RESUMEN
INTRODUCTION: After 5 years experience with the GUD (glandular urethral disassembly) technique for distal hypospadias, we present the GUDplay technique, incorporating Thiersch-Duplay tubularization of the plate till the coronal area, disassembling the glans aggressively and refurbishing the glans. METHODS: We defined the urethral plate and designed an inverted Y incision to open the glans in two wings. The glans was entirely detached from the corpora to gain a great mobility that allowed minor cranial mobilization of the urethra and caudal rotation of the wings. In sequence, there are well-known steps: Duplay urethroplasty, spongioblasts and a Dartos flap to cover the neourethra. The glans was connected to the urethra by 6.0 PDS sutures except in the ventral meatus and the glans wings are joined in the midline. RESULTS: The 5-year-old patient had midshaft hypospadias without previous surgery. The catheter was removed after a week and the healing appears to be good. DISCUSSION: We combined principles of total glans deconstruction in association to Duplay tubularization and then lifted it up to the tip of the glans divided in two wide and mobile wings. We have treated a small series of 6 cases without complications and mean follow-up of 6.2 months.
Asunto(s)
Hipospadias , Pene , Procedimientos de Cirugía Plástica , Uretra , Procedimientos Quirúrgicos Urológicos Masculinos , Hipospadias/cirugía , Masculino , Humanos , Procedimientos Quirúrgicos Urológicos Masculinos/métodos , Preescolar , Pene/cirugía , Pene/anomalías , Procedimientos de Cirugía Plástica/métodos , Uretra/cirugía , Uretra/anomalías , Escroto/cirugía , Colgajos QuirúrgicosRESUMEN
Hypospadias is an uncommon sexual development disorder in cats, in which the urethral opening is not in its anatomical location on the penis. The purpose of this report is to describe two cases of hypospadias in the feline species. The first cat was asymptomatic, had a history of bacterial cystitis, and was diagnosed with perineal hypospadias at an appointment for preoperative evaluation of orchiectomy. The second cat had clinical signs of dysuria and pollakiuria for 30 days and had glandular hypospadias. Both cats showed abnormalities in the urinalysis which were suggestive of lower urinary tract disease. For both cases, clinical treatment with antibiotic therapy was performed. In the first patient, surgical treatment consisted of orchiectomy, while in the second animal a perineal urethrostomy and orchiectomy were performed. The cats had a satisfactory recovery after the treatments. Performing a thorough physical examination is essential to diagnose cases of hypospadias and choose the best treatment for each patient.
A hipospadia é uma desordem do desenvolvimento sexual pouco comum nos gatos, na qual a abertura uretral não está em sua localização anatômica do pênis. O objetivo do presente relato é descrever dois casos de hipospadia em felinos domésticos. O primeiro gato era assintomático, tinha histórico de cistite bacteriana prévio, e foi diagnosticado com hipospadia perineal em uma consulta para avaliação pré-cirúrgica de orquiectomia. O segundo gato apresentava sinais clínicos de disúria e polaquiúria há 30 dias e apresentava hipospadia glandular. A partir dos exames complementares, pôde-se observar que os animais, além do defeito anatômico, apresentavam alterações sugestivas de doença do trato urinário inferior. Para ambos os casos, foi realizado o tratamento clínico inicial com antibioticoterapia. No primeiro paciente, optou-se pelo procedimento de orquiectomia, enquanto no segundo animal foram realizadas as técnicas de uretrostomia perineal e orquiectomia. Os gatos mostraram recuperação satisfatória após os tratamentos instituídos. Dessa forma, pode-se observar a importância de se realizar um exame físico minucioso a fim de diagnosticar os casos de hipospadia e escolher o tratamento correto para cada paciente.
Asunto(s)
Animales , Gatos , Trastornos del Desarrollo Sexual/veterinaria , Uretra/anomalías , Enfermedades Uretrales/veterinaria , Enfermedades de los Gatos , Hipospadias/veterinariaRESUMEN
A hipospadia é oresultado de falha no desenvolvimento em que não ocorre a fusão normal das dobras genitais e intumescência genital, o que causa desenvolvimento anormal da uretra peniana, do pênis, prepúcio e/ou escroto. O objetivodeste relato é descrever um caso de hipospadia peniana de um canino macho, sem raça definida (SRD), de oito meses deidade, atendido no Hospital Veterinário da Universidade Federal Rural da Amazônia, na cidade de Belém/Pará, detalhando os procedimentos cirúrgico e anestésico utilizados para resolução do caso. Ao chegar ao hospital, o animal foi submetido ao exame físicoinicial no qual observou-se uma deformidade na região perianal por onde drenava a urina e uma abertura na região prepucial. A partir disso, foi solicitada a uretrocistografia retrógrada para complemento diagnóstico da hipospadia e seu comprometimento ao sistema urinário interno. Diante disso, foi realizado procedimento cirúrgico reconstrutivo e orquiectomia terapêutica, alcançando permitindoa resolução do caso e a recuperação completa do animal. Considerando que essa deformidade impossibilita a capacidadesexual generandi, a resolução recomendada é a cirúrgica, na qual o prognóstico é bom, possibilitando qualidade de vida ao animal.(AU)
Hypospadias is the result of developmental failure where normal fusion of the genital folds and genital tumescence does not occur, which causes abnormal development of the penile urethra, penis, foreskin and/or scrotum. The objective of this report is to describe a case of penile hypospadias in an eight-month-old male mixed breed (SRD) canine, treated at the Veterinary Hospital of the Federal Rural University of Amazônia, in the city of Belém/Pará, detailing the procedures surgical and anesthetic used for resolution of the case. Upon arrival at the hospital, the animal underwent an initial physical examination in which a deformity was observed in the perianal region through which urine drained and an opening in the preputial region. From this, a retrograde urethrocystography was requested to complement the diagnosis of hypospadias and its commitment to the internal urinary system. In view of this, a reconstructive surgical procedure and therapeutic orchiectomy were performed, allowing the resolution of the case and the complete recovery of the animal. Considering that this deformity prevents the sexual capacity generandi, the recommended resolution is surgery, in which the prognosis is good, allowing quality of life for the animal.(AU)
La hipospadias es el resultado de una falla en el desarrollo donde no ocurre la fusión normal de los pliegues genitales y la tumescencia genital, lo que provoca un desarrollo anormal de la uretra peneana, el pene, el prepucio y/o el escroto. El objetivo de este relato es describir un caso de hipospadias peneana en un canino macho mestizo (SRD) de ocho meses de edad, atendido en el Hospital Veterinario de la Universidad Federal Rural de Amazônia, en la ciudad de Belém/Pará, detallando los procedimientos quirúrgicos y anestésicos utilizados para la resolución del caso. A su llegada al hospital, el animal fue sometido a un examen físico inicial en el que se observó una deformidad en la región perianal por donde salía la orina y una abertura en la región prepucial. A partir de ello se solicitó cistouretrografía retrógrada para complementar el diagnóstico de hipospadias y su afectación en el sistema urinario interno. Ante esto, se realizó un procedimiento quirúrgico reconstructivo y orquiectomía terapéutica, que permitió la resolución del caso y la recuperación completa del animal. Teniendo en cuenta que esta deformidad imposibilita la capacidadgeneradi sexual, la resolución recomendada es la cirugía, en la que el pronóstico es bueno, permitiendo calidad de vida para el animal.(AU)
Asunto(s)
Animales , Masculino , Procedimientos Quirúrgicos Operativos/veterinaria , Uretra/anomalías , Hipospadias/diagnóstico , BrasilRESUMEN
Hypospadia is the imperfect closure of the external urethra of the male which results from the incomplete fusion of the urogenital folds and abnormal openings of the penile urethra. The urethral diverticulum is the abnormal dilatation of the urethra, and can be of multifactorial origin and related to genetic, endocrinological and environmental factors. The objective of this article is to report on the clinical and surgical treatment of two cases of hypospadias and one case of urethral diverticulum in goats raised as pets, originated from cross breeding polled pairs. In Case 1 the hypospadias was characterized by two orifices on the ventral portion of the penis, separated by a 2 cm interruption of the urethra, and in Case 2 by an orifice on the ventral portion in the region of the urethra. In both cases there was an accumulation of urine in the middle ventral region of the foreskin, leading to distension of the skin. Both cases were successfully treated by urethrostomy and partial penectomy with fixation of the proximal urethral ostium to the skin. In Case 3, the animal was seen at ten days of age with presence of a diverticulum in the preputial region, however, the animal returned for new care only after four months due to urethral obstruction and died as a result of advanced azotemia. Surgical treatment of hypospadia, when performed before the development of azotemia associated with urinary retention, has been shown to be efficient, and post-operative care is important
Hipospadia é o fechamento imperfeito da uretra externa do macho, resultado da falha de fusão das pregas urogenitais e da formação incompleta da uretra peniana e o divertículo uretral é a dilatação anormal da uretra, podendo ser de origem multifatorial e relacionada a fatores genéticos, endocrinológicos e ambientais. O objetivo desse artigo é relatar o tratamento clínico-cirúrgico de dois casos de hipospadia e um de divertículo uretral em caprinos criados como animais de estimação, oriundos de cruzamentos de casal mocho. No Caso 1 a hipospadia era caracterizada por dois orifícios ventrais ao pênis, separados por uma interrupção de 2 cm da uretra e no Caso 2 por um orifício na face ventral na região da uretra, em ambos os casos ocorreu o acúmulo de urina na região médio ventral do prepúcio levando a distensão da pele. Os dois casos foram tratados com sucesso através da uretrostomia e penectomia parcial com fixação do óstio uretral proximal a pele. Já no Caso 3, o animal foi atendido com dez dias de vida com presença de divertículo na região prepucial, no entanto retornou para novo atendimento somente após quatro meses devido obstrução uretral, causando quadro grave de azotemia e a morte. O tratamento cirúrgico da hipospadia, quando realizada antes do desenvolvimento de azotemia associado a retenção urinária, mostrou-se eficiente, sendo importante atenção os cuidados pós-operatórios
Asunto(s)
Animales , Masculino , Uretra/anomalías , Cabras/anomalías , Prepucio/anomalías , Hipospadias/veterinaria , Anomalías Urogenitales/veterinariaRESUMEN
INTRODUCTION: Posterior urethral valve (PUV) is a congenital malformation characterized by a membranous structure located in the prostatic portion of the male posterior urethra that obstructs the urinary flow. Efforts have been made to determine the degree of impairment of fetal kidney function in this condition. OBJECTIVE: This study aimed to measure the levels of urinary biomarkers of glomerular and tubular functions in fetuses with PUV and to compare with the levels of the same molecules in healthy male premature newborns. STUDY DESIGN: Urine samples from 43 fetuses with PUVs were collected and compared with urine samples from 40 healthy male newborns of the same gestational age (controls). Tubular and glomerular biomarkers levels were measured in urine samples by MILLIPLEX® assay kits. Levels of the molecules were related to creatinine (Cr) measurements at same urine samples and expressed as pg/mg Cr. Results were analysed with Graphpad Prism version 7.0 and SPSS version 20.0. RESULTS: Fetuses with PUV showed a significant reduction in urine levels of Epidermal Growth Factor (EGF), Calbindin, Osteoactivin, Molecule Renal Injury 1 (KIM-1 and Factor of trefoil 3 (TFF-3) when compared to controls. On the other hand, urine levels of cystatin C and renin were higher in PUV fetuses. The levels of molecules also differed according to urine osmolality and grade of hydronephrosis. DISCUSSION: Some urinary excreted molecules may indicate kidney damage in several segments along the nephron, while others may exert important functions. Mechanical and immunological mechanisms related to PUV might significantly modulate the synthesis of cytokines related to glomerular and tubular physiology, leading to alterations in urinary concentrations of those molecules. These biomarkers can be used as future diagnostic and prognostic markers in clinical practice. CONCLUSION: Early kidney structural and functional impairment influenced the synthesis of glomerular and tubular molecules related to kidney physiological processes in fetuses with PUV.
Asunto(s)
Enfermedades Renales , Obstrucción Uretral , Biomarcadores/orina , Creatinina/orina , Feto , Humanos , Recién Nacido , Masculino , Uretra/anomalíasRESUMEN
INTRODUCTION: Diphallia is a rare anomaly. It has a range of appearances from a small accessory penile to complete duplication. METHODS: We present a 2 year-old boy with complete penile duplication. The left penile was the largest. NMR (Nuclear Magnetic Resonance) suggested one corporal body for each penile and VCUG (Voiding Cystourethrogram) showed a normal urethra in the right penile and stricture at glandular and mid penile urethra of the left penis. A Y confluence to bulbar urethra was observed confirming only one prostate and bladder. RESULTS: The cystoscopy through the right penile identified the urethral confluence in the bulbar area. We performed a meatotomy in the left penile to insert the cystoscope and confirmed the blind ending urethra. We decided to remove this penile. The penile was degloved entirely and clamped and took out the corpora at the base. DISCUSSION: Diphallia can have three presentations: only glans duplication, bifid diphallia and complete diphallia (two corpora cavernosa and a corpus spongiosum for each penile). In our case, each penile presented only one corpora cavernosa and the decision taken was based on urethral patency. CONCLUSION: The treatment should always be planned individually whereas associated anomalies with the goal of attaining satisfactory functional and cosmetic results.
Asunto(s)
Enfermedades del Pene , Preescolar , Humanos , Imagen por Resonancia Magnética , Masculino , Pene/anomalías , Pene/diagnóstico por imagen , Pene/cirugía , Uretra/anomalías , Uretra/diagnóstico por imagen , Uretra/cirugía , Vejiga UrinariaRESUMEN
BACKGROUND: To describe the perinatal outcomes of fetoscopic urethral meatotomy (FUM) in fetuses with lower urinary tract obstruction (LUTO) by congenital megalourethra. STUDY DESIGN: Between 2012 and 2020, 226 cases with LUTO were referred to our fetal surgery center in Queretaro, Mexico. We report the perinatal outcome of cases with LUTO by congenital megalourethra that were selected for FUM in an attempt to release the penile urethral obstruction. RESULTS: Congenital megalourethra was diagnosed in 10 cases (4.4%) but only 3 cases (30%) with obstructive megalourethra and megacystis were selected for fetal surgery. Fetoscopic urethral metatotomy was successfully performed in all three cases at a median gestational age (GA) of 21.4 (18.0-26.7) weeks and with a median surgical time of 27 (12-43) min. A resolution of urethral dilatation and subsequent reduction of the penile length and normalization of both the bladder size and amniotic fluid were observed in all cases. The median GA at delivery was 35.2 (range: 30.6-38.0) weeks. There were no fetal deaths but one neonatal death (33%) secondary to renal failure and preterm delivery. CONCLUSION: In fetuses with LUTO by congenital obstructive megalourethra, FUM is feasible and is associated with good perinatal outcomes.
Asunto(s)
Fetoscopía/métodos , Obstrucción Uretral/congénito , Femenino , Fetoscopía/tendencias , Edad Gestacional , Humanos , Lactante , Recién Nacido , Masculino , México/epidemiología , Pene/anomalías , Pene/cirugía , Embarazo , Ultrasonografía Prenatal/métodos , Ultrasonografía Prenatal/estadística & datos numéricos , Uretra/anomalías , Obstrucción Uretral/epidemiología , Obstrucción Uretral/cirugíaRESUMEN
OBJECTIVE: To reliably and quickly diagnose children with posterior urethral valves (PUV), we developed a multi-instance deep learning method to automate image analysis. METHODS: We built a robust pattern classifier to distinguish 86 children with PUV from 71 children with mild unilateral hydronephrosis based on ultrasound images (3504 in sagittal view and 2558 in transverse view) obtained during routine clinical care. RESULTS: The multi-instance deep learning classifier performed better than classifiers built on either single sagittal images or single transverse images. Particularly, the deep learning classifiers built on single images in the sagittal view and single images in the transverse view obtained area under the receiver operating characteristic curve (AUC) values of 0.796 ± 0.064 and 0.815 ± 0.071, respectively. AUC values of the multi-instance deep learning classifiers built on images in the sagittal and transverse views with mean pooling operation were 0.949 ± 0.035 and 0.954 ± 0.033, respectively. The multi-instance deep learning classifiers built on images in both the sagittal and transverse views with a mean pooling operation obtained an AUC of 0.961 ± 0.026 with a classification rate of 0.925 ± 0.060, specificity of 0.986 ± 0.032, and sensitivity of 0.873 ± 0.120, respectively. Discriminative regions of the kidney located using classification activation mapping demonstrated that the deep learning techniques could identify meaningful anatomical features from ultrasound images. CONCLUSION: The multi-instance deep learning method provides an automatic and accurate means to extract informative features from ultrasound images and discriminate infants with PUV from male children with unilateral hydronephrosis.
Asunto(s)
Aprendizaje Profundo , Hidronefrosis/diagnóstico , Interpretación de Imagen Asistida por Computador/métodos , Anomalías Urogenitales/diagnóstico , Reflujo Vesicoureteral/diagnóstico , Estudios de Casos y Controles , Diagnóstico Diferencial , Estudios de Factibilidad , Femenino , Humanos , Lactante , Recién Nacido , Riñón/anomalías , Riñón/diagnóstico por imagen , Masculino , Curva ROC , Reproducibilidad de los Resultados , Ultrasonografía/métodos , Uretra/anomalías , Uretra/diagnóstico por imagenRESUMEN
Abstract Objective: Posterior urethral valve is the most common lower urinary tract obstruction in male children. A high percentage of patients with posterior urethral valve evolve to end‐stage renal disease. Previous studies showed that cytokines, chemokines, and components of the renin-angiotensin system contribute to the renal damage in obstructive uropathies. The authors recently found that urine samples from fetuses with posterior urethral valve have increased levels of inflammatory molecules. The aim of this study was to measure renin-angiotensin system molecules and to investigate their correlation with previously detected inflammatory markers in the same urine samples of fetuses with posterior urethral valve. Methods: Urine samples from 24 fetuses with posterior urethral valve were collected and compared to those from 22 healthy male newborns at the same gestational age (controls). Renin-angiotensin system components levels were measured by enzyme‐linked immunosorbent assay. Results: Fetuses with posterior urethral valve presented increased urinary levels of angiotensin (Ang) I, Ang‐(1‐7) and angiotensin‐converting enzyme 2 in comparison with controls. ACE levels were significantly reduced and Ang II levels were similar in fetuses with posterior urethral valve in comparison with controls. Conclusions: Increased urinary levels of angiotensin‐converting enzyme 2 and of Ang‐(1‐7) in fetuses with posterior urethral valve could represent a regulatory response to the intense inflammatory process triggered by posterior urethral valve.
Resumo Objetivo: A válvula de uretra posterior é a obstrução do trato urinário inferior mais comum em crianças do sexo masculino. Uma alta porcentagem de pacientes com válvula de uretra posterior evolui para doença renal em estágio final. Estudos anteriores mostraram que citocinas, quimiocinas e componentes do sistema renina-angiotensina contribuem para o dano renal em uropatias obstrutivas. Recentemente, descobrimos que amostras de urina de fetos com válvula de uretra posterior tinham níveis aumentados de moléculas inflamatórias. O objetivo deste estudo foi medir as moléculas de renina-angiotensina e investigar sua correlação com marcadores inflamatórios previamente detectados nas mesmas amostras de urina de fetos com válvula de uretra posterior. Métodos: Amostras de urina de 24 fetos com válvula de uretra posterior foram coletadas e comparadas com amostras de urina de 22 recém-nascidos saudáveis de mesma idade gestacional (controles). Os níveis dos componentes de SRA foram medidos por ensaio de imunoabsorção enzimática. Resultados: Os fetos com válvula de uretra posterior apresentaram níveis urinários aumentados de angiotensina (Ang) I, Ang-(1-7) e enzima conversora de angiotensina 2 em comparação com os controles. Os níveis de enzima conversora de angiotensina eram significativamente menores e os níveis de Ang II eram semelhantes nos fetos com válvula de uretra posterior em comparação com os controles. Conclusões: O aumento dos níveis urinários de enzima conversora de angiotensina 2 e de Ang-(1-7) em fetos com válvula de uretra posterior poderia representar uma resposta regulatória ao intenso processo inflamatório desencadeado pela válvula de uretra posterior.
Asunto(s)
Humanos , Masculino , Femenino , Embarazo , Recién Nacido , Fragmentos de Péptidos/orina , Uretra/anomalías , Enfermedades Uretrales/orina , Angiotensina I/orina , Angiotensina II/orina , Peptidil-Dipeptidasa A/orina , Feto/anomalías , Uretra/embriología , Enfermedades Uretrales/diagnóstico , Enfermedades Uretrales/embriología , Biomarcadores/orina , Estudios de Casos y Controles , Técnicas de InmunoadsorciónRESUMEN
OBJECTIVE: Posterior urethral valve is the most common lower urinary tract obstruction in male children. A high percentage of patients with posterior urethral valve evolve to end-stage renal disease. Previous studies showed that cytokines, chemokines, and components of the renin-angiotensin system contribute to the renal damage in obstructive uropathies. The authors recently found that urine samples from fetuses with posterior urethral valve have increased levels of inflammatory molecules. The aim of this study was to measure renin-angiotensin system molecules and to investigate their correlation with previously detected inflammatory markers in the same urine samples of fetuses with posterior urethral valve. METHODS: Urine samples from 24 fetuses with posterior urethral valve were collected and compared to those from 22 healthy male newborns at the same gestational age (controls). Renin-angiotensin system components levels were measured by enzyme-linked immunosorbent assay. RESULTS: Fetuses with posterior urethral valve presented increased urinary levels of angiotensin (Ang) I, Ang-(1-7) and angiotensin-converting enzyme 2 in comparison with controls. ACE levels were significantly reduced and Ang II levels were similar in fetuses with posterior urethral valve in comparison with controls. CONCLUSIONS: Increased urinary levels of angiotensin-converting enzyme 2 and of Ang-(1-7) in fetuses with posterior urethral valve could represent a regulatory response to the intense inflammatory process triggered by posterior urethral valve.
Asunto(s)
Angiotensina II/orina , Angiotensina I/orina , Feto/anomalías , Fragmentos de Péptidos/orina , Peptidil-Dipeptidasa A/orina , Uretra/anomalías , Enfermedades Uretrales/orina , Enzima Convertidora de Angiotensina 2 , Biomarcadores/orina , Estudios de Casos y Controles , Femenino , Humanos , Técnicas de Inmunoadsorción , Recién Nacido , Masculino , Embarazo , Uretra/embriología , Enfermedades Uretrales/diagnóstico , Enfermedades Uretrales/embriologíaRESUMEN
BACKGROUND: Posterior urethral valves (PUVs) are associated with severe consequences to the urinary tract and are a common cause of chronic kidney disease (CKD). The aim of this study was to develop clinical predictive model of CKD in a cohort of patients with PUVs. METHODS: In this retrospective cohort study, 173 patients with PUVs were systematically followed up at a single tertiary unit. The primary endpoint was CKD ≥ stage 3. Survival analyses were performed by Cox regression proportional hazard models with time-fixed and time-dependent covariables. RESULTS: Mean follow-up time was 83 months (SD, 70 months). Sixty-five children (37.6%) developed CKD stage ≥ 3. After adjustment by the time-dependent Cox model, baseline creatinine, nadir creatinine, hypertension, and proteinuria remained as predictors of the endpoint. After adjustment by time-fixed model, three variables were predictors of CKD ≥ stage 3: baseline creatinine, nadir creatinine, and proteinuria. The prognostic risk score was divided into three categories: low-risk (69 children, 39.9%), medium-risk (45, 26%), and high-risk (59, 34.1%). The probability of CKD ≥ stage 3 at 10 years age was estimated as 6%, 40%, and 70% for patients assigned to the low-risk, medium-risk, and high-risk groups, respectively (P < 0.001). The main limitation was the preclusion of some relevant variables, especially bladder dysfunction, that might contribute to a more accurate prediction of renal outcome. CONCLUSION: The model accurately predicts the risk of CKD in PUVs patients. This model could be clinically useful in applying timely intervention and in preventing the impairment of renal function.
Asunto(s)
Modelos Biológicos , Insuficiencia Renal Crónica/epidemiología , Uretra/anomalías , Obstrucción Uretral/complicaciones , Preescolar , Progresión de la Enfermedad , Estudios de Factibilidad , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Modelos de Riesgos Proporcionales , Insuficiencia Renal Crónica/etiología , Estudios Retrospectivos , Medición de Riesgo/métodos , Factores de Riesgo , Análisis de Supervivencia , Obstrucción Uretral/congénito , Obstrucción Uretral/mortalidadRESUMEN
BACKGROUND: Posterior urethral valves (PUVs) constitute the most common infravesical urinary obstruction in boys and are often accompanied by severe consequences to the lower and upper urinary tract. Currently, about two-thirds of diagnosis of PUVs has been suspected by prenatal ultrasonography findings. The aim of this study was to compare long-term clinical outcomes in two groups of patients with PUVs, with antenatal vs. postnatal diagnosis. STUDY DESIGN: This was a retrospective cohort study of 173 patients with PUVs systematically followed up in a tertiary center. Median follow-up time was 66.5 months (interquartile range [IQ], 11.4-147.9 months) for those patients who survived neonatal period. Seventy-nine (45.6%) patients were followed up for more than 5 years and 55 (32%) for more than 10 years. For analysis, the cohort was stratified into two groups according to the clinical presentation (prenatal vs. postnatal). The events of interest were urinary tract infection (UTI), surgical interventions, proteinuria, hypertension, chronic kidney disease (CKD), and death. Survival analyses were performed to evaluate time until occurrence of the events. RESULTS: Sixty-two patients (35.8%) were diagnosed by fetal sonography. Patients of postnatal group presented a higher incidence rate of UTI episodes (6.5, 95% confidence interval [CI], 4.9-8.3) than antenatal group (1.2, 95% CI, 0.4-2.7) (P < 0.001). Thirty-six patients (21%) presented hypertension, and 77 (44.5%) had persistent mild proteinuria. There was no significant difference in the estimated incidence of hypertension (P = 0.28) and proteinuria (P = 0.78) between antenatal and postnatal groups. The cumulative incidence of CKD stage ≥3 was estimated to be about 37% at 10 years of age, and 56% at 18 years of age. By survival analysis, there was no significant difference in the estimated incidence of CKD stage ≥3 (log-rank = 0.32, P = 0.57) and CKD stage 5 (log-rank = 1.08, P = 0.28, Figure) between antenatal and postnatal groups. Of 173 patients included in the analysis, 13 (7.5%) died during follow-up with a median age of 2.6 months (IQ, 15 days-62 months). Survival analyses have not shown any significant difference in the estimated incidence of death between antenatal and postnatal groups (log-rank = 1.38, P = 0.24). CONCLUSION: The study findings did not corroborate the initial hypothesis that the rates of renal function declining in patients with PUVs would be attenuated by an early diagnosis and intervention after antenatal diagnosis.
Asunto(s)
Ultrasonografía Prenatal , Uretra/anomalías , Uretra/diagnóstico por imagen , Enfermedades Urológicas/epidemiología , Adolescente , Niño , Preescolar , Estudios de Cohortes , Femenino , Humanos , Incidencia , Lactante , Recién Nacido , Masculino , Embarazo , Estudios Retrospectivos , Resultado del Tratamiento , Uretra/cirugía , Obstrucción Uretral/complicaciones , Obstrucción Uretral/epidemiología , Obstrucción Uretral/cirugía , Enfermedades Urológicas/complicaciones , Enfermedades Urológicas/cirugíaRESUMEN
ABSTRACT Introduction and objective Urethral duplication is a rare congenital anomaly, with roughly 200 cases reported in the literature (1). It is more frequent in males, with few cases reported in females. The clinical presentation differs according to the anatomical variant present. The duplication most commonly occurs in the sagittal plane with one urethra located ventrally and the other dorsally (2). Usually the ventral urethra is the more functional of both. Duplications occurring in the coronal plane are quite rare and they are usually associated with bladder duplication (3). The purpose of this paper was to present a video of a boy with an unusual urethral duplication form. Materials and Methods Patient was born premature due to oligohydramnios at 7 months-gestational age and he has initial diagnosis of hypospadia. Since then, he presented at least 7 febrile UTI and mother complained of difficult micturition and a presence of a mass at lower abdomen. Patient was referred to our institution and we identified urethral duplication with a glandar and scrotal meatus, palpable bladder and left penile-hemiscrotum transposition. US and CT-scan showed left kidney agenesis and overdistended bladder. VCUG and retrograde urethrography showed duplication, presence of contrast in the seminal vesicles and complete catheterizing of both urethras was not possible. Results The topic urethra was dysplastic and not patent to a 4Fr plastic tube so we were unable to access it endoscopically. We performed initially a Mitrofanoff procedure to allow CIC and treat chronic retention. Six months later, we assessed both urethras surgically and concluded that dorsal urethra was dysplastic after 3cm still in the penile area and scrotal urethra was not possible to be catheterized. We excised the ventral urethra because of dribbling complaints up to bulbar area and reconstructed the scrotal transposition, keeping the topic urethra for cosmetic issues. Patient had excellent outcome, performs CIC every 4 hours and has not presented further UTI episodes. Discussion and conclusion The urethral duplication is an anomaly that has multiple anatomical presentations. There are several theories about the etiology, but none can explain all types of presentations. There is also more than one rating available, and the Effmann classification is the most detailed. The case exemplifies this varied spectrum of anatomic urethral duplication. It resembles the urethral duplication type IIa-Y, however, ventral urethra meatus was located in penoscrotal area and both urethras were at least partially hypoplastic/dysplastic associated with obstruction and bladder retention. In determining how to best manage a patient with Y-type urethral duplication, the caliber and quality of the orthotopic urethra must first be assessed. Published reports suggest that best outcomes are those using the ventral duplicated urethra for the reconstruction (4). In this case, none of urethras were functional and a supravesical outlet channel had to be provided. The treatment of this condition requires an individualized planning and a vast technical knowledge of reconstructive surgery.
Asunto(s)
Humanos , Masculino , Recién Nacido , Uretra/anomalías , Anomalías Congénitas/cirugía , Retención Urinaria/cirugía , Hipospadias/cirugía , Riñón/anomalías , Enfermedades Renales/congénito , Enfermedad Crónica , Retención Urinaria/complicaciones , Hipospadias/complicaciones , Riñón/cirugía , Enfermedades Renales/cirugía , Enfermedades Renales/complicacionesRESUMEN
INTRODUCTION: Y-type urethral duplication describes the condition in which a functional urethra is implanted in the rectum, and there is also a dysplastic topic urethra which produces mostly urinary dribbling. These patients are at risk of urinary tract complications and UTI. We aimed to present the surgical steps of a case treated by ASTRA approach in which we separated the urethra from the rectum and created a perineal urethrostomy. MATERIAL AND METHODS: We treated a 5-month-old boy with Y-type (IIA-2) urethral duplication, in whom the orthotopic urethra was patent just in the penile segment. The patient had urinary flow per anus and minimal dribbling through the orthotopic urethra. We performed a combined cystoscopy with retrograde urethrogram and managed to catheterize the dysplastic urethra with a guide-wire that showed ectopic implantation in the prostatic urethra, below the bladder neck. We performed an ASTRA procedure to separate the urethra from the rectum. The urethral stump was further mobilized to the perineum and anastomosed to a perineal skin flap to create a stoma and minimize the risk of stricture. RESULTS: The patient was followed at 2 month-intervals, and at 6 months follow-up had an excellent outcome. CONCLUSION: The ASTRA approach proved to be an excellent alternative for Y-type urethral duplication with functional urethra implanted in the rectum. We believe that further efforts to reconstruct the urethra should be avoided, with a better and simpler option being to create a definitive perineal urethrostomy.
Asunto(s)
Procedimientos de Cirugía Plástica/métodos , Recuperación de la Función/fisiología , Recto/cirugía , Uretra/anomalías , Uretra/cirugía , Anomalías Urogenitales/cirugía , Estudios de Seguimiento , Humanos , Lactante , Masculino , Posicionamiento del Paciente , Pene/cirugía , Posición Prona , Enfermedades Raras , Recto/anomalías , Medición de Riesgo , Resultado del Tratamiento , Infecciones Urinarias/prevención & control , Anomalías Urogenitales/diagnóstico , Procedimientos Quirúrgicos Urológicos Masculinos/métodosRESUMEN
INTRODUCTION AND OBJECTIVE: Urethral duplication is a rare congenital anomaly, with roughly 200 cases reported in the literature (1). It is more frequent in males, with few cases reported in females. The clinical presentation differs according to the anatomical variant present. The duplication most commonly occurs in the sagittal plane with one urethra located ventrally and the other dorsally (2). Usually the ventral urethra is the more functional of both. Duplications occurring in the coronal plane are quite rare and they are usually associated with bladder duplication (3). The purpose of this paper was to present a video of a boy with an unusual urethral duplication form. MATERIALS AND METHODS: Patient was born premature due to oligohydramnios at 7 months-gestational age and he has initial diagnosis of hypospadia. Since then, he presented at least 7 febrile UTI and mother complained of difficult micturition and a presence of a mass at lower abdomen. Patient was referred to our institution and we identified urethral duplication with a glandar and scrotal meatus, palpable bladder and left penile-hemiscrotum transposition. US and CT-scan showed left kidney agenesis and overdistended bladder. VCUG and retrograde urethrography showed duplication, presence of contrast in the seminal vesicles and complete catheterizing of both urethras was not possible. RESULTS: The topic urethra was dysplastic and not patent to a 4Fr plastic tube so we were unable to access it endoscopically. We performed initially a Mitrofanoff procedure to allow CIC and treat chronic retention. Six months later, we assessed both urethras surgically and concluded that dorsal urethra was dysplastic after 3cm still in the penile area and scrotal urethra was not possible to be catheterized. We excised the ventral urethra because of dribbling complaints up to bulbar area and reconstructed the scrotal transposition, keeping the topic urethra for cosmetic issues. Patient had excellent outcome, performs CIC every 4 hours and has not presented further UTI episodes. DISCUSSION AND CONCLUSION: The urethral duplication is an anomaly that has multiple anatomical presentations. There are several theories about the etiology, but none can explain all types of presentations. There is also more than one rating available, and the Effmann classification is the most detailed. The case exemplifies this varied spectrum of anatomic urethral duplication. It resembles the urethral duplication type IIa-Y, however, ventral urethra meatus was located in penoscrotal area and both urethras were at least partially hypoplastic/dysplastic associated with obstruction and bladder retention. In determining how to best manage a patient with Y-type urethral duplication, the caliber and quality of the orthotopic urethra must first be assessed. Published reports suggest that best outcomes are those using the ventral duplicated urethra for the reconstruction (4). In this case, none of urethras were functional and a supravesical outlet channel had to be provided. The treatment of this condition requires an individualized planning and a vast technical knowledge of reconstructive surgery.
Asunto(s)
Anomalías Congénitas/cirugía , Hipospadias/cirugía , Enfermedades Renales/congénito , Riñón/anomalías , Uretra/anomalías , Retención Urinaria/cirugía , Enfermedad Crónica , Humanos , Hipospadias/complicaciones , Recién Nacido , Riñón/cirugía , Enfermedades Renales/complicaciones , Enfermedades Renales/cirugía , Masculino , Retención Urinaria/complicacionesRESUMEN
ABSTRACT Introduction: Urethral duplication is rare. Characterized by the presence of two urethral channels. This anomaly presents a great variety of clinical findings that depend on the type of duplication that often is associated with other anomalies. Material and Methods: We report thirteen boys with urethral duplication managed in our institution between 1988-2015. Clinical findings, associated anomalies, treatment of urethral duplication and our results are described. Patients were classified according to Effmann classification. Results: Mean patient's age was 38.3±34.7 months (3-136 months). Mean follow-up was 7.7±3.4 years (3y8m-14y2m). Type II A2 was the most common pattern (8/13 patients, 61.5%), followed by type IA (3/13 patients, 23%) and IIA1 (2/13 patients, 15.3%). The most frequent clinical manifestations were urinary tract infections (UTI) observed in 11/13 patients (84.6%) and anal urinary leakage, found in 7/13 patients (53.8%). Associated anomalies were found in 9/13 patients (69.2%). Required surgeries were 3.53±2.84 procedures per patient. Considering groups: Type IIA2 4.25±3.28, type IIA1 4±1.41 and type IA 1.33±0.57 needed procedures per patient. Complications rate were 0% for type IA, 50% for type IIA1 and 75% for type IIA2. Conclusions: Patients with incomplete duplication (type I A or I B) can totally be asymptomatic, with no need of surgical correction. Type IIA2 is the most complex form of duplication to correct and multiple procedures might be required because of the very hypoplastic orthotopic dorsal urethral tissue. Surgical treatment should be individualized and parents should be advised on complications and need of multiple surgeries according to urethral duplication type.
Asunto(s)
Humanos , Masculino , Femenino , Lactante , Preescolar , Niño , Adolescente , Uretra/anomalías , Uretra/cirugía , Reoperación , Procedimientos Quirúrgicos Urológicos , Estudios Retrospectivos , Estudios de SeguimientoRESUMEN
INTRODUCTION: Severe genital abnormalities such as urogenital sinus and urethral duplication with ectopic urethra in the rectum represent a major challenge in reconstructive urology. OBJECTIVE: We aimed to review our cases presenting with functional ectopic urethra implanted in the rectum that were treated through an ASTRA approach. METHODS: We reviewed the medical records of all patients who had undergone an ASTRA approach from 2005-2016. We collected data with interest to primary diagnosis, clinical presentation, additional procedure, immediate clinical outcome, complications, bowel habits after surgery, voiding and bladder emptying pattern and long term follow-up. RESULTS: Since 2005, we treated 6 cases using this method, consisting of 3 patients with congenital aphallia and 3 with Y-type urethral duplication. Two aphallia patients underwent De Castro's neophalloplasty with simultaneous anastomosis of proximal urethra to a tubed buccal mucosa neourethra and one had a neophalloplasty with transverse skin flaps and primary perineal urethrostomy. Two patients with Y-type urethral duplication had a complete urethroplasty performed (one end-to-end anastomosis and a two-stage repair). Last patient had a definitive perineal urethrostomy. At mean follow-up of 83.5 meses, only one patient voids through the urethra, four have a Mitrofanoff channel and two have a perineal urethrostomy. Immediate follow-up was uneventful and none of our patients had any bowel complications nor fecal incontinence. DISCUSSION: Domini et al. were the first proponents of the anterior sagittal transanorectal approach (ASTRA) as an alternative to classic Peña approach. Later, De Castro popularized specifically this technique as a relevant part of his neophalloplasty procedure to treat congenital aphallia. We did not find in this series any complications related to ASTRA technique in regards to bowel habits, fecal incontinence or infection but most of cases we tried to create a new anterior urethra and connect to the proximal stump failed. Moreover, we are skeptic to support simultaneous urethral repair when there is a dysplastic bulbar segment between the proximal stump and the end of the penile urethra normally at penoscrotal junction area by urethral duplication. Treatment at two-stages is also prone to fail in the midterm. At end, most of them will have either a Mitrofanoff or perineal urethral stoma as shown in our data. CONCLUSION: The ASTRA approach proved to be useful for cases of rectal implantation of the urethra. No complications related to anterior sphincter incision were documented. We believe it should be regarded as the treatment of choice for cases alike ours.
Asunto(s)
Recto/anomalías , Recto/cirugía , Uretra/anomalías , Uretra/cirugía , Preescolar , Humanos , Lactante , Masculino , Estudios Retrospectivos , Procedimientos Quirúrgicos Urológicos/métodosRESUMEN
A imperfuração uretral associada ou não à persistência do úraco é rara; quando concomitante, o animal mantém o fluxo urinário por via umbilical, entretanto, após o tratamento de correção da persistência do úraco, ocorre o armazenamento de urina, que pode culminar em complicações como bexigoma, hidroureter e ruptura vesical. Uma bezerra Nelore, com 20 dias de idade, foi atendida com persistência de úraco. Prescreveu-se a aplicação local de tintura de iodo a 10% durante cinco dias, e indicou-se retorno para obliteração cirúrgica caso não houvesse resposta à terapia proposta. Após 30 dias, o animal retornou com distensão abdominal, histórico de diminuição gradual do fluxo urinário, com ausência de micção via vaginal e discreto gotejamento de urina através do umbigo. Após diversas tentativas de cateterismo uretral sem sucesso, diagnosticou-se a imperfuração do óstio uretral externo. O exame ultrassonográfico revelou distensão vesical com aproximadamente sete litros de conteúdo, hidroureter e hidronefrose bilateral. Realizou-se a cistocentese e o esvaziamento vesical guiado por ultrassom e optou-se pela abordagem cirúrgica para criação do óstio uretral e correção do úraco persistente. Por meio de cistotomia, realizou-se a sondagem retrógrada da uretra e a perfuração da membrana que recobria o óstio uretral no vestíbulo vaginal, a fim de criar um novo óstio. A sondagem foi mantida por 10 dias, com o intuito de evitar estenose do óstio e, após 30 dias de pós-operatório, o animal recebeu alta com óstio uretral patente no vestíbulo vaginal.(AU)
Urethral imperforation associated or not with urachal patency is rare, when concomitant, the animal maintains urinary flow through umbilical via, but when the treatment is performed storage of urine occurs and can culminate in complications like bexigoma, hydrourether, and vesical rupture. A nelore calf with 20 days of age, was attended with urachal patency, conservative treatment with searing substance (10% iodine) was prescribed. Return for surgical treatment was recommended if no resolution was achieved. After 30 days, the animal returned with abdominal distension and history of gradual decrease in urinary flow without urination via urethra. Urethral catheterization was attempted, unsuccessfully, and sonographic evaluation revealed vesical distention with approximately seven liters of content, hydrourether and hydronephrosis. cystocentesis and vesical deflation was performed. Surgical approach of the bladder was performed and a new ostium for the urethra in the vaginal vestibule was created by retrograde via. Urachal was removed and urethral catheterization was maintained for 10 days. Following 30 days of pos operative procedure, the animal was discharged.(AU)
Asunto(s)
Animales , Femenino , Bovinos , Anomalías Congénitas/veterinaria , Uraco/anomalías , Uretra/anomalías , Ultrasonografía/veterinariaRESUMEN
A imperfuração uretral associada ou não à persistência do úraco é rara; quando concomitante, o animal mantém o fluxo urinário por via umbilical, entretanto, após o tratamento de correção da persistência do úraco, ocorre o armazenamento de urina, que pode culminar em complicações como bexigoma, hidroureter e ruptura vesical. Uma bezerra Nelore, com 20 dias de idade, foi atendida com persistência de úraco. Prescreveu-se a aplicação local de tintura de iodo a 10% durante cinco dias, e indicou-se retorno para obliteração cirúrgica caso não houvesse resposta à terapia proposta. Após 30 dias, o animal retornou com distensão abdominal, histórico de diminuição gradual do fluxo urinário, com ausência de micção via vaginal e discreto gotejamento de urina através do umbigo. Após diversas tentativas de cateterismo uretral sem sucesso, diagnosticou-se a imperfuração do óstio uretral externo. O exame ultrassonográfico revelou distensão vesical com aproximadamente sete litros de conteúdo, hidroureter e hidronefrose bilateral. Realizou-se a cistocentese e o esvaziamento vesical guiado por ultrassom e optou-se pela abordagem cirúrgica para criação do óstio uretral e correção do úraco persistente. Por meio de cistotomia, realizou-se a sondagem retrógrada da uretra e a perfuração da membrana que recobria o óstio uretral no vestíbulo vaginal, a fim de criar um novo óstio. A sondagem foi mantida por 10 dias, com o intuito de evitar estenose do óstio e, após 30 dias de pós-operatório, o animal recebeu alta com óstio uretral patente no vestíbulo vaginal.(AU)
Urethral imperforation associated or not with urachal patency is rare, when concomitant, the animal maintains urinary flow through umbilical via, but when the treatment is performed storage of urine occurs and can culminate in complications like bexigoma, hydrourether, and vesical rupture. A nelore calf with 20 days of age, was attended with urachal patency, conservative treatment with searing substance (10% iodine) was prescribed. Return for surgical treatment was recommended if no resolution was achieved. After 30 days, the animal returned with abdominal distension and history of gradual decrease in urinary flow without urination via urethra. Urethral catheterization was attempted, unsuccessfully, and sonographic evaluation revealed vesical distention with approximately seven liters of content, hydrourether and hydronephrosis. cystocentesis and vesical deflation was performed. Surgical approach of the bladder was performed and a new ostium for the urethra in the vaginal vestibule was created by retrograde via. Urachal was removed and urethral catheterization was maintained for 10 days. Following 30 days of pos operative procedure, the animal was discharged.(AU)