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Resume L’hyperplasie congenitale des surrenales est une pathologie liee a une mutation genetique responsable d’une production exageree de testosterone des la vie in utero. Nous rapportons le cas d’une patiente de 15 ans atteinte de la maladie, particulierement inobservante de son traitement de substitution hormonale, chez laquelle une « prostate » a ete mise en evidence lors d’une IRM realisee pour un bilan de douleurs pelviennes chroniques et de troubles urinaires.

Objectifs Le traitement endoscopique du RVU chez l’enfant fait partie des guidelines, mais diverses molecules sont presentes sur le marche. Le but est de comparer les Resultats d’une injection endoscopique de polyacrylate-polyalcool copolymere Vantris® a ceux d’une injection de dextranomere/acide hyaluronique Deflux® en termes de recidive d’infection urinaire, chez des enfants traites pour reflux vesico-ureteral (RVU) de grade faible ou intermediaire. Methodes Une etude retrospective monocentrique a ete realisee incluant 94 enfants traites par une injection endoscopique de Vantris® pour un RVU de grade 1 a 3 associe a des pyelonephrites ou a des anomalies scintigraphiques de 2014 a 2019. Ce groupe a ete compare a 91 enfants traites par une injection de Deflux® pour la meme indication entre 2010 et 2014. Le critere de jugement principal est le taux de pyelonephrite a 6 mois postoperatoire, qui a ete compare entre les deux groupes. Resultats Les 2 groupes etaient comparables en termes d’âge moyen a l’injection (5,6 ans dans le groupe Vantris® vs. 6,6 ans dans le groupe Deflux®), de ratio garcons/filles (38/66 vs. 28/66), d’antecedent de pyelonephrite (91 vs. 88 %) et de sequelles scintigraphiques (62 vs. 56 %). Les troubles mictionnels etaient plus nombreux dans le groupe Vantris® (39 vs. 16 %). Le suivi moyen etait plus important dans le groupe Deflux® (45 mois vs. 18). Dans le groupe Vantris®, 7 enfants (7 %) ont presente une recidive de pyelonephrite, dont 3 avaient une recidive de RVU cystographique et 2 enfants ont ete reoperes. Dans le groupe Deflux®, 16 enfants (17 %, p = 0,04) ont recidive une pyelonephrite, dont 8 avaient une recidive de RVU sur la cystographie et ont ete reoperes. Conclusion Dans le traitement endoscopique du RVU, le Vantris® semble avoir une meilleure efficacite que le Deflux® sur la recidive de pyelonephrites, au moins a court terme. Cependant, le risque d’obstruction chronique est a surveiller et necessite des etudes sur les resultats du Vantris® a long terme.

The toxicity of cancer therapies can affect all organs and tissues. Some treatments damage spermatogonial stem cells (SSCs), with a risk of infertility. Storage and reimplantation of frozen testicular tissue is a recent approach tofertilitypreservationfor young boys. However, thawed frozen prepubertal testicular tissue must undergo a maturation process to restore sperm production. This process, currently being studied in animal models, can be achieved by in vivo transplantation of SSCs into seminiferous tubules or by testicular grafting, possibly following in vitro maturation.

Resume But Etudier les effets d’une preparation par erythropoietine, avant chirurgie des grands hypospades, sur le retentissement du saignement peroperatoire et le resultat de la chirurgie. Patients et methodes Vingt-et-un patients ont ete operes d’un Onlay entre janvier 2001 et septembre 2008, apres une preparation par l’erythropoietine. Sept avaient un hypospade moyen, deux un hypospade penien posterieur et 12 un hypospade penoscrotal. Les enfants ont tous ete revus a quatre mois pour juger du resultat de l’intervention. Resultats Apres chirurgie, 18 enfants avaient un meat apical (85,7 %). Trois avaient un meat balanopreputial, deux une stenose du meat, deux enfants ont ete repris, un selon la technique de Duplay, l’autre pour meatostomie. Aucune fistule, aucune necrose du lambeau n’a ete observee. L’hematocrite preoperatoire moyenne etait de 41 %, soit un gain de 3,8 points en moyenne. La perte d’hematocrite peroperatoire a ete evaluee a 6,6 points. A la fin de l’intervention, 12 enfants avaient une hematocrite inferieure a 35 %, dont quatre une hematocrite inferieure a 30 %. Aucun n’a necessite de transfusion. Conclusion La preparation par l’erythropoietine avant chirurgie d’Onlay a semble avoir plusieurs avantages : d’une part, une hematocrite plus elevee en fin d’intervention qu’apres une preparation par le fer seul et, d’autre part, un taux moindre de complications postoperatoires, notamment fistules et necrose du lambeau.

Two different protocols to thaw cryopreserved human ovarian cortex have been evaluated using a histological analysis. The slower one, based on a progressive dilution of cryoprotectants, seems to maintain an optimal follicle morphology.

Resume Objectif.– La prise en charge des testicules impalpables (cryptorchidies) est une preoccupation habituelle des equipes specialisees de chirurgie pediatrique. L'exploration cœlioscopique premiere est maintenant admise par la plupart des auteurs mais l'attitude therapeutique et les techniques d'abaissement restent discutees. Le clampage premier du pedicule spermatique dans le temps de l'exploration permet le developpement de suppleances garant d'une bonne vascularisation de la gonade lors de l'abaissement definitif quelques mois plus tard. Materiel et methode. – Une serie de 122 garcons porteurs d'une cryptorchidie a ete revue. Tous ces enfants ont eu une cœlioscopie exploratrice. Lorsque le testicule etait retrouve, un clampage du pedicule spermatique a ete effectue dans le meme temps et l'abaissement definitif a ete realise quelques mois plus tard. Resultats. – Une gonade a ete retrouvee dans 60 cas et le clampage du pedicule spermatique dans le meme temps a permis un abaissement satisfaisant quatre a six mois plus tard dans pres de 80% des cas. Conclusion. – L'etude de cette serie retrospective du clampage premier par voie cœlioscopique des testicules impalpaples confirme une meilleure fiabilite de cette technique comparee aux abaissements en un temps.

Resumen La patologia aguda mas importante de la bolsa escrotal en el nino y el adolescente es la torsion del cordon espermatico, mal llamada torsion testicular. Este cuadro frecuente provoca una necrosis testicular rapida con destruccion de la funcion seminal y, mas tarde, de la funcion endocrina. Se admite que, despues de 6 horas de evolucion, la funcion seminal se encuentra amenazada, por lo que el cuadro constituye una verdadera urgencia. El diagnostico de torsion del cordon espermatico debe ser siempre el primero en sospecharse y una vez confirmado, obliga a intervenir quirurgicamente sin demora para conservar la funcion reproductora. A pesar del desarrollo y de los avances de las pruebas complementarias de imagen (ecografia, Doppler, gam-magrafia), a menudo la cirugia es lo unico que proporciona un diagnostico de certeza. Todavia se interviene tarde a muchos ninos debido, sobre todo, al desconocimiento del diagnostico y de su gravedad por parte del paciente o de sus padres y, otras veces, a pruebas complementarias falsamente tranquilizadoras. Deben conocerse las maniobras externas de reduccion de la torsion que el cirujano puede intentar, pero que no evitan la intervencion rapida. Los otros cuadros patologicos agudos (torsion del anexo, epididimitis y orquioepididimitis) no tienen el mismo caracter de urgencia. Ahora bien, como la certidumbre diagnostica nunca es absoluta, ante cualquier bolsa escrotal dolorosa en un nino debe mantenerse el principio de la intervencion sistematica de urgencia.

Objectifs Pour les patients porteurs d’une uropathie severe, la transition de l’âge pediatrique a l’âge adulte est un enjeu majeur. Dans cette etude, nous avons voulu analyser notre activite de consultation conjointe et evaluer son efficacite dans la transition du patient des services de pediatrie vers l’urologie adulte. Methodes Etude retrospective sur dossiers, menee a partir des listes de la consultation conjointe. Celle-ci regroupe deux fois par an un urologue pediatre, un urologue d’adulte et deux infirmieres stomatherapeutes autour de jeunes patients suivis pour une pathologie urologique chronique. Depuis 2003, 92 patients ont ainsi ete recus a cette « consultation de transition ». Les donnees recueillies concernaient l’âge a la premiere consultation conjointe, la duree du suivi et le devenir des patients. Resultats Soixante-treize patients ont consulte pour vessie neurologique (52 sur dysraphisme spinal), 12 pour malformation vesicale complexe (exstrophie, agenesie du trigone et VUP), 4 pour malformation genitale, 2 pour troubles mictionnels et une pour lithiase. L’âge moyen lors de la premiere consultation etait de 23,8 ans (14–51 ans). Quarante-trois patients n’ont ete vus qu’une fois en consultation conjointe ; les autres sont venus 2,3 (2–9) fois en moyenne, soit un suivi de 45,6 (7,6–138,4 mois) en consultation conjointe. Apres cette phase de transition, 53 patients (57 %) ont poursuivi leur prise en charge dans le service d’adultes, 6 sont toujours suivis en pediatrie dont 2 en consultation conjointe, 11 consultent a la fois en pediatrie et chez les adultes, 2 sont decedes et 21 ont ete perdus de vue. Trente-trois patients ont necessite une intervention, dont une seule a ete realisee en pediatrie. Conclusion La consultation conjointe permet une transition efficace, de la pediatrie vers l’urologie adulte, dans plus de 50 % des cas. Pour les praticiens, cette consultation represente un temps privilegie de discussion entre chirurgien pediatre et urologue d’adultes sur des dossiers complexes. Pour les patients, elle est un lieu de rencontre privilegie avec les professionnels qui les suivront desormais.

The toxicity of cancer therapies can affect all organs and tissues. Some treatments damage spermatogonial stem cells (SSCs), with a risk of infertility. Storage and reimplantation of frozen testicular tissue is a recent approach tofertilitypreservationfor young boys. However, thawed frozen prepubertal testicular tissue must undergo a maturation process to restore sperm production. This process, currently being studied in animal models, can be achieved by in vivo transplantation of SSCs into seminiferous tubules or by testicular grafting, possibly following in vitro maturation.

Mucopolysaccharidosis type IH (MPS IH) is a rare autosomal recessive lysosomal storage disorder. Haematopoietic stem cell transplantation (HSCT) has been proposed for the treatment of MPS IH patients and offers the possibility to grow into their adulthood. Precocious puberty has been described in few MPS patients. We report, to the best of our knowledge and for the first time, the initiation of the first waves of spermatogenesis fortuitously observed in seminiferous tubules of a pre-pubertal 19-month-old boy, affected by MPS IH and who did not present any clinical signs of precocious puberty. This patient benefited from testicular tissue cryopreservation before HSCT. Seminiferous tubule size, germ cell differentiation and Sertoli cell expression of androgen receptor and anti-müllerian hormone corresponded to the pattern observed in a pubertal boy. The Hurler syndrome may be responsible for the precocious initiation of spermatogenesis. A specific follow-up during childhood may be useful to confirm if such abnormal testis development is common in young boys with MPS IH and if it may lead to precocious onset of puberty in survivors despite HSCT. Furthermore, we have observed that Sertoli cell maturation (up-regulation of AR expression, down-regulation of AMH expression) occurred before the clinical signs of puberty and before the increase of testosterone plasmatic level.

Objective To report our experience of assessing children with chronic voiding dysfunction (>6 months’ duration) using a minimal urodynamic evaluation, and the management of detrusor-sphincter dyscoordination (DSdc) using pelvic floor biofeedback. Patients and methods From 1994 to 1997, 120 children (mean age 7.5 years) with three predominant and associated symptoms were referred to one urologist; they had nocturnal enuresis (28 children), urge incontinence (42) or urinary tract infection (50). All patients were assessed by urinary culture, renal ultrasonography and a minimal urodynamic evaluation, i.e. urinary flowmetry with sphincter electromyography (EMG) using perineal surface electrodes. If they had urinary tract infection and/or renal dilatation, they underwent voiding cysto-urethrography. In children with DSdc, urinary training with frequent voiding was instituted initially, with subsequent pelvic floor biofeedback exercises if the improvement was deemed unsatisfactory. Results DSdc was diagnosed in 33 children (28%), none of whom had isolated nocturnal enuresis. Pelvic floor biofeedback was undertaken by 15 children (12 girls and three boys); it was well accepted because it was administered as a computer game. In all affected patients the DSdc resolved on EMG and there was a significant clinical improvement. Vesico-ureteric reflux was detected in 24 patients, associated with DSdc in 10. The reflux resolved spontaneously on antibiotic prophylaxis in six children and after urinary re-education in four. Conclusion A minimal urodynamic evaluation seems to be useful in the diagnosis of DSdc which caused urinary tract infection and/or bladder overactivity. The results with pelvic floor biofeedback were excellent in these children.

Stenosing and calcified ureteritis occurring in Henoch–Schonlein purpura remains a rare urological complication. The authors describe their own experience of the diagnosis and treatment of this rare p

Resume L'observation d'un garcon de 14 ans ayant presente une peritonite inaugurale d'une meningite a meningocoque de type C est rapportee. Il s'agissait initialement d'un diagnostic de peritonite appendiculaire, la cœlioscopie exploratrice revelant un appendice normal mais un epanchement trouble d'importance modeste, conduisant a une antibiotherapie a large spectre. L'examen direct objectivera quelques cocci Gram negatifs. Dix heures apres l'intervention, l'enfant a presente un syndrome meninge faisant realiser une ponction lombaire revelant une reaction meningee a polynucleaires, une hyperproteinorachie, mais pas de germe a l'examen direct. L'hemoculture pratiquee a l'entree a pousse a la 24 e heure mettant en evidence un Neisseria meningitidis de type C, la culture du liquide peritoneal et du LCR sera negative, mais la recherche d'ADN meningoccique sur le liquide peritoneal s'averera positif a meningocoque C.

To study the effect of a preparation by erythropoietin before Onlay's intervention on postoperative hematocrits and medium-term results of the surgery.Twenty-one patients were operated on by Onlay's technique between 2001 January and 2008 September, after being treated by erythropoietin. Seven had a midshaft hypospadias, two a posterior hypospadias and 12 a penoscrotal hypospadias. All children were examinated four months after surgery to evaluate the surgical results.After the surgery, 18 children had an apical meatus (85.7%). Three had a balanopreputial meatus, two a stenosis of the meatus. Two children were reoperated on, one with the Duplay's technique, the other for a meatostomy. No fistula, no necrosis of the preputial flap was observed. The preoperative hematocrit was measured at 41%, with an increase of 3.8% because of the preparation. Peroperative blood loss was evaluated at 6.6%. After surgery, 12 children had an hematocrit inferior than 35%, four an hematocrit inferior than 30%. No transfusion was needed.Preparation by erythropoietin before severe hypospadias surgery seemed to have several advantages: a more elevated hematocrit after surgery than with an iron preparation alone, and a lower rate of postoperative complications, including fistulas and necrosis of the preputial flap.

We evaluated quality of life in adults with bladder exstrophy-epispadias complex to improve patient and parent counseling.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.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.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.

A 15-year-old boy was first referred for a clinical presentation of revealed peritonitis. Abdominal endoscopy showed normal appendix but the presence of purulent peritoneal fluid. Antibiotics were immediately administered. Ten hours later clinical signs of meningitis occurred, and lumbar puncture was performed. CSF bacterial meningitis characteristics were present but no bacteria was observed. However Neisseria meningitidis type C was detected by direct peritoneal fluid examination and by 24(th) hour blood culture. Peritoneal and the CSF fluid culture were negative, but DNA analysis from peritoneal fluid was positive.

Impalpable and undescended testis is a very common preoccupation for specialized pediatric surgery teams. The laparoscopy as the single most accurate modality for diagnosis and localization of impalpable undescended gonad is well established by all authors. However the therapeutic attitude and the technique are still a matter of debate.A series of 122 boys presenting an impalpable and undescended testis was studied. All the children beneficiated of a primary laparoscopic exploration. During this exploration, when a testis was found, the clamping of the spermatic vessel was made and the final descent took place few months later.In our study the testis was found in 60 cases and the spermatic vessel's clamping assured the descent of a well vascularized testis in about 80% of cases.Clamping the spermatic vessel during the laparoscopic exploration assured, thanks to the development of a supply, a good vascularisation of the testis during its second step descent. Our retrospective study has showed the good reliability of laparoscopic surgery as primary treatment in front of impalpable undescended testicles in comparison to the one-step classical orchiopexy.

Objectif Etudier les troubles vesico-sphincteriens des enfants ayant un dysraphisme spinal afin d’adapter leur protocole de surveillance. Materiel et methode Etude retrospective monocentrique (1997–2003). Resultats 47 enfants ont ete inclus, âge median au recueil 7,3 ans (1–16). 24 enfants se sondent ou sont incontinents : 10/13 myelomeningoceles, 1/8 lipomes, 7/7 agenesies sacrees, 6/9 dysraphismes avec malformation anorectale (MAR). 13 ont eu une chirurgie urinaire (10 enterocystoplasties +/- cystostomie continente, 1 Mitrofanoff, 2 injections de toxine botulique). 19 sont aux lavements evacuateurs ou sont incontinents : 7/13 myelomeningoceles, 1/8 lipomes, 5/7 agenesies sacrees, 6/9 dysraphismes avec MAR. Les enfants avec agenesie sacree, myelomeningocele ou ayant une MAR associee sont plus a risque de presenter des troubles vesico-sphincteriens majeurs (p=3.10 −6 ). Conclusion La myelomeningocele, l’agenesie sacree et le dysraphisme associe a une MAR necessitent une prise en charge rapprochee : consultation et echographie regulieres, BUD tous les 2- 3 ans. Pour les lipomes, le suivi peut etre espace en conservant des BUD reguliers. Pour le sinus dermique, le suivi sera simplifie : consultation et echographie apres l’âge de la proprete.

Objectifs Etude retrospective portant sur 28 patients pris en charge pour une malformation anorectale haute sur le CHU de Rouen entre 1982 et 2011. Sujets 20 garcons et 8 filles ont ete operes par voie sacro-coccygienne posterieure selon la technique de Stephens. Le suivi a porte sur les complications digestives et urologiques avec un recul de 3 a 27 ans. Resultats principaux Un peu moins de la moitie (13) des enfants presentaient une malformation rachidienne avec 12 agenesies sacree et une moelle attachee basse pour 6 d’entre eux. 1 enfant presentait des anomalies vertebrales isolees. Seul 53% d’entre eux ont obtenu une continence satisfaisante spontanee avec la persistance de quelques fuites (3) ou a l’aide d’une caecostomie (4). Pour les 15 enfants avec une malformation anorectale haute sans anomalie sacree, les malformations urologiques etaient les malformations les plus frequemment associees. Les resultats sur la continence dans ce groupe sont evidemment meilleurs puisque seulement 3 enfants sont completement incontinents et 1 a beneficie d’une caecostomie. Conclusion La continence reste un challenge chez les enfants presentant une malformations anorectale haute, elle est essentiellement tributaire des anomalies sacrees avec une capacite d’amelioration parfois tard dans l’enfance.

Stenosing and calcified ureteritis occurring in Henoch-Schönlein purpura remains a rare urological complication. The authors describe their own experience of the diagnosis and treatment of this rare pathology along with a review of the literature. Recognition and early surgical management may prevent serious renal outcome. In cases of ureteral replacement, different procedures using ileal segment have been proposed. We report the results of the Cockett and Goodwin procedure with a follow-up of more than 25 years.

PURPOSE Bladder Exstrophy-Epispadias Complex (BEEC) is a rare congenital malformation which requires a lifetime follow-up. Quality of life (QOL) is one of the concerns of the patients and their parents. MATERIAL AND METHODS We present a multicenter study which evaluated the QOL of 25 adult patients born with a BEEC from 1957 to 1990. To evaluate the QOL, we used the Short-Form (SF-36) Health Survey questionnaire and a local social and sexual questionnaire. Of the 25 patients, 15 had undergone a stage reconstruction of BEEC and 10 had primarily or secondarily undergone a urinary diversion. RESULTS The scores obtained for our patients were statistically under the norm-based scores for 2 of the eight health concepts: limitations in physical activities because of health problems and general health perception. The results were statistically different among the patients depending on dryness, method of voiding urine, and reconstruction/diversion. The patient's scores were not different regarding the gender, number of intervention, sexual life, aesthetic aspect and renal function. Nine patients are married and 2 are in long-term relationship. Twenty-two patients achieved examination success. Seventeen patients had a full-time work. Twelve men were capable of penetrative intercourse and 6 women managed penetrative intercourse. Thirteen children were born from 13 patients (3 women and 10 men). CONCLUSIONS Patients had obtained scores under the norm-based scores only in two health concepts. Moreover, they are generally well-adjusted in society. Urinary diversions are usually well tolerated and remain a therapeutic option when incontinence interferes with social life or when renal function is compromised.

To report our experience of assessing children with chronic voiding dysfunction (6 months' duration) using a minimal urodynamic evaluation, and the management of detrusor-sphincter dyscoordination (DSdc) using pelvic floor biofeedback.From 1994 to 1997, 120 children (mean age 7.5 years) with three predominant and associated symptoms were referred to one urologist; they had nocturnal enuresis (28 children), urge incontinence (42) or urinary tract infection (50). All patients were assessed by urinary culture, renal ultrasonography and a minimal urodynamic evaluation, i.e. urinary flowmetry with sphincter electromyography (EMG) using perineal surface electrodes. If they had urinary tract infection and/or renal dilatation, they underwent voiding cysto-urethrography. In children with DSdc, urinary training with frequent voiding was instituted initially, with subsequent pelvic floor biofeedback exercises if the improvement was deemed unsatisfactory.DSdc was diagnosed in 33 children (28%), none of whom had isolated nocturnal enuresis. Pelvic floor biofeedback was undertaken by 15 children (12 girls and three boys); it was well accepted because it was administered as a computer game. In all affected patients the DSdc resolved on EMG and there was a significant clinical improvement. Vesico-ureteric reflux was detected in 24 patients, associated with DSdc in 10. The reflux resolved spontaneously on antibiotic prophylaxis in six children and after urinary re-education in four.A minimal urodynamic evaluation seems to be useful in the diagnosis of DSdc which caused urinary tract infection and/or bladder overactivity. The results with pelvic floor biofeedback were excellent in these children.

Background. In at least 15 % of dilated urinary tracts, diuresis renography fails to assess the presence or absence of urinary obstruction. Objective. To determine the shortcomings of 99 mTc-DTPA frusemide diuresis renography by reference to pressure flow studies. Materials and methods. Thirty-four patients, aged 1 month to 20 years, with questionable obstruction were evaluated by diuresis renography and pressure flow studies (the Whitaker test) as the reference method during the same short period of time. Discrepancies were analysed. Results. In patients with type I or IIIa renographic response, pressure flow studies never led to any change in management. Poor function, major dilatation and prior surgery were found to be risk factors of inaccurately obstructive pattern (type II) on renography (n = 6). In patients with type IIIb response, pressure flow studies could show low-grade (n = 3) or intermittent obstruction (n = 2). Intermittent obstruction was also demonstrated in two patients with type II response. Conclusion. In patients with risk factors, type II response was sometimes inaccurate, and urodynamic evaluation showed absence of obstruction and led to conservative management. Type IIIb response should be considered equivocal rather than partially obstructive, and pressure flow studies could be considered in such patients.