Gangrena de Fournier en un niño con síndrome nefrótico corticorresistente. A propósito de un caso / Fournier gangrene in a child with steroid-resistant nephrotic syndrome. Report of one case

La gangrena de Fournier es una fascitis necrotizante que afecta las regiones genital, perineal y perianal, de inicio súbito y diseminación rápidamente progresiva. Su diagnóstico obliga a una urgente intervención interdisciplinaria. La asociación con enfermedades nefrológicas es rara.Se presenta un caso de gangrena de Fournier en un niño con síndrome nefrótico corticorresistente y anasarca con edema escrotal grave. Recibió un esquema antibiótico de amplio espectro y se realizó un desbridamiento quirúrgico extenso e inmediato de la lesión necrótica. Posteriormente, requirió reparación por parte de Cirugía Plástica. Presentó una respuesta clínica favorable a la terapéutica instaurada.

Fournier gangrene is a necrotizing fasciitis that affects the genital, perineal and perianal regions, of sudden onset and rapidly progressive dissemination. Its diagnosis requires an urgent and interdisciplinary intervention. The association with nephrologic diseases is rare.We present a case of Fournier gangrene in a child with steroid-resistant nephrotic syndrome and anasarca with severe scrotal edema. He received a broad-spectrum antibiotic scheme and extensive an immediate surgical debridement of the necrotic lesion was carried out. Subsequently, it was repaired by Plastic Surgery. He presented a favourable clinical response

[Fournier gangrene in a child with steroid-resistant nephrotic syndrome. Report of one case]. / Gangrena de Fournier en un niño con síndrome nefrótico corticorresistente. A propósito de un caso.

Fournier gangrene is a necrotizing fasciitis that affects the genital, perineal and perianal regions, of sudden onset and rapidly progressive dissemination. Its diagnosis requires an urgent and interdisciplinary intervention. The association with nephrologic diseases is rare. We present a case of Fournier gangrene in a child with steroidresistant nephrotic syndrome and anasarca with severe scrotal edema. He received a broad-spectrum antibiotic scheme and extensive an immediate surgical debridement of the necrotic lesion was carried out. Subsequently, it was repaired by Plastic Surgery. He presented a favourable clinical response.

La gangrena de Fournier es una fascitis necrotizante que afecta las regiones genital, perineal y perianal, de inicio súbito y diseminación rápidamente progresiva. Su diagnóstico obliga a una urgente intervención interdisciplinaria. La asociación con enfermedades nefrológicas es rara. Se presenta un caso de gangrena de Fournier en un niño con síndrome nefrótico corticorresistente y anasarca con edema escrotal grave. Recibió un esquema antibiótico de amplio espectro y se realizó un desbridamiento quirúrgico extenso e inmediato de la lesión necrótica. Posteriormente, requirió reparación por parte de Cirugía Plástica. Presentó una respuesta clínica favorable a la terapéutica instaurada.

Reflujo vesicoureteral / Vesicoureteric reflux

El propósito de este artículo de revisión es compilar los factores más frecuentes asociados o vinculados al reflujo vesicouretral como: a) incidencia, b) mecanismo de transmisión genética, c) etiopatogenia del reflujo, d) su relación con la infección urinaria, e) patogénesis del daño parenquimatoso renal adquirido y congénito en niños con reflujo, f) reflujo intrarrenal y su asociación con el daño renal, g) rol de las disfunciones vesicales no-neurogénicas, h) métodos de diagnóstico y i) presentación clínica. Se analizan además, distintos aspectos del tratamiento del reflujo que siguen generando controversias; las indicaciones de la profilaxis antibiótica, el tratamiento quirúrgico clásico creando un túnel submucoso ureteral de longitud adecuada y el tratamiento endoscópico con sustancias de abultamiento. Aún persisten varios aspectos polémicos relacionados con el reflujo, en esta revisión se examinan algunos de ellos. Es el propósito de este trabajo que el lector interesado en el tema de la urología pediátrica se beneficie con la lectura de esta revisión

The intention of this review article was to compile outlining areas surrounding the effects of vesicoureteric reflux (VUR) and associated factors, such as: a) incidence, b) patterns of inheritance, c) etiology of VUR, d) relationship between reflux and urinary tract infection, e) diagnostic imaging studies, f) etiology of acquired and congenital kidney damage related to VUR, g) importance of intrarenal reflux in reflux nephropathy, h) role of high bladder pressures on the kidney and i) clinical presentation. In addition, we also discussed the management of VUR which is still controversial, the role of antibiotic therapy and indications of ureteric reimplantation or endoscopic correction of reflux; still debated in the pediatric urologic literature. Many unanswered questions remain, and are analyzed in this review. We hope the readers will profit from reading this review

Traumatic Posterior Urethral Strictures in Children and Adolescents.

Background: Management of partial or complete traumatic urethral disruptions of the posterior urethra in children and adolescents, secondary to pelvic fracture poses a challenge. Controversy exists as to the correct acute treatment of posterior urethral injuries and delayed management of PFPUDDs. We reviewed the urological literature related to the treatment of traumatic posterior urethral injuries and delayed repair of these distraction defects in children and adolescents. Material and Methods: There are few long-term outcomes studies of patients who underwent PFPUDDs repairs in childhood; most reports included few cases with short follow up. We excluded studies in which the cohort of patients was heterogeneous in terms of stricture disease, etiology and location. Results: Primary cystostomy and delayed urethroplasty is the traditional management for PFPUIs. Immediate repair is rarely possible to perform. Realignment of posterior urethral rupture in children is indicated in special situations: (a) concomitant bladder neck tears, (b) associated rectal lacerations, (c) long disruptions of the urethral ends. Before delayed reconstruction ascending urethrography and micturating cystourethrogram along with retrograde and antegrade urethroscopy define site and length of the urethral gap. However, the most accurate evaluation of the characteristics of the distraction defect is made when surgical exposure reveals the complexity of the ruptured urethra. Partial ruptures may be managed with urethral stenting or suprapubic cystostomy, which may result in a patent urethra or a short stricture treated by optical urethrotomy. The gold standard treatment for PFPUDDs in children is deferred excision of pelvic fibrosis and bulbo-prostatic tension-free anastomosis, provided a healthy anterior urethra is present. Timing of delayed repair is at 3 to 4 months after trauma. Some urologists prefer either the perineal access or the transpubic approach to restore urethral continuity in children with PFPUDDs. Substitution urethroplasties are used in children with PFPUDDs, when anastomotic repair can't be achieved due to severe damage of the bulbar urethra. Conclusion: As evidenced in this review the progressive perineo-abdominal partial transpubic anastomotic repair has advantages over the isolated perineal anastomotic approach in patients with "complex" PFPUDD. This approach provides wider exposure and facilitates reconstruction of long or complicated posterior urethral distraction defects.

Megaprepuce Reconstruction: A Single Center Experience.

INTRODUCTION: Surgical treatment of congenital megaprepuce is challenging and controversial. We report our 10-year experience treating patients with this deformity using a standardized procedure that has similarities to a technique reported by Smeulders et al. (1). Our postoperative complications and mid-term follow-up cosmetic appearance of the genitalia after reconstruction are reviewed. MATERIAL AND METHODS: Fifteen patients operated on between 2005 and 2015 were evaluated. Age at surgical repair ranged from 3 to 20 months (mean 9). Treatment included unfolding the preputial sac via a ventral approach, excision of redundant inner preputial skin, and ventral skin coverage with the outer preputial layer. Twelve patients presented associated partial scrotal engulfment, which was simultaneously treated. Mean follow-up was 4.6 years (range 2-7 years). RESULTS: Short-term complications occurred in three patients: scrotal hematoma in one patient and small skin dehiscence at the penoscrotal junction in two patients. Skin disruption healed by secondary epithelial ingrowth. All cases resulted in a satisfactory genital cosmetic outcome. There were no late complications. All patients preserved normal external genitalia appearance. CONCLUSION: Our experience is in agreement with reports of other authors; suggesting that excision of the inner preputial layer and using the external one for penile coverage provide good and durable mid-term esthetic results in megaprepuce reconstruction.

Delayed surgical repair of posttraumatic posterior urethral distraction defects in children and adolescents: long-term results.

INTRODUCTION: Various surgical techniques have been proposed to treat pelvic fracture urethral distraction defects (PFUDDs) in children (Figure): primary alignment of the acute transected urethra, substitution procedures and delayed anastomosis urethroplasties (DAU) by perineal, elaborated perineal, transpubic or perineo-abdominal/partial transpubic access. However, long-term follow-up of surgical correction for PFUDDS with DAU is infrequently reported in the literature. PURPOSE: Long-term efficacy of DAU in children and adolescents with PFUDDs was evaluated. Other surgical methods used to accomplish tension-free DAU were also described. MATERIAL AND METHODS: We reviewed records of 49 male children aged 3.5-17.5 years (median 9.6) with PFUDDS who underwent DAU from 1980 to 2006. Median PFUDDs length was 3 cm (range 2-6). Six patients had prior failed treatments: anastomotic urethroplasties (5) and internal urethrotomy (1). Surgical access was transperineal in 28 cases and perineal/partial pubectomy in 21. Urethral rerouting was performed in 8 cases. Median follow-up was 6.5 years (range 5-22). RESULTS: On review median PFUDDS length in patients treated with primary cystostomy was 3 cm compared to those initially managed with urethral alignment (4 cm). Five patients treated with perineal DAU developed recurrent strictures at the anastomosis site, successfully managed with additional perineal/partial pubectomy anastomosis (4 cases) and internal urethrotomy (1). Primary and overall success rate was 89, 7% and 100%, respectively. Urinary incontinence occurred in 9 cases. Two had overflow incontinence and performed self-catheterization; 1 developed sphincter incontinence and required AUS placement, while 4 of 6 cases with mild stress incontinence achieved dryness at pubertal age. Retrospectively, associated bladder neck lesions at trauma time were noted in 5 patients. Three patients with erectile dysfunction before DAU remained impotent. DISCUSSION: In children, several factors make management of PFUDDs more difficult than in adults: 1) restricted surgical access to reach a high lying proximal urethral end, 2) long distraction defects, 3) simultaneous bladder neck and membranous urethral lesions and 4) small urethral caliber. In our experience and that of others (Turner Warwick, 1989 and Ranjan, 2012), radiographic and endoscopic findings provide information on stricture features; however, the final choice of surgical exposure to restore urethral continuity is made at operative time based on PFUDD complexity. Perineal exposure usually allows performing DAU in 2 cm long PFUDDs. Ten percent of our patients treated with perineal DAU developed recurrent strictures attributed to inappropriate access selection or unrecognized PFUDD complexity. Failures were treated endoscopically (1) and by perineal/partial pubectomy anastomotic urethroplasty (4) with 100% final success. We used perineal/partial pubectomy DAU in 43% of the cases to excise pelvic scarring and bridge long urethral gaps, with urethral rerouting in 8 cases. Success rate of initial perineal and perineal/partial pubectomy anastomotic procedures was 82% and 100%, respectively. Koraitim (1997), Orabi (2008) and Ranjan (2012) reported excellent outcomes in children with either transperineal or transpubic anastomotic repair, as opposed to poor results in those undergoing substitution urethroplaties. Most reports rarely evaluate urinary incontinence after successful DAU. At the end of follow-up only 2 of our 9 initial incontinent cases remain with acceptable stress incontinence. Retrospectively, in 5 cases the original trauma comprised the bladder neck and the membranous sphincter mechanism. In our series erectile dysfunction after trauma did not change after DAU except in 1 patient who regained potency 1 year after repair. STUDY LIMITATIONS: All patients were referred after initial treatment was done elsewhere, thus they may represent the most severe PFUDDs cases. Additionally, erection dysfunction was not investigated in the kind of detail required due to patients' age. CONCLUSIONS: DAU has durable success rate for PFUDDs treatment in children with a healthy bulbar urethra. In childhood, additional surgical steps are frequently needed to achieve direct anastomotic repair.

Renal parenchyma abnormalities in infants with dilating vesicoureteral reflux: relation to reflux severity and pressure at onset.

PURPOSE: We used (99m)technetium dimercapto-succinic acid scans in infants to determine the relation between renal deformities and severity of primary dilating reflux. We also used videourodynamics to analyze the association between detrusor pressure at onset of reflux and degree of renal changes. MATERIALS AND METHODS: A total of 20 patients (15 males) 0.10 to 1.3 years old (median 0.75) with grade III to V reflux underwent conventional filling videourodynamics. (99m)Technetium dimercapto-succinic acid scans were done 6 months after the last febrile urinary tract infection (median age 0.58 years). RESULTS: Nine males and 1 female had bilateral refluxing ureters, and 10 patients had unilateral reflux (grade III in 10, IV in 11 and V in 9). Among the 30 refluxing ureters renal changes were present in 19 kidneys (63%). Prevalence of renal changes according to reflux grade III, IV and V was 40%, 63% and 89%, respectively (p <0.02). Reflux occurred in 13 patients (21 ureters) with detrusor pressure less than 10 cm water and in 7 (9) at 30 to 70 cm water (median 39.5). Renal changes were significantly more common in low pressure, low volume refluxing ureters (84.6%) compared to ureters refluxing at higher volumes and low pressure (37.5%, p <0.02). At higher pressure reflux renal deformities occurred in 55.5% of refluxing ureters (5 of 9). Five patients had renal function impairment (mean creatinine for age 0.92 mg/dl, range 0.60 to 1.53). CONCLUSIONS: High grade reflux and reflux occurring in the first half of bladder filling at low pressure are risk factors for renal changes in this cohort of infants.

Perineal mobilization of the common urogenital sinus for surgical correction of high urethrovaginal confluence in patients with intersex disorders.

OBJECTIVE: We report anatomical and cosmetic results of feminizing genital reconstruction in patients with a high vagina due to disorders of sexual differentiation. PATIENTS AND METHODS: Twelve patients with urogenital sinus anomalies graded as Prader IV underwent one-stage perineal clitoral vaginoplasty at a mean age of 1.6years. Seven patients had congenital adrenal hyperplasia, four partial androgen insensitivity and one mixed gonadal dysgenesis. Mobilized common sinus, opened dorsally without pubourethral ligament dissection, was used in combination with a perineal skin flap to construct the distal vagina. Clitoroplasty and labioplasty were done simultaneously. Mean follow up was 7.3years. RESULTS: In all cases the vaginal introitus was positioned in the vestibule region below the urethral meatus. One patient developed postoperative glans atrophy. Agreement between parental and physician satisfaction with postoperative cosmetic genital appearance was recorded in 11 girls. Vaginal stricture occurred in one patient, treated successfully with repeat vaginoplasty. One girl experienced urinary stress incontinence and became dry after bladder neck injection of a bulking agent. CONCLUSION: This procedure is successful in creating a feminine genital appearance in children having disorders of sexual differentiation with high vagina. Long-term follow up is needed to reassess the initial good anatomical and cosmetic results and evaluate sexual function after puberty.

Recurrent eosinophilic cystitis in a child with chronic granulomatous disease.

Eosinophilic cystitis is an uncommon disease in children, and its association with chronic granulomatous disease (CGD) has been previously reported in only five patients. In all those patients the disease showed either a self-limited benign course or a rapid response to corticosteroid treatment. The authors describe a child with X-linked CGD who developed eosinophilic cystitis with a recurrent course and difficult therapeutic management. The authors also discuss the pathogenesis of granuloma formation in CGD and review the literature for current therapies for these complications.

Videourodynamic findings in young infants with severe primary reflux.

PURPOSE: We investigated bladder function on videourodynamic studies in infants with severe primary vesicoureteral reflux (VUR) and analyzed the relationship between VUR and intravesical detrusor pressure during the micturition cycle. MATERIALS AND METHODS: From 1999 to 2001, 3 female and 9 male infants with a median age of 9 months with VUR underwent conventional filling videourodynamics at our institution. Four cases were diagnosed by prenatal detection and 8 were diagnosed after symptomatic urinary tract infections. Reflux was bilateral in 5 cases and unilateral in 7 (grades III to V in 7, 7 and 3, respectively). Controls included 10 age matched infants (median age 4 months) studied with the same urodynamic methodology. RESULTS: Median cystometric bladder capacity expected for age +/- SD was 50% +/- 58% (range 20% to 240%). Bladder capacity was low (less than 80%) in 10 patients. Detrusor overactivity during filling was found in only 1 patient. In all except 1 case voiding alternated with peaks of high detrusor pressure (median 87 +/- 25 cm water), associated with intermittent external sphincter contractions with normal coordinated micturition (median 36 +/- 7 cm water). Cystometric residual urine (30% or greater) was noted in 3 cases. In 5 patients VUR occurred with an increase in bladder pressure, while in 7 it occurred during stable filling. No significant cystometric differences were found between patients with reflux and controls. CONCLUSIONS: Most infants with VUR had decreased cystometric bladder capacity and normal detrusor activity during filling. High voiding pressure with concomitant sphincteric overactivity coexisted with normal micturition. This voiding pattern might represent a developmental stage of normal urine control and it may have a role in the etiology of reflux in some infants.

Urinary continence outcome after augmentation ileocystoplasty as a single surgical procedure in patients with myelodysplasia.

PURPOSE: Preoperative prediction of urinary continence in patients with myelodysplasia requiring augmentation cystoplasty is uncertain. To determine reliable factors to predict postoperative urinary continence, we retrospectively analyzed preoperative videourodynamic parameters and urinary continence outcome in a group of patients with myelodysplasia who underwent augmentation ileocystoplasty. MATERIALS AND METHODS: Of 75 patients with myelodysplasia with neurogenic bladder dysfunction refractory to conservative management (anticholinergic drugs and clean intermittent catheterization) who underwent augmentation cystoplasty as a single procedure we selected 14 girls and 12 boys without previous vesicostomy drainage and with preoperative and postoperative videourodynamic studies in whom detubularized ileocystoplasty was performed. Preoperative and postoperative evaluation included physical examination, assessment of renal function, urine culture, renal and bladder ultrasound and videourodynamic studies. We analyzed on preoperative videourodynamic studies bladder outlet morphology at 20 cm. water of filling detrusor pressure, leak point pressure, cystometric bladder capacity and end filling detrusor pressure. Videourodynamic bladder outlet morphology was classified as closed bladder outlet, insinuated bladder neck, bladder neck and urethra open up to the sphincter, and bladder outlet widely open. Patients were considered continent if they were able to remain dry between catheterization without pads. For analysis patients were divided into group 1-19 with preoperative incontinence and group 2-7 without urinary incontinence. RESULTS: After surgery 4 group 1 patients had urinary incontinence whereas all group 2 patients remained continent. Only 1 group 1 patients with leak point pressure less than 50 cm. H2O and 3 with leak point pressure greater than 50 cm. H2O were incontinent after surgery. Seven group 1 patients with cystometric bladder capacity greater than 75% and 8 with cystometric bladder capacity less than 75% of the age expected bladder capacity achieved continence. Two group 1 patients with filling detrusor pressure less than 40 cm. H2O and 2 with filling detrusor pressure greater than 40 cm. H2O continued with urinary incontinence after ileocystoplasty. Preoperative videourodynamic images of the 4 group 1 patients who remained incontinent after surgery demonstrated irrespective of the aforementioned urodynamic parameter widely open bladder outlet. Moreover, none of the postoperative continent patients had an open bladder neck and urethra on preoperative evaluation. CONCLUSIONS: According to our findings of the anatomical morphology of the bladder outlet (bladder neck/urethra) during the filling phase of videourodynamic studies at 20 cm. water filling detrusor pressure was the most reliable parameter to predict continence outcome after detubularized augmentation ileocystoplasty.