Pediatric Kidney Transplantation Outcomes in Children with Primary Urological Abnormalities Versus Nonurological Abnormalities: Long-Term Results.

PURPOSE: We assessed renal function, graft survival rates and the risk of graft loss in children based on etiology with a focus on differences between urological causes from congenital anomalies of the kidney and urinary tract vs other causes of end stage kidney disease. MATERIALS AND METHODS: A retrospective chart review was performed including patients younger than 18 years who underwent kidney transplantation at our institution from December 1984 to November 2010 with the last followup recorded in March 2018. Patient clinical characteristics, demographics and end stage kidney disease etiology were recorded. Patients were divided into the 2 groups of urological (congenital anomalies of the kidney and urinary tract) vs nonurological based on end stage kidney disease etiology, and survival analysis was performed. RESULTS: Of 112 kidney transplant cases 90 (80.4%) were associated with nonurological causes and 22 (19.6%) with urological causes. Median (IQR) patient age at transplantation was 12 (7-15) years. Median graft survival time was not statistically different according to end stage kidney disease etiology (nonurological 12 years 95% CI 10.01-13.99 vs urological 16 years 95% CI 7.59-24.41, p=0.532). There was a significant risk of graft loss in patients with urinary tract infections after transplantation (HR 3.15, 95% CI 1.59-6.25, p=0.001). CONCLUSIONS: Children requiring transplantation due to urological causes have no disadvantage in graft survival compared to children with end stage kidney disease with other causes. Patients with urinary tract infection after transplantation had a higher rate of graft loss.

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.

Long-Term Bowel Vaginoplasty With a Single Yang-Monti Tube.

INTRODUCTION: The bowel remains the favored tissue for neo-vaginoplasty (NeoVP) in pediatric patients with vaginal agenesis. In 2001, the first description of NeoVP using the Yang-Monti technique with a sigmoid double tubular flap was published. We present our experience of NeoVP with a single Yang-Monti tube (SYMT) flap and report on the use of different segments of colon. METHODS: We conducted a retrospective review of patients who underwent NeoVP using a bowel SYMT between 2009 and 2021. The procedure was performed under open abdominal surgery by isolating 8 to 12 cm from the rectosigmoid, cecum, or ascending colon. Subsequently, this segment was detubularized near the mesenteric border, folded, and retubularized transversally, leaving the mesentery in a cephalic position. A channel was dissected in the pelvis to accommodate the NeoVP. RESULTS: Seventeen patients were identified. The median age was 16 years old. The principal diagnosis was Mayer-Rokitansky's syndrome (47.1%). The median follow-up was 50 months. The mean harvested bowel length was 9 cm, and the sigmoid was the preferred site (65%). The cecum-ascending colon was used in 3 (17.6%) patients. Complications were recorded in 6 patients (35%). Of these 6 patients, 4 had introital stenosis. There was 1 case of urethrovaginal fistula and another of left hematometrocolpos. Satisfactory sexual function has been reported in sexually active individuals. CONCLUSIONS: We described our experience in NeoVP using a large bowel SYMT as a safe and effective technique. It allows decreased tension on the vascular pedicle and the use of shorter colon segments.

Does preoperative screening VCUG affect the outcomes and complications of pyeloplasty in patients with ureteropelvic junction obstruction?

INTRODUCTION: The role of voiding cystourethrogram (VCUG) in evaluating vesicoureteral reflux (VUR) in patients with known ureteropelvic junction obstruction (UPJO) remains unclear. While VCUG is frequently performed, the incidence of concomitant VUR and UPJO is low, and VUR is often low-grade with high rates of spontaneous regression. OBJECTIVE: To analyze the clinical relevance of VCUG in patients with UPJO by determining its incidence and studying the difference in clinical outcomes between patients with known, unknown, and negative VUR. STUDY DESIGN: Retrospective review of patients with UPJO who underwent pyeloplasty from 2012 to 2020 with <18 years-old, unilateral UPJO, postoperative follow-up of ≥2 months and had at least 1 renal ultrasound (US) after pyeloplasty. Results were compared among 3 groups: patients who underwent VCUG before pyeloplasty and were found to have VUR (group 1), patients who underwent VCUG before pyeloplasty without VUR (group 2), and patients who did not have a VCUG before pyeloplasty (group 3). RESULTS: A total of 275 patients met the inclusion criteria, of which 21 patients were classified in group 1, 166 patients in group 2, and 88 patients in group 3 (Table). The age at preoperative VCUG was 14.7 ± 32.9 months in group 1 and 15.17 ± 35.8 months in group 2 (p = 0.960). Overall, the incidence of concomitant UPJO and VUR was 11.2%. In group 1 the initial VUR grade was 5 in 2 patients, 4 in 3, 3 in 5, 2 in 7, and 1 in 4 patients. Of these, only 1 patient required ureteral reimplantation after pyeloplasty. Post-pyeloplasty, no significant differences were observed in complications (p = 0.7436), length of follow up (p = 0.3212), SFU grade 4 hydronephrosis (p = 0.2247), postoperative UTIs (p = 0.1047) and pyeloplasty success rate (p = 0.4206) among the 3 groups. Despite the use of antibiotic prophylaxis being significantly different amongst the three groups (p < 0.001), it was not associated with a lower incidence of postoperative UTIs (group 1 p = 0.068, group 2 p = 0.486, group 3 p = 1). In patients with reflux, an increase in age was associated with a decrease in the rates of complications (p = 0.019). CONCLUSION: We found no significant difference in the outcomes in patients who had a preoperative VCUG as compared to those who did not. The preoperative diagnosis of VUR by VCUG changed the management in less than 1% of the study population and thus its role in patients with UPJO should be reevaluated.

Correction: Neonatal Circumcision: What Are the Factors Affecting Parental Decision?

[This corrects the article DOI: 10.7759/cureus.19415.].

Complications after use of gastric segments for lower urinary tract reconstruction.

PURPOSE: We retrospectively reviewed our experience with the use of gastric segments for lower urinary tract reconstruction with an emphasis on long-term complications. MATERIALS AND METHODS: A total of 29 patients underwent reconstruction of the lower urinary tract using gastric segments between 1993 and 2000. Diagnoses included neurogenic bladder (21), cloacal exstrophy (5), solitary kidney/ectopic ureter (1), posterior urethral valves (1) and rhabdomyosarcoma of prostate (1). Gastric segment was used as gastrocystoplasty (21), composite gastroenteric cystoplasty (6), demucosalized gastrocystoplasty (1) and continent gastric reservoir (1). RESULTS: Mean followup was 13.9 years (range 9 to 16.5). Complications were seen in 15 (51.7%) patients. Seven patients had the hematuria-dysuria syndrome, which was intractable in 1 and necessitated excision of the gastric patch. Due to severe complications necessitating major reoperations 3 patients underwent re-augmentation with enteric segments without excision of the gastric tissue (composite). One patient who underwent demucosalized gastrocystoplasty had excision of the gastric tissue and re-augmentation with enteric segment due to contraction of the gastric patch. A stone developed in 1 patient with a composite gastroenteric reservoir. Malignancy developed in the reservoir in 3 patients 11, 12 and 14 years after gastrocystoplasty, and all 3 died of metastasis. CONCLUSIONS: We do not recommend the use of gastric segments for reconstruction of the lower urinary tract due to the high incidence of reoperations and complications. In patients in whom gastric segments were used in the past for lower urinary tract reconstruction, regular surveillance and close followup are strongly advocated.

Abdominal Evisceration: A Rare Complication of In Utero Vesicoamniotic Shunting.

Vesicoamniotic shunting (VAS) is one of the most common surgical options in selected cases of severe fetal obstructive uropathy. Abdominal evisceration secondary to VAS has been reported in only eight cases. A pregnant woman underwent VAS due to lower urinary tract obstruction with severe oligohydramnios. Abdominal evisceration was observed at birth. Subsequently, he underwent abdominal surgical repair and urethral dilations due to segmental urethral atresia. At 21-month-follow-up, the patient is breathing spontaneously, undergoing peritoneal dialysis, voiding small amounts of urine, and being fed via G-tube. Data from those previous case reports is systematically reviewed and compared with our case.

Small Intestinal Submucosa for corporeal body grafting in patients with proximal hypospadias and severe chordee: Long term follow-up assessing erectile function and genital self-perception.

BACKGROUND: Proximal hypospadias and severe ventral chordee are often challenging to repair. To preserve penile length in chordee repair, Small Intestinal Submucosa (SIS) corporal grafting is often performed with potential long-term complications including recurrent curvature and erectile dysfunction (ED). There is a paucity of data evaluating sexual function in mid, late and post-pubertal patients. OBJECTIVE: We aimed to assess long-term outcomes of genital self-perception and erectile function in mid, late and post-pubertal patients who underwent single-layer (1-ply) SIS corporal body grafting for correction of severe chordee. STUDY DESIGN: Patients with proximal hypospadias who underwent correction of severe chordee using SIS grafting between 2001 and 2015 were retrospectively identified. Patients were evaluated for erectile and sexual function using the modified erection hardness score (mEHS) and the modified sexual health inventory for men (mSHIM). Perceived function and straightness were measured with Hypospadias Objective Scoring Evaluation (HOSE). Penile self-perception was assessed using the Pediatric Penile Perception Score (PPPS). Results were compared to an age-matched healthy control group. Categorical variables were analyzed using Fisher's exact test, and continuous variables using paired and unpaired t-test and ANOVA. RESULTS: Nineteen patients with proximal hypospadias who underwent correction of severe chordee using SIS grafting and 18 controls participated in the study with a median age of 17 years for both groups. In the mEHS, 12 (63.2%) hypospadias-patients and 14 (87.5%) controls rated their erections as completely hard and very rigid. In the mSHIM, 1 (5.2%) hypospadias-patient was classified as having moderate ED. A total of 16 hypospadias-patients (84%) and 16 controls (88.9%) reported being very satisfied or satisfied with the straightness of their penis. No significant difference was observed in the mEHS, mSHIM and PPPS between groups (p < 0.05). The straightness of the erection was rated lower by participants, than by the pediatric urologist. In the HOSE, 12 (63.2%) hypospadias-patients and 16 (88.9%) controls obtained an acceptable score. DISCUSSION: Our findings indicate favorable long-term outcomes in ED and genital self-perception; only 5% of our population reported having a mild-moderate to moderate presentation of ED, and there were no reports of severe ED. The overall PPPS satisfaction rates were statistically similar for the control and hypospadias groups. The small sample population limits the significance of our findings. CONCLUSION: Corporal body grafting with 1-ply SIS suggests positive long-term outcomes in genital self-perception and erectile function, with mid, late and post-pubertal patients who underwent hypospadias repair having comparable results to age-matched healthy controls.

Total continence reconstruction using the artificial urinary sphincter and the Malone antegrade continence enema.

PURPOSE: Surgical management of children with myelomeningocele addresses 2 aspects of the disease, neurogenic bladder and neurogenic bowel. Results of total continence reconstruction using an artificial urinary sphincter and Malone antegrade continence enema are presented. MATERIALS AND METHODS: We performed a retrospective chart review of patients who underwent simultaneous artificial urinary sphincter placement and a Malone antegrade continence enema procedure. From 1997 to 2007 a total of 21 patients with myelomeningocele underwent total continence reconstruction using the artificial urinary sphincter. Mean patient age was 10.4 years (range 6 to 22) and mean followup was 4.7 years (range 0.66 to 11.7). Artificial urinary sphincter cuff was placed around the bladder neck. A Malone antegrade continence enema was performed using appendix in 19 patients and cecal based flaps in 2. Two patients underwent concomitant augmentation cystoplasty. Six patients had concomitant Mitrofanoff vesicostomy using split appendix in 4 and Monti tube in 2. RESULTS: Immediate postoperative complications were observed in 5 patients, including prolonged ileus (2), urinary tract infection (2) and superficial wound dehiscence (1). Seventeen patients (81%) achieved complete urinary continence and 5 were voiding with sphincter cycling. Improvement in urinary continence with dry intervals greater than 3 hours was reported in 2 patients. There were 19 patients (90%) who reported fecal continence, with 2 reporting soiling 1 to 2 times a week. Malone antegrade continence enema stoma stenosis occurred in 3 patients and 2 required revisions. Sixteen patients (76%) achieved complete continence of stool and urine. During followup 2 artificial urinary sphincters were explanted and 8 patients (38%) underwent bladder augmentation. CONCLUSIONS: Urinary and fecal continence in patients with myelomeningocele is achievable with a single total continence reconstruction procedure using the artificial urinary sphincter and the Malone antegrade continence enema with durable results.

Salvage dextranomer-hyaluronic acid copolymer for persistent reflux after ureteral reimplantation: early success rates.

PURPOSE: Endoscopic injection of dextranomer-hyaluronic acid copolymer is an accepted initial procedure to correct vesicoureteral reflux. Less data are available on its role in treating failed ureteral reimplantation. MATERIALS AND METHODS: We retrospectively reviewed the charts from 2002 to 2008 and identified 21 patients (26 ureteral units) with persistent reflux after reimplantation. RESULTS: Mean age was 7 years (range 2 to 13). Mean followup was 2 years (range 10 to 46 months). Of the 17 patients with a single system ureteral reimplantation was extravesical in 9 and intravesical in 8 with tapering performed in 5. Three patients underwent reimplantation of duplex systems and 1 underwent reimplantation due to ureterocele. Residual reflux grade was 1 to 4 in 3 (11%), 17 (65%), 3 (11%) and 3 ureteral units (11%), respectively. Dextranomer-hyaluronic acid copolymer was injected transurethrally. The mean volume injected was 1.2 ml (range 0.7 to 3). After 1 injection reflux resolved in 15 patients (71%) or a total of 20 ureteral units (77%), including 12 of 14 (86%) extravesically and 8 of 12 (66%) intravesically reimplanted units. The resolution rate improved to 84% after multiple injections. Two of the 6 patients with reflux after 1 injection had a single system, 2 had an obstructive megaureter with tapered reimplantation, 1 had a duplicated system and 1 had a ureterocele. Three of the 5 patients with persistent reflux underwent revision surgery. Ureteral abnormalities other than reflux and tapered reimplantation were associated with a statistically significant inferior success rate. CONCLUSIONS: Dextranomer-hyaluronic acid copolymer injection is an efficacious salvage procedure for persistent reflux after ureteral reimplantation. The success rate is inferior for ureteral abnormalities other than primary vesicoureteral reflux and after tapering.

Neonatal Circumcision: What Are the Factors Affecting Parental Decision?

INTRODUCTION: The American Academy of Pediatrics (AAP) guidelines state that the health benefits of circumcision outweigh the risks, but these benefits are not enough to recommend universal newborn circumcision. Therefore, it is the guardians' decision to circumcise their son. In this study, we assess the factors that influence the decision-making process for newborn circumcision. METHODS: A prospective study was done from January to April 2020 for newborn circumcision. AAP guidelines were used as an educational tool and given to the parents on the day of patient circumcision assessment. On procedure day, a self-reported survey regarding the reasons for circumcision and the usefulness of the guideline as an educational resource was given to guardians. RESULTS:  A total of 265 parents completed the survey. Of the study variables, the future health of the child and the circumcision status of the father were considered extremely important factors influencing the decision-making process for 168 (63.4%) and 90 (34%) guardians, respectively. The study showed that 226 (85.3%) of the parents found the AAP guidelines helpful whereas 39 (14.7%) did not. CONCLUSION:  Overall results suggest that the health of the child and the father of the child being circumcised are the primary factors that influence the guardians' decision to circumcise their child. In addition, providing parents with an educational resource such as the AAP guidelines policy statement prior to circumcision may serve as a way to supplement the discussion between parents and providers.

Predictive factors for early discharge (≤24 hours) and re-admission following robotic-assisted laparoscopic pyeloplasty in children.

INTRODUCTION: Minimally invasive pyeloplasty (MIP) for correction of ureteropelvic junction obstruction in children has significantly improved the postoperative management of these patients. In this study, we sought to examine the factors associated with early discharge (≤24 hours) in children who underwent robotic-assisted laparoscopic pyeloplasty (RALP). METHODS: We performed a retrospective chart review of all children who underwent RALP from 2012-2018 in our center. Descriptive statistics and a non-adjusted risk analysis were performed to evaluate the factors associated with early discharge (≤24h), re-admission, and complications within the first 30 days after the procedure. RESULTS: Eighty-nine patients out of 124 total pyeloplasties (72%) stayed ≤24 hours post-surgery. Of the variables analyzed, later cases were statistically associated with length of stay (LOS); the first 55 patients had a lower probability of being hospitalized for ≤24 hours (odds ratio [OR] 0.24, 95% confidence interval [CI] 0.09-0.64, p=0.004). CONCLUSIONS: RALP for children is associated with a high rate of early recovery, short hospital stay, and low re-admission and complication rates. Although not statistically significant, patients with shorter operative room time also had a shorter LOS. An increased LOS was observed in the initial patients of our series, and this is most likely explained by the initial learning curve of the team for the procedure itself and the more conservative postoperative management.

Voiding Cystourethrogram in Children With Unilateral Multicystic Dysplastic Kidney: Is It Still necessary?

OBJECTIVE: To evaluate the value of the voiding cystourethrogram (VCUG) in children with multicystic dysplastic kidney (MCDK) who have a normal versus abnormal contralateral kidney and bladder ultrasound (US), and assess the risk of having vesicoureteral reflux (VUR) or urinary tract infection (UTI) based on the US results. METHODS: A retrospective chart review including children with unilateral MCDK with postnatal US and VCUG available at our institution between January 2008 and September 2017 was performed. Analysis was done to find association between abnormal contralateral US and contralateral VUR and UTI. RESULTS: One hundred and fifty-six children were analyzed; 118(75.6%) patients had a normal contralateral kidney US, while 38(24.4%) had abnormal US. The rate of severe contralateral VUR (grade IV and V) was 2 (1.7%) and 5 (13.2%) in children with normal and abnormal contralateral US, respectively. The risk analysis demonstrated a significant association between severe VUR on the contralateral kidney and an abnormal contralateral US (odds ratio = 7.73; 95%CI: 1.43-41.81; P = 0.018) and no significant association with UTI (odds ratio = 1.58; 95%CI: 0.50-4.94; P = 0.435). CONCLUSION: Our data suggests, the rate of severe contralateral VUR in children with unilateral MCDK and normal contralateral kidney is low. VCUG should be considered for infants with proven MCKD and alterations on the contralateral kidney on US. Following patients with MCDK and normal contralateral kidney without the use of VCUG is a reasonable approach, unless there is development of signs and symptoms of recurrent UTI or deterioration of the renal function. We found that abnormal contralateral kidney US was associated with severe VUR.

Prenatal renal parenchymal area as a predictor of early end-stage renal disease in children with vesicoamniotic shunting for lower urinary tract obstruction.

BACKGROUND: Vesicoamniotic shunting (VAS) and other bladder drainage techniques for fetal lower urinary tract obstruction (LUTO) have been proven to ameliorate pulmonary hypoplasia and increase survival in patients with an initial poor prognosis. Currently there are limited prognostic tools available during gestation to evaluate and predict postnatal renal function. OBJECTIVE: The aim was to describe the prenatal growth of the renal parenchymal area (RPA) in patients with LUTO and determine its application as a predictor of renal function at one year of life. STUDY DESIGN: The study population comprised a retrospective cohort of all infants who survived the fetal VAS to birth. Renal growth and size were measured using imageJ software to calculate the RPA in sequential prenatal ultrasounds. The parenchymal area was measured from the image of each kidney with the greatest longitudinal length. These measurements were further correlated and analyzed as a predictor of end-stage renal disease (ESRD) within the first year of life. RESULTS: Etiologies of LUTO in the 15 male fetuses included eight posterior urethral valves, four Eagle-Barrett/prune belly syndrome, two urethral atresia, and one megacystis microcolon intestinal hypoperistalsis syndrome. All patients had patent shunts, in place, at birth. Furthermore, ultrasonographic parameters such as oligohydramnios, keyhole sign, and bladder wall thickness showed no statistical difference between groups. Renal parenchymal growth correlated with postnatal renal function in both the ESRD (r = 0.409, p = 0.018) and the non-ESRD (r = 0.657, p < 0.001) groups. Most notably, RPA during the 3rd trimester predicted ESRD with the best cut-off point determined to be 8 cm2 (sensitivity, 0.714; specificity, 0.882; and positive likelihood ratio, 6.071) (Table). DISCUSSION: Despite definitive VAS for LUTO, postnatal morbidity and mortality remain high, emphasizing the role of renal dysplasia in postnatal renal failure, in spite of urinary diversion. Renal growth statistically differs between groups in the 3rd trimester of gestation; RPA development appears stagnant in patients that developed ESRD within the first year of life. In contrast, patients that did not develop ESRD continued to have renal parenchymal growth in a linear fashion. This suggests that prenatal RPA may be predictive of postnatal ESRD. CONCLUSIONS: RPA measurement during the prenatal period could play an important role as a non-invasive tool to predict postnatal renal function and to anticipate postnatal clinical interventions.

Staged Fowler-Stephens and Single-stage Laparoscopic Orchiopexy for Intra-abdominal Testes: Is There a Difference? A Single Institution Experience.

OBJECTIVE: To compare single-stage laparoscopic orchiopexy (SSLO) and staged Fowler-Stephens (SFS) procedures in the management of intra-abdominal undescended testes, and to analyze postoperative atrophy and malpositioning as end points. MATERIALS AND METHODS: A retrospective chart review identified laparoscopic orchiopexy patients with intra-abdominal testes between November 2006 and November 2014. Of 167 patients who had laparoscopic orchiopexy, 73 (85 testes) were identified as having laparoscopic orchiopexy. Baseline characteristics, as well as testicular scrotal position and size at follow-up, were recorded. Regression analysis was performed to compare outcomes between patients who underwent SFS and SSLO. RESULTS: Of the 85 laparoscopic orchiopexies, 35 underwent SFS and 50 had SSLO. Patient demographics were comparable in both groups. The median age at surgery was 12 months (5-151 months), and the average follow-up was 17.3 months. On follow-up, there were 0 recorded cases of SFS patients with abnormally positioned testes postoperatively, whereas there were 10 (20.0%) SSLO patients who had abnormally positioned testes (odds ratio: 0.05, 95% confidence interval: 0.01-0.44). Differences in atrophy rates were not significant. CONCLUSION: These results suggest that there may be no difference between the 2 approaches in terms of postoperative atrophy. However, the SFS appears to be more successful in securing a favorable scrotal position. Atrophy does not seem to be associated with other patient factors. Prospective, randomized studies are indicated to further explore outcome differences between the 2 approaches.

Renal pelvis rupture in a kidney with ureteropelvic junction obstruction and extrarenal calyces.

The extrarenal calyx (ERC) is a rare congenital anomaly, associated with various other abnormalities of the urogenital system. We report a unique case of ERC in a solitary functioning kidney with a massively dilated pelvis that developed spontaneous rupture. A dismembered pyeloplasty was performed at the time of rupture. The patient did well post-operatively with a stable creatinine and stable SFU grade 2 hydronephrosis.

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.

Ventral corporal body grafting for correcting severe penile curvature associated with single or two-stage hypospadias repair.

PURPOSE: The literature on small intestinal submucosa for chordee correction in children is scarce. We reviewed our experience with 1 ply SIS for ventral corporal body grafting in cases of severe ventral penile curvature associated with proximal hypospadias in children. MATERIALS AND METHODS: From 04-2001 to 12-2007, 58 boys with proximal hypospadias and severe ventral curvature underwent single layered SIS graft to the corporal bodies to correct chordee. In 43 patients the surgery was done in the first stage of a planned 2-stage procedure. Fifteen patients underwent a 1-stage chordee correction with SIS and tubularized transverse preputial flap urethra. RESULTS: Mean follow-up was 4.8 years. A straight phallus with good cosmesis was achieved in 57/58 patients. In 51/58 patients an artificial erection was performed in the operating room as part of a second stage procedure or for complications associated with the one stage urethroplasty. One patient needed a second procedure to correct the curvature (chordee was over-corrected and needed a ventral Nesbitt plication). In fifteen patients that underwent a 1-stage genital reconstruction, the neourethral meatus was left in the lower part of the glans in 8 patients and at the coronal sulcus in 7. CONCLUSIONS: Corporal body grafting with single layer SIS is a viable option for correction of severe chordee associated with corporal body disproportion. SIS is a material with reliable results, easy availability and no donor site associated morbidity. As a result of penile elongation with a graft, simultaneous island flap urethroplasty became difficult in many patients.