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Pediatric UrologyLuis Guerra, MD

University of Ottawa

Children’s Hospital of Eastern Ontario

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Evaluation

Historically Urinalysis, IVP and cystoscopy

Recently US, CT, MRI, and endourology

Basic and most important approach starts with Complete history Thorough physical examination

Attention to genitourinary system

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History Extremely important Always address the child Main complaint, past medical and family history

Antenatal history Prenatal US (hydro, bladder distention, oligohydramnios)

Delivery Uncomplicated, voided in the first 24 hrs, any metabolic unbalance

Urologic Age toilet trained Enuresis or daytime incontinence UTI Intestinal constipation Dysfunctional voiding pattern

Previous surgery Traumas

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Physical General aspect

Syndromes, social and psycho-emotional behavior Abdomen

Palpation (bladder, kidneys, masses) Lower back

Skin dimples, hairy tufts, discolorations spots, gluteal cleft External genitals

Girls Vulva- Inflammations, labial adhesions, wetness

Boys Penis- Physiologic phimosis, hypospadias, chordee Testicles- Descended, size, texture, scrotal mass

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Urinary infection in children

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Urinary infection in children

What is UTI Bacterial infection in the urine May involve bladder and/or kidneys

Incidence: UTIs more common

First year of life Boys (peak at 6 months) >1 yr of life Girls (peak at 2-3 years)

During school age 1.2% of boys and 5% of girls develop UTI

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Most common etiology

Organisms from bowel flora E. Coli (most common) Less often

Klebsiella, Proteus, Enterococcus, Staphylo saprophyticus

Ascending route is the MC

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Pathogenesis

3% of girls and 1% of boys will get a prepubertal UTI (Winberg et al, 1974)

Of these, 17% will get infection-related renal scarring

Of those with scarring, 10% to 20% will become hypertensive, and a rare child will progress to renal failure

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UTI – Clinical presentation

Kidney (pyelonephritis) Fever, abdominal or flank pain, vomit, diarrhea, child is ill

Bladder (cystitis) Dysuria, sp pain, frequency, urgency, incontinence and foul urine

Infants May be vague and without localization Fever 66%, irritability 55%, poor feeding 40%,

vomiting 35%, diarrhea 31%, abdominal distension 10%

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UTI - Diagnosis

History – Symptoms

Physical There are no signs specific for UTI in the infant

An abdominal mass may be palpated (hydro, bladder,etc) Abdominal, flank, sp, CVA tenderness in older child Active, febrile, dehydrated, septic shock

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Methods of urine collection

Bag specimen Even after skin cleaning usually reflects perineal and rectal flora It is reliable if: Significant pyuria, grow only 1 bacteria and

child is symptomatic May be useful to “rule out bacteriuria”

Suprapubic aspiration (most reliable but less used) Catheterization (if bag urine is not definitive) Toilet trained children: Midstream void Diagnosis of UTI

At least 10.000 and preferably >100.000 colony-forming units/ml

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Reliability of urinalysis In a properly collected and processed urinary specimen, the

combination of: Positive leukocyte esterase, Positive nitrite testing, and Microscopic confirmation of bacteria

Has almost 100% sensitivity for detection of UTI

When all (or the leukocyte esterase and nitrite tests) are negative: The negative predictive value approaches 100%( Wiggelinkhuizen et al, 1988; Lohr et al, 1993)

Combination of these 3 tests helps, however the urinalysis cannot replace urinary culture

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UTI - Radiological evaluation

Recommended in all children < 5 yrs of age All boys, irrespective of age All girls with pyelonephritis (fever) After a 2nd UTI in a girls older than 5 yrs of age

(Palmer, JS and Elder, JS 2003)

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UTI - What kind of test?

Ultrasound Upper tract abnormalities (hydro, atrophic kidneys, cysts, renal

scarring, etc)

VCUG (voiding cystourethrogram) VUR and posterior urethral valve, assess bladder configuration

If both test are normal, no other evaluation is necessary

Renal scans For assess obstruction and renal function (DMSA, DTPA)

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Vesicoureteric reflux

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Vesicoureteric reflux

Wash back of urine to the kidneys (passive, active) Prevalence as high as 1 in 100 births 1:6 Male to female; present in 35-50% of children with UTI Boys are more likely to have high grades (IV and V) Causes

Primary (congenital short intramural tunnel) Normal tunnel length and ureteral orifice is 2.5:1

Secondary (high pressure – PUV, neurogenic bladder,etc) Importance of diagnosis of VUR

VUR + UTI can cause renal damage (scarring)

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International classification VUR

International classification of vesicoureteral reflux. Copyright © 2003, Elsevier Science (USA)

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VUR - Treatment

Antibiotic prophylaxis and follow up Follow up

Annual US and cystogram Spontaneous cure rate

20% per year Depends on the grade of VUR, unilateral vs bilateral

Surgery (open or endoscopic) in selected cases Breakthrough UTI (first year of life most likely to get scar) New onset or progressing of renal scarring Decreasing renal function Long follow up, parental preference

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Treatment of an UTI: 10-14 days

Depends on age and severity of UTI

Septic (pyelonephritis) Neonates or young infants

Admission IV antibiotic (ampi or cephalosporin + genta) and then according to

bacteria sensitivity (change to oral when discharged) Older children oral therapy may suffice (same as for cystitis)

Clinically well (cystitis) Oral antibiotics

(sulfa-trimethoprim, nitrofurantoin, amoxicillin, ampicillin, cephalosporin) Adequate oral intake for hydration

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Should not be used < 2 mo of age

Sulfa derivatives (Sulfamethoxasole) Displaces protein-bound bilirubin and may interfere with

bilirubin excretion, exacerbating neonatal physiologic jaundice

Nitrofurantoin May cause hemolytic anemia because of glutathione

instability in the erythorocyte

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Management following a UTI

Adequate fluid intake Avoid intestinal constipation (fiber in the diet) Girls

Proper hygiene (swipe front to back) Void spreading the legs (avoid vaginal voiding and

incomplete voiding) Timed voiding (every 2 to 3 hours)

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Who should have antibiotic prophylaxis? Patients with abnormalities of the GU tract

All VUR < 5 years of age Some risk factor for UTI (GU obstruction or urinary stasis)

Select cases of PUV, megaureters, hydronephrosis, etc

Patients with normal work-up More than 2 or 3 UTIs in 1 year

Usually sulfamethoxasole-trimethoprim, nitrofurantoin or trimethoprim alone

Little effect on stool’s bacterial flora Dose is 1/3 or 1/2 of the treatment dose

Routine C+S should not be done Amoxicillin or cephalosporine can affect the intestinal flora

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UTI.., Who I should refer?

All daytime incontinence Diagnosed or suspected of GU abnormalities

VUR, hydronephrosis, megaureters, etc Neurogenic features

Abnormal lower back exam Established neurogenic entities

Sacral agenesis, imperforate anus, tethered cord, etc Known syndromes (VACTERL, 21 trisomy)

Child with recurrent UTIs after proper management of Intestinal constipation Adequate fluid ingestion Regular voiding (every 2-3 hours)

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Scrotal mass in children

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Scrotal mass in children Causal conditions

Inguino-scrotal hernia

True scrotal swelling Cystic/Soft

Epididymal cyst (spermatocele) Hydrocele Varicocele (MC on left side)

Solid Benign or malignant tumors (testis, spermatic cord and adnexas ) Testicular torsion / appendix torsion Incarcerated/ strangulated hernias

Trauma (hematocele, hematoma) Infectious (epididymitis)

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Scrotal mass in children Acute

Testicular torsion Testicular/epidydimis appendix torsion Orchitis-epidydimitis Incarcerated / strangulated hernias Trauma Insect bits

Non-acute Hydrocele Hernia Testicular tumor Varicocele Cysts (epididymis, testis, spermatic cord)

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Inguinal hernias in children

Persistence of the peritoneal vaginalis conduct

and insertion of abdominal contents into the inguinal canal

Presentation may be Non-acute (inguinal lump, bulging) Incarcerated or strangulated (pain, vomiting, tenderness)

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Inguinal hernias in children

Treatment Elective surgery to prevent strangulation Urgent exploration if strangulated Dissection of the hernia sac up to internal ring and closure Repair of the posterior wall is generally not necessary in

children but adolescents may need it (or mesh)

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Hydrocele - characteristics

Is an accumulation of fluid within the tunica vaginalis Usually communicating in children

All hydroceles in infants and children result from persistence of or delayed closure of the processus vaginalis

Present in 1-3% of all children

Greater in premature children

Males (85%)

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Hydrocele - Anatomy

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US - Hydrocele

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Hydrocele - diagnosis Adequate history and physical examination are the hallmarks

of diagnosis

Assess the presence of Characteristic of scrotal swelling (soft or tense) Positive transillumination Inguinal bulge or incarceration (hernia) Important to feel a normal testis (US if necessary)

More frequent on the right and 10% is bilateral

Ultrasound is not usually necessary

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Hydrocele - Management

Scrotal hydroceles common in newborn Usually resolve first year of life No evidence of testicular injury (even if tense)

Older child Secondary to minor trauma or inflammation

Observation is reasonable

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Surgical management

Hernias Repaired when recognized Emergently if it is strangulated

Hydroceles Usually repaired after 18 months of age, electively

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Acute scrotum

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Testicular torsion

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Testicular torsion (TT)

Most common in Neonates (extravaginal)

Peripubertal (intravaginal)

16-42% of boys with acute scrotum have TT

Extremely challenging condition One of the true Urologic Emergencies

Diagnostic tests are not entirely reliable

Must rule out in patient with acute scrotal pain/swelling

Diagnosis usually determined from history and physical

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Testicular torsion - History

Can begins abruptly in early morning/during resting

Severe pain from onset

History of scrotal trauma is common Pain that persists >1 hr after minor trauma is not normal

Often previous episode/s of similar pain

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TT – Physical exam

Scrotum/ position of testes

Cremasteric reflex - rarely intact in patients with TT

“Hard mass” or “swelling” Depending on how long the onset

Check for flank tenderness and bladder distension

Inguinal region for hernia/spermatic cord

Prehn’s sign Lack of pain relief with elevation suggests TT

Manual detorsion (controversy)

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Bell clapper

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Testicular torsion

Don’t delay proper treatment Most of the TT can be saved before 6 hrs of onset

Urinalysis – Not reliable to rule out

Color Doppler U/S: Operator dependant May not demonstrate flow in very young boys Reduced flow may indicate torsion

Gold standard diagnosis of TT – Surgical exploration

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Doppler US - Arterial flow

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True testicular torsion

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Testicular/epididymal appendix

Wolfian duct remnant

Mullerian duct remnant

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Testicular appendix torsion

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Testicular appendix torsion Clinical presentation

Usually less severe pain and swelling Upper pole “blue dot” However, it may be mimic TT

Management Reassurance Relative rest Analgesic (acetaminophen, codeine) It takes 1-2 weeks to improve Return to ER if any change in symptoms/findings

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Testicular tumors

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(paratesticular rhabdomyosarcoma)

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Testicular tumors

Uncommon disease 0.5-2 / 100.000 children 1% to 2% of all pediatric solid tumors Incidence of childhood testicular tumors

Peaks at age 2 years Tapers after age 4 years Rises again at puberty

Germ cell tumors account for 65% of prepubertal tus Rare among black and Asian children

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Diagnosis

Painless firm testicular mass is the MC presentation of a child with a testicular tumor

Negative transillumination Disorders that must be excluded:

Epididymitis Hydrocele Hernia Spermatic cord torsion

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Investigation US testis Alpha-fetoprotein

High in 90% of Yolk sac tumor Single polypeptide chain amino acid produced by the fetal yolk

sac, liver, and gastrointestinal tract Half-life: 5 days (25-30 days)

β-hCG Made by syncytiotrophoblast Is rarely increased in preadolescent tumors Half-life: 24 hrs (5-7 days)

Serum LDH (lactate dehydrogenase) Chest x-ray and abdominal CT

Retroperitoneal lymph nodes are the 1st site of meta Lung (MC distant site of meta)

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MC types in children Yolk sac tumor

Most common type in childhood - 60% Mostly in < 2 years of age Persistent AFP elevation post orchiectomy suggests mets

Teratoma Usually benign in children Second most common in childhood (21%) Mean age: 18 months US usually contain complex cysts Germ cell tumor with more than one germ cell layer

Endoderm, ectoderm, and mesoderm Cartilage, bone, mucous glands or muscle

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Testicular tumor - Treatment

Initial treatment is radical inguinal orchiectomy Stage I disease do not receive additional adjuvant Routine RPLND and/or adjuvant chemotherapy is not

indicated (only if metastasis)

Partial orchiectomy in selected cases Teratoma: No report of metastases in prepubertal males

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Timely surgery

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Testicular mass…Who and when I should refer?

All cases of testicular mass should be referred as soon as diagnosed

Even before US is obtained In testicular cancer, time matters

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Abdominal mass in children

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Abdominal mass in neonates

Abdominal mass (75% arise in the GU tract) 1-Hydronephrosis is the MC (UPJO, VUR, UVJO, PUV) 2-Multicystic dysplastic kidney (MCDK) 3-Tumors account for 12 %

MC abdominal tumors: Neuroblasoma, congenital mesoblastic nephroma and teratoma (sacrococcygeal)

Hydronephrosis MCDK

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Abdominal mass in neonates

Neuroblastoma is the MC malignance in neonate

Wilms tumor is extremely rare

Abdominal mass + hematuria: renal vein thrombosis

Girl with abd mass + interlabial bulging: hydrocolpos

CMN is the MC renal tumor

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Abdominal mass after neonatal period

From 1 month to 1 year of age Hydronephrosis – 40% Solid masses and tumor – 40%

Older than 1 year Tumor is the MC cause of abdominal mass

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Neuroblastoma Wilms Tumor (Nephroblastoma)

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Neuroblastoma (NB)

MC malignant tumor of infancy 8% to 10% of all childhood cancers Annual incidence 10 cases per 1 million Median age at diagnosis: 22 mo 50% of cases < 2 years of age (75% <4yrs)

(Fortner et al, 1968)

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NB- What and Where?

Tumor of the neural crest cell origin Cells that form the adrenal medulla and sympathetic ganglia

75% are retroperitoneal 50% adrenal 25% sympathetic chain (from neck to pelvis)

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NB - Presentation

Often has systemic symptoms (different from WT) Fever, abdominal pain or distension, abd mass,

weight loss, anemia, bone pain, proptosis and periorbital ecchymoses (retro-orbital metastasis)

Metastases are present in 70% at diagnosis

VMA (vanilmandelic acid), HMA (homovanillic acid) Are elevated in > 90% of the neuroblastomas 24 hs urine collection (catecholamine metabolites)

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NB - Imaging

US is usually the first exam in child with abdominal mass

CT or MRI Both detect extension beyond midline and hepatic

involvement MRI: better displays the relationship with great

vessels and detects intraspinal extension (tumor of sympathetic chain)

CT may show calcifications (rare in Wilms tumor)

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NB - Treatment Generally based on risk assessment

Tumor stage Grade Biochemical risk factors Genetic risk factors

Low-stage favorable Sx alone

Higher risk tumor Adj chemo +/- Rt

Very aggressive tumor Autologous bone marrow

transplantation

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Wilms’ tumor (Nephroblastoma)

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Wilms' Tumor

MC primary malignant renal tumor of childhood Embryonal tu develops from remnants of immature

kidney Annual incidence 7 to 10 cases per million Median age 3.5 yrs 80% diagnosed < 5 yrs of age Worldwide sex ratio is close to 1

(North America girls slightly > boys)

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Congenital anomalies and WT

Genitourinary anomalies in 4.5% of WT Renal fusion anomalies Cryptorchidism Hypospadias

(Breslow et al, 1993) These are common disorders and screening for WT

is not necessary in most children with genital anomalies

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Syndromes associated with WT Without overgrowth

Denys-Drash syndrome (DDS) Male pseudohermaphroditism, renal mesangial sclerosis and WT

( Drash et al, 1970)

Aniridia (Found in 1.1% of patients with WT) WAGR syndrome

(W ilms' tumor, a niridia, g enital anomalies, mental r etardation(Clericuzio , 1993)

Horseshoe kidney (NWTSG found 7 times incidence of WT)

With overgrowth Hemihypertrophy, which may occur alone or with syndromes Beckwith-Wiedemann (BWS), Perlman, Soto, Simpson-Golabi-

Behmel ( Perlman et al, 1975; Neri et al, 1998)

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Imaging in WT

Ultrasound is the first study performed in most children with an abdominal mass. (solid nature of the lesion)

CT shows the relationship with other organs

MRI is the study of choice if extension of tumor into the inferior vena cava cannot be excluded by ultrasound (Weese et al, 1991)

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Treatment of WT

Surgical Radical nephrectomy Accurate staging for determination need Rt +/- chemo Exploration of the abdominal cavity

Liver and nodal metastases and peritoneal seeding Formal exploration of the contralateral kidney

Should be performed before nephrectomy Formal retroperitoneal lymph node dissection

Is not recommended

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Urinary incontinence - Definitions

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Urinary incontinence Normal voiding

Activation of the micturition reflex

The micturiction reflex Under voluntary control Coordinated by the pontine micturiction center

Two neurological systems involved Sympathetic system (relaxes detrusor and closes internal sphincter) Parasympathetic system (detrusor contraction) Somatic – Pudendal nerve (external urethral sphincter)

Continence Learned behavior

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Physiology of micturition

From Blaivas JG: Pathophysiology of lower urinary tract dysfunction. Clin Obstet Gynaecol 1985;12(2):295–309.) Copyright © 2003, Elsevier Science (USA). All rights reserved.

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Transition from childhood to adult pattern of urine control

Many children transiently presents Voiding disturbances or Urinary incontinence that suggest dysfunction But they are simply a normal transition phase

They are in fact a process of bladder function maturation and self limited

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What is urinary incontinence? Is the involuntary loss of urine

A careful history may determine the cause

Four main categories Continuous Stress Urgency Overflow

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Nocturnal enuresis

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Enuresis, Is it a…night or daytime problem?

Nocturnal enuresis Involuntary loss of urine during sleep

Neurological maturation and improve over time

Daytime incontinence Involuntary loss of urine during the day (awake) Need to rule out

Neurological causes Dysfunctional elimination syndrome Other causes (anatomical GU abnormalities)

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Nocturnal enuresis 15 % at 5 yrs and 1% at 15 yrs of age

It is considered normal until 6 years of age

Often children > 6 yrs should be investigated Exam of lower back

Skin dimple, hairy tufts, discolorations Ultrasound Urinalysis and urine culture

Vast majority these tests will be normal

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Nocturnal enuresis Number of theories tried to explain the cause

Behavioral, genetic, developmental, neurologic, psychological, urodynamic, and organic causes

There is no single explanation for this symptom

Multiple factors may be involved

Neurological maturation theory is the MC accepted

MNE is a symptom rather than a disease

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Urodynamics in MNE

Not indicated

Research studies Urodynamics don’t show increased bladder instability Involuntary contractions are not the cause MNE

Therapy aiming at eliminating uninhibited

contractions is generally ineffective (anticholinergic)

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Vasopressin levels

In normal children Increased production of vasopressin at night 50% less urine is normally excreted at night

Theory

Vasopressin deficiency is the cause for NE Controversy, contradictory studies

However, many children with MNE have similar levels of vasopressin during both the day and the night

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Hereditary Factors

Likelihood of MNE 77% if it occurred in both parents 43% if 1 parent had NE 15% if neither parent had NE

Twin studies Monozygotic 65-70% Dizygotyic 31-44 %

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Evaluation Sufficient evaluation for most children with primary MNE

A carefully history Physical examination Urinalysis

Check for a history of Urinary infection Diurnal incontinence Obstructive GU symptoms or signs of neuropathy

In their absence of above risk factors There is generally no indication for radiographic studies or cystoscopy The incidence of associated uropathology is low

However, some authors recommend U/S in children older than 6 yrs

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Treatment - Enuresis

Controversy Some authors recommend treatment in children after the

age of 5-6 years of age Others wait to treat until the child seems bothered by the

problem Treatment should be individualized

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Pharmacologic Therapy

Anticholinergic therapy has low effectiveness ranging from only 5% to 40% (Person-Junemann et al, 1993; Kosar et al, 1999)

Imipramine (tricyclic antidepressant) Increases functional bladder capacity Good result in 40% to 50% of cases However, discontinuation causes relapse in up to 60%

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DDAVP - Desmopressin

Rationale - Reduction of urine output at night Some enuretics have reduced nocturnal vasopressin

concentrations and have nocturnal polyuria (Norgaard et al, 1989c)

The therapeutic effect of DDAVP is temporary

50% to 90% of children relapse after stop treatment(Kahan et al, 1998)

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Behavior Modification

Should be considered the first-line approach Bladder training (increase bladder capacity) Responsibility reinforcement Classic conditioning therapy with a urinary alarm

Varied success rate (40-60%)

Most effective and reproducible rate of cure

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Daytime Incontinence

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Daytime incontinence MC causes of daytime incontinence in children

Dysfunctional voiding Neurological conditions Rarely – Obstructive (PUV or urethral stenosis), fistulas, sphincteric

incontinence, etc

Daytime wetting is of more concern than nocturnal enuresis May be caused by an organic problem

Complete physical Lower back, palpable bladder, neurogenic conditions Girls: Vulvo-vaginitis, labial adhesion (vaginal voiding),

ectopic ureter Boys: Urethral meatal stenosis, fistulas

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Daytime incontinence,Non-neurological cause Dysfunctional voiding is the MC cause Part of a “behavioral elimination syndrome”

Intestinal constipation Holding urine for too long Abnormal spastic pelvic floor

Voiding and stoolling calendars (3 days record)

Other causes Ectopic ureter, urethral stenosis (overflow), external sphincter

insufficiency, vaginal or rectal fistulas

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Daytime incontinence,Neurologic causes Tethering of the spinal cord (TC) is the MC cause

Occult spinal dysraphism (primary TC) Stigmata (skin dimple, discoloration, hairy tufts, gluteal

asymmetry) Open spina bifida (secondary TC)

Lipomyelomeningocele, dermal sinus tract, split cord malformations, syringomyelia, etc

Post-operative (myelomeningocele repair)

Other neurological causes Down syndrome, sacral agenesis, VACTERL complex,

imperforate anus, cloacal anomalies, etc

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Evaluation if Neurogenic cause is suspected

Urodynamics US kidneys and bladder MRI of the spinal cord EMG of the legs and perineum Complete neurological assessment (neurosurgeon)

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Tx of daytime incontinence

Dysfunctional voiding Increase fluid and fiber in the diet Prompt to void every 2-3 hours Maintain soft and non-painful BM

Stool softener if necessary

Neurogenic According the underlying cause Release of tethered cord Anticholinergic, Clean intermittent bladder catheterizations Bladder augmentation

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Who I should refer?

All cases of daytime incontinence should be referred to urological assessment