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.

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.

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.