Pediatr Radiol (2017) 47:1069–1078
DOI 10.1007/s00247-017-3869-y
PEDIATRIC ULTRASOUND
Ultrasound of congenital and inherited disorders of the pediatric
hepatobiliary system, pancreas and spleen
Susan J. Back 1 & Carolina L. Maya 2 & Asef Khwaja 1
Received: 30 December 2016 / Revised: 26 February 2017 / Accepted: 11 April 2017 / Published online: 2 August 2017
# Springer-Verlag Berlin Heidelberg 2017
Abstract Ultrasound is often the initial imaging examination
performed of the solid organs of the pediatric abdomen. The
sonographic appearance of the hepatobiliary system, pancreas
and spleen changes with growth and development. This article
reviews the normal US appearance of these organs in children
and illustrates, through case examples, congenital and
inherited conditions that affect them.
Keywords Liver . Pancreas . Pediatrics . Spleen . Ultrasound
Introduction
Because ultrasound (US) is widely available, well tolerated,
typically performed without sedation and lacks ionizing radi-
ation, it is commonly the first imaging modality used to assess
the pediatric abdomen. The body habitus of children also
lends itself to high-quality US examination of the abdominal
organs. The goal of this article is to provide an overview of the
normal sonographic appearance of the liver, biliary system,
pancreas and spleen in children. Here we highlight the utility
of US in the assessment of children with non-infectious and
non-neoplastic congenital and inherited conditions that affect
these organs.
* Susan J. Back
backs@email.chop.edu
1
Department of Radiology, The Children’s Hospital of Philadelphia,
Perelman School of Medicine, University of Pennsylvania,
3401 Civic Center Boulevard, Philadelphia, PA 19104, USA
2
Department of Radiology, The Children’s Hospital of Philadelphia,
3401 Civic Center Boulevard, Philadelphia, PA 19104, USA
Hepatobiliary system
Liver development begins in the 4th week of gestation as an
outpouching from the ventral foregut. The liver and biliary
ducts develop from the cranial aspect of the hepatic divertic-
ulum; the gallbladder and cystic duct develop from the caudal
portion [1]. There is an intricate relationship among the devel-
opment of the ductal plate, the portal venous and the hepatic
arterial systems [2]. In normal development, the majority of
the liver is in the right upper abdomen, with the left hepatic
segment extending across midline to the left upper quadrant.
In children with situs abnormalities, the position of the liver
and gallbladder might be abnormal. In such cases, the liver
might be midline (bridging) or in the left upper abdomen (in
the majority), and the gallbladder might be absent [3].
Normally the liver is on the right.
The US examination is performed in the fasted state with
the highest-frequency curved transducer available based on
the child’s body habitus. Linear high-frequency probes are
also used to evaluate the liver contour. The liver is examined
through a subcostal or intercostal approach in the long and
short axes to evaluate organ size and the presence of focal or
diffuse abnormality [4].
Several population studies have reported normal liver size
for age, height and weight [5–9]. While the correlation coef-
ficients between these parameters and the longitudinal liver
measurement are very similar, most report the best correlation
is with height [6]. For preterm infants, organ length correlates
slightly better for weight than height, and girls tend to be
smaller than boys [9]. Normal values are also published for
the gallbladder size and wall thickness and common hepatic
duct size. In a group of 51 children, the mean gallbladder
length for children from birth to 1 year of age was 2.5 cm
(range 1.3–3.4 cm) and from 12 years to 16 years was
6.1 cm (range 3.8–8 cm). Across all ages, the gallbladder wall
1070
thickness was always ≤3 mm and the common hepatic duct
less than 4 mm [10]. The echotexture of the normal liver
should be greater than that of the right kidney and less than
that of the pancreas and spleen (Fig. 1). This applies to older
children because in babies the renal parenchyma can be more
echogenic. The interface between the hepatic parenchyma and
the portal triads and vessel walls should be visible [11].
Congenital disorders of the hepatobiliary system present
with a wide variety of clinical signs and symptoms or bio-
chemical abnormalities. Fibropolycystic diseases comprise a
group of liver and biliary abnormalities that result from mal-
formation of the hepatic ductal plate. The specific diagnosis is
based on the size of the affected ducts. Small-duct involve-
ment leads to congenital hepatic fibrosis and biliary
hamartoma; medium-duct involvement results in autosomal-
dominant polycystic liver disease; and large-duct involvement
causes either choledochal cyst (extrahepatic) or Caroli disease
(intrahepatic) [2]. The age at presentation depends on the se-
verity of the hepatic and renal manifestations [12]. Congenital
hepatic fibrosis can progress to cirrhosis and liver failure and
portends an increased risk of hepatocellular carcinoma [2].
Typical US imaging findings include hepatomegaly,
hyperechoic parenchymal echotexture and portal triads, poor-
ly defined portal vessels, and dilated intra- and extrahepatic
bile ducts (Fig. 2). Recently US shear wave elastography
(SWE) has been used to assess liver stiffness (Fig. 2). SWE
measurements correlate with pediatric hepatic fibrosis and
therefore might serve as a noninvasive measure of liver dis-
ease [13]. No imaging finding is specific for congenital hepat-
ic fibrosis, and the diagnosis is ultimately made by liver biop-
sy [2]. Treatment is directed toward managing the complica-
tions of cirrhosis and portal hypertension [12].
Fig. 1 Normal liver. Gray-scale transabdominal US of the right upper
quadrant in a sagittal plane in a 7-year-old boy shows normal
echogenicity of the liver and kidney. The liver (L) is hyperechoic
relative the right kidney (K)
Pediatr Radiol (2017) 47:1069–1078
Choledochal cysts are dilations of the bile ducts. There are
two theories about the formation of choledochal cysts [2]. One
is that they are a ductal plate malformation of the large extra-
hepatic ducts. A second theory is that abnormal common bile
duct anatomy allows reflux of pancreatic enzymes into the
duct, resulting in cholangitis and cyst formation [2]. The
Todani classification categorizes cysts based on the number,
configuration and location (intra- or extrahepatic) of the ductal
dilation [14]. The clinical triad of abdominal pain, jaundice
and mass is present in a minority of patients. US can readily
identify right upper quadrant and intrahepatic cystic masses
and therefore these lesions might be detected in the evaluation
of abdominal pain (Fig. 3). Cystic dilation of the biliary tree
must be differentiated from hepatic cysts. MRI and magnetic
resonance cholangiopancreatography (MRCP) are often per-
formed for larger and inconclusive lesions because treatment
requires surgical resection (Fig. 3) [2].
Extrahepatic biliary atresia and neonatal hepatitis are the
cause of 60–90% of conjugated hyperbilirubinemia [15].
Despite similarities of laboratory and clinical findings in these
entities, their management is markedly different. Infants with
biliary atresia initially undergo portoenterostomy and often go
on to liver transplantation. The diagnosis is time-sensitive be-
cause portoenterostomy is less likely to be beneficial if per-
formed beyond the age of 3 months. In a meta-analysis of 23
studies published over 25 years on the sonographic diagnosis
of biliary atresia, the authors concluded that gallbladder ab-
normalities and the triangular cord sign are the most accurate
sonographic findings of biliary atresia (Fig. 4). Gallbladder
US findings are subjective and include its absence, small size,
abnormal shape, abnormal wall and absent contraction. The
triangular cord sign is a triangular or tubular echogenicity in
the region of the porta hepatis on transverse or longitudinal
US and is thought to represent the fibrous remnant of the
extrahepatic duct. The summary sensitivity of 0.95 (0.7–
0.99) for these findings was greater than either sign alone
[16]. However in a prospective study limited to children youn-
ger than 90 days, the triangular cord sign was less sensitive
than previous reports [15]. It was postulated that in this age
group the triangular cord sign might be absent or too small to
identify. Irregularity of the gallbladder wall was highly sensi-
tive and specific and absent gallbladder was always associated
with extrahepatic biliary atresia [15]. Babies with indetermi-
nate US might undergo hepatobiliary scintigraphy, intraoper-
ative cholangiogram or liver biopsy. Shear wave elastography
has shown promising results in differentiating biliary atresia
from other neonatal liver diseases and normal babies based on
increased hepatic stiffness in biliary atresia [13, 17, 18].
However, in one study there was overlap in measured liver
stiffness between those with biliary atresia, and two children
with giant cell hepatitis and one with Alagille syndrome [19].
Alagille syndrome is an autosomal-dominant disorder that
affects multiple organ systems and sometimes presents with
Pediatr Radiol (2017) 47:1069–1078
ƒFig. 2
cholestasis. It is important to distinguish Alagille from biliary
atresia because Alagille patients who undergo a
portoenterostomy have a worse prognosis [20]. Alagille syn-
drome is traditionally diagnosed based on the classic criteria
where three of the following are present: cholestasis, charac-
teristic facies, cardiac defect, vertebral anomalies and ophthal-
mologic findings. Liver pathology in Alagille syndrome
1071
Congenital hepatic fibrosis in a 3-year-old girl who presented
with abdominal distension, hepatosplenomegaly and thrombocytopenia.
Gray-scale transabdominal US image of the right upper quadrant in the
sagittal plane shows abnormal, hyperechoic and heterogeneous liver
echotexture. Liver biopsy showed portal and periportal fibrosis. a
Image of the liver (L) obtained with a curved C9–2 MHz transducer. b
Image of the liver (L) obtained with a linear 12–5 MHz transducer. c
Ultrasound elastography with increased mean shear wave velocity
(2.58 m/s) calculated from 10 velocity samples, obtained from hepatic
segment 8. This is increased relative to the normal pediatric liver stiffness
of 1.16 m/s (standard deviation 0.14 m/s) on this equipment. The green
rectangle is the region of interest where shear wave velocity was
measured
Fig. 3 Choledochal cyst in a 4-year-old boy with several weeks of
abdominal pain. a Gray-scale transabdominal US in the sagittal plane
with dilation of the common bile duct (star). The fusiform nature of the
dilation was better seen on MRI. Echogenic debris is seen in the lesion
(arrow). b Coronal T2-W half-Fourier acquisition single-shot turbo spin-
echo (HASTE) MR image with fat saturation shows fusiform extrahepatic
ductal dilation (star). There is mild dilation of the intrahepatic ducts
(arrows). Sludge was seen in the intrahepatic ducts on other images
(not shown). This boy underwent type I choledochal cyst resection and
hepaticojejunostomy. C cystic duct, G gallbladder, K kidney, L liver
1072
Fig. 4 Triangular cord sign in a 5-month-old girl with known biliary
atresia pre liver transplant evaluation. Transabdominal color Doppler
US of the right upper quadrant in the transverse plane shows an
echogenic linear region (arrow) adjacent to the portal vein that
measured up to 4 mm. The gallbladder was not visualized
shows a paucity of bile ducts, differentiated from ductal pres-
ence in congenital hepatic fibrosis, and inflammation with
proliferation of duct and glandular elements and fibrosis in
biliary atresia. Because of the normal progression of biliary
maturation, biopsies taken early in life sometimes incorrectly
conclude a lack of ducts and it is unclear at what age the ductal
findings can be confirmed with biopsy [20]. Maintaining an
index of suspicion and referral to a center that specializes in
pediatric hepatic disease is important for the diagnosis and
management of these children.
Cystic fibrosis liver disease is the most common
extrapulmonary cause of mortality in patients with cystic fi-
brosis (CF) [21]. Among these patients, 5–10% develop cir-
rhosis in childhood [21]. Other than a recent publication on
trending gamma-glutamyl transferase (GGT) to predict cir-
rhotic cystic fibrosis liver disease, biochemical markers have
not been useful in predicting risk of cirrhosis [22]. US is useful
in detecting hepatic parenchymal changes in CF before clini-
cal and biochemical markers (Figs. 5 and 6). Out of 719 base-
line US examinations of children with CF without known liver
disease performed as part of a prospective trial on US assess-
ment of cystic fibrosis liver disease, cirrhosis was detected in
3.3% [23]. Cystic fibrosis liver disease is inhomogeneous and
unevenly distributed and therefore liver biopsy is subject to
sampling error. SWE has demonstrated progressive increase
in liver stiffness in those with cystic fibrosis liver disease and
associated portal hypertension, splenomegaly and varices
compared to those without cystic fibrosis liver disease [23].
Pancreas
The pancreas develops by the 4th week of gestation from
separate ventral and dorsal buds of the duodenal endodermal
lining [24, 25]. During development, there is rotation of the
ventral pancreas, which fuses with the dorsal anlage while the
Pediatr Radiol (2017) 47:1069–1078
Fig. 5 Cystic fibrosis liver disease in a 16-year-old girl. Sagittal gray-
scale transabdominal US image shows a heterogeneous, hyperechoic
hepatic echotexture compatible with cystic fibrosis liver disease
ventral pancreatic duct merges with the central portion of the
dorsal duct. This creates the main duct (of Wirsung), which
extends from this union to the major papilla. The remaining
dorsal duct forms the duct of Santorini that drains into the
minor papilla [24].
Given its location, the pancreas can be difficult to examine
with US. Graded compression or water ingestion can displace
overlying bowel gas. The left hepatic lobe or left kidney
(when prone) acts as an acoustic window to increase pancre-
atic visibility.
The pancreas has a multi-lobulated appearance and extends
from the second portion of the duodenum toward the splenic
hilum. The normal parenchyma is homogeneous and
isoechoic or hyperechoic to the liver (Fig. 7). Newborn and
premature infants tend to have hyperechoic parenchyma
(Fig. 8) [26]. Ten percent of children have hypoechoic paren-
chyma [27]. The pancreas is relatively larger in children than
adults, with rapid growth in the first year followed by slower
growth until 18 years of age [27]. The head and tail are similar
in size, with the intervening body slightly thinner. Normal
Fig. 6 Cystic fibrosis liver disease in a 12-year-old boy. Transverse gray-
scale transabdominal US image shows a homogeneous hyperechoic
hepatic echotexture, which is suggestive of hepatic steatosis
Pediatr Radiol (2017) 47:1069–1078
Fig. 7 Normal pancreas. Transverse gray-scale transabdominal US of the
mid upper abdomen in a 7-year-old boy shows normal echogenicity of the
liver and pancreas. L liver, P pancreas. The pancreatic duct (arrow) was
normal
measurements have been described throughout childhood [3].
With improving technology, the pancreatic duct is frequently
visualized by US. The normal mean diameter of the duct is
1.65 mm (±0.45 mm) and is up to 2.2 mm in adolescents [28].
Fig. 8 Pancreas in a premature infant. Transverse gray-scale
transabdominal US images of the mid upper abdomen in a girl born at
35 1/7 weeks of gestation. a 1 day old. b 7 months old. The echogenicity
of the pancreas is hyperechoic relative to the liver at 1 day but is isoechoic
at 7 months
1073
Given its complicated development, a spectrum of varia-
tions and malformations can occur. Pancreas divisum results
from failed dorsal and ventral bud fusion and is the most
common congenital anomaly, occurring in up to 14% of the
population based on autopsy series [25]. While divisum is
most often complete without ductal communication, there
are variations ranging from a filamentous connection to ab-
sence of the ventral duct [29]. The ductal anatomy is further
delineated with MRCP, and endoscopic retrograde
cholangiopancreatography is reserved for therapeutic inter-
ventions. However conditions thought to be associated with
divisum, such as pancreatitis, can be seen with sonography.
In annular pancreas, pancreatic tissue encircles the second
portion of the duodenum due to incomplete rotation of the
ventral anlage. The prevalence is approximately 1 in 2,000
persons [24, 30]. Two types have been proposed: (a) extramu-
ral subtype where the ventral pancreatic duct encircles the
duodenum to join the main pancreatic duct and (b) intramural
subtype where pancreatic tissue intermingles with duodenal
wall muscle fibers and numerous small ducts drain directly
into the duodenum [25]. Symptoms differ between the sub-
types. Those with the extramural subtype present with ob-
structive symptoms and those with intramural subtype present
with duodenal ulceration [31]. About half of patients present
with duodenal obstruction in the neonatal period. Pancreatic
tissue encircling the descending duodenum can be seen on US
[32].
The congenitally short pancreas results from agenesis of
the dorsal pancreatic anlage. Sonography confirms the ab-
sence of the pancreatic neck, body and tail, with stomach or
bowel loops filling the normal pancreatic bed [33]. Agenesis
of the pancreas can also be seen as part of the spectrum of
anomalies in patients with situs abnormalities [3]. Pancreatic
tissue can also be found in ectopic locations, most often the
stomach, duodenum and jejunum [34, 35]. The actual tissue is
usually not visualized by ultrasound but rather the sequela of
disease involving the ectopic tissue is seen, including bowel
wall thickening in pancreatitis, cysts or malignancy [34, 36,
37].
Congenital hyperinsulinism causes hypoglycemia in the
newborn from hyperplasia of pancreatic beta cells, which
can be diffuse or focal. When 18F-dihydroxyphenylalanine
(DOPA) positron emission tomography (PET)/CT identifies
a focal lesion, surgical excision can be curative.
Intraoperative US with the transducer placed directly onto
the pancreas can localize these small, often <5-mm lesions
for excision (Fig. 9). Typically they are hypoechoic, heteroge-
neous or homogeneous with ill-defined margins [38]. Recent
advent of ultrahigh-resolution US systems and transducers has
further improved visibility of these lesions (Fig. 9).
In children with CF, inspissated pancreatic secretions lead
to obstruction of the exocrine function of the pancreas [39].
This ultimately causes pancreatic inflammation and atrophy
1074
Fig. 9 Congenital hyperinsulinism in a 1-month-old girl. A lesion was
detected at the pancreatic head–neck junction on preoperative 18F-
dihydroxyphenylalanine positron emission tomography (PET)/CT (not
shown). The lesion was not seen on preoperative transabdominal gray-
scale ultrasound (not shown). a Intraoperative transverse gray-scale US
with a linear 15–7 MHz transducer placed directly on the pancreas shows
the 3 mm by 5 mm hypoechoic lesion at the pancreatic head–neck
junction (arrows). b Intraoperative transverse gray-scale US with a 50-
MHz transducer placed directly on the pancreas shows the lesion in better
detail. Pathological evaluation described a 6x3x3-mm focus of islet cell
hyperplasia consistent with focal neonatal hyperinsulinism
followed by fibrosis and fat infiltration. Duct dilation, calcifi-
cations and cysts also occur. On US the pancreas is small and
hyperechoic, but the gray-scale appearance does not necessar-
ily correlate with function (Fig. 10) [39, 40]. Shear wave
elastography has shown decreased pancreatic stiffness in pa-
tients with pancreatic insufficiency compared to CF patients
without insufficiency as well as healthy subjects [23].
Spleen
The spleen normally resides within the left upper quadrant of the
abdomen. It is derived from mesenchyme and composed of red
and while pulp. Red pulp consists of numerous vascular sinuses
and the white pulp is composed of lymphoid follicles and cells of
the reticuloendothelial system [41]. In the fetus, the spleen is a
site of red blood cell production. However after birth the spleen
Pediatr Radiol (2017) 47:1069–1078
Fig. 10 Pancreatic insufficiency in a 12-year-old boy with cystic fibrosis.
Gray-scale transabdominal US of the mid upper abdomen in a transverse
plane shows normal echogenicity of the liver and homogeneous
hyperechogenicity of the pancreas. L liver, P pancreas + calipers denote
the pancreatic head and X calipers denote the pancreatic body
functions mainly to remove old and damaged red blood cells
from the bloodstream [42].
The spleen has a lentiform shape and clefts and lobules may
be seen. Splenic size, as with most solid abdominal organs,
changes with the child’s growth, and normal reference values
based on age and body size are available [6, 7, 43–45]. Similar
to the liver, height is more commonly reported to correlate best
with the longitudinal measurement of the spleen and no signifi-
cant difference is seen between genders. In preterm infants, body
weight is a better correlate with length and girls tend to be smaller
than boys [44, 45]. In general, the spleen does not extend beyond
the lower margin of the left kidney. On US, splenic length is
measured from dome of the spleen to its inferior tip. A splenic
measurement of 1.25 times the left kidney length suggests
splenomegaly [46].
On US imaging the spleen has uniform, homogeneous ap-
pearance with echogenicity similar to or slightly greater than
the kidneys and slightly greater than the liver (Fig. 11) [42]. A
Fig. 11 Normal spleen in a 10-year-old boy. Transverse gray-scale
transabdominal US of the left upper quadrant shows normal
echogenicity of the spleen
Pediatr Radiol (2017) 47:1069–1078
Fig. 12 Normal spleen in a 9-month-old boy. Transverse gray-scale
transabdominal US of the left upper quadrant using a linear 12–5 MHz
transducer shows a reticular nodular appearance (arrows) of the splenic
parenchyma. The spleen size was normal
pattern of hypoechoic sub-millimeter nodules (reticulonodular
pattern) can be visualized when using high-frequency transduc-
ers (Fig. 12). When correlated with histology, the nodules were
found by one group to represent lymphoid follicles of white pulp
that increase in size up to 5 years of age [47]. This could account
for the more prominent nodular appearance in some children and
should not be mistaken for pathology. The spleen is relatively
hypervascular on color Doppler imaging. Although the spleen
normally has a smooth contour, variations can occur. The fetal
spleen is lobulated and this configuration can persist. Notches or
clefts are thought to represent residua of the grooves between
fetal lobulations and can be as deep as 3 cm [48].
Anomalies of splenic location and number occur as isolated
anomalies or associated with systemic abnormalities. The
Fig. 13 Torsed wandering spleen in a 15-year-old girl who presented
with acute abdominal pain and abdominal mass. Panoramic sagittal
gray-scale transabdominal US of the left upper quadrant with the spleen
(calipers) displaced caudal to the left kidney (K). The spleen measured up
to 20.3 cm in length. Doppler images (not shown) showed decreased
parenchymal flow on color Doppler and high resistance arterial wave
forms and poor venous drainage on spectral tracings. At laparoscopy
this was found to be a torsed spleen with narrow pedicle and no
abdominal attachments
1075
Fig. 14 A 5-month-old boy with heterotaxy and polysplenia. Gray-scale
transabdominal US of the left upper quadrant in a transverse plane shows
accessory splenules (arrows)
“wandering spleen” occurs when there are lax or absent ligamen-
tous attachments that allow the spleen to move within the abdo-
men and pelvis [49, 50]. When the spleen is in an abnormal
location it sometimes presents as a mass or with pain secondary
to torsion and vascular compromise (Fig. 13) [50]. The spleen
may also be abnormally located in children with abdominal wall
or diaphragmatic defects. Accessory spleens or splenules occur
in up to 30% of patients on autopsy and are caused by failed
fusion of the splenic anlage [47, 51]. These are usually asymp-
tomatic and should be not be mistaken for a mass or lymph node
(Fig. 14). Accessory spleens are usually less than 4 cm in size,
round and near the hilum, with a similar appearance to spleen.
Polysplenia syndrome consists of a range of abnormalities in-
cluding situs ambiguous and concomitant levoisomerism [48]
(Fig. 15). As the name suggests, splenic findings include
Fig. 15 A 9-year-old girl with heterotaxy and polysplenia. Gray-scale
transabdominal US of the right upper quadrant in a sagittal plane shows a
lobular soft-tissue structure (SP) in the right upper quadrant adjacent to
the stomach (S). This tissue was similar in echotexture to additional
splenic tissue in the right upper quadrant (not shown), consistent with
polysplenia
1076
Fig. 16 Spleen imaging in a 12-year-old boy with sickle cell SS disease
and history of hydroxyurea therapy. a Gray-scale transabdominal US of
the left upper quadrant shows increased echogenicity of the periphery of
the spleen (arrow) with multiple relatively hypoechoic round foci (star) in
the central portions. Color Doppler images (not shown) showed blood
flow within the hypoechoic foci but not in the periphery of the spleen. b
Axial CT through the upper abdomen obtained after intravenous contrast
administration shows the spleen in the left upper quadrant. There is
increased attenuation (200 Hounsfield units [HU]) of the periphery of
the splenic tissue (arrow) with normal-attenuation splenic tissue (122
HU) in the central aspect (star). The liver in comparison had an
attenuation of 105 HU. The round foci were thought to represent
residual splenic tissue while the echogenic/hyperattenuating portions
were likely infarcted tissue
numerous discrete spleens varying in size that can be seen in
either the left or right upper abdomen, but always on the same
side as the stomach [48, 52, 53]. Asplenia can also occur in cases
of situs ambiguous. These patients often have associated congen-
ital heart disease, with high mortality in the first years of life [48,
54]. If no spleen is seen on US imaging, confirmatory tests in-
cluding a nuclear medicine Tc99m-labeled denatured red cell
scan should be performed [49].
The spleen can be involved in numerous systemic
abnormalities including autoimmune disorders, portal
Pediatr Radiol (2017) 47:1069–1078
hypertension, infection, storage diseases, leukemia/
lymphoma and hemolytic anemia (sickle cell disease
and trait, hereditary spherocytosis). Acute splenic se-
questration is an emergent cause of splenomegaly in
children with sickle cell disease [49]. Patients have a
rapid drop in hemoglobin and platelets as the spleen
traps large volumes of blood, with possible hypotension
and death [55]. On US imaging, the spleen appears
enlarged and heterogeneous [49, 55]. In time, the spleen
in patients with sickle cell disease unde rgoes
autoinfarction. The final result is a small, fibrotic and
calcified spleen [55]. Previously, children with sickle
cell disease had autosplenectomy by school age. As
more are treated with blood transfusions and hydroxy-
urea, more children with sickle cell disease not only
have a spleen but also abnormalities that are associated
with these therapies [56, 57]. Splenomegaly, abnormal
echotexture and nodules likely result from congestion,
fibrosis, hemosiderin deposition and formation of
intrasplenic nodules of preserved, functioning splenic
tissue (Fig. 16) [57, 58].
Conclusion
Ultrasonography can readily assess the pediatric liver, biliary
system, pancreas and spleen. A variety of congenital and
inherited disorders affect these organs. Knowledge of their
normal sonographic appearance, growth and development
are necessary for exam interpretation.
Compliance with ethical standards
Conflicts of interest The authors have no financial interests, investiga-
tional or off-label uses to disclose.
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