Pediatric Urology UpdateRama Jayanthi, M.D.Section of Pediatric UrologyColumbus Childrens Hospital

Format and purposeSelected cases in pediatric urologyStimulate discussionDiscuss management

Case 1Hypospadias noted at birthBoth testes normally descended

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

Hypospadiasabnormally positioned meatusmeatus can be located anywhere from perineum to glanschordee- associated penile curvature

Hypospadias- associated abnormalitiesEasy to remember - nothing!Normal kidneys and bladderNormal fertilityNormal sexual function

Hypospadias - management for the pediatricianDo not circumcise!No need for any imaging studiesRefer to pediatric urologist within first months of lifeAlways 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 circIf glans meets in midline proximal to meatus, not a hypo!Even if meatus appears to be largeIf a true hypo is presentWrap with Vaseline if not bleedingOtherwise close skin edges with chromic sutures

Hypospadias - management for the pediatric urologistSurgical correction at 6 - 9 months of ageAttempt one stage reconstructionOut patient surgerySuccess rates should be > 95%

EpispadiasVery rare - more often associated with bladder exstrophyNeed early referral for parental counselingPatients may be totally incontinent

Case 2: Scrotal massPainless scrotal massesStable in sizeNo increase with cryingNo inguinal bulge

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

Scrotal massesSolid vs. cystictransillumination of lightTesticular vs. extratesticularPainful vs. painless

Hernia/hydrocele - cystic scrotal massTestes develop intraabdominally and exit the abdomen at the internal ringAll males have a fascial defect at some point during gestationPersistence of defect leads to communicating hydroceles and hernias

Hernia/hydroceleWhat is the difference between a hernia and a communicating hydrocele?Both are the same anatomic defectIf opening only large enough to admit peritoneal fluid - communicating hydroceleScrotal swelling only, comes and goesIf opening large enough to admit bowel- clinical herniainguinal bulge

Hernia/hydrocele

Hernia/hydrocele - managementObservation:Noncommunicating hydrocele < 12 - 18 months of ageHernia - very premature infants with easily reducible large herniasSurgery:Hydrocele - persistent, enlarging, painfulHernia - always Surgical correction involves ligation of peritoneal sac

What is the diagnosis?Findings:

Painless right scrotal massDoes not transilluminateUltrasound: solid massDiagnosis: yolk sac tumor

Case 3A 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 recumbentWhat is the most likely diagnosis?

VaricoceleRepresents dilation of left spermatic veinsEtiology unknown? Lack of venous valves? High intravenous pressureIncidence: 15% of all teenage males rare in prepubertal males

Significance of varicocelesInfertilityMost common surgically correctable cause of male factor infertilityReason unclear? Increased temperature of scrotum? Primary endocrinopathyPainUncommon in teenagersDull ache

Management of pediatric varicoceleClinically significant varicoceles requires surgical ligationProblem:Most teenagers have varicoceles detected on routine physical examinationUsually asymptomatic

Management of adolescent varicocelesYearly measurement of testicular sizeSymmetric testes - observeIndications for intervention:Development of size discrepancy > 2ccPain

Personal opinion:Spermatic vein embolization may be the simplest and least invasive option

Case 4A 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 orchidopexyIf an US shows no testis it may be inaccurate because the child may have a small intraabdominal testis that was not detectedRegardless of US findings, the child needs explorationThus, there is no need for radiological evaluation for a nonpalpable testis

Classification of UDTIntraabdominaltestis located above internal ringusually nonpalpableCanalicular- routine undescended testisRetractile - not a UDTdue to hyperactive cremaster reflexonly in prepubertal malesno hormonal/testis defects

Management of UDTObservation until 6 -12 months of ageIf still undescended, surgical correctionNo advantage to further observation after 12 months of agetestis will not descendgerm cell fibrosis evident by three years of life

Bilateral nonpalpable testesKaryotyping essentialMain question: Is there functional testicular tissue present?No functional tissue present ifmarked elevation baseline FSH and LHno rise in serum testosterone with HCG stim

Fertility after cryptorchidismLee, Brit J Urol, 1995

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%)

Risk of NeoplasiaUDT has 10X greater riskAbdominal testis has 4X greater risk than inguinalTumors occur after pubertyMean age 25 - 30 years25% occur in normally descended testisEarly orchidopexy may be protectiveSeminoma most common, embryonal cell 2nd

Case 5A nine year-old uncircumcised boy presents with a tightly phimotic foreskin.He has had a few episodes of balanitisHis parents to do not want him to be circumcised if possibleWhat can be done?

Natural history of phimosis

Medical management of phimosisProspective trialDiprolone cream (0.05%) applied TID for 4 weeks to preputial bandPatients reevaluated at one month

Medical management of phimosisResultsn = 21Signs and symptomsUTIBalanitisPreputial ballooningAsymptomatic

Medical management of phimosisSuccess 17/21 (81%)11 complete, 7 partialFailure 4/21 (19%)

What does a bladder do??Store urineEmpty urineIn a 24 hour time periodBladder is in storage mode for 23 hours and 45 minutesThus, storage function is of greater importance than emptying

Normal bladder functionStorageStorage must take place at low pressuresIntravesical pressures must be low enough toNot impede urine transport from kidneys via the uretersHydronephrosis/renal injuryNot overwhelm sphincteric resistanceUrinary incontinence

Emptying functionFirst step in voiding is relaxation of sphincteric mechanism followed by bladder contractionNormal 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 historyDaytime wetting vs. nighttime wetting vs. bothUrgency?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 mythssmall bladder that the child has to grow intonarrow urethra that needs to be stretchedinability to sense fullnessUrgency and/or frequency in a male may be due to meatal or urethral stenosisVoiding dysfunction may be due to

Evaluation of voiding dysfunctionsHistory most importantScreening renal ultrasoundEnsure normal kidneysAlleviates parental anxietyBladder wall thicknessSubtle sign of bladder overactivityPost-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 diaryExpected bladder capacity: Age + 2 in ouncesVCUG rarely neededhistory of significant UTIsymptoms of obstruction in males

Varieties of voiding dysfunction In order of frequencyBladder instability/overactivityInfrequent voidingIncomplete emptyingHinmans syndromeNonneurogenic neurogenic bladders

Bladder instabiltyClinical manifestationswettinginfectionspelvic/vaginal painpenile/scrotal pain

Forms of bladder instabiltyUrgency incontinence syndromepredominant symptom is wettinginfections less likelyHypertonic bladder predominant symptom is UTImay also have associated wetting

Urgency incontinenceMore common than hypertonic bladderUsually associated urgency/frequencySeverity of wettings ranges from damp pants to soaking

Hypertonic bladderVCUG - trabeculated bladder, may have diverticulaeMain point: Infections (and reflux) are secondary problem

Distal urethral stenosisSpinning top urethraNOT due to obstructionA sign of bladder instability

Urethral dilation is NEVER indicated!!!

Management of bladder instabilityAnticholinergicsBowel managementConsider prophylactic antibiotics only if has recurrent infections refractory to standard managementThe older I get, the less I use prophylactic antibiotics

Choice of anticholinergicsOxybutininDitropan XL 5 -15 mg qAMAdvantages:once a day dosagefewer side effectsElixir (0.2 mg/dose/BID -TID)only if cannot swallow pills

Role of bowel dysfunctionFecal retentionIncomplete or infrequent emptying of bowelsSubtle cluesabdominal painperineal painvaginal itchingpenile pain

Relationship of constipation and wetting234 constipated/encopretics29% day and 34% night wetting pre-treatment, UTI in 11%52% had improvement in constipation89% improved day63% improved nightno more UTILoening-Baucke, Pediatrics, 1997

Importance of UTIs and bowel/bladder disturbances143 children with reflux+ breakthrough UTI77% had dysfunction- breakthrough UTI16% had dysfunction

Koff, J Urol, 1998

Infrequent voiding syndromeslazy bladder syndromean inappropriate term that incorrectly labels a child as being lazyFact of life for children:Children usually have more important things to do than urinate and defecateSensation normal - children tune out the bladder

Management of infrequent voiding syndromestimed voidingbehavioral modificationcontrolled briberyintermittent 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 7A 8 year old girl has her first episode of UTIHow 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 antibioticsRed introitusPerineal discomfortDysuria in the absence of a positive cultureA positive urinalysis is not sufficient to definitively diagnose an infectionMicroscopic hematuria

Philosophical questionsWhy do we treat urinary tract infections?What are the ramifications of UTIs?

Renal scarringmay cause hypertensionif present diffusely and bilaterally, may lead to renal failuremost likely will occur after pyelonephritic episodes in children less than 4 years of age

Thereforeif older child has episode of cystitis, recommend USif older child has pyelonephritic episode, recommend VCUG/USif younger child has any type of UTI, recommend complete workup, especially if male

Case 8Four year old girl with recurrent UTI, some with feverUS - normal, VCUG - normalRepeat nuclear cystogram also normalWhat do you do???

Non-reflux pyelonephritisThe majority of children with febrile pyelonephritis do not have reflux or any other urinary tract abnormalitiesWhat causes urinary tract infections in the absence of anatomic abnormalities?

Non-anatomic causes of UTIsticky bacteriadysfunctional bladder habitsdysfunctional bowel habitsall the above

Role of VCUG in children with UTIA VCUG is necessary to diagnose refluxTreatment of reflux is helpful to prevent pyelonephritis and renal scarringThus a VCUG is not necessarily needed in a child with normal kidneys and lower urinary tract infections

Case 9A 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 (contd)What is the diagnosis?Giggle incontinencePart of the cataplexy/narcoplexy complexTreatment consists of behavioral modificationsConsider Ritalin for nonresponders

Case 108 year old male who presented with urinary tract infectionsFever and flank pain

Case 10 (contd)On further questioning.Previously was dry but now has day and night wettingSignificant daytime urgency and occasional back painRarely has good streamParents have noted that the child also walks funny.

Case 10 (contd)Main diagnostic consideration: occult tethered spinal cordRelatively uncommonImportance in early detection in that delay in diagnosis may lead to permanent neurological deficit

Case 114 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 wetConsider ectopic ureter.Ureter does not insert into bladder. Inserts into urethra or vaginaSurgery is curativeKey is to consider the diagnosisIntravenous pyelography has very poor sensitivity.

Imaging for ectopic ureter

Imaging for ectopic ureter

Case 125 year old boy who suddenly developed severe daytime frequency. He doesnt 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 passRenal ultrasound is normal and anticholinergics have not helpedWhat is the diagnosis?

Case 12Daytime Frequency SyndromeUnknown etiologySpontaneous improvement is the rule

Thank you for listening

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