R. Besson

Sacral neuromodulation in children with urinary and fecal incontinence: a multicenter, open label, randomized, crossover study.

PURPOSE The clinical benefit of sacral neuromodulation is unclear due to the paucity of randomized trial data. The purpose of this study was to evaluate sacral neuromodulation for management of urinary and fecal incontinence in a pediatric population. MATERIALS AND METHODS This multicenter, open label, randomized, crossover study included children older than 5 years. After trial stimulation of the S3 root a neuromodulator (InterStim) was implanted on the S3 foramen. Clinical examinations, voiding and bowel diaries, and urodynamic and manometric evaluations were performed at the beginning (t1) and end (t2) of the first period, and at the beginning (t3) and end (t4) of the second period. RESULTS A total of 33 patients (24 boys) with a mean +/- SD age of 12.22 +/- 5.09 years were randomized. Etiologies were mainly of neurological origin. Incontinence was mixed urinary and fecal in 19 cases, urinary only in 9 and fecal only in 5. Cystometric bladder capacity increased during sacral neuromodulation (delta +24.27 ml vs -37.45 ml, p = 0.01). There was no significant change in other urodynamic or manometric parameters. Overall positive response rate was more than 75% for urinary (81%) and bowel (78%) function. Crossover analysis indicated that sacral neuromodulation is more effective than conservative treatment for both types of incontinence (p = 0.001). CONCLUSIONS In a pediatric population sacral neuromodulation is effective for bladder and bowel dysfunction and should be considered before irreversible surgery.

Biofeedback therapy in the treatment of bladder overactivity, vesico-ureteral reflux and urinary tract infection

OBJECTIVE Voiding disorders are commonly encountered in paediatric urology practice. Urinary incontinence often leads to the impairment of self-esteem but can also cause renal damage, when recurrent urinary tract infection (UTI) or vesico-ureteral reflux (VUR) exists. The aim of this study was to assess the efficacy of a biofeedback training program in children with a long history of voiding disorders. PATIENTS AND METHODS Between 1998 and 2002, 60 children with voiding disorders without neuropathic disease, aged 5-14, were treated. There were 48 girls and 12 boys. The main symptoms were daytime incontinence for 90%, urge syndrome for 78%, and night-time incontinence for 60%. UTIs were noted in 62% of the children, for 37% of them in association with a VUR. Ten weekly sessions were planned for all the children. They were given instructions on toilet behaviour and posture, and pelvic floor training. They had to supervise their voiding frequency and liquid intake at home using a chart. Biofeedback procedure used surface perineal electrodes. The exercises focused on relaxation of the perineum. RESULTS The first results were recorded 6 months after the last session: 96% of the children with daytime incontinence and 83% of the children with night-time incontinence were cured or improved; 84% of the children were free from infection and VUR was cured in 50% of cases. After a mean follow-up of 21 months, 8% of the children with daytime incontinence and 33% with night-time incontinence relapsed, with a significant difference between primary and secondary enuresis. A breakthrough UTI was observed in 19% of cases. CONCLUSION This non-invasive training program was effective in the treatment of daytime incontinence, UTI and VUR. There was an improvement in secondary but not primary enuresis. To prevent relapse, additional support sessions seem to be necessary.

Quality of life in adults with bladder exstrophy-epispadias complex.

PURPOSE We evaluated quality of life in adults with bladder exstrophy-epispadias complex to improve patient and parent counseling. MATERIALS AND METHODS We evaluated quality of life in a multicenter study using the SF-36® Medical Outcome Study questionnaire and a local questionnaire. A total of 47 patients born in 1957 to 1990 were followed due to bladder exstrophy-epispadias complex at the 4 participating hospitals. RESULTS Nine women and 16 men were included in analysis, including 15 who underwent staged reconstruction for bladder exstrophy-epispadias complex and 10 who underwent primary or secondary urinary diversion. Nine patients were married and 2 were in a long-term partnership. A total of 22 patients achieved a high school degree with examination success and 17 worked full time. Six women and 12 men achieved penetrative intercourse. A total of 13 children were born to 3 women and 7 men. Quality of life scores in our patients were less than the norm based scores on 2 of the 8 health concepts, including limitations in physical activity due to health problems and general health perception. Results were statistically different among patients depending on dryness, voiding and urinary reconstruction/diversion. Patient scores did not differ in regard to gender, number of interventions, sexual life, cosmesis or renal function. CONCLUSIONS Patient scores were less than norm based scores on only 2 health concepts and patients were generally well integrated into society. Urinary diversion was usually well tolerated. It remains a therapeutic option when incontinence interferes with social life or renal function is compromised.

Maternal PUFA ω-3 Supplementation Prevents Neonatal Lung Injuries Induced by Hyperoxia in Newborn Rats.

Bronchopulmonary dysplasia (BPD) is one of the most common complications of prematurity, occurring in 30% of very low birth weight infants. The benefits of dietary intake of polyunsaturated fatty acids ω-3 (PUFA ω-3) during pregnancy or the perinatal period have been reported. The aim of this study was to assess the effects of maternal PUFA ω-3 supplementation on lung injuries in newborn rats exposed to prolonged hyperoxia. Pregnant female Wistar rats (n = 14) were fed a control diet (n = 2), a PUFA ω-6 diet (n = 6), or a PUFA ω-3 diet (n = 6), starting with the 14th gestation day. At Day 1, female and newborn rats (10 per female) were exposed to hyperoxia (O2, n = 70) or to the ambient air (Air, n = 70). Six groups of newborns rats were obtained: PUFA ω-6/O2 (n = 30), PUFA ω-6/air (n = 30), PUFA ω-3/O2 (n = 30), PUFA ω-3/air (n = 30), control/O2 (n = 10), and control/air (n = 10). After 10 days, lungs were removed for analysis of alveolarization and pulmonary vascular development. Survival rate was 100%. Hyperoxia reduced alveolarization and increased pulmonary vascular wall thickness in both control (n = 20) and PUFA ω-6 groups (n = 60). Maternal PUFA ω-3 supplementation prevented the decrease in alveolarization caused by hyperoxia (n = 30) compared to PUFA ω-6/O2 (n = 30) or to the control/O2 (n = 10), but did not significantly increase the thickness of the lung vascular wall. Therefore, maternal PUFA ω-3 supplementation may protect newborn rats from lung injuries induced by hyperoxia. In clinical settings, maternal PUFA ω-3 supplementation during pregnancy and during lactation may prevent BPD development after premature birth.

Cure d’hypospadias. La forme de la plaque urétrale a-t-elle une influence sur le résultat de l’intervention de Duplay-Snodgrass ?

OBJECTIVES A number of factors potentially influence the outcome of hypospadias surgery. We evaluate the effect of catheter-size used during urethroplasty (a detail related to the depth of the urethral groove and width/elasticity of the urethral plate) on the complication rate following Duplay-Snodgrass repair. METHODS The data of 113 patients undergoing primary hypospadias repair was retrospectively reviewed. Patients were divided into two groups based on the urethral plate width evaluated by the catheter-size used during urethral reconstruction, and the rate of complications was compared in the two groups. Other factors evaluated included age of patients and severity of the defect. RESULTS Complications (urethro-cutaneous fistula and meatal stenosis) were more common in the narrow urethral plate 8F catheter group (three fistulae among 18 patients) compared to the wide urethral plate 10F catheter group (six fistulae and one meatal stenosis in 95 patients) (p=0.08). CONCLUSION The width of the urethral plate during a modified Duplay urethroplasty has a significant impact on outcome. A smaller-sized catheter for neo-urethral reconstruction, implying a shallow urethral groove and a narrow or inelastic urethral plate, is associated with a higher rate of urethrocutaneous fistula and meatal stenosis.

Short report: SF1 and spleen development: new heterozygous mutation, literature review and consequences for NR5A1-mutated-patient's management.

Steroidogenic factor 1 (encoded by SF1/NR5A1) is a transcription factor with multiple target genes involved in the development and function of multiple steroidogenic and non‐steroidogenic tissues. NR5A1 mutations lead to several phenotypes, including sex reversal, spermatogenesis failure, premature ovarian failure and adrenocortical insufficiency. The implication of NR5A1 mutations in spleen development anomalies was recently highlighted. We provide new evidence of this involvement, describing a novel heterozygous non‐sense NR5A1 mutation in a 46,XY‐DSD with polysplenia female proband and her father, who had hypospadias and asplenia.

Effects of Eicosapentaenoic Acid (EPA) and Docosahexaenoic Acid (DHA) on Fetal Pulmonary Circulation: An Experimental Study in Fetal Lambs

Background: Persistent pulmonary hypertension of the newborn (PPHN) causes significant morbidity and mortality in neonates. n-3 Poly-unsaturated fatty acids have vasodilatory properties in the perinatal lung. We studied the circulatory effects of eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) in fetal sheep and in fetal pulmonary arterial rings. Methods: At 128 days of gestation, catheters were placed surgically in fetal systemic and pulmonary circulation, and a Doppler probe around the left pulmonary artery (LPA). Pulmonary arterial pressure and LPA flow were measured while infusing EPA or DHA for 120 min to the fetus, to compute pulmonary vascular resistance (PVR). The dose effects of EPA or DHA were studied in vascular rings pre-constricted with serotonin. Rings treated with EPA were separated into three groups: E+ (intact endothelium), E− (endothelium stripped) and LNA E+ (pretreatment of E+ rings with l-nitro-arginine). Results: EPA, but not DHA, induced a significant and prolonged 25% drop in PVR (n = 8, p < 0.001). Incubation of vascular rings with EPA (100 µM) caused a maximum relaxation of 60% in the E+ (n = 6), whereas vessel tone did not change in the E− (n = 6, p < 0.001). The vascular effects of EPA were significantly decreased in LNA E+ (n = 6). Incubation with DHA resulted in only a mild relaxation at the highest concentration of DHA (300 µM) compared to E+. Conclusions: EPA induces a sustained pulmonary vasodilatation in fetal lambs. This effect is endothelium- and dose-dependent and involves nitric oxide (NO) production. We speculate that EPA supplementation may improve pulmonary circulation in clinical conditions with PPHN.

Pyéloplastie pour syndrome de la jonction pyélo-urétérale chez l'enfant: voie lombo-assistée versus lombotomie

Surgical treatment of pyelo-ureteric junction syndromes was classically at the childs, a pyeloplasty by posterior way or by lombotomy. For several years, assisted video techniques are proposed for this gesture. The purpose of our study was to compare the lombo-assisted pyeloplasty procedure with the lombotomy procedure, within the framework of this coverage. We made a retrospective study of procedures performed from January 2000 to December 2005, based on a file review of children operated for pyelo-ureteric junction syndrome. Children under the age of 2 years were excluded. Fifty-two children were divided in 2 groups: group 1: 24 children, 7 girls and 17 boys, average age of 86 months (extremes: 27-172) benefited from a lombo-assisted pyeloplasty. Group 2: 28 children, 12 girls and 16 boys, average age of 69 months (extremes: 24-129) benefited from a pyeloplasty by lombotomy. Operating times were significantly shorter in opened surgery than with the lombo-assisted procedure. There was no significant difference in terms of per- or post-operating complication, use of analgesic and hospitalization duration. On the other hand, the lombo-assisted procedure provided the same undisputable aesthetic benefit and the same muscular preservation than pure lomboscopy procedure. As a consequence, this procedure could be set up at no risk for the patient, even if it had lead to longer operating times. However, operating times stayed shorter than with the pure lomboscopy or the celioscopy procedures, compared to the literature.

CL133 - Issues des mégavessies anténatales

La megavessie (MV) antenatale peut etre due a des valves de l’uretre posterieur (VUP) ou un reflux vesico-ureteral de haut grade (RVU) alterant la fonction renale. Le but de l’etude est de determiner l’issue des foetus ayant un diagnostic antenatal de MV. Entre Janvier 2004 et Decembre 2007, 45 foetus ont ete presentes a notre Centre de Diagnostic Prenatal pour MV. Dix-huit grossesses ont ete arretees car elles presentaient un pronostic pejoratif, 2 foetus sont morts in utero. Quinze ont ete diagnostiquees au premier trimestre de grossesse. Les examens anatomopathologiques ont retrouves 9 syndromes malformatifs, 6 dysplasies renales, 2 malformations en rapport avec une dyschromosomie et un couple a refuse l’examen. Vingt-cinq enfants sont nes, 3 ont eu un diagnostic au premier trimestre. Les etiologies sont 10 VUP, 4 RVU, 3 vessies neurologiques, 3 regressions, 1 syndrome malformatif, 1 CAKUT, 1 megauretere, 1 polype du col vesical et 1 perdu de vue. Le suivi median est de 33 mois [0; 72], un seul enfant a eu une greffe pour insuffisance renale. En conclusion, si une MV est diagnostiquee au premier trimestre son pronostic est pejoratif. Si elle est isolee, elle n’engage pas la fonction renale. L’etude de la miction de ces enfants permettrait d’etudier les repercussions de ces MV.

SFCP-P42 – Chirurgie viscérale – Fibrosarcomes congénitaux, tumeur à suspecter avant et après la naissance

Les Sarcomes des tissus mous, dans la premiere annee de vie sont rares. Les plus habituels sont le rhabdomyosarcome, les tumeurs neuroectodermales primitives. Dans cette courte etude nous vous rapportons 4 cas de fibrosarcome infantile diagnostiques dans notre service sur une periode courte, de mars 2006 a avril 2007. Tous ces enfants sont actuellement en vie. Trois d’entre eux ont eu une presentation congenitale, et 2 avaient des echo-graphies ante natales anormales. La tumeur primitive se localisait pour 3 cas au niveau des membres avec une forme localisee et pour 1 sur le tronc avec une forme devant rapidement metastatique. Le diagnostic de certitude a ete fait sur l’examen anatomo-pathogique de biopsie tumorale pour les 4 cas, ainsi qu’une recherche positive du transcript de fusion ETV6/NTKR3 en biologie moleculaire sur un prelevement tumoral. Deux des enfants ont necessite une chimiotherapie preoperatoire. Dans un cas devant une evolution spontanee rapidement metastatique defavorable sur le plan loco regional et general. Dans l’autre la chimiotherapie avait pour but de permettre une chirurgie d’exerese moins mutilante. Actuellement 1 patient est en cours de chimiotherapie avant chirurgie, 1 est en vie avec une resection microscopiquement incomplete mais sans recidive ni metastase a un an de la chirurgie, 2 sont remission complete sans sequelle fonctionnelle objectivable. Le fibrosarcome congenitale est une pathologie rare (2,5/100 000 naissances) habituellement a faible potentiel malin. Cependant des cas, comme dans notre courte serie, a potentiel metastatique on t ete decrit dans la litterature et doivent nous obliger a apporter un diagnostic precoce pour optimiser leur prise en charge.