Brazilian consensus on vesicoureteral reflux-recommendations for clinical practice.

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 Refl ux 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.

Brazilian consensus on vesicoureteral reflux-recommendations for clinical practice

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.

Brazilian consensus in enuresis-recomendations for clinical practice

ABSTRACT Introduction Enuresis, defined as an intermittent urinary incontinence that occurs during sleep, is a frequent condition, occurring in about 10% of children at 7 years of age. However, it is frequently neglected by the family and by the primary care provider, leaving many of those children without treatment. Despite of many studies in Enuresis and recent advances in scientific and technological knowledge there is still considerable heterogeneity in evaluation methods and therapeutic approaches. Materials and Methods The board of Pediatric Urology of the Brazilian Society of Urology joined a group of experts and reviewed all important issues on Enuresis and elaborated a draft of the document. On September 2018 the panel met to review, discuss and write a consensus document. Results and Discussion Enuresis is a multifactorial disease that can lead to a diversity of problems for the child and family. Children presenting with Enuresis require careful evaluation and treatment to avoid future psychological and behavioral problems. The panel addressed recommendations on up to date choice of diagnosis evaluation and therapies.

Brazilian consensus in enuresis-recomendations for clinical practice.

INTRODUCTION: Enuresis, defined as an intermittent urinary incontinence that occurs during sleep, is a frequent condition, occurring in about 10% of children at 7 years of age. However, it is frequently neglected by the family and by the primary care provider, leaving many of those children without treatment. Despite of many studies in Enuresis and recent advances in scientific and technological knowledge there is still considerable heterogeneity in evaluation methods and therapeutic approaches. MATERIALS AND METHODS: The board of Pediatric Urology of the Brazilian Society of Urology joined a group of experts and reviewed all important issues on Enuresis and elaborated a draft of the document. On September 2018 the panel met to review, discuss and write a consensus document. RESULTS AND DISCUSSION: Enuresis is a multifactorial disease that can lead to a diversity of problems for the child and family. Children presenting with Enuresis require careful evaluation and treatment to avoid future psychological and behavioral problems. The panel addressed recommendations on up to date choice of diagnosis evaluation and therapies.

The Ochoa urofacial syndrome: recognize the peculiar smile and avoid severe urological and renal complications / Síndrome urofacial de Ochoa: reconheça o sorriso peculiar e evite complicações urológicas e renais graves

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.

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.

The Ochoa urofacial syndrome: recognize the peculiar smile and avoid severe urological and renal complications.

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.

Comparative analysis of the pressure profilometry of vesicocutaneous continent catheterizable conduits between patients with and without rectus abdominis neosphincter (Yachia principle).

AIMS: To assess whether crossing rectus abdominis muscle strips, as proposed by Yachia, would change urinary catheterizable conduit's pressure profilometry, in static and dynamic conditions. METHODS: Non-randomized selection of 20 continent patients that underwent Macedo's ileum-based reservoir, 10 including Yachia's technique (Study Group) and 10 without this mechanism of continence (Control Group). Demographics and cystometric data were assessed. Conduit's pressure profilometry was obtained by infusing saline through a multichannel catheter, at rest and during Valsalva maneuver. We assessed the pressure: (a) in the bladder; (b) in conduit's proximal segment; and (c) in conduit's distal segment, which is presumably the abdominal wall and crossed muscle strips site. RESULTS: Mean age at surgery was 6.1 years in the Control Group and 7.7 years in the Study Group. There was no statistically significant difference between groups regarding maximum cystometric bladder capacity and leakage point pressure. At rest, the pressure profilometry showed similar results between groups in all segments analyzed. During Valsalva maneuver, pressure profilometry showed similar results between groups in bladder and conduit's proximal segment pressure. In this condition, conduit's distal segment pressure in the Study Group (Mean = 72.9 and Peak = 128.7 cmH2 O) was significantly greater (P < 0.05) than conduit's distal segment pressure in the Control Group (Mean = 48.3 and Peak = 65.1 cmH2 O). CONCLUSIONS: Crossing muscle strips over the conduit significantly increases the pressure in its distal segment during contraction of the rectus abdominis muscle, which can be important in moments of sudden increase in abdominal pressure in order to keep continence.

Urological evaluation of patients that had undergone in utero myelomeningocele closure: A prospective assessment at first presentation and early follow-up. Do their bladder benefit from it?

AIMS: To report our data on initial urological presentation after in utero myelomeningocele (MMC) closure. METHODS: A prospective urological assessment at first presentation was designed for patients that had undergone in utero MMC closure and referred to our urological facility. The protocol consisted of detailed medical history, renal sonography, voiding cystourethrogram, and urodynamic evaluation. RESULTS: In utero MMC closure was performed in 19 patients at gestational age of 25.6 weeks 25-27. Birth occurred at a mean gestational age of 31.8 weeks 26-36. Hyperactive bladder was observed in 89.5% 17/19. Bladder compliance was normal in two cases (10.5%), was markedly reduced in 10 patients (52.6%) and not possible to be determined due to urinary leakage in 7 patients (36.8%). We observed normal bladder capacity in 8 patients (42.1%), reduced in 11 (57.9%), and detrusor-sphincter dyssynergia in 9 patients (47.4%). Underactive bladder was diagnosed in one case. Clean Intermittent Catheterization was initiated by 11 patients (57.9%) mostly in association with anticholinergics 10/11. Vesicoureteral reflux was found in 5 patients (26.3%) and 9 had pyelonephritis at a mean follow-up of 5.4 months 2-17. CONCLUSIONS: Our data suggested that despite in utero MMC surgery, patients are at risk for bladder abnormal function and renal deterioration and should be aggressively treated, not differently from those operated in the post-natal term. This study has the merit of being a prospectively set evaluation performed by one investigator, including the urodynamic study. We acknowledge the need of long-term follow up.

Role of penile biometric characteristics on surgical outcome of hypospadias repair.

PURPOSE: To assess influence of penile biometric characteristics on surgical outcome of tubularized incised plate (TIP) repair for hypospadias. METHODS: We prospectively studied 42 boys with distal hypospadias that underwent TIP urethroplasty. Biometric assessment prior to surgery consisted of classifying glans shape, urethral plate (UP) length and width, prepuce vascularization and penile size, using a caliper rule, according to previous definitions. Surgical outcome was assessed according to the occurrence of dehiscence, fistula or urethral stricture. RESULTS: There was no statistical difference among groups concerning postoperative complications. Glans shape: grooved (24/57 %), shallow (9/21 %) and conical (9/21 %). UP width: <10 mm (26/62 %) and ≥10 mm (16/38 %). UP length was evaluated in 29 patients: <10 mm in (12/41 %) and ≥10 mm (17/59 %). Prepuce vascularization: one predominant blood vessel (17/41 %), two predominant blood vessels (8/19 %), H-like form with communication between two well-developed blood vessels (6/14 %) and net-like form with no predominant blood vessels (11/26 %). Penile size was measured in 28 patients under 50th percentile (25/89 %). CONCLUSION: Glans shape, UP width, UP length, prepuce vascularization and penile size do not significantly affect the complication rate of TIP repair in distal hypospadias. Most of the patients with distal hypospadias presented with penile size under mean length for age suggesting some form of mild hypogonadism.

Cloacal Exstrophy: a complex disease

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. .

Keratinized versus non-keratinized preputial flap onlay urethroplasty: does it make any difference in the histological analysis? An experimental study in rabbits

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. .

Anthropometric measurements as an indicator of nutritional status in spina bifida patients undergoing enterocystoplasty.

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.

Two-stage complex hypospadias repair when urethral plate has to be divided: ventral corporoplasty using a tunica vaginalis flap.

OBJECTIVE: To demonstrate ventral corporoplasty, with tunica vaginalis flap to reconstruct the corpora cavernosa, in a two-stage strategy for proximal hypospadias surgery. METHODS: Assessment of residual curvature after complete urethral plate division and transverse superficial cuts in albuginea. Ventral incision of tunica albuginea to elongate the ventral surface of the penis and use of a tunica vaginalis flap to reconstruct the defect in corpora cavernosa. Dorsal preputial island flap was used to cover the penile ventral surface, to be tubularized in a second stage, together with the original urethral plate. RESULTS: Patient had an uneventful follow-up. Penile aspect was very satisfactory with no residual curvature. CONCLUSION: Tunica vaginalis is an attractive alternative for ventral corporoplasty in hypospadia repair and has the advantages of prompt disposability, autologous use, and does not represent an extra cost for treatment.

Medidas antropométricas como indicadores do estado nutricional em pacientes com espinha bífida submetidos a enterocistoplastia / Anthropometric measurements as an indicator of nutritional status in spina bifida patients undergoing enterocystoplasty

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.

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.

Age-related structural changes of the urethral plate in hypospadias.

PURPOSE: To describe age-related changes in the extracellular matrix (ECM) of the human urethral plate in patients operated on for hypospadias, specifically describing histological features and determining the differences in the major components of the ECM, and thus providing an evaluation of the quality and wound healing potential of the urethral tissue. PATIENTS AND METHODS: Urethral plate samples were obtained from 16 patients who underwent hypospadias repair (6 months-53 years of age), not previously submitted to any surgery. As a control group, male urethras were obtained from five fetuses. ECM structural characterization was performed by Hematoxylin and Eosin, Masson's trichrome, Weigert's resorcin-fuchsin, and Sirius red. The concentration of total collagen was determined by a hydroxyproline assay. RESULTS: Urethral plates were lined with squamous epithelium. Most urethral plate samples showed well-vascularized connective tissue and typical vascular sinusoids surrounded by an ECM with smooth muscle cells, collagen, elastic fibers and fibroblasts. ECM of the older urethral plates was characterized by abundant collagen content (types I and III), scarce elastic fibers, low cellular density, and no vascular sinusoids. Total collagen concentration increased significantly with aging (r = 0.798; p = 0.006). CONCLUSIONS: Urethral plates of hypospadias present important age-related structural changes. These changes may play a role in urethral healing following hypospadias repair, although this subject deserves more investigation.

Total urogenital mobilization by posterior sagittal approach and ileal neovaginoplasty in cloacal repair by a common channel of 4 cm.

OBJECTIVE: The total urogenital mobilization (TUM) technique avoids dissection of the vagina from the urinary tract. However, in the case of a long common channel, bowel interposition for vaginal replacement may be necessary. We present a video of the TUM technique in cloacal repair with ileal neovaginoplasty. METHODS: An 8-month-old female patient with cloaca, presenting a 4-cm common channel, was operated on by the TUM technique, through a perineal sagittal with additional abdominal approach. She had undergone decompressing colostomy at birth. Total urogenital sinus mobilization was performed with ileal neovaginoplasty. RESULTS: The patient had a good postoperative outcome, an endoscopy of all structures reconstructed was performed at PO day 30, and home dilatation of colon and vagina by parents was initiated. CONCLUSION: TUM with neovaginoplasty bowel interposition is an effective technique for repairing long common channels.