A síndrome de Ochoa é rara, e seus principais problemas clínicos são frequentemente não reconhecidos. Descrevem-se aqui características faciais de seis pacientes para auxiliar profissionais de saúde a reconhecer o sorriso invertido que eles apresentam e encaminhá-los para o tratamento adequado. Os prontuários médicos foram revisados e a condição urológica dos pacientes foi reavaliada clinicamente. A média de idade na última avaliação foi de 15,5 anos, variando de 12 a 32 anos. O seguimento médio foi de 35 meses (12 a 60). Os sintomas iniciais foram infecções do trato urinário em quatro pacientes (67%) associadas com enurese e incontinência urinária em três deles (50%). Um paciente apresentou infecções do trato urinário isoladamente e dois apresentaram sintomas do trato urinário inferior, porém sem infecções. O tratamento inicial consistiu em cateterismo intermitente limpo, com anticolinérgicos em todos os pacientes. Reconstrução urinária foi realizada em quatro pacientes (67%) por meio de ampliação vesical. Dois pacientes apresentaram doença renal em estágio terminal no seguimento, um recebeu transplante renal e outro manteve-se em lista de espera para transplante renal. Consanguinidade familiar esteve presente em apenas um caso. Essa condição significativa é rara, porém deve ser reconhecida por pediatras, nefrologistas e urologistas, a fim de instituir tratamento urológico agressivo precoce.

Ochoa syndrome is rare and its major clinical problems frequently unrecognized. We describe facial characteristics of six patients to help health professional recognize the inverted smile that these patients present and refer them to proper treatment. Patients’ medical records were reviewed and patients’ urological status clinically reassessed. At last evaluation patients’ mean age was 15.5 years, and age ranged from 12 to 32 years. Mean follow-up was 35 months (12 to 60). Initial symptoms were urinary tract infections in four patients (67%) associated with enuresis and incontinence in three of them (50%). One patient had only urinary tract infection and two lower urinary tract symptoms without infections. Initial treatment consisted of clean intermittent catheterization with anticholinergics for all patients. Four patients (67%) were submitted to bladder augmentation. Two patients had end-stage renal disease during follow-up, one received kidney transplantation and one patient remained on the waiting list for a renal transplantation. Familial consanguinity was present in only one case. This significant condition is rare, but it must be recognized by pediatricians, nephrologists and urologists in order to institute early aggressive urological treatment.

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.

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.

ObjectiveTo histologically evaluate, in an experimental study in rabbits, the integration process of the buccal mucosa fenestrated graft applied in the corpora cavernosa for Bracka first stage urethroplasty.Materials and MethodsA urethral defect was surgically created in 16 male rabbits of the New Zealand breed through the excision of the penile urethra. The urethral defect was corrected by applying buccal mucosa fenestrated graft through two cruciform incisions in the distal portions of its longitudinal axis. The animals were sacrificed at 2, 4, 8 and 12 weeks post surgery and their genitals were subjected to clinical and histological assessment.ResultsThe buccal mucosa fenestrated graft showed complete uptake in all groups, with keratinization squamous metaplasia and mucosal proliferation of the fenestrated areas. The fenestrated graft area represented an increase in length of 25% in length in relation to the original standard graft.ConclusionsThe fenestrated buccal mucosa graft presented total integration to the adjacent epithelia with re-epithelization of the incision areas of the graft (fenestrations) and no significant inflammatory or scarring reactions when compared to other mucosa transplanted areas; therefore its application is viable in cases of extensive urethral defect whenever the donating area might be insufficient.

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.

OBJECTIVE: This study was developed to determine whether the generation of free radicals, induced by ischemia followed by reperfusion in a model of chronic intravesical obstruction in rats, would lead to damage in the detrusor. It also investigates the possible protective action of the flavonoid galangin on the tissue lesion induced by lipid peroxidation. MATERIALS AND METHODS: Twenty-one male rats were divided into three groups of seven animals each. Group A was subjected to a sham procedure; group B to partial obstruction of the bladder neck; and group C to partial obstruction of the bladder neck, but also received a diet rich in the flavonoid galangin. All the animals were subjected to urodynamic evaluation and then sacrificed. The bladders were sent for enzymatic tests. RESULTS: The urodynamic showed that group B developed significantly greater numbers of involuntary contractions of the detrusor, greater post-micturition residue and lower compliance. The group A presented TEAC levels greater than to the group B. Comparative analysis of group A, B and C demonstrated significantly greater malondialdehyde levels in group B in relation to groups A and C. The group B presented smaller contraction amplitudes than did groups A and C, in electrically stimulated contractions. CONCLUSIONS: That oxidative stress is implicated in the damage to the detrusor musculature following a period of chronic intravesical obstruction. We show, for the first time, that administration of an antioxidant prior to and following the start of chronic obstruction makes it possible to avoid the cellular lesions that cause detrusor dysfunction.

OBJECTIVE: The purpose of this study was to compare the effects of castration on cell death rate of the adult rat prostates and to evaluate the benefic action of alpha tocopherol supplementation to avoid apoptosis post-orchiectomy. MATERIAL AND METHODS: Thirty male Wistar rats weighing 250-300g were divided into three groups: group I - they were subjected to bilateral orchiectomy and sacrificed eight weeks after the procedure; group II - subjected to bilateral orchiectomy and alpha-tocopherol supplementation for four weeks preceding the procedure; and group III - subjected to bilateral orchiectomy and alpha-tocopherol supplementation for four weeks preceding the procedure and for eight weeks afterwards. At the end of the experiment, the prostatectomy was performed in all rats. The presence of oxidative stress was determined by assaying the blood level of 8-isoprostane and the occurrence of apoptosis was evaluated by identification of active caspase-3 through immunohistochemical analysis. RESULTS: The statistic analysis of active caspase-3 showed that in the long-term castrated group the detection was higher than in groups were the alpha-tocopherol was supplemented (p=0.007). Analysis of 8-isoprostane levels showed higher concentrations of reactive oxygen species in group I compared to other groups (p<0.05). Groups II and III presented active caspase-3 lower than in group I (p<0.05). CONCLUSION: Our exploratory analyses demonstrate a method to study the aging process and its influence on oxidative stress of prostatic tissue and cells death rate. Based on our results we can suggest that alpha tocopherol supplementation can decrease the apoptotic process as well as the oxidative stress levels induced by androgen deprivation of the prostate gland.

OBJECTIVE: To analyze the impact of low levels of testosterone induced by orchiectomy and the effect of alpha-tocopherol supplementation on oxidative stress in the urethral sphincter. MATERIALS AND METHODS: Forty male Wistar rats weighing 250-300g were divided into four groups with 10 each: Sham group; Orchiectomy group: bilateral orchiectomy; Orchiectomy-pre-Tocopherol group: bilateral orchiectomy preceded by alpha-tocopherol supplementation for four weeks; Orchiectomy-full-Tocopherol group: bilateral orchiectomy with alpha-tocopherol supplementation for four weeks preceding the procedure and for eight weeks afterwards. At the protocol end, animals were euthanized and had the sphincter analyzed stereologically focusing on collagen and muscle fibers percentage. Oxidative stress levels were determined using 8-epi-PGF2. RESULTS: The 8-epi-PGF2 levels were statistically higher (p < 0.0003) in the Orchiectomy group compared to others groups while Sham and Orchiectomy-full-Tocopherol groups presented statistically similar values (p = 0.52). Collagen volumetric densities were significantly lower in Sham and Orchiectomy-full-Tocopherol groups (p < 0.022). Sham group presented statistically greater muscle fiber percent. CONCLUSION: Castration caused oxidative stress in the urethral sphincter complex, with increased collagen deposition. Alpha-tocopherol had a protective effect and its supplementation for twelve weeks provided the greatest protection.

Despite significant advances in laparoscopic technique and technologies, laparoscopic Urologic surgery remains technically demanding regarding various surgical steps including the challenge of specimen retrieval and extraction, whether to install a drainage system and the best option for wound closure. Laparoscopic specimen entrapment and extraction occurs at what is falsely considered the "end of the procedure". During open surgery, after the specimen has been mobilized, the specimen is simply lifted out of the larger incision which has been made to achieve the surgical objectives. In contrast, significant laparoscopic skill is required to entrap and safely extract laparoscopic specimens. Indeed, the Urologist and surgical team which are transitioning from open surgery may disregard this important part of the procedure which may lead to significant morbidity. As such, it is imperative that during laparoscopic procedures, the "end of the procedure" be strictly defined as the termination of skin closure and dressing placement. Taking a few minutes to focus on safe specimen entrapment and extraction will substantially reduce major morbidity. The following review focus on the technology and technique of specimen entrapment and extraction, on the matter of whether to install a drainage system of the abdominal cavity and the options for adequate closure of trocar site wounds. This article's primary objectives are to focus on how to minimize morbidity while maintain the advantages of a minimally invasive surgical approach.

RESUMO Apresentamos o caso de um adolescente de 16 anos com rabdomiossarcoma paratesticular, submetido à linfadenectomia retroperitonial por tumor clínico estágio I (tomografia computadorizada retroperitonial normal), cujo resultado cirúrgico demonstrou três linfonodos aumentados e positivos para doença metastática; o paciente foi encaminhado para tratamento quimioterápico adjuvante. Este caso sugere que o protocolo Intergroup Rhabdomyosarcoma Study Group IV é questionável para adolescentes com rabdomiossarcoma paratesticular, e que a tomografia computadorizada de abdome negativa para linfonodos não deve afastar a necessidade de linfadenectomia retroperitoneal.

ABSTRACT We report on a 16-year-old male with paratesticular rhabdomyosarcoma who underwent retroperitoneal lymph node dissection due to a stage I tumor (normal retroperitoneal computed tomoghaphy). The surgical finding was three enlarged nodes, positive for metastatic disease. Patient was referred to adjuvant chemotherapy. This case suggests that the Intergroup Rhabdomyosarcoma Study Group IV protocol is subject to questions regarding adolescents with paratesticular rhabdomyosarcoma, and that negative retroperitoneal CT does not preclude the need of lymph node dissection.

PURPOSE: To compare the efficacy and costs of circumcision versus topical treatment using a prospective pharmacoeconomic protocol. MATERIALS AND METHODS: We treated 59 patients (3-10 years of age) randomized into two groups: 29 underwent an 8-week course of topical treatment with 0.2% betamethasone-hyaluronidase cream twice a day; and 30 underwent circumcision. Topical treatment success was defined as complete exposure of the glans. In cases of treatment failure, circumcision was performed and its cost imputed to that of the initial treatment. The pharmacoeconomic aspects were defined according to the Brazilian National Public Health System database and the Brazilian Community Pharmacies Index. RESULTS: The two groups were statistically similar for all clinical parameters evaluated. Topical treatment resulted in complete exposure of the glans in 52% of the patients. Topical treatment was associated with preputial pain and hyperemia. However, treatment suspension was unnecessary. Minor complications were observed in 16.6% of the surgical group patients. The mean cost per patient was US$ 53.70 and US$ 125.20, respectively, for topical steroid treatment (including the costs related to treatment failure) and circumcision. The total costs were US$ 2,825.32 and US$ 3,885.73 for topical treatment and circumcision, respectively. CONCLUSIONS: Topical treatment of phimosis can reduce costs by 27.3% in comparison with circumcision. Therefore, topical treatment of phimosis should be considered prior to the decision to perform surgery.

OBJETIVO: Avaliar os efeitos renais das ondas de choque eletro-hidráulicas (OCEH), utilizando como parâmetros marcadores bioquímicos urinários. MÉTODOS: Foram utilizados 20 ratos machos, EPM - Wistar, distribuídos aleatoriamente em dois grupos: G1 (n=10) Animais submetidos a uma sessão de OCEH. G2 (n=10) Animais submetidos a duas sessões de OCEH separadas por um intervalo de 14 dias. Para coleta da urina os animais foram mantidos em gaiolas metabólicas 24 horas antes e depois da aplicação das OCEH. Foram medidos o pH, a creatinina, sódio, potássio, cloretos, cálcio, magnésio, fósforo, oxalato, fosfatase alcalina e citrato. Vinte e quatro horas após a coleta da urina os animais foram submetidos à nefrectomia do rim envolvido no experimento e, em seguida, sacrificados. Os rins foram então submetidos aos procedimentos de fixação e coloração histológica com hematoxilina e eosina. RESULTADOS: Pequenas variações nos marcadores bioquímicos foram detectadas nos dois grupos, sem diferenças significantes nos valores obtidos antes e após a aplicação das OCEH, exceto para os valores urinários de citrato e fosfatase alcalina. A citratúria diminuiu significantemente nos animais do Grupo 2 após a segunda aplicação das OCEH( 24,8 ± 3,0 mg/dia após a primeira sessão de OCEH e 15,3 ± 2,2 mg/dia após a segunda sessão de OCEH; p < 0.05). A fosfatase alcalina urinária aumentou de forma significante no grupo 1 após a sessão de OCEH (0,57 ± 0,02 vs. 0,79 ± 0,04 µmol/mg de creatinina; p < 0,01) e também no grupo 2 (0,69 ± 0,05 vs. 0,83 ± 0,03 µmol/mg de creatinina; p < 0,05). Os achados histológicos observados nos animais dos dois grupos foram: hemorragia glomerular, intersticial e subcapsular, acompanhada de edema perivascular. CONCLUSÕES: Observou-se um aumento significante da fosfatase alcalina urinária nos dois grupos estudados, sugerindo uma lesão dos túbulos proximais causada pelas ondas de choque eletro-hidráulicas. Nos animais submetidos a mais de uma sessão de OCEH observou-se um discreto aumento na excreção urinária de citrato, o que pode ser um fator que pode contribuir para a formação de novos cálculos.

PURPOSE: To access the effect of repeated extracorporeal shock wave (ESW) on urinary biochemical markers METHODS: 20 rats were assigned for ESW (Direx Tripter X1® - 14 KV) to one of two groups: G1 (n=10) one ESW; G2 (n=10) two ESWs within a 14-day interval. Within the twenty-four hour period before and after the application of shock waves, the animals were placed in metabolic cages for 24 hour urine collection. The ph, creatinine, sodium, potassium, chlorides, calcium, magnesium, phosphorus, oxalates, alkaline phosphatase and citrates were measured. Twenty-four hours after the material was collected for urinary determination, the animals underwent nephrectomy of the kidney submitted to the ESW applications and were, then, sacrificed. The kidneys were processed for hispatological examination. RESULTS: Small variations in the biochemical markers were found in both groups, with no significant differences between the values obtained either prior to or following the ESW applications, except for citrate and alkaline phosphatase. Citraturia decreased significantly in group 2, following the second ESWL application (24.8 ± 3.0 mg/day after the first ESWL vs. 15.3 ± 2.2 mg/day after the second ESWL; p < 0.05). Alkaline phosphatase increased significantly following ESWL in group I (0.57 ± 0.02 vs. 0.79 ± 0.04 µmol/mg creatinine; p < 0.01) and also in group 2 (0.69 ± 0.05 vs. 0.83 ± 0.03 µmol/mg creatinine; p < 0.05). Glomerular, interstitial and sub-capsular hemorrhage with perivascular edema was found in the animals in both groups studied. CONCLUSIONS: A significant increase in urinary alkaline phosphatase was found in both groups studied, suggesting a proximal tubule lesion. In the group of rats undergoing more than one ESWL application, a smaller urinary citrate excretion was noticed, which may be a factor contributing for the formation of new calculi.

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.