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ULTRASOUND
CLINICS
Ultrasound Clin 1 (2006) 431441

Jaundice in Infants and Children

Marilyn J. Siegel,
-

MD

Sonographic examination
Overview of cholestatic diseases
Neonatal jaundice
Biliary atresia
Neonatal hepatitis syndrome
Additional imaging studies to
differentiate atresia and hepatitis
Alagille syndrome
Choledochal cyst

Real-time sonography remains the screening

study of choice for the evaluation of jaundice in
infants and children and it is an important tool in
differentiating between obstructive and nonobstructive causes of jaundice [1,2]. The causes of
cholestasis are multiple, but the three major causes
are hepatitis, biliary atresia, and choledochal cyst.
Other causes include neoplastic processes, cirrhosis,
and strictures.
This article reviews the common congenital and
acquired causes of jaundice in the pediatric patient
and describes the sonographic findings associated
with these conditions. The role of correlative imaging studies is also reviewed.

Sonographic examination
The sonographic examination of infants and children who have jaundice includes a detailed examination of the liver, bile ducts, gallbladder, and
pancreas. Hepatic size and echotexture should be
thoroughly assessed. The right hepatic lobe should
extend to or just below the right costal margin
in a patient without hyperinflated lungs. The

Spontaneous perforation of the

extrahepatic bile ducts
Inspissated bile syndrome
Jaundice in older infants and children
Caroli disease
Byler disease
Hepatocellular diseases
Inflammatory diseases of the biliary ducts
Biliary tract obstruction
References

echogenicity of the normal liver is low to medium

and homogeneous, and the central portal venous
vasculature is easily seen (Fig. 1). In the neonate
and young infant, the hepatic parenchyma and renal cortex are equally echogenic. In individuals 6
months of age and older, the liver usually is more
echogenic than the kidney. The patency and flow
direction of the hepatic vessels should be documented with pulsed and color Doppler interrogation. The liver and adjacent area should also be
evaluated for evidence of end-stage liver disease, including collateral channels (varices), hepatofugal
flow, and ascites.
The diameter of the common duct should be
measured on the sagittal scan to confirm the presence or absence of ductal dilatation. The upper
limits of the common duct should not exceed 1
mm in neonates, 2 mm in infants up to 1 year of
age, 4 mm in children 1 to 10 years of age, and 6
mm in adolescents and young adults [3]. The distal
portion of the common duct is typically larger than
the proximal portion. Ductal size may increase by
1 mm or more during deep inspiration and the
Valsalva maneuver [4]. The cystic duct in children

Radiology and Pediatrics, Mallinckrodt Institute of Radiology, Washington University School of Medicine,
510 South Kingshighway Boulevard, St. Louis, MO, 63110, USA
E-mail address: siegelm@mir.wustl.edu
1556-858X/06/$ see front matter 2006 Elsevier Inc. All rights reserved.

ultrasound.theclinics.com

doi:10.1016/j.cult.2006.05.010

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Siegel

Fig. 1. Normal liver. Transverse sonogram shows homogeneous hepatic parenchyma. The echogenic portal venous vasculature (arrows) is easily seen. The
gallbladder (GB) is distended and the wall is thin
and hyperechoic. PV, portal vein.

is not routinely seen unless it is dilated, and then

usually only the distal part of the duct near its insertion into the common bile duct is seen.
Gallbladder size usually can be assessed subjectively, but measurements may be helpful in equivocal cases. The normal gallbladder length is 1.5 to
3.0 cm in neonates and young infants (younger than
1 year old) and the width is approximately 1 cm. In
older children and adolescents, gallbladder length is
3 to 8 cm and width is less than 3.5 cm. The wall of
the gallbladder should be thin, hyperechoic, and
well defined. The upper limits of wall thickness in
the fasting state are 3 mm [5].
Feeding of a fatty meal may be helpful in patients
who have enlarged gallbladders to assess cystic duct
patency. In healthy individuals, maximum emptying of the gallbladder occurs between 45 and 60
minutes after the fatty meal, and the mean volume
decreases approximately 60%. Contraction of the
gallbladder after a fatty meal supports the diagnosis
of a patent cystic duct.
Pancreatic size, echotexture, and ductal size should
be evaluated. Pancreatic size increases with increasing age of the child [6]. The mean cross-sectional diameter of the pancreatic head ranges between 1 and
2 cm, the body between 0.6 and 1.1 cm, and the tail
between 1 and 2 cm. The normal pancreas is isoechoic or minimally hyperechoic compared with
the liver. The cross-sectional diameter of the pancreatic duct should not exceed 1 to 2 mm.

Overview of cholestatic diseases

Causes of cholestasis vary with patient age. For this
review, diseases are classified into two main classes:

(1) neonatal and (2) older child and adolescent. In

the neonate, biliary atresia, the neonatal hepatitis
syndrome, and choledochal cyst are the most common causes of jaundice [79]. Other causes include
syndromic and nonsyndromic bile duct paucity, inspissated bile syndrome, and spontaneous perforation of the extrahepatic bile duct. In older children,
jaundice is most often caused by hepatocellular disease, including hepatitis and cirrhosis. Biliary tract
obstruction is a less common cause of childhood
jaundice. The possible causes of obstructive jaundice include choledochal cyst, cholangitis, stricture,
stones, and neoplasms.
The results of various laboratory tests of liver
function, in conjunction with the pertinent historical and physical findings, generally suffice to differentiate between obstructive and nonobstructive
causes of jaundice. Imaging examinations are used
to confirm the clinical impression. In patients
who have obstructive jaundice, these studies may
also often show the level and cause of obstruction.
Sonography is the preliminary imaging procedure. If the extrahepatic ducts are well visualized
by sonography and are normal in caliber and there
is no evidence of intraductal dilatation, further radiologic evaluation is rarely needed. Sonography
is supplemented by radionuclide studies using hepatobiliary agents (99mTc-IDA analogs) when functional information is needed. Hepatobiliary
scintigraphy currently is used primarily to confirm
suspected diagnoses of choledochal cysts, biliary
atresia, and neonatal hepatitis. CT or MR imaging
are reserved for cases in which more anatomic detail is needed for surgical planning or the level
or cause of obstruction cannot be determined by
sonography [1015].

Neonatal jaundice
Biliary atresia
Biliary atresia is a rare disease with an incidence of 1
in 8,000 to 10,000 live births. It is, however, the single most common cause of neonatal cholestasis, accounting for nearly 90% of the surgical causes and
for approximately 40% of all causes of cholestasis
[16]. The cause is unclear, but it is believed to be
caused by in utero inflammation that results in failure of the remodeling process at the hepatic hilum
[7]. Histologically, it is characterized by absence of
the extrahepatic bile ducts, proliferation of the
small intrahepatic bile ducts, periportal fibrosis,
and occasionally multinucleated giant cells. There
is a spectrum of changes, depending on the extent
of the obliterative process. Complete atresia is present in 75% to 85% of cases. In the remaining cases,
there may be patency of the gallbladder and cystic
duct or patency of only the gallbladder. Associated

Jaundice in Infants & Children

anomalies are common (10%20% of patients)

and include choledochal cyst, polysplenia, pre-duodenal portal vein, azygous continuation of the inferior vena cava, diaphragmatic hernia, situs inversus,
and hydronephrosis [1618].
Patients who have biliary atresia and neonatal
hepatitis usually present at 1 to 4 weeks of age
with jaundice. Distinguishing between neonatal
hepatitis and biliary atresia is important, because
biliary atresia requires early surgical intervention
to prevent biliary cirrhosis, whereas neonatal hepatitis is managed medically. Surgical treatment
varies with the level of obstruction [7,8]. When
there is extrahepatic biliary obstruction (15%
25% of cases), a direct anastomosis between the
patent portion of the extrahepatic bile duct and intestine is performed. When there is intrahepatic
biliary atresia, a Kasai hepatoportoenterostomy
(anastomosis of a segment of small bowel to the
portal region) is performed [7,8,19]. The success
rate of the Kasai procedure is inversely proportional to patient age. Bile flow can be re-established in up to 90% of infants who are younger
than 2 months of age at the time of hepatoportoenterostomy and in approximately 50% in those
who are 2 to 3 months. The success rate decreases
to less than 20% when surgery is performed after
90 days of age because of the presence of cirrhosis
[79]. Liver transplantation is often required in
older infants and children who have intrahepatic
biliary atresia.
A spectrum of findings may be seen sonographically, reflecting the underlying histology. The liver
size and parenchymal echogenicity may be normal
or increased [1]. The intrahepatic ducts are typically not dilated. The extrahepatic duct is typically
not visualized. A remnant of the extrahepatic duct,
however, may be noted in the porta hepatis [20
23]. This remnant appears as a triangular or tubular echogenic structure just superior to the portal
vein bifurcation. This finding has been termed
the triangular cord sign and correlates with fibrous
tissue in the porta hepatis at histologic examination (Fig. 2). The sign is reliable for the diagnosis
of extrahepatic biliary atresia and has a specificity
approaching 100% and a sensitivity of approximately 85%.
In biliary atresia, the gallbladder is usually small
or absent (Fig. 3), although a normal-sized gallbladder may be seen when the atresia is distal to
the insertion of the cystic duct (approximately
10% of cases). The finding of a small gallbladder
(<1.5 cm in diameter) is nonspecific and may be
seen with biliary atresia or neonatal hepatitis. Contractility and changes in gallbladder size after a milk
feeding are rare in patients who have biliary atresia
(<10% of cases) [24,25].

Fig. 2. Biliary atresia, cord sign. Transverse sonogram

through the porta hepatis shows an echogenic cord
(arrowheads) anterior to the portal vein (arrow), indicating fibrosis along the course of the common hepatic duct.

Neonatal hepatitis syndrome

The neonatal hepatitis syndrome is the term given
to nonspecific hepatic inflammation that develops
secondary to several different causes, including infection (cytomegalovirus, herpes simplex, toxoplasmosis, protozoa, syphilis), metabolic defects (alpha
1-antitrypsin deficiency, galactosemia, glycogen
storage disease, tyrosinosis), and Alagille syndrome.

Fig. 3. Biliary atresia. Transverse scan shows normal

parenchymal echogenicity. The gallbladder (arrow)
is small. The common bile duct was not visualized.

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Additional imaging studies to differentiate

atresia and hepatitis

Fig. 4. Neonatal hepatitis. Longitudinal sonogram

shows diffusely increased and coarsened echogenicity.
The gallbladder is small and filled with sludge (arrow).

Histologic examination shows multinucleated giant

cells with hepatic parenchymal disruption and little
bile within the bile duct canaliculi. Similar to biliary atresia, the cause is believed to be an in utero inflammatory process and the disease process usually
manifests with jaundice at 3 to 4 weeks of life.
At sonography, the liver size and echogenicity
may be normal or increased, and the biliary ducts
are not dilated [1,2] (Fig. 4). The gallbladder may
be small, normal, or increased in size. Changes in
gallbladder size after a milk feeding can occur in patients who have neonatal hepatitis, reflecting patency of the common hepatic and common bile
duct [24].

Hepatobiliary scintigraphy
Because the sonographic findings of biliary atresia
and hepatitis overlap, hepatobiliary scintigraphy is
usually performed to assess the presence or absence
of bile excretion into the bowel. Infants who have
biliary atresia less than 3 months of age usually
show normal hepatic extraction of tracer but no excretion of the radionuclide into the small intestine
(Fig. 5A), whereas infants older than 3 months of
age show decreased extraction of tracer and no
excretion into the bowel. In neonates who have
neonatal hepatitis, parenchymal extraction is
diminished but there is some excretion into the
bowel (Fig. 5B).
The sensitivity and specificity of scintigraphy for
the diagnosis of biliary atresia in infants less than
3 months of age is approximately 95% and 80%, respectively. The presence of small bowel activity excludes biliary atresia as the cause of jaundice.
Differentiation between biliary atresia and neonatal
hepatis is more difficult when there is poor hepatocellular function.

Magnetic resonance imaging

MR cholangiopancreatography may also be useful
in assessing the patency of intra- and extrahepatic
biliary ducts [10,12]. Complete visualization of
the extrahepatic biliary system excludes biliary atresia as the cause of cholestasis [10].

Fig. 5. Hepatobiliary imaging in neonatal jaundice. (A) Biliary atresia. Hepatobiliary scan obtained 6 hours after
injection of Tc-99m disofenin demonstrates hepatic uptake but absence of excretion into the central bile ducts
and intestine. (B) Neonatal hepatitis. Hepatobiliary scan obtained 3 hours after injection shows radioactivity in
the gallbladder (*) and within bowel (arrows). On more delayed images, there was poor clearance of radioactivity from the liver.

Jaundice in Infants & Children

Cholangiography
Cholangiography is performed when other clinical
or imaging findings suggest the diagnosis of biliary
atresia. It may be performed percutaneously, endoscopically, or intraoperatively. Contrast medium is
injected into the gallbladder.

Alagille syndrome
Alagille syndrome (also known as arteriohepatic
dysplasia) is a hereditary disorder, usually an autosomal dominant trait with variable penetrance
[8,26]. A deletion in the short arm of chromosome
20 has been seen in some patients [26]. It is associated with abnormalities of the liver (cholestatic
jaundice), heart (most commonly peripheral pulmonic stenosis), skeleton (butterfly vertebrae and
hemivertebrae), eye, kidneys, and abnormal facies
(frontal bossing, deep-set eyes, bulbous tip of the
nose, and pointed chin). The associated findings
help to distinguish Alagille syndrome from biliary
atresia. Patients typically present with jaundice in
the neonatal period. Histologic examination shows
paucity and hypoplasia of the interlobular bile
ducts. Imaging findings are similar to those described for neonatal hepatitis.

Choledochal cyst
Choledochal cyst is a congenital dilatation of the
common bile duct, with 30% of cases found to occur in the first year of life, 50% between 1 and 10
years of age, and 20% in the second decade or later
[27]. The classic clinical presentation is jaundice,
abdominal pain, and mass, although this triad is
present in only 20% to 50% of patients [27]. This
abnormality is believed to be the result of an abnormal insertion of the common bile duct into the
pancreatic duct, which allows reflux of pancreatic
enzymes into the biliary system. This reflux results in a chemical cholangitis, which weakens the
walls of the bile duct, eventually leading to ductal
dilatation [28].
Four types of choledochal cysts have been described [29]. The type 1 cyst, accounting for 80%
to 90% of cases, is subdivided into type 1A, cystic
dilatation of the common duct; type 1B, focal segmental common duct dilatation; and type 1C, fusiform dilatation of the common bile duct. The type
2 cyst, accounting for approximately 2% of cases, is
a true diverticulum arising from the common duct.
The type 3 cyst, accounting for 1% to 5% of cases, is
a choledochocele involving only the intraduodenal
portion of the duct. The type 4 cyst is subdivided
into type 4A, multiple intrahepatic cysts and an extrahepatic cyst, and type 4B, multiple extrahepatic
cysts. The type 5 cyst, or Caroli disease, consists of

multiple intrahepatic biliary cysts and is considered

to be a separate disorder (see later discussion).
Choledochal cysts in neonates and young infants
may coexist with biliary atresia [17,18].
At sonography, the choledochal cyst appears as
a fluid-filled cystic mass in the region of the porta
hepatis that is separate from the gallbladder
(Fig. 6). Intrahepatic biliary duct dilatation is present in approximately half of affected patients and
typically is limited to the central portions of the left
and right main hepatic ducts. Generalized ductal dilatation, typical of acquired obstruction, is absent.
The cysts tend to be smaller and ductal dilatation
is absent when there is concomitant biliary atresia
[17,18]. Complications associated with choledochal cysts include cholelithiasis, choledocholithiasis, ascending cholangitis, intrahepatic abscesses,
biliary cirrhosis, portal hypertension, and hepatobiliary malignancy, usually adenocarcinomas. The
risk for malignancy increases with age [27].
When a choledochal cyst is demonstrated sonographically, scintigraphy with hepatobiliary agents
is performed to confirm that the cystic mass communicates with the biliary system. Preoperative CT
is acquired to further define the anatomy of the intrahepatic biliary tree and the distal common bile
duct [13]. MR cholangiography may also be useful
in the preoperative anatomic assessment of these
lesions [1012].

Spontaneous perforation
of the extrahepatic bile ducts
Spontaneous perforation of the extrahepatic bile
ducts is a cause of neonatal jaundice and ascites,
usually affecting infants between 1 week and 4
months of age. The clinical findings include ascites,
mild jaundice, failure to thrive, and abdominal distension. The serum bilirubin level is elevated,
whereas other liver function tests are normal. The
latter feature is helpful in differentiating perforation
from neonatal hepatitis and biliary atresia, which
have similar clinical findings but abnormal liver
function tests. The most frequent site of perforation
is the junction of the cystic and common bile ducts.
Rarely the perforation involves the common hepatic duct, gallbladder, or junction of the cystic duct
and gallbladder [30].
Sonography shows generalized ascites or a loculated fluid collection in the porta hepatis [30]
(Fig. 7). Echogenic debris or fine septations may
be present within the ascitic fluid. The biliary tree
is not dilated because it is not obstructed. Gallbladder or distal common duct calculi may be associated findings. Hepatobiliary scintigraphy is useful
to confirm the diagnosis by showing leaking of radioactive tracer into the peritoneal cavity. Surgical

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Fig. 6. Choledochal cyst in a young boy with jaundice. (A) Longitudinal and (B) transverse sonograms through
the liver demonstrate a cystic mass (C), representing the choledochal cyst, in the porta hepatis separate from
the gallbladder (GB). P, pancreas. (C) Contrast-enhanced CT scan confirms the cystic mass (C), which is the dilated
common bile duct.

placement of a drainage tube in the area of perforation usually results in spontaneous closure.

cyst (see earlier discussion) and Caroli disease, diseases of the hepatocytes, and inflammatory and obstructive lesions of the biliary ducts.

Inspissated bile syndrome

The inspissated bile or bile-plug syndrome refers to
an extrahepatic obstruction of the bile ducts by biliary sludge [2]. This condition typically affects fullterm infants. Inspissated bile syndrome has been
associated with massive hemolysis, hemorrhage, total parenteral nutrition, cystic fibrosis, and various
intestinal diseases (Hirschsprung disease, intestinal
atresias, and stenoses). Sonography shows moderately or highly echogenic bile within the gallbladder and often within dilated intra- or extrahepatic
bile ducts. Although the bile is echogenic, it does
not cause acoustic shadowing. The ductal dilatation
may be difficult to recognize if the echogenicity of
the inspissated bile and liver are similar [1,2].

Jaundice in older infants and children

The causes of jaundice in older children and adolescents include cystic diseases, including choledochal

Caroli disease
Caroli disease, also known as congenital cystic dilation of the intrahepatic biliary tract, has two forms.
One form is characterized by segmental, saccular dilation of the intrahepatic bile ducts, an increased
frequency of calculus formation and cholangitis,
and the absence of cirrhosis and portal hypertension. The other form is characterized by hepatic fibrosis, cirrhosis, and portal hypertension. Both
forms of Caroli disease are associated with renal
cystic disease, including renal tubular ectasia (medullary sponge kidney), cortical cysts, and autosomal
recessive polycystic disease. Patients who have Caroli disease, like those who have choledochal cysts,
have an increased risk for developing cholangiocarcinoma. Patients may present in the neonatal period [31], but the vast majority present as young
adults who have abdominal pain, fever, and jaundice or with portal hypertension.

Jaundice in Infants & Children

Fig. 7. Spontaneous perforation of the common bile duct. (A) Transverse sonogram through the upper abdomen
demonstrates ascites (A) in the perihepatic space. (B) On a more caudad image, a loculated fluid (F) collection
and a calculus (arrow) are noted in the porta hepatis.

Sonography shows multiple dilated tubular

structures, typical of biliary radicals (Fig. 8). These
can converge, creating larger saccular areas [31].
The portal radicals may be partially or completely
surrounded by the dilated ducts (termed the central
dot sign) [32] (Fig. 9). The extrahepatic bile ducts
can be normal, narrowed, or associated with a choledochal cyst. Findings of portal hypertension may
be observed in patients who have hepatic fibrosis.

Byler disease
Byler syndrome (also known as progressive familial
intrahepatic fibrosis) is a familial intrahepatic cholestatic syndrome that is associated with cystic hepatic lesions and jaundice. Histologically, there is
periportal fibrosis, micronodular cirrhosis, and
periductal cysts. Symptoms, including jaundice,
pruritus, and hepatomegaly, usually appear by the
end of the first year of life. The sonographic findings

Fig. 8. Caroli disease. (A) Transverse Doppler sonogram shows dilatation of the intrahepatic bile ducts (arrows),
which converge toward the porta hepatis. The color Doppler image helps confirm the absence of flow in the
dilated ducts. Flow is seen in the portal vein (PV). (B) CT confirms saccular dilatation of the bile ducts. (From
Siegel MJ. Gallbladder and biliary tract. In: Siegel MJ, editor. Pediatric sonography. 3rd edition. Philadelphia:
Lippincott Williams & Wilkins; 2002. p. 276304; with permission).

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Fig. 9. Caroli disease. Transverse sonogram shows dilated ducts (arrows) that completely envelope the
portal radicals. This appearance is termed the central
dot sign.

are multiple saccular cystic lesions, some of which

may contain echogenic portal veins (the central
dot sign) [33]. Unlike Caroli disease, the cysts in Byler disease do not communicate with the bile ducts.

Hepatocellular diseases
Hepatocellular disease can be classified into two
major classes: infectious (acute and chronic hepatitis) and noninfectious (metabolic disorders, drugs,
toxins, and autoimmune diseases). The sonographic appearance of the liver depends on the severity of the insult, rather than on the causative
agent [1]. Sonography is usually normal in cases
of mild acute infectious hepatitis. Sonographic
findings in severe acute hepatitis include hepatomegaly, decreased parenchymal echogenicity, and
increased echogenicity of the portal venule walls
(starry sky liver) (Fig. 10). The gallbladder wall
may be small, thick-walled, and filled with intraluminal sludge. In chronic active hepatitis, the liver
often appears heterogeneous and hyperechoic with
irregular margins and decreased visualization of the
portal venous radicles (Fig. 11). The gallbladder
may be small and contain thick bile, sludge, or
stones, and collateral vessel formation may be
noted.
Metabolic causes of jaundice include Wilson
disease, cystic fibrosis, glycogen storage disease,
tyrosinemia, and a1-antitrypsin deficiency. The
sonographic appearance of these disorders is nonspecific and can be similar to that of acute or
chronic hepatitis. A definitive diagnosis requires
correlation with clinical information and laboratory results, and in many cases biopsy is needed
to confirm the diagnosis.

Fig. 10. Acute hepatitis. Starry sky liver. Sagittal sonogram of the liver shows brightly echogenic portal venous triads. The liver was also moderately enlarged.

Inflammatory diseases of the biliary ducts

Sclerosing cholangitis
Sclerosing cholangitis is a chronic cholestatic disorder characterized by inflammatory obliterative fibrosis of the extrahepatic and intrahepatic bile
ducts leading to biliary cirrhosis and ultimately
liver failure [34]. This entity has been associated
with chronic inflammatory bowel disease, Langerhans histiocytosis X, and immunodeficiency disorders [34]. Histologic examination shows multiple
segmental strictures, diverticula formation between
areas of stricture, and mural thickening of the bile
ducts. Clinical manifestations include jaundice
and right upper quadrant pain. Most affected patients are adolescents or adults.

Fig. 11. Chronic hepatitis. Longitudinal sonogram of

the liver shows an enlarged liver with irregular margins and diffusely coarse echotexture.

Jaundice in Infants & Children

extrahepatic bile ducts, and a dilated, thick-walled

gallbladder wall [3537]. An additional finding is
a hyperechoic nodule in the distal end of the common bile duct caused by edema of the papilla of
Vater [38].

Biliary tract obstruction

Fig. 12. Sclerosing cholangitis. Longitudinal sonogram shows a dilated, thick-walled common bile duct
(arrows) (diameter, 9 mm). PV, portal vein; SV, splenic
vein.

Sonographic findings include a thick-walled gallbladder, thick-walled dilated intrahepatic ducts

(Fig. 12), intrahepatic and intraductal stones,
cholelithiasis, and segmental ductal narrowing
secondary to stricturing. The strictures may be difficult to detect sonographically unless the ducts are
dilated. In longstanding disease, sonography may
show findings of biliary cirrhosis and portal
hypertension.

Sonographic features
Biliary obstruction resulting in jaundice is usually
the result of stone disease. Acute pancreatitis, neoplasm, and benign strictures are less common
causes of obstructive jaundice in children. The sonographic diagnosis of biliary obstruction is based
on the demonstration of dilated intrahepatic or extrahepatic bile ducts. Dilated intrahepatic biliary
radicles appear as multiple, anechoic branching
structures that enlarge as they approach the porta
hepatis. The dilated common hepatic and common
bile ducts may appear as round or tubular anechoic
structures near the porta hepatis or the head of the
pancreas (Fig. 13).

AIDS-related cholangitis
The common biliary tract abnormalities in children
who have AIDS are acalculous cholecystitis and
cholangitis. The sonographic findings in AIDSrelated cholangitis are similar to those of sclerosing
cholangitis and include ductal dilatation and wall
irregularity (Fig. 13), stricture of the intra- and

Cholelithiasis and choledocholithiasis

Stones in the common bile duct usually originate in
the gallbladder and migrate distally. Cholelithiasis
in neonates has been associated with congenital
anomalies of the biliary tract, total parenteral nutrition, furosemide therapy, phototherapy, dehydration, infection, hemolytic anemias, and short-gut
syndrome [9]. In older children, causes of cholelithiasis include sickle cell disease, cystic fibrosis,
malabsorption, total parenteral nutrition, liver disease, Crohn disease, bowel resection, and hemolytic
anemia.
Choledocholithiasis typically manifests as
brightly echogenic shadowing foci within the

Fig. 13. AIDS-related cholangitis. Longitudinal sonogram at the level of the porta hepatitis demonstrates
intrahepatic ductal dilatation (arrows). (Courtesy of
Edward Lee, MD, Boston, MA).

Fig. 14. Choledocholithiasis. Longitudinal scan at the

level of the pancreatic head (P) shows a stone
(between calipers) without shadowing. At operation,
calcium bilirubinate stones were found in the gallbladder and distal duct. Ductal diameter is 11 mm.

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Fig. 15. Cystic duct stone. (A) Longitudinal scan shows a calculus (calipers) in the cystic duct. A second calculus
(arrow) is noted in the distal common bile duct, which is dilated. (B) Endoscopic retrograde cholangiogram confirms the stone (arrow) in the cystic duct. (From Siegel MJ. Gallbladder and biliary tract. In: Siegel MJ, editor.
Pediatric sonography. 3rd edition. Philadelphia: Lippincott Williams & Wilkins; 2002. p. 276304; with
permission).

biliary ducts and is usually associated with ductal

dilatation (Fig. 14). The calculi obstruct anywhere
in the biliary duct, but most cause obstruction at
the level of the pancreatic head. The sensitivity for
detection of choledocholithiasis is lower for stones
in the distal versus the proximal duct. A calculus impacted in the distal duct can be more difficult to detect because of adjacent or overlying bowel gas and
because the calculus is surrounded by the echogenic
pancreatic head.
Cystic duct stones
Mirizzi syndrome is a rare cause of biliary obstruction in children. It is secondary to an impacted cystic duct stone, which causes extrinsic compression
or inflammatory stricture of the common duct.
Sonographic findings include calculi in the gallbladder neck or in the cystic duct and dilated intrahepatic ducts, including the common hepatic duct
(Fig. 15).
Biliary neoplasms
Rhabdomyosarcoma of the biliary tract is rare, but
it is the most common neoplasm of the biliary tract
in children. Most rhabdomyosarcomas arise in the
porta hepatis and involve the cystic duct. Sonographic findings are intra- and extrahepatic ductal
dilatation and an echogenic mass without acoustic
shadowing. Rhabdomyosarcoma spreads by direct
extension to contiguous structures or by hematogenous or lymphatic dissemination to lymph nodes,
lungs, bone, bone marrow, or liver.
Biliary duct strictures
Biliary stricture is an uncommon cause of distal obstruction, but the diagnosis needs to be considered

in patients who have biliary obstruction in whom

no calculus or other obstructing lesion can be visualized. An abrupt transition from a dilated duct to
one of normal caliber is a finding suggestive of
stricture.

References
[1] Gubernick JA, Rosenberg HK, Ilaslan H, et al. US
approach to jaundice in infants and children. Radiographics 2000;20:17395.
[2] Siegel MJ. Gallbladder and biliary tract. In:
Siegel MJ, editor. Pediatric sonography. 3rd edition. Philadelphia: Lippincott Williams & Wilkins; 2002. p. 276304.
[3] Hernanz-Schulman
M,
Ambrosino
MM,
Freeman PC, et al. Common bile duct in children: sonographic dimensions. Radiology 1982;
145:437.
[4] Wachsberg RH. Respiratory variation of extrahepatic bile duct diameter during ultrasonography.
J Ultrasound Med 1994;13:617.
[5] Wolson AH. Ultrasound measurements of the
gallbladder. In: Goldberg BB, Kurtz AB, editors.
Atlas of ultrasound measurements. Chicago: Year
Book Publishers, Inc.; 1990. p. 10812.
[6] Siegel MJ, Martin KW, Worthington JL. Normal
and abnormal pancreas in children: US studies.
Radiology 1987;165:158.
[7] Balistreri WF, Grand R, Hoofnagle JH, et al. Biliary atresia: current concepts and research directions. Summary of a symposium. Hepatology
1996;23:168290.
[8] Balistreri WF, Schubert WK. Liver diseases in
infancy and childhood. In: Schiff L, Schiff ER,
editors. Diseases of the liver. 7th edition.
Philadelphia: JB Lippincott; 1993. p. 1099203.

Jaundice in Infants & Children

[9] Roberts EA. The jaundiced baby. In: Kelly DA,

editor. Diseases of the liver and biliary system
in children. Oxford UK: Blackwell Science; 1999.
p. 1145.
[10] Guibaud L, Lachaud A, Touraine R, et al. MR
cholangiography in neonates and infants: feasibility and preliminary applications. Am J Roentgenol 1998;170:2731.
[11] Irie H, Honda H, Jimi M, et al. Value of MR cholangiopancreatography in evaluating choledochal
cysts. Am J Roentgenol 1998;171:13815.
[12] Miyazaki T, Yamashita Y, Tang Y, et al. Single-shot
MR cholangiopancreatography of neonates, infants, and young children. Am J Roentgenol 1998;
170:337.
[13] Siegel MJ. Liver and biliary tract. In: Siegel MJ,
editor. Pediatric body CT. Philadelphia: Lippincott Williams Wilkins; 1999. p. 14174.
[14] Siegel MJ. Pediatric liver imaging. Semin Liver
Dis 2001;21:25169.
[15] Roy CC, Silverman A, Alagille D. Pediatric clinical gastroenterology. St. Louis: Mosby; 1995.
[16] Abramson SJ, Berdon WE, Altman RP, et al. Biliary
atresia and noncardiac polysplenic syndrome: US
and surgical considerations. Radiology 1987;
163:3779.
[17] Kim WS, Kim IO, Yeon KM, et al. Choledochal
cyst with or without biliary atresia in neonates
and young infants: US differentiation. Radiology
1998;209:4659.
[18] Torrisi JM, Haller JO, Velcek FT. Choledochal cyst
and biliary atresia in the neonate: imaging findings in five cases. Am J Roentgenol 1990;
155:12736.
[19] Carceller A, Blanchard H, Alvarez F, et al. Past
and future of biliary atresia. J Pediatr Surg
2000;35:71720.
[20] Choi S-O, Park W-H, Lee H-J, et al. Triangular
cord: a sonographic finding applicable in the diagnosis of biliary atresia. J Pediatr Surg 1996;
31:3636.
[21] Kendrick APT, Phua KB, Subramaniam R, et al.
Making the diagnosis of biliary atresia using
the triangular cord sign and gallbladder length.
Pediatr Radiol 2000;30:6973.
[22] Park W-H, Choi S-O, Lee H-J, et al. A new diagnostic approach to biliary atresia with emphasis
on the ultrasonographic triangular cord sign:
comparison of ultrasonography, hepatobiliary
scintigraphy, and liver needle biopsy in the evaluation of infantile cholestasis. J Pediatr Surg
1997;32:15559.
[23] Park W-H, Choi S-O, Lee H-J. The ultrasonographic triangular cord couples with

[24]

[25]

[26]

[27]
[28]

[29]

[30]

[31]

[32]

[33]

[34]

[35]

[36]

[37]

[38]

gallbladder images in the diagnostic prediction

of biliary atresia from infantile intrahepatic cholestasis. J Pedatr Surg 1999;34:170610.
Ikeda S, Sera Y, Ohshiro H, et al. Gallbladder
contraction in biliary atresia: a pitfall of ultrasound diagnosis. Pediatr Radiol 1998;28:4513.
Weinberger E, Blumhagen JD, Odell JM. Gallbladder contraction in biliary atresia. Am J
Roentgenol 1987;149:4012.
Alagille D, Estrada A, Hadchouel M, et al.
Syndromic paucity of interlobular bile ducts
(Alagille syndrome or arteriohepatic dysplasia): review of 80 cases. J Pediatr 1987;110:
195200.
Kim OH, Chung HJ, Choi BG. Imaging of the
choledochal cyst. Radiographics 1995;15:6988.
Babbitt DP, Starshak RJ, Clemett AR. Choledochal cyst: a concept of etiology. Am J Roentgenol
1973;119:5762.
Savader SJ, Benenati JF, Venbrux AC, et al. Choledochal cysts: classification and cholangiographic appearance. Am J Roentgenol 1991;
156:32731.
Haller JO, Conden VR, Berdon WE, et al. Spontaneous perforation of the common bile duct in
children. Radiology 1989;172:6214.
Toma P, Lucigrai G, Pelizza A. Sonographic patterns of Carolis disease: report of 5 new cases.
J Clin Ultrasound 1991;19:15561.
Miller WJ, Sechtin AG, Campbell WL, et al. Imaging findings in Carolis disease. Am J Roentgenol
1995;165:3337.
Herman TE, Siegel MJ. Central dot sign on CT of
liver cysts. J Comput Assist Tomogr 1990;
14:101921.
Debray D, Pariente D, Urvoas E, et al. Sclerosing
cholangitis in children. J Pediatr 1994;124:
4956.
Chung CJ, Sivit CJ, Rakusan TA, et al. Hepatobiliary abnormalities on sonography in children
with HIV infection. J Ultrasound Med 1994;
13:20510.
Dolmatch BL, Laing FC, Federle MP, et al.
AIDS-related cholangitis: radiographic findings
in nine patients. Radiology 1987;163:313.
Grumbach K, Coleman BG, Gal AA, et al. Hepatic and biliary tract abnormalities in patients
with AIDS. Sonographic-pathologic correlation.
J Ultrasound Med 1989;8:24754.
Da Silva F, Boudghene F, Lecomte I, et al. Sonography in AIDS-related cholangitis: prevalence
and cause of an echogenic nodule in the distal
end of the common bile duct. Am J Roentgenol
1993;160:12057.

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