the older child gallstones are increasingly being
recognised on ultrasound. Advances in MRI techniques
enable the biliary tract to be imaged in great detail so that
more invasive techniques such as ERCP can be avoided in
many cases. Pancreatic masses are a rare cause of jaun-
dice but careful evaluation by CT or MRI can distinguish
inflammatory causes from mass lesions to enable appro-
priate management.
The causes of jaundice in children remain manifold and
often the underlying diagnosis is made by a combination
of clinical findings, laboratory tests, imaging and histol-
ogy. With close cooperation with clinicians the appro-
priate radiological investigations are performed and
unnecessary tests avoided, thereby expediting the di-
agnosis of the underlying condition.
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