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
cysts should be sought when bile duct stones are found.
Hepatic and pancreatic masses are a rare but important differential in the
investigation of jaundice and require evaluation by CT or MRI.

doi: 10.1259/imaging/
43631920
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

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 physiological 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

imaging.birjournals.org

Imaging techniques
Ultrasound
Ultrasound examination remains the imaging investigation 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 nonobstructive 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 pulmonary 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 vasculature 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
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H E Woodley

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 patency and function of the biliary tree. 99Tcm iminodiacetic 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 hepatobiliary 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 postliver 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 infusion 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 investigation 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 image quality and adaptations for children include coil selection, 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 halffourier 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, sclerosing cholangitis, pancreaticobiliary malunion and more
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recently has been advocated in the investigation of neonatal 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. Problematic 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 modalities 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 postoperative 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 enzyme glucuronosyl transferase. However, infants who
are jaundiced beyond the first 2 weeks of life require investigation 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 causes of jaundice from extrahepatic causes, in particular
excluding biliary atresia, and ultrasound will be the first
imaging modality of choice. Intrahepatic causes of jaundice 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 extrahepatic 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
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Imaging of the jaundiced child

Table 1. Causes of neonatal jaundice
Extrahepatic

Intrahepatic disorders

Biliary atresia
Choledochal cyst
Bile plug syndrome
Cholelithiasis
Spontaneous perforation of
CHD
Duodenal duplication
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
a1-antitrypsin
Cystic fibrosis
Galactosaemia
Tyrosinaemia
Endocrine
Hypothyroidism
Panhypopituitanism

CHD, common hepatic duct; CMV, cytomegalovirus; TPN,

total parental nutrition.

Type 3 the gallbladder is small or absent and contains clear mucus only. Occasionally a cyst is found
proximal or distal to the atretic bile duct at the porta
hepatis. Syndromic biliary atresia (biliary atresia
splenic malformation (BASM)) occurs in 1020% of
cases and consists of polysplenia (or rarely, asplenia),
interrupted inferior vena cava (IVC), pre-duodenal
portal vein, intestinal malrotation, situs inversus and
cardiac defects.
Early diagnosis of biliary atresia (,8 weeks old) and
prompt surgery improves outcome. Historically a combination of tests is usually required to diagnose biliary
atresia with certainty, although in experienced hands it
can be diagnosed by ultrasound alone [13]. At ultrasound
the echogenicity of the liver parenchyma is not usually
helpful but may be increased, the intrahepatic bile ducts
are not dilated and non-recognition of the CBD is not
diagnostic. A number of more specific ultrasound features have been described [14]. These include: (i) the triangular cord sign that represents the fibrotic remnant of
the hepatic duct junction and is seen as an echogenic
triangular or tubular structure just cranial to the bifurcation of the portal vein at the portal hepatis
(Figure 2a) [1517], (ii) a small or absent gallbladder with
irregular walls [18, 19] (Figure 2b) although a small gallbladder may also be seen in neonatal hepatitis, (iii) lack of
gall bladder contractibilty [21] and (iv) increased hepatic
artery dimension [21].
Occasionally a cyst is present at the porta hepatis
(Figure 2c) and there may be evidence of BASM.
imaging.birjournals.org

Figure 1. Classification of the main types of biliary atresia.

If ultrasound features suggestive of biliary atresia are

present or the diagnosis cannot be excluded, a HIDA scan
should be performed. In biliary atresia there is nonexcretion of bile into the bowel by 24 h (Figure 3). However, hepatobiliary scanning is not diagnostic as nonexcretion may indicate severe neonatal hepatitis or the
presence of interlobular bile duct paucity [22].
Following ultrasound and HIDA scan, if the diagnosis
remains in doubt but biliary atresia remains a diagnostic
possibility, percutaneous liver biopsy may be considered.
MRCP has been advocated to demonstrate the biliary
structures and diagnose biliary atresia [7, 23] and to
demonstrate the triangular cord sign [24] but this technique requires further evaluation. ERCP has been used in
the diagnosis of biliary atresia but is technically difficult
and rarely performed [11]. Percutaneous cholecystocholangiography under ultrasound guidance can also be
used to assess the biliary tree when a gallbladder is large
enough to perform this procedure safely [25].
When imaging or biopsy histology is compatible with
biliary atresia a Kasai portoenterostomy is performed.
This is usually preceeded by an operative cholangiogram
to confirm the diagnosis (Figure 4). Resolution of jaundice
after surgery is more likely if surgery is performed before
8 weeks of age when larger ductules are present at the
porta hepatis and in non-syndromic cases of biliary
atresia.

Neonatal hepatitis
Neonatal hepatitis, for which there are many underlying diagnoses (Table 1), is the main differential to
biliary atresia. Often there are no specific ultrasound
features, although the liver may be enlarged with increased echogenicity. Usually the biliary system is unremarkable with a normal sized gallbladder, although in
severe hepatocellular dysfunction the gallbladder may be
decreased in size owing to decreased volume of bile.
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H E Woodley

(a)

(b)

(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 biliary 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 prenatally, but most commonly present with jaundice with
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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 malignant change). The diagnosis is usually made by ultrasound 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 uncertainty 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].
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Imaging of the jaundiced child

Treatment is by
hepatojejunostomy.

radical

cyst

excision

and

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, infection, dehydration, total parenteral nutrition (TPN),
frusemide, gastrointestinal dysfunction, cystic fibrosis
(CF) and haemolysis. Ultrasound demonstrates dilatation 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, although persistence or complications such as cholangitis
and liver abscess can be treated by PTC or cholecystography with irrigation of the biliary tree with contrast and
saline [12].

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.

Figure 3. A 24 h

99

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 parenchymal lesions from cystic abnormalities communicating with the bile ducts (Type V).

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 indications 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

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

Intrahepatic causes of jaundice (Table 2) have nonspecific 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 normal 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)

(b)

Figure 7. Images of a type V choledochal cyst in a

5-month-old girl. (a) Longitudinal ultrasound demonstrates 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 cholangiogram confirms communication with the biliary tract.

(c)

In chronic hepatitis, ultrasound generally shows increased 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 approach involving clinical assessment, chemistry, haematology, 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] although duct stones are unusual [31]. Gallstones are
most common in adolescent girls and in this group are
imaging.birjournals.org

associated with obesity, pregnancy or the oral contraceptive pill. Other risk factors include haemolytic disease (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 diagnosis 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

(b)

(a)

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

choledochal cysts when duct stones are present. In children the choledochus may only be mildly dilated and
may be confused with obstructive dilatation secondary
to a stone. It is important to recognise this entity preoperatively as surgery consists of a cholecystectomy for
simple stones but hepatojejunostomy for a choledochal
cyst (see choledochal cyst section). Follow-up ultrasound
or MRCP pre-operatively are indicated if there is suspicion of an underlying choledochal cyst in cholelithiasis
(Figure 10).

Sclerosing cholangitis
Sclerosing cholangitis is a rare cause of chronic progressive liver disease characterised by an inflammatory obliterative fibrosis affecting the intrahepatic and extrahepatic

biliary tree. The disorder may be associated with a variety of other diseases, e.g. chronic inflammatory bowel
disease, Langerhan cell histiocytosis, immunodeficiency
disease and autoimmune hepatitis. Cases can be isolated
and neonatal idiopathic sclerosing cholangitis, a distinct
entity, is also recognised.
Imaging aims to establish the diagnosis by demonstrating the typical findings of strictures in multiple segments of
the biliary tree with intervening dilated segments. The
disease may involve both the intrahepatic and extrahepatic
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]
(Figure 11), but in negative or inconclusive cases, invasive
imaging, e.g. ERCP or PTC, is required.
Table 2. Causes of jaundice in the child
Extrahepatic disorders

Extrahepatic and
intrahepatic
bile duct lesions
Intrahepatic disorders

Figure 9. Ultrasound of a premature infant presenting with

jaundice demonstrates sludge within a dilated common bile
duct compatible with inspissated bile syndrome.

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Cholelithiasis
Choledochal cyst
Pancreatic masses
Inflammatory
Neoplastic
Liver trauma
Post-surgical
Benign strictures
Sclerosing cholangitis

Infection
viral hepatitis
Drug-induced hepatitis
Metabolic
a1-antitrypsin deficiency
Cystic fibrosis
Wilsons disease
Glycogen storage disease
Toxic
Progressive intrahepatic
cholestasis
Alagilles syndrome
Autoimmune hepatitis

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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 contribute greatly in establishing a diagnosis, namely the age of
the child (Table 3), clinical presentation, laboratory

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 imaging 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 haemangioendothelioma (IHE) may be solitary or multiple while metastatic 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 sarcoma 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 following 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

Figure 11. Coronal half-fourier acquisition single-shot

turbo-spin echo magnetic resonance cholangiopancreatography image of a 13-year-old boy with abnormal liver
function tests and ulcerative colitis shows irregular dilatation of the biliary tree and common bile duct compatible with sclerosing cholangitis.
imaging.birjournals.org

<3 years

>3 years

Hepatoblastoma
Metastases
(neuroblastoma/Wilms)
Infantile
haemangioendothioma
(85% occur in infants
,6months)
Cystic mesenchymal
hamartoma

HCC
Undifferentiated
embryonal sarcoma
Metastases or lymphoma

FNH
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

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. Imaging aims to distinguish benign from malignant lesions.

Beckwith Wiedeman
Hemihypertrophy
Foetal alcohol syndrome
Familial polyposis
Gardners syndrome
Very low birth weight
Viral hepatitis B & C
Glycogen storage disease
type 1 or 3
Tyrosinaemia
Alpha1-antitrypsin deficiency
Haemochromatosis
Spontaneous or surgical
portocaval shunts

HCC

HCC, hepatocellular carcinoma.

(Figure 12). These findings will prevent biopsy being

performed in these benign lesions, which have a natural history of involution by the age of 1218 months
and can be followed up by ultrasound.
Biopsy is necessary when the diagnosis remains problematic or tissue is required prior to commencing
chemotherapy.

Inflammatory pancreatic masses

Obstructive jaundice is an uncommon complication of
pancreatitis in children although autoimmune pancreatitis or idiopathic fibrosing pancreatitis may present with
jaundice [36]. This entity is now recognised as an important 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 pancreas on early post-contrast phase and a capsule-like rim

(a)

(b)

(d)

(e)

(c)

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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(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 postgadolinium T1 coronal oblique image demonstrates peripheral enhancement of the pancreas in keeping with autoimmune
pancreatitis.

that shows enhancement on delayed post-contrast

images. MRI shows diffuse or focal pancreatic enlargement with low signal on T1 weighted images and a capsule of low signal on T2 weighted images which shows
delayed enhancement on delayed post-gadolinium T1
images. MRCP demonstrates narrowing of the pancreatic
duct and bile duct in the intrapancreatic region with dilatation of the proximal biliary tracts (Figure 13). ERCP
demonstrates smooth stenosis of the distal CBD. Obstruction may resolve spontaneously although temporary
biliary stenting may be required and is now advocated in
preference to more invasive surgical biliary diversion
[38]. If there is diagnostic doubt, particularly if the

Table 5. Pancreatic masses in children

Ductal Cell ductal carcinoma
(very rare)
Acinar Cell pancreatoblastoma
acinar cell carcinoma
Uncertain solid
pseudopapillary tumour
ii) Endocrine Functioning Insulinoma
Gastrinoma
VIPoma
Glucagonoma
Non-functioning
Non-epithelial
Lymphoma
Sarcoma
Mesenchymal
Lymphangioma
Haemangiothelioma
Dermoid cyst
Metastatic
Lymphoma
Neuroblastoma
Epithelial i) Exocrine

imaging.birjournals.org

radiological appearances are of a focal lesion in the head

of pancreas percutaneous, ultrasound guided biopsy
should be performed. Follow-up imaging may demonstrate pancreatic atrophy with consequent exocrine and
occasionally endocrine insufficiency.

Pancreatic tumours
Pancreatic tumours are rare in children but may
present with obstructive jaundice. Pancreatic tumours
in children have a different histological spectrum and
prognosis than in adults [39] and can be divided into
exocrine tumours, endocrine tumours, non-epithelial or
mesenchymal tumours, such as haemangioma, neurofibroma or lymphangioma [4]. The pancreas can also be
involved owing to metastatic diseaselymphoma has
the highest incidence in children [40]or rarely direct
invasion e.g. neuroblastoma (Table 5). Pancreatic
masses identified on ultrasound require further evaluation by CT or MRI to characterise the lesion [4], assess
the extent of disease and look for other sites of disease
(Figure 14).
Pancreatoblastoma is the most common pancreatic
neoplasm in young children and may be associated with
a raised alpha fetoprotein in up to a third of cases. There
is a known association with Beckwith-Wiedemann syndrome. On imaging the tumour is heterogeneous with
central cystic change and is often multiloculated. Calcifications may be seen on CT and there is mild contrast
enhancement. MRI typically demonstrates high signal on
T2 weighted sequences and low to intermediate signal on
T1 sequences. Metastatic disease is rare but when it is
present prognosis is poor.
Solid pseudopapillary tumour usually presents in
adolescent or young women but is the most common
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 prominent fibrous capsule and solid and cystic haemorrhagic
internal component. Ultrasound and CT demonstrate
a well defined large mass with variable cystic components, while MRI may demonstrate a T1 low signal rim
representing the fibrous capsule and central high signal
areas consistent with haemorrhagic debris.
12 of 14

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 posttransplantation include rejection, infection, drug toxicity
and biliary complications including anastomotic strictures (Figure 15) or non-anastomotic strictures related to
Imaging 2013, 22, 43631920

Imaging of the jaundiced child

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 compensatory hypertrophy of the caudate and left lateral lobe.
There are secondary signs of cirrhosis such as ascites and
portal hypertension with splenomegaly and the formation of varices. Colour Doppler will assess direction of
flow in the portal vein and the presence of varices or
collateral venous channels.

Conclusion

Figure 15. 11-year-old boy presented with jaundice 3

months post-liver transplant. Magnetic resonance cholangiopancreatography 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 common causes of cirrhosis in children are chronic hepatitis,

This article describes the imaging findings of the major

causes of jaundice in the neonate and child. The contrasting aetiologies in neonates and infants compared
with older children necessitate a different imaging strategy (Figure 16). Ultrasound remains the initial imaging
modality in both these age groups and is used to determine the next investigation.
Ultrasound should be performed promptly in the neonate 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 jaundice but careful evaluation by CT or MRI can distinguish
inflammatory causes from mass lesions to enable appropriate 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 histology. With close cooperation with clinicians the appropriate radiological investigations are performed and
unnecessary tests avoided, thereby expediting the diagnosis of the underlying condition.

References

Figure 16. Algorithm for the investigation of neonatal

jaundice. HIDA, 99Tcm hepatic iminodiacetic acid; MRCP,

magnetic resonance cholangiopancreatography.

imaging.birjournals.org

1. Gubbernick JA, Rosenberg HK, Ilaslan H, Kessler A. US

approach to jaundice in infants and children. Radiographics
2000;20:17395.
2. Ranson M, Hiew C, Babyn PS. Pediatric biliary imaging In:
Stringer DA and Babyn PS (eds). Pediatric gastrointestinal
imaging and intervention. Hamilton: BC Decker Incorporated, 2000, pp 5523.
3. McGahan JP, Phillips HE, Cox KL. Sonography of the normal pediatric gallbladder and biliary tract. Radiology 1982;
14:8735.
4. Nijs E, Callahan MJ, Taylor GA. Disorders of the pediatric
pancreas: imaging features. Pediat Radiol 2005;35:35878.
5. Rosenberg HK, Markowitz RI, Kolberg H, Park C, Hubbard
AM, Bellah RD. Normal splenic size in infants and children:
sonographic measurements. AJR Am J Roentgenol 1991;157:
11921.
6. Majid M, Reba RC, Altman RP. Effect of phenobarbitone in
99mTc-IDA scintigraphy in the evaluation of neonatal
jaundice. Semin Nucl Med 1981;11:194204.

13 of 14

H E Woodley
7. Pallan A, Williams H. Magnetic resonance cholangiopancreatography in children. RAD Magazine 2004;30:212.
8. Guiband L, Lachaud A, Touraine R, Guibal AL, Pelizzari M,
Basset T, et al. MR cholangiography in neonates and infants:
feasibility and preliminary applications. AJR Am J Roentgenol
1998;170:2731.
9. Chavhan GB, Babyn PS, Manson D, Vidarsson L. Pediatric
MR cholangiopancreatography: principle, technique, and
clinical applications. Radiographics 2008;28:195162.
10. Jaw T, Kuo Y, Liu G, Chen S, Wang C. MR cholangiography
in the evaluation of neonatal cholestasis. Radiology 1999;
212:24956.
11. Aabakken L, Aagenaes I, Sanengen T, Aasen S, Bjornland K.
Utility of ERCP in neonatal and infant cholestasis. J Laparoendosc Adv Surg Tech A 2009 Jun;19(3): 4316.
12. Pariente D, Bernard O, Gauthier F, Brunelle F, Chaumont P.
Radiological treatment of common bile duct lithiasis in infancy. Pediat Radiol 1989;19:1047.
13. Takamizawa S, Zaima A, Muraji T, Kanegawa Y, Akasaka
Y, Satoh S, et al. Can biliary atresia be diagnosed by ultrasonography alone? J Pediatr Surg 2007;42:20936.
14. Humphrey TH, Stringer MS. Biliary atresis: US diagnosis.
Radiology 2007;244:84551.
15. Choi SI, Park WH, Lee HJ, Woo SK. Triangular cord:
a sonographic finding applicable in the diagnosis of biliary
atresia. J Pediatric Surg 1996;31:3636.
16. Koth MA, Koth A, Sheba MF, El Koofy NM, El-Karasksy HM,
Abdel-Kahlik MK, et al. Evaluation of the triangular cord sign
in the diagnosis biliary atresia. Pediatrics 2001;108:41620.
17. Lee HJ, Le SM, Park WH, Choi SO. Objective criteria of
triangular cord sign in biliary atresia on US scans. Radiology
2003;229:395400.
18. Farrant P, Meire HB, Mieli-Vergani G. Improved diagnosis
of extrahepatic biliary atresia by high frequency ultrasound
of the gallbladder. Br J Radiol 2001;74:9524.
19. Tan Kendrick AP, Phua KB, Ooi BC, Tan CE. Biliary atrsia:
making the diagnosis by the gallghost triad. Pediatr Radiol
2003;33:3115.
20. Ikeda S, Sera Y, Akagi M. Serial ultrasonic examination to
differentiate biliary atresia from neonatal hepatitis special
reference to changes in size of the gallbladder. Eur J Pediatr
1989;148:396400.
21. Kim WS, Cheon JE, Youn BJ, Yoo SY, Kim WY, Yeon KM,
et al. Hepatic arterial diameter measured with US: adjunct
for US diagnosis of biliary atresia. Radiology 2007;245:
54955.
22. Gilmour SM, Hershkop M, Reifen R, Gilday D, Roberts EA.
Outcome of hepatobiliary scanning in neonatal hepatitis
syndrome. J Nucl Med 1997;38:127982.
23. Ryeom HK, Choe BH, Kim JY, Kwon S, Ko CW, Kim HM,
et al. Biliary atresia: feasibility of mangafodipir trisodiumenhanced MR cholangiography for evaluation. Radiology
2005;235:2508.
24. Kim MJ, Park YN, Han SJ, Yoon HS, Hwang EH, et al. Biliary atresia in neonates and infants: triangular area of high
signal intensity in the porta hepatis at T2 weighted MR
cholangiography with US and histopathologic correlation.
Radiology 200;215:395401.

14 of 14

25. Nwomeh BC, Caniano DA, Hogan M. Definitive exclusion

of biliary atresia in infants with cholestatic jaundice: the role
of percutaneous cholecysto-cholangiography. Pediatr Surg
Int 2007;23:8459.
26. Kim MJ, Han SJ, Yoon CS, Kim JH, Oh JT, Chung KS, et al.
Using MR cholangiography to reveal anomaolous pancreaticobiliary ductal union in infants and children
with choledochal cysts. AJR Am J Roentgenol 2002;179:
20914.
27. Matos C, Nicaise N, Deriere J, Cassart M, Metens T, Struyren
J, et al. Choledochal cysts: comparison of findings at MR
cholangiopancreatography and endoscopic retrograde
cholangiopancreatography in eight patients. Radiology
1998;209:4438.
28. Kurtz AB, Rubin CS, Cooper HS. Ultrasound findings in
hepatitis. Radiology 1980;136:71723.
29. Cushing B, Slovis TL. Sonography of the liver and biliary
system in pediatric patients. Appl Radiol 1985;14:718.
30. Holcomb GW, Holcomb GW. Cholelithiasis in infants, children and adolescents. Pediatr Rev 1990;11:26874.
31. Rescoria FJ. Cholelithiasis, cholecystitis, and common bile
duct stones. Curr Opin Pediatr 1997;9:27682.
32. Roslyn JJ, Berquist WE, Pitt HA, Man LL, Kangarloo H,
Den Besten L, et al. Increased risk of gallstones in children
receiving total parenteral nutrition. Pediatrics 1983;71:
7849.
33. Ferrara C, Valeri G, Salvolini L, Giovagnoni A. Magnetic
resonance cholangiopancreatography in primary sclerosing
cholangitis in children. Pediat Radiol 2002;32:4137.
34. Helmberger TK, Ros PR, Mergo PJ, Tomczak KR, Reisner
MF. Pediatric liver neoplasms: a radiologic-pathologic correlation. Eur Radiol 1999;9:133947.
35. Siegel MJ, Luker GD. MR imaging of the liver in children.
MRI Clin North Am 1996;4:63756.
36. Yoshi da K, Toki F, Takeuchi T, Watanabe S, Shiratori K,
Hayashi N. Chronic pancreatitis caused by an autoimmune
abnormality: proposal of the concept of autoimmune pancreatitis. Dig Dis Sci 1995;40:156168.
37. El-Matary W, Casson D, Hodges S, Davison S, McClean P,
Elbadri A, et al. Successful conservative treatment of idiopathic fibrosing pancreatitis in children. Eur J Pediatr 2006;
165:5605.
38. Cartmell MT, Cusick E, Ashworth M, Duncan A, Huskisson
LJ. Idiopathic fibrosing pancreatitis and spontaneous resolution of pancreatic masses in children. Pediatr Surg Int
2007;23:897901.
39. Chung EM, Travis MD, Conran RM. Pancreatic tumors in
children: radiologic-pathologic correlation. Radiographics
2006;26:121138.
40. Ravindra KV, Stringer MD, Prasad KR, Kinsey SE, Lodge
JPA. Non-Hodgkins lymphoma presenting with obstructive
jaundice. Br J Radiol 2003;90:8459.
41. Yoon W, Jeong YY, Kim JK, Seo JJ, Lim HS, Shin SS, et al. CT
in blunt liver trauma. Radiographics 2005;25:87104.
42. Stringer M. Liver transplantation: complications and their
management. In: Howard ER, Stringer MD, Colombani PM
(eds). Surgery of the liver, bile ducts and pancreas in children. London: Arnold, 2002, pp 44164.

Imaging 2013, 22, 43631920