ABSTRACT Introduction Clitoroplasty constitutes an important step in feminizing surgery for congenital adrenal hyperplasia (CAH) ( 1 ). In this video we present a technique that aims to preserve clitoral sensitivity and engorgement while minimizing the risk of neurovascular lesion. Materials and methods We present a video of a three-year-old girl with history of CAH classical form, PRADER-III, who underwent clitoroplasty. After an initial endoscopic evaluation of the urogenital sinus, the clitoris was degloved and a rectangular incision was made on the ventral corpora cavernosa 15mm above the corpora bifurcation and 0.5 mm below the coronal sulcus. The cavernous tissue was partially resected. The upper and lower borders of the rectangular gap were closed by a 5-0 PDS running suture similar to the Mikulicz technique. Next, the edge of the glans was deepithelialized to reduce its size. For improved clitoral positioning, the clitoris was sutured to the pubic fat. From that point onward the procedure followed that of a standard vaginoplasty using the en-bloc technique ( 2 - 4 ). Thus far we have performed this technique in 33 patients, with 31 of them being girls with CAH and 2 being women with clitoral hypertrophy. Conclusion Corporoplasty is a simplified technique for clitoroplasty, with the advantage being that is faster and safer than the technique that involves the dissection of the neurovascular bundle. In addition, corporoplasty has the possible benefit of preserving the cavernosal blood flow that permits the engorgement of the clitoris during sexual arousal.

ABSTRACT Introduction Vesicoureteral Reflux (VUR) is characterized by a retrograde flow of urine from the bladder into the ureters and kidneys. It is one of the most common urinary tract anomalies and the major cause of urinary tract infection (UTI) in the first years of life. If not properly diagnosed and treated can lead to recurrent UTI, renal scar and, in severe cases, to end stage renal disease. Despite recent advances in scientific and technological knowledge, evaluation and treatment of VUR is still controversial and there is still considerable heterogeneity in evaluation methods and therapeutic approaches. The aim of the present consensus is to give a practical orientation on how to evaluate and treat VUR. Methods The board of Pediatric Urology of the Brazilian Society of Urology joined a group of experts and reviewed all important issues on Vesicoureteral Reflux evaluation and treatment and elaborated a draft of the document. On November 2017 the panel met to review, discuss and write a consensus document. Results and Discussion Vesicoureteral Reflux is a common and challenging problem in children. Children presenting with Vesicoureteral Reflux require careful evaluation and treatment to avoid future urinary tract infections and kidney scars. The panel addressed recommendations on up to date choice of diagnosis evaluation and therapies.

RESUMO CONTEXTO: Recentemente foi demonstrada associação entre sintomas do trato urinário inferior entre mães e filhos. No entanto, o papel da constipação funcional neste binômio não é claro. OBJETIVO: Avaliar a disfunção vésico-intestinal entre mães e filhos. MÉTODOS: Estudo transversal de base populacional. As mães e os filhos responderam a um questionário de autorresposta, composto pelos critérios de Roma IV, International Consultation on Incontinence Questionnaire - Overactive Bladder, Dysfunctional Voiding Scoring System e perguntas sociodemográficas. RESULTADOS: Foram estudados 441 pares mãe-filho. A idade média dos filhos foi de 9,1±2,7 anos, sendo 249 (56,5%) do sexo feminino. A idade média das mães foi de 35,7±6,1 anos. A constipação sem sintomas do trato urinário inferior estava presente em 35 (7,9%) crianças e 74 (16,8%) mães. Sintomas do trato urinário inferior isolados estavam presentes em 139 (31,5%) crianças e 92 (20,9%) mães e a disfunção vésico-intestinal ocorreu em 51 (11,6%) crianças e 78 (17,7%) mães. Não houve associação entre sintomas isolados do trato urinário inferior em crianças e sintomas isolados do trato urinário inferior em mães (P=0,31). Na análise univariada, houve associação entre disfunção vésico-intestinal em crianças e disfunção vésico-intestinal em mães (OR=4,8 IC 95% 2,6-9,6; P<0,001) e constipação isolada em crianças e constipação isolada em mães (OR=3,0 IC 95 % 1,4-6,4; P=0,003). Na análise multivariada, mães com disfunção vésico-intestinal foi o único fator de associação independente para disfunção vésico-intestinal em crianças (OR=5,4 IC 95% 2,5-11,6; P<0,001). CONCLUSÃO: Mães com disfunção vésico-intestinal têm maior probabilidade de ter filhos com disfunção vésico-intestinal. A associação entre constipação e sintomas do trato urinário inferior desempenha um papel importante nesta apresentação familiar.

ABSTRACT BACKGROUND: Recently it was shown an association between lower urinary tract symptoms in mothers and their children. However, the role of functional constipation in this binomial is unclear. OBJECTIVE: To evaluate bladder and bowel dysfunction between mothers and children. METHODS: A population-based cross-sectional study. Mothers and their children responded a self-administrated questionnaire composed by Rome IV criteria, International Consultation on Incontinence Questionnaire - Overactive Bladder, Dysfunctional Voiding Scoring System and demographic questions. RESULTS: A total of 441 mother-child pairs was obtained. Children’s mean age was 9.1±2.7 years, with 249 (56.5%) female. Mothers’ mean age was 35.7±6.1 years. Isolated constipation was present at 35 (7.9%) children and 74 (16.8%) mothers. Isolated lower urinary tract symptoms were present in 139 (31.5%) children and 92 (20.9%) mothers and bladder bowel dysfunction occurred in 51 (11.6%) children and 78 (17.7%) mothers. There wasn’t any association between isolated lower urinary tract symptoms in children and isolated lower urinary tract symptoms in mothers (P=0.31). In univariate analysis there were an association between bladder bowel dysfunction in children and bladder bowel dysfunction in mothers (OR=4.8 IC 95% 2.6-9.6, P<0.001) and isolated constipation in children and isolated constipation in mothers (OR=3.0 IC 95% 1.4-6.4, P=0.003). In multivariate analysis mothers with bladder bowel dysfunction was the only independent factor associated with bladder bowel dysfunction in children (OR=5.4 IC 95% 2.5-11.6, P<0.001). CONCLUSION: Mothers with bladder bowel dysfunction are more likely to have a child with bladder bowel dysfunction. Association between these two dysfunctions plays an important role in this familiar presentation.

ABSTRACT Introduction: Lower urinary tract dysfunction (LUTD) is a common clinical condition. Emotional and behavioral issues are increasing among children and adolescents, with stress indicating difficulties in personal and social functioning. This study evaluated whether urinary tract symptoms (LUTS) is associated with stress. Materials and Methods: A cross-sectional, analytical study with 6-14-year-old patients with LUTS and no anatomical/neurogenic urinary tract abnormalities was conducted using the Dysfunctional Voiding Scoring System, a psychological assessment and the Child Stress Scale. The overall stress score was analyzed in relation to the psychological assessment data. Answers to the seven specific DVSS urinary questions were compared with those for the four Child Stress Scale domains. Univariate and multivariate analyses were performed. The chi-square test and Pearson's correlation were used to determine associations. Significance was defined as p <0.05. Results: Most children were male (56%). Mean age was 9.0±2.25 years. Stress was detected in 20 out of 98 patients (20.4%; 95% CI: 13-30%). Of these, 90% were born from unplanned pregnancies and 67% were upset about their disorder. All the Child Stress Scale domains were significantly associated with urinary dysfunction, with dysuria being significantly associated with all four domains. In the multivariate analysis, dysuria was the only symptom that remained associated with stress. Associations with stress strengthened as the frequency of dysuria increased: physical reactions (p <0.01), emotional reactions (p <0.05), psychological reactions with a depressive component (p <0.01) and psychophysiological reactions (p <0.05). Conclusion: Stress levels are higher in children and adolescents with LUTS who have more severe symptoms. Dysuria was the symptom most associated with stress, both in the physical reactions domain, in the psychological reactions domains with or without a depressive component and in the psychophysiological reactions domain.

ABSTRACT Aim: To evaluate the efficacy of the onabotulinum toxin type A in the treatment of HTLV-1 associated overactive bladder and its impact on quality of life (QoL). Methods: Case series with 10 patients with overactive bladder refractory to conservative treatment with anticholinergic or physical therapy. They received 200Ui of onabotulinumtoxin type A intravesically and were evaluated by overactive bladder symptoms score (OABSS) and King's Health Questionnaire. Results: The mean (SD) of the age was 52 + 14.5 years and 60% were female. All of them had confirmed detrusor overactivity on urodynamic study. Seven patients had HAM/TSP. The median and range of the OABSS was 13 (12–15) before therapy and decreased to 1.0 (0–12) on day 30 and to 03 (0–14) on day 90 (p < 0.0001). There was a significant improvement in 8 of the 9 domains of the King's Health Questionnaire after the intervention. Hematuria, urinary retention and urinary infection were the complications observed in 3 out of 10 patients. The mean time to request retreatment was 465 days. Conclusion: Onabotulinum toxin type A intravesically reduced the OABSS with last long effect and improved the quality of life of HTLV-1 infected patients with severe overactive bladder.

Purpose To assess the prevalence of LUTS, urinary tract and urodynamics changes in patients with Friedreich's Ataxia (FA), the most common form of hereditary ataxia. Materials and Methods This study evaluated 258 patients with genetically confirmed diagnoses of FA. Of the patients, 158 responded to a questionnaire which assessed their urinary symptoms. Patients with clinical changes underwent renal function examinations, ultrasound, and urodynamic studies (UDS). Results The sample analyzed showed that 82% of the patients complained of LUTS, although only 22% related the symptoms with quality of life impairment. Twenty eight (18%) of them agreed to undergo urodynamic evaluation. Urgency was the most common symptom. The exam was normal in 4 (14%) and detrusor underactivity was the most common finding. 14% (4 patients) presented with dilatation of the upper urinary tract at ultrasound scans. None of them had creatinine alterations. Conclusions LUTS was found in a large percentage of patients with FA, but only a few related it to their quality of life impairment. Although creatinine levels was normal in this sample, some patients may show upper urinary tract abnormalities, with deserves close observation and proper care.

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.

Purpose: To review our clinical experience with urinary continent catheterizable reservoir in children under five years of age. Materials and Methods: A total of 23 patients (16 males, 7 females) with a median age of 3.64 years were evaluated. Among these, 6 (26.08%) had a posterior urethral valve, 9 (39.13%) myelomeningocele, 4 (17.39%) bladder exstrophy, 2 (8.69%) genitourinary rabdomyosarcoma, 1 (4.34%) had spinal tumor and 1 (4.34%) an ano-rectal anomaly. Results: Perioperative complications were observed in four patients consisting of one febrile urinary tract infection, one partial operative wound dehiscence, one partial stomal dehiscence and one vesico-cutaneous fistula after a secondary exstrophy repair. The overall long-term complications rate was 40.90% and consisted of two stomal stenoses (9.09%), one neobladder mucosal extrusion (4.54%), three neobladder calculi (13.63%) and persistence of urinary incontinence in three patients (13.63%). The overall surgical revision was 36.36% and final continence rate was 95.45% with mean follow-up of 39.95 months Conclusion: Continent urinary diversion is technically feasible even in small children, with acceptable rates of complications.

OBJECTIVE: To evaluate the basic knowledge of pre-school teachers who deal with children between the ages of 4 and 7 years, who present signs of lower urinary tract dysfunction (LUTD). MATERIALS AND METHODS: We performed a survey with 50 teachers from 9 private schools working with pre-school children. The criteria for selection were if teachers were certified or non-certified elementary school teachers - NCEST and the amount of professional experience. RESULTS: Thirty-three teachers considered that the normal daily urinary frequency should be from 4 to 7 times. Two of the 50 teachers considered it normal to urinate less than 4 times per day and 15 teachers considered more than 7 times per day as normal. There was no difference between the 2 categories of certified or NCEST nor between those with more or less than five years of professional experience. Thirty-three percent believed that to urinate more than 4 times during a class period (4-5 hours) could indicate a urinary problem. There was a statistically significant difference among the certified and NCEST but not in terms of time of professional experience. If during this period the child would not ask to urinate, only 18% considered that as an indication of urinary problem. When asked about the symptoms that would indicate urinary urgency and urge incontinence, only 24% of the teachers connected it with urinary problem. There was no difference in terms of professional background or professional experience in these 2 last analyses. CONCLUSION: Our data shows evidences that private pre-schools teachers are not well informed of the clinical manifestation of LUTD.

OBJECTIVE: We evaluated clinical characteristics of primary vesicoureteral reflux (VUR) in infants in a 30-year period in Brazil with special reference to the relation of renal parenchymal damage to urinary tract infection and gender. MATERIALS AND METHODS: From 1975 through 2005, 417 girls (81.6%) and 94 boys (18.4%) with all grades of reflux were retrospectively reviewed. Patients were categorized by the worst grade of reflux, maintained on antibiotic prophylaxis and underwent yearly voiding cystourethrography until the reflux was resolved. VUR was considered resolved when a follow-up cystogram demonstrated no reflux. Surgical correction was recommended for those who fail medical therapy, severe renal scarring or persistent VUR. RESULTS: Grades I to V VUR resolved in 87.5%, 77.6%, 52.8%, 12.2% and 4.3%, respectively. Renal scars were present at presentation in 98 patients (19.2%). Neither gender nor bilaterality versus unilaterality was a helpful predictor of resolution. The significant difference was found among the curves using the log rank (p < 0.001) or Wilcoxon (p < 0.001) test. CONCLUSION: Despite the current use of screening prenatal ultrasound, many infants are still diagnosed as having vesicoureteral reflux only after the occurrence of urinary tract infection in our country. Scarring may be associated to any reflux grade and it may be initially diagnosed at any age but half of the scars are noted with higher grades of reflux (IV and V). The incidence of reflux related morbidity in children has significantly diminished over the last three decades.

OBJECTIVE: To evaluate the role of elective appendicovesicostomy in association with Monfort abdominoplasty to avoid urinary tract infection (UTI) and renal damage in the post-operative follow-up of patients with prune belly syndrome. MATERIALS AND METHODS: We followed 4 patients operated in our institution (UNIFESP) (Monfort, orchidopexy and Mitrofanoff) and compared them to 2 patients treated similarly, but without an appendicovesicostomy, in a second institution (UFBA). We evaluated postoperative clinical complications, UTI and preservation of renal parenchyma. Patients were followed as outpatients with urinalysis, ultrasonography (US) and occasionally with renal scintigraphy. RESULTS: Mean follow-up was 23.5 months. Immediate post-operative course was uneventful. We observed that only one patient with the Mitrofanoff channel persisted with UTI, while the 2 patients used as controls persisted with recurrent pyelonephritis (> 2 UTI year). CONCLUSION: Our data suggest that no morbidity was added by the appendicovesicostomy to immediate postoperative surgical recovery and that this procedure may have a beneficial effect in reducing postoperative UTI events and their consequences by reducing the postvoid residuals in the early abdominoplasty follow-up. However, we recognize that the series is small and only a longer follow-up with a larger number of patients will allow us to confirm our suppositions. We could not make any statistically significant assumptions regarding differences in renal preservation due to the same limitations.

OBJECTIVES: To assess the clinical presentation of children with lower urinary tract dysfunction (LUTD) relating to objective examination data. MATERIALS AND METHODS: Forty-four children (36 girls and 8 boys with mean age of 6.8 years) with LUTD were prospectively assessed through a specific questionnaire that analyzed clinical presentation of those patients. These data were then compared to objective data, such as micturition diary and uroflowmetry with electromyography. RESULTS: A urinary tract infection (UTI) antecedent was observed in 31 cases (70.5%), and of those, 24 cases of UTI were accompanied by fever. All children presented micturition urgency. Daily urinary incontinence was observed in 33 cases (75%) and nocturnal enuresis in 23 (52.3%). As for micturition frequency, 15 (34.1%) had normal frequency 19 (43.2%) presented more than 10 daily micturition episodes and 10 (22.7%) thought they urinated less than 5 times a day. In the uroflowmetry and electromyography examination, 14 (31.8%) experienced lack of coordination during micturition. Of 10 children with infrequent micturition, 5 confirmed this in their micturition diaries and 2 listed more than 5 micturition episodes per day in the diary. Of 19 patients presenting polaciuria, only 5 confirmed this in their micturition diaries, while 7 had less than 10 micturition episodes per day. CONCLUSION: Most children with LUTD presented a previous UTI, and daily incontinence was verified in around 75% of the patients. Complaints of polaciuria or infrequent micturition are not noted completely in the micturition diaries and there is no parameter in the clinical history that offers good sensitivity or specificity for the diagnosis of lack of perineal coordination.

OBJECTIVES: The aim of this article was to report our experience with kidney transplantation in children. MATERIALS AND METHODS: From June 1980 to December 2003, 690 kidney transplants were performed in our institution, among which 50 were in patients with less than 18 years old. Technical aspects as well as clinical and surgical evolution were reviewed in this study. RESULTS: Patient's mean age was 12 years (2-17 years). Twenty-nine patients were male and 21 female. Live related donors were responsible for 75% of the cases (38 patients) and 25% (12 patients) came from cadaver donors. The main complications were ureteral fistula in 6 patients (12%), arterial stenosis in 2 (4%), wall infection and dehiscence in 1 case (2%). The overall rate of surgical complication was 20%. No case of hyperacute rejection was reported. During the follow-up 20 grafts were lost due to chronic rejection and 2 patients died. No loss of graft due to surgical complications was reported. The graft survival rate was 71% in 1 year, 64% in 3 years and 57% in 5-year follow-up. CONCLUSIONS: Kidney transplantation in children is a viable treatment option for terminal kidney disease presenting success and surgical complication rate similar to kidney transplantation in adults.