ABSTRACT Objectives High-risk bladder pattern can be defined by Urodynamic Evaluation (UE) as overactive bladder with detrusor leak point pressure higher than 40 cmH2O and/or higher filling pressures also above 40 cmH2O. We wanted to evaluate response to treatment in myelomeningocele patients operated in utero in this subgroup. Patients and Methods From our prospective cohort of in utero MMC we have identified patients in the high-risk group. Treatment consisted of anticholinergics (Oxybutynin 0.2 mg/Kg) 2 or 3 times daily in association with CIC. At every UE, patients were reclassified in high-risk or low-risk patterns. Patients not responding were proposed bladder reconstruction or diversion according to age. Results Between 2011 to 2020, we have been following 121 patients and 60 (49.6%) of them were initially categorized as high-risk. The initial UE was performed at a mean age of 7.9 months and detrusor overactivity was found in 83.3% (mean maximum pressure of 76.5cmH20). When evaluating patients with 2 or more UE, we identified 44 patients (follow-up: 36.8months). It was observed in the group of patients who underwent 2 to 5 UE, that response to treatment was validated by the finding of 40% of low-risk bladder patterns in the second UE and between 62% to 64% in the third to the fifth UE. The incidence of surgery was 13.3%. Conclusions Early urological treatment of high-risk bladder pattern was effective in approximately 60%. We reinforce the need to correctly treat every patient with myelomeningocele, in accordance with UE, whether undergoing in utero or postnatal treatment.

ABSTRACT Introduction: A challenging situation in proximal hypospadias is the presentation of patients with successful urethroplasty but with persistent or recurrent ventral curvature (VC) after multiple hypospadias repair. Materials and Methods: We present a 13 year-old boy with 7 previous surgeries (long TIP, Duplay, meatoplasty) to treat hypospadias presenting with 60 degrees of VC, in spite of a well-accepted coronally neomeatus. We degloved the penis and artificial erection clearly appointed corporal disproportion causing curvature. We disconnected urethra from corpora. After excision of remnant fibrotic tissue, there was a residual curvature so a lenghtening corporoplasty with dermal graft from groin was performed. We have adjusted the urethral meatus position into a proximal penile shaft. We used a buccal mucosa graft placed in an inverted U-shape position planning a second stage urethroplasty (1). An indwelling silicone Foley tube was left for one week. The patient was discharged the day after surgery. Results: The aspect after corporoplasty proved satisfactory curvature correction. Patient had an excellent outcome and is scheduled for a second-stage after 6 months. Discussion: Snodgrass and Bush (2) reported that on 73 patients with an average of 2.7 operations for proximal shaft to perineal hypospadias; of which, 83% had VC at re-operation averaging 50°. We do believe that some good results with minimal dorsal plicature may recur in adolescence and therefore when these procedures may be considered, they should be performed by classic Nesbit technique (3). Otherwise, the choice for primary ventral lengthening should be taken. Conclusion: Severe curvature associated with hypospadias should undergo a major procedure at early stage to avoid decompensation after dorsal plicature in adolescence. We had a very satisfactory result, the patient awaits the second stage procedure (Figure-1). Figure 1 End aspect after complete healing of buccal mucosa. Notice that penis is straight and second stage will be performed after 6 months of interval.

ABSTRACT Introduction: Vesicostomy should be considered in children with neuropathic bladder in case first-line therapies fail. This simple and reversible procedure can reduce febrile urinary tract infections and protect the upper urinary tract (1) until more definitive alternatives can be proposed. We describe in this video how we approach patients that underwent vesicostomy and want it to be converted into a continent catheterizable reservoir. Material and methods: We perform an infra-umbilical longitudinal incision with a semicircular flap where the stoma will be placed (outside vesicostomy). After releasing the bladder, we proceed with usual steps of the Macedo-Pouch technique (2). We perform the reservoir from 35cm of ileum that constructs a catheterizable channel from the same bowel segment from a 3cm width flap from anterior and posterior wall of ileum in the mid part of it. The continence mechanism of the efferent tube is based on angulation and a serous lined tunnel created with 3-4 3.0 prolene sutures. The stoma is placed in the midline (3). Results: Patient had an uneventful evolution and is continent performing CIC every 4 hours with 9 months of follow-up. We have in the last 3 years a consecutive series of 12 patients operated according to this principle. Discussion: Vesicostomy should be regarded as an alternative for patients with neurogenic bladder refractory to clinical treatment at a younger age in order to postpone definitive treatment such as any an enterocystoplasty. This option must be considered as transient, since definitive reconstructive surgery can provide preservation of renal function and continence achievement. In this context, our video demonstrates that performing a bladder augmentation on a patient with a vesicostomy is safe and feasible. We reinforce that our method precludes the need of appendix or creation of a Monti tube as the outlet channel and the whole procedure is performed from a single piece of bowel.

ABSTRACT Introduction We present an alternative procedure for distal hypospadias consisting of urethral mobilization and partial glandar disassembly, namely GUD (glandar urethral disassembly) technique. Materials and Methods A subcoronal circumcision exposes distal dysplastic urethra. We incise the Buck´s fascia on both sides of urethra releasing it partially from the corpora. We keep a thin bridge of urethral plate to the glans and disassembly almost completely the glans from the corpora, except for the bridge. The glans is incised creating two wide wings that are extremely mobile. The urethra is mobilized, advanced and sutured to the tip of the glans. The glans wings embrace the distal urethra producing a conical glans. Discussion The concept of urethral mobilization has been reported and popularized by Koff in the literature to correct distal hypospadias. One of the limitations of this procedure is the risk of urethral retraction due to extensive proximal dissection. We got inspiration from Mitchell and Bagli’ s work of penile disassembly in epispadias to develop the GUD concept. We adopt minimal urethral mobilization mainly in glandar/proximal penile shaft and complete deconstruction of the glans, detaching the corpora from the glans and rotating the wide glans wings to embrace the urethra. Therefore we avoid suture urethroplasty and refurbish the glans to a better conical shape. Conclusion We are convinced that this operation can be regarded as a genuine alternative to distal hypospadias (coronal and subcoronal) but should not be addressed to midshaft forms.

ABSTRACT Introduction and objective Urethral duplication is a rare congenital anomaly, with roughly 200 cases reported in the literature (1). It is more frequent in males, with few cases reported in females. The clinical presentation differs according to the anatomical variant present. The duplication most commonly occurs in the sagittal plane with one urethra located ventrally and the other dorsally (2). Usually the ventral urethra is the more functional of both. Duplications occurring in the coronal plane are quite rare and they are usually associated with bladder duplication (3). The purpose of this paper was to present a video of a boy with an unusual urethral duplication form. Materials and Methods Patient was born premature due to oligohydramnios at 7 months-gestational age and he has initial diagnosis of hypospadia. Since then, he presented at least 7 febrile UTI and mother complained of difficult micturition and a presence of a mass at lower abdomen. Patient was referred to our institution and we identified urethral duplication with a glandar and scrotal meatus, palpable bladder and left penile-hemiscrotum transposition. US and CT-scan showed left kidney agenesis and overdistended bladder. VCUG and retrograde urethrography showed duplication, presence of contrast in the seminal vesicles and complete catheterizing of both urethras was not possible. Results The topic urethra was dysplastic and not patent to a 4Fr plastic tube so we were unable to access it endoscopically. We performed initially a Mitrofanoff procedure to allow CIC and treat chronic retention. Six months later, we assessed both urethras surgically and concluded that dorsal urethra was dysplastic after 3cm still in the penile area and scrotal urethra was not possible to be catheterized. We excised the ventral urethra because of dribbling complaints up to bulbar area and reconstructed the scrotal transposition, keeping the topic urethra for cosmetic issues. Patient had excellent outcome, performs CIC every 4 hours and has not presented further UTI episodes. Discussion and conclusion The urethral duplication is an anomaly that has multiple anatomical presentations. There are several theories about the etiology, but none can explain all types of presentations. There is also more than one rating available, and the Effmann classification is the most detailed. The case exemplifies this varied spectrum of anatomic urethral duplication. It resembles the urethral duplication type IIa-Y, however, ventral urethra meatus was located in penoscrotal area and both urethras were at least partially hypoplastic/dysplastic associated with obstruction and bladder retention. In determining how to best manage a patient with Y-type urethral duplication, the caliber and quality of the orthotopic urethra must first be assessed. Published reports suggest that best outcomes are those using the ventral duplicated urethra for the reconstruction (4). In this case, none of urethras were functional and a supravesical outlet channel had to be provided. The treatment of this condition requires an individualized planning and a vast technical knowledge of reconstructive surgery.

RESUMO Relata-se caso de reconstrução urinária em gêmeos siameses previamente separados com apresentação clínica de bexiga extrófica e incontinência urinária. Os dois pacientes eram do sexo masculino com idade de 13 anos. O primeiro gêmeo apresentava falha da enterocistoplastia com extrusão e visualização da neobexiga extrófica, tendo sido submetido ao fechamento do colo vesical e à reconfiguração da parede abdominal. Após o procedimento, o paciente desenvolveu fístula, que foi tratada, mas persistiu. Posteriormente, optamos por bolsa cateterizável, descartando a bexiga nativa. O segundo gêmeo foi submetido à construção imediata de bolsa cateterizável, por meio da técnica de Macedo. Atualmente, ambos os pacientes estão continentes em intervalos de 4 horas. O seguimento médio foi de 8 meses. As atuais técnicas de derivação urinária oferecem novas perspectivas e esperança para esta população complexa.

ABSTRACT We report a case of secondary urinary reconstruction of previously separated conjoined twins with exstrophic bladder and urinary incontinence. Patients were male and aged 13-year-old. Twin one had a history of failed enterocystoplasty that extruded and was visible like an exstrophic neobladder. He underwent a procedure to close bladder neck and reconfigure abdominal wall. After the procedure the patient developed a fistula that was treated, but it persisted and, for this reason, a catheterizable pouch was constructed and native bladder was discarded. Twin two required the immediately construction of catheterizable pouch using the Macedo’s technique. Currently, both patients are continent at 4 hour intervals. The mean follow-up was 8 months. Modern continent urinary diversion techniques offer new perspectives and hope for such complex population.

ABSTRACT Purpose: To develop a rabbit experimental study to test the hypothesis that surgical repair of hypospadias with severe ventral curvatures might be completed in one stage, if a graft, such as buccal mucosa, could be placed over the tunica vaginalis flap used in corporoplasty for ventral lengthening, with the addition of an onlay preputial island flap to complete the urethroplasty. Materials and methods: The experimental procedure with rabbits included a tunica vaginalis flap for reconstruction of the corpora after corporotomy, simulating a ventral lengthening operation. A buccal mucosa graft was placed directly on top of the flap, and the urethroplasty was completed with an onlay preputial island flap. Eight rabbits were divided into 4 groups, sacrificed at 2, 4, 8 and 12 weeks postoperatively, and submitted to histological evaluation. Results: We observed a large number of complications, such as fistula (75%), urinary retention (50%) and stenosis (50%). There were two deaths related to the procedure. Histological evaluation demonstrated a severe and persistent inflammatory reaction. No viable tunica vaginalis or buccal mucosa was identified. Conclusions: In this animal model, the association of a buccal mucosa graft over the tunica vaginalis flap was not successful, and resulted in complete loss of both tissues.

Purpose To compare the histological characteristics of keratinized versus non-keratinized onlay island flaps in an experimental rabbit model. Materials and Methods Sixteen male rabbits were randomly allocated into two experimental groups: keratinized and non-keratinized onlay island flaps. A defect was created in the ventral aspect of the penile urethra. In the keratinized group, a longitudinal island flap was harvested from the external prepuce and rotated to cover the urethral defect. In the non-keratinized group a transverse island flap was harvested from the inner prepuce. The animals were sacrificed after 2, 4, 8 and 12 weeks. Results The flaps were viable in all animals, and no deaths were associated with the procedure. Two urethrocutaneous fistulas were identified, one in each experimental group. A similar pattern of fibrosis was identified in both groups. The keratinized epithelium of the external prepuce kept its histological aspect and keratin production. Both keratinized and non-keratinized groups presented a slight decrease on the epithelial thickness, however without a statistically significant difference between groups. Conclusions In this short-term rabbit model, we observed that the stratified squamous keratinized epithelium from the external prepuce kept its keratin production. There was no statistical influence of the flap type on the mean epithelial thickness.

Introduction Cloacal exstrophy is a rare occurrence with an incidence of 1:200,000 to 1:400,000 live births. It represents one of the most challenging reconstructive endeavors faced by pediatric surgeons and urologists. Aside from the genitourinary defects, there are other associated anomalies of the gastrointestinal, musculoskeletal and neurological systems that require a multidisciplinary approach when counseling anxious parents. Material and Methods We present a video of a patient with cloacal exstrophy treated with 21 days of life. Surgery consisted of separation and tubularization of the cecal plate from the exstrophied bladder halves and colostomy construction. The bladder was closed primarily and umbilical scar reconstructed and used for ureteral and cistostomy drainage. A urethral catheter was used to guide bladder neck tubularization. A final epispadic penis was obtained and planned for further repair in a second step. Results The patient had an initial uneventful postoperative course and immediate outcome was excellent. The bladder healed nicely but patient presented with abdominal distension in the 5th day of postoperative setting requiring parenteral nutrition. The distal colon persisted with lower diameter although non obstructive, but causing difficulty for fecal progression. Continuous colostomy dilatation and irrigation were required. Conclusions Approximating the bladder halves in the midline at birth and primary bladder closure is a viable option, intestinal transit may be a issue of concern in the early postoperative follow-up.

Objective To conduct a systematic review on single scrotal incision orchiopexy. Materials and Methods A search was performed using Pubmed, through which 16 articles were selected out of a total of 133. The following conditions were considered exclusion criteria: other surgical methods such as an inguinal procedure or a laparoscopic approach, retractile testes, or patients with previous testicular or inguinal surgery. Results A total of 1558 orchiopexy surgeries initiated with a transcrotal incision were analyzed. Patients' ages ranged between 5 months and 21 years. Thirteen studies used high scrotal incisions, and low scrotal incisions were performed in the remainder of the studies. In 55 cases (3.53%), there was a need for inguinal incision. Recurrence was observed in 9 cases, testicular atrophy in 3, testicular hypotrophy in 2, and surgical site infections in 13 cases. High efficacy rates were observed, varying between 88% and 100%. Conclusions Single scrotal incision orchiopexy proved to be an effective technique and is associated with low rates of complications.

OBJETIVO: Utilizar as medidas antropométricas para comparar o estado nutricional em crianças com bexiga neurogênica secundária à mielomeningocele submetidas a enterocistoplastia àquelas não submetidas à cirurgia. MÉTODOS: Estudo tipo caso-controle conduzido em 20 crianças, divididas em dois grupos, aquelas que realizaram enterocistoplastia (Grupo A) versus crianças não operadas (Grupo B), pareados por gênero e idade. Os parâmetros utilizados foram peso, altura, circunferência do braço e prega cutânea triciptal. A avaliação nutricional foi determinada calculando-se índices baseados na idade e no gênero. A classificação foi baseada em porcentagens, e os resultados foram comparados com valores de referência. RESULTADOS: A média da idade foi 6,41 anos no Grupo A e de 6,35 no Grupo B. O intervalo entre a cirurgia e a avaliação foi de 11 meses. As seguintes medidas foram encontradas para o Grupo A: 80% das crianças eram eutróficas, 30% maior do que no Grupo B; circunferência braquial foi adequada em 40% dos pacientes, 20% maior do que no Grupo B; a área do músculo do braço foi adequada em 90%, 30% maior do que no Grupo B. Os valores no Grupo B foram os seguintes: 60% apresentaram prega cutânea triciptal acima do valor médio, uma porcentagem 20% acima do que no Grupo A; para índice de gordura braquial, 60% dos pacientes estavam acima da média, 40% maior do que no Grupo A. CONCLUSÃO: Pacientes submetidos à enterocistoplastia demonstraram melhor estado nutricional enquanto o grupo controle apresentou índices de gordura mais elevados nas medidas antropométricas. Entretanto, a diferença entre os grupos não foi estatisticamente significativa.

OBJECTIVE: To use anthropometric measurements to compare nutritional status in children with neurogenic bladder dysfunction secondary to meningomyelocele who underwent enterocystoplasty and those who did not undergo surgery. METHODS: A case-control study was conducted in 20 children, divided into two groups: those who had enterocystoplasty (Group A) and those who did not undergo surgery (Group B), matched for genre and age. Weight, height, arm circumference, and triceps skinfold thickness were the parameters used. Nutritional assessment was determined by calculating the indexes, based on age and genre. Classification was based on the percentile and the results were compared with the reference values. RESULTS: The mean age was 6.41 years in Group A and 6.35 years in Group B. The interval between surgery and evaluation was 11 months. The following measures were found for Group A: 80% of children were eutrophic, a percentage 30% greater than that in Group B; arm muscle circumference was adequate in 40% of patients, a percentage 20% greater than that in Group B; arm muscle area was adequate in 90%, a percentage 30% greater than that in Group B. Values in Group B were as follows: for triceps skinfold thickness, 60% of patients had values above the mean, a percentage 20% greater than that in Group A; for arm fat index, 60% of patients were above the mean value, 40% greater than in Group A. CONCLUSION: Patients who had undergone enterocystoplasty showed better nutritional status, while the control group presented higher fat indexes in anthropometric measures. However, the differences between groups were not statistically significant.

INTRODUCTION: Urethral duplication is a rare congenital anomaly affecting mainly boys. Generally, the duplication develops on the sagittal plane; the accessory urethra may run dorsally or ventrally to the orthotopic one. We present a patient with urethral duplication in which the orthotopic urethra was patent in the penile segment but atresic in the bulbar and prostatic segment. The patient had urinary flow from the rectum and the ectopic urethra could be well identified by anal examination. MATERIALS AND METHODS: Age at surgery was 13 months. The procedure consisted of an ASTRA (anterior sagittal trans-ano-rectal) approach for dividing the urethra and rectum and was successful to move the urethra up to the perineal area. The rectum was reconstructed and the patient placed into a lithotomy position. A urethral catheter inserted in the penile urethra oriented us were the atresic urethra in bulbar area started. The scrotum was opened in the middle and the distance between the two urethral stumps proximal and distal defined the extension of no urethral tissue that consisted of 5 cm. We opened the right scrotal space and a tunica vaginalis flap was obtained and attached to the bulbar tissue for a two-stage urethroplasty strategy. RESULTS: Patient had a nice healing and the tunica vaginalis was nicely incorporated to the adjacent tissue, having the two urethral stumps well delineated. CONCLUSIONS: ASTRA approach in combination with a two-stage urethroplasty with tunica vaginalis dorsal flap proved to be an excellent combination for a rare case of urethral Y duplication having the main urethra into the rectum.

PURPOSE: To translate and culturally adapt the Dysfunctional Voiding Symptom Score (DVSS), questionnaire into Brazilian Portuguese. MATERIALS AND METHODS: The 10-item Dysfunctional Voiding Symptom Score (DVSS) was translated into Brazilian Portuguese according to a standard methodology: translation, synthesis, back-translation, Expert Committee, and pre-testing. After the translation process the final version was pre-tested and patient responses were analyzed to identify necessary modifications. Reliability was evaluated using the test-retest method, and internal consistency was assessed using Cronbach’s alpha. RESULTS: The Cronbach’s alpha coefficient was calculated in the test and retest phases. Internal consistency was found to be satisfactory, as confirmed by a Cronbach’s alpha coefficient of 0.76 for the test and 0.77 for the retest. A high degree of stability was found in the test/retest, with an intraclass correlation coefficient (ICC) of 0.960 (p < 0.001; 95% CI: 0.943-0.972). CONCLUSION: The cross-cultural adaptation process of the Dysfunctional Voiding Symptom Score questionnaire to be used on Brazilian children was successfully completed following internationally accepted methodologies.