CONGENITAL ABNORMALITIES OF

KIDNEY AND URETER

dr. Sri Indah Aruminingsih, Sp.Rad

Bagian Radiologi
RSUD Abdul Moeloek Lampung

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Learning Objectives

Enlist congenital abnormalities of kidney &

ureter
Describe clinical significance of these
abnormalities, ie
What problems these can cause (symptoms)
What are risks to health ( complications)
Enlist diagnostic investigation plan history,
examination, investigations
Suggest treatment plan in these conditions

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Congenital abnormalities of
kidney
Uncommon ( <1:1000)
Commonly Symptomless found incidentally
US,IVU, CT. Why ????
sometimes detected only when have caused
sufficient damage
Endanger kidneys to various complications

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Agenesis / aplasia of kidney

Bilateral fatal
Unilateral compatable
with normal life,
contralateral kidney
hypertrophed
Failure of mesonephric
duct to bud
Ureteric orifice absent on
cystoscopy
Rarely ureters & pelvis
may be present but renal
tissue is absent or so

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Hypoplasia-dysplasia

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Renal Ectopia / pelvic kidneys
Doesn't ascend, formed near
pelvic brim
No symptom
Present with pain or mass
which one may tempt to
remove as unexplained
pelvic mass
pose diagnostic problems in
case of disease or surgery
May be source of stone,
infection
Liable to trauma
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Crossed Ectopia / Crossed
Dystopia

Both kidneys lie in one loin

May be fused with each other
or separate
Ureter of lower crosses midline
to open into bladder on its
normal side

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Crossed Ectopia contrasted
with normal IVU

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Mal rotated kidneys

Calyces face anteriorly or Obstructi

on/ stasis
antrolaterally

Infecti
Have some element of on
obstruction causing Stone,
Renal
inadequate drainage failure
leading to infection &
stone formation
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 low lying ascent impeded by
inferior mesenteric artery Horse shoe kidney

Lower poles fused in mid line

in front of 4th lumber
vertebra.(isthemus).

Longitudinally lie medially and

downwards,
instead of laterally and down
wards,

Part or whole of pelvicaliceal

system is malrotated ( facing
medially),

Ureters curve over fused poles.

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 obstru
Horse shoe kidney ction

Infec
tion
Stone,
Pain, hematuria, fever,
Renal
mass failure

Exam : fixed mass below umbilicus

Diagnosis: US & IVU
Significance: Liable to disease
angulated ureters + PUJ obstruction urinary
stasis stones, infection & obstruction CRF

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Horse shoe
kidney

Treatment
Asymptomatic = nothing doing
Mild sypmtoms = treat accordingly
PUJ or ureteric obstruction, recurrent infections,
Stones surgery (pyelolithotomy + /-
reconstruction) ISTHEMECTOMY
with straightening of ureters less commonly
done.

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Polycystic kidneys

Hereditary autosomal dominant

Not manifested before 30
Kidneys enlarged, studded with cysts
Unyeilding capsule compresses renal
parenchyma causing atrophy
Liver,lungs and pancreas may be affected
Defact : not clear, many theories

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 Loin pain- weight dragging
upon peddicle or capsule Polycystic kidneys
stretch, hemorhage in cyst,
stone

abdominal mass- confused

with cystic tumor

hematuria- cyst rupture in

pelvis,moderate, episodic.

hypertention, infection, &

uremia/CRF.

Nonspecific symptoms:
anorexia, headache, vague
abdominal discomfort,
vomiting, drowsiness,
anemia.

ESRD: suddenly in middle age,

survival without RRT ( dialysis/
transplant) unlikely
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Polycystic kidneys

US and CT:
cysts in kidneys,liver & others
Simple (aquired) cysts: solitary, smooth walled &
homogeneous contants
Blood & debris cystic adenocarcinoma
FNA- cytology differentiates
IVU
Enlarged renal shadow,
Renal pelvis compressed & elongated
Calyces narrow, stretched over cysts ( spider legs /
bell shaped)

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Polycystic kidneys

Nephrologist :
BP control, infection,anemia, disturbances of Ca
metabolism, low protein diet to delay need for
DIALYSIS
Urologist:
Surgical / laproscopic deroofing of cysts ( Rovsings
operation)
Relieves pain & pressure saving kidneys
Rarely performed / not preserve function
Renal transplant/ pretransplant bilateral native
kidneys nephrectomy

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solitary / Simple (acquired)
renal cysts
Common, may be multiple ( not always 1 )
Incidentally found no treatment needed
Rarely symptomatic; pain- stretch, bleed
in cyst, infection,mass
papa-pelvic cyst at hilum presses PUJ
obs
IVU: filling defect.
US, CT :smooth, homogeneous contant
DD: hydatid, cystic adenocarcinoma
Percuteneous FNA Cytology
Treat only in case obstructing

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Infantile polycystic kidneys

Rare
Inheritance- autosomal recessive
Enlarged kidneys may obstruct labour,
Many stillborn
Die of renal failure in in early childhood

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Unilateral Multicystic
kidney
More common
Presents as nonfunctioning mass
Exploration & removal is treatment of chioce
DD: wilms, neuroblastoma, congenital
hydronephrosis

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Aberrant vessels

Variation in no of vessels common

Arteries END arteries, damage ischemia
Veis extensive colaterals = can be ligated
are not cause of hydronephrosis- though the
bulging renal pelvis in between them makes
them noticeable

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Abnormalities of renal pelvis
and ureter
Most common, harmless,
asymptomatic

DUPLICATION OF RENAL PELVIS

Common,4%, usually unilateral left

Upper pelvis small, drains upper
calyx

Asymptomatic no treatnment

If one moity severly damaged partial

nephrectomy

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Duplex kidney, double moiety

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DUPLICATION OF URETER. 3%

Often join before reaching bladder,

suffer obstruction ( esp from
stones) & YOYO reflux

May open independently, ureter

from upper moity opens distal
and medial to its fellow.

Uppermoity ureter suffers

ureterocele.

Lower moity ureter suffers VUR

Infection, calculus formation, PUJ

obst and VUR, ectopic opening

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Abnormalities of ureter
Ectopic uretric opening
Female into urethra below sphincter on vagina = incotinance
since childhood with desire and passage of urine normally as well.
IVU and cystoscopy ( indigocarmine)
Male.continent as opening is above sphincter
Opening in trigone apex, post. Urethra, seminal vesical or ejaculatory
duct functionally abnormal, infection common
Treatment:
Frequently ectopic ureter drains hydronephrotic, chronically infected
moity --- best excised - nephrectomy
Incontinence can be cured and renal function preserved by implanting
ureter into bladder ( tunneling) or joining its fellow. (URETERO-
NEOCYSTOSTOMY, URETERO-URETEROSTOMY)

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Abnormalities of ureter
CONGENITAL MEGA URETER
Uncommon, bilateral
FUNCTIONAL obstruction
at lower end dilatation
& infection
Ureteric orifices normal,
ureteric cath passes easily
Reflux not feature till
opened endoscopically
Treatment: refashioning
and tunneled reimplant
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Abnormalities of ureter
POST (retro) CAVAL URETER

Right ureter passes behind

IVC instead of lying to the
right of it (laterally)

If causing obstruction, can

be devided and joined in
front of cava long
oblique ETE anastomosis

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Abnormalities of ureter
URETEROCELE
Cystic enlargement of
intramural portion of ureter
Thought to result from
congenital atresia of ureteric
orifice
Though present since
childhood, unrecognised till
adulthood
More common in female,
cause BOO by obstructing /
prolapsing into internal
urinary meatus. May even
prolapse out of urethra

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 Adder head on IVU
Cyst wall composed of
urothelium only
confirmed on cystoscopy
Translucent cyst, enlerging
and collapsing as urine
flows
Treatment avoided unless
symptoms of infection /
stone
Endoscxopic diahermy
incision / deroofing
Postoperatove MCUG to
see VUR
Ureteral reimplant
Sever hydronephrosis,
pyonephrosis
nephrectomy
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Hydronephrosis

Aseptic
dilatation of
pelvicaliceal
system due to
complete or
partial
obstruction.
Unilateral
hydronephrosis
Epsilateral ureteric
obstruction
( unilateral
supravesical
obstruction)
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Hydronephrosis:
Bilateral
Bilateral ureteric obstruction (
bilateral supravesical
obstruction)
Urethral obstruction (
bladder outlet obstruction,
infravesical obstruction )
Detrusor hypertrophy
intramural ureteric
obstruction
VUR
Pregnancy physiologic
dilatation - progesterone,
early pregnancy 20 weeks
marked reverts 12 week
of delivery
Infection, diagnostic
difficulty in acute abdominal
pain in pregnancy
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Causes of Ureteric Obstruction

Extramural
Tumors of cervix, ovary, uterous, vagina, urinary bladder, prostate, rectum,
colon, caecum & lymphomas
Idiopathic retroperitoneal fibrosis
Retrocaval ureter
Pararenal cysts
Aberent vessels
Intraluminal
Calculus, sloughed papilla, clot, ureteric malignancy
Intramural
Congenital PUJ obstruction or stenosis
Ureterocele and congenital small ureteric orifice
Strictures ( stone, repair, tuberculosis, schistosomiasis)
Ureteric / vecsical malignanncy
Kenks & adhesions ( sec to VUR)

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Bladder outlet obstruction

Phemosis / fused synichae, Ext. meatal stenosis

Urethral Stone / foreign body impaction,
Enlarged prostate--------- benign / malignant /
inflammatory/abscess
bladder neck stenosis, Post urethral,valve Urethral
stricture
neoplasm of bladder, urethra, prostate and penis
vesical calculus, foreign body
Neurogenic
Detrusor sphincter dys-synergia ,
neurogenic bladder ,spine trauma, multiple
seclerosis. DM
Stones , vesical , urethral,

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Congenital (idiopathic)pelvi-
ureteric obstruction
Right side effected more
Female :male = 2:1
Clinical features
Insidious onset mild loin pain / dull ache /
Sensation of dragging heaviness made worse
by fluid intake
Little to call attention to renal damage
Kidney may / may not be palpable renal
failure intervenes before kidneys dilate
Intermittent hydronephrosis / Dietls crisis
Pain, swelling passage of large volume
urine pain & swelling disappears
Trauma
Mass obstructed kidney
hypertention

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Congenital(idiopathic) pelvi-
ureteric obstruction
obst
Pathogenesis ructi
Adynamic segment of ureter Inf on
Ston
Polyps, valves, kiks, angulation ect e,
High origin, abnormal relation to vessels ion
Renal
Narrowing, strecture, failur
Pathology e
Pelvicaliceal system dilates at the expense of parenchyma which is
compressed & destroyed by surrounding unyielding capsule. ( pressure
atrophy)
Resultant nonfunctioning Kidney consists of thined out cortex making
lobulated sac containing pale low specific gravity uriniferous fluid
Extrarenal pelvis
Renal damage delayed and prolonged % lesser
Intrarenal pelvis
Renal damage rapid and severe
Stasis leads to infection and stone formation pain, fever , hematuria

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 PUV
(Posterior Urethral Valve)
Type I: PUV are mucosal folds extending anteroinferiorly
from the caudal aspect of the verumontanum (colliculus
seminalis), often fusing anteriorly at a lower level. They
are derived from the plicae colliculi.
Type II: PUV are mucosal folds extending anterosuperiorly
from the verumontanum toward the bladder neck.
Type III: PUV are disk-like membranes located below the
verumontanum in two different ways:
- In the first type, the valve extends from the
verumontanum distally.
- In the second type, the valve extends from the
verumontanum towards the bladder neck.
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Matur
Nuwun Semoga
Bermanfaat

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