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pediatric urology, anomaly.pptx
1. Pediatric urology and anomalies
Embryogenesis
โ Human genitourinary system develops from the urogenital ridge on the
dorsal surface of the embryo. The latter is further differentiated into the
urinary and genital systems.
โ During pregnancy, the kidney goes through three successive stages of
development : Pronephros, mesonephros and metanephros.
โ Pronephros is a dysfunctional structure and soon degenerates.
โ Mesonephros is a temporary excretory organ. The greater part of it also
degenerates, a part (ureteric bud) produces ureter. Its termination (The
wolffian duct) produces an epididymis, ductus deferens, seminal vesicle and
ejaculatory duct in men.
โ Metanephrosis becomes the definitive kidney. In the formation of the
metanephrosis takes part:
โ Mesonephrotic ureteric bud - Upcoming pelvis and urethra;
โ Arounding mesodermic area โ Upcoming glomerular apparatus and
tubular system.
โ The definitive kidney then migrates upward and takes its place in the lumbar
region.
2.
3. โ Metanephros opens in the urogenital sinus, from
which the urinary bladder and urethra arise.
โ The following basic structures are involved in genital
embryogenesis :
โ Undifferentiated gonads ๐กช The testicles in the male
and the ovaries in the female.
โ The formed gonads then migrate from the abdominal cavity
to the pelvis (female) and scrotum (male).
โ Paramesonephric (the Mullerian) ducts ๐กช The uterus
and cervix, fallopian tubes.
โ Mesonephric (the Wolffian) ducts ๐กช Epididymis,
ductus deferenses, seminal vesicles and ejaculatory
duct.
4.
5. โ Phimosis - is a condition in which the foreskin cannot be
retracted from around the tip of the penis.
โ Treatment: Circumcision (Indication: Recurrent infections,
difficulty urinating); Short frenum correction (Frenulotomy).
โ Paraphimosis - is a condition in which the foreskin can no
longer be pulled forward over the tip of the penis.
โ Often iatrogenic: after catheterization.
โ Causes severe pain.
โ Circulatory failure can lead to necrosis of the head and
foreskin of the penis.
โ Emergency is needed : Manual correction if unsuccessful, an
emergency dorsal slit should be performed, or circumcision.
7. It is necessary to differentiate the phimosis in children
with long foreskin
8. Cryptorchidism
โ Is the absence of at least one testicle from the scrotum.
โ Types: (1) A testis retention at the level of the abdomen,
inguinal canal or outer inguinal ring; (2) Thigh, penodorsal or
epipfascial ectopia.
Descending of the testis to the
scrotum during embryogenesis
9.
10. โ Cryptorchidism is reported in 1.8-2% of children under
1 year of age.
โ The movable (retractile) testicle is the type of
cryptorchidism - The movable testicle with a very
short spermatic cord easily returns toward the
external inguinal ring and canal.
โ Increased risk of infertility and testicular cancer
โ Diagnosis - Physical Examination, Ultrasonography,
Magnetic Resonance Imaging.
โ Treatment:
โ Hormone therapy (human chorionic gonadotropin).
โ Surgery treatment: Orchidopexy (to move a testicle
in the scrotum).
15. Hypospadias
โ Abnormal localization of the urethral opening on the ventral
surface of the penis, scrotum or perineum.
โ Classification by localization : Distal (glanular, coronal,
subcoronal), midshaft, proximal (penoscrotal, scrotal, perineal)
16. โ Evaluating:
โ External urethral orifice diameter, shape, location;
โ Penis size, curved erectile penis, inflammatory changes;
โ Existence of other congenital anomalies of the genitourinary
system;
โ Treatment:
โ Surgical treatment needed: (1) Midshaft and proximal
hypospadias, (2) distal hypospadias,
if it is accompanied by penile fracture the and
Meatal stenosis.
โ Surgery: Correction of curved penis,
formation of new external urethral orifice and
put the external urethral orifice on top of the
pennis.
17. Epispadias
โ Localization of the external urethral orifice on the
dorsal surface of the penis.
โ Rare malformation of the penis than
hypospadias.
โ Often accompanied by urinary bladder exstrophy.
โ It is characterized by penile curvature and
urinary incontinence.
โ The treatment is surgical : Curvature correction,
reconstruction of the external urethral orifice.
19. Hormonal causes : Deficiency of gonadotropins, 5-alpha
reductase deficiency, decreased androgen sensitivity,
Klinefelter syndrome, Idiopathic, ...
Micropenis
Micropenis due to excess weight
20. Urinary incontinence
โ In children urinary incontinence problem solve on reaching of 2 years age,
and when they reach the age of 4, they control nighttime urinary retention
โ Classification:
โ Enuresis โ Normal urination, implemented at an inappropriate and
socially inaccessible time and place.
โ Monosymptomatic enuresis - Involuntary urination at night in sleeping
children; do not wake the liquid; has a genetic tendency.
โ Primary nocturnal enuresis - Occurs after birth and the dry period does
not exceed 6 months.
โ Secondary nocturnal enuresis - Starts after a dry period of 6 months.
โ Nocturnal polyuria, enuresis - Enuresis + according to diary of urination,
the amount of urine produced at night exceeds the functional volume of
the bladder.
โ Daytime anuresis- If the bladder is completely emptied and the urethral
and bladder function is normal, then, due to an inattention, daytime can
occur.
21. โ Urinary incontinence related:
โ Anatomical defects of the urinary tract - Ectopic ureter,
ureterocele, Prune-Belly syndrome, urinary bladder
exstrophy, epispadias, rear urethral valve, Cloacal
anomalies.
โ With neurogenic disorders โ Spinal cord disruption,
Caudal regression syndrome, myelomeningocele, other
C.N.S. disorders.
โ Functional incontinence of urine - Non-neuropathic
bladder-sphincter dysfunction(Imperative urination
syndrome and Dysfunctional voiding); Often associated
with recurrent urinary tract infections.
โ Diagnosis: Anamnesis, examination, urinalysis,
ultrasonography, voiding diary, urodynamic testing,
cystourethrography, urethrocystoscopy.
22. Dilatation of the upper urinary tract
โ Fetal hydronephrosis
โ By the 16th week of pregnancy, fetal hydronephrosis can
be diagnosed.
โ Reasons:
โ Vesicorenal reflux,
โ Ureteropelvic junction obstruction,
โ Megaureter,
โ Urethral valve,
โ Multicystic dysplastic kidney.
โ Diagnosis - Ultrasonography, voiding cystourethrography,
renal scintigraphy, excretory urography.
โ Treatment โ Cause correction.
28. Urinary reflux
โ Backflow of urine in the urinary
bladder or in the renal pelvis:
Vesicoureteral or vesicorenal
reflux.
โ The incidence of vesicoureteral
reflux in the kindergarten and
school age girls is 14-29% in
boys 30%.
โ The basic manifestation is
recurrent upper urinary tract
infections.
โ Advanced case reflux causes
hypertension, kidney failure.
29. โ Diagnosis - Ultrasonography, voiding cystourethrography,
renal scintigraphy, excretory urography.
โ The goal of treatment is to prevent infectious complications
and progressive impairment of renal function.
โ Methods of treatment - Conservative, surgical (depending on
age and reflux grade).
30. Renal anomalies
Quantitative renal abnormality
โ Renal agenesis - Condition in which one (unilateral) or both
(bilateral) fetal kidneys fail to develop.
โ Supernumerary kidney
Right renal agenesis Partially merged left extra kidney
31. Renal anomaly of position
โ Dystopia - Pelvic, lumbar, iliac,
thoracal, crossed.
โ Nephroptosis (Abnormally moving
kidney). It differs from dystopia
with ureteral length.
32. Renal anomalies of shape
โ The horseshoe kidney -
Kidneys become attached together
at the lower end or base
โ S-shaped kidney,
โ L-shaped kidney,
โ Pancake (discoid) kidney,
The horseshoe kidney
33. Volumetric and structural renal abnormalities
โ Renal hypoplasia- Small kidney(congenital)
In clinical practice it is often difficult to differentiate from
acquired atrophy.
34. โ Renal cysts
โ Mylticystic kidney - The kidney consists of irregular
cysts of varying sizes
โ Unilateral anomaly
โ Acquired
โ Reason: Absence of ureter or dull ending.
โ Polycystic kidney disease - development and growth of
multiple cysts within the kidney
โ Bilateral anomaly
โ Genetic, congenital
โ Progressive renal failure
โ Simple kidney cyst โ this kind of cysts are often
unilateral and do not require treatment unless they
cause symptoms or impaired kidney function
39. Renal pelvicalyceal system anomalies
โ Calyceal diverticulum
โ Hydrocalycosis
โ Megacalycosis
โ Unipapillary kidney
โ Extrarenal pelvis or calyces
โ Funnel-shaped pelvis
โ Duplication of renal pelvis
40. Renal vascular abnormalities
โ Additional or multiple blood vessels
โ May cause ureteropelvic segment obstruction
โ Renal artery aneurysm
โ Renal arterio-venous fistula
Multiple arteries of the right
kidney
41. Ureteral anomalies
โ Quantitative anomalies
โ Often concurring with quantitative renal
abnormalities.
โ Even a normal kidney may have a duplicated ureter
โ Ureters can be attached independently to the
bladder or to each other at different levels.
43. โ Anomalies of ureteral endings
โ Lateral ectopy โ Ureteral orifice opens on the lateral wall
of the bladder.
โ An ectopic ureteral orifice may open into the urinary
bladder neck, urethra, vagina, uterus, In men - into the
prostate part of the urethra, seminal vesicles, into the
ejaculatory duct, and into the epididymis.
44. โ Ureterocele
โ Narrowed ureteral orifice and pushed it into the
bladder lumen.
โ It may be related to the duplicated ureter.
โ Urinary Rare anomalies:
โ Rolled ureter,
โ Ureteral diverticulum,
โ Ureteric valve.
45.
46. โ Ureteropelvic segment obstruction
โ One of the most common pathologies.
โ It may be related to the presence of an accessory renal
blood vessel, which often joins the kidney to the lower
pole and causes pressure on the ureteropelvic segment.
48. Urinary bladder anomalies
โ Ectopic urinary bladder
โ The bladder has no anterior wall and the bladder mucosa
joins the abdominal skin in the suprapubic area
โ It is more common in boys and is always accompanied by
an epispadias.