Pelvic plexiform neurofibroma during bowel/bladder dysfunction in an NF1 pediatric patient: A case report.

Individuals with Neurofibromatosis I may develop plexiform neurofibromas throughout the body, however they are rarely seen in the pelvis. We present a 15-year-old patient with NF1 with a large unresectable neurofibroma located between the prostate and bladder discovered incidentally on renal/bladder ultrasound during the evaluation of bowel/bladder dysfunction. Despite the extensive nature of the mass, the patient presented with minimal symptoms and the lesion was thought to be stool or enlarged prostate on subsequent evaluations. Urologists should be aware of NF1 related pelvic masses in the evaluation of bowel bladder dysfunction and specify NF1 on radiologic requisitions.

Sigmoid vaginoplasty with a modified single Monti tube: a pediatric case series.

PURPOSE: No consensus exists regarding the most effective procedure for neovagina formation. We describe our experience with modified single Monti tube colovaginoplasty in pediatric patients with disorders of sexual differentiation. MATERIALS AND METHODS: Six patients were retrospectively identified who underwent primary sigmoid vaginoplasty with a modified single Monti tube between 2009 and 2012. Data were collected from patient charts. The procedure is performed by isolating an 8 to 10 cm segment of distal sigmoid colon or proximal rectum, which is detubularized along the anterior mesentery, folded and retubularized longitudinally, leaving the mesentery in a cephalad position. A channel is dissected in the pelvis to accommodate the neovagina. RESULTS: Mean patient age was 12.7 years (range 6 to 17). The primary diagnosis was androgen insensitivity in 3 cases (50%), and Mayer-Rokitansky syndrome, partial androgen insensitivity and persistent cloaca in 1 each (16.7%). Chromosomal analysis revealed 46XY in 4 patients (66.7%). Median followup was 7.9 months (range 3 to 41). One patient who engages in vaginal intercourse reported satisfactory vaginal length without discomfort. In 1 patient an anastomotic stricture developed, which was managed by buccal mucosal grafting. CONCLUSIONS: Modified single Monti tube sigmoid vaginoplasty is a safe, effective technique for neovagina formation in pediatric patients with disorders of sexual differentiation. Compared to other existing methods, our technique allows for the use of shorter bowel segments with decreased tension of the vascular pedicle.

Use of buccal mucosa in hypospadias repair.

Hypospadias is an embryological disorder that results in an abnormal ventral positioning of the urethral meatus. Among multiple surgical techniques described to correct this anomaly, the use of buccal mucosa grafts has gained popularity among pediatric urologists, pediatric surgeons and plastic surgeons. Buccal mucosa grafts have shown favorable histological changes that result in an excellent scaffold for urethral reconstructive surgery. This review describes the evolution of the use of buccal mucosa grafts in hypospadias repair.

Anterior urethral valves: not such a benign condition .

PURPOSE: Anterior urethral valves (AUVs) is an unusual cause of congenital obstruction of the male urethra, being 15-30 times less common than posterior urethral valves (PUVs). It has been suggested that patients with congenital anterior urethral obstruction have a better prognosis than those with PUV, with less hydronephrosis, and a lower incidence of chronic renal insufficiency (5 vs. 30%). The long-term prognosis of AUVs is not clear in the literature. In this report we describe our experience and long-term follow up of patients with anterior urethral valve. MATERIALS AND METHODS: We retrospectively identified 13 patients who presented with the diagnosis of AUVs in our institutions between January 1994 and June 2012. Two patients were excluded: one patient had no follow up after intervention; the other had a follow up <1 year. From the 11 patients included, we evaluated the gestational age, prenatal and postnatal ultrasound findings, voiding cystourethrogram findings, age upon valve ablation, micturition pattern, creatinine, and clinical follow up. RESULTS: Between 1994 and 2012 we evaluated 150 patients with the diagnosis of urethral valves. Of this group, 11 patients (7.3%) had AUVs and an adequate follow up. Mean follow up is 6.3 years (2.5-12 years). Five (45.4%) patients had prenatal diagnosis of AUV. The most common prenatal ultrasonographic finding was bilateral hydronephrosis and distended bladder. One patient showed a large perineal cystic mass, which was confirmed to be a dilated anterior urethra. The mean gestational age was 37.6 weeks (27-40 WGA). Postnatally, 90% had trabeculated bladder, 80% hydronephrosis, and 40% renal dysplasia. The most common clinical presentation was urinary tract infection in five patients (45.4%), followed by weak urinary stream found in four patients (36.3%). The age at initial surgical intervention ranged between 7 days and 13 years. Seven (63.6%) patients had primary transurethral valve resection or laser ablation and three patients (27.2%) had primary vesicostomies. One boy (9.1%) had penile urethrostomy with excision of urethral diverticulum. Two (18.2%) patients developed end-stage renal disease. CONCLUSION: Anterior urethral valve is a rare congenital entity affecting the genitourinary system in males. Early urinary tract obstruction resulted in end-stage renal disease in 18% of our patient population. In our series, the complication rate and the evolution to renal failure are high and similar to patients with PUV. In patients with AUVs we recommend long-term follow up and close evaluation of patient's bladder and renal function.