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Imaging of the Pediatric

Urinary System
Ellen M. Chung, MDa,b,*, Karl A. Soderlund, MDc, Kimberly E. Fagen, MDc

KEYWORDS
 Pediatric  Children  Vesicoureteral reflux  Pyelonephritis  Renal scarring
 Postinfectious nephropathy  Congenital anomalies of the kidney and urinary tract (CAKUT)
 Renal cyst

KEY POINTS
 New methods for imaging the pediatric urinary tract include magnetic resonance urography (MRU),
functional MRU, and contrast-enhanced voiding urosonography.
 Management of primary vesicoureteral reflux (VUR) is the subject of controversy and is evolving.
The roles of imaging include evaluation for VUR, congenital anomalies, voiding dysfunction, pyelo-
nephritis, and renal scarring.
 A new consensus nomenclature and classification system has been developed for urinary tract dila-
tion that stratifies patients by risk for progressive uropathy.
 The causes and nature of cystic renal diseases of childhood have been better elucidated in the past
decade.
 A new modified-Bosniak classification system shows promise in helping to distinguish complicated
renal cysts from cystic renal tumors in children.

Recent advances in imaging of the pediatric uri- IMAGING MODALITIES IN CHILDREN


nary system include new applications of imaging Ultrasound
modalities to reduce radiation dose; evolving un-
Because of the concerns about long-term effects
derstanding of the relationship of vesicoureteral
of ionizing radiation, the most commonly used im-
reflux (VUR), urinary tract infection (UTI), and renal
aging modality for evaluation of the urinary system
scarring with greater appreciation for the impor-
is ultrasound (US). US is valuable for evaluating
tance of dysfunctional voiding; a new consensus
renal parenchyma, renal size and growth, collect-
classification system for urinary tract dilation;
ing system dilation, congenital malformations,
recognition of a unifying cause of genetic cystic
bladder wall thickness, and prevoid and postvoid
diseases of the kidneys; and a new approach to
bladder volume. Excellent technique with the
differentiating complex cysts from cystic renal
use of modern multifrequency and multi-focus
tumors.

Disclosure Statement: The authors have nothing to disclose.


The opinions and assertions contained herein are the private views of the authors and are not to be construed
as official or as representing the views of the departments of the Army, Navy, or Defense.
radiologic.theclinics.com

a
Department of Radiology and Radiological Sciences, F. Edward Hebert School of Medicine, Uniformed Ser-
vices University of the Health Sciences, 4301 Jones Bridge Road, Bethesda, MD 20814, USA; b Pediatric Radi-
ology Section, American Institute for Radiologic Pathology, 1100 Wayne Avenue, Silver Spring, MD 20910,
USA; c Department of Radiology, Walter Reed National Military Medical Center, 8901 Rockville Pike, Bethesda,
MD 20889, USA
* Corresponding author. Department of Radiology and Radiological Sciences, F. Edward Hebert School of
Medicine, Uniformed Services University of the Health Sciences, 4301 Jones Bridge Road, Bethesda, MD 20814.
E-mail address: ellen.chung@usuhs.edu

Radiol Clin N Am - (2016) --


http://dx.doi.org/10.1016/j.rcl.2016.10.010
0033-8389/16/Published by Elsevier Inc.
2 Chung et al

transducers and harmonic imaging yields the best examination is performed using heavily
results.1 T2-weighted images that readily depict dilated
urinary tracts. For nondilated tracts, delayed
Fluoroscopy contrast-enhanced imaging is very useful. Three-
dimensional (3D) imaging with isotropic or
Fluoroscopic voiding cystourethrogram (VCUG) is
near-isotropic voxel size allows for creation of 2-
much less commonly performed than in the past
dimensional and 3D reconstructions that are help-
but remains an important examination for
ful for evaluating complex anatomy (Fig. 1).
screening and follow-up of patients with VUR.
Dynamic contrast-enhanced MR, also called
Dose reduction techniques, including use of digital
functional MR imaging, allows evaluation of renal
pulsed fluoroscopy (34 frames per second),
function and drainage using visual assessment
recording last-image hold rather than performing
as well as quantitative measures, including time-
exposures, collimation, and limited use of magnifi-
signal intensity curves, calyceal-transit time, renal
cation, should be used.2
transit time, and differential renal function using
the Patlak-Rutland method.7
Nuclear Medicine
Contrast-enhanced-voiding urosonography (ce-
Radionuclide cystogram (RNC) is another option VUS) is an available alternative to VCUG and RNC
for evaluation of suspected VUR. 99mTc-pertech- for VUR screening without ionizing radiation. Ce-
netate is instilled into the bladder. The advantages VUS is performed by instilling an US contrast
include slightly less radiation dose compared with agent mixed with saline into the bladder. Some
VCUG and increased sensitivity, as reflux is an of these agents are now available in the United
intermittent phenomenon. The disadvantage is States, but their use for ce-VUS is off label. Kid-
decreased spatial resolution, so RNC is more suit- neys, ureters, bladder, and urethra (transperineal
able for follow-up of prior positive VCUG and window for boys) are then scanned using gray-
asymptomatic sibling screen in girls for whom ure- scale, color flow, harmonic, and contrast-specific
thral abnormalities are rare. Renal cortical scintig- imaging (Fig. 2).8
raphy with 99mTc-dimercaptosuccinic acid (DMSA)
is particularly sensitive for pyelonephritis and renal VESICOURETERAL REFLUX AND URINARY
scarring. TRACT INFECTION
Computed Tomography and MR Imaging UTI is one of the most common illnesses in the pe-
diatric population. Approximately one-third of chil-
MR imaging is an appealing alternative to modal-
dren with UTI are found to have VUR.9 In young
ities involving ionizing radiation; but because of
children, most cases of VUR result from a primary
the long scan times, sedation is required in young
abnormality of the ureteral insertion into the
children. There may be long-term risks of sedation,
bladder wall via an intramural tunnel such that its
and further study of these is required.3,4 Although
ability to prevent backward flow of urine is
computed tomography (CT) uses ionizing radia-
compromised. This condition may be familial and
tion, this modality is much faster, generally does
usually resolves spontaneously by 6 years of
not require sedation, and is subject to less motion
age. On the other hand, in some children, reflux
artifact compared with magnetic resonance (MR).
is secondary to another abnormality. The most
Efforts to reduce radiation dose of diagnostic ex-
common cause of secondary reflux is bladder
aminations has led to advances in CT scan tech-
outlet obstruction, which may be caused by
nology that have allowed reduction in radiation
anatomic obstruction, such as posterior urethral
exposure associated with pediatric CT
valves, or by physiologic obstruction, as in neuro-
examinations.5
genic bladder. Another cause of secondary reflux
is ureteral insertion into or near a bladder divertic-
Future Trends
ulum. A diverticulum situated near the ureteroves-
Newer methods of imaging are available, and use ical junction is known as a Hutch diverticulum
of these is expanding. MR urography can be help- (Fig. 3). The diverticulum represents herniation of
ful in cases of complex urogenital anomalies, the urothelium through a defect in the muscle
particularly with dilated tortuous ureters that are of the bladder wall. If the ureter inserts at a site
very difficult to follow with US. Additional applica- of muscle weakness, the sphincteric function ure-
tions include ectopic ureteral insertion and urinary terovesical junction will be inadequate.
tract dilation without reflux.6 Renal parenchymal The most common examination performed for
abnormalities, such as cystic dysplasia, pyelone- reflux screening is the VCUG. Proper technique
phritis, and scars, may be seen as well. The for the performance of VCUG is important.
Imaging of the Pediatric Urinary System 3

Fig. 1. Coronal T2 maximum intensity projection (A) and 3D coronal reformatted images (B) from MR urography
demonstrate bilateral dilated ectopic ureters in a 3-month-old girl. (Courtesy of David M. Biko, MD, Childrens
Hospital of Philadelphia, Philadelphia, PA.)

 Early filling image is valuable for showing ure-  Voiding images of boys are important and
teroceles, herniations of dilated distal ureter should be obtained in a nearly lateral projection.
into the bladder usually caused by ureteroves-  Cyclic voiding, allowing the bladder to fill and
ical junction obstruction. empty several times before removing the
 Oblique views should be obtained to show the catheter, increases the sensitivity of the ex-
ureteral insertions in tangent. amination in infants.
 Ureteral insertion should be at least 1 cm from
the bladder neck (otherwise ectopic). The grading system for VUR is internationally
standardized (Fig. 4).10

Fig. 2. (A) Longitudinal image from ce-VUS shows US contrast in the intrarenal collecting system (arrowhead) and
ureter (arrow). (B) VCUG in same patient also shows VUR. (Courtesy of Jeanne S. Chow, MD, Boston Childrens
Hospital, Boston, MA.)
4 Chung et al

Fig. 3. Bilateral Hutch diverticula in a 12-year-old boy. (A) Longitudinal US image of the bladder demonstrates an
anechoic outpouching from the bladder (arrowhead). (B, C) Bilateral oblique images from a VCUG demonstrate
bilateral contrast-filled diverticula (arrowheads) into which the ureters insert.

 Grade I: reflux into ureter that does not reach VUR and infants with hydronephrosis detected in
the renal pelvis utero. Concerns about the risk of continuous anti-
 Grade II: extending into the pelvis without biotic prophylaxis selecting out resistant organisms
dilation arose. In addition, given the lack of large random-
 Grade III: mild to moderate dilation of pelvis and ized prospective trials comparing antibiotic prophy-
calyces with no or slight blunting of the fornices laxis with watchful waiting, the need for extensive
 Grade IV: moderate dilation of pelvis and screening and prophylaxis was questioned. More
calyces and blunting of fornices but presence data were needed to determine who could safely
of papillary impressions; moderate tortuosity be excluded from screening and prophylaxis.
of the ureter Studies performed in the early part of this cen-
 Grade V: marked dilation of the pelvis and tury failed to demonstrate the efficacy of antibiotic
calyces with loss of papillary impressions; prophylaxis in preventing recurrent UTI and renal
dilation and tortuosity of the ureter scarring, causing many to reject prophylaxis in
favor or watchful waiting.1922 The value of
The great concern about UTI and reflux in chil- screening imaging examinations for reflux then
dren stems from the relationship between UTI, also came into question. Based on these data, in
VUR, and renal scarring.11 Pyelonephritis in chil- 2011 the American Academy of Pediatrics (AAP)
dren is often an ascending infection related to the issued an update to its practice guideline on the
presence of a bladder infection and VUR. Pyelone- management of babies with UTI between 2 and
phritis, particularly if not treated expeditiously, can 24 months of age. In this update, VCUG is no
result in acquired renal scars with persistent de- longer recommended for first febrile UTI if the renal
fects on DMSA scans after recovery.12,13 The inci- US is normal.23 Similar guidelines were also adop-
dence of scarring is higher in cases of multiple ted in Europe leading to a marked decrease in the
episodes of infection and with dilating and higher number of reflux evaluations performed.1
grades of reflux (III and greater).13,14 Patients who On the other hand, some subsequent studies
already have parenchymal scars are at higher risk with larger numbers of subjects came to a
of developing additional scars.15 different conclusion. The PRIVENT (Prevention
VUR alone does not cause upper tract scarring of Recurrent Urinary Tract Infection in Children
without UTI, and this is the rationale for antibiotic with Vesicoureteric reflux and Normal Renal
prophylaxis to prevent recurrence of UTI while Tracts) study24 showed a significant benefit of
awaiting resolution of VUR.14 It has been shown antibiotic prophylaxis although the study
that the incidence of scarring is reduced in pa- included patients without VUR and patients who
tients treated with antibiotic prophylaxis.16,17 did not undergo imaging for VUR. The Swedish
Beginning in the 1990s, recommendations issued Reflux Trial showed a significant benefit of pro-
based on these data resulted in development of phylaxis in girls with higher-grade reflux.25,26
a robust screening program for VUR and for The RIVUR (Randomized Intervention for Children
continuous antibiotic prophylaxis.18 with Vesicoureteral Reflux) study9 was a random-
Screening expanded to include patients who had ized, placebo-controlled study of more than 600
never had UTIs, including siblings of patients with
Imaging of the Pediatric Urinary System 5

Fig. 4. Vesicoureteral reflux. (A) Grade II reflux with no blunting of the calyces is seen (arrowhead). (B) Mild
blunting of the calyces (arrowhead) characterizes grade III VUR. (C) Grade IV VUR is associated with marked ca-
lyceal blunting but preservation of the papillary impression (arrowheads). (D) Grade V reflux shows loss of the
papillary impression (arrowhead) and marked tortuosity of the ureter (arrow). Intrarenal reflux allows visualiza-
tion of the parenchyma on VCUG (curved arrow).

children, which showed that there is a benefit to abnormalities would not have been detected
antibiotic prophylaxis with a 50% reduction in with the newer screening recommendations.
recurrence of UTI. The benefit was higher for Most of these they considered significant,
those with bowel bladder dysfunction and a his- including some cases of high-grade reflux and
tory of febrile UTI.27 Furthermore, a study scarring.28,29
applying the new criteria recommended by the The inconsistency in results of the aforemen-
National Institute for Health and Care Excellence tioned studies is due to variation in study design
in the United Kingdom to an existing database of and inclusion and exclusion criteria, resulting in
children with UTI showed that 58% of all varied study populations with disparate levels of
6 Chung et al

risk and, therefore, disparate potential benefits of DMSA is also used in follow-up to evaluate for res-
prophylaxis.18 Patients at higher risk of recurrent olution of the defect versus development of perma-
UTI include those who are female and have a his- nent scar. T2-weighted MR images also depict
tory of recurrent prior UTIs, higher-grade reflux, cortical scars.34,35
and voiding dysfunction.18 The later findings Pyelonephritis has a similar appearance on all
have caused many investigators to call for recon- imaging modalities. The involvement may be
sideration of the AAPs 2011 recommendation.9 diffuse or focal (also referred to as acute bacterial
The current recommendations have caused a nephritis or lobar nephronia) (Fig. 5).
marked decrease in the number of cystograms
performed worldwide in the last decade. The effect  Enlargement, may be the only finding on US; if
on the population of this decline in screening has focal, may mimic a mass
yet to be determined.  Loss of corticomedullary differentiation
Furthermore, not all patients with scarring have  Streaky appearance of parenchyma on
reflux; the incidence of progression to scarring is contrast-enhanced CT
the same for children with DMSA defects regard-  Urothelial thickening of the renal pelvis and
less of the presence of reflux15,30 prompting for ureter on US
some a shift in focus from VUR to renal paren-  Decreased flow on US and decreased
chymal abnormalities, which are much better contrast enhancement on CT and MR relative
detected with DMSA scan than with US. Recent to unaffected kidney parenchyma
studies have shown that a significant percentage  Focal high-signal intensity on DWI and low-
of patients with renal cortical defects do not have signal on apparent diffusion coefficient map
detectable VUR, yet these patients are at risk of Failure to improve with therapy suggests the
developing further scarring and would be missed possibility of complicated infection with abscess
without DMSA scanning, which is not routinely rec- or pyonephrosis. Abscess appears as a round,
ommended by the current practice guidelines.31,32 well-defined nearly anechoic or fluid-attenuation
These data have caused some to advocate for a mass with no internal flow or enhancement
top-down approach (to evaluate for scarring with (Fig. 6). Rupture into the perinephric space may
DMSA scanning and then cystogram, if positive) be seen. Pyonephrosis appears as echogenic ma-
rather than the traditional bottom-up approach (to terial within a dilated renal collecting system
evaluate for VUR with cystogram and then possibly (Fig. 7). Drainage of the abscess or collecting sys-
for scar with DMSA in selected cases).14 tem is required for complicated infection.

Pyelonephritis Renal Scarring


Diagnosis of pyelonephritis is important, as clinical In some cases, particularly with delayed treatment,
complications of UTI are limited to those with upper pyelonephritis can progress to renal scarring or
tract infection.1 In older children, the diagnosis is postinfectious nephropathy. On all imaging modal-
generally made clinically; however, specific clinical ities the affected area is decreased in size, and other
symptoms of UTI are more difficult to detect in pre- features include the following (Figs. 810):
verbal, incontinent infants. For these infants, fever is
 Focal loss or thinning of cortex and medulla
the most important indicator of upper tract infection.
(full thickness)
Indications for imaging for pyelonephritis
 Mild dilation of the underlying calyx due to
include the following:
loss of papilla that normally preserves its
 Lacking or equivocal clinical information shape
 Suspected congenital anomaly  Diffuse cortical thinning
 Known or suspected urinary tract obstruction  Diffuse decrease in size or lack of growth, may
 Failure to respond to 48 hours of intravenous be the only finding on US
antibiotics  Compensatory hypertrophy of the adjacent
unaffected portions of the kidney, which can
US, CT, and renal cortical scintigraphy with mimic a mass (see Fig. 10)
DMSA scan may be used; but DMSA scan is the
most sensitive examination.33 Because of the VOIDING DYSFUNCTION AND NEUROGENIC
desire to limit exposure to ionizing radiation, BLADDER
some investigators advocate use of MR with
diffusion-weighted imaging (DWI), which has been Dysfunctional voiding is an important cause of UTI
recently shown to be comparable with DMSA for and incontinence. Voiding dysfunction results from
the evaluation of suspected pyelonephritis.34 failure of the detrusor muscle of the bladder neck
Imaging of the Pediatric Urinary System 7

Fig. 5. Focal pyelonephritis in a 7-year-old girl. (A) Gray-scale longitudinal US image shows focal loss of cortico-
medullary differentiation (arrowheads). (B) Relative decrease in flow is noted in the affected area (arrowhead).
(C) Contrast-enhanced CT in another patient shows focal decreased enhancement (arrowhead).

and the internal (involuntary) and external (volun- for demonstrating rare anatomic causes for
tary) sphincters to act synergistically to hold and incontinence.
release urine appropriately. The rectum and Neurogenic bladder results from failure of the
bladder are both embryologically derived from external sphincter to relax during voiding. Spinal
the cloaca and share some innervation, account- dysraphism is the most common cause of neuro-
ing for the association of constipation and genic bladder in children. Other cases of neuro-
dysfunctional bladder emptying. The underlying genic bladder include tethered cord, caudal
cause of voiding dysfunction is usually a neuro- regression, presacral mass, and spinal cord injury.
logic defect. In the remainder of cases, only about The bladder wall becomes hypertrophied from at-
1% to 2% have a demonstrable anatomic cause.36 tempts to overcome the physiologic obstruction
Imaging can be useful in patients with dysfunc- and loses compliance over time. This loss of
tional voiding who present with UTI to evaluate compliance is associated with increased intraves-
for secondary VUR and renal scarring and ical pressure, which can lead to secondary VUR.

Fig. 6. Renal abscess. (A) Longitudinal US image of the left kidney reveals a heterogeneous mass with central hy-
poechoic region (arrowhead). (B) Axial CT following intravenous iodinated contrast confirms a heterogeneous
mass with central fluid attenuation (arrowhead). (Courtesy of Marilyn J. Siegel, MD, Mallinckrodt Institute of
Radiology, St Louis, MO.)
8 Chung et al

Fig. 7. Pyonephrosis of an obstructed upper pole ureter in a 5-month-old girl. (A) Initial postnatal longitudinal
image of the bladder shows an anechoic left upper pole ureterocele (arrowhead). Asterisk indicates dilated ure-
ter. (B) US image of the opposite right kidney shows mild dilation of the upper pole collecting system (asterisk)
and normal parenchymal thickness and corticomedullary differentiation (arrow). (C) Longitudinal image of the
bladder obtained at 5 months of age when admitted for febrile UTI shows echogenic material within the ureter-
ocele and thickening of the wall (measurement calipers). Asterisk indicates dilated lower pole ureter. (D) Longi-
tudinal image of the left kidney shows marked dilation of both upper and lower pole collecting systems with
echogenic material in the upper pole pelvis and ureter (arrow).

Stasis of urine predisposes to UTI, and the combi-  Plain radiographs, constipation; spinal
nation of UTI and VUR can cause renal scarring dysraphism
and even renal insufficiency. Neurogenic bladders  Thickened, irregular bladder wall with multiple
show abnormal appearance and function on imag- small diverticula
ing (Fig. 11).  Echogenic debris, reduced bladder volume,
and large postvoid residual on US; prevoid
and postvoid bladder volumes should be ob-
tained in all renal bladder US
 Christmas tree appearance on VCUG due to
irregular wall and taller-than-wide
configuration
 Funnel-shaped bladder neck and spinning-
top appearance of the urethra
 Inability to void spontaneously or decreased
caliber of postsphincteric urethra
 Overflow incontinence
Some patients with voiding dysfunction have
findings similar to neurogenic bladder in the
absence of an underlying neurologic deficit.
Affected patients also have abnormal emptying
Fig. 8. Focal renal scar in 5-year-old boy with history
of febrile UTI at 2 years of age. Longitudinal renal of the colon. These associated conditions are
US shows a focal area of increased echogenicity encompassed in the term bowel and bladder
and cortical thinning and volume loss (arrowheads). dysfunction (BBD). BBD is a fairly common condi-
(Courtesy of Jeanne S. Chow, MD, Boston Childrens tion associated with an increased risk of UTI and
Hospital, Boston, MA.) risk of development of renal scars.18
Imaging of the Pediatric Urinary System 9

Fig. 9. (A) Delayed DMSA scan shows focal defects (arrowhead) in right upper pole after febrile UTI consistent
with scarring. (B) VCUG shows VUR.

Fig. 10. Severe renal scarring in 6-year-old girl with hypertension. (A) Longitudinal US of the right kidney shows
marked parenchymal loss in the upper pole above a mildly dilated calyx (arrowhead). (B) CT following intrave-
nous contrast reveals parenchymal loss of the anterior right kidney (arrowhead) and compensatory hypertrophy
of the posterior portion (arrow). The left kidney is severely scarred and small (curved arrow). (C) Axial T2-
weighted MR image also shows anterior parenchymal loss and mildly dilated calyx (arrowhead) and posterior hy-
pertrophy (arrow). (D) VCUG demonstrates left VUR and trabeculated bladder wall (arrow) consistent with
bladder dysfunction.
10 Chung et al

Fig. 11. Neurogenic bladder. (A) Anterior-posterior image from VCUG in a 2-year-old girl with closed spinal dys-
raphism shows a taller-than-wide configuration of the bladder with numerous small bladder diverticula. The
bladder neck is inappropriately funnel shaped in the filling phase (arrow). Note widened posterior elements
of the sacrum (arrowheads). (B, C) Transverse US and axial T2-weighted MR images of the bladder in a 5-year-
old patient show thickened and trabeculated bladder wall (arrowhead). Bladder is decompressed by a catheter
in the MR image.

Anatomic causes for voiding dysfunction are URINARY TRACT DILATION


typically diagnosed in utero; but, particularly in
the absence of ureteral dilation, these may be Fetal urinary tract dilation (UTD) occurs in up to 1%
found in older children presenting with UTI or in- to 2% of pregnancies.37 Prenatal UTD results from
continence. Half of boys with posterior urethral a wide variety of congenital anomalies of the kid-
valves are found prenatally because of bilateral uri- ney and urinary tract (CAKUTs), with the risk of
nary collecting system dilation, but the other half postnatal abnormality varying widely from 11%
have unilateral or no dilation of the ureter and diag- to 88% depending on severity (Box 1).37 CAKUTs
nosis may be delayed (Fig. 12). Girls with duplex may be associated with varying degrees of renal
kidneys with ectopic upper pole ureters with hypoplasia or dysplasia, and CAKUTs are the
obstruction and ureterocele are often identified most common cause of chronic kidney disease
prenatally; but if there is no dilation of the collect- and end-stage renal disease in the pediatric pop-
ing system, diagnosis is delayed. If the ureter in- ulation. The goal of prenatal screening for UTD is
serts into the urethra below the external to identify pathologic conditions that would require
sphincter or into the vagina, then she will be incon- postnatal therapy in order to prevent or delay
tinent of urine produced by the upper pole moiety. these complications.38
The classic clinical history of inability to achieve Clinical approaches to the diagnosis and man-
complete continence of urine with diurnal and agement of pediatric UTD are varied. Furthermore,
nocturnal wetting in a girl suggests the diagnosis, no standard nomenclature and differing classifica-
and additional imaging studies beyond US may tion systems have been adopted by practitioners
be needed (Fig. 13). of the various fields involved with the diagnosis
Imaging of the Pediatric Urinary System 11

Fig. 12. Posterior urethral valves. (A) Longitudinal US of the bladder in a newborn boy shows marked thickening
of the bladder wall (arrowhead) and dilation of the posterior urethra (asterisk). (B) Voiding image shows the
valve (arrow) just below the dilated posterior urethra. (C) Anterior-posterior image of the bladder from a
VCUG in a 10-year-old patient shows an abnormal configuration, marked irregularity of the bladder wall with
small diverticula, and dilated posterior urethra (arrow).

Fig. 13. Duplex kidney with ectopic upper pole ureter inserting into the vagina in 8-year-old girl. (A) Longitudinal
US image shows cortical echogenicity (arrow) separating the renal sinus fat into 2 parts. The upper pole paren-
chyma (curved arrow) is thinned because of renal dysplasia. (B) Coronal maximum intensity projection image
demonstrates two ureters draining the right kidney (arrowheads). (C) Coronal delayed postgadolinium image
shows the right upper pole ureter (arrowhead) draining into the vagina (asterisk) below the bladder (B). (D) Axial
early dynamic postgadolinium image of the perineum shows the fluid-filled vagina (arrowhead). (E) Delayed im-
age shows filling of the vagina with gadolinium-chelate (arrowhead).
12 Chung et al

Box 1 fluid volume for prenatal scans. Those features


Causes of pediatric urinary tract dilation are anterior-posterior renal pelvic diameter
(APRPD), calyceal dilation, parenchymal thickness
Transient/physiologic and appearance, ureteral dilation, and bladder ab-
Ureteropelvic junction obstruction normalities (Figs. 1418).
Vesicoureteral reflux
Prenatal Imaging
Ureterovesical junction obstruction
Prenatal US is typically performed during the first
Congenital megaureter
trimester for dating and repeated in the second
Multicystic dysplastic kidney disease trimester to assess the fetal anatomy. The APRPD
Posterior urethral valves is the maximum anterior-posterior diameter of the
Ureterocele intrarenal portion of the pelvis measured in the
transverse plane during second-trimester fetal
Duplex collecting system
anatomic surveys. Prenatal UTD is diagnosed
Prune belly syndrome (Eagle-Barrett syndrome) when the APRPD is greater than or equal to
Ectopic ureteral insertion 4 mm between 16 and 20 weeks gestational age
Polycystic kidney disease and greater than or equal to 7 mm at 28 to
32 weeks gestational age (see Table 1).
Follow-up prenatal sonography is warranted if
and management of UTD. Therefore, there are there is urinary tract dilation on the second-
limited data correlating the severity of prenatal trimester fetal survey. In one study, postnatal
UTD with postnatal outcomes. Terms such as pel- uropathy was diagnosed in 12% of children with
viectasis, caliectasis, and hydronephrosis are isolated second-trimester UTD but in 40% with
somewhat vague, though commonly used de- progressive (second and third trimester) UTD.40
scriptors for UTD. These nonspecific terms should Most cases of second-trimester UTD with an
be avoided in favor of the term dilation.39 APRPD between 4 and 7 mm resolved during
In 2014, a multidisciplinary committee consist- third-trimester imaging.41 Close US follow-up is
ing of pediatric radiologists, nephrologists, urolo- recommended for fetuses with second-trimester
gists, and obstetricians published a consensus APRPD greater than 7 mm, as progressive UTD
statement on the classification and management is associated with a higher rate of postnatal urop-
of prenatal and postnatal UTD (Table 1).38 In this athy.42 A prenatal APRPD of greater than 10 mm is
system, the same features are evaluated antena- highly associated with the requirement for post-
tally and postnatally with the addition of amniotic natal surgical correction.43 Fetal MR imaging

Table 1
Ultrasound parameters and normal values for the 2014 consensus urinary tract dilation classification
system

US Finding 1627 wk EGA More than 28 wk EGA Postnatal


APRPD <4 mm <7 mm <10 mm
Calyceal dilation
Central Nondilated Nondilated Nondilated
Peripheral Nondilated Nondilated Nondilated
Parenchymal thickness Normal Normal Normal
Parenchymal appearance Normal Normal Normal
Ureters Normal Normal Normal
Bladder Normal Normal Normal
Unexplained oligohydramnios Not present Not present N/A
Notes: Anterior-posterior renal pelvic diameter is measured on a transverse image at its maximal intrarenal diameter.
Parenchymal thickness is based on a subjective assessment. Parenchymal appearance describes echogenicity, corticome-
dullary differentiation, and the presence/absence of cortical cysts. Bladder evaluation should include wall thickness, pres-
ence/absence of ureterocele, and presence/absence of dilated posterior urethra.
Abbreviations: APRPD, anterior-posterior renal pelvic diameter; EGA, estimated gestational age; N/A, not applicable.
Adapted from Nguyen HT, Benson CB, Bromley B, et al. Multidisciplinary consensus on the classification of prenatal and
postnatal urinary tract dilation (UTD classification system). J Pediatr Urol 2014;10(6):98298.
Imaging of the Pediatric Urinary System 13

Fig. 14. Longitudinal (A) and transverse US (B) show renal pelvic dilation. The white line illustrates the proper
measurement of APRPD.

may be used in complex cases and for problem minor calyces, which surround the papillae (see
solving in patients with a higher severity of prenatal Fig. 15). The finding of peripheral calyceal dilation
UTD. is associated with increased risk of uropathy
compared with central calyceal dilation alone.38
Postnatal Imaging
Postnatal evaluation of pediatric UTD is performed Risk Assessment and Management
initially with US and with additional imaging and
The goal of diagnosing pediatric UTD is to prevent
functional studies if the dilation persists. Postnatal
major complications and identify patients with
US is typically performed at least 48 hours after
congenital abnormalities of the kidney and urinary
birth, as infants are typically dehydrated in the
tract. The Consensus Panel also developed a risk-
early postnatal period, leading to underestimation
stratification system based on the prenatal and
of the degree of UTD.44 However, imaging should
postnatal sonographic findings to predict potential
not be delayed in cases of oligohydramnios, ure-
for future significant uropathy and to direct man-
thral obstruction, bilateral high-grade dilatation,
agement (Tables 2 and 3). The risk classification
and concern for poor parental compliance.
group should be included in the impression of
The postnatal measurement of the APRPD
the radiographic report.
should be performed at the widest portion of the
intrarenal pelvis in the transverse plane with pa-
tients in the prone position (see Fig. 14). Subse- CYSTIC RENAL DISEASE
quent APRPD measurements should be
performed with the same patient positioning to Cystic renal disease may be sporadic or inherited.
ensure consistency of measurement. The type The former results from congenital anomalies of
and degree of calyceal dilation can be better the kidney and urinary tract that result in abnormal
assessed on the postnatal scan. The term central development of the renal parenchyma. It is known
calyces as used in the UTD system refers to major that genetic causes of cystic renal disease are
calyces or infundibula, which drain 2 or 3 minor related to mutations of primary cilia, which also
calyces. The term peripheral calyces refers to affect the liver and possibly other organ systems.

Fig. 15. Changing dilation of peripheral calyces due to VUR. (A) Initially mild dilation of a peripheral calyx is
noted (arrow). (B) Later images show more marked dilation of the peripheral calyces (arrow).
14 Chung et al

Fig. 16. Parenchymal compression in a newborn boy with PUV. (A) Longitudinal US image shows marked dilation
of the collecting system and compression of the renal parenchyma (arrowhead). (B) Longitudinal US image of the
ureter shows marked dilation (asterisk). PUV, posterior urethral valve.

Simple Renal Cyst reflux, or ectopic ureteral insertion.45 The most


severe form of obstructive uropathy is multicys-
Simple renal cysts are uncommon in children but
tic dysplastic kidney (MCDK) caused by infundi-
more common than previously thought because
bulopelvic atresia. Cysts replace the kidney;
of the increased utilization of US in the evaluation
imaging reveals a nonreniform mass of multiple
of children with UTI. A solitary cyst found prena-
variable-sized, noncommunicating cysts with
tally is usually inconsequential; but cysts may be
no identifiable normal renal parenchyma
seen in malformation syndromes, so careful evalu-
(Fig. 19). The absence of renal parenchyma dis-
ation for other anomalies should be undertaken.
tinguishes MCDK from other causes of abdom-
Simple renal cysts in children are typically solitary,
inal cysts. On the other hand, MCDK
so the finding of 2 or more cysts should prompt
occasionally may be segmental, involving the
consideration of a genetic cystic renal disease.
upper pole of a duplex kidney or the lower pole
Similar to adults, cysts that satisfy all US criteria
of a crossed-fused ectopic kidney. In such
for simple cysts require no further evaluation.
cases, there is adjacent renal parenchyma;
Otherwise, contrast-enhanced imaging is neces-
cystic tumors of the kidney must be considered
sary to exclude enhancing solid components
in the differential.
because Wilms tumor can be cystic.
Lesser degrees of cystic renal dysplasia appear
Cystic Renal Dysplasia as small cysts seen throughout the cortex and me-
dulla with hyperechoic parenchyma and poor cor-
Abnormal renal parenchymal development re- ticomedullary differentiation (Fig. 20). Additional
sults from in utero collecting system obstruction, findings of dilated urinary collecting system, ure-
terocele, or ectopic ureter inform the radiologist
of the underlying cause of the cystic disease.

Genetic Cystic Renal Disease


Inherited cystic renal disease results from muta-
tions of genes encoding primary (sensory) cilia
and are now included in the group of diseases
termed ciliopathies.46 Unlike the better-known
motile cilia that line the respiratory tract, nonmotile
primary cilia serve as the antennae of cells,
sensing the environment and sending signals to
the nucleus. The most specialized of these cilia
are found on the cells lining the retina. Primary cilia
are also found on cells lining the collecting ducts of
the kidney and the bile ducts of the liver. They are
Fig. 17. Increased parenchymal echogenicity in a
newborn boy with oligohydramnios due to ARPKD. involved in tubule development or maintenance.
Longitudinal US image of the kidney shows increased Primary cilia are also important in development
cortical echogenicity (arrowhead) compared with of the brain, thorax, and skeleton; many malforma-
the adjacent liver. (Courtesy of Jeanne S. Chow, MD, tion syndromes involving these structures are also
Boston Childrens Hospital, Boston, MA.) ciliopathies.
Imaging of the Pediatric Urinary System 15

Fig. 18. Cystic dysplasia due to posterior urethral valves in a newborn boy. (A) Longitudinal US image of the kid-
ney shows poor corticomedullary differentiation and a parenchymal cyst (arrowhead). (B) Transverse US of the
decompressed bladder reveals marked bladder wall thickening (arrowhead). A Foley catheter balloon is seen
in the bladder lumen.

Autosomal recessive polycystic kidney disease  Plain radiographic findings


Autosomal recessive polycystic kidney disease  Small, bell-shaped thorax
(ARPKD) is the most common genetic cystic renal  Bilateral pneumothoraces
disease in infants and young children. The disease  Bulging flanks with central displacement of
always involves the both the kidney and the liver bowel gas
but to different degrees. In the kidneys, the collect-  US findings
ing ducts are dilated and there is stasis of urine in  Enlarged hyperechoic kidneys
the ducts. In the liver, all patients have congenital  Poor corticomedullary differentiation
hepatic fibrosis, or ductal plate malformation,  Halo appearance of hypoechoic, relatively
leading to abnormal fibroblastic proliferation spared cortex (which has fewer collecting
around the portal veins and dilation of the intrahe- ducts) compressed to the periphery
patic biliary ducts. Over time, the fibroblastic pro-  Echogenic pyramids mimicking medullary
liferation becomes sclerotic leading to portal nephrocalcinosis in some cases
hypertension. The severity of renal disease relative  Tubular configuration of the cysts
to liver disease determines the age at presenta-  Some small, round macrocysts possible
tion, because liver disease takes longer to mani- (Fig. 24)
fest clinically.  Bilateral findings always present (vs cystic
The infant with severe renal disease presents in renal dysplasia, which is often unilateral)
the perinatal period with oligohydramnios and pul-  Segmental involvement of the kidneys in
monary hypoplasia. US evaluation is generally suf- patients with predominant liver disease
ficient to confidently diagnose ARPKD (Figs. 21
and 22). With modern high-frequency linear trans- In all patients, ARPKD is also a disease of the
ducers, it is now possible to resolve the tubular na- liver. Even in patients presenting in the perinatal
ture of these cysts, which is a defining feature of period with severe kidney disease, congenital he-
ARPKD (Fig. 23).45 patic fibrosis can be detected on liver biopsy.

Table 2
Risk-based management of prenatally diagnosed urinary tract dilation

UTD A1: Low Risk UTD A23: Increased Risk


Prenatal period Additional US after 32 wk Additional US in 46 wk
After birth 2 additional US: US between 48 h and 1 mo
 First between 48 h and 1 mo after birth after birth
 16 mo after birth
Other Aneuploidy risk modification if indicated Specialist consultation with
nephrology and/or urology

Abbreviation: A, antenatal.
Adapted from Nguyen HT, Benson CB, Bromley B, et al. Multidisciplinary consensus on the classification of prenatal and
postnatal urinary tract dilation (UTD classification system). J Pediatr Urol 2014;10(6):98298.
16 Chung et al

Table 3
Risk-based management of postnatally diagnosed urinary tract dilation

UTD P1: Low Risk UTD P2: Intermediate Risk UTD P3: High Risk
Follow-up US 16 mo 13 mo 1 mo
VCUG At clinicians discretion At clinicians discretion Recommended
Antibiotics At clinicians discretion At clinicians discretion Recommended
Functional scan Not recommended At clinicians discretion At clinicians discretion
Abbreviation: P, postnatal.
Adapted from Nguyen HT, Benson CB, Bromley B, et al. Multidisciplinary consensus on the classification of prenatal and
postnatal urinary tract dilation (UTD classification system). J Pediatr Urol 2014;10(6):98298.

Patients with predominant liver disease present in parenchyma is compressed. ADPKD is much
early school age with manifestations of portal hy- more common than ARPKD in the general popula-
pertension. Liver findings on imaging are also var- tion but not in children. Children with ADPKD may
iable depending on the severity of the disease (see be discovered on US obtained in evaluation for
Fig. 24; Fig. 25). UTI. Rarely, infants can present with enlarged
echogenic kidneys similar in appearance to those
 Hepatosplenomegaly of ARPKD. Cysts may be seen, but these are round
 Heterogeneous echotexture to the liver rather than the tubular cysts of ARPKD. On the
 Ascites other hand, ARPKD can have small macrocysts.
 Gastric varices; splenorenal shunt Screening of the parents may help by revealing
 Intrahepatic bile duct dilation, typically cysts of undiagnosed ADPKD.
peripheral
 Central dot sign: severely dilated duct
completely engulfs portal vein Medullary cystic disease and juvenile
 Extrahepatic duct and gall bladder dilation nephronophthisis
 Asymmetric enlargement of the left lobe of the Medullary cystic disease and juvenile nephronoph-
liver thisis are related genetic progressive tubulointer-
stitial diseases that also represent ciliopathies.
Autosomal dominant polycystic kidney disease Patients present with urine concentrating defects
Autosomal dominant polycystic kidney disease and progress to renal failure. Imaging features
(ADPKD) is also caused by a primary ciliary defect differ from those of the more common ciliopathies.
but one that causes abnormal tubular maintenance
rather than development.46 The kidneys are normal  Normal-sized or small kidneys (vs large kid-
at birth but progressively develop round cysts that neys in ARPKD and ADPKD)
do not communicate with the tubules. As the num-  Hyperechoic kidneys with poor corticomedul-
ber and size of the cysts increase, the adjacent lary differentiation

Fig. 20. Renal dysplasia in a 10-day-old boy with pre-


Fig. 19. Longitudinal US image shows replacement of natal diagnosis of posterior urethral valves. A longitu-
the left kidney with multiple large, noncommuni- dinal US shows enlargement of the kidney, multiple
cating cysts (arrow). There is no visible normal renal round parenchymal cysts, and loss of normal cortico-
parenchyma. medullary differentiation.
Imaging of the Pediatric Urinary System 17

Fig. 21. Newborn with severe renal involvement with ARPKD. (A) Radiograph shows small, bell-shaped thorax
due to pulmonary hypoplasia. Note deep sulcus sign of right pneumothorax (arrowhead). Bilateral flank masses
(asterisk) push the bowel gas centrally. (B) Longitudinal US image of the left kidney shows diffusely echogenic
with poor corticomedullary differentiation. The renal length was 9.8 cm, much larger than normal for a newborn.
The right kidney (not shown) had a similar appearance.

 Medullary or subcortical round rather than masses share features with complex cysts and
tubular cysts result in a diagnostic dilemma. Cystic renal tumors
 Cysts appear late in the disease course include multilocular cystic renal tumors and cystic
Wilms tumors.
Cysts associated with malformation syndromes Multilocular cystic renal tumor refers to 2 patho-
Renal cysts may also be seen in numerous malfor- logically distinct but radiologically indistinguish-
mation syndromes, including aneuploidies (tri- able tumors: cystic nephroma and cystic partially
somy 21 and XO) and Beckwith-Wiedemann differentiated nephroblastoma (CPDN). These tu-
syndrome. Additionally, renal cysts are seen in mors are now considered part of the nephroblas-
other conditions often as part of ciliopathies that toma (Wilms tumor) spectrum analogous to the
also affect brain and skeletal development. These spectrum of neuroblastic tumors, with cystic
conditions include Meckel-Gruber syndrome, Zell- nephroma at the benign end and Wilms tumor at
weger syndrome, Joubert syndrome, Bardet-Biedl the malignant end.
syndrome, and several skeletal dysplasias, Cystic nephroma and CPDN demonstrate multi-
including asphyxiating thoracic dysplasia. ple predominantly thin septations (Fig. 26).
Cystic Renal Tumors  Cysts with thin wall and septations that may
Most solid renal tumors in young children older enhance
than 6 months are Wilms tumors, but cystic renal  No nodular components
 No solid enhancing components

Fig. 22. Coronal US image of the kidneys in a 1-day-


old boy with ARPKD shows markedly echogenic
medullae mimicking medullary nephrocalcinosis (ar- Fig. 23. Transverse US image using a high-frequency
row). Note dark halo of relatively spared cortex at transducer allows resolution of the tubular cysts in
the periphery (arrowhead). the near field (arrowheads).
18 Chung et al

Fig. 24. ARPKD in a newborn girl. (A) Longitudinal US image of the right kidney reveals increased echogenicity
and poor corticomedullary differentiation with renal enlargement compared with the spine (asterisk). A macro-
cyst is also seen (arrowhead). (B) Heterogeneous echotexture of the liver (L) is also noted.

Fig. 25. ARPKD in a 1-day-old boy. Longitudinal (A) and transverse (B) US images of the liver demonstrate dilated
biliary ducts (arrowheads). Echogenic medullae of the right kidney are seen (arrow). Curved arrow indicates the
aorta.

Fig. 26. Cystic nephroma in a 10-month-old boy. (A) Transverse US image reveals a mass composed of cysts with
fairly thin walls without nodularity or soft tissue mass (arrowhead). (B) Coronal T2-weighted image shows fluid
signal in most cysts and thin, hypointense septations (arrowhead). (C) Coronal T1-weighted image with fat satu-
ration after intravenous gadolinium chelate shows only mild enhancement of the septations (arrowhead).
Imaging of the Pediatric Urinary System 19

Fig. 27. Cystic-appearing anaplastic Wilms tumor in a 9-year-old boy. (A) Longitudinal US image shows a predom-
inantly cystic mass (arrow) with thick, nodular septations (arrowhead). Asterisk indicates adjacent kidney. (B) CT
following intravenous iodinated contrast demonstrates enhancement of the peripheral solid portions of the mass
(arrowhead) and internal vascularity (curved arrow). (C) Coronal T2-weighted image shows the predominantly
fluid-signal mass (arrow) containing some dark septations. Hypointense soft tissue nodules are seen at the periph-
ery (arrowhead). Asterisk indicates the adjacent kidney.

Demonstration of nodular septations or solid prospective studies in children, early retrospective


enhancing components suggest cystic Wilms tu- studies show good interobserver agreement and
mor, so contrast-enhanced imaging with CT or suggest that this system is useful in guiding man-
MR is necessary for full evaluation (Fig. 27).47 agement of children with solitary complex renal
Recently, some investigators described a modi- cysts.4850
fication of the Bosniak criteria for renal cysts adapt- Bosniak IIF criteria are included in other grades
ed for US and pediatric patients (Table 4).48,49 in the modified system because it applies to US
Although this system awaits validation with rather than CT. In the studies of Wallis and

Table 4
Modified Bosniak criteria for pediatric solitary renal cyst on ultrasound

Wall Septa
Grade Shape Thickness Nodules Flow Number Thickness Nodules Flow Calca Content
I Round 1 mm No No None N/A No No No Anechoic
II Lobulated 1 mm No No Few 1 mm No No No Debris
III N/A 1 mm No Yes Multipleb 1 mm No Yes Yes N/A
IV N/A N/A Yes N/A N/A N/A Yes N/A N/A Soft tissue

Abbreviations: Calc, calcification; N/A, not applicable.


a
Except for mobile intracyst stone.
b
Multiple defined as greater than 4.
Adapted from Karmazyn B, Tawadros A, Delaney LR, et al. Ultrasound classification of solitary renal cysts in children.
J Pediatr Urol 2015;11(3):149.e1-6.
20 Chung et al

colleagues49 and Peng and colleagues,50 Bosniak image acquisition, postprocessing, and mathemat-
IIF criteria (multiple septations and/or minimal ical modeling steps. J Magn Reson Imaging 2011;
septal thickening) were included in modified- 33(6):127083.
Bosniak grade II. As a result, multilocular cystic 8. Darge K. Voiding urosonography with US contrast
renal tumors were included in grade II prompting agent for the diagnosis of vesicoureteric reflux in
the investigators to recommend follow-up for children: an update. Pediatr Radiol 2010;40(6):
grade II cysts. On the other hand, Karmazyn and 95662.
colleagues48 included Bosniak IIF criteria in 9. RIVUR Trial Investigators, Hoberman A,
the modified-Bosniak grade III group, because Greenfield SP, et al. Antimicrobial prophylaxis for
cysts with these features should be further evalu- children with vesicoureteral reflux. N Engl J Med
ated with CT. In their study of 212 consecutive pa- 2014;370(25):236776.
tients with solitary cysts, they found no tumors in 10. Lebowitz RL, Olbing H, Parkkulainen KV, et al. Inter-
those classified as modified-Bosniak grade I or II. national system of radiographic grading of vesi-
They recommend no follow-up for asymptomatic coureteric reflux. International Reflux Study in
grade I or II cysts. For complex cysts (grade III or Children. Pediatr Radiol 1985;15(2):1059.
IV), further evaluation with 3-phase intravenous 11. Smellie JM, Normand IC. Bacteriuria, reflux, and
contrast-enhanced CT is recommended to eval- renal scarring. Arch Dis Child 1975;50(8):5815.
uate for enhancing components.48 12. Doganis D, Siafas K, Mavrikou M, et al. Does early
treatment of urinary tract infection prevent renal
damage? Pediatrics 2007;120(4):e9228.
SUMMARY
13. Smellie JM, Normand IC, Katz G. Children with uri-
Many aspects of pediatric genitourinary imaging nary infection: a comparison of those with and those
have undergone advances and/or evolution in the without vesicoureteric reflux. Kidney Int 1981;20(6):
past 10 years that have helped to decrease radia- 71722.
tion dose and resulted in changes in imaging and 14. Pohl HG, Belman AB. The top-down approach to
management of children with UTI and renal cystic the evaluation of children with febrile urinary tract
diseases. infection. Adv Urol 2009;783409.
15. Jakobsson B, Svensson L. Transient pyelonephritic
changes on 99mTechnetium-dimercaptosuccinic
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