Congenital anomaly of kidney & Ureter.ppt

Radiological evaluation of congenital anomaly of kidney and ureter

1. Congenital Anomaly of
Kidney & Ureter
Dr. Nazia Naureem
DMRD student
Department of Radiology and Imaging
Rajshahi Medical College

2. Renal Anatomy

3. Anatomy

4. Development
•The Urinary System goes through three phases on its way to becoming fully
functioning:
1.Pronrphros
2.Mesonephros
3.Metanephros

Starting from 4
th
week of intrauterine life
•The collecting system arises from the
 ureteric bud, which arises from
the
 mesonephric duct in the fourth week of gestation. The renal parenchyma
arises from the metanephros, which appears in the fifth week, a derivative of
the intermediate mesoderm.
•

6. Imaging Anatomy

10. Renal Agenesis
•Renal agenesis 
refers to a congenital absence of one or both
kidneys.
•Occurs due to failure of the ureteric bud to reach the metanephros
and is associated with an absent ipsilateral ureter and hemitrigone.
•On IVU, the renal shadow is absent, replaced by air-filled splenic or
hepatic flexure.
•The contralateral kidney is enlarged due to compensatory
hypertrophy.
•An absent renal shadow may also be due to renal ectopia,
nephrectomy or severe atrophy from VUR/multiple infarctions.

11. Association:
a.VATER anomaly (Vertebral, Ventricular septal anomalies,
Anorectal/Tracheal/Esophageal fistula/atresia, Radial bone).
b.Agenesis of ejaculatory duct with resultant seminal vesicle
obstruction/cyst, which is called Zinner syndrome.
c.Uterine anomalies like bicornuate uterus with ipsilateral obstruction
of hemivagina and renal agenesis constitute the OHVIRA
(obstructed hemivagina and ipsilateral renal agenesis).

12. •CT of the abdomen demonstrates a
solitary left kidney, with physiological
hypertrophy. The morphology of the
left kidney is normal. The right renal
bed is empty, with no evidence of prior
surgery or renal tissue.
CT of the abdomen demonstrates a solitary left kidney, with
physiological hypertrophy. The morphology of the left kidney is
normal. The right renal bed is empty, with no evidence of prior
surgery or renal tissue.

14. Bilateral Renal Agenesis :
-Potter syndrome
-Fatal in first few days of life due to pulmonary
hypoplasia secondary to the associated
oligohydramnios

15. Renal Hypoplasia
•The kidney is small due to an intrauterine vascular insult
but otherwise normal and with normal renal function.
•Bilateral hypoplasia often have evidence of renal
insufficiency
•On IVU, the hypoplastic kidney is seen as a functioning,
small, smooth, normal shaped kidney with 5 (or < 5)
normal calyces.
•Hypoplastic kidney may be supernumary & usually left
sided & caudally positioned, with ureteric drainage into
the bladder or into the ureter of normal kidney.

16. There is a complete left renal hypoplasia, with a 3.5mm upper pole
calculus. The right kidney has undergone slight compensatory
enlargement, and contains multiple simple cysts

17. Hypoplastic non-functional kidney, axial contrast-enhanced MDCT
image (a) and coronal maximum intensity projection (b) show a left
hypoplastic non-functional kidney (arrow) and a hypertrophied right
kidney

18. Renal dysplasia
•Failure of development of normal renal tissue; usually unilateral &
affects the whole kidney
•Occasionally bilateral & leads to renal failure in infancy
•Rarely affects upper pole of one kidney only
•On ultrasound, the kidney is small with either smooth outline or
marked fetal lobulation. There is loss of corticomedullary
differentiation and tiny cortical cyst may be visible
•On IVU the kidney excrete contrast, which demonstates a lack of
papillary development, the calyces appearing clubbed.
•There is an association with vesicoureteric reflux and numerous
congenital syndromes including Beckwith-wiedmann and Laurence-
moon-Biedl

19. Supernumerary Kidney
•Very rare
•Most common on the left side and usually caudal to the
native kidney.
•Ureteric drainage may be separately into the bladder or
into the normal kidney’s ureter.
•Mostly the accessory kidney is smaller in size with
reduced excretion.
•The evaluation may be done by ultrasound,
 IVU, CT,
MRI and nuclear medicine studies like
 DMSA and DTPA
scans.

22. Rotational Abnormalities
• Also known as
 renal malrotation, refers to an anatomical variation
in the position of the
 kidneys, in particular to anomalous orientation
of the renal hilum.
•It may occur unilaterally or bilaterally.
• It is almost always an asymptomatic incidental finding.
•The renal hilum is normally directed anteromedially. The renal hilum
is initially oriented anteriorly, but during its ascent from the pelvis,
the kidney rotates 90° along its longitudinal axis to its more typical
orientation.

23. Anomalies in this process can result in:
•Incomplete rotation
 or non-rotation (most common): hilum faces
anteriorly,
 ureters are located laterally
•Excessive rotation
 (hyper-rotation): hilum faces posteriorly; renal
vessels are located posteriorly
•Reversed rotation:
 hilum faces laterally, renal vessels are located
anteriorly, ureter is located laterally
•Sagittal rotation: rotation around its hilum in the sagittal plane.

24. Anterior malrotated kidney, axial (a) and sagittal (b) contrast-enhanced
MDCT images show an anterior malrotated left kidney with
pelvicaliceal stones, volume rendering (c) shows stones in anteriorly
oriented renal pelvis

25. Lateral malrotated kidney, axial maximum intensity projection (a) and volume
rendering (b) show a lateral malrotated right kidney, the renal pelvis
shows abnormally lateral location

26. Renal Ectopia
•Pelvic Kidney: Failure of complete
ascent of the kidney to the level of
second lumber vertebra.
•Prone to trauma, VUR, and calculus
formation, may be associated with
contralateral agenesis, ectopia or
midline fusion of both unascended
kidneys as a “pancake” kidney.
•Often small and abnormally rotated
•Blood supply: From iliac artery or the
aorta

27. Pelvic kidney

30. Renal Ectopia
•Overascent is rare.
•Is almost always limited by diaphragm but there may be
some superior herniation through a localosed eventration
and very rarely a true intrathoracic kidney.
•

33. Horseshoe kidney,
•Most common fusion anomaly,
•1: 400 live births,
•Male Predominance
•Two kidneys joined at lower pole by
parenchymal or fibrous isthmus due to
contact between the metanephric tissue of
developing kidney.
•Association: PUJ obstruction (PUJO), calculi
and Wilms' tumor, as also anomalies of the GI
tract and cyclic vomiting syndrome (CVS),
and Turner's or Ellis–van Creveld syndrome.

34. Radiographic Features :
•On plain film and IVU, the characteristic horseshoe configuration
may be seen although the isthmus is poorly visualized over the
lower lumbar spine.
•Abnormal axis of each kidney with lower calyx more medial than
upper calyx
•Bilateral malrotation of renal pelvises in anterior position
•Isthmus lies anterior to aorta and inferior vena cava (IVC) but
behind inferior mesenteric artery (IMA)

35. On the scout image, there is a soft tissue mass on either side of the midline
with a central isthmus, following contrast injection the orientation of the
pelvicalyceal system is clearly outlined. Lower pole of kidneys are closest to
midline, which is the reverse of normal. Both kidneys appear rotated and
lower poles are fused with formation of isthmus at the level of L3-L4.

36. Horseshoe kidney with anteriorly oriented renal pelvis, axial contrast-
enhanced MDCT image (a) shows a horseshoe, the renal isthmus is
found anterior to the great retroperitoneal vessels, corresponding
volume rendering (b) better show the inferior renal isthmus, the
normal renal arteries, and the anterior location of the renal pelvises

37. I = isthmus, IMA (arrow)

38. Crossed fused ectopia
•It is a type of horseshoe kidney with both kidneys on the same side.
•Male predeliction (2:1)
•On IVU, the left kidney usually crosses to the right forming an enlarged
right renal shadow and an absent left renal shadow.
•Two distinct malrotated pyelograms are evident with the upper moiety
ureter orthotopically inserting into the ipsilateral side of the bladder and
the lower moiety ureter crossing the midline to insert orthotopically into the
contralateral side of the bladder
•The upper pole of the ectopic kidney is usually fused to the lower pole of
the other kidney , although fusion may occur anywhere

39. •Both kidneys are seen on the right side (fused) with adequate excretion of contrast medium in five minutes.
•Right ureter: normal in course and caliber and opens normally in an orthotopic position
•Left ureter: short, non-tortuous, normal caliber and crosses to the left side and inserts orthotopically.

41. 2-Pacake / Discoid Kidney :
-Bilateral fused pelvic kidneys , usually near
the aortic bifurcation

44. Fetal Lobulation
•Renal tissue develops as a series of 8-16
lobules & the lobulated structure remains
apparent at birth. This feature gradually
disappears over the first 5 years of life as
the kidney grows. In upto 5% of the
population, however lobulation persists.

46. Fetal renal lobulation, coronal maximum intensity projections (a, b)
show persistent fetal lobulation in both kidneys

47. Renal Pseudotumors
•Prominent areas of normal renal tissue may develop and appear as
mass lesions, particularly on IVU.
•No clinical significance but may be misdiagnosed as neoplastic
mass.
•A hypertrophied column of Bertin is a cortical prominence that
splays calyces without bulging the contour and is continuous with
renal cortex.
• Dromedary humps are focal bulges caused by the normal splenic
impression on the superolateral margin of the left kidney. The
calyces underlying the hump extend further laterally into the hump
than the other calyces, preserving a normal parenchymal thickness .

48. Dromedary Hump

50. Compensatory Hypertrophy :
-Occurs when existing healthy nephrons enlarge to
allow the healthy renal parenchyma to perform
more work
-Seen in :
a) Nephrectomy
b) Renal agenesis
c) Renal hypoplasia
d) Renal atrophy
e) Renal dysplasia

51. Megacalycosis and
Polycalycosis
•Dilatation of calyces in the absence of obstruction with a normal
caliber renal pelvis and ureter constitute magacalyces.
•Often Increased number of calyces and calyces have a polygonal
appearance when it is called megapolycalycosis. The number of
calyces increases to 20–25, where as normal kidney has 10–14
calyces.
•Renal cortical thickness is maintained and function of the kidney is
preserved.
•Increased risk of infection and stone formation due to stasis .
•Megacalycosis can sometimes coexist with primary megaureter
which is important to recognize and avoid interpreting the calyceal
dilatation as due to obstructing primary megaureter.

52. MIP Image of a CT urogram shows numerous dilated, polygonal-shaped
calyces in the left kidney with convex outer margins (red arrows), the calyces
fit together like pieces of a puzzle. The renal pelvis (white arrow)
is not dilated and the ureter is normal in size (yellow arrow) helping to
differentiate this from a cause of obstructive hydronephrosis.

55. Congenital Megaureter
•A congenital (primary) megaureter encompasses causes of an
enlarged ureter which are intrinsic to the ureter. Persistent dilatation
and ureter with diameter > 0.7cm should be consider as abnormal.
• It includes:
•Obstructive primary megaureter: Obstructive primary megaureter is
related to a distal adynamic segment with proximal dilatation and is a
common cause of obstructive uropathy in children.
•Refluxing primary megaureter: Refluxing primary megaureter is a
result of an abnormal vesicoureteric junction, which impedes the
normal anti-reflux mechanisms. This can be due to a short vertical
intramural segment, congenital paraureteric
diverticulum, ureterocele with or without associated duplicated
collecting system.
•Non-refluxing unobstructed primary megaureter: The vesicoureteric
junction is normal, with no evidence of reflux or obstruction the ureter
is enlarged. The reason for this is unknown.

57. Primary megaureter in a 7-month-old boy, longitudinal US image
through the bladder ( ) demonstrates a dilated distal ureter (
★
u) and
a narrowed juxtavesical ureteral segment corresponding to the
aperistaltic segment (arrow)

60. Pyelocalyceal Diverticulum
•Congenital outpouching from the renal collecting system lined by
transitional epithelium.
•Complication: Stone formation
•
◾
Type 1: Pyelocalyceal diverticulum communicates with a minor
calyx or infundibulum.
•
◾
Type 2: Communicates with renal pelvis or major calyx
Scheme of various arrangements of the calyceal cyst and hydronephrosis. (a) Norm; (b)
hydrocalycosis of the forniceal and minor calyx; (c) hydrocalycosis of the major calyx; (d)
hydrocalycosis of the minor calyx; (e–g) complex hydrocalycosis

61. •On USG, an anechoic cyst will be seen indistinguishable
from other cortical cysts, unless a mobile calculus is
demonstrated within.
•On IVU, the outpouching from the pelvicalyceal system
will show gradual filling-in of contrast.
•The same finding can be observed in CT urography
•Intervention and ablation is required if complicated with
infection/calculi.

62. A right renal rounded intraparenchymal hypodense lesion
with layered dependent density representing milk of calcium,
in keeping with a calyceal diverticulum.
Trans abdominal ultrasound shows large cystic structure in the
middle portion of right kidney with mobile, hyper-echoic material
in the dependent portion (milk of calcium).

63. Infundibular and
Infundibulopelvic Stenosis
•Extremely rare anomaly where there is congenital narrowing of the
infundibulum with resultant hydrocalycosis.
•Associated stenosis of pelvis can occur resulting in infundibulopelvic
stenosis .
•Unilateral or bilateral.
•Considered as a milder form of disease in the spectrum of
obstructive renal dysplasias, the most severe form being
infundibulopelvic atresia causing multicystic dysplastic kidney
(MCDK)

64. Pelvi-Ureteric Junction
Obstruction (PUJO)
•PUJO partially obstructs the passage of urine from the renal
pelvis to the ureter. It is the most frequent cause of
hydronephrosis in the fetus and can also present in adulthood. 
•Spectrum of severity ranging from severe antenatal hydronephrosis
with global cortical loss to an adult asymptomatic PUJO.
•Common anomaly and is found in men with a 2:1 predominance
•The left kidney is affected twice as frequently as the right
•Association: Multicystic disease of kidney, renal agenesis, renal
duplication and VUR
•On plain radiograph, a massive hydronephrosis from PUJO can
displace bowel, creating a paucity of bowel on the side of the
abnormal kidney as well as reveal an absence of the renal shadow.

65. Cause:
Intrinsic Causes:
a)Intrinsic narrowing or stenosis
b)Failure of canalization
c)High insertion of ureter into the pelvis
d)Excessive collagen deposition in the PUJ wall
e)Abnormal arrangement of smooth muscle(Impaired peristalsis)
Extrinsic causes:
a)Aberrant or accessory renal artery/vein crossing the PUJ
b)Bands or kinks at PUJ
c)Extrinsic compression by abnormal vessels or fibrous adhesions

67. Tc99m-MAG3 demonstrate progressive and persistent accumulation
of radio-tracer in the right kidney, in keeping with a pelviureteric
junction obstruction.

68. • ■ On IVU, there is severe acute obstruction with a delayed,
increasingly dense nephrogram and delayed excretion resulting in a
negative pyelogram: dilated PCS lucency within a dense
nephrogram. The opacified PCS shows clubbed calyces, dilated
pelvis and “crescents” peripheral to calyces due to stasis in
displaced collecting ducts. The PUJ appears tightly closed with
ureter unopacified; the large round dilated pelvis, round dilated
calyces and thin rim of parenchyma have the appearance of an
elephant's foot

70. •■ In chronic obstruction, cortical loss, calculus formation
and pyonephrosis can be demonstrated. Subtle PUJO
presents as a large extra-renal pelvis that balloons
following frusemide-induced diuresis
• ■ Following pyeloplasty, IVU shows a characteristic
straight vertical medial edge to the pelvis with the ureter
arising from the inferomedial angle of the pelvis.
Dilatation of the collecting system persists despite
improvement of urinary drainage

71. Ureteropelvic junction stenosis, coronal maximum intensity projections
(a, b) show left ureteropelvic junction stenosis, close to crossing
vessels of uncertain involvement (arrow), pyelocalycial dilatation
and decreased cortical nephrogram are seen

72. Duplication Abnormalities
•These range from a bifid renal pelvis to completely duplicated pelvis and
ureters.
•■ In incomplete duplication, bifid ureter with ureteric fusion at varying points,
forming a common distal ureter and orifice in the bladder.
•Bifid pelvis: Fusion occurs at proximal ureters
•Bifid Ureter: Fusion occurs at more distal point

73. •In complete duplication, the orthotopic lower moiety ureter refluxes while the
ectopic upper moiety ureter is associated with a ureterocoele that obstructs.,
•the upper pole moiety ureter inserts medially and at a lower level in the
urinary bladder compared to the lower pole moiety ureter. This upper moiety
ureter could also have an ectopic insertion and can sometimes be
associated with a ureterocele, causing obstruction of the upper pole moiety.
•The lower pole moiety inserts at a higher and lateral position in the bladder
making it prone for VUR. This pattern of ureteric insertion in complete
duplication of ureters is called the Weigert Meyer rule.

74. • On IVU, the duplex causing renal enlargement on plain radiograph.
Evidence of lower moiety reflux nephropathy is seen in the form of
cortical scarring over clubbed calyces, while upper moiety
obstruction, marked hydronephrosis, parenchymal thinning and
delayed excretion causes inferior displacement of the lower moiety
(“drooping lily” sign)

75. • “
◾
Faceless kidney” sign is a CT sign described in duplex kidney.
On axial images, a section through the intervening parenchyma
between the two collecting systems do not show the normal sinus
signature. Since the normal face of the kidney is lacking, it has been
termed the “faceless” kidney

76. • MRU is the technique of choice to clearly delineate the urinary tract
anatomy, course of the ectopic ureter and site of ectopic insertion,
especially when there is a poorly functioning moiety
• Isotope studies/dynamic MRU help to assess the function of the
two moieties
•Scintigraphy helps to assess drainage and split renal function in an
obstructed system. Though dynamic MRI can also be used for this
assessment, its performance in comparison to scintigraphy needs to
be evaluated prospectively.

78. Incomplete duplicated collecting system, coronal maximum intensity
projection (a) and volume rendering (b) show an incompletely
duplicated left pyelocalyceal system and ureter, the left ureters
converge and fuse near the bladder

79. Ureterocele
•Cystic dilatation of terminal ureter in the intravesical intramural
segment, either orthotopic or ectopic, the latter occurring at an
ectopic site of insertion of ureter.
•On ultrasound, cystic dilatation of the terminal ureter can be
demonstrated in continuity with the dilated proximal ureter
• On MCU, the ureterocele is seen as a filling defect in a contrast
filled bladder.
•On IVU, contrast filled rounded or oval density is seen within the
bladder with a surrounding radiolucent halo representing the wall of
ureterocele forming the classic “cobra head”/“onion bulb”
appearance.

80. Types:
Intravesical ureterocele (~25%): occur at the normal vesico
ureteric junction position known as simple/orthotopic
ureterocele .
– Considerably less common than the ectopic variety.
–There is a congenital prolapse of a dilated distal ureter into
the bladder lumen.
–Bilateral in about 30% of cases .
Extravesical ureterocele (~75%): occur ectopically low and
medial, near bladder neck/urethra
–Also known as "ectopic" ureterocele.
– Almost always associated with a duplicated collecting
system and the result of abnormal embryogenesis. There is an
abnormality in the early development of the intravesicular
ureter, the ipsilateral kidney and its collecting system .

81. Cobra head sign

84. Ureterocele, early anteroposterior voiding cystourethrography (VCUG)
demonstrates small ureterocele

85. Ureterocele, coronal curved (a) and axial (b) contrast-enhanced MDCT
images show distal ureter balloons at its opening into the bladder,
forming a sac-like pouch

86. Anomalies related to Vascular
Development :
1-Aberrant vessels
2-Retrocaval ureter

87. Aberrant vessels
•As the kidney ascends during embryologic development , it derives
its blood supply from the aorta at successively higher levels with
regression of the lower level vessels
•If the lower level vessels persist , aberrant renal arteries will be
present
•Aberrant vessels can compress the ureter anywhere along its
course , giving rise to obstruction
•With color Doppler , aberrant vessels may be seen crossing the
ureter at the level of ureteric obstruction.

88. (a) Excretory urogram after 30 min shows severe dilatation of the right pelvocalyceal
system with cortical thinning and smooth obstruction of the right ureteropelvic junction,
(b) CTA with a maximum intensity projection technique demonstrates two renal
arteries originating from the aorta, an inferior aberrant artery (arrow) is crossing over
the ureteropelvic junction

89. Retrocaval ureter
•Also known as circumcaval ureter
•Right ureter swings medially over pedicle of L3/4, passes behind
IVC, then exits anteriorly between IVC and aorta returning to its
normal position, produces varying degrees of proximal
hydroureteronephrosis
•Rare but well recognized congenital anomaly , there is a 3:1
predominance in men , with most patients presenting with pain in
the 2
nd
to 4
th
decade of life

91. Intravenous urogram showing right-sided hydronephrosis and the
dilation of the proximal ureter up to the level of the L3 transverse
process, the medial deviation of the ureter at this level (arrow) gives
rise to the typical fish hook or reversed S appearance

94. Ectopic ureter
•An ectopic ureter is a congenital renal anomaly that occurs as a
result of abnormal caudal migration of the ureteral bud during
its insertion to the urinary bladder.
• Normally the ureter drains via the internal ureteral orifice at the
trigone of the urinary bladder. 
•In females, the most common sites forectopic ureter insertion
are the bladder neck and upper urethra (33%), vaginal vestibule
between the urethra and introitus (33%), vagina (25%), and
cervix and uterus (<5%) . 
•In males, the ureter may insert into the lower urinary bladder,
posterior urethra, seminal vesicle, ductus deferens, ejaculatory
duct, and rarely the rectum.

95. Cystic Renal Diseases
•Multicystic Dysplastic Kidney (MCDK) : is a
nonhereditary developmental abnormality in which the kidney is
replaced by multiple cysts with no functioning intervening
parenchyma. Postulations include an in utero insult/obstruction
resulting in maldevelopment of kidney and replacement by bizarre
cysts. Embryologically, it is thought to be due to failure of the
ureteric bud to induce the metanephric blastema to form the nephric
vesicle and its subsequent development into nephrons.
•On imaging, cluster of multiple varying sized non communicating
cysts are seen replacing the kidney. There is no functioning normal
renal parenchyma.
•Commonly diagnosed on antenatal scan.

97. Polycystic Kidneys
•Autosomal recessive polycystic kidney disease
(ARPKD): also known as infantile polycystic kidney disease is a
hereditary disorder characterized by cystic dilatation of renal
tubules. This occurs due to a genetic abnormality that causes
abnormal development of renal tubules due to alteration in primary
cilia.
•Consists of bilateral enlarged kidneys containing numerous tiny (1–8
mm) cysts.
• Renal function improves with age but associated periportal fibrosis
worsens and causes liver failure.

99. •Autosomal dominant polycystic kidney disease
(ADPKD): is a bilateral, progressive disease with numerous cysts
of varying size that can grow extremely large, replace normal renal
parenchyma and lead to renal failure.
•ADPKD is associated with cysts in the liver, pancreas, spleen and
lung, berry aneurysms, coarctation, and valvular heart disease.
• On plain radiograph, curvilinear cyst calcification and enlarged
kidneys are seen with multiple, well-defined, low density, round foci
in the nephrogram (“Swiss cheese” nephrogram).
• On IVU, the kidneys are enlarged, with cysts splaying, thinning, and
stretching the calyces, broadening the infundibula and give rise to a
spider leg PCS deformity.

102. Others
•Medullary Cystic Kidney Disease/Nephronothisis
•Glomerulocystic Kidney Disease (GCKD)

103. Medullary Sponge Kidney
•This is a congenital abnormality of tubules in the
• medullary pyramid, where concretions within fusiform
• and cystic ducts produce stippled and bristle medullary
•calcifications that can progress to medullary nephrocalcinosis.
• It can be associated with Caroli disease and Ehlers-Danlos
syndrome.
•On IVU, multiple linear, brush-like (“paintbrush” sign) and saccular
contrast collections are seen within the medulla.
•This differs from the normal papillary blush where distinct and
resolvable tubular structures are not evident. Small calculi within the
ectatic tubules can progress to medullary nephrocalcinosis and the
formation of ureteric calculi.

106. Prune Belly Syndrome :
•Related to bladder outflow obstruction in utero secondary to urethral
valves causing hydroureteronephrosis in Male
•It consists of three major findings :
1-Gross ureteric dilatation
2-Anterior abdominal wall underdevelopment (resulting in the
prune belly appearance)
• 3-Bilateral undescended testes (cryptorchidism) in males
•There is often an association with other respiratory, gastrointestinal,
musculoskeletal, and cardiovascular anomalies
•It is also known as
 Eagle Barrett syndrome or triad syndrome

107. Urinary tract abnormalities include :
1-Bilateral hydroureteronephrosis: often with extremely dilated,
tortuous ureters
2-Varying degrees of renal dysplasia
3-Enlarged urinary bladder, often with
 urachal diverticulum
4-Vesicoureteral reflux is common
5-Poor bladder contractility
6-Dilated posterior urethra without urethral obstruction

108. The abdomen of an infant with prune belly syndrome shows marked
distention of the abdomen and bulging flanks secondary to a large
urinary system and the absence of abdominal wall musculature

109. •X ray findings:
•globular shape of abdomen
•bilateral bulging flank

111. Prune-belly syndrome, IVU
 
(a) 
and VCUG 
(b) 
images show the characteristic
large floppy bladder ( ) and marked bilateral hydronephrosis and
★
hydroureter (u) with renal parenchymal wasting, note the anomalies in the
pelvic bones and both femurs due to renal osteodystrophy in
 
b

112. Take Home Message
•Congenital anomalies of the kidney and ureter are not rare and may
remain silent until complications arise.
•Imaging plays a pivotal role from screening to precise anatomical
delineation and functional assessment.
•A structured, multimodality imaging approach ensures accurate
diagnosis, appropriate management planning, and preservation of
renal function.
•Ultimately, the radiologist’s role is crucial in guiding clinicians toward
timely intervention and long-term patient outcomes.