patience wildenfels, md - amazon web services · 2019-07-20 · frequent voiding with low pressure...

Patience Wildenfels, MD

Division of Pediatric Urology

Ochsner Health System

[email protected]

July 20, 2019

mailto:[email protected]

GUIDELINE STATEMENTS

Evaluation and Treatment of Cryptorchidism

(AUA Guidelines published 2014)

Guideline Key Points▪ Cryptorchidism or undescended testis (UDT) is one of the most common

pediatric disorders

▪ 1% - 4% of full-term and 1% -45% of preterm newborn

▪ The main reasons for treatment of cryptorchidism

▪ Increased risks of impairment of fertility potential

▪ Testicular malignancy

▪ Testicular torsion

▪ +/- associated inguinal hernia

▪ Successful scrotal relocation of the testis, however, may reduce but

does not prevent these potential long-term sequelae in susceptible

individuals.

Guideline Key Points

▪ Cryptorchidism, or undescended testis (UDT), is defined as failure of a testis

to descend into a scrotal position.

▪ Normal scrotal position has been defined as positioning of the midpoint of

the testis at or below the midscrotum (Dependent portion of scrotum)

Guideline Key Points

▪ Although “high scrotal testes” are not routinely considered

undescended by most clinicians, they are included in the definition of

UDT in some epidemiologic studies.

▪ Because…. Some of these are ok & stable- however some are not!

▪ Stable descended testes that reside above the scrotal midpoint

▪ Retractile

▪ Undescended “gliding” testes , which are Not stable

GUIDELINE STATEMENTS

▪ Guideline Statement 1

▪ Providers should obtain gestational history at initial evaluation of

boys with suspected cryptorchidism.

▪ (Standard; Evidence Strength: Grade B)

▪ Guideline Statement 2

▪ Primary care providers should palpate testes for quality and position

at each recommended well-child visit.


Guideline Statement 3

▪ Providers should refer infants with a history of

cryptorchidism (detected at birth) who do not have

spontaneous testicular descent by six months (corrected

for gestational age) to an appropriate surgical specialist

for timely evaluation.



▪ Providers should refer boys with the possibility of newly

diagnosed (Acquired) cryptorchidism after six months

(corrected for gestational age) to an appropriate surgical

specialist.



▪ Providers must immediately consult an appropriate specialist for

all phenotypic male newborns with bilateral, nonpalpable testes

for evaluation of a possible disorder of sex development (DSD).

▪ (Standard; Evidence Strength: Grade A)


▪ In boys with bilateral, nonpalpable testes who do not have

congenital adrenal hyperplasia (CAH),

▪ providers should measure Müllerian Inhibiting Substance (MIS)

or Anti- Müllerian Hormone [AMH] and consider additional

hormone testing to evaluate for anorchia.

▪ (Option; Evidence Strength: Grade C)


▪ Providers should assess the possibility of a Disorder of Sex

Development (DSD) when there is increasing severity of

hypospadias with cryptorchidism.

▪ (Recommendation; Evidence Strength: Grade C)


▪ Providers should not perform ultrasound (US) or other

imaging modalities in the evaluation of boys with

cryptorchidism prior to referral, as these studies rarely

assist in decision making.



▪ In boys with retractile testes, providers should assess the

position of the testes at least annually to monitor for

secondary ascent.


RETRACTILE TESTES

▪ - initially extrascrotal on examination or moves easily out

of scrotal position, but can be manually replaced in

stable, dependent scrotal position and remain there

without tension at least temporarily.

▪ often associated with a vigorous cremasteric reflex

GUIDELINE STATEMENT 10

▪ Providers should not use hormonal therapy to induce

testicular descent as evidence shows low response rates

and lack of evidence for long-term efficacy.


Guideline Statement 11- Surgery

▪ In the absence of spontaneous testicular descent by six

months (corrected for gestational age), specialists should

perform surgery within the next year.



▪ Providers should counsel boys with a history of

cryptorchidism and/or monorchidism and their parents

regarding potential long-term risks and provide education

on infertility and cancer risk.

▪ (Clinical Principle)

Posterior Urethral Valves

Still here and living longer


Anatomy 101

“Bladder Neck”

“Bladder Outlet”

“Verumontanum”

(seminal colliculus)

“Perineal Membrane”

What is the posterior urethra anyway?

▪ Urethra

▪ two broad segments: the anterior urethra and

posterior urethra

▪ posterior urethra

begins distal to the

bladder neck & runs

to the perineal

membrane

▪ anterior urethra

begins at the

perineal membrane

& continues distally

to urethral meatus

PUV is a

Congenital

Obstruction of

Bladder Outlet

(within the posterior

urethra)

ANTEGRADE problem!


Is most common cause of bladder outlet obstruction in

infants: presentation is often antenatal, and

pathognomonic findings on post natal images typically

confirm diagnosis

Few problems like this in a newborn male: ironic actually

Its easy to detect, easy for initial treatment with valve

ablation but not easy! life long problems

PUV- EPIDEMIOLOGY▪ Congenital Anomalies of the Urinary Tract affect of 1 in 500 pregnancies and

obstructive type issues are the most common (UPJ, PUV, etc)

▪ The incidence (risk of having PUV) is between 1.6 and 2.1 per 10,000 births.

▪ The prevalence (proportion of PUV in the population at any given time) is

2.10 per 10,000 births.

▪ Consider this …

▪ In USA alone - expect 300 to 500 new cases of infants with PUV born

annually—of which 1/3 will go on to End-Stage Renal Failure(ESRF)

▪ COST of THIS IS GINORMOUS!

▪ Abortions, still births, access to care…

Classification of PUV types

▪ 1st proposed in 1919

▪ 3 Types

▪ TYPE 1- most common 90-95% cases

▪ “LEAFLETS” that out pouch with voiding and obstruct

▪ Type 2- Unicorns

▪ Type 3- 5-10 % cases

▪ Annular ring with small hole in center

TYPE 1 PUV- 90-95% casesTYPE 1 variant “leaflets” that arise from the verumontanum and fuse in

the midline just proximal to the external urethral sphincter.

TYPE 2 – Unicorns

TYPE 3

concentric ring with central opening (5-10%)

Antegrade voiding

DIAGNOSTIC TOOLS

Fetal MRI

Ultrasound

VCUG

LABS

Renal Scan

Bilateral severe

pelvicaliectasis & bladder

Keyhole sign, thickened

bladder

Fetal MRI

Being used more mostly at major centers- utility is limited

High Alert

▪ Do NOT let this baby go home without

workup!

▪ Needs renal u/s and when stable VCUG

BEFORE discharge

Pathognomonic VCUG

Voiding phase will show contrast

traveling across a hypertrophied,

elevated bladder neck and grossly

dilated posterior urethra

The urethra funnels abruptly at a

transverse membrane, or “leaflets”

Clinical Presentation

▪ Neonatal

▪ Early Infancy

▪ Dribbling / poor urinary stream

▪ Urosepsis

▪ Dehydration

▪ Electrolyte abnormalities

▪ Uremia

▪ Failure to thrive; (renal insufficiency)

Clinical presentation

▪ Toddlers

▪ Better renal function (less obstruction)

▪ Febrile UTI

▪ Voiding dysfunction – incontinence

▪ Daytime incontinence may be the only symptom in boys

with less severe obstruction

Pathophysiology

Why are PUV bad?

Clinical Comorbidities

PULMONARY HYPOPLASIA

Most profound complication & Most common cause of perinatal mortality in infants affected

by severe LUTO

Lifelong potential for issues

Pulmonary abnormalities start BEFORE oligohydramnios

Pathophysiology

▪ Despite early cystoscopy and valve ablation

there are a lifetime of consequences from

the in utero obstruction

▪ Bladder dysfunction

▪ Renal dysplasia

▪ Polyuria

▪ Worsening bladder dysfunction….

▪ ESRD/Transplant

Bladder Problems in PUV

Major Importance:

The potential comorbidities arising from PUV—

renal dysfunction, urine reflux, worsening

hydronephrosis—are due to bladder

dysfunction

Compliance- bladder has to be able to store

urine at low pressure and has to be able to

empty urine sufficiently

Bladder dysfunction- “Valve Bladder”

Pathophysiologic cascade of changes due to early

exposure to obstruction, cascade continues…

Voiding dysfunction

Urinary reflux

Worsening of renal dysplasia

All conspire to manifest as the dysfunction of :

VALVE BLADDER SYNDROME

Valve Bladder Syndrome

Throughout childhood the bladder progresses through 3

distinct contractility cycles

1. Detrusor hyperreflexia in infancy-early childhood (over active

bladder)

2. Decreasing intravesical pressures and improved compliance of

bladder in childhood

3. Increased bladder capacity with hypocontractility and atony in

adulthood (maybe adolescence) (BIG FLOPPY BAD BLADDERS)

Valve Bladder Dysfunction/Syndrome

▪ Must always suspect valve bladder even when not

suspected by history alone!

▪ Looking for INCONTINENCE- VOIDING PROBLEMS-FREQUENCY….

▪ Reliance on clinical examination or patient questionnaires alone may grossly

underestimate bladder dysfunction

Vesicoureteral Reflux and Dysplasia

(VURD)

▪ VURD

▪ The hypothesis: reflux served as a “pop-off” mechanism in which the

dysplastic kidney with reflux served as a pressure reservoir mitigating

damage to the contralateral kidney, and coined the term

vesicoureteral reflux and dysplasia

▪ This appears to be a short-term protective mechanism

▪ Cuckow et al found that, whereas 67% of patients affected by VURD

during year 2 of life had a normal serum creatinine, only 30% of these

children had normal values between ages 8 and 10 years

Valve Bladder- Follow up

▪ Obtaining a uroflow and checking postvoid residuals should be a routine part

of follow-up in toilet-trained children with a history of posterior urethral

valves

▪ Upper tract assessment with renal ultrasonography

▪ Renal function panel (GFR critical to follow)

We are a team: observation, clinical history,

imaging, labs, and urodynamics

Valve Bladder Dysfunction/Syndrome

POTTY TRAINING

▪ Counsel Parents: Do Not aggressively push an affected child toward toilet training & expect a lag compared to the normal population.

▪ Daytime incontinence is not uncommon, ranging from 7% to 35%

▪ Nocturnal enuresis is expected in 1 of 4 children with a hx of PUV

▪ Once toilet training is achieved, children and caregivers are educated to ensure adequate fluid intake, to void on a timed regimen, and to practice double voiding.

▪ Biofeedback therapy and home pelvic floor exercises have also been shown to be useful

PUV Bladder- medical management

▪ Plan should be from Pediatric Urologist

▪ Frequent voiding with low pressure

▪ Timed and double voids

▪ (extended release preferred but is pill)

▪ Alpha-blocker (pills)

▪ Monitor Post Void Residuals!!

▪ Uroflow/urodynamic testing

▪ CIC- clean intermittent catheterization- for bladder myogenic failure

▪ Overnight Bladder Catheter for at risk patients with renal dysfunction

Catheterization

▪ CIC

▪ The utilization of CIC can be challenging in PUV patients due to the sensate urethra

and bladder neck hypertrophy

▪ IF You Don’t Need, and do it- no change

▪ BUT if You NEED it, and Don’t do it, progression of renal failure

Medical Management

just to stress…

❖ Given the increased risk of ESRD, critical to follow glomerular filtration rate

(GFR)

❖ Adolescents need follow up too! - metabolic demand of the associated

growth spurt and decreased renal reserve

❖ Delayed myogenic/detrusor failure due to “valve bladder cycle” should

always be investigated when urologic testing reveals increased PVR or

worsening upper tract dilation.

Prognostic Indicators for Renal Function

▪ Lifetime prevalence of end-stage renal disease in boys

with posterior urethral valves is between 20% and 50%

▪ Age at diagnosis

▪ Renal dysplasia with or without vesicoureteral reflux

▪ Nadir creatinine during 1 year of life,

▪ Recurrent urinary tract infections,

▪ Bladder dysfunction.

Nadir Creatinine

▪ A serum creatinine of less than 0.8 mg/dL appears to

indicate a minimal risk, whereas a value greater than

1.2 mg/dL at 1 year of age predicts a higher risk of

developing end-stage renal failure

▪ The nadir creatinine value measured at 1 year of life appears to be more

accurate as a predictive tool than the value obtained at 1 month of age

▪ In those studies suggesting that a 1-month post-treatment serum creatinine is

a more accurate predictor of renal function, again the value of less than

0.8 mg/dL at 1 month after treatment seems to indicate better long-term

outcomes

Risk

▪ In McLeod(2019) series by 10 years of follow-up

▪ a nadir serum creatinine <0.4 mg/dL was associated with 0% risk of

RRT whereas 100% of patients with a nadir serum creatinine >1.0

mg/dL experienced outcome of requiring Renal Replacement

Therapy

▪ Additional ranges showed intermediate risk illustrating the variable

renal outcomes of the PUV patient and need for extended follow up

Transplant

▪ The prevalence of end-stage renal disease in boys with a history of

posterior urethral valves is up to 50%

▪ At any given time- 50% of the boys with PUV have ESRD!

▪ 2006 annual report of the North American Pediatric Renal Trials and

Collaborative Studies listed obstructive uropathy as the second most

common cause for transplantation, accounting for 1424 of 8990

transplantation cases (15.8%) since 1987

Transplant

▪ Transplant recipients in whom bladder dysfunction is incompletely managed

or the bladder reservoir is not optimized have significantly higher

complication rates and graft loss rates

Transplant

▪ Pediatric Urologist should be critical part of team

▪ Video urodynamics

▪ Determine safe bladder pressures

▪ Contractile function

▪ Determine proper bladder management skills

▪ CIC

▪ Overnight catheter drainage

▪ Good skills critical for graft survival

MOST IMPORTANTLY…

We are nice people!

We Want to Help You!

We are a phone call or text or email or

anything away 24/7!

PLEASE CALL ANYTIME

Patience Wildenfels, MD

504-343-0380 cell

[email protected]

(NOLA, BR, Covington)

Frank Cerniglia, MD

504-417-1540 cell

[email protected]

(NOLA, Slidell)

PEDI URO Office/Clinic- DIRECT

504-703-0809

504-703-0808

504-842-4083- main urology



patience wildenfels, md - amazon web services · 2019-07-20 · frequent voiding with low pressure...

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