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2.
J Pediatr Urol ; 14(2): 157.e1-157.e8, 2018 04.
Artigo em Inglês | MEDLINE | ID: mdl-29398588

RESUMO

INTRODUCTION: Prior studies of outcomes following genitoplasty have reported high rates of surgical complications among children with atypical genitalia. Few studies have prospectively assessed outcomes after contemporary surgical approaches. OBJECTIVE: The current study reported the occurrence of early postoperative complications and of cosmetic outcomes (as rated by surgeons and parents) at 12 months following contemporary genitoplasty procedures in children born with atypical genitalia. STUDY DESIGN: This 11-site, prospective study included children aged ≤2 years, with Prader 3-5 or Quigley 3-6 external genitalia, with no prior genitoplasty and non-urogenital malformations at the time of enrollment. Genital appearance was rated on a 4-point Likert scale. Paired t-tests evaluated differences in cosmesis ratings. RESULTS: Out of 27 children, 10 were 46,XY patients with the following diagnoses: gonadal dysgenesis, PAIS or testosterone biosynthetic defect, severe hypospadias and microphallus, who were reared male. Sixteen 46,XX congenital adrenal hyperplasia patients were reared female and one child with sex chromosome mosaicism was reared male. Eleven children had masculinizing genitoplasty for penoscrotal or perineal hypospadias (one-stage, three; two-stage, eight). Among one-stage surgeries, one child had meatal stenosis (minor) and one developed both urinary retention (minor) and urethrocutaneous fistula (major) (Summary Figure). Among two-stage surgeries, three children developed a major complication: penoscrotal fistula, glans dehiscence or urethral dehiscence. Among 16 children who had feminizing genitoplasty, vaginoplasty was performed in all, clitoroplasty in nine, external genitoplasty in 13, urethroplasty in four, perineoplasty in five, and total urogenital sinus mobilization in two. Two children had minor complications: one had a UTI, and one had both a mucosal skin tag and vaginal mucosal polyp. Two additional children developed a major complication: vaginal stenosis. Cosmesis scores revealed sustained improvements from 6 months post-genitoplasty, as previously reported, with all scores reported as good or satisfied. DISCUSSION: In these preliminary data from a multi-site, observational study, parents and surgeons were equally satisfied with the cosmetic outcomes 12 months after genitoplasty. A small number of patients had major complications in both feminizing and masculinizing surgeries; two-stage hypospadias repair had the most major complications. Long-term follow-up of patients at post-puberty will provide a better assessment of outcomes in this population. CONCLUSION: In this cohort of children with moderate to severe atypical genitalia, preliminary data on both surgical and cosmetic outcomes were presented. Findings from this study, and from following these children in long-term studies, will help guide practitioners in their discussions with families about surgical management.


Assuntos
Hiperplasia Suprarrenal Congênita/cirurgia , Transtornos do Desenvolvimento Sexual/cirurgia , Anormalidades Urogenitais/cirurgia , Hiperplasia Suprarrenal Congênita/diagnóstico , Pré-Escolar , Estudos de Coortes , Transtornos do Desenvolvimento Sexual/diagnóstico , Estética , Feminino , Genitália Feminina/anormalidades , Genitália Feminina/cirurgia , Genitália Masculina/anormalidades , Genitália Masculina/cirurgia , Humanos , Lactente , Masculino , Complicações Pós-Operatórias , Estudos Prospectivos , Qualidade de Vida , Procedimentos de Cirurgia Plástica/métodos , Medição de Risco , Cirurgia Plástica/métodos , Resultado do Tratamento , Anormalidades Urogenitais/diagnóstico , Procedimentos Cirúrgicos Urogenitais/efeitos adversos , Procedimentos Cirúrgicos Urogenitais/métodos
3.
J Pediatr Urol ; 14(1): 42-46, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29150195

RESUMO

INTRODUCTION: Pre-operative physical examination of male epispadias allows for classification of epispadias level as glanular (GE), penile (PE) or penopubic (PPE), and for delineation of anatomic anomalies. The incidence of associated extragenital abnormalities, such as vesicoureteral reflux (VUR), bladder neck (BN) abnormality and abnormal pubic diastasis (PD), and their impact on urinary continence has not yet been systematically studied. OBJECTIVE: The goal of this study was to evaluate whether the more proximal level of epispadias correlated with associated extragenital anatomic anomalies seen on initial imaging or endoscopic evaluation, and whether these pre-operative findings contributed to subsequent surgical management and impacted on achieving urinary continence. It was hypothesized that the more severe forms of epispadias may be associated with a higher frequency of associated anomalies. STUDY DESIGN: The study was an IRB-approved, retrospective case study of all male patients treated initially for isolated epispadias at the current institution between 1994 and 2011. Data collection was achieved by chart and radiology review evaluating PD, BN appearance, presence of VUR, surgical treatment, and urinary continence. RESULTS: A total of 26 patients were identified and divided into three groups based on appearance at physical examination: four glanular (GE), eight penile (PE), and 14 penopubic (PPE); 17 patients had an abnormal BN. Reflux was noted in nine of 20 patients who had a voiding cystourethrogram (VCUG), two of which had an episode of pyelonephritis. Of the 22 patients past the age of toilet training, 17 were continent (64% (9/14) penopubic, 63% (5/8) penile, and 75% (3/4) glanular). DISCUSSION: Anatomic classification for male epispadias did not provide sufficient information regarding extragenital findings. This study provided new information regarding PD, BN appearance, presence of reflux, and ultimate urinary continence. Pubic diastasis and BN abnormalities were more frequently seen in more severe forms of epispadias, whereas VUR seemed more prevalent in less severe forms. A template for pre-operative evaluation was outlined. Limitations of the study were its retrospective design and relatively small cohort of patients, which reflected the rarity of the condition. CONCLUSION: Based on the information generated, additional anatomic information was generated regarding boys with epispadias. This information will help guide the evaluation and the management of these patients in the future.


Assuntos
Epispadia/diagnóstico , Epispadia/cirurgia , Qualidade de Vida , Incontinência Urinária/cirurgia , Procedimentos Cirúrgicos Urológicos/métodos , Criança , Pré-Escolar , Estudos de Coortes , Bases de Dados Factuais , Epispadia/psicologia , Humanos , Lactente , Imageamento por Ressonância Magnética/métodos , Masculino , Cuidados Pré-Operatórios/métodos , Prognóstico , Procedimentos de Cirurgia Plástica/métodos , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento , Uretra/anormalidades , Uretra/cirurgia , Incontinência Urinária/diagnóstico , Incontinência Urinária/etiologia
4.
J Pediatr Urol ; 13(3): 293.e1-293.e6, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28215832

RESUMO

BACKGROUND: Bifid scrotum and hypospadias can be signs of undervirilization, yet boys presenting with these findings often do not undergo genetic evaluation. In some cases, identifying an underlying genetic diagnosis can help to optimize clinical care and improve guidance given to patients and families. OBJECTIVES: The aim of this study was to characterize current practice for genetic evaluation of patients with bifid scrotum, and to identify approaches with a good diagnostic yield. METHODS: A retrospective study of the Boston Children's Hospital electronic medical records (1993-2015) was conducted using the search term "bifid scrotum" and clinical data were extracted. Data were abstracted into a REDCap database for analysis. Statistical analysis was performed using SPSS, SAS, and Excel software. RESULTS: The search identified 110 subjects evaluated in the Urology and/or Endocrinology clinics for bifid scrotum. Genetic testing (including karyotype, microarray, or targeted testing) was performed on 64% of the subjects with bifid scrotum; of those tested, 23% (15% of the total cohort of 110 subjects) received a confirmed genetic diagnosis. Karyotype analysis, when performed, led to a diagnosis in 17% of patients. Of the ten instances when androgen receptor gene sequencing was performed, a pathogenic mutation was identified 20% of the time. CONCLUSION: This study demonstrated that the majority of individuals with moderate undervirilization resulting in bifid scrotum do not receive a genetic diagnosis. Over a third of the analyzed subjects did not have any genetic testing, even though karyotype analysis and androgen receptor (AR) sequencing were both relatively high yield for identifying a genetic etiology. Increased utilization of traditional genetic approaches could significantly improve the ability to find a genetic diagnosis.


Assuntos
Hipospadia/complicações , Hipospadia/genética , Escroto/anormalidades , Virilismo/complicações , Feminino , Testes Genéticos , Humanos , Lactente , Masculino , Seleção de Pacientes , Estudos Retrospectivos , Virilismo/genética
5.
J Pediatr Urol ; 13(1): 28.e1-28.e6, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27887913

RESUMO

INTRODUCTION: Little data exist about the surgical interventions taking place for children with disorders of sex development (DSD). Most studies that have evaluated cosmetic outcomes after genitoplasty have included retrospective ratings by a physician at a single center. OBJECTIVE: The present study aimed to: 1) describe frequency of sex assignment, and types of surgery performed in a cohort of patients with moderate-to-severe genital ambiguity; and 2) prospectively determine cosmesis ratings by parents and surgeons before and after genital surgery. STUDY DESIGN: This prospective, observational study included children aged <2 years of age, with no prior genitoplasty at the time of enrollment, moderate-to-severe genital atypia, and being treated at one of 11 children's hospitals in the United States of America (USA). Clinical information was collected, including type of surgery performed. Parents and the local pediatric urologist rated the cosmetic appearance of the child's genitalia prior to and 6 months after genitoplasty. RESULTS: Of the 37 children meeting eligibility criteria, 20 (54%) had a 46,XX karyotype, 15 (40%) had a 46,XY karyotype, and two (5%) had sex chromosome mosaicism. The most common diagnosis overall was congenital adrenal hyperplasia (54%). Thirty-five children had surgery; 21 received feminizing genitoplasty, and 14 had masculinizing genitoplasty. Two families decided against surgery. At baseline, 22 mothers (63%), 14 fathers (48%), and 35 surgeons (100%) stated that they were dissatisfied or very dissatisfied with the appearance of the child's genitalia. Surgeons rated the appearance of the genitalia significantly worse than mothers (P < 0.001) and fathers (P ≤ 0.001) at baseline. At the 6-month postoperative visit, cosmesis ratings improved significantly for all groups (P < 0.001 for all groups). Thirty-two mothers (94%), 26 fathers (92%), and 31 surgeons (88%) reported either a good outcome, or they were satisfied (see Summary Figure); there were no significant between-group differences in ratings. DISCUSSION: This multicenter, observational study showed surgical interventions being performed at DSD centers in the USA. While parent and surgeon ratings were discordant pre-operatively, they were generally concordant postoperatively. Satisfaction with postoperative cosmesis does not necessarily equate with satisfaction with the functional outcome later in life. CONCLUSION: In this cohort of children with genital atypia, the majority had surgery. Parents and surgeons all rated the appearance of the genitalia unfavorably before surgery, with surgeons giving worse ratings than parents. Cosmesis ratings improved significantly after surgery, with no between-group differences.


Assuntos
Doenças dos Genitais Femininos/cirurgia , Doenças dos Genitais Masculinos/cirurgia , Genitália/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Procedimentos Cirúrgicos Urogenitais , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Prospectivos
6.
J Pediatr Urol ; 11(2): 73.e1-4, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25837706

RESUMO

INTRODUCTION: Varicoceles in prepubertal boys are uncommon and little is known of the natural history. Historically, a large percentage of these boys have undergone surgical repair with the belief that such early presentation carried a worse prognosis, making assessment of longitudinal outcomes difficult. OBJECTIVE: While there may be concern that varicocele could represent a progressive disease and therefore prepubertal presentation would portend a worse prognosis, we hypothesized that there would be no difference between the prepubertal boys and other adolescents with varicocele. STUDY DESIGN: We retrospectively reviewed a database of boys at a single institution with a documented left-sided varicocele between 1995 and 2011. Inclusion criteria were one or more of the following: 1. Clinician-documented Tanner 1 status, 2. Right testis orchidometric or ultrasound calculated volume of ≤3 cc's. Patients were drawn from a prospectively maintained database of all boys presenting to the outpatient urology clinic receiving a diagnosis of varicocele. A cohort of adolescent boys was assembled by matching as closely as possible with respect to testis volume disparity and grade of varicocele. All matches were within 2% of volume difference. Volume was calculated using the length*width*height*0.71 formula. Testis size disparity was set to a threshold of ≥20% using the Lambert formula: (VolumeRight - VolumeLeft)/VolumeRight*100%. Our primary outcome was defined as hypotrophy or the need for surgery for hypotrophy at the termination of the study. We planned a single subgroup analysis of boys based on presentation with or without hypotrophy. The decision for surgery or observation was made by the individual clinician at the time of patient assessment. RESULTS: On presentation, the prepubertal cohort was younger (10.8 vs 14.1 years), and with smaller left (2.4 vs 11.6 cc) and right (2.4 vs 11.6 cc) testis volume. There were no significant differences with respect to varicocele grade and volume differentials at presentation. At the end of the study, 76% of the prepubertal cohort had neither hypotrophy nor the requirement for operation, compared with 83% of the matched cohort (P = 0.71, Fisher's exact test). Similarly, there were no significant differences in outcome when comparing prepubertal boys with initial symmetry or hypotrophy to their matched cohort of older adolescents. DISCUSSION: The prepubertal varicocele is a rare clinical problem for which little data exists to guide the clinician. In a review of Pubmed indexed English language manuscripts, we were only able to find five papers with information on Tanner stage; only 31 prepubertal boys have longitudinal data reported. This study approximately doubles the number of boys for whom such data is available in the literature. Our chief limitation was sample size. A power analysis indicated that a final-analysis cohort of 90 prepubertal boys would be required to detect a 20% difference in outcome between that group and a matched cohort of pubertal or post-pubertal boys. We propose that given the lack of evidence for worse outcomes in prepubertal boys with varicocele that prepubertal status, in and of itself, not be considered an additional indication for correction of varicocele. CONCLUSION: In our retrospective cohort of prepubertal boys with left testis varicocele and their matched cohort, we did not detect a difference in the rate of good outcomes, defined as the absence of hypotrophy and lack of need for surgical intervention. While we may have suspected, as have others, that prepubertal presentation would have conveyed a more pressing need to intervene, it is likely that these boys represent the very same patients that we see more commonly later in their adolescence, and should thus be managed in a similarly conservative fashion.


Assuntos
Testículo/crescimento & desenvolvimento , Varicocele/epidemiologia , Varicocele/fisiopatologia , Adolescente , Fatores Etários , Idade de Início , Estudos de Casos e Controles , Criança , Humanos , Masculino , Tamanho do Órgão , Prognóstico , Puberdade , Valores de Referência , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Testículo/fisiologia , Ultrassonografia , Varicocele/diagnóstico por imagem , Varicocele/cirurgia
7.
Kidney Int ; 69(1): 137-43, 2006 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-16374434

RESUMO

The renal histologic changes associated with congenital ureteropelvic junction obstruction (UPJO) and the relationship to clinical imaging have not been well studied. In order to better understand the histologic alterations of congenital UPJO and their relationship with clinical imaging and outcomes, we examined renal biopsies from 61 patients undergoing pyeloplasty for congenital UPJO. Glomeruli were analyzed for various injury patterns and the tubulointerstitium was examined for tubular atrophy/simplification and fibrosis. Two methods were used to evaluate tubular mass: glomerular density and morphometric measurement of tubular size and density. Control specimens were obtained from age-matched autopsy specimens without renal pathology. Glomerular changes were identified in 73% of all biopsies and were present in a range from 1.7 to 91% of glomeruli in each patient. Overt tubulointerstitial changes were present in 26% of all biopsies. Fibrosis was noted to occur with tubulointerstitial changes in a significantly greater fraction of children over the age of 1 year (P=0.026). Increased glomerular density was associated with severe hydronephrosis (P<0.02). Normal glomerular density was inversely correlated with age (P<0.001), but this relationship was more variable in UPJO (P<0.01). Among patients with intact differential function preoperatively (>45%), postoperative functional decline was predicted only by increased glomerular density. 20 biopsies without overt tubulointerstitial changes were analyzed morphometrically and showed a significant reduction in proximal tubular (PT) size, but unchanged density. Distal tubular (DT) size was unchanged in UPJO, but density was increased. The PT/DT ratio was therefore markedly decreased in UPJO (P<0.0001). Both PT and DT sizes were significantly larger in children with a diuretic renogram washout time less than 20 min than those with greater than 20 min, a common threshold for functionally significant obstruction (P<0.05). Capsular thickness was significantly increased in UPJO. In all, 36% of biopsies had a thickness >0.5 mm and this was associated with greater degrees of tubulointerstitial changes and glomerular alterations. Congenital UPJO produces a variety of renal parenchymal changes, which may in part reflect abnormal development. Some of these alternations are seen in clinical imaging and may help predict outcomes, but there is significant discordance between conventional imaging and histological findings.


Assuntos
Pelve Renal/anormalidades , Rim/anormalidades , Rim/patologia , Obstrução Ureteral/congênito , Obstrução Ureteral/patologia , Adolescente , Biópsia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Glomérulos Renais/patologia , Túbulos Renais/patologia , Masculino
8.
ScientificWorldJournal ; 4 Suppl 1: 103-7, 2004 Jun 07.
Artigo em Inglês | MEDLINE | ID: mdl-15349535

RESUMO

OBJECTIVE: The study compared two populations of patients undergoing bladder neck reconstruction using the silastic sheath in two major pediatric centers. The success with this technique was markedly different in the two centers. The purpose of the study was to determine factors that might explain the divergent results. PATIENTS AND METHODS: Fifteen patients treated in Indianapolis were compared with 94 patients treated in London with the silastic sheath technique of bladder neck reconstruction. Eighty-seven percent of the Indianapolis patients had myelomeningocele whereas 86% of the London group had exstrophy/epispadias. Median age of the Indianapolis patients was 11 years whereas it was 8.4 years in London. Seventy-three percent of patients in Indianapolis were female and 79% in London were male. Patients were followed for a minimum of eight years in Indianapolis and a mean of seven years in London. Similar surgical technique was employed in the two centers but, over time, the London approach included use of a non-reinforced silastic wrapped loosely around the bladder neck with the interposition of omentum. RESULTS: Both groups achieved continence rates exceeding 90%. Of the Indianapolis patients, two-thirds experienced erosion of the silastic at a mean of 48 months. With modifications in the London technique, the erosion rate of silastic was lowered from 100% to 7%. CONCLUSION: Direct, snug wrap of silastic without omentum around the Young-Dees tube as well as simultaneous bladder augmentation placed patients at increased risk for erosion. The silastic sheath technique may be less applicable to myelomeningocele patients. It seems most applicable to older male patients with exstrophy or epispadias undergoing Young-Dees bladder neck reconstruction who have the ability to void.


Assuntos
Dimetilpolisiloxanos , Epispadia/cirurgia , Meningomielocele/cirurgia , Procedimentos de Cirurgia Plástica/instrumentação , Próteses e Implantes , Silicones , Incontinência Urinária/cirurgia , Procedimentos Cirúrgicos Urológicos/instrumentação , Criança , Epispadia/complicações , Análise de Falha de Equipamento , Feminino , Humanos , Indiana , Londres , Masculino , Meningomielocele/complicações , Implantação de Prótese/métodos , Procedimentos de Cirurgia Plástica/métodos , Recuperação de Função Fisiológica , Resultado do Tratamento , Incontinência Urinária/prevenção & controle , Procedimentos Cirúrgicos Urológicos/métodos
10.
BJU Int ; 91(7): 675-7, 2003 May.
Artigo em Inglês | MEDLINE | ID: mdl-12699483

RESUMO

OBJECTIVE: To describe the clinical features of neonatal scrotal haematoma and distinguish them from those of neonatal testicular torsion. PATIENTS AND METHODS: Five neonates presenting with an acute scrotum and initial diagnosis of neonatal testicular torsion were found to have neonatal scrotal haematoma. In one case the diagnosis was surgical and in four subsequent cases the diagnosis was by colour Doppler ultrasonography, and surgery was avoided. Four of the five children had risk factors associated with neonatal scrotal haematoma, including bleeding diathesis, birth trauma and high birth weight. CONCLUSIONS: The importance of including haematoma in the differential diagnosis of the acute neonatal scrotum is emphasized, as is the value of contemporary Doppler ultrasonography in making this diagnosis.


Assuntos
Doenças dos Genitais Masculinos/diagnóstico por imagem , Hematoma/diagnóstico por imagem , Escroto/diagnóstico por imagem , Diagnóstico Diferencial , Humanos , Recém-Nascido , Masculino , Análise Multivariada , Torção do Cordão Espermático/diagnóstico por imagem , Ultrassonografia Doppler
11.
BJU Int ; 91(6): 525-7, 2003 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-12656908

RESUMO

OBJECTIVE: To assess the efficacy and safety of laparoscopically assisted ureterocystoplasty (LAU) in children. PATIENTS AND METHODS: From 1999 to 2001, five patients (mean age 7 years, range 3.5-13) from four centres underwent LAU with laparoscopic mobilization of the small kidney and upper ureter combined with ureterocystoplasty, with exposure of the bladder through a Pfannenstiel incision. The details and outcomes are reviewed. RESULTS: The LAU was successful in all five patients; there were no complications. A large midline incision was avoided and the LAU carried out through the better tolerated and less painful Pfannenstiel incision. CONCLUSION: LAU is an appealing technique that is safe with the added benefit of a reduced abdominal incision and acceptable operative duration. This represents the first published report of LAU.


Assuntos
Nefropatias/cirurgia , Laparoscopia/métodos , Doenças Ureterais/cirurgia , Doenças da Bexiga Urinária/cirurgia , Coletores de Urina , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Meningomielocele/complicações , Resultado do Tratamento , Ureter/anormalidades
12.
BJU Int ; 89(9): 923-7, 2002 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-12010241

RESUMO

OBJECTIVE: To describe the tolerability and efficacy of clean intermittent catheterization (CIC) in the management of dysfunctional voiding in patients who are neurologically and anatomically normal. PATIENTS AND METHODS: The medical records were reviewed in 23 patients (16 girls, mean age 9 years, range 6-14.5, and seven males, mean age 8 years, range 5-20.5) with urinary incontinence and/or urinary tract infection (UTI) who were offered CIC because they had a large postvoid residual urine volume (PVR). All had extensive instruction before starting CIC. All patients underwent urodynamic studies, and urinary and fecal elimination habits were recorded. Detrusor hyperactivity, when present, was treated with anticholinergic medication. The follow-up evaluation included tolerance of CIC, continence status and the incidence of UTI. Behavioural modification or biofeedback training was not used in any patient. RESULTS: Of the 23 patients, 13 presented with both UTI and urinary incontinence, five with incontinence only, four with UTI only, one with frequency and no incontinence, and one with haematuria. Associated symptoms included frequency/urgency, constipation or soiling, and straining to void or incomplete emptying (in nine each), and infrequent voiding in six. CIC was performed within 2 days by 15 patients, while four others required up to 2 weeks to master CIC. However, three of the four patients (all older girls) who needed 2 weeks to learn the technique did not tolerate CIC and discontinued it within 3 weeks. Four other adolescents (three girls and one boy) refused to learn CIC. Of the 16 patients remaining on CIC only three had cystitis; no patient had a febrile UTI. Once successfully instituted, all patients became continent while on CIC. Six boys (mean follow-up 4 months) had a marked decrease in their PVR. CIC was discontinued in three girls who voided normally to emptiness within 6 months of starting CIC; they remained dry and infection-free 16 months (two) and 6 years later. CONCLUSION: CIC is a viable therapeutic option for the treatment of dysfunctional voiding, associated with a large PVR, in the absence of any neurological abnormality. CIC is well tolerated in the sensate patient and provides a means for expeditiously achieving continence and improving bladder emptying cost-effectively.


Assuntos
Cateterismo Urinário/métodos , Incontinência Urinária/terapia , Infecções Urinárias/terapia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Estudos Longitudinais , Masculino , Autocuidado , Resultado do Tratamento , Doenças da Bexiga Urinária/terapia , Incontinência Urinária/fisiopatologia , Infecções Urinárias/fisiopatologia , Urodinâmica/fisiologia
13.
J Urol ; 166(2): 658-61, 2001 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-11458113

RESUMO

PURPOSE: While a fascial sling for treating children with intractable urinary incontinence is often successful in girls, its effectiveness in boys remains unclear. We determined the long-term efficacy of a rectus fascial sling in boys with neurogenic sphincteric incontinence and defined its urodynamic characteristics for achieving continence. METHODS AND METHODS: We reviewed the charts of all boys who underwent a rectus fascial sling procedure for neurogenic incontinence to determine urinary continence status at the most recent office visit or by telephone interview, the type and dose of anticholinergic and sympathomimetic medications, the frequency of intermittent clean intermittent catheterization, status of the upper urinary tract and comparative urodynamic findings preoperatively and postoperatively. RESULTS: We evaluated 7 boys 7 to 19 years old, of whom 4 were postpubertal, who fulfilled study criteria and had a followup of 1 to 9 years. In 4 patients a continent stoma was created concurrently at surgery. Postoperatively all patients were dry during the first 3 months after surgery. At the last followup 1 patient was completely dry, 3 had occasional nighttime wetting, 2 had occasional stress incontinence, and 1 had frequent daytime and nighttime wetting requiring subsequent bladder neck closure. Prepubertal and postpubertal males performed catheterization without difficulty and all required less frequent clean intermittent catheterization and medication postoperatively compared to preoperative status. None had hydronephrosis. Postoperatively urodynamic evaluation revealed normal bladder compliance, improved urethral resistance that did not decay with bladder filling and no uninhibited contractions. CONCLUSIONS: The rectus fascial sling is effective for increasing bladder outlet resistance and decreasing the degree of incontinence in prepubertal and postpubertal males with neurogenic sphincteric incontinence. It has no long-term deleterious effects on bladder function and does not impair the ability to catheterize postoperatively. A fascial sling is an effective alternative to bladder neck closure when creating a continent stoma.


Assuntos
Bexiga Urinaria Neurogênica/cirurgia , Adolescente , Adulto , Criança , Fasciotomia , Seguimentos , Humanos , Rim/cirurgia , Masculino , Resultado do Tratamento , Bexiga Urinaria Neurogênica/fisiopatologia , Urodinâmica/fisiologia
14.
J Urol ; 166(1): 233-5, 2001 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-11435876

RESUMO

PURPOSE: We present a modified technique of pyeloplasty that seems ideally suited for reoperative as well as primary repair of ureteropelvic junction obstruction due to high ureteral insertion. MATERIALS AND METHODS: This repair combines the dismembered technique with creation of a V-flap of renal pelvis by dividing and incising through the ureteropelvic junction superolateral onto the pelvis to a height above the most dependent portion of the pelvis just exceeding the length of ureteral spatulation. The V-flap is completed by an inferolateral incision directed toward the lateral aspect of the pelvis at its most dependent portion. The tip of the V-flap is then flipped down and approximated to the apex of the posterior ureteral spatulation. Of the 12 cases managed by this procedure 2 were reoperative and 10 involved select primary repair with high ureteral insertion at the ureteropelvic junction. Patient age was 3 months to 17 years (median 11 months). Two procedures were performed via a dorsal lumbar incision in the oldest patients and 10 were done via the standard anterior extraperitoneal approach. Radiological evaluation, including mercaptoacetyltriglycine renal scan and/or excretory urography, was performed in all patients preoperatively and postoperatively. RESULTS: In all patients postoperative mercaptoacetyltriglycine renal scan and/or excretory urography at 3 months and 1 year showed excellent drainage after dismembered V-flap pyeloplasty. CONCLUSIONS: Dismembered V-flap pyeloplasty has proved to be successful with a number of advantages over the Anderson-Hynes dismembered technique in select patients. These advantages include the avoidance of tissue tension that make mobilization of the kidney and ureter unnecessary, aggressive ureteral spatulation, creation of a dependent, funneled configuration and automatic tapering of a redundant pelvis in the routine course of closure without excision of tissue. This technique also has the advantage of being simpler than other flap repairs and it combines the physiological virtues of dismembered repair with the anatomical advantages of flap pyeloplasty.


Assuntos
Pelve Renal/cirurgia , Obstrução Ureteral/cirurgia , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Nefropatias/diagnóstico , Nefropatias/cirurgia , Pelve Renal/fisiopatologia , Masculino , Estudos Prospectivos , Retalhos Cirúrgicos , Resultado do Tratamento , Obstrução Ureteral/diagnóstico , Procedimentos Cirúrgicos Urológicos/métodos
15.
J Pediatr ; 138(6): 949-51, 2001 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-11391350

RESUMO

In boys with familial male-limited precocious puberty, an activating mutation of the luteinizing hormone receptor causes Leydig cell hyperplasia, resulting in excess testosterone production. There are no reports of Leydig cell masses in boys with familial male-limited precocious puberty. We describe a 10-year-old boy with familial male-limited precocious puberty who developed Leydig cell nodules.


Assuntos
Células Intersticiais do Testículo/patologia , Puberdade Precoce/genética , Puberdade Precoce/patologia , Criança , Humanos , Hiperplasia , Masculino , Puberdade Precoce/metabolismo , Testículo/diagnóstico por imagem , Testosterona/biossíntese , Ultrassonografia
16.
J Urol ; 165(6 Pt 2): 2224-7, 2001 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-11371949

RESUMO

PURPOSE: Endoscopic correction of vesicoureteral reflux continues to attract research of many autologous and nonautologous substances. We previously reported on the initial results of a clinical trial using endoscopic injection of autologous chondrocytes to correct vesicoureteral reflux in children and now present our greater than 1-year followup results. MATERIALS AND METHODS: A total of 29 children (47 ureters) with grades II to IV vesicoureteral reflux were treated at 2 centers. Each child underwent cystoscopy and posterior auricular cartilage harvesting at the initial setting. Chondrocytes were grown in culture during a 6-week period. Patients returned for transurethral injection of autologous chondrocytes into the ureterovesical junction of the refluxing ureters. Ultrasound was performed at 1 month, 1 year, and 2 and 3 years after implantation, and a voiding cystourethrogram or radionuclide cystogram at 3 months and 1 year after injection. If reflux persisted re-treatment with stored chondrocytes was offered. RESULTS: At 3-month followup initial chondrocyte injection corrected reflux in 55% of ureters (27 of 47) while a second or third injection was successful in additional 15 of 29 patients, resulting in an overall success rate of 86% (42 of 49) ureters and 25 of 29 patients. At 1-year followup reflux correction was maintained in 70% of ureters (32 of 46) and 65% of patients (19 of 29). The 1-year followup results after re-treatment of 3 ureters were not available. In those patients in whom implantation failed cystoscopy revealed evidence of volume loss and shifting of subureteral mounds to account for loss of the antireflux effect. Three patients underwent successful open ureteroneocystostomy for failed autologous chondrocyte implantation. There were no significant complications. CONCLUSIONS: Transurethral injection of autologous chondrocytes to correct vesicoureteral reflux in children is safe and reasonably effective. There is a relapse rate which must be considered. Changes in the formulation of the material have been made to enhance implant reliability and increase long-term success.


Assuntos
Condrócitos , Cistoscopia , Refluxo Vesicoureteral/cirurgia , Adolescente , Células Cultivadas , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Resultado do Tratamento , Ultrassonografia , Refluxo Vesicoureteral/diagnóstico por imagem
17.
Curr Opin Pediatr ; 13(2): 138-41, 2001 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-11317055

RESUMO

Prenatal hydronephrosis is diagnosed with an incidence of 1:100 to 1:500 maternal-fetal ultrasonographic studies. Although ultrasonography accurately describes dilation of the renal collecting system, it cannot define mechanical obstruction, a functional characteristic. The purpose of prenatal screening has changed from simple detection of hydronephrosis to selection for specific diagnosis-based management. The natural history of prenatal hydronephrosis is difficult to determine, and therefore physicians who take care of the fetus and infant are challenged with the following question: when does dilation of the renal collecting system indicate that serious pathology (ongoing renal deterioration) exists? The diagnosis of prenatal hydronephrosis, indications for and timing of prenatal intervention, and recommendations for treating children with a history of prenatal hydronephrosis are included in this review.


Assuntos
Hidronefrose/diagnóstico , Ultrassonografia Pré-Natal , Feminino , Humanos , Hidronefrose/terapia , Recém-Nascido , Masculino , Cuidado Pós-Natal , Gravidez , Cuidado Pré-Natal
18.
BJU Int ; 87(6): 484-9, 2001 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-11298039

RESUMO

OBJECTIVE: To combine and analyse the results from centres with a large experience of laparoscopy for the impalpable testis with small series, to determine the expected success rate for laparoscopic orchidopexy. METHODS: A questionnaire was distributed to participating paediatric urologists; each contributor retrospectively reviewed the clinical charts for their cases of therapeutic laparoscopy for an impalpable testis, detailing 36 variables for each patient. The data were collated centrally into a computerized database. For inclusion, the testis was intra-abdominal (including 'peeping' at the internal ring) at laparoscopic examination, was not managed through an open approach and did not undergo orchidectomy. Three surgical groups were assessed, with success defined as lack of atrophy and intrascrotal position: group 1, primary laparoscopic orchidopexy; group 2, a one-stage Fowler-Stephens (F-S) orchidopexy; and group 3, a two-stage F-S orchidopexy. RESULTS: Data were gathered from 10 centres in the USA, covering the period 1990-1999; 252 patients representing 310 testes were included and overall, 15.2% were lost to follow-up. There was no significant difference between success rates in the larger and smaller series. Atrophy occurred in 2.2% of 178 testes, 22.2% of 27 testes and 10.3% of 58 testes in groups 1-3, respectively. Testes were not in a satisfactory scrotal position in 0.6%, 7.4% and 1.7% of groups 1-3, respectively. The mean follow-up for each group was 7.7, 8.6 and 20.0 months, respectively. The overall success for all groups was 92.8% (97.2% group 1; 74.1% group 2; 87.9% group 3), with an atrophy rate of 6.1%. CONCLUSION: Laparoscopic orchidopexy for the intra-abdominal testis, in both large and small series, can be expected to have a success rate higher than that historically ascribed to open orchidopexy. Within this series, single-stage F-S laparoscopic orchidopexy resulted in a significantly higher atrophy rate than the two-stage repair. However, when considering both F-S approaches, the laparoscopic approach gave greater success than previously reported for the same open approaches. Despite the weaknesses inherent in a retrospective unrandomized study, we conclude that laparoscopic orchidopexy is, if not the procedure of choice, an acceptable and successful approach to the impalpable undescended testicle.


Assuntos
Criptorquidismo/cirurgia , Laparoscopia/efeitos adversos , Testículo/cirurgia , Pré-Escolar , Seguimentos , Humanos , Laparoscopia/métodos , Laparoscopia/normas , Masculino , Estudos Retrospectivos , Inquéritos e Questionários , Resultado do Tratamento
19.
J Urol ; 165(2): 581-5, 2001 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-11176441

RESUMO

PURPOSE: We evaluated the impact of tubularized incised plate urethroplasty on primary and repeat hypospadias repair. MATERIALS AND METHODS: We retrospectively reviewed the medical records of all boys who underwent hypospadias repair at our institution during a recent 3-year period. The level of the hypospadias defect, technique of repair, primary repair versus reoperation, age at surgery and complications were recorded. RESULTS: A total of 520 hypospadias repairs were done from May 1996 through June 1999. We began to perform tubularized incised plate urethroplasty in November 1996. During the ensuing consecutive 32 months 181 primary and 25 repeat hypospadias repairs were done using this technique. Mean patient age at surgery was 22 months (range 3 months to 30 years). During the 6 months immediately before we began to use this method the Mathieu flip-flap procedure was the most commonly performed technique, accounting for 38% of all hypospadias repairs. In contrast, during the last 6 months reviewed tubularized incised plate urethroplasty accounted for 63% of all repairs, including 41 of 65 primary operations (63%) and 4 of 6 reoperations (67%), while no Mathieu procedures were performed. Postoperative followup was 6 to 38 months for tubularized incised plate repair. Overall meatal stenosis and a urethrocutaneous fistula developed in 1 and 14 boys, respectively (7% complication rate). CONCLUSIONS: Tubularized incised plate urethroplasty has become the preferred technique of primary and repeat hypospadias repair at our institution. The technique has few complications as well as proved success and versatility that continues to expand its applicability and popularity.


Assuntos
Hipospadia/cirurgia , Uretra/cirurgia , Adolescente , Adulto , Criança , Pré-Escolar , Humanos , Lactente , Masculino , Reoperação , Estudos Retrospectivos , Procedimentos Cirúrgicos Urológicos Masculinos/métodos
20.
Urol Clin North Am ; 27(3): 529-41, 2000 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-10985152

RESUMO

The spectrum of Sertoli cell tumors in children covers a wide range of testis and ovarian tumors classified as sex cord-stromal tumors. Sertoli cell tumor of the testis is extremely rare in the pediatric population. The American Academy of Pediatrics Section on Urology Prepubertal Testicular Tumor Registry has reported a total of six cases of Sertoli cell tumor of the testis, accounting for 1.3% of the 430 cases reported to the registry as of October 1996. Despite their rarity, Sertoli cell variants of sex cord-stromal tumors have generated keen interest because of their variable histologic appearance and biologic behavior, including endocrine activity. Because sex cord-stromal tumors occur in the ovary and testis, a primitive cellular origin to these tumors is likely in males and females.


Assuntos
Neoplasias Ovarianas/patologia , Tumor de Células de Sertoli/patologia , Neoplasias Testiculares/patologia , Criança , Feminino , Humanos , Masculino , Tumores do Estroma Gonadal e dos Cordões Sexuais/patologia
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