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Congenital Abnormalities

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									 Congenital Abnormalities
    Of the Genitourinary System




    Dr.V.Kasiviswanathan MS
Formerly Sr.DMO / S.Rly. / Palakkad
Let us have a review of the normal
anatomy of the urinary system before
having a knowledge of the congenital
abnormalities
The most frequent congenital defects
and abnormalities of the
genitourinary tract are
hydronephrosis, undescended
testicles (cryptorchidism),
hypospadias and epispadias
Absence of one kidney
 Congenital aplasia
 Failure to develop one kidney
 Can be found during ulatrasound
 examination, CT scanning and Pyelogram
 studies
 Ureter absent
 No ureteric orifice found during cystoscopy
 Or ureter and renal pelvis are present but
 the kidney absent
Absent left
kidney
CT Urography
CT Urography has almost completely
replaced conventional excretory urography,
popularly called IVU.
With the current multi-slice scanner,
especially 64-slice CT scanners, it is
possible to obtain high-quality images of
the kidneys, ureters and bladder,
Angiographic, parenchymal and excretory
phases.
Renal Ectopia

 Kidney does not ascend
 Usually near the pelvic brim ; usually
 left
 If it is not symptomatic the only
 problem is that during the abdominal
 operations the pelvic kidney should
 not be mistaken for any abnormal
 tumour and be injured
Renal
ectopia –
pelvic kidney
Horseshoe Kidney

 Situated usually in front of fouth
 lumbar vertebra
 Fused lower poles common
 Ureters angulated
 Infection
 Nephrolithiasis
 Fixed mass below umbilicus
Horseshoe Kidney
Horseshoe Kidney - pyelogram
Horseshoe Kidney – CT scan
Horseshoe Scintigram
Horse shoe kidney
Unilateral Fusion
 Both kidneys are in one loin
 Usually fused
 Ureter of the lower kidney crosses the
 midline to enter the bladder on the
 contralateral side.
 Both renal pelves may lie one above each
 other medial to the renal
 parenchyma(unilateral long kidney - or the
 pelvis of the crossed kidney faces laterally
 (unilateral S-shaped kidney)
Crossed
fused kidneys
Congenital cystic kidneys –
polycystic kidneys
 Hereditary
 Autosomal dominant trait
 Not usually detectable until the second or third
 decades of life and never manifests before the age
 of 30
 Irregular upper quadrant mass
 Loin pain
 Haematuria
 Infection
 Hypertension
 Uraemia
 CT image : multiple cysts in both kidneys
Bilateral polycystic kidneys ct
Polycystic kidney
      Simple Renal Cyst

Common
Multiple
Diagnosed on ultrasound
Rarely require treatment
Treat only if causing obstruction
Renal Cyst
Aberrent renal vessels

 Two or more renal arteries are most
 common on the left
 Functional end arteries – infarction if
 divided
 Veins can be divided because they
 have collaterals
Normal Renal Arteries
Two Right Renal Arteries
Congenital abnormalities of the
renal pelvis and ureter
Duplication of a renal pelvis
 Common
 Usually unilateral
 Duplication of a ureter
The ureters usually join before they
 reach the bladder

Less commonly the ureters open
  indepedently into the bladder

Congenital megaureter
Duplication of the
      ureter
Congenital defects of the bladder

 Ectopia vesicae – exstrophy of the bladder
 Easily recognised at birth
 Umbilicus absent, protruding due to the
 intraabdominal pressure
 In addition epispadias
 Mons and clitoris bifid
 In the neonate the bladder should be
 covered with Saran Wrap or clingfilm to
 prevent trauma to the delicate mucosa
Exstrophy of
the bladder
Congenital abnormalities of the
urethra and penis
 Meatal stenosis
 Congenital stricture
 Congenital valves
 Hypospadias
 epispadias
             Meatus

Congenital stenosis of the external
urethral meatus – normally the
narrowest part of the male urethra
Associated with phimosis – at times
pin hole meatus
Back pressure effects
Spraying, dribbling
Congenital Urethral Stricture

 Rare
Congenital valves of the posterior
urethra
 Folds of urothelium
 Obstuction in boys
 Within prostatic urethra
 Catheter will pass easily
 Micturating cystourethrogram
 Pass catheter
Posterior Urethral Valve
Micturating
cystourethrogram
Posterior
Urethral Valve
with proximal
dilatation of the
prostatic urethra
         Hypospadias

Most common urethral abnormality
Glandular hypospadias
Coronal hypospadias
Penile and penoscrotal hypospadias
Perineal hypospadias
Avoid circumcision
Normal External Urethral Meatus
Hypospadias types
Hypospadias
Abnormalities of the testes and
scrotum
  Incompletely Descended Testis
Testis is not present in the scrotum
In about 4 % of all newborns
50% descend during the first month of life
The genitals all newborns must be examined
May be associated with inguinal hernia
Should be corrected well before puberty
Otherwise atrophies
Various Positions of the
incompletely descended testis
         Retractile Testis
Sometimes the testis intermittently
disappears upwards.
 This phenomenon is called 'retractile
testis'.
wait for the boy to grow
careful followup
if the testis prefers to stay higher or if the
testis is under tension when brought down,
surgical correction is recommended.
          Ectopic testis

The sites of ectopic testes are
At the superficial inguinal ring.
In the peruneum
At the root of the penis
In the feroral ring
            Phimosis

At birth foreskin adherent to the
surface of the glans penis
Separate spontaneously with time
Can wait for 4 years to separate
Gentle retraction at bath permitted
Forcible retractions injure
Phimosis

								
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