[From paediatric urological care to adult urology. Assessment of a transition consultation for adolescents]. / De l'urologie pédiatrique à l'urologie adulte, quelle prise en charge de l'adolescent ? État des lieux d'une consultation de transition en urologie.

To provide an adequate lifelong urological care in the complex period of adolescence, a transition consultation conducted by a paediatric surgeon and an urologist was developed in our institution. As a real rite of passage, it allows the follow-up and the adapted care of urological conditions, sometimes complex, and permits the transition between childhood and the world of grown-ups. We reported our experience at the Children Hospital of our institution (paediatric surgery and urology departments). During a 6 months period (January-July 2015), forty-five young adults with a mean age of 17.8±3.6 years were seen in transition consultation. Eight patients had neurogenic voiding disorders (4 spina bifida, 1 multiple sclerosis, 1 mitochondrial encephalopathy, 1 metachromic leucodystrophy, 1 paraplegia), 9 patients had idiopathic voiding disorders, 1 patient had a non obstructive malformative uropathy; and 30 patients had surgery during infancy and childhood: hypospadias in 17 young men and malformative uropathy in 13 patients. This consultation occurred within 4.6±4.5 years after the last consultation with paediatric surgeon. For 6 patients, the transition consultation was the first for the urological problem. After this consultation, 8 patients stayed in paediatric surgery and 37 patients were referred to adult urologist. Among those 8 patients: 2 patients had cognitive and psychiatric disorders; 4 patients refused to be transferred to adult unit; 2 patients wanted to come back at transition consultation. Among the 37 patients transferred in adult urological care: 6 patients had urological surgery, and one patient was referred to a sexology consultation. The remaining 30 patients have initiated long-term monitoring. All reconvened patients came back at the follow-up visit (at least 12 months follow-up). A 16-year-old patient (spina bifida with polymalformative syndrome) developed a depressive syndrome at the end of the consultation, in the motive of an awareness of the definitive nature of his handicap and the need of medical follow-up throughout his life. Transition consultation makes easier the passage from paediatric care to adult urological care. It allows a smooth change of interlocutors, facilitates subsequent care and improves compliance to medical follow-up. It requires a good collaboration between paediatric and adult care units. Transition responds to an increasing request of adolescents, families, and medical teams, since care rupture during adolescence can have functional and psychological consequences. LEVEL OF PROOF: 4.

[Long-term outcomes after hypospadias surgery: Sexual reported outcomes and quality of life in adulthood]. / Vie sexuelle des jeunes adultes opérés dans l'enfance d'un hypospadias postérieur.

OBJECTIVE: To evaluate outcomes and long-term sexual quality of life after hypospadias surgery. Seventeen-years-old patients operated for a posterior hypospadias in childhood were included in a transversal study. PATIENTS AND METHODS: Fifteen patients, among the forty children treated since 1997, accepted to participate. These young men (mean age at the first surgery was 27.9±20months) were clinically reviewed and responded to questionnaires (EUROQOL 5, IIEF15 and non-validated questionnaire). This study arises about 8.4±5years after the last visit in paediatric department. RESULTS: Mean study age was 21.2±4.7years. One third of patients thought that global quality of life was distorted. Although 33% of the patients had erectile dysfunction, 80% were satisfied with their sexual quality of life. The most important complains were relative to the penile appearance. Number of procedures was not predictive of patient's satisfaction about penile function and appearance. Thirty-three percents of the patients would have been satisfied to have psychological and medical support. They would be interested in having contact with patients who suffered from the same congenital abnormality. CONCLUSION: These patients had functional and esthetical disturbances. This visit leads to a specific visit in 20% cases. In this study, medical follow-up does not seem to be counselling and had to be adapted. Adequate follow-up transition between paediatric and adult departments especially during adolescence seems to be necessary.

[Tibial nerve transcutaneous stimulation for refractory idiopathic overactive bladder in children and adolescents]. / Stimulation transcutanée du nerf tibial dans le traitement des hyperactivités vésicales réfractaires de l'enfant et de l'adolescent.

INTRODUCTION: The purpose of this study was to evaluate safety and tolerability of transcutaneous tibial nerve stimulation (TENS) in patients under 15years of age with refractory overactive bladder. MATERIALS AND METHODS: A retrospective analysis was conducted on outcomes of TENS (1daily 20-minute session, 10Hz) in patients with refractory overactive bladder, excluding patients with neurogenic bladder. Treatment efficacy was evaluated on symptomatic improvement and voiding schedule. Healing was defined as following: no recurrence of urinary tract infection, normal urodynamic voiding parameters, no nighttime continence disorder, normal uroflowmetry. RESULTS: Nineteen consecutive patients with refractory overactive bladder were treated from November 2010 to March 2012 (11girls, 8boys, age 12.1±2.7 years). Three patients reported only daytime voiding disorders, the others reported daytime and nighttime voiding disorders. Ten patients reported febrile urinary tract infection (1 boy, 9 girls). The average length of treatment was 6 months. Two patients were lost to follow-up. Thirteen patients had only tibial TENS; 3 patients had tibial TENS and trospium chloride or desmopressin. At 1-month assessment, 16 patients out of 17 (94%) reported symptomatic improvement. At the end of treatment, 12 patients out of 17 (70%) met healing criteria (5 boys, 7girls), without relapse within 9 months. Three boys (18%) had partial improvement (no daytime wetting, but increased daytime frequency). No patient reported side effects. CONCLUSION: Tibial TENS is a safe, non invasive and effective treatment in refractory overactive bladder in children. The success rate is 70%, with no side effect and no relapse at the end of the treatment in our study.

Free tubularised vesical mucosa graft for congenital stenosis of the urethra in children.

BACKGROUND: Reconstruction of urethral strictures in children remains a challenge to the pediatric surgeon as these are often related to different rare congenital anomalies with various clinical presentations that endanger renal function and should be repaired in young children. Multiple techniques have been described for their repair. We aimed to determine whether the use of a free tubularised bladder mucosal graft associated to a prior tubeless vesicostomy was feasible and sure, as this technique of reconstruction using tubularised grafts has not been described yet in young children. RESULTS: Two newborn male patients were referred to our department. Both presented a congenital stenosis of the urethra as a part of a complex urethral malformation. Surgery involved prior tubeless vesicostomy, free bladder mucosal graft for urethral reconstruction, and vesicostomy closure for both children. Postoperative evolution was satisfying in both children and cystourethroscopy showed permeable urethra. Satisfying cosmetic and functional results have been obtained in the two cases. CONCLUSIONS: The prior vesicostomy prevents kidney damage in the context of complex genital and urinary malformations. Bladder mucosa's immunohistological properties are the most similar to those of the urethral tissue, and are appropriate for this type of correction, making our technique feasible and sure. LEVEL OF EVIDENCE: 5.

[Study of renal veins by multidetector-row computed tomography scans]. / Étude radiologique des variations anatomiques des veines rénales par scanner multidétecteurs en coupes fines.

PURPOSE: To determine the prevalence of renal vein variants. To investigate the distribution of renal veins. METHODS: We retrospectively reviewed spiral computed tomography (CT) scans of the abdomen performed during a two-month period. The same protocol was used for all CT scans: same multidetector-row CT scanner (Siemens(®)), 1 to 2-mm section thickness, injection of intravenous iomeprol. The study group included 121 patients, aged 21.7 to 93.4 years (mean age 60.9 ± 15.4 years). The sex ratio was 2/1, with 80 men and 41 women. RESULTS: Seventy-three percent of the study group (88 patients) had no variants of the renal veins. Indeed almost 40% (48 patients) had one artery and one vein on each side, with typical course, and 33% (40 patients) had course and/or number variants of the renal arteries. Variants of the right renal vein consisted in multiple veins in 20.6% (25 cases). We detected no case of multiple left renal veins, but we described variations of its course in 9.1% (11 cases): 5 cases of retroaortic left renal vein (4.1%) and 6 cases of circumaortic left renal vein (5%). Three of these 11 patients had an associated double right renal vein. The probability to have a right renal vein variant was significantly higher than a left one (OR = 2.6, P = 0.01). And we found a significantly higher risk of having a venous variant in women (OR = 2.4, P = 0.04). We detected no case of inferior vena cava variant. CONCLUSION: In our study, prevalence of a circum- or retroaortic left renal vein appeared higher than previously reported in the literature (9.1%). Knowledge of anatomical variants of renal vasculature is crucial and this study puts the emphasis on variations of course and number of renal vessels. Those variations are not so uncommon and should be known by radiologists and also by surgeons. Their knowledge has major clinical implications in practice and it contributes to the safety of renal and retroperitoneal surgery.

[Primary obstructive megaureters: long-term follow-up]. / Méga-uretères primitifs : étude rétrospective sur dix ans.

INTRODUCTION: The objective of this study was to evaluate the clinical outcome of primary megaureters and to evaluate predictive factor for surgery need based on ultrasound values. METHODS: A total of 43 primary obstructive megaureters detected in 41 patients have been evaluated between January 2000 and may 2010. RESULTS: Mostly boys (73%) were concerned by megaureters, mainly on the left side (66%). Twenty patients were prenatally diagnosed. Surgical indications were: pyonephrosis (3), recurrent pyelonephritis (14), scintigraphic damage (3), ureteric diameter aggravation (7), disease occurring on unique kidney (2). Regarding the 30 patients who benefit surgery, the retrovesical ureter measured, before surgery 19.15 mm (± 7.17) on average and 3.18 mm, 44 months later. Over the 10 patients treated medically, the initial diameter was 9.91 mm and at the end of the study, five patients had megaureter completely regressed, three patients had a diameter greater than 10mm at the end of the study and two faced a worsening evolution with sudden and complete renal damage on scintigraphic nephrogram, after recurrent pyelonephritis despite antibioprophylaxis. CONCLUSION: We recommend a careful watch-fulling of primary megaureters; mainly for those with recurrent infections and whenever the retrovesical ureter diameter exceeds 14 mm at first ultrasound.

[Testicular torsion in children: Factors influencing delayed treatment and orchiectomy rate]. / Torsion du cordon spermatique chez l'enfant : impact du mode de consultation sur le délai de prise en charge et le taux d'orchidectomie.

INTRODUCTION: Acute scrotal pain is a true surgical emergency as patients presenting with acute scrotal pain may suffer from spermatic cord torsion and gonadal loss. We assessed whether the type of consultation (first consultation in our center or secondary transfer from a peripheral hospital or primary care practice), distance from home to hospital, and duration of pain had an impact on the orchiectomy rate. PATIENTS AND METHODS: We retrospectively reviewed the medical records of all patients under 15years of age suffering from acute scrotal pain who had surgical exploration between January 2007 and January 2010 in our center. Patient demographics, transfer status, time to consultation in our center, time to surgery, operative findings and clinical outcome were reviewed. RESULTS: Of the 76 patients with acute scrotal pain in whom surgical exploration was performed, 59 had acute spermatic cord torsion, 16 had torsion of the testicular appendage, and 1 had orchitis. In patients with acute spermatic cord torsion, the median age was 13 years (range: 0.18-14.97). In patients with acute spermatic cord torsion, 32 came straight to our center (direct admission group, 54.2%), and 27 (45.8%) came after a prior consultation out of side the center (transfer group). The median journey was 19km (range: 2.5-113) in the direct admission group and 44km (range: 2.5-393) in the transfer group (P=0.0072). The median time between pain onset and consultation at our center was 4.3h (range: 0.5-48) in the direct admission group, and 11h (range: 2-48) in the transfer group (P=0.6139). The median time between admission at our center and surgery was 2.5h, with no difference between the 2 groups (P=0.8789). The orchiectomy rate was 25% in the direct admission group and 14.8% in the transfer group (P=0.5177). In children who underwent orchiectomy, the duration of pain was consistently over 6h. The duration of pain was greater in patients with orchiectomy (12h [range: 1-72]) than in patients without orchiectomy (12h [range: 6-48]; P=0.0001). CONCLUSION: In this study, the orchiectomy rate depended on the duration of pain but not on transfer status. Acute scrotal pain must lead to surgical exploration as soon as possible, requiring close collaboration between peripheral hospitals, primary care physicians, and referral centers.

[Is there an outcome difference between posterior urethral valves diagnosed prenatally and postnatally at the time of antenatal screening?]. / Impact de l'âge au diagnostic sur le devenir à long terme des patients opérés de valves de l'urètre postérieur.

PURPOSE: Posterior urethral valves (PUV) diagnosed during childhood have classically been associated with a better outcome than antenatally diagnosed PUV. The aim of our study was to compare long-term outcome of these two patients' groups. MATERIAL AND METHODS: We retrospectively reviewed the medical records of boys with PUV managed between 1990 and 2010. Patient demographics, clinical background, radiographic data (including prenatal ultrasonography data when available), renal and bladder functional outcomes, surgical procedures and urinary tract infections (UTI) were abstracted. Impaired renal function (IRF) was defined as glomerular filtration rate less than 90 mL/min/1.73 m(2) at last follow-up. RESULTS: We identified 69 patients with confirmed PUV. Thirty-eight were diagnosed prenatally (group 1) at 30.5 weeks of gestation and 31 had a delayed diagnosis (group 2) at a median age of 6.31 years. At diagnosis, 20 patients in group 1 had renal insufficiency versus two in group 2 (P<0.05). At the end of mean follow-up of 7.2 ± 0.5 years, in group 1, 26.3% developed IRF versus 6.3% in group 2 (mean follow-up 2.3 years). Mean age at last follow-up was 7.3 years in group 1 versus 8.3 in group 2 (P>0.05). In group 1, 27% had voiding dysfunction versus 30% in group 2 (NS). In group 1, 35% had UTI during follow-up versus 10% (P=0.01). CONCLUSION: During the follow-up, the patients with delayed diagnosis VUP have developed fewer complications related to the initial obstruction than the population who was detected antenatally and managed from the early hours of life. However, the rate of IRF and voiding disorders in our study, associated with the data of the literature, highlights the potential persistence and worsening of these conditions. That is why, whatever the age at diagnosis, VUP patients require a close monitoring.

[Distal and late ureteral obstruction: a rare complication following dextranomer/hyaluronic acid injection for vesicoureteral reflux in children]. / L'obstruction urétérale distale secondaire : une complication rare du traitement endoscopique par Deflux(®) du reflux vésico-urétéral de l'enfant.

Surgically relevant obstruction after dextranomer/hyaluronic acid injection (Dx/Ha, Deflux(®)) for the treatment of vesicoureteral reflux (VUR) is rare with a 0.6% incidence. It occurs usually during the early postoperative period. We report here the case of a 9-year-old boy with a history of VUR who was previously treated with Deflux(®) and was referred more than 2 years later with acute flank pain (as he already did 2 weeks after surgery with a spontaneous relief under medical treatment). Initial radiological investigations showed hydronephrosis caused by distal ureteral obstruction which required open surgery removal of the Dx/Ha and Cohen procedure. This is the second case of delayed symptomatic obstruction due to Dx/Ha reported in the literature.

[Proteus syndrome: Case report of bladder vascular malformation causing massive hematuria]. / Une malformation vasculaire vésicale rare dans le cadre du syndrome de Protée.

Proteus syndrome is a rare, sporadic disorder consisting of disproportionate overgrowth of multiple tissues, vascular malformations, and connective tissue or epidermal nevi. Due to mosaic pattern of distribution, the phenotypes are variable and diverse. Vascular malformations are part of the major criteria used to define and diagnose this syndrome. It can involve the gastrointestinal tract, spleen, or the urinary tract but bladder malformations are rare. We report here a case of bladder vascular malformation in a 12-year-old boy known to have Proteus syndrome and review the literature on bladder malformations or tumors in this syndrome.

[Functional damages after blunt renal trauma in children]. / Séquelles fonctionnelles après traumatisme rénal chez l'enfant.

PURPOSE: The purpose of this study was to determine the long-term functional outcome of renal injuries. METHODS: We retrospectively reviewed the charts of patients under 16 sustaining renal trauma and admitted to our department between 1990 et 2010. There were 66 renal lesions categorized as follows: grade I to III, 33, grade IV, 28 and grade V, 5. Whatever their initial status, all children were followed using ultrasonography or computed tomography. After complete healing, data of technetium-99m-dimercaptosuccinic acid nuclear were collected. RESULTS: There was no bilateral injury. Thirteen patients proceeded to laparotomy leading to nephrectomy in three cases, partial nephrectomy in two others cases. Four renovascular injuries required interventional radiologic management. Nine urinomas were managed with eight stentings and one percutaneous drainage. Percentage of renal function by technetium-99m-dimercaptosuccinic acid nuclear scanning concerning 26 patients was analysed. Split percentage of renal function was 43.4% (±6.2%), 35.7% (±5.3%) et 30.3% (±12.2%) (mean±SD); P=no significant, for grade I-III, IV and V, respectively. CONCLUSION: Functional outcome after blunt renal trauma appeared influenced by injury grade. Functional sequelae existed even with low-grade traumas. In keeping with literature, these results justified a close follow-up in which radionuclide study was the essential element.

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.

[Retrotracheal left pulmonary artery: case report with an embryologic and anatomic update]. / Artère pulmonaire gauche rétrotrachéale : mise au point embryologique et anatomique.

The accidental discovery of a retrotracheal left pulmonary artery in a 4-month-old infant encouraged us to review the various embryologic theories concerning this very rare anomaly and perform an anatomic update in order to better define surgical treatment. Nathan underwent surgery for a bilateral inguinal hernia at the age of 4 months. The postoperative period was marked by malaise associated with dyspnoea, stridor, tachycardia and sweating. A X-ray of the thorax, oesophageal transit and angio scan presented an intertracheo-oesophageal left pulmonary artery and a reimplantation of the left pulmonary artery was successfully performed. A retrotracheal left pulmonary artery is a very rare malformation. From development of pulmonary vascularisation, three embryologic theories have been advanced to explain this anomaly. From an anatomic point of view, Landing et al. proposed in 1982 a classification system of retrotracheal left pulmonary artery. Today, current radiological techniques not only provide a precise diagnosis but also make it possible to define appropriate care for the different types of this malformation.

Congenital anterior urethrocutaneous fistula associated with a stenosis of the bulbar urethra in the context of high anorectal malformation without fistula.

SUMMARY: Congenital anterior urethrocutaneous fistulas are infrequent. We report a case of a congenital anterior urethrocutaneous fistula associated with a stenosis of the bulbar urethra in the context of a high anorectal malformation. We describe the surgical technique for the reconstruction of the urethra.

Quality of life and continence in patients with spina bifida.

PURPOSE: Spina bifida (SB) is the most common congenital cause of incontinence in childhood. This study attempts to determine the relationships between urinary/faecal incontinence, methods of management, and Health Related Quality of Life (HRQoL) in people with SB. PATIENTS AND METHOD: A total of 460 patients (300 adults and 160 adolescents) from six centres in France have taken part in this cross-sectional study. Clinical outcome measures included walking ability, urinary/faecal continence, and medical management. HRQoL was assessed using the SF36 in adults and the VSP in adolescents and their parents. Univariate and multivariate analysis was used to determine the relationships between clinical parameters and HRQoL. RESULTS: HRQoL were significantly lower than in the general population. Adult women had significantly lower scores than men, and adolescent females had significantly lower scores for psychological well being. We did not found strong relationship between incontinence and HRQoL in this population. Moreover patients surgically managed for urinary/fecal incontinence did not show significantly higher scores of HRQoL. CONCLUSION: Using generic HRQoL measures, urinary/faecal incontinence and their medical management may not play a determinant role in HRQoL of persons with SB. However many other factors affect HRQoL in these patients. A longitudinal study design is recommended to assess whether incontinence management is associated with improved HRQoL.

Apparent mismatch between extended x-ray absorption fine structure and diffraction structures of crystalline metastable WO3 phases.

The local structure of monoclinic, monohydrate, hexagonal, and pyrochlore WO3 phases was investigated by the extended x-ray absorption fine structure spectroscopy as preliminary studies of model compounds of amorphous and thin film WO3 based electrochromic species. In the four cases, we found a large W-O distribution of distances ranging from 1.70 to 2.35 A. The apparent discrepancy of these results and previously published crystal structures are discussed and interpreted as the detection of vacancies and local distortion disorder.

A multicentre study of the management of disorders of defecation in patients with spina bifida.

Patients with spinal dysraphism may have severe constipation and faecal incontinence. The impact of antegrade colonic enema (ACE) in the management of patients with spina bifida (SB) is analysed. In a multicentre cross-sectional study, constipation, faecal incontinence and faecal management were described. Cases surgically treated were identified. Data were collected from 423 patients, of whom 230 did not use any manoeuvre or laxatives to assist evacuation. Conventional treatment was used in 193 patients, including digital extraction in 39%, retrograde enema in 21% and oral laxatives in 52%. For intractable constipation and overflow of faecal incontinence, 47 patients were treated with ACE, of whom 41 used the method at a mean time of interview of 4.1 +/- 1.9 years after ACE operation; six abandoned ACE for conventional management. With ACE, faecal continence was significantly improved compared with conventional management, and neither retrograde rectal enema nor digital extraction were required. The conduit was fashioned to the right colon in 32 cases and to the left colon in nine cases. This study provides information on a multicentre experience in bowel management in SB patients. Whatever the technique used, ACE has improved faecal status compared with conventional therapy.