Congenital Abnormalities of the Urinary Tract

The most frequent  congenital defects and abnormalities of the  genitourinary tract are hydronephrosis, undescended testicles (cryptorchidism), hypospadias and epispadias.

Congenital Abnormalities of the Kidney
o        Hydronephrosis is distention and dilation of the renal pelvis, usually caused by obstruction of the free        flow of urine from the kidney.
o        loin or flank pain
o        kidney may be palpable on examination.
o        may cause renal failure

Absence of one kidney
o        Congenital aplasia
o        Failure to develop one kidney
o        Can be found during ulatrasound examination, CT scanning and Pyelogram studies
o        Ureter absent
o        No ureteric orifice found during cystoscopy
o        Or ureter and renal pelvis are present but the kidney absent

       CT Urography
o        CT Urography has almost completely replaced conventional excretory urography, popularly called IVU.
o        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,

Renal Ectopia
o        Kidney does not ascend
o        Usually near the pelvic brim ; usually left - called pelvic kidney
o        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

Horseshoe Kidney
o        Situated usually in front of fouth lumbar vertebra
o        Fused lower poles common
o        Ureters angulated
o        Infection
o        Nephrolithiasis
o        Fixed mass below umbilicus

       Intra venous pyelogram
       CT scan

Unilateral Fusion
o        Both kidneys are in one loin
o        Usually fused
o        Ureter of the lower kidney crosses the midline to enter the bladder on the contralateral side.
o        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)

Congenital cystic kidneys - polycystic kidneys
o        Hereditary
o        Autosomal dominant trait
o        Not usually detectable until the second or third decades of life and never manifests before the age of 30
o        Irregular upper quadrant mass
o        Loin pain
o        Haematuria
o        Infection
o        Hypertension
o        Uraemia

       CT scan

Simple Renal Cyst
o        Common
o        may be Multiple
o        Diagnosed on ultrasound
o        Rarely requires treatment
o        Treat only if causing obstruction

Aberrent renal vessels
o        Two or more renal arteries are most common on the left
o        Functional end arteries - infarction if divided
o        Veins can be divided because they have collaterals
       Two Right Renal Arteries

Congenital abnormalities of the renal pelvis and ureter
       Duplication of a renal pelvis
o        double renal pelvis
o        Common
o        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
o        dilatation of the ureter

Congenital Defects of the bladder
       Ectopia vesicae - exstrophy of the bladder
o        Easily recognised at birth
o        Umbilicus absent, protruding due to the intraabdominal pressure
o        In addition epispadias
o        Mons and clitoris bifid
o        In the neonate the bladder should be covered with Saran Wrap or clingfilm to prevent trauma to the        delicate mucosa

Congenital abnormalities of the urethra and penis
       Meatal Stenosis
o        Congenital stenosis of the external urethral meatus - normally the narrowest part of the male urethra
o        Associated with phimosis - at times pin hole meatus
o        Back pressure effects
o        Spraying, dribbling

       Congenital Urethral Stricture
o        Rare
       Congenital valves of the posterior urethra
o        Folds of urothelium
o        Obstuction in boys
o        Within prostatic urethra
o        Catheter will pass easily
o        Micturating cystourethrogram done
o        Pass catheter

o        Most common urethral abnormality
o        Glandular hypospadias
o        Coronal hypospadias
o        Penile and penoscrotal hypospadias
o        Perineal hypospadias
o        Avoid circumcision

Abnormalities of the testes and scrotum
       Incompletely Descended Testis
o        Testis is not present in the scrotum
o        In about 4 % of all newborns
o        50% descend during the first month of life
o        The genitals of all newborns must be examined
o        May be associated with inguinal hernia
o        Should be corrected well before puberty
o        Otherwise atrophies
o        The testis may be found at various positions

       Retractile Testis
o        Sometimes the testis intermittently disappears upwards.
o         This phenomenon is called 'retractile testis'.
o        wait for the boy to grow
o        careful followup
o        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
o        At the superficial inguinal ring.
o        In the perineum
o        At the root of the penis
o        In the feroral ring

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