Pediatric Urology Update

Rama Jayanthi, M.D.Section of Pediatric Urology

Columbus Children’s Hospital

Format and purpose

Selected cases in pediatric urology Stimulate discussion Discuss management

Case 1

Hypospadias noted at birthBoth testes normally descended

Questions:What type of work up?What is initial management?When do you refer to pediatric

urologist?

Hypospadias

abnormally positioned meatus meatus can be located anywhere

from perineum to glans chordee- associated penile

curvature

Hypospadias- associated abnormalities

Easy to remember - nothing! Normal kidneys and bladder Normal fertility Normal sexual function

Hypospadias - management for the

pediatrician Do not circumcise! No need for any imaging studies Refer to pediatric urologist within

first months of life Always consider intersex if

hypospadias associated with undescended testis

Who is a boy and who is a girl?

Is it a hypo or not?

Retract foreskin completely off glans during circ

If glans meets in midline proximal to meatus, not a hypo!• Even if meatus appears to be large

If a true hypo is present• Wrap with Vaseline if not bleeding• Otherwise close skin edges with chromic

sutures

Hypospadias - management for the pediatric urologist

Surgical correction at 6 - 9 months of age

Attempt one stage reconstruction Out patient surgery Success rates should be > 95%

Epispadias

Very rare - more often associated with bladder exstrophy

Need early referral for parental counseling

Patients may be totally incontinent

Case 2: Scrotal mass

Painless scrotal massesStable in sizeNo increase with cryingNo inguinal bulge

Questions:What is the diagnosis?What should be done?

Scrotal masses

Solid vs. cystic• transillumination of light

Testicular vs. extratesticular Painful vs. painless

Hernia/hydrocele - cystic scrotal mass

Testes develop intraabdominally and exit the abdomen at the internal ring

All males have a fascial defect at some point during gestation

Persistence of defect leads to communicating hydroceles and hernias

Hernia/hydrocele

What is the difference between a hernia and a communicating hydrocele?• Both are the same anatomic defect• If opening only large enough to admit

peritoneal fluid - communicating hydrocele• Scrotal swelling only, “comes and goes”

• If opening large enough to admit bowel- clinical hernia• “inguinal bulge”

Hernia/hydrocele

Hernia/hydrocele - management

Observation:• Noncommunicating hydrocele < 12 - 18

months of age• Hernia - very premature infants with easily

reducible large hernias Surgery:

• Hydrocele - persistent, enlarging, painful• Hernia - always

Surgical correction involves ligation of peritoneal sac

What is the diagnosis?

Findings:

Painless right scrotal massDoes not transilluminateUltrasound: solid mass

Diagnosis: yolk sac tumor

Case 3

A 15 year old boy is noted to have a left scrotal mass during a sports physical.

The mass is soft, painless, located above the testis and disappears when the boy is recumbent

What is the most likely diagnosis?

Varicocele

Represents dilation of left spermatic veins

Etiology unknown• ? Lack of venous valves• ? High intravenous pressure

Incidence: 15% of all teenage males • rare in prepubertal males

Significance of varicoceles

Infertility• Most common surgically correctable cause

of male factor infertility• Reason unclear

• ? Increased temperature of scrotum• ? Primary endocrinopathy

Pain• Uncommon in teenagers• “Dull ache”

Management of pediatric varicocele

“Clinically significant varicoceles” requires surgical ligation

Problem:• Most teenagers have varicoceles detected

on routine physical examination• Usually asymptomatic

Management of adolescent varicoceles

• Yearly measurement of testicular size• Symmetric testes - observe• Indications for intervention:

• Development of size discrepancy > 2cc• Pain

• Personal opinion:• Spermatic vein embolization may be the

simplest and least invasive option

Case 4

A 4 month old boy on routine examination is found to have a normally descended right testis but no palpable left testis. His exam is otherwise normal.

What workup is needed? When should he be referred?

What to do with a missing testis?

Issues:• palpable or nonpalpable?• Unilateral or bilateral?• Associated hypospadias?• Associated syndromes?

• Most will have isolated unilateral undescended testis

Should an ultrasound be performed?

If an US reveals a testis, then surgery is required for orchidopexy

If an US shows no testis it may be inaccurate because the child may have a small intraabdominal testis that was not detected

Regardless of US findings, the child needs exploration

Thus, there is no need for radiological evaluation for a nonpalpable testis

Classification of UDT

Intraabdominal• testis located above internal ring• usually nonpalpable

Canalicular- “routine” undescended testis Retractile - not a UDT

• due to hyperactive cremaster reflex• only in prepubertal males• no hormonal/testis defects

Management of UDT

Observation until 6 -12 months of age If still undescended, surgical

correction No advantage to further observation

after 12 months of age• testis will not descend• germ cell fibrosis evident by three years

of life

Bilateral nonpalpable testes

Karyotyping essential Main question: Is there functional

testicular tissue present? No functional tissue present if

• marked elevation baseline FSH and LH

• no rise in serum testosterone with HCG stim

Fertility after cryptorchidism

Formerly unilateral

UDT

Formerly bilateral

UDT

Control

Number 313 50 336

Married 244 (78%)

38 (76%)

269 (80%)

Married with

children

183 (75%)

20 (53%)

203 (76%)

Lee, Brit J Urol, 1995

Risk of Neoplasia

UDT has 10X greater risk• Abdominal testis has 4X greater risk than

inguinal Tumors occur after puberty

• Mean age 25 - 30 years 25% occur in normally descended testis Early orchidopexy may be protective Seminoma most common, embryonal cell

2nd

Case 5

A nine year-old uncircumcised boy presents with a tightly phimotic foreskin.

He has had a few episodes of balanitis

His parents to do not want him to be circumcised if possible

What can be done?

Natural history of phimosis

Medical management of phimosis

Prospective trial Diprolone cream (0.05%) applied

TID for 4 weeks to preputial band Patients reevaluated at one month

Medical management of phimosis

Results• n = 21• Signs and symptoms

• UTI• Balanitis• Preputial ballooning• Asymptomatic

Medical management of phimosis

Success 17/21 (81%)• 11 complete, 7 partial

Failure 4/21 (19%)

What does a bladder do??

Store urine Empty urine In a 24 hour time period

• Bladder is in storage mode for 23 hours and 45 minutes

Thus, storage function is of greater importance than emptying

Normal bladder function

Storage• Storage must take place at low

pressures• Intravesical pressures must be low

enough to…• Not impede urine transport from kidneys via

the ureters• Hydronephrosis/renal injury

• Not overwhelm sphincteric resistance• Urinary incontinence

Emptying function

First step in voiding is relaxation of sphincteric mechanism followed by bladder contraction

Normal voiding is a “passive” process with no involvement of the abdominal muscles

Case 6

A 7-year-old girl complains of new onset daytime wetting. She has always been a bed wetter. She has never had any urinary tract infections. She does note that she often will leak while running and exerting herself. She furthermore does not realize that she has to go prompting her parents to wonder whether the child can even tell that she needs “to go”. Sometimes the family will see her doing the “pee-pee dance” and sometimes they will see her suddenly squat on her heel. Occasionally she will have a precipitous urge to void but when she makes it the bathroom nothing comes out. Her leakage can vary from damp spots on the underpants to complete soaking of her clothes. When the family is out they will often have to stop to find a restroom for her prompting the family to wonder whether her bladder is “too small”. She occasionally will complain of mild nonspecific abdominal pain.

What kind of evaluation is required?

Aspects of the history

Daytime wetting vs. nighttime wetting vs. both Urgency? Frequency? Infrequent voiding? Damp pants vs. soaking? Does leakage occur prior to going to restroom

or after voiding ? Does the child care if he/she is wet? Frequency of bowel movements?

Common myths

“small bladder” that the child has to grow into

“narrow urethra” that needs to be stretched

“inability to sense fullness” Urgency and/or frequency in a male

may be due to meatal or urethral stenosis

Voiding dysfunction may be due to

Evaluation of voiding dysfunctions

History most important Screening renal ultrasound

• Ensure normal kidneys• Alleviates parental anxiety

• Bladder wall thickness• Subtle sign of bladder overactivity

• Post-void residual• ? Incomplete sphincter relaxation

Voiding cystourethrography??

A child should almost never have a catheter inserted in the initial evaluation of pure incontinence!!!

“Functional bladder capacity” better evaluated by voiding diary• Expected bladder capacity: Age + 2 in ounces

VCUG rarely needed• history of significant UTI• symptoms of obstruction in males

Varieties of voiding dysfunction

In order of frequency•Bladder instability/overactivity• Infrequent voiding• Incomplete emptying•Hinman’s syndrome

•“Nonneurogenic neurogenic bladders”

Bladder instabilty

Clinical manifestations•wetting•infections•pelvic/vaginal pain•penile/scrotal pain

Forms of bladder instabilty

Urgency incontinence syndrome•predominant symptom is wetting•infections less likely

Hypertonic bladder • predominant symptom is UTI• may also have associated wetting

Urgency incontinence

More common than hypertonic bladder

Usually associated urgency/frequency

Severity of wettings ranges from damp pants to soaking

Hypertonic bladder

VCUG - trabeculated bladder, may have diverticulae

Main point: Infections (and reflux) are secondary problem

“Distal urethral stenosis”

Spinning top urethra

NOT due to obstruction

A sign of bladder instability

Urethral dilation is NEVER indicated!!!

Management of bladder instability

Anticholinergics Bowel management Consider prophylactic antibiotics

only if has recurrent infections refractory to standard management• The older I get, the less I use

prophylactic antibiotics

Choice of anticholinergics

Oxybutinin• Ditropan XL 5 -15 mg qAM

• Advantages:• once a day dosage• fewer side effects

• Elixir (0.2 mg/dose/BID -TID)• only if cannot swallow pills

Role of bowel dysfunction

Fecal retention• Incomplete or

infrequent emptying of bowels

• Subtle clues• abdominal pain• perineal pain• vaginal “itching”• penile pain

Relationship of constipation and wetting

234 constipated/encopretics 29% day and 34% night wetting

pre-treatment, UTI in 11% 52% had improvement in

constipation• 89% improved day• 63% improved night• no more UTILoening-Baucke, Pediatrics, 1997

Importance of UTIs and bowel/bladder disturbances

143 children with reflux + breakthrough UTI

• 77% had dysfunction - breakthrough UTI

• 16% had dysfunction

Koff, J Urol, 1998

Infrequent voiding syndromes

“lazy bladder syndrome”• an inappropriate term that incorrectly

labels a child as being lazy Fact of life for children:

• Children usually have more important things to do than urinate and defecate

Sensation normal - children “tune out” the bladder

Management of infrequent voiding syndromes

timed voiding behavioral modification

• controlled bribery intermittent catheterization

The overwhelming majority of patients can be evaluated with only a careful history. Only a small number may need “objective” measurements of bladder function.

Case 7

A 8 year old girl has her first episode of UTI

How do you evaluate her?• Observation? • US? • VCUG?• DMSA scan?

What is a urinary tract infection?

Positive culture in a child with appropriate symptoms

What is not an infection, and thus should not receive antibiotics

Red introitus Perineal discomfort Dysuria in the absence of a positive

culture• A positive urinalysis is not sufficient

to definitively diagnose an infection Microscopic hematuria

Philosophical questions

Why do we treat urinary tract infections?

What are the ramifications of UTI’s?

Renal scarring

may cause hypertension if present diffusely and

bilaterally, may lead to renal failure

most likely will occur after pyelonephritic episodes in children less than 4 years of age

Therefore

if older child has episode of cystitis, recommend US

if older child has pyelonephritic episode, recommend VCUG/US

if younger child has any type of UTI, recommend complete workup, especially if male

Case 8

Four year old girl with recurrent UTI, some with fever

US - normal, VCUG - normal Repeat nuclear cystogram also

normal What do you do???

Non-reflux pyelonephritis

The majority of children with febrile pyelonephritis do not have reflux or any other urinary tract abnormalities

What causes urinary tract infections in the absence of anatomic abnormalities?

Non-anatomic causes of UTI

“sticky bacteria” dysfunctional bladder habits dysfunctional bowel habits all the above

Role of VCUG in children with UTI

A VCUG is necessary to diagnose reflux

Treatment of reflux is helpful to prevent pyelonephritis and renal scarring

Thus a VCUG is not necessarily needed in a child with normal kidneys and lower urinary tract infections

Case 9

A 15 year old girl notes that she leaks only when she laughs. She is a cheerleader and never wets during her routines. She is also is a star soccer player and never wets during her games.

Case 9 (cont’d)

What is the diagnosis?• “Giggle incontinence”• Part of the cataplexy/narcoplexy

complex• Treatment consists of behavioral

modifications• Consider Ritalin for nonresponders

Case 10

8 year old male who presented with urinary tract infections• Fever and flank

pain

Case 10 (cont’d)

On further questioning….• Previously was dry but now

has day and night wetting• Significant daytime

urgency and occasional back pain

• Rarely has good stream• Parents have noted that

the child also “walks funny.”

Case 10 (cont’d)

Main diagnostic consideration: occult tethered spinal cord

Relatively uncommon Importance in early detection in

that delay in diagnosis may lead to permanent neurological deficit

Case 11

4 year old girl who is always wet. She has no urgency, voids regularly, and has failed treatment with empiric anticholinergics.

Key is the history of being “always wet” Consider ectopic ureter.

• Ureter does not insert into bladder. Inserts into urethra or vagina

• Surgery is curative• Key is to consider the diagnosis

• Intravenous pyelography has very poor sensitivity.

Imaging for ectopic ureter

Imaging for ectopic ureter

Case 12

5 year old boy who suddenly developed severe daytime frequency. He doesn’t have any associated wetting, has had no infections, will occasional wake up at night to void.

He literally will void every 10 minutes and each time he voids a small amount of urine will pass

Renal ultrasound is normal and anticholinergics have not helped

What is the diagnosis?

Case 12

“Daytime Frequency Syndrome”•Unknown etiology•Spontaneous improvement is the

rule

Thank you for listening