526 the prostatic utricle: an under-recognized cause of morbidity in boys with both hypospadias and...
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526THE PROSTATIC UTRICLE: AN UNDER-RECOGNIZED CAUSEOF MORBIDITY IN BOYS WITH BOTH HYPOSPADIAS ANDNORMAL EXTERNAL GENITALIA
Stanley Kogan*, Bronx, NY
INTRODUCTION AND OBJECTIVES: Pediatric presentationsof a prostatic utricle have received only scant attention. Though recog-nized with increased frequency in boys with hypospadias little is de-scribed about incidence and potential morbidity in boys with normalexternal genitalia. Our experience with symptomatic utricles in boyswith both situations led us to review the frequency, causes for morbidityand indications and results of surgical repair.
METHODS: Initial retrospective review of 64 patients with hy-pospadias seen between 2003-2006 was done to determine frequencyof lower urinary tract studies (VCUG and/or cystoscopy) and utricleidentification. Children with genetic disorders of sexual differentiationwere excluded. This initial cohort was extended to a larger group withhypospadias, also identifying a group of 23 boys with normal externalgenitalia presenting with various signs and symptoms who were foundto have an unsuspected utricle. Symptomatology was the indication forevaluation in the latter group contrasting with the hypospadias groupmostly investigated because of hypospadias presence alone. Method-ology for identification and choices of surgical treatment and outcomeswere reviewed.
RESULTS: Of 64 patients only 25 (37.5%) underwent an inves-tigative study, mostly because of symptomatic urinary infection orbacteriuria. Utricle frequency in this group was 9.4% (6). Three (50%)required surgical excision with excellent outcome allowing their hypo-spadias repair to proceed. Results in the more expanded hypospadiasgroup confirmed these findings with increased rates of investigationand identification noted. Boys with normal external genitalia all requiredsurgical treatment since the symptoms resulted from the utricle alone.Penile pain with voiding, hematuria, epididymitis and urinary infectionwere the most common causes. At surgery considerable variation inutricle size and location of vas deferens insertion were noted.
CONCLUSIONS: The prostatic utricle should be considered asa potential cause of morbidity in boys with both normal external geni-talia as well as those with hypospadias. Endoscopic or radiologicalevaluation should be undertaken in all boys with proximal hypospadiasand those with distal hypospadias with associated symptoms or signs.Boys with normal external genitalia with persisting signs or symptomsnot explained by radiological investigations should undergo cystoscopyas an unidentified unsuspected utricle may be the underlying cause.Utricle size should determine the best route for surgical intervention.
Source of Funding: None
527HIGH INCIDENCE OF HYPOSPADIAS IN GRAND-SONS OFWOMEN EXPOSED TO DIETHYSTILBESTROL (DES) DURINGPREGNANCY: A NATIONWIDE MULTIGENERATIONAL STUDY
Nicolas Kalfa*, F Paris, MO Soyer-Gobillard, RB Galifer, JP Daures,C Sultan, Montpellier, France
INTRODUCTION AND OBJECTIVES: DES was prescribed for2-8 millions of pregnancies until 1970s with the mistaken belief that itwould prevent miscarriage. Unfortunately, this synthetic estrogen wasfound to have harmful effects. Prenatal DES exposure has been shownto increase the incidence of genital malformation in males, especiallyhypospadias. More intriguingly, DES-exposed mice have raised suspi-cion on a transgenerational effect, questioning whether the harmfuleffects of DES may be transmitted to subsequent generations. The aimof this work was to evaluate the impact of prenatal DES treatment ongrandsons of women treated with DES during pregnancy.
METHODS: A nationwide epidemiological study in collabora-tion with a French association of DES treated women was conducted.529 families, representing 1180 pregnancies, were included. In 1000pregnancies an exposition to DES was reported. 180 pregnancies were
conducted without DES. The second generation study included 465boys. Mutational analysis of androgen receptor (AR), 5 alpha reductase(5aR) and MAMLD1 genes in hypospadiac boys was performed. Othermaternal environmental expositions were investigated.
RESULTS: First generation: the incidence of hypospadias waslow among boys non-exposed to DES in utero (0/180). On the opposite,the incidence of the malformation in the exposed boys was 3.57%(isolated hypospadias, severe n�12, non severe n�4). Second gen-eration: the incidence of hypospadias in boys born from mothers orfathers non-exposed to DES was low (n�0/360). In boys born fromexposed fathers, the incidence of hypospadias was low too (n�0/8). Onthe opposite, boys born from DES daughters exhibited hypospadias in6.1% of cases (severe n�4, non severe n�2). No mutation of the AR,5aR and MAMLD1 were identified. Other professional exposition of themother was not significant during pregnancy.
CONCLUSIONS: Beyond confirming the impact of prenatalexposure to synthetic estrogens on the development of male genitalia,these results are cause for concern. They show a strong transgenera-tional effect of DES in boys of the second generation. DES sons areunlikely to predispose their own sons to hypospadias, whereas DES-related pathology of reproductive structures in DES daughters mayinterfere with normal fetal development during pregnancy.
Source of Funding: University grant PHRC UF8270
528THE UTILITY OF UROFLOWMETRY IN THE LONG-TERMFOLLOW UP OF DISTAL HYPOSPADIAS REPAIR
Richard Ashley*, Ahmed BaniHani, Julia Barthold, T. ErnestoFigueroa, Wilmington, DE
INTRODUCTION AND OBJECTIVES: Uroflowmetry has beenutilized as an objective measure of successful distal hypospadiasrepair. However, no guidelines exist for how to use uroflow data in orderto identify those patients who need repeat intervention. We sought todetermine if this test could be used as a predictive tool for whichpatients would require a repeat operation.
METHODS: A retrospective chart review was conducted toidentify patients with at least 36 months of follow-up after distal hypo-spadias repair. Additional inclusion criteria included successful potty-training and one complete uroflow study with post-void residual. Uro-flow parameters for each child were compared to the previouslypublished Miskolc nomograms. Univariate and multivariate analyseswere performed to determine factors associated with the need forrepeat intervention with p�0.05 considered significant
RESULTS: A total of 329 patients underwent distal hypospa-dias repair from 2000-2006. 107 patients met inclusion criteria for thestudy and had median follow-up of 63 months (range 36-146mo). Themedian age at surgery was 7mo (range 3-149mo) and median age atpost-operative uroflow was 5.7y (range 3-17yr). Several hypospadiasrepair techniques were utilized: tubular incised plate (TIP, 12/107,11%), Theirsh-Duplay (TD, 44/107, 41%), Mathieu (Ma, 31/107, 29%),sleeve advancement (SA, 11/107, 10%), MAGPI (M, 9/107, 8%). Whenthe entire cohort was analyzed and the Qmax was compared to thepublished norms on the Miskolc nomograms, 38% (41/107) of thepatients had Qmax below the 5th percentile. However, only 13%
e208 THE JOURNAL OF UROLOGY� Vol. 183, No. 4, Supplement, Sunday, May 30, 2010