Urethral duplication II-A Y type with rectal urethra: ASTRA approach and tunica vaginalis flap for first stage repair

INTRODUCTION: Urethral duplication is a rare congenital anomaly affecting mainly boys. Generally, the duplication develops on the sagittal plane; the accessory urethra may run dorsally or ventrally to the orthotopic one. We present a patient with urethral duplication in which the orthotopic urethra was patent in the penile segment but atresic in the bulbar and prostatic segment. The patient had urinary flow from the rectum and the ectopic urethra could be well identified by anal examination. MATERIALS AND METHODS: Age at surgery was 13 months. The procedure consisted of an ASTRA (anterior sagittal trans-ano-rectal) approach for dividing the urethra and rectum and was successful to move the urethra up to the perineal area. The rectum was reconstructed and the patient placed into a lithotomy position. A urethral catheter inserted in the penile urethra oriented us were the atresic urethra in bulbar area started. The scrotum was opened in the middle and the distance between the two urethral stumps proximal and distal defined the extension of no urethral tissue that consisted of 5 cm. We opened the right scrotal space and a tunica vaginalis flap was obtained and attached to the bulbar tissue for a two-stage urethroplasty strategy. RESULTS: Patient had a nice healing and the tunica vaginalis was nicely incorporated to the adjacent tissue, having the two urethral stumps well delineated. CONCLUSIONS: ASTRA approach in combination with a two-stage urethroplasty with tunica vaginalis dorsal flap proved to be an excellent combination for a rare case of urethral Y duplication having the main urethra into the rectum.Universidade Federal de São Paulo (UNIFESP) Department of UrologyUNIFESP, Department of UrologySciEL

Cloacal Exstrophy: a complex disease

Introduction Cloacal exstrophy is a rare occurrence with an incidence of 1:200,000 to 1:400,000 live births. It represents one of the most challenging reconstructive endeavors faced by pediatric surgeons and urologists. Aside from the genitourinary defects, there are other associated anomalies of the gastrointestinal, musculoskeletal and neurological systems that require a multidisciplinary approach when counseling anxious parents. Material and Methods We present a video of a patient with cloacal exstrophy treated with 21 days of life. Surgery consisted of separation and tubularization of the cecal plate from the exstrophied bladder halves and colostomy construction. The bladder was closed primarily and umbilical scar reconstructed and used for ureteral and cistostomy drainage. A urethral catheter was used to guide bladder neck tubularization. A final epispadic penis was obtained and planned for further repair in a second step. Results The patient had an initial uneventful postoperative course and immediate outcome was excellent. The bladder healed nicely but patient presented with abdominal distension in the 5th day of postoperative setting requiring parenteral nutrition. The distal colon persisted with lower diameter although non obstructive, but causing difficulty for fecal progression. Continuous colostomy dilatation and irrigation were required. Conclusions Approximating the bladder halves in the midline at birth and primary bladder closure is a viable option, intestinal transit may be a issue of concern in the early postoperative follow-up.Universidade Federal de São Paulo (UNIFESP) Division of UrologyUNIFESP, Division of UrologySciEL

Elective appendicovesicostomy in association with monfort abdominoplasty in the treatment of prune belly syndrome

OBJECTIVE: To evaluate the role of elective appendicovesicostomy in association with Monfort abdominoplasty to avoid urinary tract infection (UTI) and renal damage in the post-operative follow-up of patients with prune belly syndrome. MATERIALS AND METHODS: We followed 4 patients operated in our institution (UNIFESP) (Monfort, orchidopexy and Mitrofanoff) and compared them to 2 patients treated similarly, but without an appendicovesicostomy, in a second institution (UFBA). We evaluated postoperative clinical complications, UTI and preservation of renal parenchyma. Patients were followed as outpatients with urinalysis, ultrasonography (US) and occasionally with renal scintigraphy. RESULTS: Mean follow-up was 23.5 months. Immediate post-operative course was uneventful. We observed that only one patient with the Mitrofanoff channel persisted with UTI, while the 2 patients used as controls persisted with recurrent pyelonephritis (> 2 UTI year). CONCLUSION: Our data suggest that no morbidity was added by the appendicovesicostomy to immediate postoperative surgical recovery and that this procedure may have a beneficial effect in reducing postoperative UTI events and their consequences by reducing the postvoid residuals in the early abdominoplasty follow-up. However, we recognize that the series is small and only a longer follow-up with a larger number of patients will allow us to confirm our suppositions. We could not make any statistically significant assumptions regarding differences in renal preservation due to the same limitations.Federal University of São Paulo Division of UrologyFederal University of Bahia Division of UrologyUNIFESP, Division of UrologySciEL

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

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.Univ Fed Sao Paulo, Sao Paulo, BrazilUniv Fed Sao Paulo, Sao Paulo, BrazilWeb of Scienc

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

Objetivo: Utilizar as medidas antropométricas para comparar o \ud estado nutricional em crianças com bexiga neurogênica secundária \ud à mielomeningocele submetidas a enterocistoplastia àquelas não \ud submetidas à cirurgia. Métodos: Estudo tipo caso-controle conduzido \ud em 20 crianças, divididas em dois grupos, aquelas que realizaram \ud enterocistoplastia (Grupo A) versus crianças não operadas (Grupo B), \ud pareados por gênero e idade. Os parâmetros utilizados foram peso, \ud altura, circunferência do braço e prega cutânea triciptal. A avaliação \ud nutricional foi determinada calculando-se índices baseados na idade \ud e no gênero. A classificação foi baseada em porcentagens, e os \ud resultados foram comparados com valores de referência. Resultados: \ud A média da idade foi 6,41 anos no Grupo A e de 6,35 no Grupo B. O \ud intervalo entre a cirurgia e a avaliação foi de 11 meses. As seguintes \ud medidas foram encontradas para o Grupo A: 80% das crianças eram \ud eutróficas, 30% maior do que no Grupo B; circunferência braquial \ud foi adequada em 40% dos pacientes, 20% maior do que no Grupo \ud B; a área do músculo do braço foi adequada em 90%, 30% maior \ud do que no Grupo B. Os valores no Grupo B foram os seguintes: \ud 60% apresentaram prega cutânea triciptal acima do valor médio, \ud uma porcentagem 20% acima do que no Grupo A; para índice de \ud gordura braquial, 60% dos pacientes estavam acima da média, \ud 40% maior do que no Grupo A. Conclusão: Pacientes submetidos à \ud enterocistoplastia demonstraram melhor estado nutricional enquanto \ud o grupo controle apresentou índices de gordura mais elevados nas \ud medidas antropométricas. Entretanto, a diferença entre os grupos \ud não foi estatisticamente significativaObjective: To use anthropometric measurements to compare nutritional \ud status in children with neurogenic bladder dysfunction secondary \ud to meningomyelocele who underwent enterocystoplasty and those \ud who did not undergo surgery. Methods: A case-control study \ud was conducted in 20 children, divided into two groups: those who \ud had enterocystoplasty (Group A) and those who did not undergo \ud surgery (Group B), matched for genre and age. Weight, height, arm \ud circumference, and triceps skinfold thickness were the parameters \ud used. Nutritional assessment was determined by calculating the \ud indexes, based on age and genre. Classification was based on the \ud percentile and the results were compared with the reference values. \ud Results: The mean age was 6.41 years in Group A and 6.35 years \ud in Group B. The interval between surgery and evaluation was 11 \ud months. The following measures were found for Group A: 80% of \ud children were eutrophic, a percentage 30% greater than that in Group \ud B; arm muscle circumference was adequate in 40% of patients, a \ud percentage 20% greater than that in Group B; arm muscle area was \ud adequate in 90%, a percentage 30% greater than that in Group B. \ud Values in Group B were as follows: for triceps skinfold thickness, 60% \ud of patients had values above the mean, a percentage 20% greater \ud than that in Group A; for arm fat index, 60% of patients were above \ud the mean value, 40% greater than in Group A. Conclusion: Patients \ud who had undergone enterocystoplasty showed better nutritional \ud status, while the control group presented higher fat indexes in \ud anthropometric measures. However, the differences between groups \ud were not statistically significan

One-stage complex primary hypospadia repair combining buccal mucosa graft, preputial flap and tunical vaginalis flap (the three-in-one technique)

Objective: Complex hypospadia repair can be performed according to different strategies, mostly in one or two stages. We present a detailed video of one patient operated according to the three-in-one technique, which combines dorsal buccal mucosa grafting for reconstruction of the incised urethral plate and a preputial flap onlay urethroplasty covered by a tunica vaginalis graft.Method: After sectioning of the urethral plate to correct ventral curvature, the original plate is anchored to the proximal penile shaft. Buccal mucosa is harvested from the lower lip and sutured to the ventral penile shaft area. A transverse preputial flap is obtained and anastomosed 'onlay' to the reconstructed neouretha. the scrotal fascia is opened at the site opposite to the placement of the pedicle of the preputial flap and a careful dissection of the tunica vaginalis and cremasteric tissue is performed. This second flap is used to cover the neourethra and is fixed to the corpora by angular interrupted 6-0 PDS sutures.Results: Our series of 35 patients resulted in 68.5% success after a single operation, whereas 31.5% needed a second repair.Conclusion: the technique gives excellent results and can be considered an alternative to one-stage repair in primary cases. (C) 2010 Journal of Pediatric Urology Company. Published by Elsevier B.V. All rights reserved.Universidade Federal de São Paulo, Dept Urol, São Paulo, BrazilUniversidade Federal de São Paulo, Pediat Urol Sect, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Urol, São Paulo, BrazilUniversidade Federal de São Paulo, Pediat Urol Sect, São Paulo, BrazilWeb of Scienc

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

Objective: To demonstrate ventral corporoplasty, with tunica vaginalis flap to reconstruct the corpora cavernosa, in a two-stage strategy for proximal hypospadias surgery.Methods: Assessment of residual curvature after complete urethral plate division and transverse superficial cuts in albuginea. Ventral incision of tunica albuginea to elongate the ventral surface of the penis and use of a tunica vaginalis flap to reconstruct the defect in corpora cavernosa. Dorsal preputial island flap was used to cover the penile ventral surface, to be tubularized in a second stage, together with the original urethral plate.Results: Patient had an uneventful follow-up. Penile aspect was very satisfactory with no residual curvature.Conclusion: Tunica vaginalis is an attractive alternative for ventral corporoplasty in hypospadia repair and has the advantages of prompt disposability, autologous use, and does not represent an extra cost for treatment. (C) 2013 Journal of Pediatric Urology Company. Published by Elsevier B.V. All rights reserved.Universidade Federal de São Paulo, Div Urol, São Paulo, BrazilUniversidade Federal de São Paulo, Div Urol, São Paulo, BrazilWeb of Scienc