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462
GENITOURINARY IMAGING
Congenital Anomalies of the
Upper Urinary Tract: A Compre-
hensive Review
Abdallah P. Houat, MD
Cassia T. S. Guimarães, MD
Marcelo S. Takahashi, MD
Gustavo P. Rodi, MD
Taísa P. D. Gasparetto, MD, PhD
Roberto Blasbalg, MD, PhD
Fernanda G.Velloni, MD
Abbreviations: CRE = crossed renal ectopia,
SK = supernumerary kidney, UPJ = ureteropel-
vic junction, UPJO = ureteropelvic junction ob-
struction, VCUG = voiding cystourethrography,
URA = unilateral renal agenesis
RadioGraphics 2021; 41:462–486
https://doi.org/10.1148/rg.2021200078
Content Code:
From the Department of Radiology, Diagnósti-
cos da América SA (DASA), Av Juruá 434,
Alphaville Industrial, Barueri, SP 06455-010,
Brazil. Presented as an education exhibit at the
2019 RSNA Annual Meeting. Received April 19,
2020; revision requested June 25 and received
July 23; accepted August 4. For this journal-
based SA-CME activity, the authors, editor, and
reviewers have disclosed no relevant relation-
ships. Address correspondence to A.P.H. (e-
mail: abdallah_houat@hotmail.com).
©
RSNA, 2021
SA-CME LEARNING OBJECTIVES
After completing this journal-based SA-CME
activity, participants will be able to:
„ Describe the normal anatomy and em-
bryology of the kidneys and ureters.
„ Identify the spectrum of radiologic
findings related to congenital anomalies
of the kidneys and ureters.
„ Discuss the clinical manifestations and
potential complications associated with
congenital anomalies of the kidneys and
ureters.
See rsna.org/learning-center-rg.
An earlier incorrect version of this article
appeared online and in print. This article
was corrected on August 13, 2021.
The upper urinary tract is the most common human system affected
by congenital anomalies. Congenital anomalies of the kidneys and
ureters comprise a wide spectrum of disorders ranging from simple
variants with no clinical significance to complex anomalies that may
lead to severe complications and end-stage renal disease. They may
be classified as anomalies of renal form, which are subclassified as
structural anomalies (eg, persistent fetal lobulation, hypertrophied
column of Bertin, and dromedary hump) and fusion anomalies (eg,
horseshoe kidney and pancake kidney); anomalies of renal posi-
tion (eg, renal malrotation, simple renal ectopia, and crossed renal
ectopia) and renal number (eg, renal agenesis and supernumerary
kidney); and abnormalities in development of the urinary collecting
system (eg, pyelocaliceal diverticulum, megacalycosis, ureteropelvic
junction obstruction, duplex collecting system, megaureter, ectopic
ureter, and ureterocele). US is usually the first imaging modality
used because of its low cost, wide availability, and absence of ionizing
radiation. Intravenous urography and voiding cystourethrography
are also useful, mainly for characterization of the collecting system
and vesicoureteral reflux. However, intravenous urography has been
replaced by CT urography and MR urography. These imaging meth-
ods not only allow direct visualization of the collecting system but
also demonstrate the function of the kidneys, the vascular anatomy,
adjacent structures, and complications. Comprehension of congenital
anomalies of the upper urinary tract is crucial for an accurate diag-
nosis and correct management. The authors discuss the spectrum of
these anomalies, with emphasis on embryologic development, imag-
ing findings, clinical manifestations, and complications.
Online supplemental material is available for this article.
©
RSNA, 2021 • radiographics.rsna.org
Introduction
Congenital anomalies of the upper urinary tract correspond to a wide
spectrum of disorders that are related to embryonic defects with spo-
radic, genetic, and environmental causes. They occur in 3%–11% of
the population, accounting for 50% of all congenital abnormalities (1).
They are frequently associated with genital tract malformations due to
the common embryologic origin of these structures and show a broad
variety of phenotypes, ranging from a complete absence of renal tissue
to minor alterations of the structural parenchyma. Approximately two-
thirds of patients with congenital anomalies of the upper urinary tract
have associated anomalies of other organ systems such as the skeletal,
cardiovascular, gastrointestinal, and central nervous systems.
Congenital anomalies of the upper urinary tract may be classi-
fied according to embryologic development as anomalies of renal
form, position, and number and abnormalities in development of
the urinary collecting system. Thus, understanding the embryologic
development and knowing the anatomic structures of the upper
RG • Volume 41 Number 2
TEACHING POINTS
„ Although many congenital anomalies of the upper urinary
tract are diagnosed in the prenatal and neonatal periods, de-
tection also occurs frequently in children or adults as either
an incidental finding in asymptomatic individuals or a com-
plication (eg, related to upper urinary tract obstruction, stone
formation, infection, hypertension, or renal failure).
„ Anomalies of renal form comprise structural anomalies and
fusion anomalies. Structural anomalies are anatomic variants
that are related to embryologic defects during the final stages
of kidney development, including persistent fetal lobulation,
a hypertrophied column of Bertin, and a dromedary hump.
Fusion anomalies occur during the cranial migration of the
kidneys from the pelvis to the lumbar region. The kidneys
cross the umbilical arteries, and any change in arterial position
may cause fusion of nephrogenic blastema. The fusion may
be partial (eg, horseshoe kidney and crossed renal ectopia
with fusion) or total (ie, pancake kidney).
„ Anomalies of renal position occur because of defects in the
cranial migration of the kidneys from the pelvis to the lumbar
region, which begins in the 4th week and ends in the 9th
week of gestation. They include renal malrotation, simple re-
nal ectopia, and crossed renal ectopia.
„ Anomalies of renal number occur due to defects in the devel-
opment of the ureteric bud or its interaction with the meta-
nephric blastema at approximately the 5th week of gestation.
These anomalies include renal agenesis and supernumerary
kidney (SK).
„ Congenital anomalies of the urinary collecting system are
related to defects in the embryologic development of the
ureteric bud starting during the 5th week of gestation and in-
clude pyelocaliceal diverticulum, megacalycosis, ureteopelvic
junction obstruction, duplex collecting system, megaureter,
ectopic ureter, and ureterocele.
urinary tract are extremely important for com-
plete comprehension of these anomalies.
Although many congenital anomalies of the
upper urinary tract are diagnosed in the prena-
tal and neonatal periods, detection also occurs
frequently in children or adults as either an
incidental finding in asymptomatic individuals or
a complication (eg, related to upper urinary tract
obstruction, stone formation, infection, hyperten-
sion, or renal failure).
In this article, we describe the embryologic de-
velopment, anatomic structures, defects, and find-
ings related to congenital anomalies of the kidneys
and ureters with different imaging methods.
Normal Anatomy of the Kidneys and
Ureters
The kidneys are paired bean-shaped retroperi-
toneal organs that are covered by a fibrous cap-
sule and surrounded by perirenal fat, which is
encompassed by the renal fascia (Gerota fascia).
They lie on quadratus lumborum muscles at the
level of T12–L3, lateral to the psoas muscle. The
oblique course of the psoas muscle results in the
upper poles oriented medially in relation to the
Houat et al 463
lower poles. The right kidney is 1–2 cm lower ow-
ing to displacement caused by the liver. Usually,
the kidneys are 10–12 cm long and 3–5 cm wide.
The adrenal glands lie superior and medial to the
kidneys. The liver, hepatic flexure of the colon,
and duodenum are in close contact with the right
kidney, while the tail of the pancreas, splenic flex-
ure of the colon, and spleen abut the left kidney.
The renal parenchyma is composed of the cortex
and the medulla. The cortex is the outer part that
contains glomeruli and the proximal portion of the
collecting tubules. The medulla corresponds to the
inner part and contains the renal pyramids and the
distal portion of the collecting tubules. The papilla
is the apex of the renal pyramids, and each papilla
indents to a minor calyx. Approximately 2–4
minor calyces merge to form a major calyx, and
2–3 major calyces merge to form the renal pelvis,
a funnel-shaped structure in the renal hilum that
drains to the ureters (Fig 1a).
Ureters are thin muscular tubes that are 25–30
cm long, coursing along the retroperitoneum,
originating from the ureteropelvic junction, and
lying on the psoas muscles in the upper abdo-
men. They deviate anteriorly, cross the common
or external iliac vessels, and run along the lateral
pelvic wall near the internal iliac vessels until the
ureterovesical junction. At this level, they pass
obliquely through the muscular bladder wall,
creating a valve mechanism that prevents urine
reflux (Fig 1b).
Embryology
Three germ layers are present at the beginning
of morphogenesis: the ectoderm, mesoderm, and
endoderm. The urogenital ridge arises from the
intermediate mesoderm during the 4th week of
intrauterine life, giving rise to the nephrogenic
cord. Three successive kidneys form rostrally and
progress caudally over a few weeks in the neph-
rogenic cord: the pronephros, mesonephros, and
metanephros.
At the beginning of the 4th week, the proneph-
ros, a nonfunctional primitive kidney that totally
regresses by day 25, forms at the cervical region.
At the end of the 4th week, the intermediate
mesoderm condenses to form the mesonephros
in the thoracolumbar region. The mesoneph-
ros is functional for a brief period and develops
excretory tubules, which drain into the meso-
nephric duct, which is also called the wolffian
duct. The mesonephric duct grows toward the
caudal region and fuses with the cloaca during
the 5th week of embryogenesis, giving origin to
primordial renal tissue known as the metanephric
blastema from the sacral intermediate mesoderm.
Later, the mesonephros and mesonephric duct
develop into the epididymis, vas deferens, seminal
464 March-April 2021 radiographics.rsna.org

Figure 1. Anatomy of a healthy kidney (a) and ureter (b). UPJ = ureteropelvic junction, UVJ = ureterovesical junction.

vesicles, and ejaculatory duct in males, and they

degenerate in females.
At the 5th week, the metanephros forms in
the sacral region and persists as the permanent
kidney (Fig 2). At this moment, the primordial
renal tissue metanephric blastema begins the
secretion of a protein that stimulates the lateral
growth of the ureteric bud in the mesonephric
duct (2), invading the metanephric blastema
and leading to reciprocal interactions between
both, called the induction of the kidney. The
metanephric blastema then forms the nephrons
(ie, the glomeruli, proximal tubules, and distal
tubules) and the ureteric bud starts to branch,
creating the collecting tubules. The first bifurca-
tion forms the renal pelvis as well as the cranial
and caudal lobes of the kidney. The next bifur-
cations continue until the 32nd week, progres-
Figure 2. Embryologic illustration shows that in the
sively forming the major calyces and minor 5th week, the kidneys begin to develop by means of
calyces and then forming approximately 1–3 fusion of the ureteric bud with the metanephric blas-
million collecting tubules (3). tema of the intermediate mesoderm.
Initially, the kidneys are located close to each
other in the sacral region of the embryo, with the
hila directed anterior in the pelvis. As the abdomen anteromedially. The ureters elongate, opening into
and pelvis grow, the kidneys are drawn apart and the bladder superiorly (Fig 3).
gradually ascend to the lumbar region, reaching
their adult position adjacent to the adrenal gland Imaging Techniques
by the 9th week of gestation. During ascension, The first imaging modality that is usually per-
the renal arterial supply undergoes a continuous formed for anomalies of the urinary tract system
process of replacement, receiving irrigation from is US because it is noninvasive, widely available,
successively higher branches, first from common low cost, and lacks ionizing radiation. However,
iliac arteries and later from the aorta, while caudal US is limited by its operator and patient depen-
branches continue to degenerate. Once the kidneys dence and limited visualization of the entire col-
reach their final position, one branch becomes the lecting system. Contrast-enhanced US, and more
main renal artery, and the rest regress. Less com- specifically voiding urosonography, is a technique
monly, more than one branch persists, giving rise that is gaining popularity, especially for pediat-
to accessory renal arteries. As the kidneys ascend, ric patients, for the evaluation of vesicoureteral
they rotate medially almost 90°, directing the hila reflux and urethral abnormalities.
RG • Volume 41 Number 2
Figure 3. Embryology of the urinary system. During the 6th week, the primitive kidneys are close to each other in the
pelvis. Later, the kidneys migrate upward, move apart, and rotate medially almost 90 degrees, reaching the definitive
position close to the adrenal glands by approximately the 9th week of gestation. The development of the renal pelvis,
calyces, and collecting tubules occurs from the sequential branching of the ureteric bud during the 5th to 8th weeks. B =
bladder, K = kidney, S = suprarenal gland.
The use of intravenous urography as a diag-
nostic tool has drastically decreased in recent
decades, especially because of improvements in
other modalities. Currently, it is almost always
used as a second or third choice when other
examinations are either inconclusive or not avail-
able for the patient.
Voiding cystourethrogram (VCUG) is an
established method for diagnosing both vesicoure-
teral reflux and urethral abnormalities. Recently,
specialized centers have begun performing voiding
urosonography as a reliable first-line alternative to
VCUG for both vesicoureteral reflux and urethral
evaluation. Both examinations require bladder
catheterization, but voiding urosonography has the
advantage of being a radiation-free examination
that allows simultaneous evaluation of morpho-
logic features and reflux, while VCUG has the
advantage of depicting the whole collecting system
at once.
CT and MRI are excellent modalities for
identification of anomalies of the upper urinary
tract because they allow direct characterization of
renal vascularity, adjacent abdominal and pelvic
structures, and complications (eg, renal stones,
infection, and malignancies). MRI is limited in
characterization of nonobstructive renal stones.
CT and MR urography have been increasingly
used to assess the upper urinary tract because
they allow visualization of the entire collecting
system anatomy in great detail. MRI also pro-
vides information about perfusion and excretion
functions. MR urography may be preferable for
pediatric patients and those who need frequent
repeated imaging, because the modality does not
involve exposure to ionizing radiation, and in
individuals with a history of allergy to iodinated
contrast agents.
Houat et al 465
Classification of Congenital Anomalies
of the Upper Urinary Tract
The Table provides a comprehensive overview
of the classification of anomalies of the upper
urinary tract, which include renal anomalies of
form, position, and number and anomalies in the
development of the urinary collecting system.
Anomalies of Renal Form
Anomalies of renal form comprise structural
anomalies (Fig 4) and fusion anomalies (Fig 5).
Structural anomalies are anatomic variants that
are related to embryologic defects during the final
stages of kidney development, including persistent
fetal lobulation, a hypertrophied column of Bertin,
and a dromedary hump. Fusion anomalies occur
during the cranial migration of the kidneys from
the pelvis to the lumbar region. The kidneys cross
the umbilical arteries, and any change in arterial
position may cause fusion of nephrogenic blastema.
The fusion may be partial (eg, horseshoe kidney
and crossed renal ectopia with fusion) or total (ie,
pancake kidney). Crossed renal ectopia is explained
in the section on anomalies of renal position.
Structural Anomalies
Persistent Fetal Lobulation.—Persistent fetal
lobulation, which is also known simply as loba-
tion, is characterized by fine linear and smooth
indentations of the renal outline between the
pyramids (Fig 4) (4). It is difficult to estimate its
real incidence because anomalies of no clini-
cal significance are often not reported or even
detected. Despite that, it has been estimated to
be present in 4% of the population (5).
Embryologically, fetal kidneys are divided
into lobes that are made of a medullary pyramid
466 March-April 2021 radiographics.rsna.org

Classification of Congenital Anomalies of the Upper Urinary Tract

Type of Renal Anomaly Key Features

Renal form
Structural
Persistent fetal lobulation Smooth indentations of the renal outline between the pyramids
Hypertrophied column of Bertin Hypertrophied cortical tissue projecting into the renal sinus
Dromedary hump Focal bulge on the lateral contour of the left kidney
Fusion
Horseshoe kidney Fusion across the midline of two distinct functioning kidneys
Pancake kidney Complete fusion of both kidneys in the pelvic cavity
Renal position
Renal malrotation Abnormal position of the kidneys in relation to the hilum
Ectopic kidney Abnormal kidney location: simple ectopia or crossed ectopia
Crossed renal ectopia Kidney is located on the opposite side and its ureter has normal insertion
Renal number
Renal agenesis Complete absence of one or both renal tissue
Supernumerary kidney Accessory organ (typically one additional kidney)
Development of the urinary collecting
system
Pyelocaliceal diverticulum Cystic dilatation of renal pelvis or calyces
Megacalycosis Enlargement of the renal calices without renal pelvis dilatation
UPJO Obstruction of the normal flow of urine in the UPJ
Duplex collecting system Two separate pyelocaliceal systems: complete or incomplete
Megaureter Ureteral dilatation with or without pyelocaliceal dilatation
Ectopic ureter Ureter with an insertion beyond the bladder trigone
Ureterocele Cystic dilatation of the intravesical segment of the distal ureter
Note.—UPJ = ureteropelvic junction, UPJO = ureteropelvic junction obstruction.

surrounded by a cortex and separated by inter-
lobular grooves. The lobulation usually remains
until the end of the fetal period, becoming un-
noticeable during the 3rd trimester and resulting
in a smooth renal surface. Incomplete fusion of
interlobular folds can result in the appearance of
persistent fetal lobulation (4,6).
Persistent fetal lobulation does not have any
clinical implication and can mimic other abnor-
malities at diagnostic imaging, such as scarring or
kidney tumors. At US, uniform vascular distribu-
tion is observed in the parenchyma (7). At CT
(Fig 6a) and MRI, attenuation and signal intensity,
respectively, are similar to those of the cortex, as
is contrast enhancement (7–9). However, renal
scarring is not smooth, appears as a loss of renal
cortex, and is located overlying the medullary
pyramids (7,8). Parenchymal tumors show differ-
ent enhancement in at least one of the phases (9).
Hypertrophied Column of Bertin.—A hypertro-
phied column of Bertin, or focal cortical hyperplasia,
is characterized by hypertrophied cortical tissue
located between the pyramids of the renal me-
dulla, projecting into the renal sinus (Fig 4). The
tissue is often located in the middle one-third of
the kidney between the upper and middle calyces.
It more commonly occurs on the left side (7,8).
A hypertrophied column of Bertin is a normal
variant with no exact incidence, although some
authors (10) state that it can be present in almost
50% of the population, and 20% of those diag-
nosed have hypertrophied columns of Bertin in
both kidneys. Double hypertrophied columns of
Bertin in one kidney are rare, occurring in approx-
imately 5% of individuals (10). The cause is an
incomplete fusion of the fetal lobes, resulting from
the merging of two adjacent septa into a large
column with double thickness (11,12).
A hypertrophied column of Bertin has no clini-
cal importance. At US, it appears as a well-defined
indentation in the renal sinus in continuity with
and with similar echogenicity as the adjacent renal
parenchyma (Fig 6b) (13). However, sometimes
it can have hyperechoic areas in the side that
represent interlobular vessels (11,12) and have
similar vascularity to that of the renal parenchyma
(13). In some patients, a hypertrophied column
of Bertin can have an indefinite appearance at US
and can mimic a renal mass. In these cases, CT
RG • Volume 41 Number 2
or MRI can be diagnostic, preventing an unneces-
sary invasive procedure and showing, respectively,
attenuation and signal intensity similar to that
of the parenchyma on nonenhanced images and
enhancement identical to that of the renal paren-
chyma on contrast-enhanced images (8,12).
Dromedary Hump.—A dromedary hump is a focal
bulge on the lateral contour of the left kidney (Fig
4). It is caused by the splenic impression on the
superolateral kidney (8,14). Its frequency has been
estimated at 0.5% (5).
Similar to other renal variants, a dromedary
hump shows characteristics identical to those of
the renal parenchyma at imaging, regardless of
the imaging modality (Fig 6c). Although a drom-
edary hump can also mimic renal masses, tumors
commonly show different signal intensity, attenu-
Houat et al 467
Figure 4. Anomalies of
renal form. Illustration
shows structural anom-
alies of the kidneys.
Figure 5. Anomalies of renal form. Il-
lustration shows fusion anomalies of the
kidneys.
ation, and/or enhancement during at least one of
the phases of the imaging study (14,15).
Fusion Anomalies
Horseshoe Kidney.—A horseshoe kidney is formed
by fusion of two distinct functioning kidneys,
one on each side of the midline (Fig 5) (16).
Horseshoe kidney is the most common congeni-
tal anomaly of the upper urinary tract and can
be found in one in 400 adults (17). It is twice as
common in men than it is in women (18).
The theory of mechanical fusion suggests that
the metanephric blastema of the two kidneys come
in contact in the fetal pelvis during the 4th week of
gestation. This may be a consequence of abnormal
flexion or growth of the fetal spine and pelvic or-
gans. At this stage, because of the lack of the renal
468 March-April 2021
Figure 6. Structural anomalies of the kidney in three patients. (a) Coronal nonenhanced CT image in a 36-year-old
woman shows a persistent fetal lobulation (arrow). (b) US image in a 30-year-old woman shows a hypertrophied col-
umn of Bertin (*). (c) Coronal T2-weighted MR image in a 40-year-old man shows a dromedary hump (arrowhead)
from the splenic impression (curved arrow).
capsule, the blastema of the immature kidneys fuse
at the point of contact, resulting in the forma-
tion of a fibrous isthmus. As the horseshoe kidney
ascends, the isthmus is trapped under the inferior
mesenteric artery, arresting further ascent and ro-
tation, resulting in a lower location of the kidneys
and an anteriorly facing pelvis (16).
In almost 90% of cases, the fusion is between
the lower poles. In the other 10%, the superior or
both the superior and inferior poles are fused. The
most common position of the pelvis and ureters is
anterior (18). The isthmus (fused portion) may lie
over the midline (ie, symmetric horseshoe kidney)
or lateral to the midline (ie, asymmetric horseshoe
kidney). Depending on the degree of fusion, the
isthmus can be composed of renal parenchyma
or a fibrous band. The blood supply of the fused
kidney is variable and may come from the iliac ar-
teries, aorta, and less often from the middle sacral
and hypogastric arteries. Horseshoe kidney can be
isolated in 30% of those who have this anomaly,
although a wide variety of other anomalies are
frequently associated with it (19).
The majority of patients with a horseshoe
kidney are asymptomatic (20), although some
complications can occur such as ureteropelvic
junction obstruction (UPJO), lithiasis (20%–60%
of patients with horseshoe kidney) (Fig E1) (21),
and renal infections (27%–41% of patients with
horseshoe kidney). In patients with a horseshoe
kidney, there is a higher risk of renal lesions after
abdominal trauma and an increased incidence of
renal malignancies (although they are rare) such
as renal cell carcinoma, transition cell carcinoma,
Wilms tumors, and, less commonly, carcinoid
tumors (Fig E2) (18).
US can be useful for diagnosis and to estab-
lish the presence of the isthmus and its continu-
ity with the lower poles (Fig 7a). CT (Fig 7b,
7c) and MRI can help in mapping the anatomy
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of the collecting system and vascular anatomy.
Functional analysis with MRI helps in differen-
tiation of a functioning isthmus from fibrous tis-
sue and in early detection of complications. Al-
though MRI has advantages, including its ability
to show high soft-tissue contrast and the absence
of radiation, complications related to stones and
trauma are better evaluated with CT (19).
Pancake Kidney.—A pancake kidney is character-
ized by an absence of a renal capsule and com-
plete fusion of the superior, middle, and inferior
poles of both kidneys in the pelvic cavity (Fig 5)
(22). Each kidney has its own excretory system,
with two ureters that do not cross the midline
and are not connected with each other. The in-
sertion of the ureters into the bladder is normal
(22,23). Pancake kidney is rare, with an esti-
mated incidence of one in 65 000–375 000 indi-
viduals (24) and is often associated with other
genitourinary and vertebral anomalies (22). It
is possible that pancake kidney arises during
development, when the umbilical arteries press
the nephrogenic primordia, and the kidneys fuse
in the pelvis and do not ascend (23,25).
The blood supply can be anomalous in the
number and origin of arteries. The arteries
originate from the distal aorta or iliac artery.
The collecting systems are normally rotated to
face anteriorly and can be associated with many
configurations (24,25).
Most cases are asymptomatic, although patients
with pancake kidney are prone to recurrent urinary
tract infections and stone formation, given the like-
lihood of anomalous collecting system rotation and
the potential for stasis or obstruction (25).
US shows a large and lobulated renal mass
located in the pelvic cavity, consisting of the two
fused lateral lobes, without an intervening sep-
tum (Fig E3). Each lobe usually has a separate
RG • Volume 41 Number 2 Houat et al 469

Figure 7. Fusion anomalies of the kidneys: horseshoe kidney in two patients. (a) US image in a 10-week-old
infant girl shows fusion of the renal parenchyma, with two distinct kidneys on each side of the midline (*). The
aorta is seen posteriorly (arrow). (b, c) Axial corticomedullary phase (b) and coronal volume-rendered (c) CT
images in a 31-year-old man show fusion of the renal parenchyma across the midline (*) of two distinct kidneys.
Arrow in b = aorta.

Figure 8. Fusion anomalies: pancake kidney in a 59-year-old man. Coronal (left) and axial (right) portal venous
phase CT images show complete fusion of both kidneys in the pelvic cavity (arrows).

pelvicalyceal system. The renal pelvis is anteri-
orly placed with usually short ureters entering
normally into the bladder, without crossing the
midline. CT (Fig 8) and MR urography can
allow confirmation of the diagnosis and are bet-
ter choices than US for evaluation of vascular
anatomy, complications, and other associated
abnormalities (25).
Anomalies of Renal Position
Anomalies of renal position occur because of de-
fects in the cranial migration of the kidneys from
the pelvis to the lumbar region, which begins in
the 4th week and ends in the 9th week of gesta-
tion. They include renal malrotation, simple
renal ectopia, and crossed renal ectopia (Fig 9).
Renal Malrotation.—Renal malrotation is de-
fined as an abnormal position of the kidneys in
relation to the hilum. It can be unilateral or bi-
lateral and is usually related to other anomalies.
It is more common in male individuals and has
a prevalence of one in 2000 autopsies, but this is
probably underestimated, because many patients
470 March-April 2021
Figure 9. Anomalies of renal position. Il-
lustration shows simple and crossed renal
ectopia.
have no clinical symptoms (26,27). It may occur
when the ureteric bud inserts into an abnormal
region of the metanephric blastema. Because of
its association with renal ectopia, the process of
ascent and rotation are probably related (28).
Renal malrotation is asymptomatic in most
patients. Diagnosis is usually made inciden-
tally with US, CT, or MRI during evaluation
of abdominal pain or urinary abnormalities
(eg, hydronephrosis, urinary tract infection, or
hematuria) (29).
Renal malrotation consists of nonrotation or
incomplete rotation, reverse rotation, hyperrota-
tion or excessive rotation, and sagittal rotation
(Fig 10) (28).
Incomplete rotation and nonrotation are the
most common, in which the hilum is in an anterior
position or between the anterior and normal medial
position, with the ureter located laterally (Fig 11).
Reverse rotation and hyperrotation are also com-
mon types. Reverse rotation is characterized by
lateral pelvic rotation, with renal vessels crossing the
kidney anteriorly to reach the hilum and the ureter
located laterally. In hyperrotation, the kidney is ro-
tated more than 180°, but less than 360°. The renal
pelvis faces laterally, but the renal vessels are carried
posteriorly into the hilum (26,29). Sagittal rotation
is rare, and only a few cases have been described in
the literature in which the kidney rotates around its
hilum in the sagittal plane with its long axis in the
horizontal plane (28).
Simple Renal Ectopia.—Ectopic kidney or renal
ectopia is characterized by an abnormal location
radiographics.rsna.org
of the kidneys and occurs when the kidney fails
to ascend to the retroperitoneal renal fossa dur-
ing embryologic development (30,31). Ectopic
kidney can be simple (ie, when it is located at
the same side of the ureter) or crossed (ie, when
the kidney is on the opposite side of the ureteric
orifice) and unilateral or bilateral. Simple renal
ectopia, in order of frequency, can be located in
the pelvis, iliac region, abdomen, or chest (1).
A pelvic kidney (Fig 9) is the most common
form of simple renal ectopia. The incidence is
between one in 2200 to one in 3000 autopsies, and
it usually occurs between the 6th and 9th week of
gestation. It can be bilateral (Fig E4) in 10% of
those who have it and can be mistaken for a horse-
shoe kidney or pancake kidney (1,30). The blood
supply is usually from the iliac or infrarenal aorta
with multiple arteries. It is prone to poor drainage.
The most common position is opposite the sacrum
and below the aortic bifurcation (Fig 12a) (30).
The position of the kidney in renal ectopia
can be higher in the body, as in racic or subdia-
phragmatic kidney (Fig 13). Renal ascension
ends when the kidneys reach the adrenal glands
and are physically hindered from ascending to a
higher position. Also, the ascension of the right
kidney can also be prevented, to some extent, by
the liver. When changes in the development of the
adrenal glands and liver occur, with the coexis-
tence of intrinsic factors in the kidney such as
persistence of the nephrogenic cord, the devel-
oping kidney may rise to a higher location (32).
An intrathoracic ectopic kidney is a partial or
complete renal protrusion above the level of the
RG • Volume 41 Number 2
Figure 10. Illustration shows the different types of renal malrotation.
Figure 11. Anomalies of renal position: renal malrota-
tion in a 68-year-old man. Axial portal venous phase (a)
and coronal volume-rendered (b) CT images show
bilateral renal malrotation, in which the hilum faces
anteriorly (arrows).
diaphragm, while the subdiaphragmatic kidney
has a location higher than normal but below the
diaphragm. High renal ectopia is a rare condi-
tion, with few cases described, and intrathoracic
kidneys represent less than 5% of all cases of
renal ectopia, and the incidence is less than five
in 1 000 000 births. High renal ectopia shows
male predominance and occurs more commonly
on the left, probably because of early fusion of
the pleural-peritoneal channel on the right and
by the presence of the liver as a physical barrier.
Almost 10% of cases can be bilateral (33,34).
Houat et al 471
A patient with simple renal ectopia has normal
function and no symptoms and the ectopia is fre-
quently noticed as an incidental imaging finding
(35). The risk of hydronephrosis may be higher
in patients with renal ectopia because of malrota-
tion of the kidney and an anteriorly placed renal
pelvis, leading to impaired urinary drainage (Fig
12b) (30,35). Infection and renal stones can
affect the ectopic kidney, leading to nonflank
pain. Ectopic kidneys are also prone to traumatic
injuries because of their abnormal location in the
retroperitoneum (35).
472 March-April 2021 radiographics.rsna.org

Figure 12. Anomalies of renal position: simple renal ectopia in two patients. (a) US image in a 5-month-
old male infant shows a pelvic kidney that is located posterior to the bladder (curved arrow, upper image)
and anterior to the lumbosacral vertebrae (arrowhead, lower image). (b) Sagittal portal venous phase
CT image in a 93-year-old man shows a pelvic kidney with abnormal rotation, in which the hilum faces
anteriorly (*). There is associated UPJO (arrow).

Once a simple renal ectopia is detected, evalua-

tion should include a careful physical examination
in search of other anomalies. Radiologic imaging is
recommended to detect other urologic abnormali-
ties that may affect renal function. Imaging often
leads to the discovery of a small and abnormally
rotated pelvic kidney. CT can give details of the
anatomy, mainly the vascular anatomy (30,35).

Crossed Renal Ectopia.—Crossed renal ectopia

(CRE) is defined as a kidney that is located on the
opposite side in relation to its embryologic posi-
tion (Fig 9). Despite the ectopic renal position, the
ureter of an ectopic kidney has normal insertion in
the bladder (ie, on its original embryologic side).
Patients with renal ectopia have a two to three
times higher chance of having left-to-right ectopia
(36). It is a rare entity that is seen in almost 7.5 of
10 000 newborns, with a male-to-female predomi-
nance of three to two (37).
Abnormal migration of the ureteric button and
metanephric blastema across the midline culmi-
nates in the kidney being located on the opposite
side of its normal position. There are some theo-
ries to explain why this happens, such as an abnor-
mal position of the umbilical artery influencing the
cephalic migration of the kidney, which follows the
path of least resistance and, consequently, migrates
to the contralateral side. Others suggest that the
Figure 13. Anomalies of renal position: sub-
diaphragmatic kidney in a 53-year-old woman.
Coronal portal venous phase T1-weighted MR
image shows the right kidney in a subdiaphrag-
matic position (arrow).
ureteric bud crosses over because of overbend-
ing and rotation of the caudal end of the embryo,
preventing the fusion of the ureteric bud with the
metanephric blastema. There is a stimulation of
the contralateral metanephric blastema while the
ipsilateral metanephros regresses (38).
In the majority of cases, patients are asymptom-
atic. CRE is incidentally diagnosed during exami-
nations performed for other indications. However,
this anomaly may be clinically significant owing to
RG • Volume 41 Number 2
Figure 14. Illustration shows the classification of CRE.
complications such as hydronephrosis, infections,
vesicoureteral reflux, and nephrolithiasis (39) or
its association with other abnormalities, mainly
skeletal, genital, anorectal, or cardiovascular ab-
normalities. In these cases, follow-up is important
(40). If any surgical intervention is indicated, then
CT or MR angiography is extremely important
to evaluate the blood supply of the CRE, which is
prone to variations (41).
US can show that the kidney is not in its
respective renal fossa, but careful examination of
the opposite side reveals its position, mainly in the
lumbar or iliac region. CT or MR angiography
also shows the abnormal position of the kidney
and allows easier identification of the vascular-
ity and details of the collecting system, which are
important in cases of surgical procedure planning
to avoid the added risk of performing catheter
angiography before surgery (42).
Four types of crossed renal ectopia (Fig 14)
can be seen at imaging, mainly at CT and MRI,
including CRE with fusion characterized by the
ectopic kidney crossing over to the opposite side,
with its upper pole fused to the lower pole of the
normal kidney and its ureter crossing the midline
to insert in the usual position in the bladder (Fig
15); CRE without fusion, which occurs when the
kidney crosses to the other side but does not fuse
with the other kidney (Fig E5); crossed ectopia of
a solitary kidney that is associated with unilateral
renal agenesis (URA) and the kidney crossing to
the opposite side; and bilateral CRE.
The crossed kidney is fused to the normally
positioned kidney in most cases (approximately
90%), with an incidence of one in 2000 cases. Six
different forms of fusion have been described (Fig
16) including, in decreasing order of frequency,
Houat et al 473
(a) inferior crossed fusion kidney (ie, fusion of the
upper pole of the ectopic kidney with the lower
pole of the normal kidney), (b) sigmoid or S-
shaped kidney (ie, the ectopic kidney positioned
inferiorly, with the pelvis directed laterally and the
normal kidney with the pelvis turned medially),
(c) lump kidney (ie, both kidneys completely fused,
forming a lump on one side), (d) L-shaped kidney
(ie, ectopic kidney lying transversely and inferiorly
to the normal kidney), (e) disc kidney (ie, kidneys
fused along the medial edge of each pole), and
(f) superiorly crossed fused kidney (ie, ectopic kid-
ney positioned superiorly and its lower pole merg-
ing with the upper pole of the normal kidney) (43).
Anomalies of Renal Number
Anomalies of renal number occur due to defects in
the development of the ureteric bud or its interac-
tion with the metanephric blastema at approxi-
mately the 5th week of gestation. These anomalies
include renal agenesis and supernumerary kidney
(SK) (Fig 17).
Renal Agenesis.—Renal agenesis is defined as the
complete absence of one or both kidneys due to
unsuccessful formation of the embryonic kidney
(44). Bilateral agenesis is a rare congenital anom-
aly that occurs in one of 3000–4000 births. It is
incompatible with life and is most often associ-
ated with typical facial features (ie, “Potter face”)
and pulmonary hypoplasia, which is known as
the Potter sequence (45). URA (Fig 17) is not an
uncommon anomaly, and the incidence has been
estimated as one in 2000 births (46).
URA occurs because of failure of the interac-
tion between the ureteric bud and metanephric
blastema. Consequently, there is a failure in the
474 March-April 2021 radiographics.rsna.org

Figure 15. Anomalies of renal po-

sition: CRE with fusion in a 40-year-
old man. Coronal portal venous
phase (a) and coronal volume-
rendered (b) CT images show the
left kidney (straight arrow in a and
b) on the right flank and fused (ar-
rowhead in a and b) with the right
kidney (* in a and b). The ectopic
kidney ureter crosses the midline
(curved arrow in b) to insert into
the normal bladder position.

Figure 16. Illustration

shows the classification
of crossed renal ectopia
with fusion.

induction of the metanephric blastema to form
nephrons (44).
URA is usually asymptomatic and is nor-
mally found incidentally at imaging performed
for other indications. It is often associated with
other anomalies in the contralateral kidney (eg,
UPJO and vesicoureteral reflux, the latter being
the most commonly reported for almost 25% of
cases) or other organs (eg, cardiac, genital, or
gastrointestinal organs) (44).
RG • Volume 41 Number 2
Genital anomalies are also frequently associ-
ated with URA. In females, these include Mül-
lerian duct anomalies, an absent or hypoplastic
ovary or fallopian tube, and abnormal external
genitalia (47). The Mayer-Rokitansky-Küster-
Hauser syndrome is a congenital anomaly of
the female genital tract that is characterized by
complete paramesonephric duct (ie, Müllerian
duct) agenesis. It occurs in approximately one in
4500 births, and women present with primary
amenorrhea and otherwise normal pubertal
development. The findings are the absence or
reduced development of the uterus and the up-
per two-thirds of the vagina (ie, Müllerian duct
anomalies), normal external genitalia, ovaries,
and female karyotype (ie, 46, XX) (48–50).
The Mayer-Rokitansky-Küster-Hauser syn-
drome is subdivided into type I (ie, isolated, typi-
Houat et al 475
Figure 17. Anomalies of renal number.
Illustration shows renal agenesis and a su-
pernumerary kidney.
Figure 18. Anomalies of renal
number: Mayer-Rokitansky-Küster-
Hauser syndrome type II (renal
agenesis) in a 55-year-old woman.
Coronal T2-weighted MR image (a)
shows the absence of the right kid-
ney, and sagittal T2-weighted MR
image (b) shows the absence (ar-
row) of the uterus.
cal form, or Rokitansky sequence), which is char-
acterized by only Müllerian duct anomalies, and
type II (ie, atypical form), which includes genital
anomalies associated with other malformations,
such as renal, skeletal, or cardiac anomalies (49).
Congenital anomalies of the upper urinary tract
are found in approximately 40% of patients with
this syndrome, and the most common abnormali-
ties are URA (23–28%) and ectopia of one or both
kidneys (17%) (51).
Transabdominal US is usually the first im-
aging investigation performed in patients with
primary amenorrhea. US shows an absence of
the uterus and normal ovaries and may show
any associated renal tract anomaly. MRI is more
sensitive and more specific than US and should
be performed when US is inconclusive. MRI (Fig
18) allows an accurate evaluation of aplasia of the
476 March-April 2021 radiographics.rsna.org

Figure 19. Anomalies of renal number: Zinner syndrome triad

renal agenesis in a 36-year-old man. (a) Coronal T2-weighted
MR image shows the absence of the right kidney. (b) Axial T2-
weighted MR image shows ipsilateral seminal vesicle cysts (ar-
rows). (c) Sagittal T2-weighted MR image shows ejaculatory duct
dilatation (arrowhead).

uterus (rudimentary uterine bud) and the upper
two-thirds of the vagina and allows characteriza-
tion of the normal aspect of the ovaries (52,53).
In male patients, renal agenesis includes
Zinner syndrome, which comprises the triad of
URA, ipsilateral ejaculatory duct obstruction,
and ipsilateral seminal vesicle cysts. It occurs
in two-thirds of male patients with URA and is
usually diagnosed in the 2nd to 3rd decade of
life. Zinner syndrome is most commonly related
to dysuria, frequency of micturition, and perineal
pain (54). Zinner syndrome probably occurs be-
cause of a failure in the development of the distal
part of the mesonephric duct before the 7th week
of gestation. It causes atresia of the ejaculatory
duct, leading to obstruction and dilatation of the
seminal vesicle, and the ureteric bud leads to re-
nal agenesis (55). At US, in addition to unilateral
absence of a kidney, an anechoic structure in the
pelvis is found (ie, ejaculatory duct obstruction
and seminal vesicle cysts), and low-level echoes
indicating hemorrhagic or thick content can be
found (56).
CT can accurately show pelvic anatomy, but
MRI is better than CT for characterization of the
genital anatomy, owing to its greater tissue con-
trast resolution, allowing a detailed assessment.
CT and MRI show a periprostatic cystic mass ipsi-
lateral to the renal agenesis, without enhancement.
The seminal vesicle cyst appears hypointense
at T1-weighted MRI and hyperintense at T2-
weighted MRI, although it sometimes may show
hyperintensity at T1-weighted MRI because of the
presence of hemorrhage or proteinaceous content.
In addition, sometimes an ectopic ureteral inser-
tion into the seminal vesicle, ejaculatory duct,
prostatic urethra, or vas deferens or agenesis of the
vas deferens may be associated (Fig 19) (57,58).
Supernumerary Kidney.—An SK is an additional
accessory organ that manifests most commonly
caudally to the left kidney (Fig 17) (59). SK on
the right side (Fig E6) or bilateral SKs are rare,
with fewer than 100 cases reported in the litera-
ture (60–62).
Many theories exist to explain the embryogenesis
of SKs. They are thought to arise from abnormal
division of the nephrogenic cord during the 5th
to 7th week of gestation, with each branch of the
ureteral bud penetrating independently in the meta-
nephric blastema and resulting in the development
of two kidneys. Another theory suggests that they
initiate as two independent ureteral buds, arising
separately from the mesonephric duct, penetrating
the metanephric blastema, and dividing in two. It is
also possible that fragmentation of the metaneph-
ric blastema can occur because of linear infarcts,
resulting in two independent kidneys (62,63).
SK is usually asymptomatic, although it may
manifest as abdominal pain and a palpable mass
(59). Urinary complications may occur, such
as urinary incontinence, pyelonephritis, pyone-
RG • Volume 41 Number 2
Figure 20. Anomalies of renal number:
left supernumerary kidney in a 26-year-
old woman. (a) Coronal portal venous
phase T1-weighted MR image shows a
normal right kidney and two left kidneys
(*) fused by parenchymal tissue and ar-
terial branches from the abdominal aorta
supplying the cranial kidney (arrow) and
from the left common iliac artery supply-
ing the caudal kidney (arrowhead). (b) Axial portal venous phase T1-weighted MR images show venous
drainage via the inferior vena cava to both the cranial (arrow, upper image) and caudal (arrowhead, lower
image) kidneys.
phrosis, renal and ureteral calculi, and malignant
changes (ie, Wilms tumor, clear cell carcinoma).
Management depends on the function, symp-
toms, and complications of the SK (60). SK can
be associated with many congenital anomalies
(eg, vaginal atresia, duplication of the penis or
the female urethra, coarctation of the aorta, and
other abnormalities) (64,65).
An SK is normally smaller than the native kid-
ney and has its own arterial supply originating from
the aorta or the common iliac artery. The venous
drainage occurs via the inferior vena cava, with a
distinct collecting system and an encapsulated pa-
renchyma. It may be completely separated from the
ipsilateral kidney or attached to it by parenchymal
or fibrous tissue. These features can be seen at im-
aging, mainly at CT and MRI (Fig 20) (61,62,66).
The main differential diagnosis of the SK includes
evaluation for a duplex collecting system.
Abnormalities of the Developing Urinary
Collecting System
Congenital anomalies of the urinary collecting
system are related to defects in the embryologic
development of the ureteric bud starting during
the 5th week of gestation and include pyelocaliceal
diverticulum, megacalycosis, ureteopelvic junction
obstruction, duplex collecting system, megaureter,
ectopic ureter, and ureterocele (Fig 21).
Houat et al 477
Pyelocaliceal Diverticulum.—A pyelocaliceal diver-
ticulum, which is also called a pyelogenic cyst, is a
cystic dilatation of the renal pelvis or calyces, with
narrow collecting system communication, lying in
the renal parenchyma. The incidence is approxi-
mately 0.3% of the adult population (67). There
is no consensus about the congenital cause of the
pyelocaliceal diverticulum, although proposed
theories relate to failure in the bifurcation and de-
generation of ureteric bud branches (67–69).
Minor calyceal diverticuli are more common
and are often located in the poles and asymptom-
atic. Pelvic and major pyelocaliceal diverticuli are
generally central, larger, and more associated with
symptoms, mainly stones and infection related to
urinary stasis (70). The average size of a pyelocali-
ceal diverticulum is 0.5–7.5 cm. Stones have report-
edly been found in 9.5%–50% of cases (68,71).
At imaging, the definitive diagnostic find is the
characterization of the connection with the pelvi-
calyceal system, although the narrow connecting
neck may be difficult to characterize. Therefore,
the more accurate methods are intravenous urog-
raphy or CT and MR urography (Fig 22), which
show the contrast media filling the diverticulum
during the excretory phase. Often, diverticuli are
indistinguishable from cysts at US or CT and
MRI without excretory phase imaging. However,
when they are filled with stones, the mobility and
478 March-April 2021
Figure 21. Illustration shows classification of different abnormalities of the developing urinary collecting system. DCS = duplex col-
lecting system.
decubitus change can be a clue for the diagnosis,
principally at US.
Megacalycosis.—Megacalycosis is a congenital
malformation that manifests with enlargement
of the renal calyces, the number of which may
also be increased, without dilatation of the renal
pelvis (Fig 21). Although there is no established
cause for megacalycosis, one proposed theory is
that calyces that develop from a later generation
of the collecting system than usual result in larger
polygonally shaped and more numerous calyces
(72). Megacalycoses are rare, with approximately
100 cases described in the literature (73). They
are usually incidental findings, but reported
symptoms include renal calculi, hematuria, and
infection.
Imaging diagnosis is useful for differentiation of
megacalycosis from other obstructive or reflexive
causes of calyceal dilatation. At imaging, calyces
appear enlarged, with flattening of the renal pyra-
mids and with no or mild pelvic dilatation (Fig
23). Functional imaging such as diuretic renogra-
phy or functional MR urography and voiding uro-
sonography or voiding cystourethrography should
radiographics.rsna.org
be performed to exclude obstruction or reflux,
respectively, from the differential diagnosis.
Ureteropelvic Junction Obstruction.—UPJO
is defined as an obstruction of the normal flow
of urine from the renal pelvis into the proximal
ureter (Fig 21). It is the most common cause of
postnatal hydronephrosis and the second most
common cause of prenatal hydronephrosis (after
transient or physiologic hydronephrosis), with
an estimated incidence of one in 750–1500 live
births (74,75). It is more common in males
(male-to-female ratio, 2:1 to 4:1), has a left-sided
predominance, manifests bilaterally in up to 16%
of the cases, and is often associated with other
urologic malformations (15%–20%).
There is no consensus on the cause of UPJO,
which is most likely multifactorial (74). The
causes are classically divided into either intrinsic
or extinct causes. Intrinsic causes include ureteric
scarring and ureteric muscular hypoplasia. Ex-
trinsic causes include a crossing lower pole renal
vessel, which can compress the ureter and is more
common in older children (76), and an abnormal
course of the ureter related to a high insertion at
RG • Volume 41 Number 2
Figure 22. Abnormalities of the developing urinary collecting system: pyelocaliceal diverticu-
lum in a 35-year-old woman. (a) Axial nonenhanced CT image shows a renal cyst with hyper-
attenuating foci, which may correspond to parietal calcification or a stone (arrow). (b) Axial
excretory phase CT image shows infilling of the cyst, which allows confirmation that the cyst is
connected with the collecting system (arrowhead).
Figure 23. Abnormalities of the developing urinary
collecting system: megacalycosis in a 34-year-old man.
Coronal excretory phase maximum intensity projection
CT image shows dilated left renal calyces (arrow) with a
normal-appearing renal pelvis (arrowhead).
the renal pelvis, abnormal renal rotation, or other
more complex congenital abnormalities such as a
duplex kidney or horseshoe kidney.
Most cases of UPJO are diagnosed antenatally
during routine gestational US. In cases in which
UPJO diagnosis is delayed, patients may present
with intermittent abdominal pain related to dila-
tation of the collecting system, a condition also
known as a Dietl crisis (77).
At imaging, UPJO appears as a dilated renal
pelvis with an abrupt thinning of the collecting
system at the ureteropelvic junction and calyceal
dilatation. Parenchymal thinning and dysplasia
may be associated with it. US (Fig 24a) is the
first-line examination, but CT (Fig 24b) and
especially MR urography are more suited for a
more detailed evaluation of the ureteropelvic
junction, especially if proximal ureteral kinking or
abnormal kidney rotation is suspected. Crossing
vessels should always be evaluated carefully with
Doppler US, CT, or MRI, not only as a causative
factor but also to facilitate eventual surgical cor-
rection (78).
Identification and quantification of collecting
system obstruction are key to patient treatment,
and obstruction is usually evaluated with either
Houat et al 479
nuclear medicine with mercaptuacetyltriglycine
(MAG3) or functional MR urography (79).
After diagnosis, imaging is also helpful in
patient follow-up as an ancillary tool to assess
changes in the degree of dilatation and morpho-
logic parenchymal changes such as thinning or
increased echogenicity that may indicate worsen-
ing obstruction (80,81).
Duplex Collecting System.—A duplex collecting
system is defined by the presence of two separate
pyelocaliceal systems. It can be classified as either
complete or incomplete. When complete, each
duplex system has a whole-length distinct ureter
(Figs 21, 25). Incomplete duplex systems are
those in which these distinct ureters fuse together
before the ureterovesical junction. Incomplete
duplex systems can be further subdivided into a
bifid pelvis, in which fusion occurs in the proxi-
mal ureters (Figs 21, 26) or a bifid ureter, in
which the fusion occurs at a more distal point
(Figs 21, 27) (82). A bifid ureter with a blind-
ending branch is a rare and important variant,
resulting from a ureteral bud without contact
with the metanephric blastema (83).
A duplex collecting system is the most com-
mon congenital ureteral abnormality, with a
prevalence of 0.8% of the population at autopsy
(84). Embryologically, incomplete duplications
are the result of abnormal bifurcation of the ure-
ter after its origin from the mesonephric duct,
while complete duplications are a result of two
distinct ureteral buds arising from the meso-
nephric duct.
Duplex collecting systems are usually asymp-
tomatic, are incidentally diagnosed with no as-
sociated renal abnormalities, and are considered
a normal variant (85). When renal abnormalities
are present, they tend to affect the whole kidney
in incomplete duplications and can affect either
one or both poles in complete duplications.
In complete duplex collecting systems, the lower
pole follows the normal anatomic structure, with an
orthotopic ureter implantation, while the ureter of
480 March-April 2021 radiographics.rsna.org

Figure 24. Abnormalities of

the developing urinary collecting
system: UPJO. US image (a) in a
1-month-old male infant and coro-
nal portal venous phase image (b)
in a 40-year-old man show abrupt
narrowing at the ureterovesical
junction (arrow), with pelvicalyceal
dilatation (*) and thinning of the
renal parenchyma (arrowheads).

Figure 25. Abnormalities of the

developing urinary collecting sys-
tem: complete duplex collecting
system. Coronal volume-rendered
CT image in a 35-year-old woman
shows complete ureteral duplica-
tion (arrows).

Figure 26. Abnormalities of the

the upper pole has an ectopic bladder implantation,
inferiorly and medially displaced, and, therefore, is
prone to be associated with other abnormalities.
The Weigert-Meyer rule states that the ectopic
ureter implants itself along an ectopic pathway,
which, in girls, can be either above the external
urethral sphincter (ie, bladder) or beneath the
external urethral sphincter (ie, the urethra [Fig
28a, 28b], upper vagina, uterus, or fallopian
tubes) and in boys, is always above the external
urethral sphincter (ie, the bladder, prostatic ure-
thra [Fig 28c, 28d]), epididymis, seminal vesicles,
vas deferens, or ejaculatory ducts). Abnormalities
that can be related to this ectopic ureter include
urinary incontinence in girls, renal dysplasia (Fig
E7), urinary tract dilatation, ureterocele (Fig 29),
and vesicoureteral reflux (Fig 30) (85).
At US, when no renal abnormalities are as-
sociated, the kidney with the duplex collecting
system is larger than the normal one. The renal
developing urinary collecting sys-
tem: bifid pelvis in a 32-year-old
woman. Coronal excretory phase
maximum intensity projection CT
image shows two distinct ureters
fusing before the ureterovesical
junction (arrows).
parenchyma can be seen crossing the entire
renal pelvis, separating the upper from the lower
moiety. Mild asymmetric pelvicalyceal dilatation
between the upper and lower moiety is also a sign
that can suggest a duplex collecting system in an
otherwise normal kidney. CT and MR urography
are useful to determine if the duplication is com-
plete or incomplete and also in suspected ureteral
ectopic insertion, because the whole ureteral
trajectory can usually be determined.
Megaureter.—Megaureter is a broad term used to
describe ureteral dilatation with or without asso-
ciated pyelocaliceal dilatation (Fig 21). Transient
dilatation of the ureter can be seen in healthy
patients and can be considered normal, especially
when the diameter is less than 0.5 cm. Persistent
dilatation and, more specifically, ureters with a
RG • Volume 41 Number 2
Figure 27. Abnormalities of the developing uri-
nary collecting system: incomplete duplex col-
lecting system in a 48-year-old woman. Coronal
volume-rendered CT image shows two distinct ure-
ters on the right (arrows) fusing at the level of the
distal segment, above the ureterovesical junction
(arrowhead).
diameter larger than 0.7 cm should be considered
abnormal (86,87).
Megaureter can be classified as either primary
or secondary. Primary megaureter encompasses
all cases related to idiopathic congenital abnor-
malities of the vesicoureteral junction region.
It can be further subclassified into obstructive
megaureter, in which an adynamic short justaves-
ical portion of the ureter leads to dilatation of the
proximal normal ureteral segments (ie, an analog
to achalasia and Hirschsprung disease); refluxing
megaureter, in which the vesicoureteral valve is
incompetent due to a short intravesical segment;
a congenital parauteric diverticulum or other
abnormalities of the vesicoureteral junction; and
a nonrefluxing and nonobstructed megaureter (in
which neither vesicoureteral reflux nor focal ste-
nosis are present). The latter is the most common
cause of neonatal megaureter. It is usually self-
limited and thought to be related to physiologic
immaturity of the ureter. Secondary megaureter
refers to ureteral dilatation resulting from other
abnormalities such as a neurogenic bladder, pos-
terior urethral valve, or ureteral calculus.
Similarly to other upper urinary tract malfor-
mations, many cases of megaureter are diagnosed
prenatally. In older patients, a megaureter can be
Houat et al 481
asymptomatic or may be related to urinary tract
infections, hematuria, and flank pain.
The role of imaging in patients with a mega-
ureter is to define whether it is secondary or
primary. In patients with a primary megaureter,
imaging is used to determine if there is associated
obstruction (Fig 31) or reflux (Fig 32). Differen-
tiating between primary and secondary megaure-
ter is generally straightforward. The normal ca-
pacity and shape of the bladder and the absence
of evident obstructive factors indicate that the
cause is most likely a primary megaureter.
For an obstructive megaureter, the imaging
pattern is similar among US, CT, and MR urog-
raphy, which show a diffusely dilated ureter with a
narrow and usually short distal segment near the
ureterovesical junction. Identifying the presence
and degree of obstruction is paramount in deter-
mining treatment options. It is usually done with
functional MR urography or diuretic renography,
(ie, usually with MAG3). A refluxing primary
megaureter is usually associated with some degree
of pyelocaliceal dilatation. Diagnosis can be made
with either voiding cystourethrography or voiding
US. In patients with nonrefluxing and nonob-
structed megaureter, there is no evidence of reflux
or obstruction. The ureter is dilated beginning at
a point immediately above the bladder.
Ectopic Ureter.—Ectopic ureter is defined as a
ureter with an insertion beyond the bladder tri-
gone (Fig 21). It is a relatively rare abnormality,
with a prevalence of approximately one in 1900
autopsies. It is more common in female patients
(female-to-male ratio, 5:1). Most ectopic ureters
are related to a duplex collecting system, although
up to 20% can be found in a single collecting
system (88).
Ectopic ureters are believed to be a conse-
quence of failure of the ureteral bud to separate
from the mesonephric duct, which leads to a
more caudal insertion. Since it is an abnormality
that is related to the embryogenesis of the geni-
tourinary tract, anatomic and clinical manifesta-
tions vary between male and female patients.
In male patients, ectopic ureters always insert
above the external sphincter and, consequently,
are not related to urinary incontinence. In these
patients, abnormal implantation occurs more often
into the bladder, prostatic urethra (Fig 28b), or
epididymis, but the ureter can also insert into the
seminal vesicles, vas deferens, or ejaculatory ducts.
In female patients, ectopic ureters occur either
above or beyond the external sphincter and can be
related to urinary incontinence. Abnormal implan-
tation in female patients is more often located in
the bladder, urethra, or upper vagina (Fig E8), but
it can also insert into the uterus or fallopian tubes
482 March-April 2021 radiographics.rsna.org

Figure 28. Abnormalities of

the developing urinary collecting
system. (a, b) Complete duplex
system with an ectopic ureter in
a 74-year-old woman. Coronal ex-
cretory phase CT image (a) shows
complete ureteral duplication (ar-
rows). The lack of excreted con-
trast agent in the ectopic ureter
that is associated with upper pole
atrophy (*) suggests obstruction.
T2-weighted MR image (b) shows
ectopic ureter insertion in the
urethra (arrowhead). (c, d) Coro-
nal T2-weighted MR image in a
43-year-old man with a complete
duplex system (c) and illustration
(d) show ureter insertion in the
prostatic urethra (curved arrow in
c, arrowhead in d).

Figure 29. Abnormalities of the developing urinary collecting system: complete duplex sys-
tem with a ureterocele in a 4-month-old female infant. US images show complete ureteral du-
plication (arrowheads, left) associated with a ureterocele on the ectopic ureter (arrow, right).
B = bladder.

(87). Regardless of sex, symptoms also include
urinary tract obstruction, which may lead to renal
urge incontinence, recurrent urinary tract infec-
tions, and atrophy or dysplasia (in duplex collect-
ing systems, usually of the upper moiety). In atro-
phy or dysplasia, the degree of renal impairment is
usually related to the degree of ectopia.
Detecting the insertion of the ectopic ureter
is often not possible with US, because a normal
US examination cannot exclude the diagnosis.
VCUG can be of diagnostic value only if ureteral
insertion is in the bladder or urethra and reflux
is present. MR urography is an excellent method
for detection of ectopic ureters, which can often
be identified without intravenous contrast mate-
rial (89). CT urography is a useful alternative for
detection of ectopic ureteral insertion, especially
because of the fast examination time that often
does not require sedation but does require injec-
tion of iodine contrast material and exposure to
RG • Volume 41 Number 2 Houat et al 483

Figure 30. Abnormalities of the devel-

opment of the urinary collecting system:
complete duplex system with vesicoure-
teral reflux in a 3-month-old female in-
fant. VCUG image shows vesicoureteral
reflux (arrowheads), exclusively to the
lower pole of the left kidney, which can
be identified by the absence of contrast
material in the upper calyces (*).

Figure 31. Abnormalities of the development of the urinary collecting system: obstructive megaureter in two
patients. US image (a) in a 3-month-old male infant and coronal T2-weighted MR image (b) in a 1-year-old boy
show left pyelocaliceal dilatation (*), with a diffusely dilated ureter (arrow) that tapers near the ureterovesical
junction (arrowhead). B = bladder.

ionizing radiation. A split-bolus technique for CT
urography, in which simultaneous nephrographic
and excretory phase images are acquired, should
always be considered, especially in children. At
CT urography, attention should be paid to the
need for a second delayed phase acquisition in
patients with a slow-filling collecting system (77).
Ureterocele.—A ureterocele is a congenital malfor-
mation characterized by cystic dilatation of the
intravesical segment of the distal ureter (Fig 21),
with an estimated incidence of one in 4000 chil-
dren (female-to-male ratio, 4–6:1). Ureteroceles
are classically categorized as either orthotopic
ureteroceles (ie, occurring at the expected vesi-
coureteral junction) or ectopic ureteroceles (ie,
occurring outside of the expected vesicoureteral
junction, usually lower and medial).
Orthotopic ureteroceles are almost always seen
in adults. They are usually accidental findings in a
simple collecting system and have no substantial
renal repercussion.
Ectopic ureteroceles are much more common
in children (ie, four times more frequent than an
orthotopic ureterocele) and are more frequently
related to other abnormalities such as urinary tract
infection, a duplex collecting system (in up to 80%
of cases), or urinary tract dilatation. Thus, they are
more often diagnosed in either routine prenatal
examinations or during workup for urinary tract
484 March-April 2021 radiographics.rsna.org

Figure 32. Abnormalities of the

development of the urinary col-
lecting system: refluxive megaure-
ter in a 3-month-old male infant.
(a) Color Doppler US images show
mild dilatation of the distal ureter
without tapering and bidirectional
flow at the vesicoureteral junc-
tion (curved arrows), which is di-
agnostic for vesicoureteral reflux.
(b) Contrast-enhanced voiding
subtraction US image shows ure-
teropelvic dilatation (arrows) and
microbubble contrast material
filling the ureter and pelvis (*),
indicating vesicoureteral reflux.
(c) VCUG image shows bilateral
vesicoureteral reflux that is more
pronounced on the left, with ure-
teral tortuosity and dilatation (black
arrowhead) and pyelocaliceal dila-
tation (outlined arrowheads).

Figure 33. Abnormalities of the

development of the urinary collect-
ing system: ureterocele in two pa-
tients. Axial T2-weighted MR im-
age (a) in a 40-year old man and
VCUG image (b) in a 9-year-old
girl show ureteroceles (arrowheads
in a, * in b).

infection. Diagnosis in adults is usually accidental,
with the finding of a simple collecting system and
no substantial renal repercussion.
There is no consensus on the cause of uretero-
celes, but one of the most widespread theories
is that they are a consequence of the persistence
of an embryologic membrane around the distal
ureter (90).
Ureteroceles can vary in size from less than a
centimeter to a large structure that fills the entire
bladder. They can be asymptomatic or related
to urinary tract infection, pelvic pain, or ureteral
obstruction, and in extreme cases, they can even
be related to urethral obstruction.
At US (Fig 29), CT, and MRI (Fig 33a), they
appear as an intravesical cystic structure continu-
ous with the distal ureter. At VCUG (Fig 33b),
they manifest as a round filling defect inside the
bladder. Given the dynamic nature of the ure-
terocele, which may change in size and shape
because of filling and emptying, VCUG and
especially US, which allow continuous imag-
ing, usually allow more accuracy in making a
diagnosis. Ureterocele eversion into the ureter
can sometimes happen, simulating a periureteral
diverticulum at VCUG (91).
Conclusion
The upper urinary tract is the most common sys-
tem involved in congenital anomalies. Detection
of these anomalies can occur at prenatal US, but
diagnosis in the postnatal period also occurs fre-
quently, mainly as an incidental imaging finding
and also as a cause of complications. Compre-
RG • Volume 41 Number 2
hension of embryologic defects of upper urinary
tract development and the anatomic changes they
can cause is important, and radiologists must
recognize the imaging findings and possible com-
plications of these anomalies to aid in accurate
diagnosis and optimal treatment.
Acknowledgment.—The authors would like to thank Júlio
Leite for collaboration in preparing the illustrations.
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ERRATA
E165

Errata
September-October 2021 • Volume 41 • Number 5

Originally published in:

RadioGraphics 2021; 41(2):462–486
https://doi.org/10.1148/rg.2021200078
Congenital Anomalies of the Upper Urinary Tract: A Comprehensive Review
Abdallah P. Houat, Cassia T. S. Guimarães, Marcelo S. Takahashi, Gustavo P. Rodi, Taísa P. D. Gasparetto,
Roberto Blasbalg, Fernanda G. Velloni

Erratum in:
RadioGraphics 2021; 41(5):E165
https://doi.org/10.1148/rg.2021219009
Figures 1, 4, 5, 9, 14, 16, 17, 21: These figures have been replaced in the online article to correctly depict
the position of the right renal artery.