New frontiers on the molecular underpinnings of hypospadias according to severity.

Hypospadias, which is characterised by the displacement of the urethral meatus from its typical anatomical location in males, shows various degrees of severity. In this systematic review, we surveyed our current understanding of the genetics of isolated hypospadias in humans according to the severity of the condition. We found that sequencing and genotyping approaches were the preferred methods of study and that single nucleotide polymorphisms were the most common finding associated with hypospadias. Most genes fell into four gene-pathway categories related to androgens, oestrogens, growth factors, or transcription factors. Few hypospadias studies classify their findings by severity. Taken together, we argue that it is advantageous to take into consideration the severity of the condition in search of novel candidates in the aetiology of hypospadias. Abbreviations: AR: androgen receptor; ATF3: activating transcription factor 3; BMP4: bone morphogenetic protein 4; BMP7: bone morphogenetic protein 7; CYP17: steroid 17-alpha-hydroxylase/17,20 lyase; CYP1A1: cytochrome P450 1A1; CYP3A4: cytochrome P450 3A4; CNVs: copy number variants; DGKK: diacylglycerol kinase kappa; ESR1: oestrogen receptor 1; ESR2: oestrogen receptor 2; FGF8: fibroblast growth factor 8; FGF10: fibroblast growth factor 10; FGFR2: fibroblast growth factor receptor 2; HOXA4: homeobox protein Hox-A4; HOXB6: homeobox protein Hox-B6; HSD17B3: hydroxysteroid 17-beta dehydrogenase 3; MAMLD1: mastermind-like domain-containing protein 1; SF-1: splicing factor 1; SHH: sonic hedgehog; SNPs: single nucleotide polymorphisms; SOX9: SRY-box 9; SRD5A2: steroid 5 alpha-reductase 2; SRY: sex-determining region Y protein; STAR: steroidogenic acute regulatory protein; STARD3: StAR-related lipid transfer protein 3; STS: steryl-sulfatase; WT1: Wilms tumour protein; ZEB1: zinc finger oestrogen-box binding homeobox 1.

Current Concepts in Pediatric Robotic Assisted Pyeloplasty.

Robotic surgery in pediatric urology has been gaining popularity since its introduction almost two decades ago. Robotic assisted pyeloplasty is the most common robotic procedure performed in pediatric urology. Advances in robotic technology, instrumentation, patient care and surgical expertise have allowed the correction of ureteropelvic junction (UPJ) obstruction in most patients using this minimally invasive technique. The excellent experience with robotic assisted pyeloplasty has challenged other approaches as a new standard for the treatment of UPJ obstruction. In this review, we will describe the technique as it relates to the different robotic platforms, review the surgical experience and compare its results to other surgical approaches. Also, we will discuss patient and parent satisfaction, cost and financial considerations, along with evaluating the future of robotic surgery in the treatment of UPJ obstruction.

Evaluation and Management of the Undescended Testis in Puerto Rico: A Single Surgeon's 10 Years of Experience.

OBJECTIVE: Cryptorchidism is an abnormality of the male genitourinary tract in which one or both testes fail to descend into the scrotum. The American Urological Association (AUA) clinical guidelines for the evaluation and treatment of cryptorchidism were recently published. We reviewed our experience with the evaluation and management of our patients and examined our findings with respect to the AUA and European Association of Urology (EAU) guidelines. METHODS: Data were obtained from pediatric patients who underwent a surgical intervention for an undescended testis from 2007 through 2017 at HIMA Hospital and the University Pediatric Hospital (both in Puerto Rico); all the surgeries were performed by the same surgeon. A total of 754 patients were identified; 142 patients were excluded due to lack of follow-up data (N = 612). The data obtained included age, testes locations, radiologic and surgical findings, and postoperative results. RESULTS: At their initial evaluations, a large proportion of the patients (46.4%) came accompanied with radiographic imaging. These findings were consistent with those of the physical examination in 58.5% of the patients and with the surgical findings in 63.1% (sensitivity 77.9%, specificity 45.8%). Our data showed that the median referral age was 24 months, which suggests that there was a significant delay in diagnosis. At the time of surgery, the average age of the patients who required an orchiectomy was 3.93 years, while those who underwent an orchiopexy had an average age of 3.28 years. CONCLUSION: Our data reveal that, despite its lack of usefulness, radiologic imaging continues to be included in the diagnostic workups of children newly identified with cryptorchidism in Puerto Rico. In addition, and contrary to the guidelines, there tends to be a significant delay in treatment with surgical intervention. It is important to continue to educate our referring physicians on the AUA and EUA guidelines in order to create awareness and encourage the proper diagnostic and treatment approach for cryptorchidism.

Can regional anesthesia have an effect on surgical outcomes in patients undergoing distal hypospadia surgery?

BACKGROUND: Caudal and penile blocks are the most popular regional anesthetic techniques used in infants and children undergoing urological surgery. A recent report has suggested that penile venous pooling resulting from caudal blocks could affect surgical outcomes after hypospadias operations. OBJECTIVE: The aim was to report our experience in patients with distal hypospadias undergoing repair with caudal versus penile block. STUDY DESIGN: A retrospective clinical database was constructed for patients who underwent distal hypospadias repair by a single surgeon (M.P.B.) at our sponsoring institutions for the time period 2008-2013 (n = 192). Collected data included hypospadias classification (glanular, coronal, subcoronal), chordee status, perioperative anesthesia (caudal vs. penile), and assessment of postoperative complications (fistula and meatal stenosis). RESULTS: Risk ratio (RR) analysis for all distal hypospadias cases revealed that there is a higher risk of developing complications in patients who underwent caudal anesthesia than in patients who underwent penile block RR for a complication was 3.70 (95% CI 1.05-13.03; p < 0.04) (Figure). DISCUSSION: Similar to other papers in the literature, we found that patients who underwent caudal anesthesia had more complications than those who underwent penile block. The limitations of this study include not adjusting the results according to the severity of hypospadias. CONCLUSION: The main goal of this study was accomplished by demonstrating that, in our series, caudal anesthesia is associated with a higher risk of fistula formation after undergoing distal hypospadias repair than penile block.

Age of the mother as a risk factor and timing of hypospadias repair according to severity.

BACKGROUND & OBJECTIVES: Hypospadias is characterized by a displacement of the urethral opening in males that can change from the typical position within the glans penis to a subcoronal position (Type I), to anywhere along the ventral shaft (Type II), to penoscrotal, scrotal, or perineal positions (Type III). We and others have previously reported that age of the mother (≥ 40 years old) is a risk factor for having a child with hypospadias, but there is a scarcity of reports on whether such risk is higher for having a child with the mild (Type I) or the more severe forms (Types II and III). In addition, we aimed to assess the timing of hypospadias repair according to severity. METHODS: Parents of children with hypospadias were interviewed by using a series of questionnaires (n = 128 cases). Severity was confirmed in the clinic and age of the mother was self-reported. Number of surgeries, age of child by the first and the last intervention was also assessed. Ordered logistic regression and the Brant test were employed to calculate risk between mild (Type I) and severe cases (Types II and III), and the assumption of proportional odds, respectively. The Mann-Whitney U Test was used to compare number of surgeries and age by the last repair between mild and severe cases. One-way ANOVA was employed to compare age of the child at the time of first surgery across severities (Types I - III). RESULTS: Women ≥ 40 years of age are 3.89 times [95% CI: 1.20-12.64] at a higher risk for having a child with the more severe forms of the condition than younger women. Repair of Type I was accomplished with 1 intervention whereas more severe cases required 1 - 4 (2 ± 0.5) surgical interventions. The timing for hypospadias repair of Type I cases occurred at an average age of 16.2 ± 4.88 months, of Type II cases occurred at an average age of 20.3 ± 8.15 months whereas the average age of the first hypospadias repair among Type III cases was 12.68 ± 2.52 months. Number of surgeries according to severity (p ≤ 0.0018, z-ratio = 2.91) and age difference for the timing of last repair (p ≤ 0.045, z-ratio = 1.69) were statistically different, but not the age difference for the first repair. CONCLUSIONS: Increased maternal age is associated with the most severe forms of hypospadias. There is room for improvement for the timing of hypospadias repair according to severity.

Concordance of Expert and Parental Opinion about Hypospadias Surgical Outcome Is Severity Dependent.

INTRODUCTION: Hypospadias is a male congenital condition where the opening of the urethral meatus is not located in the typical anatomical position. It has been a challenge for empirical studies to ascertain the level of concordance of opinion among parents and urologists with regard to surgical outcomes according to hypospadias severity. MATERIALS AND METHODS: Parents of children who had undergone hypospadias repair were recruited for this study (n = 104). A set of questionnaires that included some items with Likert scale were created to evaluate postsurgical satisfaction by parents and urologists. SAHLSA-50, a validated instrument for adult Spanish-speaking adults, was used to assess health literacy. Cohen's kappa (κ) coefficient was used to assess interobserver agreement and Chi Square "Goodness of Fit" Test was used to determine probability of satisfaction. FINDINGS: Concordance on the level of satisfaction with surgical outcomes for Type cases I was slight (κ = 0.20; CI 95% 0-0.60), for Type II cases was moderate (κ = 0.54; CI 95% 0.13-0.94), and for Type III cases was substantial (κ = 0.62; CI 95% 0-0.56). However, the probability of satisfaction did not change according to severity (Chi Square "Goodness of Fit" Test; parents, p = 0.84; pediatric urologists, p = 0.92). These results cannot be explained by parental health literacy according to SAHLSA-50 test scores. CONCLUSION: The level of concordance of opinion among parents and urologists with regard to their level of satisfaction with surgical outcomes is related to hypospadias severity, whereby the greatest level of concordance of opinion was achieved among most severe cases. This study underscores the need for longer follow-up to properly assess satisfaction with hypospadias repair, especially for the less severe forms of the condition.

Multi-institutional Experience in Laparoendoscopic Single-site Surgery (LESS): For Major Extirpative and Reconstructive Procedures in Pediatric Urology.

OBJECTIVE: To review peri-procedural outcomes from a large, multi-institutional series of pediatric urology patients treated with laparaendoscopic single-site surgery (LESS) for major extirpative and reconstructive procedures. MATERIALS AND METHODS: Consecutive LESS cases between January 2011 and May 2014 from three free-standing pediatric referral centers were reviewed. Data include age, sex, operative time, blood loss, length of stay, and complications according to the modified Clavien-Dindo classification. Hasson technique was used for peritoneal entry, GelPOINT advanced access platform was inserted, and standard 5mm laparoscopic instruments were used. RESULTS: Fifty-nine patients (median age 5 years, 4 months-17 years) met inclusion criteria: 29 nephrectomies, 9 nephroureterectomies, 3 bilateral nephrectomies, 5 heminephrectomies, 5 renal cyst decortications, 3 bilateral gonadectomies, 2 Malone antegrade continence enema, 2 calyceal diverticulectomy, and 1 ovarian detorsion with cystectomy. Median operative times for each case type were comparable to published experiences with traditional laparoscopy. Overall mean and median length of stay was 36.2 hours and 1 day, respectively. There were two complications: port site hernia requiring surgical repair (Clavien IIIb) and a superficial port site infection that resolved with antibiotics (Clavien II). Cosmetic outcomes were subjectively well received by patients and their parents. Operative time was significantly shorter between the first half of the experience and the second half (102 vs 70 minutes, P < .05). CONCLUSION: LESS approach can be broadly applied across many major extirpative and reconstructive procedures within pediatric urology. Our series advances our field's utilization of this technique and its safety.

Laparoscopic Single-Site Surgery in Pediatric Urology: Where Do We Stand Today?

Since the first description of the laparoendoscopic single-site surgery (LESS) in the pediatric urology population, various authors have shared their experiences and results. We aim to provide a review of current studies of LESS and share our experience with this modality. The current literature describes the use of LESS for most surgeries performed in the pediatric urology population with similar results to open and standard laparoscopic surgery. The authors have described their experiences with transabdominal and retroperitoneal nephrectomy, nephroureterectomy, pyeloplasty, orchidopexy, varicocelectomy, and renal cyst decortication. In our experience, LESS has taken a role for extirpative surgery since we use other modalities for upper tract reconstruction. The data available has validated LESS as safe and has demonstrated similar results to standard laparoscopic surgery and open surgery with better cosmetic results.

Tumor in bladder reservoir after gastrocystoplasty.

PURPOSE: To our knowledge the risk of malignancy in patients with previous bladder augmentation with stomach is unknown. We report 3 cases of gastric adenocarcinoma and 1 of transitional cell carcinoma after augmentation cystoplasty with stomach with long-term followup. MATERIALS AND METHODS: Between August 1989 and August 2002, 119 patients underwent augmentation cystoplasty with stomach at our 2 institutions (University of Miami School of Medicine, and Seattle Children's Hospital and Regional Medical Center). Medical records, urodynamic studies, radiographic imaging and laboratory evaluations were reviewed retrospectively and cases of malignancy were analyzed in detail. RESULTS: Four male patients had carcinoma after augmentation gastrocystoplasty. Preoperative diagnosis was neurogenic bladder in 3 patients and posterior urethral valve in 1. Three patients had gastric adenocarcinoma, while the other had poorly differentiated transitional cell carcinoma. Each case progressed to malignancy more than 10 years after augmentation (11, 12, 14 and 14 years, respectively). CONCLUSIONS: Patients who undergo bladder augmentation with a gastric remnant are at increased risk for malignancy, probably similar to that in patients with enterocystoplasty. Therefore, they require close long-term followup. Patients should be followed annually with ultrasound, and cystoscopy should be performed annually starting 10 years after gastrocystoplasty unless they have abnormal ultrasound, hematuria or another cancer risk factor. Any suspicious lesions should be biopsied, especially at the gastrovesical anastomotic site.

Severe congenital midureteral dilatation.

Congenital midureteral stricture is an uncommon cause of prenatal hydronephrosis, and it is generally associated with proximal hydroureteronephrosis . We report a unique case of a congenital severe midureteral dilatation associated with mild proximal ureteral dilatation and normal distal ureteral diameter. The dilated midureter was excised, and an open end-to-end ureteroureterostomy was performed. Follow-up 6 months after surgical correction revealed marked improvement in the left-sided hydronephrosis and complete resolution of the hydroureter.

Salvage extravesical ureteral reimplantation after failed endoscopic surgery for vesicoureteral reflux.

PURPOSE: Open intravesical ureteral reimplantation has been reported to be uncomplicated following dextranomer/hyaluronic acid implantation. However, there are no known reports regarding extravesical ureteral reimplantation following dextranomer/hyaluronic acid failure. We reviewed our experience with extravesical ureteral reimplantation after dextranomer/hyaluronic acid failure. MATERIALS AND METHODS: We reviewed the charts of 30 patients who underwent extravesical ureteral reimplantation after dextranomer/hyaluronic acid failure. If reflux was initially bilateral and only a unilateral cure was achieved, the cured ureter was not reimplanted. Intraoperative complications, need for stenting or prolonged bladder catheterization, length of hospitalization and radiographic results were noted. RESULTS: At cystoscopy the dextranomer/hyaluronic acid implants were not seen in half of the patients. In the remaining 15 patients the blebs had moved caudally and/or were seen extravesically at the ureteral hiatus or along Waldeyer's sheath. Unilateral extravesical ureteral reimplantation was performed without difficulty in all patients and there were no intraoperative complications. Stents were left indwelling only in those patients who had undergone dismembered ureteral reimplantation. No patient required prolonged bladder drainage. All patients except 2 who had undergone additional procedures were discharged home within 24 hours postoperatively. There was no evidence of obstruction on postoperative renal sonography, and extravesical ureteral reimplantation was confirmed to be successful in all 24 patients with postoperative voiding cystourethrograms. CONCLUSIONS: Extravesical ureteral reimplantation can be performed without difficulty following dextranomer/hyaluronic acid implantation. Since extravesical ureteral reimplantation is less morbid and better tolerated than intravesical reimplantation, it is an excellent treatment option for patients with persistent unilateral vesicoureteral reflux following dextranomer/hyaluronic acid implantation. Furthermore, in cases in which vesicoureteral reflux is initially bilateral our data suggest that reimplantation of the successfully treated contralateral ureter can be avoided.

Segmental multicystic dysplasia and ureteropelvic junction obstruction in a nonduplicated kidney.

A 2-month-old child presented for evaluation of prenatal hydronephrosis. Imaging studies were consistent with a right duplex system with a dysplastic, nonfunctioning upper pole and lower pole ureteropelvic junction obstruction. We proceeded with removal of the upper pole and pyeloplasty and were surprised to find a single collecting system with a cystic, dysplastic upper pole segment and the absence of an upper pole pelvis or ureter. The rare diagnosis of a segmental multicystic dysplastic kidney with ipsilateral ureteropelvic junction obstruction was made. We present a review of the case and of previous literature on this topic.

New contralateral vesicoureteral reflux following dextranomer/hyaluronic Acid implantation: incidence and identification of a high risk group.

PURPOSE: To our knowledge the incidence of NCVUR following the endoscopic treatment of VUR with Dx/HA has not been reported previously. We evaluated the outcomes in a group of patients to determine the incidence, and to attempt to identify risk factors. MATERIALS AND METHODS: A total of 126 children with primary unilateral VUR underwent unilateral Dx/HA implantation at our institutions. The incidence of NCVUR was determined by postoperative VCUG. Indications for surgery, patient age and gender, preoperative grade of VUR and volume of Dx/HA injected were assessed as possible risk factors for NCVUR. RESULTS: Of the patients 96 (76.2%) were female, and mean age was 4.8 years. The principal indications for Dx/HA implantation were persistent reflux in 56 patients (44.4%) and primary therapy in 51 (40.5%). At followup VCUG 17 patients (13.5%) had NCVUR. No variable independently appeared to influence the incidence of NCVUR. Statistical analysis suggests that females younger than 5 years have an increased incidence of NCVUR (13 of 62, or 21% vs 4 of 64, or 6.3% of the remaining patients, p = 0.016). CONCLUSIONS: NCVUR occurred in approximately 13% of our patients. Patients with higher preoperative VUR grade or a lower number of preoperative VCUGs and those undergoing treatment as primary therapy did not have an increased incidence. Girls younger than 5 years had the highest incidence of NCVUR, and initial bilateral injection may be a consideration for this group. Further effort directed at identifying the etiology and risk factors for NCVUR is needed.

Endoscopic treatment with dextranomer/hyaluronic acid for complex cases of vesicoureteral reflux.

PURPOSE: The surgical correction of primary vesicoureteral reflux (VUR) is highly successful. This success decreases in more complex cases and often involves reoperation and increased morbidity. We present our experience with the use of subureteral injection of dextranomer/hyaluronic acid (Dx/HA) in complex cases of vesicoureteral reflux in which open surgery would have been indicated. MATERIALS AND METHODS: Between October 2001 and July 2003, 72 patients 9 months to 31 years old (mean age 5.6 years) underwent subureteral injection of Dx/HA for complex VUR at our institutions. Dx/HA was injected submucosally within the intramural ureter (modified STING) in most cases. A guidewire was used to manipulate the ureteral orifice and a retrograde ureterogram was used to delineate the anatomy in selected cases. The average volume of injected material was measured for each ureter. Renal sonography was performed to determine if hydronephrosis was present. At 3 months fluoroscopic voiding cystourethrograms were used to evaluate for the presence of VUR. RESULTS: A total of 93 ureters were treated in 55 girls and 17 boys. All cases were considered to be complex as 17 had persistent reflux after open surgery (7 megaureters repairs, 2 extravesical repairs, 7 intravesical reimplants and 1 blind ureter), 11 had persistent reflux and neurogenic bladder, 7 had ectopic ureters to bladder neck, 6 had bilateral Hutch diverticulum, 6 had persistent stump reflux, 5 had ureterocele after puncture or incision, 15 had duplications, 1 had the prune belly syndrome, 2 had posterior urethral valve following resection, 1 had epispadias and 1 had urogenital sinus. The average maximum reflux grade was IV. An average of 1.1 cc (range 0.4 to 2) was injected per ureter. Of the patients 69 had 3-month followup results. The overall success rate was 68% after 1 implantation (47 of 69). CONCLUSIONS: Submucosal intraureteral implantation with Dx/HA corrected complex vesicoureteral reflux in 68% of patients. In all of these patients open surgery would have potentially been difficult. The use of fluoroscopy and/or guidewires is a useful adjunct in these cases. We believe that this minimally invasive approach is warranted as an initial step in the management of complex cases of VUR before resorting to more difficult open surgical procedures.

The modified sting procedure to correct vesicoureteral reflux: improved results with submucosal implantation within the intramural ureter.

PURPOSE: With the advent of tissue bulking agents, in particular dextranomer/hyaluronic acid copolymer (Dx/HA), for endoscopic implantation for vesicoureteral reflux (VUR), there has been a major shift in the surgical paradigm throughout Europe, and more recently, in the United States. We describe a modification of the technique used for implantation that has significantly improved our results. MATERIALS AND METHODS: Between October 2001 and October 2003, 285 children 7 months to 15 years old (mean age 4.6 years) underwent endoscopic implantation of Dx/HA for VUR at our institutions. A modified STING (subureteral transurethral injection) procedure (implantation submucosally within the intramural ureter) was introduced during the last year of the study. The average volume of injected material was measured for each ureter. Renal sonography was performed to determine if hydronephrosis was present. At 3 months flouroscopic voiding cystourethrograms were used to evaluate for the presence of VUR. A subset of 122 patients treated with STING (52) were compared to those treated with modified STING (70). RESULTS: A total of 459 ureters in 231 girls and 54 boys were treated (174 bilateral cases). Mean maximum grade per patient was 2.5/5. Mean injected volume was 0.9 cc ureter. There were 181 patients with at least 3 months of followup. After 1 treatment 76% (137 of 181) of cases were cured (grade 0 reflux), while 54% (24 of 44) of the failures were improved. The overall cure rate was 94% for grade I, 85% for grade II, 78% for grade III and 71% for grade IV reflux. The patients treated with STING had a mean age of 4.8 years, mean maximum reflux grade was 2.5 and success rate was 71% (37 of 52; 86% grade I, 89% grade II, 70% grade III and 63% grade IV reflux). The patients treated with a modified STING had a mean age of 5.5 years, mean maximum grade was 2.8 and a success rate was 89% (62 of 70; 100% grade I, 92% grade II, 91% grade III and 90% grade IV reflux). Ureteral success rates were significantly (p <0.01) greater for the modified STING (92%) vs the standard STING (79%). There were no cases of hydronephrosis at 3 months postoperatively. CONCLUSIONS: The majority of patients undergoing minimally invasive therapy for VUR with Dx/HA are cured after 1 treatment. The modified STING is our preferred method of implant injection for the correction of VUR and in our hands produces a resolution rate of 89% (92% of ureters). The technique optimizes ureteral coaptation, is easy to perform and is not associated with any significant short-term complications. Persistence of VUR in a minority of patients continues to be the only significant adverse effect of endoscopic implantation.

A prospective study comparing ultrasound, nuclear scintigraphy and dynamic contrast enhanced magnetic resonance imaging in the evaluation of hydronephrosis.

PURPOSE: A combination of nuclear medicine, ultrasound and voiding cystourethrography is typically used to investigate hydronephrosis in children. A major problem is the lack of an accepted gold standard to assess obstruction. In some cases the anatomy is unclear. By combining anatomical and functional information in one study, magnetic resonance imaging (MRI) has the potential of providing superior information regarding hydronephrosis. We evaluate the role of dynamic enhanced MRI and compare it to other imaging modalities in the investigation of pediatric hydronephrosis. MATERIALS AND METHODS: One hundred dynamic contrast enhanced MRIs were performed in 96 children (35 girls and 61 boys, mean age 4 years [range 1 month to 17 years]). The information from the various imaging modalities was compared. RESULTS: The MRI protocol was acceptable to all families and was performed without complications in all patients. The morphological imaging with MRI was superior to conventional imaging in all cases. The split renal function as calculated by nuclear and MRI scans was compared in 71 cases, and the correlation coefficient was r2 = 0.93. The combination of ultrasound and a nuclear medicine study correlated with the final diagnosis of the MRI in 50 of 64 studies (78%). Of the 14 studies that differed 8 were obstructed on MRI but not on the nuclear studies, and 5 were not obstructed on MRI but were obstructed on the nuclear studies. One patient believed to have ureteropelvic junction obstruction on nuclear medicine scan had ureterovesical junction obstruction on MRI. The final diagnoses by MRI were ureteropelvic junction obstruction in 26 children, primary ureterovesical junction obstruction in 14, dilated but not obstructed systems in 35, duplex systems in 13, multicystic dysplastic kidneys in 5, unilateral small scarred kidney in 1, acute pyelonephritis in 2, renal mass in 1, bilateral polycystic kidneys in 1 and normal study in 9. Sedation was administered safely without any complications. CONCLUSIONS: Dynamic contrast enhanced MRI provides equivalent information about renal function but superior information regarding morphology in a single study without ionizing radiation. MRI has the potential to replace the currently used combination of other imaging modalities in the investigation of hydronephrosis in children.

Minimally invasive treatment of vesicoureteral reflux with endoscopic injection of dextranomer/hyaluronic acid copolymer: the Children's Hospitals of Atlanta experience.

PURPOSE: Emerging data demonstrate that injection of dextranomer/hyaluronic acid (Dx/HA) copolymer (Deflux, Q-Med Scandinavia, Uppsala, Sweden) is a safe and effective treatment for most patients with vesicoureteral reflux (VUR). We sought to determine the efficacy and factors predictive of outcome in patients treated with Dx/HA. MATERIALS AND METHODS: A total of 180 children 7 months to 15 years old (mean age 4.6 years) underwent subureteral injection of Dx/HA for VUR between October 2001 and February 2003. Dx/HA was injected submucosally within or beneath the intramural ureter. The average volume of injected material was measured for each ureter. At 2 weeks and 3 months postoperatively bladder ultrasounds were performed to measure the volume of Dx/HA (mm3) in the trigone using the volume of an ellipsoid (V = 4/3pir(1)r(2)r(3)). Renal sonography was performed to determine whether hydronephrosis was present. At 3 months fluoroscopic voiding cystourethrograms were used to evaluate for the presence of VUR. RESULTS: A total of 292 ureters in 180 children were treated (112 bilateral cases). There were 141 girls and 39 boys. Mean maximum grade per patient was 2.6 (out of 5). Average injected volume per ureter was 0.83 +/- 0.03 ml (830 +/- 30 mm3). At 2 weeks the average measured volume was 663 +/- 70 mm3 (18% decrease from original volume), which decreased an additional 1% by 3 months to 656 +/- 103 mm3. There were no cases of hydronephrosis at up to 12 months postoperatively.There were 134 patients with at least 3 months of followup. After 1 treatment 72% (96) were cured (grade 0), while 55% of the failures (21 of 38) were improved. New contralateral VUR was seen in 6 patients (4.5%) who had neither a history of VUR nor an abnormal appearing ureteral orifice at cystoscopy. A lower success rate (60%) was seen in the first 20 patients compared with the last 20 patients (80%). The cure rate per grade was 90% for grade I, 82% for grade II, 73% for grade III and 65% for grade IV reflux. Local migration of material caudal to the ureteral orifice was seen in 61% of patients (11 of 18) at the time of reinjection of Dx/HA after initial treatment failure. There was no statistically significant difference in age, grade, volume injected, bilaterality or gender when successes were compared with failures. CONCLUSIONS: The majority of patients (72%) undergoing minimally invasive treatment of VUR with Dx/HA are cured after 1 treatment. Contralateral treatment of nonrefluxing ureters should be considered in view of the increased incidence of new reflux (4.5%) and absence of morbidity with Dx/HA injection. There is a definite learning curve with injection therapy. The location of injected material and experience with the technique appear to correlate with the outcome of the procedure.

Complete bladder, urethral, and vaginal duplication in a 50-year-old woman.

We present a rare case of a 50-year-old woman with complete bladder, urethral, and vaginal duplication without previous urogenital system reconstruction. Our patient demonstrated a rare embryologic defect with complete bladder, urethral, and vaginal duplication without any functional dysfunction. She was able to reach adulthood without reconstructive surgery. This case allowed the unique opportunity of evaluating these urogenital anomalies at a later point in life.

Metabolic risk factors for stone formation in patients with cystic fibrosis.

PURPOSE: Cystic fibrosis is characterized by chronic pulmonary disease, insufficient pancreatic and digestive function, and abnormal sweat concentrations. Patients with cystic fibrosis also have an increased incidence of nephrolithiasis. We compared the results of metabolic evaluation in patients with cystic fibrosis with and without nephrolithiasis. MATERIALS AND METHODS: A total of 496 patients were evaluated at our center, including 98 with a mean age of 25 years who had cystic fibrosis and complete metabolic evaluation available between 1996 and 2000. Of these patients 13 (13%) had a history of nephrolithiasis. The records were reviewed for clinical characteristics and all patients underwent metabolic evaluation, including serum electrolyte measurement and 24-hour urine collection. Statistical analysis was done to compare the stone versus nonstone groups. RESULTS: The incidence of nephrolithiasis in our study was 3%. We identified 13 patients 16 to 41 years old (mean age 27) with nephrolithiasis, of whom 62% had had multiple episodes. Flank pain was the presenting symptom in 9 of the 13 cases (69%). Renal ultrasound and computerized tomography were the most common imaging modalities. In 9 cases stones were passed without intervention, extracorporeal shock wave lithotripsy was required in 2 and ureteroscopy with stone extraction was done in 2. Calcium oxalate was the dominant stone composition in the 9 patients in whom stone analysis was performed. Metabolic evaluation of the stone versus no stone groups showed elevated urinary oxalate (45.5 versus 42.5 mg./24 hours), relative calcium oxalate supersaturation (5.3 versus 7.2) and decreased urinary citrate in the 2 groups. There was a statistical difference in citrate excretion with lower levels in stone formers (102 versus 218 mg./24 hours, p = 0.0007). CONCLUSIONS: Patients with cystic fibrosis have an increased incidence of nephrolithiasis and are at high risk for recurrence. Metabolic evaluation is indicated in all patients with cystic fibrosis and urolithiasis since most have hyperoxaluria, urinary calcium oxalate supersaturation and decreased urinary citrate. Correcting detected stone risk factors in these cases may decrease stone recurrence.