PEDI GU REVIEW lower tract i


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  • First diagnosed in 1970 with prenatal u/s of polycystic kidney by William Garrett in a 31 week GA fetus Mean age at diagnosis of uropathy is 24 weeks Urine Formation begins at 8 weeks and continues through gestation; by 22 weeks volume of amniotic fluid is maintained by fetal urine production
  • -Urethral Atresia: complete obstruction caused by a membrane that is located at the distal end of the prostatic urethra -Incompatible with life unless alternative ccommunication develops: patent urachus, vesico-amniotic fistula, fistula to the rectum -Phenotypically similar to prune belly -Sagittal level III ultrasound at 15 weeks of gestation shows classic “keyhole” sign -Combined antegrade via vesicostomy/retrograde urethrogram reveals 2 cm. atretic urethral segment @ 14 months; Subsequently serial dilations and BVUR with later renal transplantation
  • -2 nd trimester=13 th -27 th weeks -Enlarged Fetal bladder with thickening, Echogenic renal parenchyma, Bilateral hydro and decreased amniotic fluid volume, extrarenal fluid collections such abdominal ascites or perirenal urinoma -Perinephric urinoma associated with echogenic kidneys and posterior urethral valves
  • -Dr. Hampton Young was creditted for the first clear description and classification of PUV in 1919 -Type I is an obstructing membrane that arises from the posterior and inferior edge of the veru and radiates distally towards the membranous urethra, inserting anteriorly near the proximal margin of the membranous urethra -Type II urethral valves were initially described as folds radiating in a cranial direction from the veru to the posterolateral aspect of the bladder neck; these are not obstructive -Type III valves are incomplete dissolution of the urogenital membrane. The obstructing membranes are situated distally to the veru at the level of the membranous urethra
  • -Type I valves make up more than 95% of the lesions whereas Type III make up the remainder -During voiding the fused anterior portion of the membrane bulges into the membranous urethra leaving only a narrow opening that is compressed along the posterior wall of the urethra
  • -Abnormal migration of the terminal ends of the wolffian duct orifices -Normally remains lateral and posterior but abnormal migration places it anteriorly -Disputed theories on development; some suggest they are the remnant of the urogenital membrane
  • 93% are boys, mostly with a history of PUV Leading cause of mortality in obstructive uropathy-Lung development begins at 3-4 weeks and ends near week 24; Reduced external pressure leads to lung fluid being draw freely into amniotic space and therefore reducing the stenting pressure in the lung and impairing normal growth High mortality rate is generally not relfected in postnatal series and represents the “hidden mortality” since these infants do not survive postnatally before transfer to a pediatric specialty center for treatment Late-Onset Oligo >30 weeks not associated with pulmonary insufficiency Fetuses with lower urinary tract obstruction and oligo represent the most severe end of the obstructive uropathy spectrum and are the fetuses one would expect to idenify earliest in gestation and to be the highest risk of pulmonary hypoplasia and renal dysplasis - -
  • -Bladder dysfunction includes 1)myogenic failure with overflow incontinence 2) Detrusor hyper-reflexia 3) Bladder hypotonia in 31% -Renal Dysfunction: GOS 10 year follow-up: 10% died of RF, 15% ESRD, 6% CRI with progression independent of type of primary surgical treatment Late onset RF  metabolic demands of puberty, hyperfiltration injury with decreased functional renal mass , polyuria with impaired urinary concentrating capacity (RF increases with age 6.5% at 5; 25% at 10, 50% at 5. -Decreased libido/potency could be due to CRF and HD; - EJ  48% c/o slow/dry ejaculation and 10/21 patients studied had oligospermia which could be due to failure of dilated posterior urethra to generate enough pressure -Infertility could be associated with cryptorhidism which is in 12% of these patients
  • Mention speaker at BWH with different incidence Mention speaker at BWH with different incidence m/f almost equal; wilms tumor rate may be higher, no known risk factors ?maternal progestin use
  • Usually advocate c section because of prolapse issue. LOTS of variation
  • PEDI GU REVIEW lower tract i

    1. 1. CONGENITAL DISORDERS OF THE LOWER URINARY TRACT Pediatric GU Review UCSD Pediatric Urology George 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)
    2. 2. Vesicoureteral Reflux
    3. 3. Introduction <ul><li>Primary VUR </li></ul><ul><ul><li>Abnormal anatomy of the UVJ </li></ul></ul><ul><li>Secondary VUR </li></ul><ul><ul><li>Acquired condition from increased intravesical pressure </li></ul></ul><ul><ul><ul><li>Neurogenic bladder dysfunction </li></ul></ul></ul><ul><ul><ul><li>Non-neurogenic bladder dysfunction </li></ul></ul></ul><ul><ul><ul><li>Outlet obstruction </li></ul></ul></ul><ul><li>VUR is really a marker of a heterogenous condition of the whole urinary tract versus a simple causal relationship </li></ul>
    4. 4. History <ul><li>Galen and Leonardo da Vinci were first to propose that normal UVJ allowed unidirectional flow </li></ul><ul><li>Hutch observed that VUR and PN might be related in parapalegic patients </li></ul>
    5. 5. Anatomical Features <ul><li>Ureter enters bladder wall at an oblique intramural path </li></ul><ul><ul><li>Flap-valve mechanism causing intramural and submucocal ureter to be comrpessed with filling against detrusor backing </li></ul></ul><ul><li>UVJ mechanism is a passive and active process </li></ul><ul><ul><li>Extension of ureteral longitudinal muscles into the trigone (Bell’s muscle) </li></ul></ul><ul><ul><ul><li>Down to veru in males and dorsal urethral in females </li></ul></ul></ul><ul><ul><ul><li>At trigone these muscle fibers extend fanwise and at midline intermingle with contralateral ureter </li></ul></ul></ul><ul><ul><li>Middle circular ureteral muscle is not seen beyond the ureteral hiatus </li></ul></ul><ul><ul><li>Waldeyer’s sheath =fibromuscular sheath that anchors the ureter within the hiatus </li></ul></ul>
    6. 6. Anatomical Features <ul><li>Ureteral muscles extend into trigone and are innervated by noradrenergic receptors similar to vas </li></ul><ul><li>Ureteral maldevelopment may be tied together with abnormal trigonal development </li></ul><ul><li>Vit A signalling deficient mice demonstrate abnormal trigonal development with high incidence of distal ureteral anomalies </li></ul>
    7. 7. Anatomical Features <ul><li>Functional integration of the ureter and trigone is important to normal UVJ mechanism </li></ul><ul><ul><li>Tanagho  unilateral sympathectomy in cats induces VUR </li></ul></ul><ul><ul><li>Increasing trigonal tone/contractility (electrical stim, bladder dist, NE) all resulted in functional occlusion of the ureteral orifices </li></ul></ul>
    8. 8. Anatomical Features <ul><li>Summary </li></ul><ul><ul><li>Natural tone of ureteral muscle provides a mild passive closure of the intramural ureter </li></ul></ul><ul><ul><li>With filling, there is a progressive obliquity of the intramural/submucosal ureter  flap-valve </li></ul></ul><ul><ul><li>Micturition stimulates trigonal tone, preventing lateral displacement of the orifices </li></ul></ul>
    9. 9. Anatomical Features <ul><li>Intravesical ureter lengthens with age </li></ul><ul><ul><li>Neonates average length=0.5 cm, adults=1.3 cm </li></ul></ul><ul><ul><li>Mature length at 10-12 yrs </li></ul></ul><ul><ul><li>Length varies with reflux in other species </li></ul></ul><ul><li>Trigonal competency is important to prevent lateral displacement </li></ul><ul><ul><li>Displacement occurs from abnormal ureteric bud origin along the developing mesonephric duct during metanephric development </li></ul></ul><ul><ul><li>Mackie and Stephens found a correlation between lateral displacement of the UO and degree of renal dysplasia and hypoplasia </li></ul></ul><ul><ul><li>VUR can be a primary trigonal disorder </li></ul></ul><ul><ul><ul><li>Trigone develops as a result of the common nephric duct and the distal ureter incorporating into the developing bladder </li></ul></ul></ul>
    10. 10. Anatomical Features <ul><li>Endoscopic observations </li></ul><ul><ul><li>Ratio of submucosal tunnel length to ureteral diameter </li></ul></ul><ul><ul><ul><li>Paquin  normal =5:1 children with VUR 1.4:1 </li></ul></ul></ul><ul><ul><ul><li>Lyon et al: 4 basic orifices </li></ul></ul></ul><ul><ul><ul><ul><li>Cone 4% VUR </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Stadium 28% </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Horseshoe 83% </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Golf-hole 100% </li></ul></ul></ul></ul><ul><ul><ul><li>Lateral pillar defect (between horseshoe and golf) </li></ul></ul></ul>
    11. 11. Demographics <ul><li>True incidence difficult to ascertain but probably 0.4-1.8% </li></ul><ul><li>Incidence of VUR in children with UTI=30-50% </li></ul><ul><ul><li>Female predominance </li></ul></ul><ul><li>Antenatal hydronephrosis  25% VUR </li></ul><ul><ul><li>Infant male predominance </li></ul></ul><ul><li>Boy present at younger ages with higher grades (secondary to transient urethral obstruction and high intravesical pressures) </li></ul><ul><ul><li>Higher voiding pressures and small functional capacity </li></ul></ul><ul><li>Sibling VUR as high as 40-50% </li></ul><ul><ul><li>Dominant inheritance pattern with 60% parent to child </li></ul></ul><ul><ul><li>Asian and African Americans have a lower rate vs caucasians (3.4 to 1) </li></ul></ul>
    12. 12. Primary vs Secondary <ul><li>Patients presenting with VUR must be managed with global view </li></ul><ul><ul><li>UVJ mechanism </li></ul></ul><ul><ul><li>Bladder dysfunction </li></ul></ul><ul><ul><li>Predisposition to UTI </li></ul></ul><ul><ul><li>Renal Anomalies </li></ul></ul><ul><li>Secondary VUR from bladder anomalies is common and BOO </li></ul><ul><ul><li>Chronic elevation of bladder storage pressure and distortion at UVJ (paraureteral diverticulum) can cause secondary VUR </li></ul></ul><ul><ul><li>Most common anatomic cause of secondary VUR is PUV </li></ul></ul><ul><ul><li>Anatomic obstruction is rare in females except obstructing ureterocele </li></ul></ul><ul><ul><li>Altered bladder function with poor compliance and dyssynergy can lead to increased intravesical pressures </li></ul></ul><ul><ul><ul><li>If pressure >40cm incidence of VUR is >80% with myelo/neurogenic bladders </li></ul></ul></ul>
    13. 13. UTI and VUR <ul><li>Lower urinary tract inflammation may affect the mildly deficient UVJ </li></ul><ul><li>New VUR was reported in 6/7 piglets after E. Coli infection </li></ul><ul><ul><li>Some E. coli strains directly affect the urinary tract smooth muscle </li></ul></ul><ul><ul><li>Bacterial endotoxin has been found to inhibit alpha-adrenergic receptors of the trigone musculature </li></ul></ul><ul><li>Gross and Lebowitz surveyed 600 children undergoing cystographic eval for VUR and found no evidence of increased VUR with positive urine culture </li></ul><ul><ul><li>VUR not caused by UTI </li></ul></ul>
    14. 14. Lower urinary tract function <ul><li>Infantile bladder has higher voiding pressures from imperfect coordination between detrusor contraction and sphincter relaxation (disappears at 2 yrs) </li></ul><ul><ul><li>Lack of integration between bladder/sphincter/brainstem/cortical centers </li></ul></ul><ul><ul><li>Versus normal infants, those with VUR have higher voiding pressures, and abnl bladder function even without UTI </li></ul></ul><ul><ul><li>Discoordinated voiding pattern most common UDS parameter in male/females with VUR </li></ul></ul><ul><ul><li>Bladder hypercontractility in infant boys with VUR </li></ul></ul><ul><ul><li>Transient urethral obstruction may be the cause of infantile VUR </li></ul></ul><ul><ul><li>Abnormal UDS may imply a global maldevelopment of the trigone and proximal urethral mechanism </li></ul></ul>
    15. 15. Lower urinary tract function <ul><li>Pediatric voiding dysfunction </li></ul><ul><ul><li>Frequency/urgency/enuresis are manifestations of detrusor instability and/or DSD </li></ul></ul><ul><ul><li>360 dysfunctional voiders  60% with UTI and 20% VUR </li></ul></ul><ul><ul><li>International Reflux Study  presence of dysfunctional voiding in 18% of VUR with UTI </li></ul></ul><ul><ul><li>UDS has shown up to 60% of children with VUR have DI </li></ul></ul><ul><ul><li>DI may exaggerate VUR and can cause delayed resolution </li></ul></ul><ul><ul><ul><li>Treatment with anticholinergics can facilitate resolution </li></ul></ul></ul>
    16. 16. Non-Surgical Management of VUR
    17. 17. Non-Surgical Therapy <ul><li>Antibiotic prophylaxis </li></ul><ul><ul><li>Daily </li></ul></ul><ul><ul><li>Intermittent (I.e. treatment of UTIs) </li></ul></ul><ul><li>Bladder training </li></ul><ul><li>Treatment of voiding dysfunction </li></ul><ul><li>Periodic assessment of reflux and child well-being </li></ul>
    18. 18. Reflux Resolution <ul><li>Higher likelihood in younger children and those with lower reflux grade </li></ul><ul><li>IRS Study </li></ul><ul><ul><li>At 5 yrs </li></ul></ul><ul><ul><ul><li>92% Grade 1 </li></ul></ul></ul><ul><ul><ul><li>81% Grade 2 </li></ul></ul></ul><ul><ul><ul><li>Grade 3: resolution highest for children<2 and unilateral (70%) whereas bilateral reflux >5 (12.5%) </li></ul></ul></ul><ul><ul><ul><li>Grade 4: Unilateral 58% at 5 yrs; 10% with bilateral </li></ul></ul></ul><ul><li>Beyond 5 years, limited data </li></ul>
    19. 19. Antibiotic Prophylaxis <ul><li>Treating each symptomatic UTI </li></ul><ul><ul><li>In children with existing scars, 66% developed new scars </li></ul></ul><ul><li>Observation </li></ul><ul><ul><li>50% of girls had a UTI during observation and even 23% with Grade 1 had scarring </li></ul></ul><ul><li>Girls more likely to develop renal scars, scars could occur at any age, and children with mild reflux are susceptible to scar formation </li></ul>
    20. 20. Antibiotic Prophylaxis <ul><li>Prospective eval of 203 children; 3% developed scars </li></ul><ul><li>Skoog et al-->545 children with Grade 1-4 on prophylaxis only 0.5 % scarring </li></ul><ul><li>Ideal antibiotic still uncertain </li></ul>
    21. 21. Breakthrough UTI <ul><li>Girls with reflux and voiding dysfunction are most likely to develop breakthrough UTI </li></ul><ul><li>In boys with reflux, uncircumcised are most likely </li></ul><ul><li>In both sexes, prior renal scarring is the greatest risk for breakthrough UTI </li></ul><ul><li>A breakthrough UTI is an indication of failure and surgical therapy should be considered </li></ul>
    22. 22. Expectant Management <ul><li>In selected patients it seems reasonable to dc prophylaxis </li></ul><ul><li>Normal voiding pattern and age of 8.6 yrs </li></ul><ul><ul><li>Grades 1-4 </li></ul></ul><ul><ul><li>UTI in 5/40 girls and 1/11 boys (Grade 3 VUR) </li></ul></ul><ul><li>Expectant management may be employed if voiding pattern is normal </li></ul><ul><li>It is unresolved whether sibling reflux or screening after MCKD or renal agenesis have a lower risk of reflux-related complications </li></ul><ul><li>Patients should understand risk of PN with pregnancy and sexual activity </li></ul>
    23. 23. Expectant Management <ul><li>115 adult women with VUR discovered after 16 years of age; 73% with refluxing kidneys had reflux nephropathy </li></ul><ul><li>Women with VUR have a higher risk of febrile UTI versus those without VUR </li></ul><ul><li>Persistent VUR in females should be corrected </li></ul>
    24. 24. Bladder Training <ul><li>Reduce the likelihood of developing UTI by lowering voiding pressure </li></ul><ul><li>Infrequent voiding, DSD and constipation can increase the likelihood of bacteriuria </li></ul>
    25. 25. Voiding Dysfunction <ul><li>VUR and dysfunctional elimination have a higher rate of breakthrough UTI </li></ul><ul><li>Up to 4x increase </li></ul><ul><li>Longer time to spontaneous resolution </li></ul><ul><li>Unsuccessful surgical outcomes with voiding dysfunction </li></ul><ul><li>High VD symptom score with training and decreased score predicted VUR improvement </li></ul>
    26. 26. Anticholinergic Therapy <ul><li>Children with overactive bladder may be treated with combination of anticholinergic and timed voiding </li></ul><ul><ul><li>Children experience incontinence 24hrs/day </li></ul></ul><ul><li>Increased spontaneous VUR resolution with anticholinergics </li></ul>
    27. 27. Alpha-Blocker Therapy <ul><li>Pelvic floor overactivity and significant PVR </li></ul><ul><ul><li>Doxazosin may reduce incontinence </li></ul></ul>
    28. 28. Biofeedback <ul><li>May be useful to establish a normal pattern of sphincteric relaxation and reduce anticholinergic need </li></ul><ul><li>Uroflow study, PVR check, 1 hr session with EMG probes </li></ul><ul><li>Children may have difficulty isolating external sphincter and may allow them to learn sphincteric relaxation </li></ul><ul><li>Pelvic floor exercises may help unstable bladder contractions </li></ul>
    29. 29. Biofeedback <ul><li>Utilize in children with symptoms of OAB that do not respond to anticholinergics </li></ul><ul><li>Or, as alternative to anticholinergics </li></ul>
    30. 30. Follow-Up <ul><li>Screening for UTI </li></ul><ul><ul><li>No evidence that periodic screening of asymptomatic children with VUR is beneficial </li></ul></ul><ul><li>Cystography </li></ul><ul><ul><li>Every 12-18 months </li></ul></ul><ul><ul><li>Non-compliance with follow-up visits up to 33% </li></ul></ul><ul><ul><li>Alternative is every year for mild reflux and every 3 years with severe reflux </li></ul></ul><ul><ul><li>Give oral or nasal midazolam for VCUG although 31% of children still remember </li></ul></ul>
    31. 31. Follow-Up <ul><li>Upper-tract Imaging </li></ul><ul><ul><li>No standards </li></ul></ul><ul><ul><ul><li>If no febrile UTI, should be no change in upper tract </li></ul></ul></ul><ul><li>Other assessment </li></ul><ul><ul><li>Follow-up should be annually for height/weight </li></ul></ul><ul><ul><li>BP check if presence of renal scarring </li></ul></ul>
    32. 32. Fetal Obstructive Uropathy <ul><li>Obstruction of urinary system </li></ul><ul><ul><li>Oligo/anhydramnios </li></ul></ul><ul><ul><li>Bilateral Hydronephrosis </li></ul></ul><ul><ul><li>Distended Bladder </li></ul></ul><ul><li>Timing of diagnosis </li></ul><ul><ul><li>9% of uropathies apparent at 17 weeks </li></ul></ul><ul><ul><li>90% apparent at 33 weeks </li></ul></ul>Cusick EL et al. Mortality after an antenatal diagnosis of fetal uropathy. J Pediatr Surg 1995; 30:463-466.
    33. 33. Lower Tract Obstruction <ul><li>Urethral Atresia </li></ul><ul><ul><li>Bladder Distension </li></ul></ul><ul><ul><li>Vesicoureteral reflux </li></ul></ul><ul><ul><li>Hydronephrosis </li></ul></ul><ul><ul><li>Renal Dysplasia </li></ul></ul><ul><ul><li>95% Mortality </li></ul></ul>Herndon et al. Early Second Trimester Intervention In A Surviving Infant With Postnatally Diagnosed Urethral Atresia. J Urol. 168 pp 1532-1533 October 2002   
    34. 34. <ul><li>Incidence 1 in 5000 to 1 in 8000 </li></ul><ul><li>25-50% seen in neonatal period </li></ul><ul><li>50-70% present within 1 st year </li></ul><ul><li>Hydronephrosis is present in 90% of infants with valves </li></ul>Posterior Urethral Valves Agarwal, S. Urethral Valves. BJU International 84 pp 570-578 1999
    35. 35. <ul><li>Presentation </li></ul><ul><ul><li>Prenatal ultrasound in 2 nd trimester </li></ul></ul>Posterior Urethral Valves
    36. 36. <ul><li>Types </li></ul>Posterior Urethral Valves Gonzales, ET.. Posterior Urethral Valves and Other Urethral Abnormalities. Campbell’s Urology; 8 th Edition
    37. 37. Posterior Urethral Valves <ul><li>Type I valves: 95% of the lesions </li></ul><ul><li>Type III make up the remainder </li></ul>Gonzales, ET.. Posterior Urethral Valves and Other Urethral Abnormalities. Campbell’s Urology; 8 th Edition
    38. 38. <ul><li>Embryology </li></ul>Posterior Urethral Valves Gonzales, ET.. Posterior Urethral Valves and Other Urethral Abnormalities. Campbell’s Urology; 8 th Edition
    39. 39. <ul><li>1/3 of children with ESRD <4 yo have obstruction as the underlying cause </li></ul><ul><li>Pulmonary Hypoplasia from AF </li></ul><ul><li>95% mortality with early mid-gestational oligohydramnios </li></ul>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
    40. 40. Posterior Urethral Valves <ul><li>Mortality 45% with presentation at birth </li></ul>Courtesy of Drs. Cendron and Borer
    41. 41. <ul><li>Long term complications </li></ul><ul><ul><li>Bladder dysfunction: “Valve Bladder” </li></ul></ul><ul><ul><li>Renal dysfunction </li></ul></ul><ul><ul><ul><li>Late onset of renal failure </li></ul></ul></ul><ul><ul><li>Sexual function/fertility </li></ul></ul><ul><ul><ul><li>Decreased libido/potency </li></ul></ul></ul><ul><ul><ul><li>EJ dyfunction </li></ul></ul></ul><ul><ul><ul><li>Cryptorchidism </li></ul></ul></ul>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
    42. 42. Exstrophy
    43. 43. Epidemiology <ul><li>1/20,000 -1/40,000 live births </li></ul><ul><li>3.3/100,000 </li></ul><ul><li>Sibling: 1-3.6% risk </li></ul><ul><li>Child: 1.4% </li></ul><ul><li>M>F 1.5-2:1 </li></ul><ul><li>Controversial epdemiology </li></ul>Kiddoo et al, Urol Clin N Am 31:417-426, 2004
    44. 44. Epidemiology <ul><li>Multifactorial </li></ul><ul><li>18 case reports of familial exstrophy </li></ul><ul><li>1% sibling risk </li></ul><ul><li>1.4% child risk </li></ul><ul><li>Historically poor fertility – difficult to define family risk </li></ul>Grady et al, Urol Clin N Am 26(1)95-109, 1999
    45. 45. Epidemiology <ul><li>205/9,452,110 (2.15/100,00 live births) </li></ul><ul><li>Whites > non-whites </li></ul><ul><li>M=F </li></ul><ul><li>Increased prematurity, cleft palate, spina bifida, GI abnormalities </li></ul><ul><li>$10,000,000 annual expenditure </li></ul><ul><li>$62,302 per case </li></ul>Nelson et al, J Urol 174:1099-1102, 2005
    46. 46. Associated Anatomic Variation <ul><li>Shortened umbilical -anal distance </li></ul><ul><li>Widened inguinal canal </li></ul><ul><li>Bilateral reflux – absent submucosal tunnel </li></ul><ul><li>Pubic diastasis </li></ul><ul><li>Rectal prolapse </li></ul><ul><li>Duplex system </li></ul>Kiddoo et al, Urol Clin N Am 31:417-426, 2004
    47. 47. Bladder <ul><li>Varying capacity – up to 15-30 cc </li></ul><ul><li>Early changes to transitional epithelium </li></ul><ul><li>Polypoid change </li></ul><ul><li>Metaplasia  malignancy if untreated </li></ul><ul><li>? Poor intrinsic detrusor function </li></ul>Kiddoo et al, Urol Clin N Am 31:417-426, 2004
    48. 48. Male genitalia <ul><li>Short/wide penis </li></ul><ul><li>Short, deep corpora </li></ul><ul><li>Dorsal chordee </li></ul><ul><li>Lateral displacement of neurovascular bundles </li></ul><ul><li>Vas, SV, ducts usually normal </li></ul>
    49. 49. Classic Male Exstrophy
    50. 50. Classic Male Exstrophy Grady et al, Urol Clin N Am 26(1)95-109, 1999
    51. 51. Female genitalia <ul><li>Hemiclitoris </li></ul><ul><li>Narrow introitus </li></ul><ul><li>Anterior displacement of vaginal orifice </li></ul><ul><li>Duplicated vagina, uterus </li></ul><ul><li>Adnexal normal </li></ul><ul><li>Higher rates of vaginal/uterine prolapse </li></ul>Kiddoo et al, Urol Clin N Am 31:417-426, 2004
    52. 52. Embryology <ul><li>Persistence of cloacal membrane (proven wrong) </li></ul><ul><li>Failure of mesoderm to ingrow between ectoderm/endoderm of cloacal membran  rupture  exstrophy </li></ul><ul><li>Caudal displacement of genital tubercle </li></ul>Grady et al, Urol Clin N Am 26(1)95-109, 1999
    53. 53. Walsh, et al Campbells Urology p. 2139
    54. 54. Walsh, et al Campbells Urology p. 2138
    55. 55. Epidemiology Nelson et al, J Urol 174:1099-1102, 2005
    56. 56. Ultrasound <ul><li>Prenatal diagnosis possible after 20 weeks </li></ul><ul><li>High false negative rate </li></ul><ul><li>Absence of bladder filling, nl kidneys, low set umbilical cord, anterior abdominal mass </li></ul><ul><li>Ddx – omphalocele, gastrochisis </li></ul>Grady et al, Urol Clin N Am 26(1)95-109, 1999
    57. 57. Skeletal Defects <ul><li>Pubis diastasis </li></ul><ul><li>Shortened pubic rami </li></ul><ul><li>Retroverted acetabulum </li></ul><ul><li>External rotation of posterior pelvis </li></ul>Walsh, et al Campbells Urology p. 2141
    58. 58. Skeletal Defects <ul><li>Waddling gait </li></ul><ul><li>Gait defects usually improve without intervention </li></ul><ul><li>Osteotomy helps GU reconstruction </li></ul><ul><li>Osteotomy decreases osteoarthritis </li></ul>Walsh, et al, Campbells Urology p. 2141
    59. 59. Osteotomy Rationale <ul><li>Approximate symphysis allows tension free abdominal wall closure </li></ul><ul><li>Facilitate urethra placement deep in pelvis </li></ul><ul><li>Bring pelvic floor musculature medial, which aids in bladder neck suspension and continence </li></ul><ul><li>Minimize tension on perineal neurovascular bundle </li></ul><ul><li>Reduces risk for dital corporeal / glannular ischemia </li></ul>Walsh, et al, Campbells Urology p. 2141 Kiddoo et al, Urol Clin N Am 31:417-426, 2004
    60. 60. Initial Management <ul><li>Silk tie on umbilicus (clamps irritate mucosa) </li></ul><ul><li>Saran wrap </li></ul><ul><li>Change saran wrap with diaper changes; irrigate with saline </li></ul><ul><li>Keep moist! </li></ul>
    61. 61. Goals of Reconstruction Renal function Continence Decreased UTI Functional genitalia genitalia Cosmetic
    62. 62. Reconstruction <ul><li>1900’s urinary diversion (ie ureterosigmoidoscopy) preferred </li></ul><ul><li>Bladder is functional </li></ul><ul><ul><li>Normal detrusor activity </li></ul></ul><ul><ul><li>Normal muscarinic receptors </li></ul></ul><ul><ul><li>Sphincter can be functional and coordinated </li></ul></ul><ul><li>Improved surgical technique </li></ul>
    63. 63. Staged Reconstruction 0-72 hours 4-6 months 4-5 years I Primary closure; osteotomy; approximation of symphysis II Epispadias repair, urethroplasty, penile reconst. III Bladder neck reconstruction., reimplant
    64. 64. Exstrophy Practice Patterns Nelson, et al, J Urol 174(3): 1099-1102, 2005
    65. 65. Primary Closure <ul><li>Typically within 72 hours </li></ul><ul><li>If bladder too small for closure  wait 4-6 months for template to grow </li></ul><ul><li>Joint orthopedic procedure - osteotomy </li></ul>
    66. 66. Primary Closure
    67. 67. Primary Closure
    68. 68. Primary Closure
    69. 69. Primary Closure
    70. 70. Primary Closure
    71. 71. Primary Closure
    72. 72. Primary Closure
    73. 73. Primary Closure
    74. 74. Primary Closure
    75. 75. Primary Closure
    76. 76. Primary Closure
    77. 77. Staged Reconstruction 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 I Primary closure; osteotomy; approximation of symphysis II Epispadias repair, urethroplasty, penile reconst. III Bladder neck reconstruction., reimplant
    78. 78. Outcomes <ul><li>Continence Definitions </li></ul><ul><ul><li>Continent : daytime dry for > 3 hrs, no stress or nocturnal incontinence </li></ul></ul><ul><ul><li>Partially continent : daytime dry 1-3 hours, occasional stress or nocturnal incontinence </li></ul></ul><ul><ul><li>Incontinent : daytime dry < 1 hour, more than occasional stress or nocturnal incontinence </li></ul></ul>Shaw, et al, J Urol 172:1450-1453, 2004
    79. 79. Results <ul><li>43/48 (90%) continent </li></ul><ul><li>4/48 (8%) partially continent </li></ul><ul><li>1/48 (2%) incontinent </li></ul><ul><li>13/38 (34%) continent after BN reconstruction alone (trend towards higher volumes) </li></ul><ul><li>6/6 (100%) later augmented became continent </li></ul><ul><li>4/9 redo BNR incontinent, 5/9 redo with augment became continent </li></ul>Shaw, et al, J Urol 172:1450-1453, 2004
    80. 80. Results <ul><li>10/43 (23%) continent patients with urethral voiding only </li></ul><ul><li>8/43 ( %) continent patients CIC + partial void per urethra </li></ul><ul><li>24/43 ( %) exclusively CIC </li></ul>Shaw, et al, J Urol 172:1450-1453, 2004
    81. 81. Conclusions <ul><li>Poor overall continence rates </li></ul><ul><li>Often requires augmentation </li></ul><ul><li>Redo bladder neck reconstruction with poor outcomes </li></ul>Shaw, et al, J Urol 172:1450-1453, 2004
    82. 82. Fertility <ul><li>Decreased overall fertility in men </li></ul><ul><li>10X increase in cyptorchidism (controversial) </li></ul><ul><li>Women with normal sexual function and fertility </li></ul><ul><li>C-section advocated (risk of uterine prolapse) </li></ul>Kiddoo et al, Urol Clin N Am 31:417-426, 2004
    83. 83. QUESTIONS
    84. 84. <ul><li>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: </li></ul><ul><li>Transplant into the existing ileal conduit </li></ul><ul><li>Bladder augmentation before transplant </li></ul><ul><li>Transplant into native bladder </li></ul><ul><li>Neobladder construction before transplant </li></ul><ul><li>Indiana pouch construction before transplant </li></ul>
    85. 85. <ul><li>A 14 year old boy with incontinence had posterior urethral valves resected as a neonate. The most likely etiology for his incontinence is: </li></ul><ul><li>Occult neurogenic bladder </li></ul><ul><li>Sphincteric injury </li></ul><ul><li>Hyperreflexic bladder </li></ul><ul><li>Hypertonic bladder </li></ul><ul><li>Myogenic failure </li></ul>
    86. 86. <ul><li>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: </li></ul><ul><li>Observation </li></ul><ul><li>Cannulation and injection of contrast </li></ul><ul><li>VCUG </li></ul><ul><li>Closure of fistula </li></ul><ul><li>Urethral catheter drainage </li></ul>
    87. 87. <ul><li>In a child with febrile UTI, the antimicrobial drug that achieves therapeutic levels in the urine but not in the tissue </li></ul><ul><li>Ampicillin </li></ul><ul><li>Gentamicin </li></ul><ul><li>Nitrofurantoin </li></ul><ul><li>Trimethoprim </li></ul><ul><li>Cephalexin </li></ul>
    88. 88. <ul><li>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: </li></ul><ul><li>Continent urinary diversion </li></ul><ul><li>Augmentation cystoplasty </li></ul><ul><li>Bladder neck reconstruction </li></ul><ul><li>Injection of collagen at the bladder neck </li></ul><ul><li>Bladder neck reconstruction and augmentation cystoplasty </li></ul>
    89. 89. <ul><li>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: </li></ul><ul><li>Staged urethroplasty </li></ul><ul><li>Pedicle-flap urethroplasty </li></ul><ul><li>Repeat DVIU </li></ul><ul><li>Primary excision and end to end anastamosis </li></ul><ul><li>Urethral dilation with CIC </li></ul>
    90. 90. <ul><li>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: </li></ul><ul><li>Laser fulguration </li></ul><ul><li>Corticosteroids </li></ul><ul><li>Long term antibiotics </li></ul><ul><li>TUR of lesion </li></ul><ul><li>Cystectomy and diversion </li></ul>
    91. 91. <ul><li>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: </li></ul><ul><li>Cystoscopy </li></ul><ul><li>CT urogram </li></ul><ul><li>Urinary myoglobin </li></ul><ul><li>Urinary calcium/creatinine ratio </li></ul><ul><li>Urinalysis 72 hrs later </li></ul>
    92. 92. <ul><li>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: </li></ul><ul><li>Renal/bladder ultrasound </li></ul><ul><li>CMG </li></ul><ul><li>CBC with eosonphil count </li></ul><ul><li>VCUG </li></ul><ul><li>Observation </li></ul>
    93. 93. <ul><li>A 6 month old boy has microscopic hematuria. His calcium to creatinine ratio is 0.6. The best treatment is: </li></ul><ul><li>Double fluid intake </li></ul><ul><li>Thiazides </li></ul><ul><li>ACE inhibitor </li></ul><ul><li>Potassium citrate </li></ul><ul><li>Observation </li></ul>
    94. 94. <ul><li>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: </li></ul><ul><li>Hyperechoic kidneys </li></ul><ul><li>Oligohydramnios </li></ul><ul><li>Renal cortical cysts </li></ul><ul><li>Abnormal urine electrolytes </li></ul><ul><li>Perinephric urinoma </li></ul>
    95. 95. <ul><li>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 </li></ul><ul><li>Cystoscopy </li></ul><ul><li>Incision,drainage and packing </li></ul><ul><li>Excision of urachus and partial cystectomy </li></ul><ul><li>Delayed excision of urachus </li></ul><ul><li>Chemical sclerosis of the sinus tract </li></ul>
    96. 96. <ul><li>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: </li></ul><ul><li>Reassurance </li></ul><ul><li>Antibiotic suppression </li></ul><ul><li>Intravesical BCG </li></ul><ul><li>Laser ablation </li></ul><ul><li>Partial cystectomy </li></ul>
    97. 97. <ul><li>Antenatally discovered VUR differs from VUR discovered in older symptomatic children, because in antenatal VUR:: </li></ul><ul><li>Grades I-IV show mid-kidney scars </li></ul><ul><li>Function of the refluxing kidney is diminished </li></ul><ul><li>Severe damage is mostly in males </li></ul><ul><li>Kidney damage is usually left-sided </li></ul><ul><li>Resolution rates are slower </li></ul>
    98. 98. <ul><li>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: </li></ul><ul><li>VCUG </li></ul><ul><li>Cystoscopy </li></ul><ul><li>Non-contrast CT scan </li></ul><ul><li>Urinalysis and blood pressure measurement in one year </li></ul><ul><li>Urine cytology </li></ul>
    99. 99. <ul><li>A cutaneous vesicostomy in infants results in:: </li></ul><ul><li>Continent urinary diversion </li></ul><ul><li>Effective drainage of the bladder </li></ul><ul><li>Bladder dysfunction from disuse </li></ul><ul><li>Increased risk of symptomatic UTI </li></ul><ul><li>Inability to void per urethra </li></ul>
    100. 100. <ul><li>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: </li></ul><ul><li>Rectourethral fistula </li></ul><ul><li>Rectovesical fistula </li></ul><ul><li>VUR </li></ul><ul><li>Neuorogenic bladder </li></ul><ul><li>Urethral stricture </li></ul>
    101. 101. <ul><li>Persistent urinary incontinence following resection of posterior urethral valves is most frequently due to: </li></ul><ul><li>Detrusor instability </li></ul><ul><li>VUR </li></ul><ul><li>Non-compliant bladder </li></ul><ul><li>Incompetent bladder neck </li></ul><ul><li>Damaged external urethral sphincter </li></ul>
    102. 102. <ul><li>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: </li></ul><ul><li>Sepsis </li></ul><ul><li>Renal dysplasia </li></ul><ul><li>Severe hydronephrosis </li></ul><ul><li>Neuorogenic bladder dysfunction </li></ul><ul><li>Electrolyte absorption from large bowel </li></ul>
    103. 103. <ul><li>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: </li></ul><ul><li>Ileal augmentation cystoplasty </li></ul><ul><li>Ileal augmentation with appendicovesicostomy </li></ul><ul><li>Bladder cycling via the vesicostomy </li></ul><ul><li>Primary closure of the vesicostomy </li></ul><ul><li>Autoaugmentation cystoplasty </li></ul>
    104. 104. <ul><li>Compared to other children with bladder exstrophy, those who undergo prepubertal intestinal bladder augmentation are: </li></ul><ul><li>More likely to have nocturnal enuresis </li></ul><ul><li>Less likely to have bladder calculi </li></ul><ul><li>More likely to be short in stature </li></ul><ul><li>More likely to develop TCC of the bladder </li></ul><ul><li>More likely to develop vitamin B and K deficiencies </li></ul>
    105. 105. <ul><li>A 25 year old man with a history of PUV will most likely have: </li></ul><ul><li>Arteriogenic erectile dysfunction </li></ul><ul><li>Normal sperm count </li></ul><ul><li>Normal semen quality </li></ul><ul><li>Neuorgenic erectile dysfunction </li></ul><ul><li>Abnormal sertoli cell function </li></ul>
    106. 106. <ul><li>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: </li></ul><ul><li>CT Scan with contrast </li></ul><ul><li>IVP </li></ul><ul><li>VCUG </li></ul><ul><li>MRI scan with gadollinium </li></ul><ul><li>cystoscopy </li></ul>
    107. 107. <ul><li>A 38 week gestation newborn with PUV has a serum creatinine of 1.8. That level: </li></ul><ul><li>Initially falls with a rapid rise in GFR </li></ul><ul><li>Is an ominous predictor of future renal function </li></ul><ul><li>Will decrease with completion of nephrogenesis </li></ul><ul><li>Is not reflective of the degree of renal function impairment </li></ul><ul><li>Will result in increased active sodium absorption from the descending limb of the loop of Henle </li></ul>
    108. 108. <ul><li>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: </li></ul><ul><li>Incompetent catheterizable stoma </li></ul><ul><li>Fistula at site of bladder neck closure </li></ul><ul><li>Incomplete reservoir emptying </li></ul><ul><li>High-amplitude rhythmic contractions </li></ul><ul><li>Decreased reservoir capacity </li></ul>