High grade persistent ventral curvature after multiple hypospadias surgery: how to correct?

ABSTRACT Introduction: A challenging situation in proximal hypospadias is the presentation of patients with successful urethroplasty but with persistent or recurrent ventral curvature (VC) after multiple hypospadias repair. Materials and Methods: We present a 13 year-old boy with 7 previous surgeries (long TIP, Duplay, meatoplasty) to treat hypospadias presenting with 60 degrees of VC, in spite of a well-accepted coronally neomeatus. We degloved the penis and artificial erection clearly appointed corporal disproportion causing curvature. We disconnected urethra from corpora. After excision of remnant fibrotic tissue, there was a residual curvature so a lenghtening corporoplasty with dermal graft from groin was performed. We have adjusted the urethral meatus position into a proximal penile shaft. We used a buccal mucosa graft placed in an inverted U-shape position planning a second stage urethroplasty (1). An indwelling silicone Foley tube was left for one week. The patient was discharged the day after surgery. Results: The aspect after corporoplasty proved satisfactory curvature correction. Patient had an excellent outcome and is scheduled for a second-stage after 6 months. Discussion: Snodgrass and Bush (2) reported that on 73 patients with an average of 2.7 operations for proximal shaft to perineal hypospadias; of which, 83% had VC at re-operation averaging 50°. We do believe that some good results with minimal dorsal plicature may recur in adolescence and therefore when these procedures may be considered, they should be performed by classic Nesbit technique (3). Otherwise, the choice for primary ventral lengthening should be taken. Conclusion: Severe curvature associated with hypospadias should undergo a major procedure at early stage to avoid decompensation after dorsal plicature in adolescence. We had a very satisfactory result, the patient awaits the second stage procedure (Figure-1). Figure 1 End aspect after complete healing of buccal mucosa. Notice that penis is straight and second stage will be performed after 6 months of interval.

Conversion of vesicostomy into continent catheterizable reservoirs in myelomeningocele

ABSTRACT Introduction: Vesicostomy should be considered in children with neuropathic bladder in case first-line therapies fail. This simple and reversible procedure can reduce febrile urinary tract infections and protect the upper urinary tract (1) until more definitive alternatives can be proposed. We describe in this video how we approach patients that underwent vesicostomy and want it to be converted into a continent catheterizable reservoir. Material and methods: We perform an infra-umbilical longitudinal incision with a semicircular flap where the stoma will be placed (outside vesicostomy). After releasing the bladder, we proceed with usual steps of the Macedo-Pouch technique (2). We perform the reservoir from 35cm of ileum that constructs a catheterizable channel from the same bowel segment from a 3cm width flap from anterior and posterior wall of ileum in the mid part of it. The continence mechanism of the efferent tube is based on angulation and a serous lined tunnel created with 3-4 3.0 prolene sutures. The stoma is placed in the midline (3). Results: Patient had an uneventful evolution and is continent performing CIC every 4 hours with 9 months of follow-up. We have in the last 3 years a consecutive series of 12 patients operated according to this principle. Discussion: Vesicostomy should be regarded as an alternative for patients with neurogenic bladder refractory to clinical treatment at a younger age in order to postpone definitive treatment such as any an enterocystoplasty. This option must be considered as transient, since definitive reconstructive surgery can provide preservation of renal function and continence achievement. In this context, our video demonstrates that performing a bladder augmentation on a patient with a vesicostomy is safe and feasible. We reinforce that our method precludes the need of appendix or creation of a Monti tube as the outlet channel and the whole procedure is performed from a single piece of bowel.

The GUD technique: glandar urethral disassembly. An alternative for distal hypospadias repair

ABSTRACT Introduction We present an alternative procedure for distal hypospadias consisting of urethral mobilization and partial glandar disassembly, namely GUD (glandar urethral disassembly) technique. Materials and Methods A subcoronal circumcision exposes distal dysplastic urethra. We incise the Buck´s fascia on both sides of urethra releasing it partially from the corpora. We keep a thin bridge of urethral plate to the glans and disassembly almost completely the glans from the corpora, except for the bridge. The glans is incised creating two wide wings that are extremely mobile. The urethra is mobilized, advanced and sutured to the tip of the glans. The glans wings embrace the distal urethra producing a conical glans. Discussion The concept of urethral mobilization has been reported and popularized by Koff in the literature to correct distal hypospadias. One of the limitations of this procedure is the risk of urethral retraction due to extensive proximal dissection. We got inspiration from Mitchell and Bagli' s work of penile disassembly in epispadias to develop the GUD concept. We adopt minimal urethral mobilization mainly in glandar/proximal penile shaft and complete deconstruction of the glans, detaching the corpora from the glans and rotating the wide glans wings to embrace the urethra. Therefore we avoid suture urethroplasty and refurbish the glans to a better conical shape. Conclusion We are convinced that this operation can be regarded as a genuine alternative to distal hypospadias (coronal and subcoronal) but should not be addressed to midshaft forms.

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.

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. .

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

OBJECTIVE: To use anthropometric measurements to compare nutritional status in children with neurogenic bladder dysfunction secondary to meningomyelocele who underwent enterocystoplasty and those who did not undergo surgery. METHODS: A case-control study was conducted in 20 children, divided into two groups: those who had enterocystoplasty (Group A) and those who did not undergo surgery (Group B), matched for genre and age. Weight, height, arm circumference, and triceps skinfold thickness were the parameters used. Nutritional assessment was determined by calculating the indexes, based on age and genre. Classification was based on the percentile and the results were compared with the reference values. RESULTS: The mean age was 6.41 years in Group A and 6.35 years in Group B. The interval between surgery and evaluation was 11 months. The following measures were found for Group A: 80% of children were eutrophic, a percentage 30% greater than that in Group B; arm muscle circumference was adequate in 40% of patients, a percentage 20% greater than that in Group B; arm muscle area was adequate in 90%, a percentage 30% greater than that in Group B. Values in Group B were as follows: for triceps skinfold thickness, 60% of patients had values above the mean, a percentage 20% greater than that in Group A; for arm fat index, 60% of patients were above the mean value, 40% greater than in Group A. CONCLUSION: Patients who had undergone enterocystoplasty showed better nutritional status, while the control group presented higher fat indexes in anthropometric measures. However, the differences between groups were not statistically significant.

OBJETIVO: Utilizar as medidas antropométricas para comparar o estado nutricional em crianças com bexiga neurogênica secundária à mielomeningocele submetidas a enterocistoplastia àquelas não submetidas à cirurgia. MÉTODOS: Estudo tipo caso-controle conduzido em 20 crianças, divididas em dois grupos, aquelas que realizaram enterocistoplastia (Grupo A) versus crianças não operadas (Grupo B), pareados por gênero e idade. Os parâmetros utilizados foram peso, altura, circunferência do braço e prega cutânea triciptal. A avaliação nutricional foi determinada calculando-se índices baseados na idade e no gênero. A classificação foi baseada em porcentagens, e os resultados foram comparados com valores de referência. RESULTADOS: A média da idade foi 6,41 anos no Grupo A e de 6,35 no Grupo B. O intervalo entre a cirurgia e a avaliação foi de 11 meses. As seguintes medidas foram encontradas para o Grupo A: 80% das crianças eram eutróficas, 30% maior do que no Grupo B; circunferência braquial foi adequada em 40% dos pacientes, 20% maior do que no Grupo B; a área do músculo do braço foi adequada em 90%, 30% maior do que no Grupo B. Os valores no Grupo B foram os seguintes: 60% apresentaram prega cutânea triciptal acima do valor médio, uma porcentagem 20% acima do que no Grupo A; para índice de gordura braquial, 60% dos pacientes estavam acima da média, 40% maior do que no Grupo A. CONCLUSÃO: Pacientes submetidos à enterocistoplastia demonstraram melhor estado nutricional enquanto o grupo controle apresentou índices de gordura mais elevados nas medidas antropométricas. Entretanto, a diferença entre os grupos não foi estatisticamente significativa.

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.

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.

Hipertensäo arterial renovascular na estenose da junçäo pieloureteral / Renovascular arterial hypertension in stenosis of the pyeloureteral joint

Mostra-se um caso de hipertensäo arterial secundária a hidronefrose unilateral. Encontraram-se níveis de renina elevados em veia periférica e a relaçäo entre renina na veia renal do lado afetado e do lado contralateral estava elevada. A nefrectomia curou a hipertensäo arterial