pediatric urology h

8/3/2019 Pediatric Urology H

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Pediatric Urology


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Introduction

Pediatric urology is the diagnosis and

treatment of congenital and acquired

urological conditions and diseases in

children.

Pediatric urologists treat conditions of the

male reproductive tract and the male and

female urinary tracts.


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Embryology

Most of the genitourinary tract is derived from

mesoderm.


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The mesonephric (Wolffian) ducts develop

laterally, and advance downwards to fuse

with the primitive cloaca (hindgut).

At week 5, a ureteric bud grows from the

distal part of the mesonephric ducts and

induces formation of the metanephros in the

overlying mesoderm . caudal growth .. Abdominal organ .

Urine production starts at week 10.


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Thus, in both males and females, the

mesonephric duct forms the ureters and renal

collecting system.


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Content

Cryptorchidism

Vesicoureteral reflux

Ureteral anomalies Bladder anomalies

Penile and urethral anomalies


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Cryptorchidism

Definition:

Cryptorchidism literally means hidden or

obscure testis and generally refers to an

undescended or maldescended testis.


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Undescended testes

It is the most common abnormality of male sexualdevelopment. In this condition, the testis is not located inthe scrotum.

The testis can be ectopic, incompletely descended,

retractile, and absent oratrophic . A palpable undescended testis is found in 3%-5% of

newborns

3030% in premature male neonates.% in premature male neonates.

only 0.7%-1% of 1-year-old infants have a persistentundescended testis.

Spontaneous descent after the first year of life isSpontaneous descent after the first year of life isuncommonuncommon


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The maldescented testes may be arrested at any

point on its path of descent from the posterior

abdominal wall to the scrotum;

Abdominal

Inguinal canal

At theexternal ring

Upper part of the scrotum

Perineal


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Failure of testicular descent is likely to be caused by

either

1. A disturbance of the hormonal environment

(LHRH -> LH-> testicular hormone axis)

incompletely descended testis2. Mechanical factors obstructing the progress of

testicular migration. ectopic testis.


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Complications

1. Testicular malignancy: the risk is increased40 times more. Surgical correction doesnt

reduce the risk, however it is more likely to be

discovered early if the testis is in the scrotum2. Subfertility: unless corrected, all bilaterally

cryptorchid adult males become sterile.

3. Traumatic injury4. Torsion


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Predisposing factors:

1. Prematurity

2. Low birth weight,3. Small size for gestational age

4. Twinning

5. Maternal exposure to estrogen during the firsttrimester.


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Clinical features

The condition is unilateral in the right in 50% and

on the left in 30%. Arrested descent of both testes

occur in 20%.

secondary sexual characteristics are normal but

other abnormalities of the genitourinary tract may

be present including: hypospadias, patent

processus vaginalis


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Treatment

Hormonal vs. Surgical


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Hormonal

HCG or LH-releasing hormones are mainlyused.

HCG and LHRH have been used incombination, with initial success rates of 14-

65%.


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Surgical : Orchidopexy

Orchidopexy is the surgical correction of thecondition that is best performed by the age of2

years

Principles of the operative procedure consist of :

mobilizing the spermatic cord,

placing the testes in a subcutaneous serotal pouch

outside the dartos muscle.


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Retractile testis

Here the testes dont appear to be fully descended,and can be palpated in the scrotal neck and gently

manipulated into its correct position.

It is due to an overactive cremasteric muscle in

children under 3 years of age and the small testes.

It is a variant of normal

It requires no treatmentprovided that the testesbecome less retractile as the boy grows.


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2. Vesicoureteric Reflux

It is the retrograde flow of urine from the bladder into the ureter,

Primary reflux is VUR in an otherwise normally functioning lowerurinary tract (anomalies as ectopic U, double U,congenital megaU,ureterocele

secondary reflux is VUR that is associated with or caused by anobstructed or poorly functioning lower urinary tract. (neuropathicdys, bladder outlet obs, Iatrogenic etc )

In both conditions, the ureterovesical junction fails to function as aone-way valve, giving lower urinary tract bacteria access to the

normally sterile upper tracts. ratio of tunnel length to ureteral diameter must be at least 5:1


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Aims of treatment:

1. prevention of episodes of acute pyelonephritis2. to prevent the scarring of the kidney associated

with VUR (reflux nephropathy), which

increases the risk of HTN and renal failure.


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Clinical presentation:

1. Hydronephrosis, often prenatally identified using U/S.

2. Urinary tract infection (UTI),Children often present with

nonspecific signs and symptoms.- failure to thrive, with or without fever

- vomiting and diarrhea

- Anorexia

- lethargy.

Older children may report voiding symptoms orabdominal pain.

Pyelonephritis ; vague abdominal discomfort, fever and chills.


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Work up:

Laboratory studies

Urine analysis and urine cultures

serum chemistries to assess for baseline renal

function.

Imaging Studies

renal and bladder ultrasonography

voiding cystourethrography (VCUG).


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The International Classification System for vesicoureteral reflux

Grade I - Reflux into nondilated lower ureter

Grade II - Reflux into renal pelvis and calyces without

dilation

Grade III - Reflux with mild-to-moderate dilation and

minimal blunting of fornices

Grade IV - Reflux with moderate ureteral tortuosity and

dilation of pelvis and calyces

Grade V - Reflux with gross dilation of ureter, pelvis, and

calyces, loss of papillary impressions, and ureteral tortuosity


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A voiding

cystourethrogr

am (VCUG) of apatient with

grade III

vesicoureteral

reflux


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grade Vvesicoureteral

reflux (VUR)


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Management

When there is no ureteral dilation, there is an 85%chance of spontaneous resolution as the childgrows. In the meantime, the urinary tract must bekept free of infection, and this is done by:

Regular voiding High fluid intake

Avoiding constipation

Maintaining perineal hygiene

Anti-bacterial chemotherapy

Regular follow up, with charting of growth anddevelopment.


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In obstructive 2 reflux (as post. Urethral valve), release

of obstruction may cure reflux.In neuropathic reflux, intermittent catheterization for

control of infection may allow return of valvularcompetence.

Surgical correction, it is done by re-implanting theureter in the bladder wall so that a length of it lies deepto the bladder mucosa. This is indicated for:

Severe reflux with dilated ureters For other anatomical abnormalities

For children who fail to progress on conservativemanagement.


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3. Ureteral anomalies

Ureteral duplication is the most common renal

abnormality, occurring in approximately 1% of

the population and 10% of children who are

diagnosed with UTIs

Other abnormalities include, ectopic ureteral

orifice, ureterocele, and congenital obstructionof the ureter.


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A ureterocele is a cystic dilatation of the terminal

intravesical ureter.

Intravesical ureteroceles: entirely contained within

the bladder.

Ectopic uretrocele: if any portion is permanently

situated at the bladder neck or the urethra, regardless

of the position of the orifice.


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4. Bladder Anomalies


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Bladder agenesis is rare and incompatible with

life

Associated with hydroureteronephrosis and renal

dysplasia are present.


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Bladder duplication is rare.

Duplication can be completeorpartial, with

complete duplication more common than

incomplete duplication.

The 2 halves of the bladder are on either side of the

midline, with the corresponding ipsilateral ureter

draining each bladder half


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Urachal anomalies

A urachal cyst is a fluid-filled structure occurring in between

the two obliterated ends of the urachus, ie, the umbilicus and

bladder dome.

A urachal sinus : a persistently patent urachus. The sinus

drains to the umbilicus.

A patent urachus is a communication from the umbilicus to

the bladder. Infants present with continuous or intermittentdrainage from the umbilicus. The tract may become

inflamed, resulting in tenderness, periumbilical swelling, and

serosanguineous or purulent discharge.


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Extrophy ofthe bladder

It results from a complete ventral defect of the UG sinus

and the overlying inferior abdominal wall

musculature.


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Presentation:

- The lower central portion is devoid of skin and muscles.

- The anterior bladder wall is absent, the posterior wall iscontiguous with the surrounding skin.

- The rami of the pubic bone are widely separated, and

the open pelvic ring may affect gait.

- Urine drains onto the abdominal wall

- In males, the penis is shortened, and the urethra is

epispadiac

- The exposed bladder mucosa tends to be chronicallyinflamed.


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Treatment:

- Closure of the bladder in the newborn period

- Urethral closure and penile reconstruction

- Ureteral reimplantantion


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5. Penile and urethral anomalies

Hypospadias

Epispadias

Uretheral strictures Posterior uretheral valves


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Hypospadias

Results from failure of fusion of the uretheral folds on theundersurface of the genital tubercle.

Incidence is 1 in 400 male births

Hypospadias is classified according to the position of themeatus into:

1. Glandular hypospadias

2. Coronal.

3. Penile and penoscrotal.

4. Perineal. It's the most severe abnormality; the scrotum issplit and the urethra opens between it's two halves


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The remnant of urethraltissue distal to the meatus

is fibrotic, causing the

penis to bend downwards-

chordee. The more

proximal is the uretheral

meatus, the worse is the

chordee.


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The ventral part of theforeskin is absent giving

rise to a hooded

appearance.


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Treatment

Create a straight penis by repairing any chordee Create a urethra with its meatus at the tip of the

penis (urethroplasty)

Reform the glans into a more natural conical

configuration (glansplasty)

Achieve cosmetically acceptable penile skin

coverage

Create a normal-appearing scrotum.


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Timing of surgery:

child is aged 4-18 months, trending toward earlierintervention.

Late hypospadias repair in the pubertal and

postpubertal period is associated with

complications, primarily urethro cutaneousfistula, in nearly half of patients.


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Epispadias

The urethra opens on the dorsum of the penis,

with deficient corpus spongiosum and loosely

attached corpora cavernosa

The pubic bones are separated

Marked dorsiflexion of the penis

Associated with bladder extrophy, and if present

alone is considered as a mild degree of theextrophy complex.


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Treatment

Correction of penilecurvature

Reconstruction of the

urethra and bladderneck.


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Urethral Strictures

Most common in the fossa navicularis & in

bulbomembranous urethra

They are thin diaphragms that respond to simple

dilation or direct vision internal uretherotomy,

and rarely open surgery is required


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Posterior Urethral Valves

The most common obstructive urethral lesions in

newborn and infant males.

The most common cause of ESRF in boys

PUV are obstructive mucosal folds, seen only in males,which originate at the veru muntanum at the prostatic

urethra.

Clincal manifestations: difficult voiding, weak stream,

lower abdominal mass, palpable kidneys, incontinence,and UTI.

Up to 70% have VUR


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Lab findings

BUN and creatinine

Evidence of UTI

U/S shows evidence of bladder thickening and

trabeculation, hydroureter, and hydronephrosis.

Voiding CUG demonstrating the urethral valves

establishes the diagnosis.

Treatment

Endoscopic destruction of the valves as soon aspossible.

pediatric urology h

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