Pe d i a t r i c I m a g i n g • R ev i ew

Mileto et al.
Imaging of Fetal Urinary Tract Anomalies

Pediatric Imaging
Review
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Fetal Urinary Tract Anomalies:

FOCUS ON:

Review of Pathophysiology,
Imaging, and Management
Achille Mileto1 OBJECTIVE. Common fetal anomalies of the kidneys and urinary tract encompass a
Malak Itani1 complex spectrum of abnormalities that can be detected prenatally by ultrasound. Common
Douglas S. Katz 2 fetal anomalies of the kidneys and urinary tract can affect amniotic fluid volume production
Joseph R. Siebert 3 with the development of oligohydramnios or anhydramnios, resulting in fetal pulmonary hy-
Manjiri K. Dighe1 poplasia and, potentially, abnormal development of other fetal structures.
CONCLUSION. We provide an overview of common fetal anomalies of the kidneys and
Theodore J. Dubinsky 1
urinary tract with an emphasis on sonographic patterns as well as pathologic and postnatal
Mariam Moshiri1 correlation, along with brief recommendations for postnatal management. Of note, we render
Mileto A, Itani M, Katz DS, et al. an updated classification of fetal abnormalities of the kidneys and urinary tract based on the
presence or absence of associated urinary tract dilation. In addition, we review the 2014 clas-
sification of urinary tract dilation based on the Linthicum multidisciplinary consensus panel.

he development of the fetal uri- well as pathologic and postnatal correlation,

T nary tract is a complex process

Keywords: amniotic fluid, congenital abnormalities,
cystic renal disease, fetal, ultrasound, urinary tract,
urinary tract dilation
doi.org/10.2214/AJR.17.18371
Received April 12, 2017; accepted after revision
October 27, 2017.
Based on a presentation at the Radiological Society of
North America 2015 annual meeting, Chicago, IL.
1
Department of Radiology, University of Washington
School of Medicine, Box 357115, 1959 NE Pacific St,
Seattle, WA 98195. Address correspondence to
A. Mileto (amileto@uw.edu).
2
Department of Radiology, NYU Winthrop Hospital,
Mineola, NY.
3
Department of Pathology, University of Washington,
Seattle, WA.
Supplemental Data
Available online at www.ajronline.org.
AJR 2018; 210:1–12
0361–803X/18/2105–1
© American Roentgen Ray Society
and is, therefore, associated with
an inherent substantial risk of
congenital defects. Fetal renal anomalies rep-
resent approximately 20% of all fetal congeni-
tal defects and are seen in 3–4% of pregnan-
cies. The frequency of prenatally detected
common fetal anomalies of the kidneys and
urinary tract is approximately 0.1–2.3% [1, 2].
Urine produced by the fetal kidneys is a
major contributor to the volume of amniot-
ic fluid in the second and third trimesters of
pregnancy. Therefore, any structural or func-
tional defects in the fetal urinary tract can
result in a substantial reduction of amniotic
fluid volume (oligohydramnios or anhydram-
nios), which, in turn, can cause fetal pulmo-
nary hypoplasia, as well as abnormal devel-
opment of several other fetal structures [3].
Fetal oligohydramnios or anhydramnios is
associated with a poor outcome, with a re-
ported mortality rate of 30–60% [4].
Accurate diagnosis of common fetal anom-
alies of the kidneys and urinary tract anoma-
lies is essential for parental counseling, ap-
propriate pregnancy management, and proper
delivery planning via a multidisciplinary
team approach, in which the radiologist plays
a major role. In this article, we review imag-
ing of fetal genitourinary tract abnormalities
with an emphasis on sonographic patterns, as
and we briefly discuss postnatal management.
In addition, we provide an updated classifi-
cation of fetal abnormalities of the kidneys
and urinary tract based on the presence or ab-
sence of associated urinary tract dilation. We
also review the 2014 classification of urinary
tract dilation based on the Linthicum multi-
disciplinary consensus panel.
Organogenesis
The urogenital system in the fetus origi-
nates from the intermediate mesoderm, which
develops into three early renal structures: the
pronephros, the mesonephros, and the meta-
nephros. Of these structures, the pronephros
is a transient excretory structure. The meso-
nephros regresses in male fetuses and gives
rise to some parts of the genital organs in fe-
male fetuses. The metanephros gives rise to
nephron units of the fetal kidneys. The ure-
teric buds give rise to the renal collecting sys-
tems, the major and minor calyces, the renal
pelves, and the renal collecting tubules. The
urogenital sinus gives rise to the urinary blad-
der. The fetal kidneys develop in the sacral re-
gion and then migrate to the renal fossa.
The fetal kidneys begin to make urine by
10 weeks of gestation and become function-
al by 12 weeks of gestation. By 14 weeks of
gestation, they become a major contributor to
the volume of amniotic fluid, with a minor

AJR:210, May 2018 1

 Mileto et al.
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A B
Fig. 1—Second-trimester female fetus with normal kidneys.
A, and B,Long-axis (A) and short-axis (B) ultrasound images show normal kidneys (between calipers, A) with
early corticomedullary differentiation. Note normal pelvicalyceal systems and renal pelves (arrowheads, B).
Aorta (a) and inferior vena cava (v) are seen anterior to vertebral body (b).
C, Coronal T2-weighted MR image shows normal position and appearance of fetal kidneys. RK = right kidney,
LK = left kidney.
C

contribution from the lungs, which produce
approximately one-third of the fluid. Neph-
ron development in the kidney continues un-
til birth. The number of nephrons then re-
mains static, although they increase in size
after birth [1].
Imaging and Normal Anatomy
Ultrasound is the primary imaging mo-
dality for the assessment of the fetal urinary
tract. The fetal kidneys can be identified by
approximately 10 weeks of gestation, espe-
cially on transvaginal sonography. In the
transverse plane, the fetal kidneys appear as
round hyperechoic structures adjacent to the
fetal spine (Fig. 1). In the longitudinal plane,
the fetal kidneys are elliptical. The kidneys
continue to grow throughout pregnancy, with
the renal length remaining directly propor-
tional to the gestational age. In later gesta-
tion, the renal medullary pyramids appear
more hypoechoic compared with the renal
cortexes. The ratio of fetal renal circumfer-
ence to abdominal circumference remains at
approximately 0.3 throughout the pregnancy
[5, 6]. The adrenal glands are seen superior
to the kidneys. In the longitudinal plane, they
are triangular, whereas they appear as oval
or tubular in the transverse plane. The adre-
nal medulla appears hyperechoic, surround-
ed by hypoechoic cortex [5, 6]. The fetal uri-
nary bladder is seen at approximately 10–12
weeks of gestation as a small fluid-filled
structure in the pelvis. The normal urinary
bladder voiding cycle is approximately every
55–155 minutes [7].
When additional information is needed,
or when ultrasound is limited because of the
absence of amniotic fluid, MRI is an excel-
lent diagnostic adjunct because of its abili-
ty to provide high-quality tissue contrast and
wider anatomic coverage, especially in com-
plex anomalies. The volume of amniotic flu-
id, fetal position, and maternal conditions are
typically not limitations of MRI, in contrast
to sonography. The normal fetal renal paren-
chyma is of intermediate signal intensity on
T2-weighted images, whereas the renal col-
lecting systems are of higher signal intensity.
The adrenal glands are low in signal inten-
sity in the suprarenal fossae. The fetal uri-
nary bladder is a high-signal-intensity round
or ovoid structure in the pelvis [8].
Classification of Fetal
Genitourinary Anomalies
Common fetal anomalies of the kidneys
and urinary tract encompass a broad spec-
trum of conditions with complex pathophysi-
ology and diverse clinical outcome and man-
agement implications [9]. Assessment of fetal
urinary tract anomalies can be approached
in various ways. One method entails divid-
ing the anomalies by their resultant chang-
es in renal size, whereas another approach
distributes them into categories according
to the presence or absence of associated uri-
nary tract dilation. In this article, we follow
the latter approach, because it is currently the
most commonly used classification.
Nonhydronephrotic Renal Anomalies: No
Urinary Tract Dilation
These structural abnormalities do not result
in dilation of the renal pelvis. Most of these
anomalies affect renal shape and position [5].
Abnormal Renal Number: Renal Agenesis
Renal agenesis refers to congenital ab-
sence of the kidney, which can be bilateral or
unilateral. Bilateral renal agenesis is a lethal
anomaly, occurring in 0.1–0.3 of 1000 live
births [9]. In a parent with a unilateral kid-
ney, the fetus has a 1% chance of developing
bilateral agenesis.
Bilateral renal agenesis can be an isolat-
ed anomaly but can also be seen in associa-
tion with other fetal syndromes and genetic
anomalies, including cardiovascular (15%)
and musculoskeletal (40%) malformations,
such as clubbed feet. Less commonly seen
associations include diaphragmatic hernia,
tracheoesophageal fistula, hydrocephalus,
myelomeningocele, and duodenal atresia.
The occurrence of renal agenesis with other
anomalies has been described as the VAC-
TERL association, which refers to the asso-
ciation of vertebral anomalies, anal atresia,
cardiac defects, tracheoesophageal fistula or
esophageal atresia, renal and radial anoma-
lies, and limb defects [10, 11].
Fetuses with bilateral renal agenesis dis-
play the Potter phenotypical sequence, which
is characterized by a receding chin, low-set
ears, widely spaced eyes with epicanthal
folds, and a broad nasal bridge. These facial
features are due to the paucity of amniot-
ic fluid, which in normal development cush-
ions the fetus’s face from direct trauma by the
uterine walls [12, 13]. The absence of both
kidneys and renal arteries can be detected in
the paravertebral regions at ultrasound (Fig.
2). In an attempt to occupy the uninhabited
renal fossae, the adrenal glands stretch lon-
gitudinally, taking an elongated shape (i.e.,

2 AJR:210, May 2018

 Imaging of Fetal Urinary Tract Anomalies

aging feature can be of clinical usefulness in

predicting the absence of the other kidney.

Abnormal Renal Position

Renal ectopia refers to any circumstance
in which the kidney is not positioned in the
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renal fossa. It is between the sixth and the

ninth weeks of gestation that the metaneph-
ros ascend to the renal fossae at the level of
the 12th thoracic vertebra [18]. Ectopia of the
kidneys, which occurs as a consequence of al-
teration at any stage of renal ascent, is usual-
ly defined as simple renal ectopia (as opposed
A B to cross-fused renal ectopia, which is covered
in the next section), because this condition is
the consequence of either a defective ascent,
most commonly resulting in a pelvic kidney
or, much less commonly, in an excessive cau-
docranial ascent into the thorax [19]. Simple
renal ectopia is seen in 1/1200 births. If not
promptly suspected, this anomaly can simu-
late unilateral renal agenesis [19].
Renal ectopia can be associated with other
genitourinary tract anomalies, including ob-
structive uropathy and reflux. Additionally,
similar to renal agenesis, other developmental
anomalies of the gynecologic, gastrointesti-
nal, cardiovascular, and musculoskeletal sys-
C D tems can also occur [14, 20, 21]. In cases of
Fig. 2—Male fetus with bilateral renal agenesis. renal ectopia, MRI can be useful to promptly
A, Oblique coronal gray-scale ultrasound image through abdomen and pelvis obtained at 23 weeks’ gestation
shows absence of both kidneys in paravertebral regions. Note adrenal glands (arrowheads).
locate the ectopic kidney and to differentiate
B, Coronal oblique color Doppler image through abdomen and pelvis obtained at 23 weeks’ gestation shows this condition from renal agenesis [21].
agenesis of both renal arteries.
C, Axial color Doppler image through fetal pelvis obtained at 29 weeks’ gestation shows lack of visualization of Abnormal Renal Size, Shape, or Position
urinary bladder due to absence of urine.
D, Postnatal ultrasound obtained at 1 day of life shows straightening of right adrenal posterior to hepatic As opposed to simple renal ectopia, fusion
parenchyma (arrowheads), compatible with lying-down adrenal gland. Neonate died at day 2 of life because of and malposition of the two kidneys can oc-
pulmonary hypoplasia. cur because of either abnormal position of
the umbilical artery or an aberrant ureteral
the lying-down appearance). In some cases, plasia is the main cause of death of newborns bud. Under such circumstances, the kidneys
the elongated adrenal glands can assume an with renal agenesis and the Potter sequence assume various abnormal shapes, including
ovoid shape, simulating the appearance of [14, 15]. Hypoplasia or absence of the uri- horseshoe kidney, crossed-fused renal ecto-
the kidneys. Severe oligohydramnios is usu- nary bladder is another expected feature in pia, and pancake kidney [22].
ally seen in the setting of bilateral renal agen- the setting of bilateral renal agenesis [1, 16]. Horseshoe kidney is the most common
esis. Oligohydramnios tends to be more evi- Unilateral renal agenesis is 4–20 times fusion abnormality, estimated at a rate of
dent after 16 weeks of gestation, because the more common than bilateral renal agenesis 1/500 births (i.e., approximately 90% of all
initial urine production by the placenta pro- and conveys a favorable prognosis [14, 15]. In renal morphologic abnormalities). Horse-
gressively declines by then, without viable the case of unilateral agenesis, the empty re- shoe configuration results from two normal-
kidneys being able to undertake this function. nal fossa with absence of the ipsilateral renal ly functioning kidneys positioned on either
Pulmonary hypoplasia with a small tho- artery can be revealed by ultrasound and col- side of the midline and are connected by a
rax is another feature commonly associated or Doppler imaging. A hypertrophied contra- parenchymal or fibrotic bridge. Most com-
with bilateral renal agenesis and the Potter lateral kidney supplied by a normal renal ar- monly, the parenchymal or fibrotic isthmus
sequence [12, 13]. Although the pathophysi- tery is usually seen (Fig. S1 can be viewed in connects the lower renal poles, with a con-
ologic mechanism causing pulmonary hypo- the AJR electronic supplement to this article, nection between the upper renal poles be-
plasia has not been entirely elucidated, to our available at www.ajronline.org). Cho and col- ing much less common [22, 23]. A horse-
knowledge, it is hypothesized that the absent leagues [17] suggested that, when the compen- shoe kidney can be challenging to diagnose
flow of amniotic fluid into the lungs along satory hypertrophy of the contralateral kidney at prenatal ultrasound because only a thin
with limited space in the fetal thorax impairs results in anteroposterior-to-transverse diam- parenchymal band is usually present (Fig.
pulmonary development. Pulmonary hypo- eter ratio greater than or equal to 0.9, this im- S2 can be viewed in the AJR electronic sup-

AJR:210, May 2018 3


plement to this article, available at www.­
ajronline.org). Urinary tract dilation can be
seen in one or both renal moieties.
Cross-fused renal ectopia is the second
most common fusion anomaly, with a fre-
quency of 1/7500 births [22]. This condi-
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tion refers to fusion of the two kidneys on
the same side of the spine. The ureter of
the ectopic kidney crosses the midline and
joins the urinary bladder at the orthotopic
site. Depending on the fusion site and final
renal shape, different variants of crossed-
fused renal ectopia are described in the lit-
erature, including type A (inferior crossed
fusion), type B (sigmoid kidney), type C,
(lump kidney), type D, (disc kidney), type
E, (L-shaped kidney), and type F (superior-
ly crossed-fused). Type A is the most com-
monly seen variant, where there is fusion
between the upper pole of the cross-fused
ectopic kidney (located more inferiorly rela-
tive to the other kidney) and the lower pole
of the other orthotopic kidney (located in a
more superior position) [22, 23].
Pancake kidney is an extremely rare ab-
normality of renal shape caused by complete
fusion of the two kidneys in the pelvis, with
two distinct collecting systems joining the
urinary bladder on either side of the mid-
line [24]. Occasionally, the pancake kidney
is drained by a single ureter [25, 26].
Hydronephrotic Renal Disease:
Urinary Tract Dilation
Dilation of the urinary tract is seen in
1–2% of all pregnancies and is estimated to
affect approximately 40,000–80,000 new-
borns in the United States every year [27].
Although urinary tract dilation is only tran-
sient or physiologic in 50–70% of cases, it
can also reflect a wide variety of underly-
ing pathologic conditions. The latter in-
clude ureteropelvic junction obstruction
(10–30%), vesicoureteral reflux (10–40%),
ureterovesical junction obstruction or mega-
ureter (5–15%), multicystic dysplastic kid-
ney (MCDK) disease (2–5%), and posteri-
or urethral valves (1–5%). Less common are
ectopic ureter and ureterocele in the setting
of collecting system duplication, polycystic
kidney disease, and prune-belly syndrome
[28]. Prenatal identification of the aforemen-
tioned conditions is of critical importance
to prevent the development of complications
(e.g., urinary tract infection, renal calculi,
and renal failure) and to allow planning of
postnatal management strategies in a timely
manner [28].
4 AJR:210, May 2018
Mileto et al.
Classification of Urinary Tract Dilation
Historically, there has been a lack of cor-
relation between prenatal and postnatal ul-
trasound findings of urinary tract dilatation,
with inconsistency in description, classifica-
tion, and grading, which ultimately affects
clinical assessment and treatment planning.
For this reason, a consensus meeting on uri-
nary tract dilation classification system was
convened in March 2014 in Linthicum, MD,
that was attended by representatives from
eight major scientific societies [27]. The con-
sensus panel had a twofold objective: to pro-
pose a unified description of urinary tract
dilation prenatally and postnatally and to de-
velop a standardized framework for prenatal
evaluation of isolated urinary tract dilatation
based on ultrasound findings, which can be
applied in the postnatal period.
In an attempt to avoid confusion, the con-
sensus panel agreed on specific terminology
to be used. Of note, the consistent adoption
of the term “urinary tract dilation” was rec-
ommended, whereas the use of other com-
monly used clinical terms (e.g., “hydrone-
phrosis,” “pyelectasis,” and “pelviectasis”)
was discouraged [27]. In addition, owing to
the emerging substantial variability among
grading systems traditionally used—descrip-
tive (e.g., mild, moderate, or severe), quantita-
tive (e.g., grades 1–5 based on anteroposteri-
or renal pelvic diameter), or semiquantitative
(e.g., the Society for Fetal Urology system)—
the consensus panel underscored the need
for using a single widely accepted method
to be used for describing pre- and postnatal
ultrasound findings. Therefore, the panel es-
tablished a standardized checklist of sono-
graphic features to report anteroposterior re-
nal pelvic diameter measured on transverse
images at maximal diameter of the intrare-
nal pelvis; calyceal dilation, either central
(major) or peripheral (minor); parenchymal
thickness, to be reported with a subjective
assessment; parenchymal appearance, in-
cluding the evaluation of echogenicity, the
presence of cortical cysts, and corticomedul-
lary differentiation; ureter description, in-
cluding transient visualization and normal
versus abnormal dilation; and urinary blad-
der description, including the assessment of
wall thickness, and the presence of a uretero-
celes or posterior urethral valves [27].
The 2014 Linthicum consensus panel also
issued a risk stratification model to be used
for both pre- and postnatal urinary tract dila-
tion [27]. On the basis of the aforementioned
risk stratification models, a scheme for man-
agement was also developed [27]. Overall,
the 2014 Linthicum recommendations pro-
vide added value because they are based on
a thorough analysis of the existing evidence-
based literature, as well as the opinion of au-
thorities reflecting the accepted standard of
care in clinical practice.
Renal Pelvis Obstruction
Obstruction of the renal pelvis at the level
of the ureteropelvic junction is the most ac-
cepted explanation for fetal urinary tract di-
lation [14, 16, 29]. Of note, obstruction of the
ureteropelvic junction is estimated to repre-
sent approximately 50% of all urinary tract
abnormalities seen in newborns [14]. This is
usually a sporadic unilateral (90% of cases)
anomaly due to aberrant collagen or fibrotic
bands at the level of the ureteropelvic junc-
tion. However, kinking of the ureteral course,
the presence of ureteral valves, aberrant ves-
sels, and abnormal insertion of the ureter
with varying configurations of the pelviure-
teral outlet have also been described as pos-
sible causes. In a minority of cases (10–30%),
obstruction of the ureteropelvic junction can
be bilateral, often in association with other
nongenitourinary abnormalities (e.g., esoph-
ageal atresia, Hirschsprung disease, and im-
perforate anus) [14].
Fetal ultrasound shows dilation of the re-
nal pelvis with or without dilation of renal
calyces; the degree of dilation depends on
the type and severity of the obstruction. In
high-grade severe obstruction, development
of perirenal urinomas and ascites can be ob-
served. This explains why both oligohydram-
nios and polyhydramnios can be seen in the
setting of ureteropelvic junction obstruction;
however, oligohydramnios is exceedingly
rare. If left untreated, long-standing obstruc-
tion can lead to increased echogenicity and
cystic changes associated with atrophy of the
renal parenchyma at ultrasound [14, 16, 29].
Overall, the prognosis of ureteropelvic junc-
tion obstruction depends on many factors,
including the presence or absence of other
anomalies, amniotic fluid volume, the pres-
ence or absence of a urinoma, and cystic dys-
plasia of the ipsilateral kidney [14, 16, 29].
Ureteral Obstruction
Dilation of the urinary tract can also be due
to obstruction at the level of the ureterovesical
junction. This condition, which is more com-
mon among male than female patients, has a
familial pattern of inheritance and is usually
due to occurrence of megaureter or stenosis of
 Imaging of Fetal Urinary Tract Anomalies
the ureterovesical junction as a result of dys-
plastic or aberrant smooth muscle with fibro-
sis. Megaureter, by definition, is a dilated ure-
ter with or without coexistent dilation of the
renal pelvis and calyces and refers to a con-
genital anomaly of the ureterovesical junction.
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Primary megaureter, also known as congen-
ital megaureter, is bilateral in 20% of cases
with contralateral genitourinary abnormal-
ities in 5% of cases. Although this anomaly
can be identified during the fetal period, de-
finitive diagnosis of primary megaureter can
be made only postnatally [30]. Primary mega-
ureter can be classified into three subcatego-
ries: obstructive, refluxing, and nonrefluxing
unobstructed. The obstructive megaureter
is characterized by ureteral dilation proxi-
mal to a short aperistaltic juxtavesical por-
tion of the ureter with a normal urinary blad-
der. The refluxing megaureter is usually due
to a small or absent portion of the intravesical
ureter, whereas the nonrefluxing unobstructed
megaureter refers to lack of obstruction or re-
flux with dilatation of the ureter proximal to
the level of the urinary bladder [14, 31]. The
nonrefluxing unobstructed variant is the most
common subtype (Fig. 3).
In all cases of ureteral obstruction, with or
without megaureter, ultrasound shows a dilat-
ed fetal ureter proximal to the ureterovesical
junction. The dilated ureter is displayed as an
anechoic elongated structure with a tortuous
course, which can be followed throughout its
entire length from the renal pelvis to the ure-
terovesical junction [31]. Postnatal evaluation
with MRI may be warranted to better char-
acterize the type of megaureter, as well as to
monitor dilation of the pelvicalyceal system
and renal parenchymal abnormalities (e.g.,
cortical thinning and scarring) [32]. Most pa-
Fig. 3—3-month-old boy with primary megaureter.
A, Sagittal ultrasound image of right kidney (between calipers) shows parenchymal thinning (arrowheads) and pelvicalyceal dilation.
B, Sagittal ultrasound image at level of right ureterovesical junction shows tapered narrowing of distal right ureter, consistent with primary megaureter.
AJR:210, May 2018 5
tients with primary megaureter can be man-
aged conservatively, because this anomaly
usually resolves spontaneously by the age of
7 years. If the anomaly does not resolve, in
the minority of such patients the treatment is
surgery, with resection of the aperistaltic seg-
ment and ureteral reimplantation.
Other causes of obstruction of the ure-
terovesical junction include an ectopic ure-
ter with or without a ureterocele. An ectopic
ureter is the result of the aberrant migration
of the ureteral bud, usually with caudal ec-
topia of the ureteral insertion, which can be
into the lower portion of the urinary blad-
der, urethra, vestibule, or vagina in female
patients, or in the seminal vesicles, vas def-
erens, or ejaculatory ducts in male patients.
Often, especially if the ectopic ureter is as-
sociated with a duplicated ureter, imaging
shows a thin-walled cystic structure arising
from the distal portion of the ureter project-
ing into the urinary bladder lumen (i.e., ure-
terocele) [14, 31]. Postnatal MR urography
can be useful to exactly depict the course
and termination of the ectopic ureter within
a ureterocele [21].
Vesicoureteral Reflux
Vesicoureteral reflux consists of the back-
ward flow of urine from the urinary bladder
into the ureters and kidneys due to abnormal
maturation of the vesicoureteral antireflux
mechanism [33]. Normally, a 7- to 12-mm
portion of the distal ureter enters the urinary
bladder wall obliquely at the vesicoureteral
junction, thus serving as a continence valve,
to prevent backward flow of urine from the
urinary bladder. If the maturation of the ure-
terovesical junction is incomplete, an abnor-
mally short intravesical ureteral segment
A B
results, causing failure of the continence
mechanism [33–35]. Although vesicoureteral
reflux can be also seen in the setting of com-
plex genitourinary abnormalities (e.g., du-
plication of the collecting system), it is more
commonly an isolated condition.
The retrograde flow of urine into the ure-
ters and kidneys (the latter is termed “in-
trarenal reflux”) increases the risk of upper
urinary tract infection caused by ascending
bacteria and can result in renal parenchymal
scarring and dysfunction. Early diagnosis of
vesicoureteral reflux is important to prevent
renal damage. Vesicoureteral reflux accounts
for up to 40% of cases of urinary tract dila-
tion detected prenatally at ultrasound [31]. In
the absence of other anomalies, prenatal doc-
umentation of urinary tract dilation should
raise the suspicion for vesicoureteral reflux,
therefore warranting postnatal evaluation.
Precise grading of vesicoureteral reflux is
based on voiding cystourethrography, which
should be promptly performed after the first
episode of urinary tract infection. According
to the International Reflux Committee Study
System [36], vesicoureteral reflux is classi-
fied as grade 1 when the backward urine flow
involves the ureter only; grade 2 when ure-
ter and renal collecting system are involved,
but without dilatation; grade 3 when there is
moderate dilation of the ureter and renal col-
lecting system, with no or slight blunting of
the caliceal fornices; grade 4 when there is
moderate dilation of the ureter and renal col-
lecting system with an associated tortuous
course, and complete blunting of the caliceal
fornices with maintenance of papillary im-
pressions; and grade 5 when there is marked
dilation of the ureter and renal collecting
system with an associated tortuous course,
 Mileto et al.
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A B

C D

E F
Fig. 4—Two fetuses with duplicated renal collecting systems.
A–F, Sagittal ultrasound image of left kidney (between calipers, A) and transverse image of urinary bladder (B) obtained at 18 weeks’ gestation show dilation of left
pelvicalyceal system (asterisk, A) and ureterocele in urinary bladder (arrowheads, B). Ultrasound images were obtained again at 23 weeks’ gestation (C and D). Upper
pole of left kidney (between calipers, C) shows cortical atrophy and cystic changes (arrows, C), and lower pole shows pelvicalyceal dilation (asterisks, C). Urinary bladder
ureterocele is now more conspicuous at site of ureteropelvic junction (arrowheads, D), and left ureter is dilated (between calipers, D). Postnatal sagittal ultrasound image
obtained at day 2 of life (E) shows cystic dysplasia of upper pole of left kidney (arrows, E), which is related to early high-grade in utero obstruction. There is pelvicalyceal
dilation in lower pole (asterisks, E). Ultrasound image of urinary bladder (F) shows left ureterocele Sex of fetus is unknown.

(Fig. 4 continues on next page)

6 AJR:210, May 2018

 Imaging of Fetal Urinary Tract Anomalies
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G H
Fig. 4 (continued)—Two fetuses with duplicated renal collecting systems.
G, 2-day-old infant (sex unknown, same as in A–F). Fluoroscopic retrograde cystourethrogram shows large filling defect in urinary bladder (arrowheads), corresponding
to known ureterocele.
H, 3-month-old boy who had bilateral ureteral and collecting system duplication. Photograph of pathologic specimen is shown. Patient also had cardiofaciocutaneous
syndrome from underlying MEK2 mutation.

with complete blunting of the caliceal forni-
ces and papillary impressions.
Although voiding cystourethrography and
cystoscintigraphy have been historically
used to diagnose and grade vesicoureteral re-
flux, contrast-enhanced sonography (also re-
ferred to as “voiding urosonography”) is be-
ing increasingly adopted. Owing to advances
in tissue-harmonic and second-generation
ultrasound contrast agents, this technique
renders an accurate diagnosis and permits
long-term follow-up of patients with vesico-
ureteral reflux without exposure to ionizing
radiation. Ascenti and co-workers [37] re-
ported that voiding urosonography improved
diagnostic accuracy for vesicoureteral reflux
grading compared to cystoscintigraphy.
Renal Duplication
Duplication of the collecting system,
also termed “renal duplication” or “ureter-
al duplication,” is a common developmental
anomaly. It is estimated to occur in approxi-
mately 1% of the newborn population and is
identified in 10% of children with recurrent
urinary tract infections. Renal duplication is
more common among female than male pa-
tients (2:1 ratio) and is bilateral in 40% of pa-
tients. This anomaly can be isolated or, much
less commonly, is associated with complex
syndromes, including the cardiofaciocutane-
ous syndrome (cardiac abnormalities, cra-
niofacial features accompanied by xerosis,
hyperkeratosis, and ichthyosis) [31, 38, 39].
A critical step in renal development is rep-
resented by the encounter of the metanephric
blastema with the ureteral bud, with the latter
originating from the mesonephric Wolffian
ducts. Normally, one ureteral bud joins the
metanephric blastema, giving rise to a nor-
mal kidney with a single collecting system.
Renal duplication, which can be incomplete
or complete, occurs when more than one ure-
teral bud meets the metanephros [31, 33, 39].
Incomplete duplication is the result of bi-
furcation of a single ureteral bud joining the
metanephric blastema with two branches, at
either the renal pelvis or the ureter. In the
former case, the incomplete duplication is
termed a “bifid pelvis,” and in the latter case,
it is termed a “bifid ureter.” In either type of
incomplete duplication, there is one ureter
distally joining the urinary bladder. In rare
instances, one ureteral ramification can ter-
minate in a blind pouch [31, 38].
Complete renal duplication occurs when two
distinct ureteral buds join a single metanephros.
Under this circumstance, there will be two ure-
ters separately traveling from the renal pelvis
to urinary bladder. The ureter arising from the
lower portion of the renal pelvis usually joins
the urinary bladder orthotopically at one cor-
ner of the vesical trigone. In contrast, the ureter
originating from the upper pole of the collect-
ing system joins the urinary bladder wall inferi-
or and medial to the orthotopic ureteral meatus
(i.e., the Weigert-Meyer rule). The upper pole
ureter most often terminates in a ureterocele,
whereas vesicoureteral reflux is more common-
ly seen in the ureter draining the lower pole re-
nal moiety, although there is variability of these
findings in individual patients [31, 38].
Most commonly, ultrasound findings in-
clude urinary tract dilation involving the up-
per pole system, with a normal appearance
of the lower pole moiety (Fig. 4). In the case
of long-standing upper pole urinary tract di-
lation, atrophy of the upper pole renal pa-
renchyma can be seen. When the complete
duplication is further complicated by devel-
opment of a ureterocele involving the upper
pole system, this ureterocele (i.e., bladder-
within-bladder appearance), with varying
degrees of ureteral dilation, can be identified
at imaging [14, 31, 38] (Fig. 4).
Fetal MRI with urographic (i.e., fast heav-
ily T2-weighted multiplanar) pulse sequences
can be used to confirm the diagnosis of renal
duplication, to assess the renal parenchyma
for concurrent dysplastic changes, and to de-
termine the level of obstruction, especially
in cases of ureterocele [21, 32]. Prenatal de-
tection or high suspicion of renal collecting
system duplication mandates further postna-
tal imaging with ultrasound and voiding cys-
tourethrography [31, 38, 39]. Postnatal MR
urography can be a useful complement to bet-
ter delineate the urinary tract anatomy, par-
ticularly the ureteral course and insertion [32].

AJR:210, May 2018 7

 Mileto et al.

Cystic Renal Disease
Cystic renal disease comprises a heteroge-
neous group of renal parenchymal disorders
that can be hereditary, acquired, or develop-
mental in origin. In some cases, obstructive
uropathy can be seen in the setting of cystic
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etiology [40–42]. Among inherited renal cys-
tic diseases, the ciliopathies—also known as
hereditary defects of primary nonmotile cil-
ia—have been increasingly implicated in re-
nal developmental abnormalities, including
ARPKD and ADPKD. Renal stigmata of cil-
Cysts can develop both in the renal cortex
and medulla, while the kidney itself main-
tains its excretory function (Fig. S3 can be
viewed in the AJR electronic supplement to
this article, available at www.ajronline.org).
Oligohydramnios and a nonvisualized uri-

renal disease.
The Potter classification (Potter types I–
IV) has been historically used to categorize
the different types of cystic renal disease:
Potter type I, infantile polycystic kidney dis-
ease, is now more commonly termed “auto-
somal recessive polycystic kidney disease”
(ARPKD); Potter type II, cystic dysplas-
tic kidney disease, is now more commonly
termed “multicystic dysplastic kidney dis-
ease” (MCDK); Potter type III is now termed
“autosomal dominant polycystic kidney dis-
ease” (ADPKD); and Potter type IV is now
termed “partial or intermittent urinary out-
flow obstruction” or “obstructive dysplasia.”
The Potter category system has been super-
seded by differentiation of the various con-
ditions on the basis of genetic or nongenetic
iopathies can be associated with hepatic (i.e.,
hepatorenal cystic disease spectrum), skele-
tal, and CNS disorders [40, 41].
Autosomal Recessive Polycystic Kidney Disease
(Formerly Potter Type I)
ARPKD is the most common renal paren-
chymal abnormality seen in utero and is among
the most frequently encountered cystic diseas-
es in the newborn. This condition is caused by a
mutation of chromosome 6p12, causing abnor-
mal ciliary signaling pathway and aberrant dif-
ferentiation of the renal tubules epithelial cells.
This condition is transmitted via autosomal re-
cessive inheritance [41].
ARPKD is manifest phenotypically with
many rounded cysts, ranging in size between
a few millimeters up to several millimeters.
nary bladder can also be seen on imaging.
Kidneys in patients with ARPKD typically
appear diffusely enlarged and hyperintense
on T2-weighted MR images [21]. A streaky
enhancement pattern can be seen after IV
gadolinium administration. Hepatic involve-
ment is also commonly noted with multiple
hepatic cysts [41]. As a rule of thumb, the
degree of renal and hepatic involvement has
an inverse relationship. Portal hypertension,
hepatic parenchymal fibrosis, and pulmo-
nary hypoplasia can be observed in the set-
ting of ARPKD, with pulmonary hypoplasia
leading to a higher rate of perinatal mortal-
ity. ARPKD can be diagnosed with ultra-
sound at 16–18 weeks of gestation, but usu-
ally manifests itself in the third trimester.
Treatment of ARPKD is supportive, with di-

A B C

D E
Fig. 5—Two fetuses with Meckel-Gruber Syndrome.
A–D, Transverse ultrasound image through abdomen obtained at 17 weeks’ gestation (A) shows bilateral enlarged kidneys with multiple
cysts. Sagittal ultrasound image (B) shows massive enlargement of right kidney, which is extending from lower chest to pelvis. There is
oligohydramnios. Transverse ultrasound image through skull (C) shows occipital encephalocele (arrowhead, C). Ultrasound shows that there
are at least 6 fingers on left hand (D). Autopsy confirmed polydactyly with rudimentary fingers. Sex of fetus is unknown.
E, 20 weeks’ gestation male fetus with Meckel-Gruber syndrome. Photograph of pathologic specimen shows marked enlargement of bilateral
kidneys with innumerable cysts.

8 AJR:210, May 2018


alysis and renal transplant usually required
later in life [14, 40, 42].
Multicystic Dysplastic Kidney Disease
(Formerly Potter Type II)
MCDK is among the most common causes
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of end-stage renal disease and renal failure in
the newborn. This condition is thought to result
from early ureteral or pelvoinfundibular atresia
(at 8–10 weeks), with subsequent development
of multiple noncommunicating cysts in the kid-
neys. At imaging, the kidneys lose their reni-
form shape and appear as paraspinal masses
containing multiple noncommunicating cysts
(Fig. S4 can be viewed in the AJR electronic
supplement to this article, available at www.
ajronline.org). On the basis of the overall size
of the kidneys, this condition was initially sub-
categorized as Potter type IIA (i.e., enlarged) or
type IIB (i.e., atrophic or normal). MCDK can
also have focal or segmental distribution pat-
terns, with unilateral or bilateral (only in 20%
of cases) renal involvement [14]. Occasion-
ally, absence or atresia of the renal artery can
be seen. The prognosis of unilateral MCDK
is excellent, with natural involution of the af-
fected kidney and development of compensa-
tory hypertrophy of the contralateral kidney
[43]. Nevertheless, MCDK can be fatal when it
involves both kidneys or when it is associated
with T13 or T18 chromosomal aneuploidies or
other complex fetal syndromes, including Ap-
ert syndrome or Meckel-Gruber syndrome [14,
40]. In Meckel-Gruber syndrome, vermian hy-
poplasia, occipital encephalocele, polydactyly,
omphalocele, oligo- to anhydramnios, inter-
rupted aortic arch, liver ductal plate abnormal-
ities, placentomegaly, and hypoplastic urinary
bladder can also be seen [14, 40, 44] (Fig. 5).
At MRI, kidneys of patients with MCDK show
multiple noncommunicating T2-hyperintense
cysts of different sizes [21]. Although a mild
degree of parenchymal enhancement can be
seen after IV gadolinium administration, no
opacification of the collecting system is typi-
cally observed [32].
Autosomal Dominant Polycystic Kidney
Disease (Formerly Potter Type III)
ADPKD is the most common type of cys-
tic renal disease and is inherited via autoso-
mal dominant trait mutations of the PKD1
gene on chromosome 16p or of the PKD2
gene on chromosome 4q, which cause aber-
rant production of polycystin-1 or polycys-
tin-2, proteins that are responsible for cal-
cium channel interactions on the surface of
renal tubules cilia. Abnormal polycystin-1 or
AJR:210, May 2018 9
Imaging of Fetal Urinary Tract Anomalies
polycystin-2 affects the physiologic mecha-
nisms of differentiation and repair of renal
tubules [41]. At sonography, ADPKD in the
fetus or newborn can present with either nor-
mal-appearing or hyperechoic kidneys. How-
ever, detection in adults is the most common
manifestation, when enlarged kidneys are
noted with many cysts of varying size. He-
patic and seminal vesicles cysts, as well as
intracranial aneurysms, are other common
features of ADPKD. Prenatal ultrasound
screening is valuable in the diagnosis of this
condition [14, 45]. MRI is increasingly being
used in patients with ADPKD to monitor re-
nal cysts and renal parenchymal volume. The
latter has been advocated as a potential met-
ric to quantify disease progression [41].
Obstructive Dysplasia (Formerly Potter Type IV)
This condition is caused by early paren-
chymal abnormality resulting from urinary
obstruction at the ureteropelvic, ureterovesi-
cal, or urinary bladder outlet levels. Although
this condition can mimic MCDK disease, the
identification of urinary obstruction is the
key to the diagnosis and has critical prognos-
tic implications. The latter depends on the
timing of detection of the obstruction (i.e.,
early vs late), with long-standing obstruction
causing irreversible parenchymal damage,
possibly complicated with urinomas, oligo-
hydramnios, and ascites, resulting in a poor
prognosis. Common ultrasound findings in
obstructive dysplasia are small echogenic yet
reniform kidneys, with cortical cysts and oli-
gohydramnios. Although this condition can
be an isolated anomaly, an association with
cardiac abnormalities or the VACTERL se-
quence can also be seen [14, 40].
Urinary Bladder Outlet Obstruction
Obstruction of the urinary bladder outlet
can occur as a result of many anatomic ab-
normalities, most commonly posterior ure-
thral valves (only in male patients), urethral
atresia (only in female patients), cloacal atre-
sia, megacystis-microcolon-intestinal hypo-
peristalsis syndrome (typically in female pa-
tients, although not a true obstruction), and
prune-belly syndrome (typically in male pa-
tients, although also not a true obstruction)
[14, 31, 46, 47].
Posterior urethral valve is characterized
by a fibrotic diaphragm embryologically de-
riving from Wolffian ducts, which travels
obliquely from the verumontanum through
the prostatic portion of the urethra. Three
subtypes of posterior urethral valves were in-
cluded in the Young classification; however,
only the Young type 1 is recognized to exist
today. In this category, the rudimental mem-
brane becomes progressively stiffer, causing
urinary bladder outlet obstruction with dis-
tention of the posterior urethra (i.e., the key-
hole appearance at ultrasound) and thick-
ening of the urinary bladder wall, with or
without ureteral dilation (Fig. 6). Complica-
tions that can be identified at imaging include
formation of urinoma and urinary ascites, uri-
nothorax, peritoneal calcifications, and renal
dysplasia. Detection of a posterior urethral
valve warrants postnatal imaging with void-
ing cystourethrography. Treatment of con-
firmed posterior urethral valve requires endo-
scopic ablation of the membrane [31, 46, 47].
Urethral atresia refers to the congenital
absence of the urethra, usually observed in
the setting of urinary bladder agenesis and
prune-belly syndrome. Posterior urethral
valves and urethral atresia are the two most
common conditions causing fetal lower uri-
nary tract obstruction, which may not be
compatible with life, depending on the se-
verity and associated findings, unless an al-
ternate urinary pathway is created between
the urinary bladder and the amniotic sac. In
this setting, urinary drainage from the uri-
nary bladder through vesicocentesis or cre-
ation of an intrauterine vesicoamniotic surgi-
cal shunt have been advocated to bypass the
urethral blockage and decompress the col-
lecting systems, thereby avoiding renal im-
pairment, development of oligohydramnios,
and pulmonary hypoplasia [48]. Percutane-
ous creation of a vesicoamniotic shunt is the
most commonly performed procedure to by-
pass the urethral obstruction [40, 49]. This
procedure consists of ultrasound-guided
placement of a double-ended pigtail cathe-
ter (i.e., either a Harrison or Rodeck-Rock-
et catheter) with its distal and proximal tips
within the fetal urinary bladder and amniotic
cavity, respectively [49, 50]. Less common-
ly used procedures to attempt to repair lower
urinary tract obstruction are fetal cystosco-
py with ablation of posterior urethral valves
and open fetal surgery with fetal vesicosto-
my [49, 50]. Owing to the inherently highly
invasive nature and high frequency of mater-
nal and fetal complications, these procedures
are performed at only a few specialized cen-
ters, with the yield of vesicocentesis remain-
ing widely controversial and debated in the
literature, to our knowledge [49, 50].
Cloacal atresia represents a condition in
which the urinary, genital, and gastrointes-

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tinal systems all open into a perineal me-
atus with a normal abdominal wall [31]. This
anomaly, which is seen exclusively in fe-
male patients, is embryologically different
than urinary bladder exstrophy (which can
be seen in either sex), in which persistence
of the rudimentary cloacal membrane affects
closure of the inferior abdominal wall with
extrusion of the urinary bladder [31].
Megacystis-microcolon-intestinal hypope-
ristalsis syndrome is a rare congenital disease
that mainly affects female patients (70%).
Severe abdominal distention is caused by a
markedly dilated but unobstructed urinary
bladder (i.e., megacystis) due to absent or ab-
normal urinary bladder wall motility. Colonic
and small-bowel peristalsis are nearly absent
in this condition as well [51].
Prune-belly syndrome (also known as Ea-
gle-Barrett syndrome) is a condition where
the absence of the rectus abdominis muscu-
lature causes laxity and outward protrusion
of the anterior abdominal wall, accompanied
with multisystem anomalies. Urinary tract
involvement is usually characterized by re-
nal parenchymal abnormalities with severely
dilated ureters, urinary bladder, and urethra
(i.e., megalocystic). Gastrointestinal, cardio-
respiratory, and musculoskeletal involve-
ment can also be seen [52].
Fetal Renal and Adrenal Masses
Mesonephric blastoma is the most com-
mon fetal renal neoplasm. It is considered a
benign hamartoma without associated anom-
10 AJR:210, May 2018
Mileto et al.
A B
alies. At ultrasound, it appears as complex
solid or multicystic mass that is associated
with polyhydramnios [53–56]. The appear-
ance of the mesonephric blastoma at MRI is
nonspecific; however, T1-hyperintense foci
can be identified because of intratumoral
hemorrhage. Restricted diffusion can be ob-
served within the solid component of meso-
nephric blastomas [57].
Adrenal neuroblastoma is the most com-
mon fetal malignancy and is usually uni-
lateral. Prenatal diagnosis can be challeng-
ing; however, this tumor must be suspected
when maternal symptoms due to increased
catecholamines are present. A prenatal adre-
nal neuroblastoma appears as an echogenic
mass at sonography located just cephalad to
the kidneys, separate from the liver and the
spleen [58]. The Oreo-cookie appearance,
which is seen in normal adrenal glands or
adrenal hyperplasia, is not identified in this
condition. At MRI, adrenal neuroblastoma
appears as complex heterogeneous mass with
mixed signal intensity characteristics due to
the coexistence of solid components and cys-
tic spaces. In some cases, adrenal neuroblas-
toma can be highly aggressive, with a ten-
dency to rapidly metastasize to the kidneys
and to the spine and other osseous and non-
osseous structures. MRI in general is pref-
erable for detection of metastases [53]. Fetal
hydrops can be seen in the setting of fetal ad-
renal neuroblastoma [59].
Adrenal hemorrhage is rarely seen in ute-
ro but is usually observed in the setting of
Fig. 6—Two patients
with posterior urethral
valve.
A, Second trimester
male fetus. Transverse
ultrasound image of
urinary bladder shows
dilated posterior
urethral valve, which is
also known as keyhole
appearance of urinary
bladder. This is typically
seen in urinary bladder
outlet obstruction.
B, 8-day-old boy.
Photograph of postnatal
pathologic specimen
of urinary tract is
shown. Infant had
severe obstruction
from posterior urethral
valve, with subsequent
dysplasia of both
kidneys.
uterine ischemic events (e.g., difficult labor
or delivery, asphyxia, or septicemia) or hem-
orrhagic disorders [60]. Adrenal hemorrhage
can be seen in newborns affected by the
Beckwith-Wiedemann syndrome, a congen-
ital overgrowth syndrome characterized by
hemihypertrophy, visceromegaly, Wilms tu-
mor, macrosomia, macroglossia, nevus flam-
meus, and hypoglycemia [48]. At prenatal
ultrasound, the adrenal glands appear thick-
ened, masslike, and echogenic, especially
in the acute phase of hemorrhage. With ag-
ing of the hemorrhage, the adrenal glands
decrease in size, and hypoechoic cystic ar-
eas can be observed. The absence of flow at
Doppler evaluation is a key feature to distin-
guish an enlarged adrenal with a masslike
appearance due to hemorrhage from a neu-
roblastoma, which usually displays normal
or greater Doppler flow. Owing to the typical
behavior of hemoglobin breakdown byprod-
ucts, MRI can be of further help to diagnose
adrenal hemorrhage. Intraadrenal T1-hyper-
intense foci can be seen in the acute phase,
along with fluid-fluid levels on T2-weighted
imaging. After initial detection in a newborn,
adrenal hemorrhage typically resolves spon-
taneously within the first month of life [53].
Conclusion
Appropriate and normal function of the fe-
tal urinary system has a major influence on fe-
tal well-being and development. Fetal urinary
tract anomalies can be discovered in isolation
or in conjunction with other syndromes and
 Imaging of Fetal Urinary Tract Anomalies
conditions or anomalies. We have reviewed
the concepts related to normal fetal urinary
tract development and have highlighted as-
sociated anomalies that can develop owing to
faulty or abnormal embryologic development.
We have presented the classification of fetal
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abnormalities of the kidneys and urinary tract
based on the presence or absence of associat-
ed urinary tract dilation. Ultrasound is the pri-
mary imaging modality for the assessment of
fetal anomalies of the urinary tract. Fetal MRI
has a complementary role in cases of equivo-
cal or inconclusive ultrasound findings, to aid
in definite diagnosis, thereby improving the
imaging-based management of the pregnancy.
In this article, we have reviewed the 2014 clas-
sification of urinary tract dilation based on the
Linthicum multidisciplinary consensus pan-
el. To provide the readership with a practical
synopsis, we also propose an algorithmic ap-
proach for the evaluation of fetal urinary tract
and renal parenchymal abnormalities (Fig. S5
can be viewed in the AJR electronic supple-
ment to this article, available at www.ajron-
line.org). This approach relies on the presence
or absence of the kidneys on initial ultrasound
evaluation, as well as on the size and appear-
ance of the kidneys, and includes the most re-
cent recommendations for follow-up of each
abnormality. Familiarity of the radiologist
with the anatomy and classification of urinary
tract and renal pathologic abnormalities, dif-
ferential diagnoses, and recommendations for
in utero management are of utmost impor-
tance for those health care practitioners per-
forming and interpreting prenatal and perina-
tal imaging.
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