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53.2.

Cross-Sectional Imaging: Ultrasonography, Computed


Tomography, and Magnetic Resonance Imaging-Utility
in the Analysis of Abdominal Masses and Other
Gastrointestinal Conditions
Karen I Norton, MD
Keith J. Benkov, MD
Robert H. Squires, Jr, MD

The subspecialty of pediatric gastroenterology substitute for a good history and physical exami- ination. In children, especially younger infants, it
began almost 30 years ago, coinciding with revo- nation, and there is probably no more important is ideal because the general lack of internal body
lutionary technological advances being made in a colleague than a trusted pediatric radiologist to fat facilitates imaging. In certain settings, US is
the eld of diagnostic imaging. Concurrent with help direct the imaging evaluation. superior to other modalities, especially for delin-
the introduction of ultrasonography (US) in the Identi cation of an abdominal mass in a child eation of cystic lesions. Generally, no sedation
early 1970s, gastrointestinal endoscopy was elicits a barrage of diagnostic considerations that is needed, and even with young infants and chil-
introduced. Rapidly following the introduction of range from benign conditions to life-threatening dren, little or no restraint is required.
US, computed tomography (CT) and then mag- malignancies. An urgent need arises to establish Technically, US uses sound above the audible
netic resonance imaging (MRI) were developed. the diagnosis, to provide an accurate prognosis range of frequencies of 20 kHz or 20,000 cycles
Theseadvances in imaging, which allow complex and to devise a treatment plan for the child and per second. In practice, diagnostic US uses a much
images to be stored, manipulated, and retrieved in family. Approximately 60% of abdominalmasses higher frequency, from l to 20 MHz (a million
microseconds, have now become routine radiol- identi ed by physical examination in child- cycles per second). These sound waves are emit-
ogy procedures rather than what might have been hood are attributable to organomegaly, with the ted from a transducer that contains crystals with
envisioned in science ction stories 30 years ago. remainder representing anomalies of develop- piezoelectric properties. When the crystals are
The detail of anatomic information gathered by ment, neoplasms, or in ammatory conditions. subjected to an electric current, they emit sound
these radiology examinations often approaches Important clues to the diagnosis include the waves at a particular frequency depending on the
what is depicted at direct surgical exploration or age of presentation (Table 1) and symptomatic size of the crystals and the current. Once the sound
pathology. Pediatric-sized endoscopes have made complaints. Conditions associated with pain or is emitted, it is directed through the body and is
direct visualization and intervention possible gastrointestinal dysfunction generally present to either refected, refracted, scattered, or absorbed,
even in very small children. It is now implausible medical attention carlier in their course, whereas depending on the properties of the tissues that
to envision practicing the subspecialty of pedi- asymptomatic masses may be well tolerated by are encountered, producing the equivalent of a
atric gastroenterology without these remarkable the child for years before clinical detection. Thus, re ected echo. This re ected echo, when returmed
complementary technologies. developmental anomalies, such as an omental to the transducer crystal, causes a vibration that
To successfully practice pediatric gastroenter- cyst, are usually present in the young infant but generates an interpretable electric pulse. The
ology requires more than casual familiarity with may not be recognized for years or even decades. returning sound beam will have a different speed
cross-šectional imaging modalitics. Although the The classi cation of abdominal masses by age and intensity from the original, which is referred to
clinical gastroenterologist is usually not required at presentation is arbitrary, and overlap is obvi- as attenuation, which depends on the properties of
to perform or independently interpret these stud- ous, but the approach is clinically useful. A large the encountered tissues. "Real-time" images can
ies,an understanding of the indications, strengths, focus has been placed on cross-sectional imag- begeneratedif rapid sound emissions are done ata
and limitations of particular investigative studies ing. It is essential however, to follow a logical, rate of at least 15 image frames persecond.
is extremely useful. In a rapidly evolving eld, it clinical approach before ordering any study, and US can also be used to determine blood ow
is sometimes dif cult to know the true bene t of a to limit exposure of children to ionizing radia- moving through vessels and structures, based on
new technique until years later, when it has been tion, given the growing awareness that diagnostic the "Doppler shift principle." Sound re ecting
fully applied and compared with other techniques. exposures may increase a child's lifetime risk of off a moving target will change in frequency,
Especially important is to know when to use these developing fatal cancer. proportional to the speed of the moving target.
often expensive technologies, which may involve The returning echo can be detected as audible
exposure to ionizing radiation, the injection of sound or as a traceable wave pattern depending
potentially nephrotoxic contrast agents, and the ULTRASONOGRAPHY on velocity. The use of color allows determina-
risks of conscious sedation or general anesthesia. tion of direction of ow, conventionally with red
Diagnostic studies are often requested by other In children, US is often the initial choice of radio- indicating ow toward the transducer and blue
inter-disciplinary colleagues, including surgery, logic investigation because it does not require ion- indicating ow away from the transducer. Some
hematology and oncology, endocrinology, urol- izing radiation, is not painful, requires little or no investigators are using contrast agents such as sta-
O8Y, and gynecology. In this regard, there is no preparation, and is a relatively inexpensive exam- bilized intravascular microbubbles to accentuate
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1432 Part VI / Diagnosis of Gastrointestinal Disorders

complete revamping of the CT protocols used in


Table 1 Abdominal Masses in lnfants and Children
pediatric studies to minimize dosage while still
Neonates Liver-cystic hepatobiliarydiscase delivering high-quality images. Newer multislice
Retroperitoncalkidney Choledochal cyst CT Scanners are now capable of better imaging
Hydronephrosis Caroli disease with less radiation exposure than older machines
Multicystic dysplastickidney Caroli syndrome and are preferable. Motion degrades the images,
Autosomal recessive polycystic kidney disease Congenital cysts usually making some form of sedation or even
Autosomal dominant polycystic kidney disease Alimentary tract
Stomach
anesthesia necessary in younger children. Intra-
Mesoblastic nephroma
Renal vein thrombosis Carcinoma venous injection of iodinated contrast is useful
Retroperitoneal-other Leiomyosarcoma in most body cases, requiring adequate venous
Adrenal abscess Rhabdomyosarcoma access. Although relatively safe, these agents can
Fetus in fetu Myosarcoma be nephrotoxic, and occasional allergic and ana-
Pelvic Fibrosarcoma phylactoid reactions are reported. Adequate inges-
Hydrometrocolpos Small bowel
tion of oral contrast, which is dif cult to obtain in
Ovarian cyst Anomalies: duplication, Meckel, malrotation
Gastrointestinal children who require sedation, increases the accu-
Lymphoma
Intestinal duplication, malrotation, obstruction Colon racy of the study. Young children and sedated
Sacrococcygcal teratoma Fecal mass subjects cannot maintain rectal contrast. The lack
Infants and Children Adenocarcinoma of internal body fat in children often makes inter-
Retroperitoneal Omentum and mesentery pretation of CT of the abdomen and pelvis chal-
Wilms tumor Cysts lenging. CT is a more expensive test than US. The
Neuroblastoma Mesenteric bromatosis
greatest advantage of CT is the high-resolution
Pancreatoblastoma In ammatory pseudotumor
Liposarcoma
images it produces, including the ability to evalu-
Rhabdomyosarcoma
Leiomyosarcoma ate air-containing structures such as bowel that
Lymphoma
Ewing sarcoma Fibrosarcoma are relatively deep to the surface and may not be
Germ cell neoplasm Mesothelioma readily palpable or accessible to US.
Liver -benign solid tumors Metastatic tumor
Adenoma Adolescents
Mesenchymal hamartoma Retroperitoneal MAGNETIC RESONANCE IMAGING
Focal nodular hyperplasia Renal cell carcinoma
Liver-malignant tumors Pelvic
This technique is based on the interaction between
Hepatoblastoma Hematocolpos
B2 Hepatocellular
carcinoma Ovarian cyst
atomic nuclei of various tissues and radio waves
Germ cell neoplasm Teratoma that are directed through the body in a strong
s Angiosarcoma Germ cell tumor magnetic eld. How the mobile nuclei are modi-
ille: Intrahepaticmesenchymaltumor Choriocarcinoma ed is labeled magnetic relaxation times (T, and
Embryonal rhabdomyosarcoma Gonadoblastoma T;), which can be measured as a change in the
Liver-vascular lesions Embryonal carcinoma
electromagnetic eld. The hydrogen atom isused
Capillary hemangioendothelioma Liver
Hepatocellular carcinoma
for imaging purposes because it is the mostabun-
Solitary cavernous hemangioma
dant nucleus with a strong magnetic signal. A
superconductor-type magnet generates the elec-
tromagnetic eld by use of large coils reducedto
detection of blood ow, but these techniques are struct an image via complex computer programs near absolute zero to offer no resistance. In the
not widely used in clinical practice. to create a cross-sectional image. Sophisticated presence of the strong magnetic eld, the ran-
US has limitations that should be addressed hardware and software advances have enabled domly oriented protons are aligned in the longi-
and understood. US cannot penetrate air-contain- nely detailed images to be displayed by a gray- tudinal axis. When subjected to a radio frequency
ing structures well, as in bowel or lungs. Struc- scale, with a spectrum from air, seen as black, in a perpendicular transverse axis, the protons are
tures underlying bowel gas may be completely to water, seen as gray, to bone, seen as white. disrupted in their alignment. Once the pulse is
obscured. US has little ability to penetrate bone, With use of the newer spiral and helical technol- discontinued, they will precess until theyresume
other calci ed structures, and metal, such as ogy, the image is generated quickly and accu- their alignment in the magnetized eld, which
sutures or plates.' The images are obtained in rately as the table moves while the scan is being can be detected as a voltage. l° The rate of return
small sectors or wedges of information, and depth done. Now with the advent of multislice CT, the to the magnetized state is the T, relaxation time
of penetration is limited, especially in older and images can be acquired so quickly that CT " uo- and the rate of decay of the transverse signal is
larger patients. The spatial relationships and reso- roscopy" to assist biopsies and catheter insertions the T, parameter.!
lution are not as great as in CT or MRI, which are possible. Vascular studies can be obtained Variations can be added to the magnetic eld
sometimes leads nonradiologists to not fully from a single rapid injection in a matter of sec- or the radio waves to highlight various featuresof
understand or trust and therefore not fully appre- onds. Traditionally, CT images are obtained in the scan. The detail afforded by MRI allows pre-
ciate the anatomic information offered by US. the axial plane, but images can be reconstructed cise characterization in different planes. With the
in any plane desired at modern workstations after injection of materials with distinctspectroscopic
8
processing. qualities, detailed images can be obtained. The
COMPUTED TOMOGRAPHY Before ordering a CT scan, the clinician most commonly used agent, gadolinium chelate, Is
should be aware of several issues related to the useful in evaluation of solid organs, andenhance-
Similar to routine radiographic studies, CT uses risks versus the bene ts of the modality. CT does ment characteristics often aid in differential diag
electromagnetic radiation to obtain images. A involve exposure to radiation, raising important nosis. With rapid injection of gadolinium, blood
well-collimated beam is passed through the sub- safety concerns that should be carefully consid- ow through various vessels can be highlighted,
ject and, depending on the characteristics of the ered, especially in children. Recent evidence pos-
producing an angiography-like study.
tissues and spaces, the X-ray will be subject to tulating an increased cancer risk in adulthood in
No ionizing radiation is involved in MRI,and
differing attenuation, making it possible to recon- children exposed to diagnostic CT² has led to from
there are no known biologic sidG viis Tects
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CHAPTER 53.2 / Cross-Sectional Imaging 1433

hisimaging modality, making it excellent for On CT, the pancreas is better de ned, with bor-
nediatricapplications. Images are, however, very ders well highlighted when the adjacent bowel is
a ectedby movement, often requiring conscious opaci ed with oral contrast and blood vessels are
sedationor anesthesia, and, at times, extended highlighted by intravenous contrast, especially
hreath-holding is needed to optimize certain if there is retroperitoneal fat. The splenic vein
acouisitions, something many young children and superior mesenteric vein can be seen poste-
cannotdo. Even peristalsis from bowel can pro- rior to the body. The lateral aspect of the head
Anceartifacts. Recent advances have reduced the is nestled by the second and thírd portions of the
acouisitiontimes for MRI substantially, making duodenum, with the third and fourth portions of
MRI a more practical modality in pediatric set- the duodenum extending inferior to the pancreas.
inos l3 MRI examinations are relatively expen- The attenuation of the pancreas on CT is less than
ie and sometimes less readily available than that of the liver. On MRI, the pancreas can also
CT. Finally, although gadolinium is generally be well displayed; however, thin-section CT is
safeand well tolerated, it should not be admin- usually easier to obtain and requires shorter study
isteredto patients in renal failure, as nephrogenic times to produce high-resolution images.
svstemic brosis/nephrogenic brosing dermopa-
thy (NSF/NFD) has been reported.l4 Gastrointestinal Tract
The gastrointestinal tract is a hollow tube that is
NORMAL APPEARANCE either uid or air lled, and the gas pattern on
AND ANATOMY AS SEEN WITH plain lm often dictates the imaging plan. On US,
CROSS-SECTIONAL IMAGING the mucosa of the bowel will appear as an echo-
Figure 1 Normal coronal magnetic resonance cholan- genic interface with the echolucent muscularis.
giopancreatogram reveals a normal extrahepatic bile duct Bowel wall thickening can be appreciated, espe-
Somebaseline ndings need to be appreciated
(arrow).
beforelooking for abnormalities on any of these cially if the bowel is uid lled. Although, more
imaging modalities. Each modality has some traditionally, the bowel is studied by upper gastro-
advantageswhen compared with the other modal- normal state is thin walled and enhances after intestinal (UGI) and small bowel follow-through
ities,and they are very often complementary. intravenous contrast on CT. On MRI, bile has (SBFT) or by barium enema (BE), the bowel can
the same signal as water but may have a higher be well studied by CT after the administration of
Liver intensity if it is concentrated in the gallbladder.!9 oral and sometimes intravenous contrast. On CT,
On heavily T,-weighted images, the entire biliary bowel wall thickening may be appreciated, and
Normal hepatic parenchyma appears homoge- tree can be imaged without the use of any con- in amed mucosa will enhance after the adminis-
neouson US, with relative increased echogenicity
trast agents, producing MRCP of striking detail tration of intravenous contrast. The lumen can be
comparedwith the renal cortex and echogenicity
(Figure 1). delineated by oral contrast, but mucosal details
similarto that of the spleen. The vessels of the
are better de ned by UGI, SBFT, or BE. In am-
liverare determined by their point of origin and Spleen matory changes in the mesentery and associated
course. Color Doppler US easily separates ves-
The normal spleen on US shows a homogeneous lymphadenopathy are well displayed. MRI is less
sels,which have slightly echogenic walls, from
echogenicity, similar to that of the liver, and lies useful at present for evaluating the bowel, but
the biliary tree. On nonenhanced CT, normal
adjacent to the left hemidiaphragm and stomach. newer MRI techniques are being investigated to
liverparenchyma is slightly higher in attenua-
The hilum is generally directed medially, and help better de ne the bowel.
tionthan the spleen, with contrasting hypodense
Vessels.With contrast, the parenchyma enhances both the splenic vein and artery are casily seen
uniformly, with vessels that become hyper- on US. CT will also show homogeneous density Genitourinary Tract
with attenuation equal to or slightly lower than
densecompared with the parenchyma. On MRI, The kidneys are easily imaged by US. The nor-
the hepatic parenchyma varies with imaging that of the liver. Rapid injection of contrast will mal neonatal kidneys are slightly echogenic and
show heterogeneous uptake initially as a mani-
Sequences,with T,-weighted images showing a may retain fetal lobulation, but by 6 months of age,
higher signal intensity than the spleen and the festation of variable owpatterns.2°On MRI, T,- the echogenicity of the normal renal cortex is usu-
reversebeing true with T,-weighted images.6 weighted images show signals of lower intensity ally slightly less echogenic than that of the liver
On previous classi cation schemata, the liver than the liver, and T,-weighted images will show and the contours are smooth. Fetal lobulation may
Wasbroken down by anatomic lobar divisions; brighter intensity. Intravenous gadolinium causes sometimes persist into adulthood. Ureters are not
a similar enhancement pattern of the spleen, as typically imaged unless dilated. MR has replaced
nowever,a more useful subdivision for the sur-
geonis based on the vessels that supply the vari- seen on CT scan. intravenous pyelograms (IVP) in the evaluation
Oussegments." The right and left hepatic ducts of renal anomalies and urograms can be obtained
Pancreas
areSeen on all cross-sectional imaging studies, without the administration ofcontrast by heavily T,
anteriorto the portal vein bifurcation. The extra- In children, the entire pancreas is usually not weighting the images. MR urography (MRU) can
hepaticducts should not measure more than 4 to well de ned on US because it lies obliquely or also be obtained after the administration of gado-
mm in diameter. In fact, the range within the transversely in the retroperitoneum and is often linium, and with multiformatting, images can be
pediatricpopulation is substantially smaller, with obscured in parts by gas in the bowel. The nor- produced at workstations in a variety of planes to
Omal ducts usually less than 4 mm.' The com- mal pancreas is of uniform, cross-hatched echo- produce high-quality images of the entire collect-
ion bile duct is important to identify and can be texture and of an cchogenicity similar to that of ing systenm, without ionizing iradiation or iodin-
SCenat the level of the pancreas on MRI or Cl. the liver, but it can also be normal and hyper- ated contrast inherent in CT or IVP examinations.
US or magnetic resonance cholangiopan- echoic or hypoechoic, especially in children. The The female gynecologic organs are well delin-
alography (MRCP), the entire course of the normal pancreatic duct, if visualized, is a 2-mm eated by US or MRI but are poorly seen on CT.
com
nmon duct can be determined, although it is or less tubular structure that runs through the pan- The imager must utilize age-dependent criteria
chnically often dif cult with US owing to over creatic body and tail. The common bile duct, as for size, shape, and appearance when determin-
ng bowelgas.The gallbladder is generally pear well as either one or two gastroduodenal arter- ing what constitutes normal or abnormal ovaries
aped atthe inferior border of the liver and in its ies within the head of the pancreas, can be seen. and uterus for age.
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1434 Part VI / Diagnosis of Gastrointestinal Disorders

ABDOMINAL MASSES IN -0
INFANTS AND CHILDREN OF
NONGASTROINTESTINAL ORIGIN

Statistically, most detectable abdominal masses


in neonates originate from the genitourinary sys-
tem. Hydronephrosis and multicystic dysplastic
kidney (MDK) make up 50 to 75% of abdominal
masses reported in the neonatal period.' Physical
ndings associated with renal masses include iso-
lated anomalies of the external ears or develop-
mental features associated with oligohydramnios,
RK SAG
such as Potter facies.
Hydronephrosis constitutes 25% of neonatal Figure 4 Sagittal image of the right kidney (upper, lef)
Figure 3 Sagittal real-time ultrasound image of the right
abdominal masses and is the most common renal renal fossa reveals multiple cysts surrounded by a small roveals a markedly echogenic enlarged kidney with loss of
mass in the newborn period.?3,2 Hydronephrosis amount of solid tissue, consistent with a MDK. The cysts do all normal sonographic architecture, typical for ARPKD.
may be due to obstruction, vesicoureteral re ux, not communicate, distinguishing it from hydronephrosis. Coronal MRCP (bottom, righ) reveals the enlarged,high
or a combination of both. It rarely is caused by water content kidneys. A focal area of biliary ectasia in the
liver is consistent with congenital hepatic brosis (arrow).
none of these factors and constitutes congen- Myoneural dysfunction, myelomeningocele, and
tial megacalyces. Dilated collecting systems are prune-belly syndrome are also associated with
more prone to infection, owing to stasis, and/or two abnormal kidneys.
increased intrapelvic pressure, which can lead US is the best initial study to evaluate the
to renal damage. Obstruction to the urinary tract upper urinary system. If hydronephrosis is pres-
occurs primarily in three locations: (1) uretero- ent, a radiographic voiding cystourethrogram can
pelvic junction, (2) ureterovesical junction, and be diagnostic for re ux or distal obstruction as in
(3) bladder outlet or urethra. PUV. MRU is recommended to evaluate complex
Ureteropelvic junction obstruction is a com- congenital anomalies of the urinary system.
mon cause of unilateral hydronephrosis (Figure 2). MDK is the most common form of cystic
In the majority of cases, the cause is unknown. disease in infants and accounts for 20% of all
When unilateral hydronephrosis is identi ed, urinary tract malformations.2$,26 Most MDK are
the contralateral kidney is often at increased risk prenatally discovered on routine obstetrical US
for developmental anomalies, including renal examinations. MDK presents as an asymptomatic
absence and multicystic dysplasia. Other con- ank mass. The kidney contains cysts of vari-
Figure 5 Coronal MRCP of a patient with ARPKD reveals
genital anomalies associated with hydronephrosis able size throughout the organ, which can be pal-
extensive beading of the extrahepatic biliary duct (arow).
include imperforate anus, spinal dysraphism, and pated as a multilobulated and immobile cystic
congenital heart disease. Bilateral hydronephro- mass (Figure 3). Other anomalies associated with
sis is frequently associated with distal obstruc- MDK include esophageal atresia, imperforate hepatic biliary disease has been described inasso-
tion, such as is seen in males with posterior ure- anus, and tracheoesophageal stula. Respiratory ciation with ARPKD (Figure 5).31
thral valves (PUV). It should be remembered, distress and gastric outlet obstruction owing to Clinicians have traditionally identi ed four
however, that PUV can present with unilateral the large abdominal mass have been described 27 classi cations of the condition, based primar-
hydronephrosis as well, when one competent Examination of the mass by US reveals the diag- ily on age and symptoms. Infants with the pre-
ureterovesical junction maintains the integrity of nostic cluster of noncommunicating cysts and a natal form, which is the most severe, are often
that side. Male children will present with a dis- variable amount of solid tissue. It is important delivered stillborn as a result of massive cystic
tended bladder, poor urinary stream, dribbling, or to evaluate the contralateral kidney as there is kidneys and pulmonary hypoplasia. Potter facies
urosepsis. Occasionally, the initial presentation a higher incidence of renal abnormalities of the (wide-set eyes, beaked nose, low-set ears, promi-
will be renal failure, failure to thrive, or urinary contralateral side, such as re ux.2 The lesion is nent fold arising from the inner canthus), usually
ascites. A de ned obstructing structural lesion is not premalignant. Although for years the treat- associated with renal agenesis, can also be seen
not the only cause of bilateral hydronephrosis. ment was surgical removal of the affected kidney in this severe form of ARPKD.!
to avoid problems with hypertension, abdomi- Autosomal dominant polycystic kidney dis-
nal pain, infection, or mass effect, it has been ease (ADPKD), formally known as "adult
demonstrated that these risks were greatly over- polycystic disease, is increasingly recognized in
estimated and in fact, many MDK completely infants, although typically the cysts do notappear
involute with time. Routine serial US is generally in abundance until adolescence or early adult-
used to follow these lesions and only those that hood." Ultrasonographic features that distinguish
grow, become infected, or are associated with the it from ARPKD include the presence of renal
development of hypertension, are removed. The cysts within the enlarged renal masses (Figure 6).
outcome is generally good when only one kidney A number of extrarenal anomalies associated
is involved 29 with ADPKD include endocardial broelastosis,
Autosomal recessive polycystic kidney disease intracerebral vascular anomalies, pyloric steno
(ARPKD), formally known as "infantile" poly- sis, and hepatic brosis.
cystic disease, has an incidence of approximately Mesoblastic nephroma is the most common
RT I to 2 per10,000births. .0 Thecondition is char- renal tumor in the neonatal period.32 It presents
acterized by bilateral renal enlargement caused by
Figure 2 Ureteropelvic Sagittal ultrasound image of the as a massive ank mass with accompanyıng
generalized dilation of the collecting tubules and is
right kidney reveals signi cant hydronephrosis with a hematurla, hypertension, and vomiting and
large conically shaped extrarenal pelvis (arrow), consis- is invariably associated with congenital hepatic with
cured by nephrectomy. Rarely, it presents
tent with a UPJ obstruction. brosis (Figure 4). Recently, predominant extra- and
atypical features such as polyhydramnio
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CHAPTER53.2 / Cross-Sectional Imaging 1435

Renal vein thrombosis (RVT) can present as Neuroblastoma is an embryonic neural crest
palpable ank mass, hematuria, thrombocytope- tumor that can arise from sympathetic nerve tis-
nia, and a consumptive coagulopathy,'* although sue from the brain to the bifurcation of the aorta,
more often, the infant presents with minimal although 65% arise from the adrenal medulla.
symptoms, such as peripheral edema or signs of The peak incidence is between 2 and 3 years of
a hypercoagulable state. RVT results in infarc- life. It most commonly presents with an abdomi-
tion of variable amounts of renal parenchyma. nal mass associated with irritability and constitu-
Factors that predispose the newborn to develop tional symptoms. It is associated with Beckwith-
RVT include hemoconcentration from dehydra- Wiedemann Syndrome, neuro bromatosis, fetal
tion, polycythemia, and low perfusion states alcohol syndrome, fetal hydantoin syndrome, and
with secondary venous congestion, local tissue Hirschsprung's disease. In 80% of cases there
swelling and hypoxia, and cellular disruption and is an abnormal short arm of chromosome 1, the
hemorrhage. Maternal factors that predispose the 1-proto-oncogene N-myc." Neuroblastoma is
Figure 6 Sagittal ultrasound of the right kidney (upper, newborn to RVT include maternal diabetes, tox- typically a poorly de ned retroperitoneal tumor
lef) of a 12 year old boy with ADPKD reveals multiple emia, and the use of medications such as steroids that spreads by direct extension across the mid-
simplecysts of variable size in a slightly enlarged kidney and thiazide diuretics during pregnancy.0 In line and metastasizes to bone, bone marrow,
forage. Cortical echogenicity remains normal. Noncon- older children, RVT is associated with nephrotic liver, lung, lymph nodes and skin. Calci ca-
trastCT (bottom, right) of another patient reveals exten-
syndrome, shock, poor cardiac output, or other tions on plain lm are seen in approximately half
sivereplacement both kidneys by multiple cysts.
causes of decreased renal perfusion. US reveals of cases. Although US is suggestive, contrast-
hypercalcemia." Although generally considered a swollen kidney with decreased echogenicity, enhanced CT or MR is needed for full assessment
benign, they occasionally recur. There are even which suggests focal or diffuse disruption of renal of extent of tumor. Calci cations are present on
rarerreports of metastasis to the brain, skeletal parenchyma. Use of duplex Doppler technology CT in approximately 90% of cases. Bone scan is
structures, lungs, and heart.23,34,35 can assess ow within the renal vein and will useful to de ne bony involvement and [l3l MIBG
detect the thrombus." Partial occlusions result scanning, which is transported and stored like a
Wilm's tumor is the most common solid child-
hood malignancy, with a peak incidence between
in abnormal arterial and venous wave forms on catecholamine, is also frequently employed prior
2 and 3 years of life. It is believed to arise from duplex Doppler US, with sharp systolic peaks and to surgery (Figure 9),37
retrograde diastolic ow seen in the main renal Ovarian cysts and neoplasms are uncommon
primitive metanephric blastemal tissue and fre-
quently presents as a large, asymptomatic unilat-
artery and its proximal branches and increased but occur in infancy, and their incidence increases
blood velocity and turbulence seen in narrowed throughout childhood. Cystic lesions are usually
eral abdominal mass. Less commonly it can pres-
ent with hematuria, hypertension, or constitutional
sections of the renal vein. Diuretic renography follicular and likely attributable to ovarian stimu-
and CT have also been used for diagnosis, how- lation by maternal hormones.' Eighty- ve percent
sysmptoms.It is usually sporadic, although familial
casesare reported. It is bilateral in approximately
ever MRI is an alternative to evaluate the patency of ovarian cystic lesions are benign. Solid lesions
of the renal vein. Gadolinium MRA/MRV is are more likely to be malignant. In the newborm,
5% of cases, with a higher incidence in familial
cases. Association with Beckwith-Wiedemann
largely replacing the usage of the more invasive ovarian lesions are displaced out of the pelvis
gold standard of venography. and present as an abdominal mass (Figure 10).
Syndrome, sporadic aniridea, hemihypertrophy,
Soto'ssyndrome, and a short arm of chromosome
Adrenal hemorrhage presents with an They sometimes arise out of the pelvis and cause
ll arereported.% Wilm's tumor spreads by direct
abdominal mass, shock, anemia, and prolonged upper GI obstruction.
jaundice, but the condition may be deceptively Hematocolpos (blood in the upper vagina) and
extensioninto the renal vein, inferior vena cava and
nghtatrium and metastasizes to the adjacent lymph
asymptomatic (Figure 8).* Differential diagno- hematometrocolpos (blood in the upper vagina
nodes,lung, liver, and rarely to bone and brain. On
sis for the physical and radiographic ndings and uterine cavity) are uncommon conditions that
in this condition would include lymphatic cyst, presents as a mass in the lower midline of the
Imaging, it usually is a well-de ned tumor aris-
Ing from the kidney. Ultrasound is typically the neuroblastoma, Wilms tumor, renal duplication, abdomen. It can be associated with a number
hrst modality employed. Careful assessment of
and hydronephrosis. Rarely, an extrapulmonary of syndromes, such as Bardet-Biedl, McKusick-
the contralateral kidney and vascular structures sequestration of the lung will mimic an adrenal Kaufman, and oral-facial-digital.o An imper-
aremandatory. Contrast-enhanced CT of the chest abscess.44 Neonatal adrenal abscess is a rare con- forate hymen is the most common cause and is
andabdomen are then performed prior to surgical dition that most likely begins with a hemorrhage revealed as a bulging hymen detected on physical
exploration
(Figure 7)" into the adrenal gland.

Kidney Len Sogtol

Figure 9 Axial contrast-enhanced CT of a 4-year old


Figure 7
contra AXial CT after the intravenous administration of male demonstrates extensive low-attenuation masses
dst in a 4-year-old boy with Wilm's tumor revcals a extending across the midline in the supra-renal retroperi-
F masscentrally within the left kidney (white arrow). Figure 8 Sagital ultrasound of a l-day-old female infant toneum. Multiple similar masses are present in the peri-
Slon oftumor into the left renal vein ispresent(black reveals a solid, nonvascularized mass (arrow) superior to the portal region and within the liver in this child with dis-
arrow).
left kidney, consistent with adrenal hemorrhage. Spleen (S). seminated neuroblastoma.
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1436 Part VI / Diagnosis of Gastrointestinal Disorders

ABDOMINAL MASSES OF children. Spontaneous regression occurs in less


GASTROINTESTINAL ORIGIN than 20% of cases and most likely occurs in con-
genital cases. Cytogenctic analysis may improve
Intestinal distention can simulate an abdominal the classi cation of discase in the future.S3,$4
mass, especially if there is an anatomic blockage. Peritoneal metastases are associated with a
Volvulus, intussusception, and other common wide variety of primary diagnoses and will havea
causes of intestinal obstruction, including meco- mass-like appearance on abdominal CT.$ Primary
nium ileus, meconium plug, and Hirschsprung tumors are rare and include liposarcoma, leiomyo-
disease, may all present with mass like ndings, sarcoma, brosarcoma, and mesothelioma,S6
but these conditions are diagnosed by plain lm An in ammatory pseudotumor is a rm, pain-
and uoroscopy and rarely by cross-sectional less, well-circumscribed, nonencapsulated mass
modalities. that commonly adheres to and in ltrates sur-
rounding viscera.57 The etiology is obscure, but
Figure 10 Coronal T, weighted fat saturated imaged after such pseudotumors are associated with an in am-
intravenous administration of gadolinium of a newborn
MASSES OF THE OMENTUM
matory response in conditions such as Hodgkin
infant female reveals a large cystic mass in the left lower AND MESENTERY
disease, Castleman discase, and peptic ulcer dis-
quandrant that proved to arise from the left ovary (arrow).
ease. There are no reports of malignant change.
Omental and mesenteric cysts likely result as a
Multiple local recurrences may plague the patient
consequence of obstructed or ectopic lymphat-
ics. The lesions are often detected on routine
following initial surgical resection.
prenatal US. When discovered in children and
young adults they are often incidental ndings, as
up to 90% of patients have minimal or no symp-
CONGENITAL HEPATOBILIARY
ANOMALIES
toms. Symptoms can vary between a vague, non-
speci c "pulling" sensation with mild abdominal
Anatomic anomalies of the hepatic lobar struc-
discomfort and fullness to an acute abdominal
ture do exist but generally are not of great clinical
crisis resulting from intestinal obstruction, volvu-
signi cance and can be evaluated by CT, US, or
lus, or rupture of the cyst. An abdominal mass is
MRI. Atresia of a speci c lobe of the liver isusu-
not always palpable but, when present, is a soft,
ally associated with hypertrophy of the remain-
thin-walled, freely mobile mass that is typically
ing lobes and can be an isolated nding or may
located within the mesentery near the terminal
Figure 11 Sagittal ultrasound of the pelvis in a 13-year-
be associated with other anomalies.$ Venous
ileum (Figure 13).
old girl with abdominal pain reveals a distended uterus anomalies most frequently involve variations in
Abdominal CT and US will demarcate the
lled with echogenic uid consistent with blood (top. the branching of the portal vein, with the only
extent of the cyst. Omental cysts should be com-
le ). Coronal MR con rmed hematometrocolpos second- real clinical signi cance reserved for the tech-
pletely resected, but recurrences are reported.0
ary to an imperforate hymen. nical aspects that arise during liver transplant.
Mesenteric bromatosis is a rare benign intraab-
Color Doppler US is probably the best modality
examination. In the absence of an imperforate dominal tumor that has an aggressive tendency
to in ltrate surrounding structures.$1.52 It is often for demonstrating most of these vascular varia-
hymen, vaginal and cervical stenosis or atresia
associated with Gardner syndrome (after colec- tions. Congenital absence of the portal vein,
should be considered in the differential diagnosis.
which can also be documented by color Doppler
Although usually initially diagnosed by US, MRI tomy), previous trauma, or prolonged estrogen
US, is rare and is associated with a multitude of
is indicated to de ne complicated anatomic prob- intake but can occur as a primary condition in the
absence of predisposing factors. Possible present-
clinical associations, including cardiac defects,
lems prior to surgical correction (Figure 11).
extrahepatic biliary atresia, and polysplenia.,60
Sacrococcygeal teratoma is the most common ing complaints include abdominal pain, a non-
tender abdominal mass, weight loss, or evidence
Also included under congenital vascular
neoplastic abdominal mass in the neonatal period.
anomalies are vascular malformations, which
It has a large external component and is usually of intestinal obstruction or perforation. The con-
dition represents 12% of the soft tissue tumors in consist mostly of hemangiomas and, less fre-
obvious at birth as most have an exophytic com-
quently, arteriovenous malformations. Both can
ponent. The vast majority are benign, but a delay
present with high-output heart failure in the new-
in diagnosis is associated with malignant trans-
born period. Infantile hemangiotheliomas and
formation (Figure 12)."7,4$ Hemangiomas can
cavernous hemangiomas are both mesenchymal
present similarly.
tumors that demonstrate a considerable amount

RK
of variability ofendothelialproliferation-hence
their potential for both growth and involution."
Hemangioendotheliomas are the more common
vascular liver tumors in infants, almost always
presenting before 6 months of age with hepa-
tomegaly and sometimes heart failure, massive
bleeding, and uncontrollable coagulopathy.
Hemangioendotheliomas can occur as solitary
lesions or as multiple hepatic lesions, and pro0
ably half are associated with cutancous heman-
Figure 13 Sagittal ultrasound (top, lef) of a 10 day-old
giomas.^ Hemangioendotheliomas (Figure 14)
infant with projectile vomiting reveals a cystic mass in
on US are predominantly hypoechoic, whereas
the right lower quadrant. A "double-wall" sign suggest-
ing enteric origin is noted anteriorly (arrow). Liver (L); cavernous hemangiomas are hyperechoic, with a
Figure 12 Axial and sagittal T,-weighted MR of a new- Right Kidney (RK), CT scan revealed the cyst and no very high-velocity ow that can be demonstrated
born infant reveals the extensive endo- and exo-phytic other abnormalities. A duplication cyst arising from the by color ow Doppler US.65 On unenhanced
components of this sacrococcygeal teratoma. terminal ileum was resected. CT scan, hemangioendotheliomas will shoW
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CHAPTER53.2 / Cross-Sectional Imaging 1437

alci cationin 40%. After the intravenous injcc- Choledochal cysts can be diagnosed at any
ion of contrast, both hemangioendothcliomas age and are classi cd by location and shape. The
and hemangiomas classically show peripheral most common are fusiform dilatation of the com-
enhancementand delayed enhancement of the mon duct (type ), but other variants, such as sac-
ntral areas (Figure 15).°* Infarction can create cular diverticulum, choledochoceles that extend
nsiderable variations in enhancement patterns. into the wall of the duodenum, and multiple cys-
Small lesions demonstrate homogeneous low tic dilatations, as in Caroli discase (Figure 20),
signalintensity on T,-weighted MRI, but larger occur.° US is the study of choice for suspected
lesionscan be more heterogeneous (Figure 16). A cases (Figures 21 and 22), with MRCP used for
T-weightedMRI will show high signal intensity con rmation and pre-preoperative de nition,
ofsmall lesions and more heterogeneity of large replacing percutaneous transhepatic cholangi-
lesions (Figures 17 and 18).67 ography and endoscopic retrograde cholangiog-
raphy (Figure 23).70 The presentation of chole-
dochal cysts during the neonatal period can be
confused with biliary atresia, in which a cystic

Figure 17 Axial T-weighted image of the liver of an


infant with disseminated hemangiomas reveals multiple
soft tissue masses in the liver (one indicated by a black
arrow) and a soft tissue mass that arises from the posterior
back (white arrow).

Figure14 Transverse sonogram of the liver of a newborn


infant with a hemangioendothelioma reveals a well-de ned
bypoechogenicmass with echogenic borders (arrowheads). L
Severalechogenic foci with associated shadowing within
themassrepresent areas of calci cation (arrow).
Figure 20 Axial contrast-enhanced computed tomo-
graphic scan of the abdomen shows multiple cystic
branching structures within the liver in this patient with
Caroli disease.

Figure 18 Axial T,-weightcd magnetic resonance image


reveals that the lesions are of bright signal intensity
(arrows).
LIVER
LOGB
RAO

FIgure15 Contrast-enhanccd axial computcd tomographic


Seanof an infant with hemangiocndothelioma reveals a large
nassin the liver with peripheral enhancement (arrow). Figure 21 Transverse sOnogran of the liver in an infant
with a choledochal cyst reveals signi cant intrahepatic
biliary dilatation, seen anterior to the portal vein (PV).

Figure 19 Routine sagittal ultrasound of the right upper


quadrant revcals a muliseptated gallbladder. The patient
was asymptomatic

Anomalies of gallbladder position and


number are more common than total agenesis,
hypoplasia, duplication, and lell-sided position
(Figure 19). Although US is still the modality
0 T,-weighted magnetic resonance image of the
have In Figure 14 revcals the large, well-de ned mass to
of choice to screen for biliary anonmalies, radio- Figure 22 Sagittal sonogram of an infant with cholestasis
1able signal characteristics. Calci cation appcars nuclide scans and MRI can also be useful tor reveals enlargement of the common duct (CD), consistent
asa signal void (arrow).
delineating anatomic abnormalities." with a clholedochal abnormality. PV = portal vein.
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1438 Part VI/ Diagnosis of Gastrointestinal Disorders

dictive rate and roughly a 90% negative predictive


rate"" for biliary atresia. MRCP (Figure 24) docs
have the advantages of not requiring pretreatment
and not using ionizing radiation," and it has been
shown to be extremely helpful in supporting the
CD diagnosis of biliary atresia in late-prescnters, in
whom the morbidity and mortality of exploratory
laparotomy are especially increased."

CONGENITAL SPLENIC ANOMALIES

The shape and position of the spleen can be quite


variable and are not of clinical signi cance. Figure 27 Axial contrast-cnhanced computed tomo-
Splenic clefts are common, as is persistent fetal graphic scan of the abdomen reveals several smallspleens
(s) on the right, inferior to the liver (L) in this patientwith
lobulation, and both can occasionally be con-
heterotaxy syndrome.
fused with lacerations. "US is generally adequate
for most examinations, but CT will yield better
Figure 23 Coronal magnetic resonance cholangiopan-
images. The orientation of the hilum can be vari- immunode ciency. Because of the complexity
creatogram of an infant with jaundice reveals marked
enlargement of the extraheptic bile duct consistent with a able, and laxity of the ligamentous attachment can and variability of the heterotaxy syndromes,US,
type I choledochal cyst. A signal void in the distal end was result in a mobile spleen. Occasionally, a wander- followed by MRI, CT, and sometimes red blood
caused by a stone (arrow). CD = common duct. ing spleen" can be confused for a neoplasm or can cell-tagged radionuclide scanning, is helpful for
result in torsion." Accessory spleens or splenules full delineation. 78 True congenital splenic cysts
remnant of the atretic biliary system can remain. (Figure 25) occur in 10% or more of healthy indi- are extremely rare, and those less than 5 cm can
Choledochal cysts in later childhood can pres- viduals and are typically near the splenic hilum."6 be managed conservatively."9
ent with signi cant obstruction, and other causes These are usually at least 2 cm in diameter to be
of biliary dilatation, such as stone, stricture, or visualized by radionuclide scanning. Although
CONGENITAL PANCREATIC
tumor, should be excluded." not clinically signi cant, accessory spleens can
ABNORMALITIES
US is often the preliminary study done when hypertrophy after splenectomy or in patients with
biliary atresia is suspected; however, the absence splenomegaly (Figure 26). Primary asplenia is not
Most congenital anomalies of the pancreas
of a normal-size gallbladder and failure to delin- common and with polysplenia falls into the cat-
involve anatomic variations of the ductalsystem.
eate the extrahepatic duct on US is suggestive but egory of situs anomalies or heterotaxy syndromes
Numerous series have reported the inadequa-
not conclusive. Documentation of the triangular (Figure 27), half of which are associated with
cies of US and CT and the need for endoscopic
cord, a periportal band of brotic tissue, both on cardiac and renal malformations and many with
retrograde cholangiopancreatography (ERCP)
US and more recently on CT," has been shown to delineate ductal anatomy. Most recently.
to correlate well with the documentation ofbiliary MRCP has proved useful in de ning pancreatic
atresia at the time of surgical exploration. There duct anatomy. Pancreas divisum (Figure 28) is
was initial enthusiasm for MRCP for evaluating a common anomaly that arguably may predis-
infants with cholestasis, but MRI is no more sen- pose the patient to pancreatitis. Hypoplasia
sitive or speci c than other modalities, including and agenesis of the pancreas have beenreported
radionuclide scanning, with a 75% positive pre- but are extremely rare. Isolated congenital cysts
and cystic disease of the pancreas areassociated
with polycystic disease of the kidneys andliver,
as well as the rare case of von Hippel-Lindau
disease (Figures 29 and 30). Annular pancreas,
which typically presents in the newborn period

Figure 25 Axial contrast-enhanced computed tomo-


graphic scan of the abdomen reveals a small, well-de ned
mass (arrow) in the splenic hilum, of identical attenuation
to normal spleen, consistent with a splenule.

STOMACH

LEEN
GB

SA L

40CE8SCORY
SPLEEN

10 20m

Figure 24 Coronal magnetic resonance cholangiopancre- Figure 26 Sagittal sonogram of the left upper quadrant Figure 28 Coronal magnetic resonancecholangiopancte
atography failed to reveal the extrahepatic biliary system of a patient with Jeune syndrome, a rare cause of hepatic atography was perfomed in this S-ycar-old female wu
in this infant evaluated for cholestasis. A cord of tissue brosis, reveals an enlarged spleen. A large mass of iden- recurrent pancreatitis. A dual drainage (arrows) stenm
was present in the anticipated location (arrow). Biliary tical echogenicity in the splenic hilum is consistent with was secn in the head of the pancreas consistent withpan
atresia was con rmed at laparotomy. an accessory spleen. creas divisum. GB = gallbladder.
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CHAPTER53.2 / Cross-Sectional Imaging 1439

TOMAGE

Fioure29 Transverse sonogram of 10-month-old male


Mih a palpable mass reveals that the pancreas is replaced
bv multiple cysts (arrow). Figure 31 Axial computed tomographic scan of the lower
chest of a S-year-old boy with a history of dysphasia Figure 34 Axial magnetic resonance cholangiopancre
reveals a cystic mass adjacent to the esophagus (arrow), atogram of a 4-month-old girl with a history of vomiting
consistent with a duplication.
and cystic mass seen on ultrasonography demonstrates
that the mass (arrow) arises in close proximity to the
lesser curvature of the stomach and appeared separate
from the gallbladder (GB), biliary system, and liver. A
gastric duplication cyst was successfully resected.

Figure 32 Contrast-enhanced multislice computed tomo-


Figure 30 Follow-up contrast-enhanced axial computed graphic angiography demonstrating an aberrant subcla-
tomographic scan of boy con rms that the pancreas is vian artery.
replacedby multiple cysts (arrow) and no other cysts were
presentin other intraabdominal organs. A diagnosis of von
Hipple-Lindaudisease was subscquently made. the detection of intraabdominal uid. Absence
of uid suggests recannalization and resolution
in utero, allowing these infants to be fed and Figure 35 Hypertrophic pyloric stenosis. Transverse
with bilious vomiting, is generally not diagnosed clinicallHy monitored. Encysted collections of sonogram of the abdomen in an infant with nonbilious
vomiting reveals both elongation of the pyloric channel
by cross-sectional imaging but rather by UGI meconium will showa heterogeneousappearance
(x) and thickening of the muscle (+), consistent with the
series.Variations of annular pancreas have been on US. Duplication and intestinal cysts, unless
diagnosis of hypertrophic pyloric stenosis.
reportedin adults and can be imaged by MRI. detected on prenatal US, generally present later
in childhood. Duplication cysts most commonly
Occur in the distal ileum and esophagus, usually ing gastric duplication cysts from cysts of biliary
CONGENITAL ABNORMALITIES OF
do not communicate with the intestinal lumen, origin (Figure 34).
THE GASTROINTESTINAL TRACT
and may or may not cause obstruction. US can Although hypertrophic pyloric stenosis does
be useful to de ne these uid- lled lesions when not usually present at birth but typically after
Numerouscongenital malformations ofthe esoph-
intraabdominal, and the presence of a double- the rst month of life, it can be classi ed under
agusand the remaining gastrointestinal tract exist,
walled sign helps distinguish gastrointestinal congenital lesions of the stomach. The condition
Including esophageal duplication cysts, which
cysts from single-walled simple mesenteric cysts results from hypertrophy of the circular muscle
may be detected on CT (Figure 31),3 Vascular
(Figure 33).$" MRCP is helpful in distinguish- of the pylorus, causing both thickening and elon-
Tings and impinging vessels, traditionally sug-
gating. The typical nding on physical exami-
gestedby UGI series and con rmed by angiogra-
nation is the palpated "olive" of hypertrophied
Phy,can now be well demonstrated by either CT
muscle. When this cannot be felt, US is the study
or MRI (Figure 32),%4 Although antral webs and
of choice to demonstrate the increased thickness
pyloric atresia can occasionally be seen on US as
and the increased length of the pyloric muscle
anintraluminal diaphragm of lincar echo density
(Figure 3S), S*
within a uid- led cavity,85 UGI or endoscopy
Temainsthe mainstay of diagnosis. High obstruc-
Uộns of the gastrointestinal tract, such as duode- CROSS-SECTIONAL IMAGING IN
a atresia, are readily suggested by a uid- led ABDOMINAL TRAUMA
"double bubble" on prenatal US but are typically
Con rmed in the newborn period by UGI and In pediatric settings, there is much less experi-
otcross-sectional imaging. Low obstruction in ence with penetrating injuries than with blunt
heonatal period may be caused by intrauter- trauma. With the increasing tendency toward
Figure 33 Sonogram of the right lower quadrant was
Ineperforation, resulting in meconium peritoni-
performed on a newborn infant because of' an abnormal
conservative, nonoperative management of
is, which calci es and is evident on plain lm. prenatal sonogram. The prescnce of a cystic mass (arrow)
abdoninal trauma, precise imaging of the abdo-
In this clinical situation, US is extremely use- was con rmed. The double wall suggests that it arises men is of the utmost importance. The liver is still
In distinguishing active peritonitis and ssur- from the bowel as opposed to a simple cyst of another the most frequently injured organ; however, in
emergency from a healed perforation by origin, such as the ovary or mesentery. 20% of cases, multiple organs are involved. In
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1440 Part VI / Diagnosis of Gastrointestinal Disorders

general, contrast-enhanced CT is the recom-


mended modality for diagnosing traumatic injury,
with US reserved for serial follow-up examina-
tions. The use of imaging is both to detect spe-
ci c injuries such as lacerations and subcapsular
hematomas and to search for peritoneal uid that
might indicate impending clinical compromise.
Parenchymal disruption to the liver can vary
from small lacerations to extensive fractures with
signi cant extravasation of blood. Lacerations
can be simple or complex and on CT (Figure 36)
appear hypodense in either a linear, round, or stel-
late shape. Subscapular hematomas (Figure 37) Figure 40 Contrast-enhanced axial computedtomographic
vary in density according to the amount of blood scan of 13-ycar-old boy who was struck by anautomobile
Figure 38 Contrast-enhanced axial computcd tomo-
loss and hematocrit, and tears in the capsule will reveals a complete transection of the pancreas (arrow),
graphic scan performed after a motor vehicle accident
allow blood to ll the peritoneal spaces. Trau- on this ll-ycar-old boy reveals a fractured spleen (thick
matic injuries to the spleen (Figure 38) are very arrow) and free ascites, presumably blood, in Morrison's
similar in CT appearance and are easily missed on pouch (thin arrow).
initial US because acute injuries may appear iso-
echoic. US should be reserved for serial examina-
tions of nown injuries (Figure 39).
Much debate has surrounded the management
of patients with blunt abdominal trauma. Most
clinicians agree that in clinical settings in which
surgical intervention is not necessary, there is lit-
tle need for follow-up CT scans." US is usually SPLEEN TR

adequate for following the evolution from poorly

Figure 41 Multiple axial images from acontrast-enhanced


CT of the abdomen in a 3-ycar-old boy with vomiting and
Figure 39 Transverse ultrasonography was used to fol- hypovolemic shock syndrome reveals extensive highatten-
low the fractured spleen serially. Six months after the ini- uation ascites, proven to be a hemoperineum. High attentu-
tial trauma, a hypoechoic area was still visualized (arrow) ation material is present in the gallbladder., consistent with
consistent with an organizing hematoma. hematobilia (arrowhead). There is intense enhancementof
both adrenal glands (arrows) and the walls of uid led
distended bowel loops. The abdominal aorta and inferior
de ned and iso- or hyperechoic to less echogenic vena cava are diminutive in caliber. Free air is present ris-
and smaller because liquefaction and organiza- ing anteriorly. A perforated jejunum secondary tononac-
tion occur over time."" Complications, such as cidental trauma was discovered at laparotomy.

L the formation of cysts, bile lakes, infarctions, and


calci cations, can also be determined by US but and SBFT, and water-soluble studies should be
are best de ned by CT with contrast. obtained if clinically suspect. Focusedabdomi-
Contrast-enhanced multidetector CT angiog- nal US for trauma has been advocated, but the
Figure 36 Axial contrast-enhanced computed tomo- raphy" has largely replaced angiography in the high false-negative rate supports the use of CT
graphic scan of the abdomen was performed in this lI- screening for arterial vascular injury, and interven- for all signi cant injuries (Figure 41)."
year-old boy after a fall. Laceration of the left lobe of the tional angiography with embolization has reduced
liver is evident (arrow). the need for surgery in a signi cant number of
cases,4 Contrast-enhanced multidetector CT can
TUMORS
be very useful in determining active hemorrhage
but is inadequate in diagnosing disruption of the Approximately 5% of all intraabdominalmasses,
benign and malignant, occur in the liver, and of
bile ducts, whereas radionucleotide scanning is
useful in diagnosing suspected bile leaks.5 all pediatric malignancies, almost 2% are primary
hepatic. S US is the preferred initial examina-
CT is the modality of choice for detecting
tion for lesions that are either purely cystic or
trauma to the pancreas, which can be manifested
typical for benign hemangiomata, seen as small,
by enlargement, edema, or complete transection
well-de ncd, echogenic masses. Serial US is
(Figure 40), as well as pseudocyst formation. US
is useful to follow pseudocysts, but pancreatic recommended to follow these benign ndings.
duct injury is best seen on MRCP.% Contrast-enhanced CT and MRI both provide
Hollow viscus gastrointestinal tract, namely further de nition of all other liver masses. C1-
99,I00
the stomach and small and large intestine, is also guided biopsies may be used for diagnosis.
L
subject to blunt trauma. CT is able to detect bowel
and mesenteric injuries in greater than 90% of MALIGNANT HEPATIC TUMORS
cases but cannot necessarily differentiate the need
Figure 37 Contrast-enhanced computed tomographic
scan of the liver of 10-year-old after a sledding accident
for surgical intervention." Free air from perfora- Hepatoblastoma is the most common primary
reveals a large subcapsular hematoma peripheral to a lac- tion is readily identi ed on CT. Bowel wall hema- malignancy of the gastrointestinal tract in chil
erated liver (arrow). tomas are sometimes more readily seen on UGI dren and is the third most common abc
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CHAPTER53.2 / Cross-Sectional Imaging 1441

Figure 45 Axial T,-weighted magnetic resonance image


ioure 42 Axial T,-weighted magnctic resonance image of a 15-year-old girl with a rm liver mass reveals a
fan ll-year-old girl wih a rm liver reveals a multi- poorly de ned mass (arrows) with a large central scar of
obulatedsolid mass within the liver (arrow), which was Figure 44 Axial contrast-enhanced computed tomo- increascd signal after the administration of gadolinium.
ubsequentlyproven to be a hepatoblastoma. graphic scan of the abdomen of a 12-year-old girl who The patient underwent a computed tomography-guided
was hepatitis B positive reveals a well-de ned mass in the biopsy that con rmed brolamellar carcinoma. Enhance-
posterior right lobe (arrows). Biopsy revealed hepatocel- ment of the central scar is more typical for focal nodular
malignancy after Wilms tumor and neuroblas-
lular carcinoma. hyperplasia.
oma." Hepatoblastoma usually presents at a
median of 1 year of age as a painless, solitary
esion, but it may be multifocal. Serum alpha- parenchyma on all imaging modalities. Small ally solitary and echogenic, with small anechoic
etoprotein is elevated in 90% of patients, and lesions are typically hyperechoic on US, whereas areas that probably represent cystic necrosis. "
metastasisoccurs in up to 20%. Plain lms wil larger lesions are generally mixed. Color Doppler Nonenhanced CT shows a well-demarcated, low-
show calci cations in up to 55%. On US, there US is useful to demonstrate low-resistance tumor attenuation mass. On contrast-enhanced CT, sep-
is usually a large, well-outlincd, predominantly ow, as well as thrombosis of the adjacent veins tations become evident. The lesion tends to be of
echogenicmass. CT will show any calci cations by tumor extension if present.02.103Nonenhanced low signal intensity on both T- and T,-weighted
present in the mass well, and with intravenous CT will show poorly de ned masses, but after MRIS, with enhancement of the tumor contrast-
contrast, there will be heterogencous enhance- the intravenous administration of contrast, HCC ing with low signal of the septa' after gado-
ment, usually related o hemorrhage and/or usually demonstrates intense enhancement sec- linium administration.
necrosis within the tumor. MRI also reveals a ondary to its hypervascular nature. Calci cations As in adults, tumors of the biliary tree are
solid mass that typically enhances irregularly are unusual. HCC is typically of low signal much less common than hepatic tumors, and
after the administration of gadolinium (Fig- intensity on T,-weighted MRI and hyperintense the prognosis is much worse. Embryonal rhab-
ures42 and 43). Calci cations are not well seen on T,-weighted imaging. Gadolinium-enhanced domyosarcoma is probably the only one in this
on MRI but rather appear as signal voids. imaging demonstrates a hypervascular tumor, category that is seen at all in children, and it
Hepatocellular carcinoma (HCC) (Figure 44) similar to a contrast-enhanced CT scan l05,106 usually presents with jaundice. US will show
ISrare in childhood and more common in older Fibrolamellar carcinoma is a very rare hepatic biliary obstruction as well as an inhomogeneous
children. It is typically associated with some pro- neoplasm but is often discussed in the differential echogenic mass, typically in the porta hepatis.
gressive,preexisting liver disease, such as chronic diagnosis of HCC, despite very different features CT shows a low-attenuation mass with some
hepatitis B, tyrosinemia, or glycogen storage and presentation. In contrast to HCC, patients enhancement with contrast, and MRCP will show
disease.HCC is more often multifocal and in l- with brolamellar carcinoma are usually symp- irregular, berry-like lling defects within the
rative. In the setting of end-stage liver disease, tomatic, have normal serum alpha-fetoprotein, do biliary system, i!!
I can be di cult to distinguish from diseased not have predisposing liver disease, and carry a Secondarymalignant tumors of the liver occur,
much poorer prognosis. The tumors are usu- most commonly from neuroblastoma, Wilms
ally solitary and lobulated, with a distinguishing tumor, lymphoma (Figure 46), and leukemia.
central brous scar that is typically hyperechoic All can be diffuse or focal. Immunosuppressed
on US and at times calci ed. On nonenhanced
CT, the lesion, and especially the central scar, is
well demarcated and of low attenuation relative
to the surounding normal liver. With the admin-
istration of intravenous contrast, the entire tumor,
but not the central scar, will cnhance. On MRI,
the lesion will appear of low signal intensity on
T-weighted images and of high signal intensity
on T,-weighted images, with failure of the central
scar to enhance after the injection of gadolinium
(Figure 45). 0% The presence of a central scar,
although helpful, is not diagnostic because it can
also be seen in focal nodular hyperplasia (FNH)
and giant hemangiomas.
The fourth most common pediatric hepatic
Figure 43 tumor is the undifferentiated embryonal sar-
T,-weighted axial magnetic resonance image Figure 46 Axial contrast-enhanced computed tomo-
coma, which may be dif cult to differentiate
bdomen of an infant reveals a large, well-de ned graphic scan of the abdomen of 6-ycar-old female patient
from a mesenchymal hamartoma. Unlike the
ma (arrow) with a central arca of high signal, which with night sweats reveals multiple low-attenuation masses
Was
he oven to represent a hepatoblastoma with central latter, embryonal sarcoma o en presents with within the liver. Bone marrow biopsy was consistent with
hemorrhage. symptoms of pain or mass. On US, it is usu- B-cell lynmphoma.
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1442 Part VI / Diagnosis of Gastrointestinal Disorders

patients can develop posttransplant lymphopro- (Figure 51) and MRI (Figure 52)."" Occasionally,
liferative disease (PTLD) with various lesions mesenchymal hamartomas present as purely cystic
that range from polyclonal B-cell hyperplasia to lesions (Figure 53). Scrial US examinations aid
malignant lymphoma. Hepatic metastases from in distinguishing simple liver cysts (Figures 54
other tumors occur rather late in the disease pro- and 55) from cystic hamartoma; the latter tend to
cess and carry a poor prognosis. The value of US grow and change in appearance, and may develop
in detecting lesions depends on the primary tumor more solid components with time."9
and the pattern of metastasis, and even the pres-
ence of hepatomegaly is not useful.12 CT with
contrast can be more useful but may require dual-
phase arterial and venous scanning for full evalu-
ation. MRI with and without gadolinium is also
sensitive for detection of focal liver lesions.
LIVER TR
Figure 48 After the intravenous administration of gado-
linium, there is intense enhancement of the mass (arrow)
BENIGN HEPATIC TUMORS but not the central scar, a nding suggestive of brola-
mellar carcinoma rather than focal nodular hyperplasia.
FNH is an uncommon epithelial lesion in child- Alpha-fetoprotein remained normal, and the mass has
hood, more often seen in middle-aged women. remained stable in size for 5 years, ndings more sugges-

In children, it is usually an incidental nding, as tive of focal nodularhyperplasia.


a solitary lesion of less than 5 cm in size, with-
tocellular adenomas are also reported in asso-
out internal hemorrhage or necrosis. The distin-
ciation with glycogen storage disease type la Figure 50 Sonogram of the liver of a newborn with an
guishing feature is a central brous scar, which is
and diabetes. In 80% of cases, hepatocellular enlarged liver reveals a complex cyst within the liver, con-
vascular and extends outward into brous septa.
adenoma is a solitary, well-circumscribed, and taining multiple septations. It was resected, and histopa-
On US, FNH is a well-demarcated, either iso- or thology revealed a cystic hamartoma.
encapsulated mass. The US appearance can be
hyperechoic lesion, with a central scar demon-
variable, depending on the presence of lipid or
strated in 20% of cases. Associated calci cations internal hemorrhage. On nonenhanced CT, the
are rare enough to suggest another pathology.!13
lesions are generally of decreased attenuation,
On nonenhanced CT, the lesion appears as a
with 509% containing calci cations (Figure 49).
hypo- or isodense lesion that enhances after the
After the injection of intravenous contrast, feed-
administration of contrast but becomes isodense ing vessels may be identi ed. 15 The lesions have
on delayed scans. The central scar in FNH will
a variable appearance on MRI. Gadolinium is
enhance in 70% of cases, helping to distinguish useful to identify feeding vessels.6 Serial US
FNH from brolamellar carcinoma, in which the
examinations, in conjunction with monitoring the
scar typically does not enhance. On T,-weighted serum alpha-fetoprotein level, are recommended
MRIS, FNH appears as a lesion of decreased or
to screen for malignant degeneration, seen as
isointensity, and on T,-weighted images, the sudden growth, necrosis, ora change to a hetero-
signal intensity increases. After the administra-
geneous appearance.17
tion of gadolinium, over 80% of cases will show Mesenchymal hamartomas, like most hamar-
enhancement of the central scar, again distin- tomas, should probably be considered as develop- Figure 51 Contrast-enhanced computed tomographic
guishing FNH from brolamellar carcinoma mental anomalies rather than true neoplasms. They scan of the liver demonstrates that a large portion of the
(Figures 47 and 48).
contain gelatinous serous uid in cystic spaces, liver has been replaced by cystic and solid tumor. Periph-
Hepatocellular adenoma is a rare epithelial intermixed with biliary ductal and connective tis- eral enhancement was seen. The patient underwent a
tumor with an increasing incidence secondary three-quadrant resection for this hamartoma. The arrow
sue. On US, mesenchymal hamartomas are pre-
to oral contraceptive and androgen use. Hepa- demonstrates the cystic hamartoma within normal liver
dominantly cystic, with multiple echogenic septa
parenchyma.
(Figure 50). Their appearance is similar on CT scan

Figure 47 Axial magnetic resonance image of a 14-year Figure 52 T,-weighted axial magnetic resonance image of
old asymptomatic girl followed for a liver mass discov- Figure 49 Noncontrast-enlhanced computed tomographic the abdomen of this infant with an enlarged liver reveals a
ered incidentally during renal ultrasonography shows scan of 8-year-old girl with type IV glycogen storage large cystic mass (arrow). It was unroofed, but pathology
well-de ncd low signal lesion (arrow) with a central scar. disease reveals a large low-attenuation mass within the was consistent with a cystic hamartoma rather than a simple
Differential diagnosis was brolamellar carcinoma versus liver. A central calci cation (arrow) was present. It was liver cyst. It regrew with more solid componcnts, and the
focal nodular hyperplasia. resectcd, and the diagnosis of giant adenoma was made. patient underwent a successful wide resection of this tumor.
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CHAPTER53.2 / Cross-Sectional Imaging 1443

SPLENIC TUMORS

Splenic tumors are in general rare and can be


divided between cystic lesions and solid neopla-
sia. Cysts are classi ed as true cysts, pseudocysts,
and parasitic cysts. True cysts (Figures 56 and
57) have an epithelial lining and are sometimes
termed congenital epidermoids (Figure 58). They
may contain brightly re ective cchoes within them
from oating crystals. Pseudocysts, which contain
no epithelial lining, are believed to be secondary
to infection, trauma, or infarction and cannot be
distinguished from true cysts radiologically. Some
Fieure 53 Axial contrast-cnhanced computed tomo-
neoplasms, such as lymphangioma, hemangioma,
oraphicscan of the abdomen of a 5-year old with right
upperquadrant pain reveals a multilobulated cystic mass Figure 59 Axial T,-weighted magnetic resonance image
within the liver, which was subsequently proven to be a of the abdomen revcals a solid mass with a central scar
mesenchymal hamartoma. arising from the spleen, which was subsequently proven to
be a hamartoma (arrow).

and hamartoma, may appear largely cystic on US.


Septations and rim calci cations may be pres-
ent.20 Hemorrhage or protein content can increase
the attenuation on CT. MRI will also demonstrate
LIVER
true solid lesions (Figure 59).
Splenic involvement by lymphoma and leu-
kemia presents in several ways. On US, homoge-
neousenlargement with normal echogenicity, sol-
itary or multifocal hypoechoic lesions, or diffuse
Figure 54 Transverse sonogram of the liver of an Figure 56 Sagittal sonogram of the left upper quadrant in ltration with heterogeneous echogenicity can
8-month-old girl shows a multilobulated cyst within the reveals a simple cyst (arrow) arising from the spleen (SP). be seen. CT and MRI demonstrate similar nd-
liverabove the portal vein (PV). ings, although MRI may be less useful because
lymphomatous tissue can have signal intensity
similar to that of normal splenic tissue.2

PANCREATIC TUMORS

Pancreatic tumors are a very small percentage


of pediatric intraabdominal tumors and usually
present with either a mass, abdominal distention,
or a variety of endocrine abnormalities. Atypi-
cally, they present with the more usual adult pre-
sentation of bowel obstruction, weight loss, or
jaundice.2l These tumors can be classi ed into
Figure 57 Non-contrast-cnhanced computed tomo- nonfunctioning and functioning tumors, as well
graphic scan demonstrates a large cyst arising from the as malignant or benign tumors.2 Pancreato-
GB spleen (arrow). blastomas usually occur before 8 years of age
as large, well-de ned masses, with both cystic
and solid components arising from the body or
tail. Although pancreatoblastomas can be seen
on US, CT is better at fully de ning these tumors
and demonstrating any associated calci ca-
tions.2 Solid and papillary epithelial neoplasm
Figure 55 Follow-up coronal magnetic resonance chol- is a rare, low-grade malignancy that typically
ngiopancreatogram con rms that the cysts (long arrow) occurs in young adult women, but a third of
are within the liver and separate from the biliary system. cases are described in adolescents. These tumors
Ine normal extrahepatic duct is demarcated by the short typically have cystic areas, as well as areas of
arrow. A portion of the gallbladder (GB) is also seen. hemorrhage and calei cation, producing a very
heterogeneous appearance on imaging studies
Related and more common entities are mes- (Figure 60). 124 Acute lymphocytic leukeumia is
Cnchymal tumors, which include hemangionmas, a rare cause of diftuse pancreatic intiltration and
nemangioendotheliomas, and lipomas. The rst enlargement (Figure 6),2$ Thin-section con-
WOhave been discussed in part above. Cavernous trast-enhanced CT is the best modality to image
emangiomas are more commonly seen in adults Figure 58 Sagittal sonogram of the spleen reveals a cen- the pancreas because it is unaftected by overly-
an inchildren. Lipomas. benign masses ofmature tral cyst with particular cchoes dispersed throughout. This ing bowel gas, which can obscure the pancreas
adiposetissue, are rarely seen in childret is a typical appearance for an epidermoid cyst (arrow). on US. Functioning pancreatic tumors, including
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|444 Part VI DragnosIs of Gastontestmal Dısorders

children, those with human immunode ciency


virus (HIV), chronic granulomatous disease.
or leukemia or on immunosuppression therapy
following organ transplant or for treatment of
diseases such as in ammatory bowel disease
(IBD). US is an excellent initial study and a use.
ful guide for diagnostic biopsy. Fungalabscesses
are usually small and multiple and appear on
US as target" lesions, with a central echogenic
focus surrounded by a hypochoic rim. On con-
trast-enhanced CT, the central area enhances
within the low-attenuation lesions. "2 Bacterial
abscesses tend to be singular, round, and located
Figure 60 Contrast-enhanccd axial computed tomo- Figure 62 Contrast-enhanced axial CT ofa 14 year of girl
in the periphery. A hypoechoic halo may be
graphic scan of the abdomen of a 13-year-old girl with seen on US.3* Similarly on CT and MRI, pyo-
with abdonminal pain reveals a multilobular mass (arrows)
abdominal pain reveals a solid tumor in the head of the
surrounding the stomach and narrowing the lumen. Biopsy genic abscesses are well-de ned, low-density
pancreas (arrow). The patient underwent a modi ed
revealed a Burkitt's variant tumor. lesions that demonstrate peripheral enhancement
Whipple procedure for what proved to be a cystadenonma.
after contrast administration. 3* Staphylococ-
cal abscesses can also present as target lesions.
Amebic or hydatid abscesses can be dif cult to
differentiate from pyogenic abscesses.35Echino-
coccosis is endemic in certain parts of the world,
TX PANCR and two-thirds of cases will involve only the
liver. US, CT, or MRI will show either single or
a few very large cysts, sometimes with calci ca-
tions (Figure 65). Perforation into the biliary tree
has been reported. 136
In general, most children with acute hepatitis
have a normal US examination, although diffuse
hepatomegaly may be present and echogenicity
may be cither increased or decreased. l57 Thicken-
Figure 63 Axial contrast-enhanced computed tomo-
graphic scan of the lower abdomen of a l4-year-old boy ing of the gallbladder wall is sometimes seen in
Figure 61 Transverse sonogram of a 3-week-old female
infant showed marked enlargement of the pancreas. The who underwent a total colcctomy for familial polypo- acute hepatitis as well as a contraction of the total
echotexture appears decreased. The patient was diagnosed sis demonstrates bulky soft tissue masses (arrows). The volume of the gallbladder. l$ In certainsetings,
with acute lymphocytic leukemia. Arrows indicate the patient wvas diagnosed with Gardner syndrome and des- as with ingestion of a fatty meal, paradoxical
head of the pancreas. moid tumors. dilatation of the gallbladder can also be seenon
US.13 CT and MRI will show similarnonspeci c
changes. Assorted neonatal infections, including
insulinoma, gastrinoma, and VIPoma, are notori-
cytomegalovirus, toxoplasmosis, and coxsacki-
ously dif cult to image, especially because these
evirus, have been associated with parenchymal
lesions may be very small. Gadolinium-enhanced
liver calci cations. Interestingly, infections in
MRI, which can depict subtle changes in tissue
planes, may be of bene t in the detection of small older children rarely result in calci cations. 14
126 Splenic abscesses appear hypoechoic on US,
tumors.
with low attenuation on CT and with high uid
content on MRI. Peripheral enhancement after
GASTROINTESTINAL TUMORS contrast administration is seen on CT and MRI.
Fungal abscess may be very small, multiple, and
Primary malignancies such as adenocarcinoma, occasionally calci ed and is seen better on CT
lymphoma, and sarcoma are rarely reported in
childhood. Cross-sectional imaging is most use-
ful for staging to determine the extent of tumor Figure 64 Axial contrast-enhanced computed tomo-
graphic scan of the pelvis of 9-ycar-old female heart
and the presence of metastatic disease. In general,
recipient on immunosuppression shows a large soft tissue
intraabdominal lymph nodes in excess of I cm
mass encasing loops of bowel (arrow). Biopsy con rmed
should raise suspicion.2" Primary lymphoma is the diagnosis of monoclonal lymphoproliferative discase. STONACH
rare, occurring most commonly in the ileocecal
region, followed by gastric origin.2% Polyps can
organs, or lymph nodes. US and CT connbined
sometimes be detected on US as incidental nd-
will detect up to three-quarters of cases of PTLD
ings (Figure 62). The use of compression US in
(Figure 64), 31
unprepared patients can be useful. Juvenile pol-
yps may appear almost cystic on US but vascular
on color ow Doppler US.9 Desmoid tumors, INFECTIOUS PROCESSES
encountered in association with familial polypo-
sis, are not malignant but can be quite aggressive The most common infectious processes that
affect the liver are abscesses and hepatitis. Fun- Figure 65 Contrast-enhanced computed tomogt«
aphic
(Figure 63)30 PTLD can present in the immuno-
scan of a 14-year-old boy reveals recurrent cysticmaSs
suppressed patient as nonspeci c hepatospleno- gal, bacterial, or other infectious abscesses are
(arrow) arising anterior to multiple clips, status postiresec-
megaly or focal masses within the bowel wall, more commonly seen in immunocompromised tion of ccchinococcalcysts.
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CHAPTER 53.2 / Cross-Sectional Imaging 1445

LIVER

RK TX

SPLEEN

Figure 67 Transverse sonogram of the right upper quad-


rant shows the liver to be of increased echogenicity com-
pared with the right kidney, suggesting fatty in ltration.
Fjgure66 Axial computed tomography of a 16-year-old-
Normally, the liver is of decreased attenuation compared
bov with ALL and right lower quadrant pain reveals a
with the kidney.
multiloculatedabscess (arrow) which extends into the ile-
Figure 70 Axial contrast-enhanced computed tomo-
onsoasmuscle. A ruptured appendicitis was the etiology. graphic scan of the abdomen of a l6-year-old female
patient with autoimmune hepatitis reveals splenomegaly,
scan.
41,142The spleen is subject to opportunistic varices (arrow), and the inferior portion of a small liver.
infections, including Pneumocystis and Myco- Clinically, the patient had portal hypertension.
bacterium, especially in immunocompromised
patients l43
Primary pancreatic abscesses do not occur, portal hypertension, with splenomegaly, varices
butthere can be secondary infection of pseudo- (Figure 70), and ascites. Recannalization of the
cysts,usually occurring 4 to 6 weeks after acute umbilical vein, hypertrophy of the hepatic artery
pancreatitisand appearing as well-circumscribed signal, and biphasic and eventual reversal of ow
complex cysts on imaging studies. This occur- A in the portal vein are demonstrated by color Dop-
rence usually requires surgical debridement. 144 pler US. Enlarged coronary veins and retroperito-
Intraabdominal abscesses occur with some neal varices can be seen with portal vein throm-
frequency in children and are usually second- bosis and cavernous transformation. CT and MRI
ary to ruptured appendicitis. They can also be Figure 68 Axial contrast-enhanced computcd tomo- can show collaterals and clots extending into
secondaryto trauma and Crohn's disease.14s US graphic scan of the abdomen of 12-year-old with cystic vessels.s Increased attenuation of parenchyma
has limited usefulness in searching for intraab- brosis reveals that the liver has lower attenuation to can be seen with iron deposition, with glycogen
dominalabscess, and contrast-enhanced CT of the spleen rather than the normally expected similar or storage disease, and after the administration of
theabdomen and pelvis with both oral and rectal increased attenuation, a nding suggestive of fatty in ltra- cisplatinum for chemotherapy.49 MRI may be
tion. The spleen is mildly enlarged.
contrastis the modality of choice (Figure 66). more sensitive than CT for detecting early depo-
Various bacterial enteritides, including Cam- sition. Because deposition can be preceded by the
pylobacterjejuni and Yersinia enterocolitica, can cirrhosis. Such ndings include macronodular accumulation of fat, the liver may initially appear
alsocause thickening and in ammation of the contours, regenerating relative enlargement of of decreased attenuation on CT.!50
bowel wall, especially in the ileum, and may be the caudate and left lobes, 4 and sclerotic nod- Budd-Chiari syndrome is a relatively rare
associatedwith mesenteric adenitis, making them ules (Figure 69), as well as the development of disorder caused by the acute or chronic obstruc-
difcult to distinguish from Crohn's disease." tion of the hepatic veins, usually by membranous
obstruction. It may also be caused by hyperco-
agulability associated with hematologic and sys-
ACQUIRED CONDITIONS
temic disease. The presentation of Budd-Chiari
Liver Disease
syndrome is similar to other liver diseases, with
right upper quadrant pain, tender hepatomegaly,
Parenchymal liver disease from various pro- and possibly ascites. Astute clinical suspicion
Cesses,including metabolic diseases, drug and and proper imaging are essential for diagnosis,
environmental toxicities, and congenital hepatic Angiography had been the principal diagnostic
nbrosis,does not offer speci c imaging ndings. modality; however, noninvasive duplex Doppler
Someof theseprocesses cause fatty in ltration, US is now preferred to show the venous anoma-
brosis,andeven cirrhosis, all of which have fur- lies, with a sensitivity that approachess angiogra-
i imagingndings,albeitnonspeci c.Fatty phy. !5! Contrast-enhanced CT and MRI do not
Inhltration with enlargement of the organ is a demonstrate venous obstruction as well but do
COmmon nding in early liver disease, seen on detect the associated parenchymal disease, the
USas diffuse increased echogenicity (Figure 67). relative preservation of the caudate lobe, and
UnCT, the liver with fatty in ltration has lower Figure 69 Spoiled gradiant axial magnetic resonance large regenerative nodules (Figures 71 and 72).!52
atenuation to the spleen rather than the or- image of the liver in 17-year-old male patient with chronic Small venule veno-0cclusive disease (VOD) is
Y expected similar or increased attenuation active hepatitis B demonstrates multiple nodules within also seen as a complication of treatment of hema-
the liver. Most were of low signal characteristic and did tological malignancies. Duplex Doppler US may
ameo8). As liver parenchyma is destroyed
not enhance after gadolinium, similar to the nodule demar-
d tneorgan becomes brotic, there is a com- detect decreased portal venous ow secondary
cated by the thin arrow. These were presumed to represent
ensurate decrease in size and a heterogeneous siderotic type nodules. One lesion was of bright signal on
to increased vascular resistance within the liver.
Crease in echogenicity on US. multiple sequences but also did not enhance (short arrow) In more severe VOD, there may be pulsatile
OS, CT, or MRI can also demonstrate and was thought to represent a regenerating nodule. All reversed ow in the portal vein and diminished
nges associatedwith chronic liver diseaseand nodules remaincd stable over a 3-ycar follow-up, systolic arterial ow in the hepatic artery.
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1446 Part VI / Diagnosis of Gastrointestinal Disorders

Figure 74 Acute cholecystitis. Sagittal sonogram of the


gallbladder revcals a thick wall and a scant amount of
Figure 71 Budd-Chiari syndrome. Axial computed Figure 73 Axial magnetic resonance cholangiopancrea- percholecystic uid (arrow).
tomographic scan after the intravenous administration of tography performed on a year-old girl with rising liver
contrast reveals heterogeneous enhancement of the liver, enzymes 5 years following a segmented liver transplant
except for the caudate lobe (c), which appears preserved. reveals mild intrahepatic biliary dilatation and beading
(arrow).

promptly and corrected in the immediate post-


operative period, can signi cantly improve graft GB TX
success and decrease the incidence of biliary
compromise. Bile duct dilatation can be related
to vascular compromise or anastomotic stric-
ture. Dilatation or beading of the biliary system
on a chronic basis may also be related to pro-
longed cold-ischemia at the time of transplant.
Biliary dilatation will be seen on US or CT if Figure 75 Transverse sonogram of the liver of a16-year-
moderate to severel5 and on MRCP even if mild old female paticnt with right upper quadrant painreveals
(Figure 73). Small focal areas of infarction are a thick-walled, contracted gallbladder (arrow) lled
not uncommon in the immediate postoperative with shadowing stones. The patient was treated foracute
Figure 72 Delayed axial computed tomographic scan period and can be detected on US as hypoechoic, cholecystitis.
after the intravenous administration of contrast reveals
peripheral, wedge-shaped areas of decreased
ling defects in the liver corresponding to thrombosed
hepatic veins (short arrow) in this 15-year-old patient perfusion but may be more evident on CT scan,
with Budd-Chiari syndrome. The long arrow demonstrates where they appear to be of decreased attenua-
ascitic uid surrounding the liver. tion. 50 US-guided biopsies and interventional
percutaneous transhepatic drainage of bilomas
or abscesses play an important role in the post-
surgical management.Contrast-cnhanced CT of
Liver Transplant the abdomen and pelvis remains the mainstay
The most signi cant and dramatic acquired con- of evaluating for post-transplantabscess, collec-
dition of the liver is transplant. Accurate imag- tions, and PTLD.
ing is crucial for both pre- and posttransplant
evaluations. Potential recipients are typically fol-
lowed by serial US examinations to document BILIARY DISEASE
progression of disease, the development of portal
hypertension, and the patency of vessels essential The most common form of acquired biliary Figure 76 Sagittal sonogram of the porta hepatisreveals
for graft anastomosis. Congenital anomalies that disease is cholelithiasis, and although the inci- an cchogenic focus within the common duct (arrow), con-
might complicate the surgical procedure, such dence is not as common as in adults, it does sistent with a stone.
as a preduodenal portal vein, are documented. occur with some frequency in children, espe-
CT and MRI are reserved for excluding the pos- cially in the setting of sickle cell disease, hercdi- although hypoalbuminemia and infections may
sibility of malignancyl* and establishing accu- tary spherocytosis, and related hematologic dis- cause sinmilar ndings,69
rate liver volume for surgical planning. MRCP, eases (Figure 74). In children, both gallstones Primary sclerosing cholangitis, a chronic
in conjunction with liver biopsy, is particularly and sludge can be well seen on US (Figure 75), in ammatory process affecting the extrahepatic
important in excluding large duct primary scle- although the clinical signi cance of each is dif- and/or medium to large bile ducts, is seen in
rosing cholangitis as the cause of liver failure ferent. SIludge is more often associated with children and has features suggesting an autom1-
because this diagnosis requires that a Roux-en- prior parenteral nutrition or antibiotics and can mune pathogenesis, It is associated with IBD,
Y portoenterostomy be performed rather than a appear and resolve in a matter of days. Most histiocytosis, and, less commonly, lymphoma
duct-to-duct anastomosis. stones are related to hemolytic processes and, and infections related to immunode ciencies.159
Serial US examinations are crucial in the unlike in adults, do not necessarily require sur- MKCP Is as sensitive as ERCP in demonstrat-
postoperative period to access for patency of gery.'S$ Common duct stones are rare in chil- ing large duct primary sclerosing cholangitis
in

the newly anastomosed vessels. Vascular com- dren (Figure 76). Acalculous cholecystitis may Chilaren by demonstrating beading of the ducts
plications of stenosis or occlusion, if diagnosed show thickening of the gallbladder wall on US, (Figure 77), l60
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CHAPTER53.2 / Cross-Sectional Imaging 1447

Pancreatic Disease

Of the identi able causes of pancreatitis, trauma


is the most common in childhood, and abuse
should always be excluded. Nontraumatic pan-
creatitis may be caused by viral infection, hyper-
cholesterolemia, and obstruction. Obstruction
as an etiology is much less common in children
than in adults. It may appear as focal or dif-
fuse enlargement, which is sometimes dif cult
to de ne because there is great variability in the
size of the normal pancreas. US is an excellent
RK modality for the initial study; however, owing to
Figure 81 Axial magnetic resonance cholangiopancrea-
obscuring bowel gas, demonstration of the entire
togram of a 5-ycar-old boy with a history of abuse demon-
pancreas can present challenges. Associated nd- strates a large pseudocyst in the pancreas (arrow).
ings of acute pancreatitis include ductal enlarge-
ment, either decreasced or increased echogenicity
of the entire gland, or heterogeneous echogenicity
within the same gland. Peripancreatic uid can
Figure 77 Coronal magnctic resonance cholangiopan-
creatogramof an 8-year-old boy with autosomal dominant be detected loculated adjacent to the pancreas or
poly-cystic kidney discase reveals dilatation and beading free in the peritoneal space as ascites and pleural
ofthe extra-hepatic bile duct (arrow), as well as milder effusions (Figure 79). Phlegmons, stulae, infarc-
intrahepatic biliary beading, suggesting the appearance tions, and pseudocysts can complicate pancre-
ofprimary sclerosing cholangitis. Note the multicystic atitis. CT with contrast has been used to evaluate
enlargedkidncys and splenomegaly.
pancreatic morphology, detect pancreatic necro-
sis, and depict retroperitoneal complications,
resulting in a CT staging severity index that has
SPLENIC DISEASE
proven to be a reliable indicator of prognosis (Fig-
ure 80).6s In children with recurrent episodes of
Thespleen is not usually the primary organ of
idiopathic acute pancreatitis, MRCP allows visu- Figure 82 Axial contrast-enhanced computed tomo-
diseaseprocesses, but it is frequently second-
alization of the pancreatic duct anatomy, includ- graphic scan of 16-year-old female patient with cystic
arily enlarged by neoplasia, infection, venous
ing side branches, ductal narrowing, cndoluminal brosis reveals complete fatty replacement of the pan-
engorgementfrom portal hypertension, or in l-
lling defects, irregular ductal contour, cavities, creas (arrow). The patient clinically had insulin-depen-
trative disease as in storage diseases. Gaucher dent diabetes.
diseaseis associated with some of the largest
documentedspleens (Figure 78). On all modali-
ties, the parenchyma is usually
uniform. Com- and pancreas divisum, thus increasing the ability
to diagnose risk factors associated with chronic
plicating splenomegaly, infarction appears as
hypoperfused,wedge-shaped, round, or irregular pancreatitis (Figure 81),64 Chronic pancreatitis,
areason US and as low-attenuation areas on con-
relatively rare in childhood, shows ductal dilata-
trast-enhanced CT unless superimposed hemor- tion, irregularity, calci cation, pseudo-cyst for-
rhage causes an increase in attenuation. When mation, and, ultimately, a smaller gland. In cystic
followed serially, infarcts tend to contract and brosis, pancreatic atrophy and fatty in ltration
progressively liquefy, and may calcify. In sickle may be dramatic (Figure 82), e Diffuse enlarge-
celldisease, multiple, recurrent infarctions result ment or focal alteration of the gland is seen in
Ineventual destruction of the entire spleen.' brosing pancreatitis and may result in biliary
obstruction, l66

Figure 79 Transverse sonogram of the abdomen reveals Disorders of the Gastrointestinal Tract
an enlarged head ofthec pancreas with a central pseudocyst
Cross-sectional imaging of the gastrointestinal
(arrow)
tract has dramatically altered the standard of care
in gastrointestinal disorders over the past several
decades. Various imaging studies are routinely
ordered for diagnosing entities such as appen-
dicitis, intussusception, IBD, and even bowel
obstruction, although plain lms and contrast
studies, coupled with clinical judgment, still play
a very large role in the diagnosis and manage-
ment of these disorders.
Up to 20% of small bowel obstruction will
not be evident on plain lms, but it is unclear
whether these undetected cases can be managed
78 Axial
noncontrast-enhanced
computedtomo- conservatively or indeed need surgical man-
8Taphicscan of the abdomen of a 13-year-old female
Figure 80 Axial contrast-enhanced computed tomo- agement. Contrast studies are at times not use-
ent with Gaucher disease demonstrates marked sple-
Omegaly with multiple peripheral low-attenuation arcas graphic scan of a 12-year-old boy with familial hypercho- ful owing to delayed transit time and dilution
scnt with infarcts (arrow), A thin calci ed rim is lesterolemia and pancreatitis revcals an enlarged head of of contrast. US examination of small bowcl
present.Free ascites (A) is also preset the pancreas and a central pscudocyst (arrosw). obstruction will demonstrate either hyperactive
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1448 Part VI / Diagnosis of Gastrointestinal Disorders

or hypoactive, atonic and dilated, uid- lled in the typical age group, 90% are ileocolic and
bowel, but the same ndings can be seen in gas- probably only 5% have a lead point, whereas the
troenteritis. lo US may suggest malrotation based occurrence under the age of6 months and over
on demonstrating the superior mesenteric vein to 3 years of age can be located in other segments
the left of the superior mesenteric artery.' CT of the bowel and may have a lead point.IM Not
can reveal high-grade obstruction with dilated all children will have the typical clinical presen-
loops of bowel and closed-loop strangulating tation of episodic cramps, currant jelly stools,
obstruction, and some authors advocate CT as the and abdominal pain, making it most important
investigative modality of choice. Speci c causes to maintain a high clinical suspicion and have
of small bowel obstruction can be seen on cross- sensitive imaging techniques to con rm. Find-
sectional imaging studies, although traditional ings of intussusception may be evident on plain
SBFT remains helpful, l67 lm radiographs, but in 25% of cases, the bowel
Causes of obstruction include congenital pattern is normal. Enema, using either barium or
duplication and mesenteric cysts. If the cyst con- Figure 83 Axial cóntrast-enhanced computed tomo- air, is the gold standard, both for diagnosis and
tains gastric mucosa, it can ulcerate, bleed, or even graphic scan with oral and rectal contrast reveals a uid- treatment.
perforate.'6s Another cause of intestinal obstruc- lled, swollen appendix in the right lower quandrant US is a reliable study for diagnosing intussus-
tion is Meckel diverticulum, which can contain (arrow). The in ammed appendix wall is enhancing. ception, although it may not reach the high sen-
gastric mucosa and therefore can be detected by sitivity and 100% negative predictive values that
radionucleotide scan. Occasionally, the diverticu- have been reported. 178 The characteristic nding
lum can be imaged by US or CT'0 as the lead is an intraabdominal mass that demonstrates a
point for an intussusception, with a blind-ended, hypoechoic ring of bowel wall mucosasurround-
thick-walled bowel segment projecting beyond ing an echogenic center of trapped mesenteric fat
the apex of the intussusceptum." and vessels, the "doughnut sign" (Figure 86)."9
Multiple concentric circles may be seen when
there are multiple layers of both intussusceptum
APPENDICITIS and intussuscipiens. In the plane longitudinal to
the mass, there are alternating hypoechogenic and
The diagnosis of appendicitis,172 once a clinical cchogenic layers, the *sandwich"' or "pseudokid-
one with a large margin of error, has been con- ney" sign.!0 Thickening of the echolucent rim
siderably changed by advances in cross-sectional greater than 8 mm and uid within the intussus-
imaging. The US nding of an appendix greater Figure 84 Axial computed tomographic scan of the ceptum, especially if there is a dilated apex,have
than 6 mm in diameter, with a blind-ending pelvis reveals a thick tubular structure in the right lower been suggested as indications of signi cant isch-
lumen that is noncompressible and hyperemic, quandrant consistent with a swollen appendix. A calci ed emia and irreducibility by enema reduction. 'Sl
became the hallmark of acute appendicitis, with appendicolith (arrow) is present. Free intraperitoncal uid and bowel thickness did
up to a 90% sensitivity and 95% speci city.!"3 not alter outcome of reduction in another large
The presence of an appendicolith and periappen- series; however, the presence of small bowel
diceal uid supports the diagnosis, " although obstruction on plain lm reduced the successrate
documentation of an appendicolith may be asso- from 90 to 65%. I82
ciated with normal appendices in up to l4% Clinical peritonitis is a contraindication to
of surgically explored cases.175 US is useful to hydrostatic or air reduction. Anintussusception
exclude ovarian pathology in female patients and
can demonstrate abscess formation suggesting
perforation. Unfortunately, as in physical exami-
nation, US is limited by overlying bowel gas and
the fact that many appendices are not in a typical
location. The inability to demonstrate an in amed
appendix does not exclude acute appendicitis on
US. Because the technique uses graded compres-
sion, US is uncomfortable and can be dif cult to Figure 85 Axial contrast-enhanced computed tomo-
perform in uncooperative children. Furthermore, graphic scan of the pelvis revcals a uid collection with
a thin enhancing rim in the right lower quandrant (arrow)
US of the appendix is highly operator dependent.
adjacent to thick-walled bowel. At surgery, a perforated
Because of these limitations, CT of the abdo- appendix with a periappendiceal abscess was found.
men and pelvis has largely replaced US for the
diagnosis of acute appendicitis. Various tech-
(Figure 85). With the widespread use of US and
niques are advocated, including contrast- and
noncontrast-enhanced techniques. Some radi- CT, the negative appendectomy rate has dropped
from 15 to 4%, whereas the rate of perforation
ologists prefer oral, rectal, and intravenous con-
has decreased from 35 to l6% in one reporting
trast, whereas others perform the examination
with only rectal contrast. Despite these differ- institution. 176
ences in technique, the objective is to visualize
Intussusception
the enlarged and in amed appendix (Figure 83)
and an appendicolith if present (Figure 84) and to The most common cause of intestinal obstruction
identify any associated ndings of pericecal uid from 6 months to 6 years of age is intussuscep-
tion, which most commonly occurs before age Figure 86 Sonogram of the abdomen in a ar old
and in ammation. CT is also used extensively in
2 years, with a peak incidence between 3 and with erampy abdominal pain reveals a soft tissuemasS
to document suspected complications such as and
the right upper quadrant. The alternating echogenic
abscess formation both pre- and postoperatively 9 months. Of the intussusceptions that occur
hypocchoic layers are typical for anintussusceptioM.
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CHAPTER53.2 / Cross-Sectional Imaging 1449

pres
oscent for greater than 48 hours or one in an
infant less than 6 months with bowel obstrue-
on plain lms has a high likelihood of per-
foration.s Lead points have included most
poly Meckel diverticulum, duplication cysts,
andsubmucosal blecds associated with Henoch-
Schonlein purpura. Although the vast majority
of intussusceptions are reduced under conven-
ional uoroscopic guidance, recent attempts at
air reduction under US guidance have been met
with some success I84
Figure 88 Axial contrast-cnhanced computed tomo-
graphic scan of the abdomen in an adolescent female with
INFLAMMATORY CONDITIONS Crohn disease reveals an abscess arising from the wall of Figure 92 Coronal reformatted image from axíal CT per-
the transverse colon (arrow). formed after the oral ingestion of contrast and adminis-
Various in ammatory processes demonstrate tration of intravenous contrast in a patient with Crohn's
bowel wall thickening on cross-sectional imag- discase reveals a long segment of diseased small bowel.
"Comb" sign (arrow) produced by marked vascularity and
ing. Although this is usually caused by an infec-
strati ed enhancement of the wall suggest active disease.
tious process and is accompanied by mesenteric
(Courtesy ofJoseph Maklansky, MD.)
adenitis, IBD can cause similar ndings. Using
bowel wall thickness as a criterion for diagnos- 32
ing IBD on US and comparing it with colono-
scopic ndings as the gold standard, US showed
a sensitivity of 88% and a specifcity of 939%.I85
In addition, CT can demonstrate transmesenteric
diseaseassociated with Crohn's disease, such as
R
creeping apposition of fat, mesenteric lymph-
adenopathy,and stula formation helping to dis- Figure 89 Axial contrast-enhanced computed tomo-
graphic scan of the pelvis reveals a collection with an
tinguish Crohn's disease from ulcerative colitis
enhancing rim abutting the psoas muscle (long arrow) in
(Figures 87 and 88). Intravenous contrast will this adolescent male with Crohn's disease and right lower
enhancethe margins of in amed bowel wall or quadrant pain. Note the thick-walled bowel adjacent to the
an in ammatory mass (Figure 89), whereas oral abscess (short arrow).
or rectal contrast will identify lumen. Another
study, which looked at a combined group of Figure 93 Coronal reformatted image from axial CT per-
IBD, infectious colitis, pseudomembranous coli- formed after the oral ingestion of contrast and adminis-
tration of intravenous contrast in a patient with Crohn's
tis, Henoch-Schonlein purpura, and hemolytic
disease reveals fatty in ltration of the wall of the diseased
syndrome, could not differentiate one condition
transversc colon and lack of enhancement, ndings sugges-
fromanother. 86 Bowel thickening without trans- tive of olddisease.(Courtesy ofJoseph Maklansky, MD.)
mesentericdisease is, however, more typical of
colitis from causes other than Crohn's disease In necrotizing enterocolitis, US has been
(Figure 90),IS7 Recently, contrast-enhanced CT demonstrated to be sensitive in detecting bowel
enterography has gained popularity, replacing wall thickening, pneumatosis intestinalis, portal
conventional SBFT, particulariy in evaluating venous gas, and perforation causing in amma-
patients with known Crohn's disease, because it tory mass; however, serial plain lms remain
Figure 90 Contrast-cnhanced computed tomographic
can potentially identify areas of active disease the mainstay of diagnosis. In an investigative
scan of the lower abdomen reveals a thick-walled ascend-
by demonstrating bowel wall hyper-enhancement ing (up arrow) and descending colon (down arrow) in this study, MRI showed good correlation with opera-
and strati cation into separate layer, ndings that teenager with known colitis. tive ndings in a small group of premature infants
are not present in diseased brotic areas (Fig- determinedtohavenecrotizingenterocolitis.l
ures91 to 93), I58 Currently, MRI is less effective However, in view of the technical and practical
189
in demonstrating bowel pathology. considerations, cross-sectional imaging probably
does not have a great deal to contribute to the
care of these critically ill infants.

CONCLUSION

Imaging technology has markedly altered the tradi-


tional workup of gastrointestinal disorders. Ongo-
ing advances in US, CT, and MRI have revolution-
ized the approach to many common diseases and
greatly increased the accuracy of diagnosis. The
risks of radiation exposure, contrast toxicity, and
Figure 91 Coronal relormatted image from axial CT per-
sedation should always be considerations, espe-
formed after the oral ingestion of contrast and administra-
tion of intravenous contrast in a patient with Crohn's discase
cially when a pediatric patient is concerned. As
rigure 87 Contrast-enhanced computed tomographie
Scan
the pelvis in this 15-ycar-old male paticnt with reveals marked thickening of the terminal ileum and nar- with any modality, an understanding of the risks,
CrOin colitis reveals contrast extending into a perirectal rowing of the lumen. Enhancement of wall suggests active benetits, and limitations of a procedure is needed
stula (arrow). disease (arroN). (Courtesyof Joseph Maklansky, MD.) to effectively use these sophisticated technologies.
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1450 Part VI / Diagnosis of Gastrointestinal Disorders

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