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Evaluation of repeated (at least 4) intra-detrusor injections of toxin botulinum A (IDI-TBA) for neurogenic bladder in a pediatric cohort.Patients who underwent at least 4 IDI-TBA between 2005 and 2017 for neurogenic bladder related issues were included (detrusor overactivity and low compliance). Clinical and cystometric data were collected before and after the first injection and after the last injection. The primary endpoint was the proportion of patients with non-abnormal cystometry (no detrusor overactivity and normal compliance). Secondary outcomes were the evolution of the observed bladder capacity/expected ratio, surgical complications and acquired kidney impairment.From the 832 patients referred to our institution for neurogenic bladder, 48 underwent IDI-TBA, and 17 at least 4 injections. Among them, a total of 95 procedures were performed (median per patient 5 [4-8]). While the first injection had a significant effect for 82.3% patients, the last injection improved the medical condition for only 53.0% cases. The bladder capacity ratio, initially 36.1%, increased to 80.3% after the first injection but decreased to a level of 57.1% at last. After a median follow-up of 57 [34-102] months, no severe complications were reported but 11.8% of patients presented with repeated pyelonephritis. A bladder augmentation surgery was finally indicated for 35.3% cases.Despite a low complication rate and impressive cystometric results after the first injection, IDI-TBA efficacy decreased with time and repetition. These findings prone a long-term follow-up and a "à-la-carte" management of this specific population depending on the long-term response to IDI-TBA.

Resume La reforme du 3e cycle des etudes medicales, en vigueur depuis la rentree 2017 au terme de 8 annees de gestation, a pour principaux objectifs de rendre coherente et equitable la formation des futurs specialistes et de substituer a une qualification basee sur une duree, une formation basee sur l’acquisition progressive de competences. Pour les 13 colleges de specialites chirurgicales, cette reforme a ete l’occasion, pour la premiere fois peut-etre, de partager une reflexion sur ce qu’etait un chirurgien et ce que devait etre la formation aux specialites chirurgicales. Celle-ci leur a permis de s’adapter aux contraintes du cahier des charges et de construire des maquettes de formation coherentes entre elles. Cet article aborde l’historique de cette reforme, ce qui change dans la formation des futurs chirurgiens, ainsi que quelques points de vigilance. Cette reforme du 3e cycle a enclenche la reforme du second cycle, qui devrait entrer en vigueur pour la rentree universitaire 2020. Son objectif sera en particulier de supprimer le couperet que constituent les epreuves de l’ECN et de permettre aux etudiants de mieux apprehender et tester leurs appetences et leurs competences pour une specialite donnee. Il appartiendra a ces memes colleges de specialites chirurgicales de definir comment y parvenir pour les specialites de la discipline « chirurgie ».

Summary Objective Total small-intestinal volvulus with malrotation (TSIVM) classically presents in the neonatal period; it occurs much less frequently in the adult and is often misdiagnosed. Prognosis is directly related to the degree and duration of intestinal ischemia. Our goal is to describe our experience with TSIVM in the adult, to identify any specific findings and to discuss its management. Method Eleven patients who had undergone surgery for TSIVM at three centers between 1992 and 2012 were included. Surgery was performed as an emergency for five patients and surgery was elective for six. Results Mean follow-up was 63 months (range: 12–270). Six patients had had previous abdominal surgery. In nine cases, the diagnosis of TSIVM was made preoperatively, mainly by CT scan in eight cases. Seven patients had associated congenital failure of retroperitoneal fixation of the right colon and all of these underwent a Ladd procedure. The mortality rate was zero. Of the five patients who underwent emergency surgery, three required intestinal resections, one of whom developed a short bowel syndrome. The six patients who underwent surgery electively had no surgical complications. Conclusion TSIVM is a very unusual finding in adult patients. The diagnosis can be made by CT scan with IV and oral contrast, but it often comes to light only at the time of surgery, even though the patients have often had recurrent episodes of abdominal symptomatology that dated back to childhood. The Ladd procedure, consisting of division of Ladd's bands, widening of the mesentery, and incidental appendectomy, remains the standard surgical repair. Digestive surgeons who care for adults should be familiar with this procedure, and it should be performed, as often as possible, with the assistance of a pediatric surgeon.

Resume Objectif Le volvulus total du grele sur malrotation (VTGM) est une pathologie classique en periode neonatale, mais beaucoup plus rare et souvent meconnue chez l’adulte. Son pronostic est directement lie au risque d’ischemie intestinale. Le but de cette etude est de decrire notre experience du VTGM chez l’adulte, d’identifier d’eventuelles specificites et de discuter sa prise en charge a l’âge adulte. Methode Onze patients operes de VTGAR ont ete etudies retrospectivement sur trois centres entre 1992 et 2012. Cinq patients ont ete operes en urgence et 6 ont ete operes a froid. Resultats Le suivi moyen etait de 63 mois (12–270). Six patients avaient eu une chirurgie abdominale prealable. Le diagnostic de VTGM etait fait en preoperatoire dans 9 cas, par tomodensitometrie (TDM) dans 8 cas. Sept patients avaient un defaut d’accolement du colon droit associe. Tous ont ete traites par une procedure de Ladd. Le taux de mortalite etait nul. Parmi les 5 patients operes en urgence, 3 ont eu une resection intestinale dont un a developpe un syndrome du grele court. Les 6 patients operes a froid n’ont eu aucune complication chirurgicale. Conclusion Le VTGM est exceptionnel a l’âge adulte. Le diagnostic peut etre fait par TDM avec injection et opacification haute, mais n’est souvent evoque qu’en peroperatoire, chez des patients qui presentaient pourtant une symptomatologie abdominale recurrente, parfois depuis l’enfance. La procedure de Ladd, qui consiste en une mise en mesentere commun complet avec appendicectomie, reste la technique de reference. Cette procedure devrait etre mieux connue des chirurgiens digestifs d’adultes et realisee, autant que faire ce peut, avec le concours d’un chirurgien pediatre.

Resume Introduction La duplication digestive est une heterotopie de muqueuse digestive. Sa localisation linguale est rare, elle se presente sous la forme de lesion kystique. Nous presentons une serie de cinq patients avec une lesion kystique de la langue, diagnostiquee duplication digestive. Patients et methodes Cette etude retrospective a repris les cas de duplication digestive de localisation linguale diagnostiques entre 1997 et 2008. Nous avons repertorie leur âge, leur sexe, les circonstances de decouverte, la localisation de la lesion, les caracteristiques cliniques, radiologiques et anatomopathologiques, le traitement et l’evolution de ces lesions. Resultats Quatre garcons et une fille ont ete inclus. Le diagnostic avait ete evoque en antenatal pour deux d’entre eux, en periode neonatale pour deux et a l’âge de 12 ans pour un patient (diagnostique au decours d’une surinfection). Dans les cinq cas, le diagnostic n’a pu etre confirme qu’apres exerese chirurgicale. Discussion Les duplications digestives de localisation linguale sont rares, mais leur incidence est probablement sous-estimee. Ce diagnostic doit etre evoque devant des lesions congenitales kystiques de la langue. Le traitement est chirurgical, et l’examen anatomopathologique confirme le diagnostic.

Resumen Tras recordar las diferentes formas anatomoclinicas, se exponen las indicaciones y modalidades tecnicas del tratamiento de los ureteroceles en el nino. El tratamiento endoscopico ocupa un lugar cada vez mas importante en el tratamiento de los ureteroceles con duplicacion en el lactante, frecuentemente descubiertos antes de que aparezcan las infecciones gracias a la ecografia antenatal; si bien esto resulta logico en los ureteroceles intravesicales, parece cuestionable en los ureteroceles extravesicales debido a la mala calidad de la pelvis renal superior y a la frecuencia de reflujo postendoscopico. La ureterocelectomia quirurgica, indicada sobre todo en los ureteroceles intravesicales, conlleva la reconstruccion del trigono y la reimplantacion del o de los ureteres con eventual remodelamiento. En la mayoria de los ureteroceles extravesicales es suficiente la nefroureterectomia polar superior con aspiracion del ureterocele.

The objective of this article is to inform health-care personnel, especially those of pediatric hospitals (pediatricians, surgeons, anesthetists, etc.), about the incidence of latex allergy in children and the adverse effects that may result if avoidance measures are not taken. The prevalence of this allergy is increasing because of repeated exposure to this ubiquitous material (in medical equipment or household products such as bottle teats or balloons). The risks are allergic reactions, ranging from benign local dermatitis to anaphylactic shock. This problem is well known and progress has been made: there is increased production of latex-free products and screening for latex-related allergy or sensitization during the pre-anesthetic evaluation to take preventive actions when planning surgery. The younger the patient exposed to latex, the higher the risk of sensitization. Therefore, the main issue raised in this article is the potential benefit of moving to latex-free health-care facilities so as to minimize the risk of allergic incidents. Many studies showed a significant decrease in the risk of allergy (sensitization and allergic reaction) when avoiding latex, but we must also consider the feasibility, the cost, and the effectiveness of such a policy.

Resume Introduction. – La lithiase biliaire est frequente chez l’enfant drepanocytaire. Ses complications sont difficiles a differencier des crises douloureuses abdominales vaso-occlusives et elles peuvent parfois menacer le pronostic vital du patient. Materiel et methode. – Nous avons etudie les resultats de l’echographie abdominale de 185 enfants drepanocytaires âges de zero a 18 ans et leur suivi a l’hopital d’enfants Armand-Trousseau (Paris) de 1982 a 1998. Resultats. – Une lithiase biliaire etait presente chez 26 patients. La lithiase a ete decouverte chez 12 patients devant une manifestation clinique. Vingt-huit pour cent des patients asymptomatiques porteurs d’une lithiase ont developpe secondairement des manifestations cliniques dans un delai maximum de deux ans et demi. Une cholecystectomie par cœlioscopie a ete effectuee dans neuf cas et par laparotomie dans 17 cas. La duree d’hospitalisation postoperatoire etait en moyenne plus courte dans le groupe de patients operes par cœlioscopie que dans le groupe de patients operes par laparotomie. L’examen anatomopathologique montrait des lesions de cholecystite subaigue ou chronique dans 85 % des cas. Conclusion. – Chez l’enfant drepanocytaire, nous conseillons de rechercher attentivement une lithiase biliaire devant toute manifestation abdominale. Nous preconisons d’effectuer une echographie abdominale annuellement des l’âge de sept ans. Nous recommandons de faire une cholecystectomie des la decouverte d’une lithiase biliaire.

Resume But de l’etude : Evaluer les resultats chez l’enfant de l’anomyoplastie unilaterale de Pickrell pour incontinence anale secondaire a une malformation anorectale. Patients et methode : Entre 1975 et 1997, 23 enfants ont ete operes ; leur âge moyen lors de l’intervention etait de 8,5 ans. Les resultats fonctionnels ont ete evalues systematiquement chez 20 patients par le score clinique de Kelly, complete chez certains enfants par une manometrie anorectale et une electromyographie de l’appareil sphincterien. Resultats : Il n’y a pas eu de mortalite postoperatoire. Une colostomie a ete realisee au debut de l’experience pour un abces local recidivant. Le resultat fonctionnel avec un recul moyen de six ans a ete juge bon dans 25 % des cas, moyen dans 45 % des cas et mauvais dans 30 % des cas. Une amelioration clinique sur la continence a ainsi ete obtenue chez 70 % des patients. Conclusion : L’amelioration de la continence apres intervention de Pickrell depend essentiellement des criteres de selection des patients, de la reeducation pre et postoperatoire et de la realisation de lavements evacuateurs reguliers. En cas de resultat insuffisant, les auteurs proposent une bilateralisation de l’anomyoplastie qui a permis chez neuf enfants d’ameliorer huit fois le resultat.

Resume La hernie ombilicale de l'enfant est une pathologie benigne. Son evolution spontanee se fait le plus souvent vers la regression complete dans les quatre premieres annees, et l'etranglement herniaire est tres rare. Ce n'est que lorsque la hernie est symptomatique ou jugee definitive qu'une intervention est proposee.

Infant small-bowel intussusceptions, most of the time idiopathic, may exceptionally reveal a severe digestive disease. We report the case of a 4-month-old infant who presented multiple and simultaneous ileal intussusceptions associated with severe acute gastroenteritis. Initially, the infant showed a protein-losing enteropathy with a clear alteration of the general state of health and undocumented fever, resistant to broad-spectrum antibiotic therapy. Skin and splenic nodules associated with hepatosplenomegaly and pancytopenia set in progressively. The etiologic evaluation led to the diagnosis of a Mycobacterium bovis BCG infection related to severe combined immune deficiency. The treatment consisted in anti-tuberculosis quadruple therapy in association with immunoglobin supplementation. Secondarily, the patient underwent gene therapy in a clinical trial. An early BCG vaccine in the first weeks of life, before the outbreak of infection revealing the immune deficiency, is a risk factor in triggering a disseminated BCG infection in immunodepressed infants. This clinical case is the first reported of severe combined immune deficiency revealed by small-bowel intussusceptions related to a disseminated BCG infection.

Resume Les stenoses des enterocolites ulceronecrosantes (ECUN) s'obserent a la fois chez les nouveau-nes traites chirurgicalement pour perforation et chez les patients traites medicalement, l'occlusion etant alors revelatrice. En raison des risques de perforation a distance et de septicemie, il para't interessant de dṕrister ces stenoses par un laverment opaque avant toute alimentation. L'objectif de ce travail est de comparer les delais, les caracteristiques cliniques et anatomopathologiques des cas chirurgicaux et des cas medicaux, d'une part revelees par une occlusion et, d'autre part, par une opactification systematique. Patients et methodes. — Quinze enfants ayant presente une ou plusieurs stenoses ont ete etudies a partir de 50 ECUN observees de 1984 a 1994. Le diagnostic d'ECUN a ete pose devant les symptomes cliniques habituels, l'existence d'une pneumatose radiologique dans 43 cas et/ou d'une perforation dans 16 cas. Parmi ces 16 cas chirurgicaux, 12 malades ont survecu a la periode initiale. Parmi les 34 cas medicaux, 11. etudies avant 1989, n'ont pas beneficie d'un lavernent opaque systematique, 23 observes de 1989 a 1994 ont eteetudies de faccon prospective par un lavement aux hydrosolubles avant toute alimentation. Resultats. — Une ou plusieurs slenoses ont ete observees quatre fois chez les 12 malades chirurgicaux et quatre fois chez les 11 malades medicaux sand lavement opaque prospectif (un deces) el, enfin, sept fois sur 23 par depistage systematique. Les 26 stenoses observees chez les 15 nourrissons siegeaient dix fois sur le colon droit, cinq fois sur le tranverse et 11 fois sur le colon gauche. Une stenose ileale est survenue chez un patient en enterostomie. Les delais de constitution des stenoses ont ete comparables dans les trois groupes: de 3 semaines a 3 mois. Les examens anatomopathologiques ont montre, sur 15 cas, le meme type de lesions: fibrose dans 15 cas, aedeme dans neuf cas et inflammation chronique dans 12 cas. Conclusion. — Le pourcentage de 30 % de stenose est identique sur le colon exclu apres perforation ou chez les patients medicaux apres revelation par une occlusion ou par le lavement opaque. Le lavement opaque permet de depister des stenoses organiques et definitives avant qu'elles ne soient symptomatiques, evitant le risque vital lie a toute occlusion et permettant une chirurgie en un seul temps.

The urological management of urinary incontinence in neurogenic bladder due to spinal cord lesions in children is intended to achieve social continence while preserving the upper urinary tract, combining clean intermittent catheterization with anticholinergic agents. The objective of this study was to report the results on continence of endoscopic management of bladder and/or sphincter of children with failure or intolerance to first intention therapy.Of the 364 children followed for neurologic bladder in our institution, 22 required endoscopic management between 2000 and 2012. Urinary incontinence was related to detrusor overactivity in 16 children and sphincter deficiency in 13 children, requiring one or several intradetrusor injections of botulinium toxin-A (BTA) and/or dextranomer/hyaluronic acid (Dx/Ha) injection in the bladder neck. Continence was reassessed between six and eight weeks after the last injection using the Schulte-Baukloh score.At the end of the mean follow-up of four years, 16 children received 54 injections of BTA and 13 children had 24 injections of Dx/Ha. Social continence, defined as a score between 0 and 1, was acquired quickly after injection of BTA and required to repeat the injections every 8.7 months (6-12) with a very low morbidity. After the first injection of Dx/Ha, 69% of the children improved significantly their incontinence score (from 1 to 0 or from 2 or 3 to 1) with better results for girls.An appropriate endoscopic management has enabled an improvement of the continence of two-thirds of children who fail first intention treatment for their neurogenic bladder. This is an alternative to delay or avoid major surgery.

Collagen-coated Vicryl mesh (C.C.V.M.) was used in 28 children who needed repair of thoracic and abdominal wall defects. Herein the authors report the clinical experience, surgical technique, and outcome in these cases.

To clarify bladder and bowel function of children with lipomas of the conus, without, before and after neurosurgery.Retrospective analysis of 114 children with a lipomas of the conus, followed in our pediatric neuro-urology department from 1993 to 2010. Several data were collected: bladder and bowel symptoms, bladder and anorectal continence, neurosurgical indication and age, clinical modification after neurosurgery, investigations carried out in pre- and post-surgery treatment, associated bladder and bowel treatment.Forty-nine of the 77 children (63.6%) operated on had never been seen before surgery in our neuro-urology department. Seventy-seven children (67.5%) underwent a neuro-surgery, 60% indicated due to a neurogenic bladder. Before neurosurgery, 66 children (85.7%) had spontaneous miction. Five children (6.5%) had bladder intermittent catheterization. Forty of these patients (56.3%) were continent. After neurosurgery and a specialized consultation in neuro-urology, 54 children (70.1%) were continent. Thirty-seven children (48%) had spontaneous miction. Thirty-seven children (48%) had bladder intermittent catheterization and drug of overactive detrusor. Fifty-two children (67.5%) were constipated after surgery. Seventy-seven percent of the treatments for bowel symptoms were effective in terms of continence.The existence of a neurogenic bladder was one of the main indications for neurosurgery. These results suggest that the complexity of care requires neurosurgical, urological surgeon and neuro-urology physician to achieve the explorations and urinary and digestive treatment in order to preserve renal function and both continences.

The association of a pelvic hemangioma and malformations in the pelvic region are described as SACRAL or PELVIS syndrome. More recently, the acronym of LUMBAR syndrome has been used to describe the association of lower-body hemangioma and other cutaneous defects, urogenital anomalies, ulceration, myelopathy, bony deformities, anorectal malformations, arterial anomalies, and renal anomalies. We report herein 2 representative cases.Two girls presented with infantile hemangioma associated with genital malformation. One case was associated with spinal anomalies and the other one with the anus in the genital position.Segmented hemangiomas are commonly associated with extracutaneous abnormalities. By analogy with PHACE syndrome, PELVIS/SACRAL/LUMBAR syndromes describe the association of segmented hemangioma of the lower extremities associated with other trunk or lower-body malformations. The syndrome is often incomplete.

Foregut duplication is a heterotopy of the digestive mucosa. The tongue localization is relatively uncommon, it presents as a cystic lesion. We report a series of five patients presenting with cysts of the tongue.This retrospective study was made on cases of foregut duplication of the lingual area, diagnosed between 1977 and 2008. We documented the patient's age, gender, symptoms, lesion localization, clinical radiological and pathological features, treatment, and outcome.Four boys and one girl were included. Two cysts were diagnosed during antenatal screening, two during early infancy, and one at the age of 12 (after infectious complication). In every case, the diagnosis could be confirmed only after surgical removal.Foregut duplication of the tongue is rare and its incidence is probably underestimated. This diagnosis should be suggested in case of congenital intraoral cysts. Treatment is surgical excision, and pathological examination confirms the diagnosis.

To describe the outcome of neonates with prenatal intestinal volvulus.All neonates with prenatal intestinal volvulus managed in our institution between May 2004 and December 2010 were retrospectively studied. All neonates with prenatal or neonatal diagnosis of prenatal intestinal volvulus were included. We analyzed age at diagnosis, fetal ultrasound (US) scan and magnetic resonance imaging (MRI) findings, clinical signs at birth, surgical findings, management, and postoperative outcome.Ten neonates with prenatal intestinal volvulus were identified. Prenatal US scans or MRI demonstrated evidence of meconium peritonitis in one fetus and bowel dilatation in 2 others. The mean gestational age at birth was 36 weeks (range, 31-38 weeks) and the mean birth weight was 2811g (range, 2050-3700g). One premature neonate developed respiratory distress and required ventilatory support at birth. In 7 neonates, clinical examination showed distended abdomen and emesis, whereas plain abdominal radiographs showed intestinal obstruction. All neonates underwent surgery and all had normal intestinal rotation, except one with total intestinal volvulus secondary to malrotation. Other causes of volvulus were suspected in 4 neonates: mesenteric defect (n=1), intestinal atresia (n=2) and narrow mesentery (n=1). Detorsion of total volvulus, ileostomy, or intestinal resection with primary anastomosis was performed in 2, 5, and 3 neonates, respectively. One patient with total intestinal volvulus secondary to malrotation died, whereas all other neonates survived. In one patient, the postoperative course was complicated by intestinal dysmotility of the distal small bowel requiring a secondary jejunoileostomy. Stoma closure was subsequently performed at 1 year of age with good outcome. One patient developed angiocholitis treated successfully with antibiotics. Median time to initiate enteral feeds was 7 days (range, 4-16 days) and all patients were subsequently weaned from parenteral nutrition. Median duration of parenteral nutrition was 29 days (range, 6-667 days). None of the patients had cystic fibrosis. Unlike postnatal volvulus, most prenatal volvulus occurs without malrotation. Although prenatal volvulus is a life-threatening condition, our results suggest that good long-term outcome can be achieved in most cases.

Abnormalities of the male genitalia have increased in the last 2 decades in numerous developed countries and remain a frequent reason of consultation in pediatric surgery. The diagnostic spectrum is wide, and surgeons should pay particular attention to these abnormalities because of their potential psychological effect. Anatomically, these abnormalities can affect one of three parts of the penis. First, the foreskin may not be fully retracted. This is normal at birth and can be caused by prepuce adherents that can continue until adolescence. Today, true phimosis is treated with topical corticoids from the age of 3 years. If medical treatment fails, a surgical procedure is required. Second, the urethra can be affected by hypospadia, which is the most frequent abnormality of the urethra. It is associated with ectopic urethral meatus, hypoplastic foreskin, and penis curvature. Its pathogenic background is not clearly understood. Surgery options differ according to the type of hypospadia and according to the surgeon's experience. It is sometimes hard to deal with, especially in a perineal form, where genetic and hormonal studies are recommended. These interventions can lead to complications ranging from stenosis to fistula. Therefore, parents have to be informed of the benefits and risks of the surgical procedures. Epispadias is rare but more serious because of the increasing risk of urinary incontinence. Finally, abnormalities of the corpora cavernosa - often associated with hypospadias - can include penis curvature and micropenis, for which an endocrinological analysis is essential.

Choledocho-ureteral anastomosis is a new experimental model of total bile diversion in the rat. The anastomosis is carried out using a polyethylene tutor drain and requires neither an external drain nor restraint of the animal. After 14 days of diversion, bile flow fell by 53% and there were no biological signs of cholestasis. The rats survived for up to 2 months. Choledocho-ureteral anastomosis is a reliable and simple experimental model which can be used to study the effects of prolonged interruption of enterohepatic bile-acid circulation.

Ovarian teratoma (OT) is the most common ovarian neoplasm in children. Oophorectomy has been the standard treatment but may impair fertility. The aim of this study was to investigate the feasibility and outcome of ovarian-sparing surgery (OSS) for OT.We retrospectively studied all children treated for OT at a pediatric teaching hospital in Paris, France, between March 1992 and July 2006. OSS was performed when deemed technically feasible in patients who had no lymphadenopathy by preoperative imaging or surgical exploration, normal tumor marker levels, and calcifications on radiographs.We identified 30 patients, including 29 with unilateral OT and 1 with synchronous bilateral OT. Emergent surgery was performed in five patients, among whom four had ovarian torsion requiring oophorectomy and one underwent OSS. Of the 26 OTs in the 25 remaining patients, 10 were managed with OSS and 16 with oophorectomy. Subsequently, ultrasound monitoring detected OT development in the contralateral ovary in 4 (14%) patients, after a median of 3 years (range, 1-14 years); OSS was performed in all four cases. The patient with bilateral synchronous OT, managed by OSS initially, underwent unilateral oophorectomy 3 years later for a recurrence. Overall OSS was performed for 15 (42%) OTs.Our results suggest recommendations for preserving fertility whenever possible without compromising the oncological prognosis. In particular, OSS should be reserved for patients who meet all criteria for localized mature teratoma. Long-term follow-up is crucial.

The aim of this study was to evaluate the prognostic significance of intrarenal reflux (IRR) regarding urinary tract infection (UTI), renal scarring and spontaneous resolution after 3 years of follow up.33 patients (42 refluxing units) with IRR were compared to 27 children (44 refluxing units) with high-grade vesicoureteral reflux (VUR) without IRR (controls) matched for gender, age and VUR grade. All patients received antibiotic prophylaxis during observation and antireflux surgery was performed in children with recurrent UTI. DMSA scan was performed at study entry, and 18 and 36 months.DMSA scores at entry showed a higher proportion of moderate and severe damage in the IRR group (25/42) compared to the control group (16/44) (Chi squared, P 0.03). During follow up the incidence of UTI was similar in the two groups, as well as the stability of DMSA scintigraphy and the rate of spontaneous disappearance of the reflux. A similar proportion of patients underwent surgery (18/33 patients with IRR and 13/27 control patients; Chi squared, not significant).Under medical management, the prognosis for IRR is not different from high-grade VUR without IRR. The presence of IRR does not justify more aggressive management than a high-grade VUR without IRR.

Surgical indications for vesico-ureteral reflux have become more selective and endoscopic treatment has now become part of therapeutic arsenal. However, it is important to know when to operate with uretero-vesical replantation if the ureters are severely dilated, before the acute pyelonephritis becomes recurrent in the setting of persisting reflux in girls or higher grade reflux.

Pyelo-ureteral junction abnormalities in children are most frequently discovered by prenatal ultrasound investigation. Most pyeloplasties by resection-anastomosis of the pyelo-ureteral junction are performed in young infants, usually with a posterior approach, patient prone. The indication and type of urinary pyelic drain remain debated: simple nephrostomy or double J drain. Ureteral-caliceal anastomosis is an interesting approach to rare surgical reoperations, particularly with major pyelo-ureteral dilatation. Laparoscopic surgery may prove usefulness in less small children. Endopyelotomy gives good results for post-operative stenoses.

With the progress of foetal ultrasonography, diagnosis of foetal tumors can be made before birth. We report five cases of prenatally detected neuroblastoma of the adrenal glands. Diagnosis was made during the third trimester in all cases. At birth no clinical sign related to the tumor was present, and urinary catecholamines were within normal ranges. On ultrasound scans the tumor was cystic in 1 case, solid in 2 cases and of mixed echogenicity in 2 cases. All children were operated on without pre or postoperative chemotherapy. In each setting the tumor was a stage I neuroblastoma according to the Evans classification. All children are alive and disease-free with a follow-up of 32 months at 14 years.

Achalasia of the cardia is rare in children. We report our experience in the management of 20 children with achalasia.Twenty children (seven girls including two sisters) with achalasia were studied, seven of them had a morbid association. Age at diagnosis ranged from eight months to 18 years (med: 6.4 yrs). Duration of symptoms prior to diagnosis ranged from one to 62 months (med: 8 months). Regurgitations, weight loss and recurrent pneumonias were the most common presenting symptoms. Diagnosis was established using esophageal manometry, which showed aperistalsis throughout the esophageal body with impaired relaxation of lower esophageal sphincter in all children, and chest x-ray and barium esophagram, which were abnormal in 11 and 18 children respectively.Nifedipine used in nine children was ineffective. Two balloon dilatations were performed in one child with a poor result. Surgery with Heller's myotomy combined with an antireflux procedure was performed in 19 children. One child died six months later due to an hypoglycemic coma. During the follow-up (6 months-21 years) symptoms reappeared in five children. Seventy-two percent of the children had no symptoms one year after the surgery and 45%, five years after. Esophageal manometry performed after surgery in seven children showed a normal lower esophageal sphincter pressure but with impaired relaxation, and an aperistalsis throughout the esophageal body. These results justify prolonged follow-up of children with achalasia of the esophagus.

Antenatal diagnosis of urinary tract anomalies raises the question of appropriate therapeutic management. Two risks should be avoided: excessive treatment, belated treatment. Postnatal management of these infants should be determined before birth. The aim of this study is to define indications more precisely.A comprehensive review of the literature on postnatal treatment of antenatally diagnosed urinary tract anomalies was performed in order to propose a model of immediate management.Neonates with hydronephrosis should be placed on antibiotic prophylaxis. Postnatal sonographic exploration should be done immediately after birth in case of bilateral pathology, and if renal function is compromised: posterior urethral valves, bilateral dilatation with parenchymal thinning; exploration is done at day 10 in other cases, when diuresis is normalized. Voiding cystourethrogram is performed soon after birth if posterior urethral valves are suspected, later in other cases. In cases of intermittent renal pelvic dilatation, often an indirect sign of reflux, the need for a voiding cystogram is controversial. Diuretic renogram with mercaptoacetyl triglycine (MAG 3) is used to access renal function and efficiency of kidney drainage, both are needed for later monitoring. Surgical treatment is indicated in the event of deteriorated renal function with permanent obstruction. The most common uropathies are ureteropelvic junction obstruction, vesicoureteral reflux, posterior urethral valves, and primary obstructive megaureter.Antenatal diagnosis of urinary tract anomalies allowed immediate prophylactic treatment of urinary infections, and decrease the risk of severe complications previously observed. Long-term beneficial effects on preserving the renal parenchyma are yet to be determined.

Pour etudier le risque d'abces de paroi chez les enfants pris en charge en hospitalisation de jour, deux series d'enfants operes par voie inguinale durant 5,2 ans dans le meme departement, soit en hospitalisation ambulatoire, soit en hospitalisation traditionnelle, sont comparees. Il apparait que la frequence des abces de paroi inguinaux est moins importante en hospitalisation de jour (1 %; 18/1816) qu'en hospitalisation traditionnelle (4 %; 17/427). Cette difference est due d'une part au mode d'hospitalisation ambulatoire, et d'autre part, a la difference de population induite par les criteres de selection pour l'admission en hospitalisation de jour

Gallstones are frequently encountered in sickle cell disease. Their complications are difficult to distinguish from vaso-occlusive abdominal pain and they can sometimes threaten the patient's life. The aim of this study was to describe our local experience with cholelithiasis in children with sickle cell disease.We analyzed the follow-up records and abdominal sonography results of 185 children with sickle cell anemia, aged zero to 18 years, followed up in Trousseau Children's Hospital (Paris) from 1982 to 1998.Cholelithiasis was detected in 26 patients. The youngest patient was five years old. Cholelithiasis was discovered because of clinical manifestations in 12 patients. Asymptomatic cholelithiasis patients developed clinical manifestations in 28% cases in a maximum delay of two and a half years after its diagnosis. Laparoscopic cholecystectomy was performed in nine cases and open cholecystectomy in 17 cases. The mean postoperative length of stay was significantly shorter in the group of patients with laparoscopy in comparison with the group with open cholecystectomy. Histologic analysis of the gallbladders noted 85% of acute or chronic cholecystis.We suggest that cholelithiasis should be carefully sought in the presence of abdominal manifestations in sickle cell patients. We recommend that annual abdominal sonography be performed in sickle cell patients as early as seven years of age and elective cholecystectomy be performed on patients with cholelithiasis.

Brachytherapy has been widely used at the Institut Gustave-Roussy since 1972 in pediatric oncology. In genitourinary rhabdomyosarcoma, because of its ballistic and physical characteristics, it represents the optimal treatment whenever irradiation is required and brachytherapy feasible. Between 1976 and 1998, 23 children with bladder or prostate rhabdomyosarcoma were treated with a protocol including brachytherapy, with five of them treated with a salvage brachytherapy. All but one brachytherapy was performed during the surgery. Among the 18 brachytherapies performed as a first-line treatment, eight presented a tumoral evolution: five presented a local evolution, one a local and nodal evolution and two a nodal evolution. Brachytherapy allowed a conservative treatment among ten out of 11 children alive with no evidence of disease. Among the five patients with salvage brachytherapy, two presented a second recurrence. Sequelae were minimal, consisting of one grade I rectitis and one asymptomatic vesical and ureteral reflux. These results are consistent with the published data using more radical treatment. Brachytherapy can represent an alternative to radical surgery, when indications are clearly defined in bladder or prostate rhabdomyosarcoma. This type of treatment can be performed only integrated with other treatments, more particularly with surgery. This approach requires a close cooperation between the different specialists: pediatricians, surgeons and brachytherapists.

The aim of this study was to report the results of unilateral Pickrell procedure in children with fecal incontinence secondary to anal atresia.Twenty three children were operated between 1975 and 1997. The mean age at the time of the operation was 8.5 years. Functional results were systematically evaluated by Kelly's clinical score and by anal sphincter electromyography and anal manometry for some patients.There was no postoperative mortality. One colostomy was performed for recurrent local sepsis. The functional result with a mean follow-up of 6 years was estimated to be good in 25%, intermediate in 45% and poor in 30%. Clinical improvement of continence was obtained in 70% of cases.The improvement of continence after Pickrell procedure essentially depends on the patient selection criteria, pre- and postoperative rehabilitation and regular enemas. In the case of poor results, the authors propose bilateral Pickrell procedure which improved the results in eight out of nine children in this group.

Torsion of the spermatic cord is frequent in neonates suffering from acute scrotal distress. A retrospective study was carried out to determine the viability and the outcome of testis treated by surgical exploration.Surgical exploration of 30 neonates presenting acute scrotal distress found: 18 torsions of the spermatic cord, six intrascrotal bleedings or hematoma due to delivery trauma, five tunica vaginalis inflammations and one ischemia without torsion. The torsion occurred in utero in 16 cases and after birth in two cases. The contralateral uninvolved testis was fixed to the scrotum in all cases. The torsion was extravaginal in 17 cases and intravaginal in one case. In all cases of prenatal torsion, there was total necrosis of the testis. One case of postnatal torsion operated on four hours after the beginning of the torsion was saved.Although the viability of the torsioned testis is compromised in cases of prenatal torsion, surgical exploration is necessary to confirm diagnosis and to avoid any contralateral torsion because bilateral torsion exists. Only emergency surgical exploration can save a testis that has undergone post-natal torsion as reported in others series.

This method was used in 14 children, seven boys and seven girls, aged from 2.7 to 14 years, with acute appendicitis and peritonitis.Conversion to median laparotomy was necessary in order to perform adhesiolysis in two cases in which small bowel was observed. Postoperative complications occurred in four patients involving three wound infections and one mechanical intestinal obstruction. There was no intra-abdominal abscess.This procedure allows appendectomy by an open classical approach to be performed, and subsequently allows the treatment of the generalized peritonitis by video laparoscopy, usually without median laparotomy.

To evaluate the morbidity of ambulatory repair of anterior hypospadias with a transurethral vesical catheter (TUVC) draining the urine between 2 diapers.34 children divided into two comparable groups were operated for anterior hypospadias. Group A comprised 17 children operated by ambulatory surgery, with catheter drainage of urine between 2 diapers. Group B consisted of 17 children operated by a conventional inpatient procedure, with the catheter connected to a urine collector. Postoperative complications and the results of a satisfaction questionnaire sent to the parents of children in group A are reported.In group A, one case of TUVC dysfunction was reported and 13 parents declared to be satisfied. One urethral fistula occurred in group B.Ambulatory repair of anterior hypospadias with TUVC draining urine between 2 diapers does not induce a higher complication rate than when repair is performed by conventional hospitalisation with the TUVC connected to a urine collector. Urine drainage by TUVC is not a contraindication to ambulatory surgical repair of anterior hypospadias in children.

The aim of this prospective study was to evaluate the laparoscopic diagnosis of contralateral patent processus vaginalis in children with unilateral inguinal hernia.Between November 1995 and February 1998, 91 consecutive children (78 boys, 13 girls) under the age of 1 year were operated on for a unilateral inguinal hernia. A contralateral hernia was diagnosed by a laparoscopy through the inguinal hernia sac before ligation. Results of this technique were correlated with those of herniography (79 cases) or with inguinal exploration (12 emergency).Laparoscopy was performed in 88 patients. Laparoscopy was impossible in three cases: one inguinal sac too thin, two cases of ectopic testis in the inguinal canal. Sensitivity was 73% and specificity 92%. The only complication arising from the procedure was wound infection in two patients.This method is a simple, safe and accurate procedure in order to select children for contralateral surgical exploration.

Definition of prognostic factors for torsion of the testis in children.From January 1986 to December 1996, 91 children were operated for torsion of the testis. Torsions were observed at all ages of childhood, but with two frequency peaks: during the neonatal period and in adolescence. Typical clinical features were observed in only three-quarters of cases.Thirty-nine testes were considered to be necrotic versus 52 with a chance of recovery. Factors of poor prognosis were: neonatal forms, delayed management or tight torsion with several turns. 28 of the necrotic testes were conserved: two were complicated by purulent necrosis, but 14% retained normal trophicity on long-term examination.The possibility of torsion at all ages of childhood and the frequently incomplete clinical features means that emergency surgical exploration must always be performed in the case of testicular pain. A conservative attitude is motivated by the possibility of recovery of testes considered to be necrotic at operation.

This study evaluates the place of percutaneous nephrostomy (PCN) in neonates with posterior urethral valves (PUV).11 neonates with PUV, 10 of which were diagnosed antenatally, were treated. PCN drainage was performed in 9 cases, prior to endoscopic resection. Six PCN were bilateral and 3 were unilateral, for the following indications: renal failure in 5 cases, severe infection in 2 cases, evaluation of a dilated kidney in 2 cases.These PCN were left in place for an average of 18 days. Two cases of asymptomatic postoperative bacteriuria were observed. One nephrectomy and one cutaneous ureterostomy followed by reimplantation for megaureter were performed on the diverted kidneys.PCN has become the method of upper tract diversion in most cases of neonatal PUV. In addition to the classical indications (severe urinary tract infection, renal failure), it allows perfectly safe resection of valves in the case of upper tract dilatation and assessment of the kidneys.

Intestinal stenosis following necrotizing enterocolitis (NE) occurred both in surgically-treated neonates after perforation, distal to an enterostomy and in medically-treated patients developing symptoms of obstruction. It has been proposed to detect stenosis by contrast enema before refeeding in those medically-treated patients. The aim of this study was to compare delay, clinical and pathological characteristics of surgical and medical patients, both after occlusion and prospective contrast studies.Fifteen patients out of 50 with NE observed from 1984 to 1994 developed one or several intestinal stenosis. Diagnosis of NE was based on usual clinical signs, X-ray pneumatosis (43 to 50) and/or perforation in 16 cases. Among these 16 surgical patients, 12 survived the initial perforation. Among the 34 medical patients, 11 were seen before 1989 and did not have contrast studies before refeeding; 23 seen after 1989 had a contrast enema before.One or several stenosis occurred in four out of 12 surgical patients, four out of 11 medical patients without prospective contrast studies (one of them died from sepsis) and seven out of the 23 of the prospective group. On the whole, 26 stenosis occurred in 15 neonates: ten to the right colon, five to the transverse and 11 to the left colon. One ileal stenosis followed enterostomy. Delay of stenosis development was comparable in the three groups (between 3 weeks and 3 months). Pathologic examination showed similar lesions in the three groups (fibrosis 15, edema nine to 15 and chronic inflammation 12 to 15).Among 46 neonates who survived the initial period, 15 developed stenosis, a 30% proportion similar in patients operated on for perforation or in medically-treated patients whose diagnosis was made after occlusion or after contrast enema as well. These results suggest that systematic stenosis detection by contrast enema may avoid complications and permit programmed one-stage surgery.

Eight cases of rupture of the posterior urethra were observed in children and treated via a trans-symphyseal approach. Repair was easy in the cases seen rapidly (2 to 10 days), urethral suture was effective and the result was excellent. On the other hand, in cases seen later (6 cases), between 6 months and 5 years, marked fibrous callus had to be resected. Urethral repair was not always possible (only one failure out of 6 late cases). This approach preserves genital functions.

The congenital uro-enteral fistula are observed essentially in boy and quite exclusively with anorectal malformation. Cloacal anomaly in girl and cloacal exstrophy in both sexes are so exceptional than complex malformations.

The authors report a case of laparoscopic nephroureterectomy in a four year old girl with a small kidney destroyed by vesicoureteric reflux.

Objectifs La fistule uretrale est une complication frequente apres urethroplastie pour hypospadias, en particulier posterieur. L’objectif de cette etude est d’evaluer la technique de fermeture de fistule avec lambeau de vaginale vascularise (FFLVV). Materiels et Methodes 18 garcons operes a l’origine d’un hypospade posterieur ou moyen ont ete traites a l’âge median de 3 ans (decembre 2005 – 2009) pour fistule uretrale selon la technique de FFLVV par un meme operateur. L’abaissement d’un testicule ectopique a ete associe a la FFLVV dans 6 cas. La technique initiale etait une urethroplastie de Koyanagi dans 10 cas et 16 patients etaient de premiere main; 8 patients avaient eu prealablement un echec de fermeture directe. La topographie de la fistule etait penienne posterieure (11), penoscrotale (4) ou penienne moyenne (3). Resultats Aucune complication post-operatoire ni recidive de fistule n’est survenue avec un recul moyen de 22 mois. Aucune atrophie testiculaire n’a ete observee. Conclusion La FFLVV est une technique tres fiable pour les fistules survenant apres chirurgie des hypospadias posterieurs et moyens. Cette constatation amene a discuter l’interet preventif de l’interposition de ce lambeau lors de l’urethroplastie initiale.