An unusual presentation of urethral duplication presenting with chronic bladder retention, left scrotal transposition and left renal agenesis

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.

Hypospadias why complications! When redo surgery! The impact of relevant factors

Great advances have been made in surgery for the correction of hypospadias. Proximal hypospadias remains the greatest challenge, but despite many innovations and much progress, surgery can fail. The new generation of pediatric surgeon has high ambitions in managing patients with hypospadias. More than 200 reconstructive procedures have been described for hypospadias repair. Despite large number of operative techniques for hypospadias repair, the complication rate is very high. To evaluate the factors that may influence the results of surgery after hypospadias repair at The Maternity and Child Teaching Hospital, pediatric surgery unit, Al-Qadisiya/ Iraq. It was a retrospective observational study. Files of all patients who had Hypospadias repair were retrieved and analyzed with a view to identify the factors which may influence the results of surgery for Hypospadias. Patients with complete record available were included in the study, whereas those with incomplete data were excluded. Forty cases [out of 115 cases of hypospadias] of fistula, stricture, retrusive meatus, meatal stenosis, torsion, open distal urethra and urethral diverticula with combination of two or more, were managed by from the 1[st] of January 2005 to the end of December 2009. There were 40 patients, aged 1-12 [mean 3.8] years, with failed hypospadias repairs. All operated on by the same surgeon, with a minimum of 12 months of follow-up. Twenty-seven of 40 patients had 1 redo operation, 9 had 2 redo operations, 3 had 3 redo operations and 1 had 4 redo operations, for a total of 58 redo operations. Of these, 34 were TIP techniques [58.6%], 8 were Mathieu [13.7%], 8 were TIP repairs [13.7%], 4 were onlay island flaps [6.8%] and 4 were buccal mucosal grafts [6.8%]. Follow-up was 1-3 years [mean 1.5 yr]. Complications after redo surgery comprised 4 urethrocutaneous fistulae, 2 meatal stenoses, 1 urethral stricture and 3 dehiscences]. Surgical techniques for the repair of hypospadias are being developed continuously, implying that no single procedure is considered a panacea for hypospadias repair. Admittedly, it is difficult to follow patients for a long time, and it is even harder to predict who will have complications that will merit closer follow-up. Success in hypospadias surgery depends on good team work. All types of repair involve straightening the penis by removal of chordee. The pediatric surgeon selects the appropriate operative procedure and carries it out with meticulous techniques

Achados audiológicos e eletrofisiológicos de indivíduos com a síndrome G/BBB / Auditory findings and electrophysiologics in individuals with G/BBB syndrome

A síndrome G/BBB é uma condição rara, caracterizada por hipertelorismo, fissura de lábio e palato e hipospádia. Não foram encontrados trabalhos sobre a audição em indivíduos com esta síndrome. OBJETIVO: Investigar a função auditiva em pacientes com síndrome G/BBB quanto à ocorrência ou não de perda auditiva e a condução nervosa auditiva periférica e central. MATERIAL E MÉTODO: Catorze pacientes de 7 a 34 anos, do gênero masculino, com a síndrome G/BBB, foram avaliados por meio de otoscopia, audiometria, timpanometria e potenciais evocados auditivos de tronco encefálico (PEATE). Forma de Estudo: Estudo de série clínico prospectivo. RESULTADOS: Limiares audiométricos normais em 12 (66,7 por cento) pacientes da amostra e alterados em dois (33,3 por cento), sendo um com perda condutiva e um neurossensorial. Quanto ao PEATE, foram encontrados: latências absolutas da onda I normais em todos os pacientes, aumento das latências absolutas da onda III e V em dois e seis pacientes respectivamente; latências interpicos I-III, III-V e I-V aumentadas em quatro, três e oito pacientes, respectivamente. CONCLUSÃO: Perdas auditivas periféricas podem ocorrer na síndrome G/BBB. Há evidências de comprometimento das vias auditivas centrais em nível do tronco encefálico. Estudos com exames de imagem são necessários para maior clareza dos achados clínicos.

The G/BBB syndrome is a rare condition characterized by hypertelorism, cleft lip and palate, and hypospadias. No studies were found on the hearing of individuals with this syndrome. AIM: To investigate the auditory function in patients with G/BBB syndrome, such as the occurrence of hearing loss, and central and peripheral auditory nerve conduction. METHODS: Fourteen male patients aged 7-34 years with the G/BBB syndrome were assessed by otoscopy, audiometry, tympanometry and evoked auditory brainstem response (ABR). Model: A retrospective clinical series study. RESULTS: Audiometric thresholds were normal in 12 (66.7 percent) of the sample and altered in two (33.3 percent), one with conductive and one with sensorineural loss. ABR resuts were: all patients had normal absolute wave I latencies; absolute wave III and V latencies were increased in two and six patients, respectively; interpeak latencies I-III, IV and V interpeak latencies were increased in four, three and eight patients respectively. CONCLUSION: Hearing loss can occur in the G/BBB syndrome. There is evidence of central auditory pathway involvement in the brainstem. Imaging studies are needed to clarify the clinical findings.

Meatobalanoplastia asociada a movilización de uretra para corregir hipospadias distales / Meatobalanoplastia associated with mobilization of the urethra to correct distal hypospadias

Objetivo: Demostrar que la meatobalanoplastia asociada a movilización, por liberación parcial o total de la uretra (MEBAMU) es una conducta técnico quirúrgica funcional y estéticamente útil para corregir hipospadias distales.Material y Métodos: 63 de 81 niños de entre 8 y158 m. de edad (promedio 37,4 m. mediana 25 m.) operados por el mismo cirujano entre los años 2000 y 2007 cumplieron con los criterios de inclusión. Es un estudio clínico prospectivo de tipo experimental, con controles históricos. Los detalles técnicos son descriptos minuciosamente. 15 (23,8%) eran hipospadias balánicas, 26 (39,7%) coronales y 22 (36,5%) subcoronales. 9 pacientes habían sido operados previamente, 8 con otras técnicas y 1 con la misma técnica. De acuerdo con las necesidades, en 18 pacientes la uretra se iberó parcialmente y en 45 totalmente (13 en un corto trayecto y 32 extensamente) . El seguimiento promedio fue de 33 m (6 a 99m). A 30 niños de 2 a 14 años de edad (Promedio 75,2 m) se les realizó uroflujometría, 36,6 m promedio después de operados. El análisis estadístico se realizó con software de SAS que considera un error de tipo I del 5% (alfa=0.05%). La comparación entre los grupos, cuando las variables fueron numéricas se realizó mediante el test de Wilcoxon Sun Run. Las variables nominales (cualitativas) mediante test de Chi cuadrado o Fisher Exact test. Resultados: Preponderó la liberación parcial de la uretra en las formas balánicas y la total en las coronales y subcoronales (p<0,01). 5 pacientes (7,9%) se complicaron, 2 fístulas y 3 estenosis. De los 9 pacientes con cirugías previas, 7 evolucionaron satisfactoriamente.

Fistula repair after hypospadias surgery using buccal mucosal graft

The aim of this study was to evaluate the success rate of urethrocutaneous fistula repair using buccal mucosal graft in patients with a previous hyposadias repair. We reviewed records of our patients with urethrocutaneous fistula developed after hypspadias repair in whom buccal mucosal graft fistula repair had been performed. All of the patients had been followed up for 24 postoperative months. A successful surgical operation was defined as no fistula recurrence or urethral stricture. Retrograde urethrography and urethrocystoscopy would be performed in patients who had any history of decreased force and caliber of urine or any difficulty in urination. Fistula repair using buccal mucosa patch graft had been done in 14 children with urethrocutaneous fistula developing after hypospadias reconstruction. The mean age of the children was 8.70 +/- 1.00 years old [range, 4 to 11 years]. Seven fistulas were in the midshaft, 4 were in the penoscortal region, and 3 were in the coronal region. Repair of the fistulas was successful in 11 of 14 patients [78.6%]. In the remaining children, the diameter of the fistula was smaller than that before the operation, offering a good opportunity for subsequent closure. Our findings showed that fistula repair using buccal mucosal graft can be on the acceptable techniques for repairing fistulas developed after hypopadias repair

Tratamento de hipospádia distal pela técnica de snodgrass / Treatment of distal hypospadias for snodgrass technique

Introdução: hipospádia é uma malformação congênita que se caracteriza pela localização ectópica do meato uretral e a anomalia genital mais comum no sexo masculino. Dentre as formas de apresentação a distalrepresenta 70% dos casos. O tratamento é estritamente cirúrgico, associado às complicações significativas que o tornam um desafio. Objetivo: avaliar o resultado cirúrgico no tratamento da hipospádia distal no serviço de cirurgia pediátrica do HIJG.Métodos: estudo prospectivo, observacional, com seguimento pós-operatório de 90 dias. Resultados: no período de março de 2006 a maiode 2007, 50 pacientes portadores de hipospádia distal foram submetidos à uretroplastia pela técnica de Snodgrassno HIJG. A média de idade foi de 4,08 anos (6m - 14anos). O tempo de retirada da sonda uretral no pós-operatório foi em média de 11,28 (7 - 20) dias e a moda de 7 dias. As intercorrências no pós-operatório ficaram assim distribuídas: sangramento de meato uretral (60%), dor na ferida operatória (40%). As complicações ocorridas foram: estenose de meato uretral (6%) e fístula uretrocutânea (10%). O aspecto cirúrgico foi considerado satisfatório em 48 dos pacientes (96%). Conclusão: A taxa complicação foi de 16% e a aparênciado trato genital externo foi considerada normal na grande maioria dos casos.

Background: hypospadia is a congenital anomaly characterized by ectopic location of the urethral meatus. It’s the most common anomaly of male genitalia. Among its forms of presentation, the distal form responds for 70% of the total. The treatment of this anomaly is strictly surgical, associated with significant complications which may interfere in the results. Objective: evaluate the surgical result of the treatment of distal hypospadia in the Joana de Gusmão Children’s Hospital, Florianópolis, Brazil. Method: prospective study, observational, with postsurgical follow-up of 90 days.Results: in the period of March 2006 and May 2007, 50 patients with distal hypospadia where submitted toSnodgrass uretroplasty. The average age was 4,08 years (6 months to 14 years). The removal of the urethralcatheter in the post-surgical period occurred in mean time of 11,28 (7 - 20) days and its mode was 7 days. Thepost-operative problems where: bleeding from urethral meatus (60%), pain in the operatory wound (40%). Thecomplications where: urethral meatus stenosis (6%) and urethra-cutaneous fistula (10%). The surgical aspect wassatisfactory in 48 patients (96%).Conclusions: the complication rate was 16% and the appearance of the external genital tract was satisfactory in the majority of the patients.

Evaluación del comportamiento y evolución clínica de los pacientes con hipospadias manejados quirúrgicamente en el Hospital Manuel de Jesús Rivera “La Mascota”, Managua, durante le 2006-2007 / Performance assessment and clinical course of patients with hypospadias managed surgically at the Hospital Manuel de Jesus Rivera

El objetivo de este estudio fue evaluar el comportamiento y evolución clínica de los pacientes con hipospadias manejados quirúrgicamente en el Hospital Manuel de Jesús Rivera “la Mascota”, Managua, durante el 2006-2007. El tipo de estudio fue descriptivo de serie de casos, en el Servicio de Urología del Hospital Manuel de Jesús Rivera “La Mascota”, Managua. Se consideró como caso a todos aquellos pacientes ingresados e intervenidos quirúrgicamente en el Servicio de Urología con diagnóstico de hipospadias, correspondiente a los códigos Q54.0 y Q54.9 de la Clasificación Internacional de Enfermedades, décima revisión (CIE-10).24 0). La fuente de información fue primaria, basada en los expedientes clínicos de los pacientes. El software utilizado fue el SPSS versión 10.0. La mayoría de pacientes operados tenían más de 5 años de edad, contrario a lo recomendado por la Sección de Urología de la Academia Americana de Pediatría. Las principales hipospadias fueron anteriores, media y posterior, respectivamente. Las principales cirugías fueron Bracka y Snodgrass. La incidencia de complicaciones fue de 51.4%, predominando las fístulas, dehiscencias, estenosis del meato e infección de la herida quirúrgica. Las cirugías con mayor índice de complicaciones fueron Byars, Snodgrass, y técnicas sin especificar. El tiempo quirúrgico fue de 140.7 minuto y la estancia hospitalaria de 7.9 días En el 11.4% de caso se registraron anomalías congénitas, predominando la criptorquidia. Se recomienda realizar estudios para determinar las razones por las cuales estos pacientes buscan atención quirúrgica tardíamente; y estudios de evaluación de la atención en salud. Siempre y cuando se mejore el registro en los expedientes clínicos. Además, promover en la atención primaria la detección temprana para referirlos oportunamente a consulta especializada...

Short hospital stay versus day-care Mathieu hypospadias repair

Objective: This study compared the outcome of Mathieu repair between patients who went home within 24 hours on catheter and dressing and patients who were managed in hospital for 48 hours and had their catheters and dressings removed before going home. Patients and Methods: A retrospective study of Mathieu hypospadias repair performed by the same surgeon for 11 years. Outcome measures were catheter and dressing related problems/complications. Results: Sixty five patients were included in the study; 43(66.2) were managed in-hospital for the first 48 hours (Group A); while 22(33.8) were managed as day-care cases (Group B). Complication rate was 6(14.0) and 3(13.6) respectively; with fistula rate of 2(4.7) in Group A and 1(4.5) in Group B. Conclusion: Day care Mathieu repair of hypospadias does not increase the occurrence of complications

role of vascularized dorsal subcutaneous flap for decreasing fistula formation following hypospadias repair: a preliminary experience

To evaluate the value of covering the neourethra using vascularized dorsal subcutaneous flap in order to decrease urethrocutaneous fistula in hypospadias repair. This is a retrospective study of 130 children [aged 1-14 years] who had different types of hypospadias and underwent different types of repair between August 2004 and December 2006 at King Hussein Medical Center. The study sample includes 66 children with distal penile hypospadias, 28 children with midshaft, 8 children with proximal hypospadias, of which 3 underwent first stage repair, 20 children with urethrocutaneous fistula, and 8 with complete failure of previous repair. Longitudinal vascularized dorsal subcutaneous flap was harvested from the excessive dorsal preputal and penile hypospadiac skin, and then used in all cases as a covering for different types of urethral repair in double breastign Byar's flap fashion which sutured to the glans wings around the neomeatus and to the corpora adjacent to the neourethra using 7/0 and 6/0 polyglactin sutures, resulting in complete covering of the neourethra with well-vascularized dorsal subcutaneous flap. Tubularized Incised Plate [TIP] repair was used in the majority of case, known as Snodgrass repair, while Mathieu repair and urethrocutaneous fistula repair were done in the rest of cases. The chordee was corrected when present, and the glans closure was finalized in 2 layers. Most cases performed were over urethral stent, and in few complicated cases sialastic foley catheter and suprapubic cystocath for urinary diversion were used. In monitoring results during the follow-up period, which extended over 18 months with a median of 6 months, the operations were successful. Three children had urethrocutaneous fistula, of which 2 had previous repair, and one had complete failure. Eight children had metal stenosis which responded to dialatation in 6 children and meatoplasty in 2 children. We suggest that in hypospadias surgery, covering of the neourethra with well-vascularized dorsal or adjacent subcutaneous flap should be part of the procedure. It decreases urethrocutaneous fistula formation especially if the careful harvesting technique is utilized

Tubularized incised plate (TIP) urethroplasty : extended use in hypospadias re-operation

Objective: Recently; tubularized incised plate (TIP) urethroplasty (Snodgrass repair) has gained popularity for the primary repair of distal and proximal hypospadias. This study was carried out to evaluate TIP urethroplasty in the repair of failed and complicated hypospadias cases. Patients and Methods: This study was carried out in the pediatric surgery unit; Departments of General Surgery and Urology; Zagazig University Hospital. Twenty-four patients with failed and complicated hypospadias with an intact urethral plate were included in this work. Thirteen patients (54) were younger than 3 years; and 3 patients (13) older than 6 years. The original hypospadias was coronal in 4; midshaft in 11 and penoscrotal in 9 cases. Wide fistula was present in 8 cases (32) and dehiscence was found in 6 cases (26). Previous procedures included meatal advancement and glanuloplasty incorporated (MAGPI) in 5 (21) cases; Mathieu procedure in 4 (17); and preputial flaps in 13 (55) cases. Two patients (8) had been subjected to previous TIP urethroplasty. Twenty cases had had a single preceding procedure; while 4 cases had been subjected to two previous trials of correction. The time that had elapsed before re-operation was less than 6 months in 14 cases (58). Results: Fistula occurred in 3 cases; two of them had had preoperative fistula. Meatal and anastomotic strictures occurred in 2 cases each and were completely managed by dilatation for 3-6 months. Wound infection occurred in 5 cases. Good cosmetic and functional results were achieved in 15 cases (63). TIP urethroplasty was found to be suitable for re-operating previously failed hypospadias cases because it reconstructs the urethra entirely from the urethral plate which is the least affected part after all types of repair. Also; no relation was found between the morphology of the urethral plate and the success of TIP repair. Most of the cases had an intact urethral plate which had not been incised in the primary procedure. Further studies are needed to assess the possibility of using a urethral plate which was previously incised in a primary procedure. Conclusion: TIP urethroplasty could be a reasonable procedure in failed and complicated hypospadias cases. It provides good cosmetic and functional results and can be done using minimal residual tissues remaining after primary procedures

Preputial glanuloplasty for an apical meatus in second-stage hypospadias repair

This work aimed to study the possibility of achieving a truly apical meatus by minor technical modification of the second-stage Denis-Browne hypospadias repair. It included 76 hypospadias aged 7 months to 25 years, with an average age of 6.3 years, who already had a penile curvature correction procedure done. They were then operated upon by a preputial glanuloplasty, the author's modification of the second-stage Denis-Browne hypospadias repair, mobilizing and suturing the lateral skin flaps to the very tip, of the glans. A truly apical meatus was achieved in 43 patients [56.6%] and overall fair to excellent results were obtained in 95% of cases. Failure, necessitating re-do surgery for the distal part of the neo-urethra, occurred in only 4 patients. Preputial glanuloplasty adds only a few minutes to the total operating time. It does not cause additional complications and achieves satisfactory anatomical and functional results. It is to be recommended when a Denis-Browne procedure is to be performed

Complicaciones de las plastías de hipospadias en 54 pacientes / Complicationes of hypospadias plasties in 54 patients

El hipospadias es un defecto congénito del pene que resulta de desarrollo incompleto de la uretra. La incidencia es de 1 en 300 varones nacidos vivos. El tratamiento ha evolucionado en forma importante en el último decenio decenio. En este artículo se informa la experiencia de los autores con 54 pacientes sometidos a 121 procedimientos para corregir esta malformación dentro de un periodo de tres años. Fueron elevados los índices de fistulización (61 por ciento) y estenosis (9 por ciento), en comparación con los datos informados en la literatura internacional. Se específican ampliamente los resultados de técnicas, materiales de sutura y drenajes utilizados en la plastias, y se concluye que la alta tasa de complicaciones es multifactorial y no se debe a ninguna variable específica.

Hipospadias femenina con incontinencia urinaria: uretroplastía distal transvaginal como tratamento quirúrgico / Female hypospadia with urinary incontinence: distal transvaginal uretroplasty as surgical treatment

El hipospadias masculino es una entidad no poco frecuente, las manifestaciones clínicas, el diagnóstico y el tratamiento del hipospadias en el varón son familiares a la mayoría de los urólogos. Sin embargo, en la mujer esta entidad es poco frecuente y diagnosticada, solamente, cuando presenta complicaciones o fortuítamente en el examen físico. Se presenta un caso tratado exitosamente mediante uretroplastía distal transvaginal con colgajo himeneal, se revisa la técnica quirúrgica y se hace una discusión sobre el hipospadias femenino, su etiología, diagnóstico, tratamento y manejo

Correçäo de fístula uretrocutânea através de interposiçäo de retalho desepitelizado de pele / Correction of urethrocutaneous fistula through the interposition of de-epithelized skin flap

Quinze crianças portadoras de fístula uretrocutânea pós-neouretroplastia foram submetidas a correçäo cirúrgica, através de interposiçäo de retalho de pele desepitelizado. Obteve-se cura em 15 de 17 fístulas operadas (88%); somente em 7 crianças utilizou-se derivaçäo urinária