CONGENITAL DISORDERS OF THE LOWER URINARY TRACT

Pediatric GU Review

UCSD Pediatric UrologyGeorge Chiang MD

Sara Marietti MD

Outlined from The Kelalis-King-Belman Textbook of Clinical Pediatric Urology 2007

(not for reproduction, distribution, or sale without consent)

Vesicoureteral Reflux

Introduction

• Primary VUR – Abnormal anatomy of the UVJ

• Secondary VUR– Acquired condition from increased intravesical pressure

• Neurogenic bladder dysfunction• Non-neurogenic bladder dysfunction• Outlet obstruction

• VUR is really a marker of a heterogenous condition of the whole urinary tract versus a simple causal relationship

History

• Galen and Leonardo da Vinci were first to propose that normal UVJ allowed unidirectional flow

• Hutch observed that VUR and PN might be related in parapalegic patients

Anatomical Features

• Ureter enters bladder wall at an oblique intramural path– Flap-valve mechanism causing intramural and submucocal ureter to

be comrpessed with filling against detrusor backing

• UVJ mechanism is a passive and active process– Extension of ureteral longitudinal muscles into the trigone (Bell’s

muscle)• Down to veru in males and dorsal urethral in females• At trigone these muscle fibers extend fanwise and at midline

intermingle with contralateral ureter

– Middle circular ureteral muscle is not seen beyond the ureteral hiatus

– Waldeyer’s sheath =fibromuscular sheath that anchors the ureter within the hiatus

Anatomical Features

• Ureteral muscles extend into trigone and are innervated by noradrenergic receptors similar to vas

• Ureteral maldevelopment may be tied together with abnormal trigonal development

• Vit A signalling deficient mice demonstrate abnormal trigonal development with high incidence of distal ureteral anomalies

Anatomical Features

• Functional integration of the ureter and trigone is important to normal UVJ mechanism– Tanaghounilateral sympathectomy in cats

induces VUR– Increasing trigonal tone/contractility (electrical

stim, bladder dist, NE) all resulted in functional occlusion of the ureteral orifices

Anatomical Features

• Summary– Natural tone of ureteral muscle provides a mild

passive closure of the intramural ureter– With filling, there is a progressive obliquity of the

intramural/submucosal ureterflap-valve– Micturition stimulates trigonal tone, preventing

lateral displacement of the orifices

Anatomical Features

• Intravesical ureter lengthens with age– Neonates average length=0.5 cm, adults=1.3 cm– Mature length at 10-12 yrs– Length varies with reflux in other species

• Trigonal competency is important to prevent lateral displacement– Displacement occurs from abnormal ureteric bud origin along the

developing mesonephric duct during metanephric development– Mackie and Stephens found a correlation between lateral

displacement of the UO and degree of renal dysplasia and hypoplasia

– VUR can be a primary trigonal disorder• Trigone develops as a result of the common nephric duct and the distal

ureter incorporating into the developing bladder

Anatomical Features

• Endoscopic observations– Ratio of submucosal tunnel length to ureteral

diameter• Paquinnormal =5:1 children with VUR 1.4:1• Lyon et al: 4 basic orifices

– Cone 4% VUR

– Stadium 28%

– Horseshoe 83%

– Golf-hole 100%

• Lateral pillar defect (between horseshoe and golf)

Demographics

• True incidence difficult to ascertain but probably 0.4-1.8%• Incidence of VUR in children with UTI=30-50%

– Female predominance• Antenatal hydronephrosis25% VUR

– Infant male predominance• Boy present at younger ages with higher grades (secondary to

transient urethral obstruction and high intravesical pressures)– Higher voiding pressures and small functional capacity

• Sibling VUR as high as 40-50%– Dominant inheritance pattern with 60% parent to child– Asian and African Americans have a lower rate vs caucasians (3.4

to 1)

Primary vs Secondary• Patients presenting with VUR must be managed with

global view– UVJ mechanism– Bladder dysfunction– Predisposition to UTI– Renal Anomalies

• Secondary VUR from bladder anomalies is common and BOO– Chronic elevation of bladder storage pressure and distortion at

UVJ (paraureteral diverticulum) can cause secondary VUR– Most common anatomic cause of secondary VUR is PUV– Anatomic obstruction is rare in females except obstructing

ureterocele– Altered bladder function with poor compliance and dyssynergy

can lead to increased intravesical pressures• If pressure >40cm incidence of VUR is >80% with myelo/neurogenic

bladders

UTI and VUR

• Lower urinary tract inflammation may affect the mildly deficient UVJ

• New VUR was reported in 6/7 piglets after E. Coli infection– Some E. coli strains directly affect the urinary tract smooth

muscle– Bacterial endotoxin has been found to inhibit alpha-

adrenergic receptors of the trigone musculature

• Gross and Lebowitz surveyed 600 children undergoing cystographic eval for VUR and found no evidence of increased VUR with positive urine culture– VUR not caused by UTI

Lower urinary tract function

• Infantile bladder has higher voiding pressures from imperfect coordination between detrusor contraction and sphincter relaxation (disappears at 2 yrs)– Lack of integration between bladder/sphincter/brainstem/cortical

centers– Versus normal infants, those with VUR have higher voiding

pressures, and abnl bladder function even without UTI– Discoordinated voiding pattern most common UDS parameter in

male/females with VUR– Bladder hypercontractility in infant boys with VUR– Transient urethral obstruction may be the cause of infantile VUR– Abnormal UDS may imply a global maldevelopment of the trigone

and proximal urethral mechanism

Lower urinary tract function

• Pediatric voiding dysfunction– Frequency/urgency/enuresis are manifestations of detrusor

instability and/or DSD– 360 dysfunctional voiders60% with UTI and 20% VUR– International Reflux Studypresence of dysfunctional

voiding in 18% of VUR with UTI– UDS has shown up to 60% of children with VUR have DI– DI may exaggerate VUR and can cause delayed resolution

• Treatment with anticholinergics can facilitate resolution

Non-Surgical Management of VUR

Non-Surgical Therapy

• Antibiotic prophylaxis– Daily– Intermittent (I.e. treatment of UTIs)

• Bladder training

• Treatment of voiding dysfunction

• Periodic assessment of reflux and child well-being

Reflux Resolution

• Higher likelihood in younger children and those with lower reflux grade

• IRS Study– At 5 yrs

• 92% Grade 1

• 81% Grade 2

• Grade 3: resolution highest for children<2 and unilateral (70%) whereas bilateral reflux >5 (12.5%)

• Grade 4: Unilateral 58% at 5 yrs; 10% with bilateral

• Beyond 5 years, limited data

Antibiotic Prophylaxis

• Treating each symptomatic UTI– In children with existing scars, 66% developed new scars

• Observation– 50% of girls had a UTI during observation and even 23%

with Grade 1 had scarring

• Girls more likely to develop renal scars, scars could occur at any age, and children with mild reflux are susceptible to scar formation

Antibiotic Prophylaxis

• Prospective eval of 203 children; 3% developed scars

• Skoog et al-->545 children with Grade 1-4 on prophylaxis only 0.5 % scarring

• Ideal antibiotic still uncertain

Breakthrough UTI

• Girls with reflux and voiding dysfunction are most likely to develop breakthrough UTI

• In boys with reflux, uncircumcised are most likely• In both sexes, prior renal scarring is the greatest risk

for breakthrough UTI• A breakthrough UTI is an indication of failure and

surgical therapy should be considered

Expectant Management

• In selected patients it seems reasonable to dc prophylaxis

• Normal voiding pattern and age of 8.6 yrs– Grades 1-4– UTI in 5/40 girls and 1/11 boys (Grade 3 VUR)

• Expectant management may be employed if voiding pattern is normal

• It is unresolved whether sibling reflux or screening after MCKD or renal agenesis have a lower risk of reflux-related complications

• Patients should understand risk of PN with pregnancy and sexual activity

Expectant Management

• 115 adult women with VUR discovered after 16 years of age; 73% with refluxing kidneys had reflux nephropathy

• Women with VUR have a higher risk of febrile UTI versus those without VUR

• Persistent VUR in females should be corrected

Bladder Training

• Reduce the likelihood of developing UTI by lowering voiding pressure

• Infrequent voiding, DSD and constipation can increase the likelihood of bacteriuria

Voiding Dysfunction

• VUR and dysfunctional elimination have a higher rate of breakthrough UTI

• Up to 4x increase• Longer time to spontaneous resolution• Unsuccessful surgical outcomes with voiding

dysfunction• High VD symptom score with training and

decreased score predicted VUR improvement

Anticholinergic Therapy

• Children with overactive bladder may be treated with combination of anticholinergic and timed voiding– Children experience incontinence

24hrs/day

• Increased spontaneous VUR resolution with anticholinergics

Alpha-Blocker Therapy

• Pelvic floor overactivity and significant PVR– Doxazosin may reduce incontinence

Biofeedback

• May be useful to establish a normal pattern of sphincteric relaxation and reduce anticholinergic need

• Uroflow study, PVR check, 1 hr session with EMG probes

• Children may have difficulty isolating external sphincter and may allow them to learn sphincteric relaxation

• Pelvic floor exercises may help unstable bladder contractions

Biofeedback

• Utilize in children with symptoms of OAB that do not respond to anticholinergics

• Or, as alternative to anticholinergics

Follow-Up

• Screening for UTI– No evidence that periodic screening of asymptomatic

children with VUR is beneficial

• Cystography– Every 12-18 months– Non-compliance with follow-up visits up to 33%– Alternative is every year for mild reflux and every 3 years

with severe reflux– Give oral or nasal midazolam for VCUG although 31% of

children still remember

Follow-Up

• Upper-tract Imaging– No standards

• If no febrile UTI, should be no change in upper tract

• Other assessment– Follow-up should be annually for height/weight– BP check if presence of renal scarring

Fetal Obstructive Uropathy• Obstruction of urinary system

– Oligo/anhydramnios– Bilateral Hydronephrosis – Distended Bladder

• Timing of diagnosis– 9% of uropathies apparent at 17 weeks – 90% apparent at 33 weeks

Cusick EL et al. Mortality after an antenatal diagnosis of fetal uropathy. J Pediatr Surg 1995; 30:463-466.

Lower Tract Obstruction

• Urethral Atresia– Bladder Distension

– Vesicoureteral reflux

– Hydronephrosis

– Renal Dysplasia

– 95% Mortality

Herndon et al. Early Second Trimester Intervention In A Surviving Infant With Postnatally Diagnosed Urethral Atresia. J Urol. 168 pp 1532-1533 October 2002   

• Incidence 1 in 5000 to 1 in 8000

• 25-50% seen in neonatal period

• 50-70% present within 1st year

• Hydronephrosis is present in 90% of infants with valves

Posterior Urethral Valves

Agarwal, S. Urethral Valves. BJU International 84 pp 570-578 1999

• Presentation– Prenatal ultrasound in 2nd trimester

Posterior Urethral Valves

• Types

Posterior Urethral Valves

Gonzales, ET.. Posterior Urethral Valves and Other Urethral Abnormalities. Campbell’s Urology; 8th Edition

Posterior Urethral Valves

• Type I valves: 95% of the lesions • Type III make up the remainder

Gonzales, ET.. Posterior Urethral Valves and Other Urethral Abnormalities. Campbell’s Urology; 8th Edition

• Embryology

Posterior Urethral Valves

Gonzales, ET.. Posterior Urethral Valves and Other Urethral Abnormalities. Campbell’s Urology; 8th Edition

• 1/3 of children with ESRD <4 yo have obstruction as the underlying cause

• Pulmonary Hypoplasia from AF

• 95% mortality with early mid-gestational oligohydramnios

Posterior Urethral Valves

Peters, C. Perinatal Urology in Campbell’s Urology 8 th Edition

Agarwal, S and Nicholas Fisk. In utero therapy for lower urinary tract obstruction. Prenatal Diagnosis 21. pp 970-976. 2001

Posterior Urethral Valves

•Mortality 45% with presentation at birth

Courtesy of Drs. Cendron and Borer

• Long term complications– Bladder dysfunction: “Valve Bladder”– Renal dysfunction

• Late onset of renal failure

– Sexual function/fertility• Decreased libido/potency

• EJ dyfunction

• Cryptorchidism

Posterior Urethral Valves

H.T. Nguyen and C.A. Peters. The long-term complications of posterior urethral valves. BJU Int 83 Suppl 3 pp23-28. 1999

Woodhouse, CR. The fate of the abnormal bladder in adolescence. J Urol 166 pp2396-2400. Dec 2001

Exstrophy

Epidemiology

• 1/20,000 -1/40,000 live births

• 3.3/100,000• Sibling: 1-3.6% risk• Child: 1.4%• M>F 1.5-2:1 • Controversial

epdemiology

Kiddoo et al, Urol Clin N Am 31:417-426, 2004

Epidemiology

• Multifactorial

• 18 case reports of familial exstrophy

• 1% sibling risk

• 1.4% child risk

• Historically poor fertility – difficult to define family risk

Grady et al, Urol Clin N Am 26(1)95-109, 1999

Epidemiology

• 205/9,452,110 (2.15/100,00 live births)

• Whites > non-whites

• M=F

• Increased prematurity, cleft palate, spina bifida, GI abnormalities

• $10,000,000 annual expenditure

• $62,302 per case

Nelson et al, J Urol 174:1099-1102, 2005

Associated Anatomic Variation

• Shortened umbilical -anal distance

• Widened inguinal canal• Bilateral reflux – absent

submucosal tunnel • Pubic diastasis• Rectal prolapse• Duplex system

Kiddoo et al, Urol Clin N Am 31:417-426, 2004

Bladder

• Varying capacity – up to 15-30 cc

• Early changes to transitional epithelium

• Polypoid change• Metaplasia

malignancy if untreated• ? Poor intrinsic detrusor

function

Kiddoo et al, Urol Clin N Am 31:417-426, 2004

Male genitalia

• Short/wide penis• Short, deep corpora• Dorsal chordee• Lateral displacement

of neurovascular bundles

• Vas, SV, ducts usually normal

Classic Male Exstrophy

Classic Male Exstrophy

Grady et al, Urol Clin N Am 26(1)95-109, 1999

Female genitalia

• Hemiclitoris

• Narrow introitus

• Anterior displacement of vaginal orifice

• Duplicated vagina, uterus

• Adnexal normal

• Higher rates of vaginal/uterine prolapse

Kiddoo et al, Urol Clin N Am 31:417-426, 2004

Embryology

• Persistence of cloacal membrane (proven wrong)

• Failure of mesoderm to ingrow between ectoderm/endoderm of cloacal membran rupture exstrophy

• Caudal displacement of genital tubercle

Grady et al, Urol Clin N Am 26(1)95-109, 1999

Walsh, et al Campbells Urology p. 2139

Walsh, et al Campbells Urology p. 2138

Epidemiology

Nelson et al, J Urol 174:1099-1102, 2005

Ultrasound

• Prenatal diagnosis possible after 20 weeks

• High false negative rate

• Absence of bladder filling, nl kidneys, low set umbilical cord, anterior abdominal mass

• Ddx – omphalocele, gastrochisis

Grady et al, Urol Clin N Am 26(1)95-109, 1999

Skeletal Defects

• Pubis diastasis• Shortened pubic

rami• Retroverted

acetabulum• External rotation of

posterior pelvis

Walsh, et al Campbells Urology p. 2141

Skeletal Defects

• Waddling gait• Gait defects usually

improve without intervention

• Osteotomy helps GU reconstruction

• Osteotomy decreases osteoarthritis

Walsh, et al, Campbells Urology p. 2141

Osteotomy Rationale

1. Approximate symphysis allows tension free abdominal wall closure

2. Facilitate urethra placement deep in pelvis3. Bring pelvic floor musculature medial, which aids in

bladder neck suspension and continence4. Minimize tension on perineal neurovascular bundle5. Reduces risk for dital corporeal / glannular

ischemia

Walsh, et al, Campbells Urology p. 2141

Kiddoo et al, Urol Clin N Am 31:417-426, 2004

Initial Management

• Silk tie on umbilicus (clamps irritate mucosa)

• Saran wrap• Change saran wrap

with diaper changes; irrigate with saline

• Keep moist!

Goals of Reconstruction

Renal function

Continence

Decreased UTI

Functionalgenitalia

genitaliaCosmetic

Reconstruction

• 1900’s urinary diversion (ie ureterosigmoidoscopy) preferred

• Bladder is functional– Normal detrusor activity– Normal muscarinic receptors– Sphincter can be functional and coordinated

• Improved surgical technique

Staged Reconstruction

I Primary closure; osteotomy; approximation of symphysis

II Epispadias repair, urethroplasty, penile reconst.

III Bladder neck reconstruction., reimplant

0-72 hours

4-6 months

4-5 years

Exstrophy Practice Patterns

Nelson, et al, J Urol 174(3): 1099-1102, 2005

Primary Closure

• Typically within 72 hours

• If bladder too small for closure wait 4-6 months for template to grow

• Joint orthopedic procedure - osteotomy

Primary Closure

Primary Closure

Primary Closure

Primary Closure

Primary Closure

Primary Closure

Primary Closure

Primary Closure

Primary Closure

Primary Closure

Primary Closure

Staged Reconstruction

I Primary closure; osteotomy; approximation of symphysis

II Epispadias repair, urethroplasty, penile reconst.

III Bladder neck reconstruction., reimplant

1 day (12 hrs-8 mos.), +/- osteotomy

21 months (8-63 mos.)

51 months (21-105 mos.), +\- augment (6/48)

Shaw, et al, J Urol 172:1450-1453, 2004

Outcomes

• Continence Definitions– Continent: daytime dry for > 3 hrs, no

stress or nocturnal incontinence– Partially continent: daytime dry 1-3 hours,

occasional stress or nocturnal incontinence

– Incontinent: daytime dry < 1 hour, more than occasional stress or nocturnal incontinence

Shaw, et al, J Urol 172:1450-1453, 2004

Results

• 43/48 (90%) continent

• 4/48 (8%) partially continent

• 1/48 (2%) incontinent

• 13/38 (34%) continent after BN reconstruction alone (trend towards higher volumes)

• 6/6 (100%) later augmented became continent

• 4/9 redo BNR incontinent, 5/9 redo with augment became continent

Shaw, et al, J Urol 172:1450-1453, 2004

Results

• 10/43 (23%) continent patients with urethral voiding only

• 8/43 ( %) continent patients CIC + partial void per urethra

• 24/43 ( %) exclusively CIC

Shaw, et al, J Urol 172:1450-1453, 2004

Conclusions

• Poor overall continence rates

• Often requires augmentation

• Redo bladder neck reconstruction with poor outcomes

Shaw, et al, J Urol 172:1450-1453, 2004

Fertility

• Decreased overall fertility in men

• 10X increase in cyptorchidism (controversial)

• Women with normal sexual function and fertility

• C-section advocated (risk of uterine prolapse)

Kiddoo et al, Urol Clin N Am 31:417-426, 2004

QUESTIONS

A 28 year old man with an ileal conduit for 20 years is scheduled for a renal transplant. He had posterior urethral valves treated as an infant. A CMG shows a high capacity bladder with uninhibited contractions, and the maximum voiding pressure is 50 c. After 5 days of bladder cycling, his bladder capacity increases to 200 cc. The best management is:

a) Transplant into the existing ileal conduit

b) Bladder augmentation before transplant

c) Transplant into native bladder

d) Neobladder construction before transplant

e) Indiana pouch construction before transplant

A 14 year old boy with incontinence had posterior urethral valves resected as a neonate. The most likely etiology for his incontinence is:

a) Occult neurogenic bladder

b) Sphincteric injury

c) Hyperreflexic bladder

d) Hypertonic bladder

e) Myogenic failure

A 10-day old infant male is hospitalized for FTT. After his umbilical stump fell off, fluid has intermittently drained from the umbilicus. The umbilical fluid has a creatinine of 10 mg/dl and grows >100,000 cfu of E.Coli. The next step should be antibiotics and:

a) Observation

b) Cannulation and injection of contrast

c) VCUG

d) Closure of fistula

e) Urethral catheter drainage

In a child with febrile UTI, the antimicrobial drug that achieves therapeutic levels in the urine but not in the tissue

a) Ampicillin

b) Gentamicin

c) Nitrofurantoin

d) Trimethoprim

e) Cephalexin

A five year old boy with bladder exstrophy underwent neonatal closure with bilateral posterior iliac osteotomies. He remains totally incontinent despite repair of his epispadias at 2 years of age. Studies reveal a bladder capacity of 50 cc with mild hypertonicity. The next step is:

a) Continent urinary diversion

b) Augmentation cystoplasty

c) Bladder neck reconstruction

d) Injection of collagen at the bladder neck

e) Bladder neck reconstruction and augmentation cystoplasty

An 8 year old uncircumcised boy has previously undergone a DVIU for a urethral stricture. The stricture recurs after 10 months. Evaluation reveals a 2 cm bulbar stricture. The next step is:

a) Staged urethroplasty

b) Pedicle-flap urethroplasty

c) Repeat DVIU

d) Primary excision and end to end anastamosis

e) Urethral dilation with CIC

A 12 year old boy has severe dysuria and hematuria. Ultrasound shows a bladder mass and mild right hydro. Urine culture is sterile. Cysto shows a diffuse, erythematous bullous mass at the bladder base. Biopsy shows intense inflammation, granulomatous reactions and an eosinophilic infiltrate. The best management is:

a) Laser fulguration

b) Corticosteroids

c) Long term antibiotics

d) TUR of lesion

e) Cystectomy and diversion

A 17 year old boy has bright red urine after prolonged exercise. He has no history of GU disease and is otherwise asymptomatic. U/A collected revealrs 1+ proteinuria and >50 RBC/hpf. There are no casts. Renal u/s is normal. The next step is:

a) Cystoscopy

b) CT urogram

c) Urinary myoglobin

d) Urinary calcium/creatinine ratio

e) Urinalysis 72 hrs later

A six year old girl has a 1 week history of marked urinary frequency and urgency. Physical exam and urinalysis are normal. The next step should be:

a) Renal/bladder ultrasound

b) CMG

c) CBC with eosonphil count

d) VCUG

e) Observation

A 6 month old boy has microscopic hematuria. His calcium to creatinine ratio is 0.6. The best treatment is:

a) Double fluid intake

b) Thiazides

c) ACE inhibitor

d) Potassium citrate

e) Observation

Prenatal u/s of a 22 week old male fetus shows bilateral hydroureteronephrosis and a thick walled bladder. The finding that supports early intervention is:

a) Hyperechoic kidneys

b) Oligohydramnios

c) Renal cortical cysts

d) Abnormal urine electrolytes

e) Perinephric urinoma

A 3 year old boy has a fever of 100, WBC of 17,000 and prurulent umbilical drainage. A pelvic u/s reveals a thickened structure between the dome of the bladder and the umbilicus which containes a central 2 cm complex mass. There is no obstruction or extravasation on a VCUG. The next step is antbiotics followed by

a) Cystoscopy

b) Incision,drainage and packing

c) Excision of urachus and partial cystectomy

d) Delayed excision of urachus

e) Chemical sclerosis of the sinus tract

A ten-year old girl with a history of bilateral high grade VUR was treated with bilateral cross-trigonal ureteral reimplantation surgery at 2 years of age. She is evaluated for gross hematuria and found to have nephrogenic adenomas of the bladder that are resected. The next step is:

a) Reassurance

b) Antibiotic suppression

c) Intravesical BCG

d) Laser ablation

e) Partial cystectomy

Antenatally discovered VUR differs from VUR discovered in older symptomatic children, because in antenatal VUR::

a) Grades I-IV show mid-kidney scars

b) Function of the refluxing kidney is diminished

c) Severe damage is mostly in males

d) Kidney damage is usually left-sided

e) Resolution rates are slower

A 6 year old boy has persistent asymptomatic microhematuria. Urine calcium/creatinine ratio, ASO, and C3 complement are normal. Renal and bladder ultrasound is normal. The next step is:

a) VCUG

b) Cystoscopy

c) Non-contrast CT scan

d) Urinalysis and blood pressure measurement in one year

e) Urine cytology

A cutaneous vesicostomy in infants results in::

a) Continent urinary diversion

b) Effective drainage of the bladder

c) Bladder dysfunction from disuse

d) Increased risk of symptomatic UTI

e) Inability to void per urethra

A boy with high imperforate anus undergoes a diverting colostomy. Over the next 6 months he has recurrent UTIs. U/S shows a normal spine and kidneys. The most likely cause for these UTIs is:

a) Rectourethral fistula

b) Rectovesical fistula

c) VUR

d) Neuorogenic bladder

e) Urethral stricture

Persistent urinary incontinence following resection of posterior urethral valves is most frequently due to:

a) Detrusor instability

b) VUR

c) Non-compliant bladder

d) Incompetent bladder neck

e) Damaged external urethral sphincter

A 10m day old boy with a transverse colostomy performed for a high imperforate anus has a serum chloride of 115, sodium of 145, potassium of 4.5, and C02 of 17. The most likely explanation for these findings is:

a) Sepsis

b) Renal dysplasia

c) Severe hydronephrosis

d) Neuorogenic bladder dysfunction

e) Electrolyte absorption from large bowel

A 4 year old with PUV has a vesicostomy. Serum Cr is 0.6. Ultrasound shows minimal hydronpehrosis. UDS shows a bladder capacity of 30cc with a pressure of 14 cm H20 when leakage occurs from the vesicostomy with no reflux. Undiversion is considered. The best management is resection of the PUV and:

a) Ileal augmentation cystoplasty

b) Ileal augmentation with appendicovesicostomy

c) Bladder cycling via the vesicostomy

d) Primary closure of the vesicostomy

e) Autoaugmentation cystoplasty

Compared to other children with bladder exstrophy, those who undergo prepubertal intestinal bladder augmentation are:

a) More likely to have nocturnal enuresis

b) Less likely to have bladder calculi

c) More likely to be short in stature

d) More likely to develop TCC of the bladder

e) More likely to develop vitamin B and K deficiencies

A 25 year old man with a history of PUV will most likely have:

a) Arteriogenic erectile dysfunction

b) Normal sperm count

c) Normal semen quality

d) Neuorgenic erectile dysfunction

e) Abnormal sertoli cell function

A 2 year old boy with stranguria develops gross hematuria. Urine culture is negative. Ultrasound shows normal kidneys and a polypoid mass at the base of the bladder. The next step is:

a) CT Scan with contrast

b) IVP

c) VCUG

d) MRI scan with gadollinium

e) cystoscopy

A 38 week gestation newborn with PUV has a serum creatinine of 1.8. That level:

a) Initially falls with a rapid rise in GFR

b) Is an ominous predictor of future renal function

c) Will decrease with completion of nephrogenesis

d) Is not reflective of the degree of renal function impairment

e) Will result in increased active sodium absorption from the descending limb of the loop of Henle

A 7 year old boy with cloacal exstrophy undergoes gastric augmentation with a catheterizable stoma and closure of the bladder neck. He was initially continent but now has diurnal leakage despite regular catheterization and saline irrigation. The most likely causeof the incontinence is:

a) Incompetent catheterizable stoma

b) Fistula at site of bladder neck closure

c) Incomplete reservoir emptying

d) High-amplitude rhythmic contractions

e) Decreased reservoir capacity