Long-term urological outcomes of spinal lipoma after prophylactic untethering in infancy: real-world outcomes by lipoma anatomy.

STUDY DESIGN: Cohort study. OBJECTIVES: Long-term urological outcomes in patients with spinal lipoma after prophylactic tethered cord release (TCR) in infancy were investigated. SETTING: Children's hospital in Yokohama, Japan. METHODS: Children under one year of age with spinal lipoma who underwent TCR between 1990 and 2010 were investigated. According to Arai's classification, lipomas other than filar lipoma were classified into four types: caudal, dorsal, transitional, and lipomyelomeningocele. The level of the conus medullaris was divided into three categories: L3-5, L5/S1, and sacral. Urological outcomes, including the need for clean intermittent catheterization (CIC), urinary incontinence, presence of renal deterioration, and the need for bladder augmentation, were investigated by both lipoma type and level of the conus medullaris. RESULTS: Fifty-three patients met the inclusion criteria. The median follow-up period was 14.2 years (interquartile range 9.6-17.6 years). Of the 53 patients, ten (19%) were on CIC, and six (11%) were incontinent. Overall, two patients (4%) had renal deterioration detected by DMSA renal scan, and two (4%) needed augmentation cystoplasty. Of the lipoma types, transitional type showed the worst outcomes with respect to need for CIC (54%) and urinary incontinence (38%). There were no significant differences in renal deterioration and the rate of bladder augmentation by lipoma type. No urological outcomes were significantly associated with conus level. CONCLUSIONS: Even after prophylactic TCR in infancy in children with spinal lipoma, 19% of patients needed CIC in long-term follow-up. Of the lipoma types, transitional type showed the worst outcomes with respect to need for CIC and urinary incontinence.

Treatment guidelines for persistent cloaca, cloacal exstrophy, and Mayer-Rokitansky-Küster-Häuser syndrome for the appropriate transitional care of patients.

We developed treatment guidelines (TGs) for appropriate transitional care of the genitourinary system in patients with persistent cloaca (PC), cloacal exstrophy (CE), or Mayer-Rokitansky-Küster-Häuser syndrome (MRKH). These TGs are in accordance with the Medical Information Network Distribution Service (Minds), published in 2014 in Japan. Clinical questions (CQs) concerning treatment outcomes of the genitourinary system, pregnancy and delivery, and quality of life in adulthood were prepared as six themes for PC and CE and five themes for MRKH. We were able to publish statements on chronic renal dysfunction, hydrometrocolpos, and pregnancy, based on four CQs about PC, four about CE, and two about MRKH, respectively. However, due to the paucity of proper manuscripts, we were unable to make conclusions about the correct timing and method of vaginoplasty for patients with PC, CE, and MRKH or the usefulness of early bladder closure for patients with CE. These TGs may help clarify the current treatments for PC, CE, and MRKH in childhood, which have been carried out on an institutional basis. To improve clinical outcomes, systematic clinical trials revealing comprehensive clinical data of the urinary and reproductive systems, especially the length of the common channel in PC, are essential.

Can Bladder Wall Thickness Predict Videourodynamic Findings in Children with Spina Bifida?

PURPOSE: We investigated whether bladder wall thickness measured at specifically defined bladder volumes could predict videourodynamic findings in children with spina bifida. MATERIALS AND METHODS: We prospectively investigated patients with spina bifida on intermittent catheterization who underwent ultrasound examination simultaneously with videourodynamics. We evaluated the association between bladder wall thickness measured at maximum cystometric capacity and parameters including age, maximum detrusor pressure during filling or at leak and bladder compliance. Differences in bladder wall thickness measured at each percent maximum cystometric capacity were compared between patients with and without unfavorable videourodynamic findings. Maximum detrusor pressure 40 cm H2O or greater during filling or at leak, bladder compliance less than 10 ml/cm H2O, detrusor overactivity, bladder trabeculation and vesicoureteral reflux were defined as unfavorable videourodynamic findings. RESULTS: A total of 23 males and 30 females with spina bifida (median age 7.8 years) underwent measurement of bladder wall thickness at maximum cystometric capacity. Mean ± SD bladder wall thickness measured at maximum cystometric capacity was 1.7 ± 0.5 mm. Only age had a weak correlation with bladder wall thickness measured at maximum cystometric capacity (p <0.05). In 31 patients bladder wall thickness was measured at each percent maximum cystometric capacity. Regarding unfavorable videourodynamic findings, there were no significant differences in bladder wall thickness measured at each percent maximum cystometric capacity, except for bladder trabeculation. CONCLUSIONS: Even if bladder wall thickness is measured at specifically defined bladder volumes, it cannot predict videourodynamic findings other than bladder trabeculation in children with spina bifida.

Expression of parathyroid hormone/parathyroid hormone-related peptide receptor 1 in normal and diseased bladder detrusor muscles: a clinico-pathological study.

BACKGROUND: To investigate the expression of parathyroid hormone (PTH)/PTH-related peptide (PTHrP) receptor 1 (PTH1R) in clinical specimens of normal and diseased bladders. PTHrP is a unique stretch-induced endogenous detrusor relaxant that functions via PTH1R. We hypothesized that suppression of this axis could be involved in the pathogenesis of bladder disease. METHODS: PTH1R expression in clinical samples was examined by immunohistochemistry. Normal kidney tissue from a patient with renal cancer and bladder specimens from patients undergoing ureteral reimplantation for vesicoureteral reflux or partial cystectomy for urachal cyst were examined as normal control organs. These were compared with 13 diseased bladder specimens from patients undergoing bladder augmentation. The augmentation patients ranged from 8 to 31 years old (median 15 years), including 9 males and 4 females. Seven patients had spinal disorders, 3 had posterior urethral valves and 3 non-neurogenic neurogenic bladders (Hinman syndrome). RESULTS: Renal tubules, detrusor muscle and blood vessels in normal control bladders stained positive for PTH1R. According to preoperative urodynamic studies of augmentation patients, the median percent bladder capacity compared with the age-standard was 43.6% (range 1.5-86.6%), median intravesical pressure at maximal capacity was 30 cmH2O (range 10-107 cmH2O), and median compliance was 3.93 ml/cmH2O (range 0.05-30.3 ml/cmH2O). Detrusor overactivity was observed in five cases (38.5%). All augmented bladders showed negative stainings in PTH1R expression in the detrusor tissue, but positive staining of blood vessels in majority of the cases. CONCLUSIONS: Downregulation of PTH1R may be involved in the pathogenesis of human end-stage bladder disease requiring augmentation.

Vesicoscopic ureteral reimplantation with a modified Glenn-Anderson technique for vesicoureteral reflux.

INTRODUCTION: Vesicoureteral reflux (VUR), commonly referred to as urinary reflux, is one of the most common congenital urological anomaly to occur during childhood. Historically, open vesicoureteral reimplantation has been the gold standard in the surgical treatment of VUR. Currently, vesicoscopic ureteral reimplantation is used as a minimally invasive alternative to open ureteral reimplantation. Most vesicoscopic procedures are performed using the transtrigonal Cohen technique. As a non-transtrigonal technique, the vesicoscopic Politano-Leadbetter technique is also performed, but requires dissection outside the bladder under a narrow surgical field, and in boy carries a risk of vas deferens injury. OBJECTIVE: This study evaluated surgical outcomes and perioperative findings for vesicoscopic ureteral reimplantation using a modified Glenn-Anderson technique in children with VUR. STUDY DESIGN: Eighteen consecutive children who underwent vesicoureteral ureteral reimplantation using a modified Glenn-Anderson technique were included in this study. The surgical procedure was explained and surgical outcomes and perioperative findings were evaluated. RESULTS: Patients comprised 9 boys and 9 girls with 29 cases of ureteral reflux (7 unilateral cases, 11 bilateral cases). All procedures were performed laparoscopically, with no cases requiring conversion to open surgery. Median operative time was 143 min for unilateral VUR and 194 min for bilateral VUR. Only one case showed a complication (Clavien-Dindo grade 1). The remaining 17 cases showed no complications, with removal of the urethral catheter and discharge 2 days postoperatively. Seventeen of the 18 cases underwent postoperative voiding cystourethrography, showing no VUR in all cases. DISCUSSION: Vesicoscopic ureteral reimplantation is a minimally invasive alternative to open surgery, with most procedures performed using a transtrigonal Cohen technique. Regardless of whether the surgery is open or laparoscopic, a disadvantage of the Cohen technique is that postoperative transureteral treatment may not be possible. The Politano-Leadbetter technique has been reported as a non-transtrigonal technique. However, this requires dissection outside the bladder under a narrow surgical field, and carries a risk of vas deferens injury in boys. In this study, vesicoscopic ureteral reimplantation using a modified Glenn-Anderson technique provided good surgical outcomes with minimal perioperative complications and easy manipulation under a wide field of view. CONCLUSION: Although many minimally invasive treatments are available, vesicoscopic ureteral reimplantation using a modified Glenn-Anderson technique is safe and effective for patients with VUR 4 years old. To demonstrate the further utility of this procedure, long-term outcomes and safety evaluations are needed in a larger number of cases.

The modified Belt-Fuqua procedure using an asymmetric "furisode" sleeve for proximal hypospadias with severe curvature.

INTRODUCTION AND OBJECTIVES: Proximal hypospadias repair is a challenge in the pediatric urology field. Although the previous reports showed that the Belt-Fuqua staged procedure is reliable with a low complication rate for midshaft hypospadias, this procedure has not been generally applied to more proximal hypospadias with severe ventral curvature due to technical limitations. To solve these technical limitations, we developed a modified Belt-Fuqua procedure using an asymmetric long skin sleeve looking like a Japanese long-sleeved Kimono called a "furisode". The aim of this study was to evaluate the outcomes of this new modification of the Belt-Fuqua procedure for the repair of proximal hypospadias with severe curvature in children. STUDY DESIGN: The study retrospectively reviewed consecutive patients with proximal hypospadias with severe curvature underwent this new technique. The major modifications included are proximal degloving beyond the urethral meatus, asymmetrical ventral transposition of dorsal preputial skin like a furisode sleeve, and circumferential proximal anastomosis of preputial skin to native meatus in the first stage. Hypospadias severity was evaluated objectively in two ways: preoperatively by the Glans-Urethral Meatus-Shaft score and intraoperative direct measurement of ventral curvature. The primary outcome was urethroplasty complications. RESULTS: A total of 66 patients completed both stages of the furisode technique. The median Glans-Urethral Meatus-Shaft score was 11. Overall, 60 (91%) patients showed ventral curvature greater than 60 degrees after degloving, and 35 (53%) underwent ventral grafting with a dermal graft in the first stage. Median urethral length constructed at the second stage was 41.5 mm. The median follow-up period was 25 months. Complications occurred in 9 patients (14%); 7 had urethral diverticula, 1 showed a fistula and 1 had a urethral stricture. Neither glans dehiscence nor meatal stenosis occurred. DISCUSSION: There are a couple of advantages of this furisode technique to other tunneled flap techniques like the Ulaanbaatar procedure. The first is the ease of glans reshaping with a Firlit collar at the first stage because of no urethra in the glans. The second is that a long neourethra could be made by the same skin flap with one anastomosis to the native urethra.The weakness of this procedure was that urethral diverticula were prone to occur less than one year after urethroplasty. CONCLUSION: The furisode technique, a new modification of the Belt-Fuqua procedure, provides an alternative staged repair of proximal hypospadias. This technique was easily applied for hypospadias with severe curvature requiring ventral grafting.

A case of large bladder hemangioma successfully treated with endoscopic yttrium aluminium garnet laser irradiation.

Bladder hemangioma is a rare benign tumor. While partial cystectomy remains an effective treatment option for large bladder hemangioma, the combined method of neodymium:yttrium aluminium garnet and holmium:yttrium aluminium garnet laser irradiation is an effective and less invasive treatment option. We report a case of large bladder hemangioma in an otherwise healthy 4-year-old boy, successfully treated with serial endoscopic yttrium aluminium garnet laser irradiation. There was no recurrence, and normal bladder function was preserved. Serial yttrium aluminium garnet laser irradiation is thus a useful, less invasive method for cases of large tumors.

Application of response surface method using rapid screening, support vector machine, and multiple regression on the acidity and activity of Si-Al-Zr ternary oxide.

To study the synergistic effect on the acidity and activity of Si-Al-Zr ternary oxide system, the mixture design of experiments was applied to prepare the ternary oxides by a sol-gel method, and rapid screening on dehydration of isopropanol and Friedel-Crafts reaction was conducted to determine the activities. These activities, amount of acid site determined by pyridine pulse, and specific surface area, were correlated with the oxide composition by means of support vector machine. Clear synergy among the Si-Al-Zr was observed on the acidic character and the surface area, whereas no synergy between Al-Zr was observed on the catalytic activities. Multiple regression was then conducted to find a relationship between the activities and the characters. The term of Si molar fraction in the oxide was essential for good regression, and acid strength determined by peak temperature of NH(3) desorption was related to the Si fraction. Thus, simple and rapid technique for activity test and characterization can be integrated by means of support vector machine and multiple regression, and some insights of the active site were obtained.

The role of the retrograde colonic enema in children with spina bifida: is it inferior to the antegrade continence enema?

OBJECTIVES: The aim of this study was to evaluate the efficacy of the retrograde colonic enema relative to the Malone antegrade continence enema. METHODS: We retrospectively investigated 25 children with spina bifida and fecal incontinence. Thirteen children had started retrograde colonic enema and twelve had started Malone antegrade continence enema. Fecal continence, water volume, time to washout, procedure frequency, pain during procedure, performance independence and demographical data were compared between the two groups. RESULTS: Fecal continence was achieved for 10 of 13 (76.9%) in the retrograde group and 9 of 12 (75.0%) in the antegrade group. In the antegrade group 8 of 12 (66.7%) performed procedure independently, while 3 of 13 (23.1%) did so in the retrograde group. Achievement of fecal continence did not differ between the groups, but procedure independence was significantly better in the antegrade group. CONCLUSIONS: Our results suggest that retrograde colonic enema was not inferior to Malone antegrade continence enema on fecal continence. We recommend considering retrograde colonic enema prior to introduction of Malone antegrade continence enema in children with spina bifida.

[Dextranomer beads in stabilized non-animal sodium hyaluronate gel (NASHA/Dx gel) for vesicoureteral reflux: multi-center study in Japanease patients].

PURPOSE: To investigate the efficacy and safety of endoscopic treatment with the injectable gel of dextranomer beads in stabilized non-animal sodium hyaluronate (NASHA/Dx gel) administered submucosally close to the proximity of ureteral orifice, we performed the multi-center open study of Japanese patients with vesicoureteral reflux (VUR). We herein report the results of the study. SUBJECTS AND METHODS: Patients aged > or = 1 year with grade II-IV VUR underwent endoscopic injection with NASHA/Dx gel. Post-treatment assessment was done by voiding cystourethrography (VCUG) at 3 and 12 months. Patients with VUR grade II-IV at 3 months underwent re-treatment, with VCUG assessment 3 and 12 months after retreatment. Positive response to treatment was defined as reflux grade 0 or 1. RESULTS: The initial treatment was conducted to 116 ureters in 73 patients. The per-protocol efficacy population included 97 ureters in 71 patients. On a per-ureter basis, the positive response rate at 12 months after the last endoscopic treatment was 69.1%, compared with 62.0% on a per-patient basis. Improvement in reflux grade was shown to be statistically significant at both 3 months post initial treatment and 12 months post last treatment. Positive response rate decreased with increasing baseline reflux grade. There were only two mild adverse events (AEs) and one moderate laboratory fluctuation which were potentially relating to NASHA/Dx gel. CONCLUSIONS: This study has shown that endoscopic injection of NASHA/Dx gel is effective and well tolerated in Japanese patients with VUR. First-line use of this treatment for VUR could potentially be considered for Japan also.

A simple screening tool for an unfavorable bladder in children with myelomeningocele: Is the height to width ratio of the cystogram useful to predict high-pressure bladder?

INTRODUCTION: Videourodynamics is the key follow-up examination of children with myelomeningocele. However, it has been performed in specific institutions focused on the urological management of children due to the difficulty in its interpretation. Although a neurogenic bladder frequently appears elongated vertically and trabeculated, no objective study has clearly shown the relationship between bladder shape on the cystogram and urodynamic parameters in children with myelomeningocele. OBJECTIVES: The aim of this study was to investigate the usefulness of the height to width ratio of cystogram (HWR) as a screening tool for finding high-pressure bladder in children with myelomeningocele.Study design the medical records of children with myelomeningocele aged less than 13 years who underwent videourodynamics were reviewed. Maximum detrusor pressure (MDP) was defined as the maximum detrusor pressure at end-filling or at leak. HWR was calculated by the maximum height/maximum width of the cystogram appearance at maximum cystometric capacity (Figure) The children were categorized into two groups: children with high-pressure bladder (MDP≥40 cmH2O) and low-pressure bladder (MDP<40 cm H2O). Age, sex, videourodynamics variables, and HWR were compared between the 2 groups. Using the results of the above, receiver-operating characteristic (ROC) curves were constructed. RESULTS: A total of 81 children who were on clean intermittent catheterization met the study criteria. All children were on CIC, and their median age was 81 months (IQR 54-128 months). The HWR was significantly higher for high-pressure bladders than for low-pressure bladders (median 1.50 vs 1.37, p = 0.004). The sensitivity and specificity of the HWR for discriminating children with high-pressure bladder from all children were 87% and 56.9%, respectively. The area under the curve (AUC) was 0.71 with a cutoff score of 1.40. DISCUSSION: Bladder deformity was objectively evaluated by the HWR, and measurement of the HWR was straightforward. The main drawback of this evaluation method for bladder shape is to disregard the presence or absence of bladder trabeculation, which has been considered a key finding of an unfavorable bladder in this population. Based on the HWR ROC curves, the AUC was 0.71, which meant that the HWR would be considered to be fair at screening for high-pressure bladder. CONCLUSION: The height to width ratio of the cystogram was a useful tool for objectively evaluating bladder shape in children with myelomeningocele, and a cut-off point of 1.40 could be used as a simple screening tool for high-pressure bladder in this population.

The significance of 99mtechnetium dimercapto-succinic acid renal scan in children with spina bifida during long-term followup.

PURPOSE: (99m)Technetium dimercapto-succinic acid renal scans are ideal for demonstrating renal scarring in children with spina bifida. However, doubt persists about the need for routine application. We assessed the associations among abnormal (99m)technetium dimercapto-succinic acid renal scans, vesicoureteral reflux and urodynamic findings in patients with spina bifida during long-term followup. MATERIALS AND METHODS: We retrospectively reviewed the records of 64 patients with spina bifida followed at our center. All patients were older than 10 years (mean 15.8, range 10 to 23). Dimercapto-succinic acid renal scans were considered abnormal with differential function of less than 40% or focal defects. Patient age, gender, previous febrile urinary tract infections, positive vesicoureteral reflux history, timing of clean intermittent catheterization initiation and the latest urodynamic findings were noted. Patients were grouped based on normal/abnormal scan results. Statistical analysis included univariate and multivariate regression analyses and chi-square tests. RESULTS: A total of 16 patients (25%) had abnormal scans. Mean patient age, male-to-female ratio, leak point pressure, bladder compliance and timing of clean intermittent catheterization initiation did not differ between groups. Rates of previous febrile urinary tract infections differed significantly (11 of 16 in the abnormal group vs 9 of 48 in the normal group, p <0.01), as did positive reflux history (100% vs 31%, p <0.01). No patient with a negative reflux history had an abnormal dimercapto-succinic acid renal scan. Multivariate analysis identified previous febrile urinary tract infections as a significant risk factor for an abnormal scan. CONCLUSIONS: A positive vesicoureteral reflux history and febrile urinary tract infections were associated with abnormal dimercapto-succinic acid renal scan in followup of patients older than 10 years with spina bifida. Thus, these factors are indicators of proactive evaluation of renal function using dimercapto-succinic acid renal scanning.

Predicting early recurrent urinary tract infection in pretoilet trained children with vesicoureteral reflux.

PURPOSE: We identified independent factors predicting recurrent urinary tract infection within 1 year after the first urinary tract infection in pretoilet trained children with vesicoureteral reflux. MATERIALS AND METHODS: We retrospectively reviewed the records of infants younger than 2 years with primary vesicoureteral reflux and a history of febrile urinary tract infection. Patients were divided into 2 groups based on the presence or absence of recurrent febrile urinary tract infection. Analysis included age, gender, reflux laterality and grade, abnormalities on dimercapto-succinic acid renal scan and prophylactic antibiotic type. Univariate and multivariate analyses were performed to identify risk factors for recurrent febrile urinary tract infection. RESULTS: From 2004 to 2007, 78 children met study inclusion criteria. Mean age at the first urinary tract infection was 4 months (range 1 week to 16 months). None of the males were circumcised. Of 78 children 25 (32%) had a recurrent febrile urinary tract infection during 1 year of followup. Univariate analysis showed that bilateral reflux, high grade reflux (IV-V) and abnormal dimercapto-succinic acid scan were statistically significant predictors of early recurrent urinary tract infection (p <0.05). However, on multivariate analysis only an abnormal dimercapto-succinic acid scan showed a significant association with early recurrent urinary tract infection (OR 8.01, 95% CI 2.10-30.51, p = 0.002). CONCLUSIONS: Abnormal dimercapto-succinic acid renal scan is an important predictor of early recurrent urinary tract infection in pretoilet trained children with vesicoureteral reflux. Whether the explanation lies in congenital or infection related damage, in this patient subgroup careful clinical followup or early surgical management for reflux should be considered.

Catalyst screening for oxidative reforming of methane in direct route using high pressure HTS reactor with syngas detection system by colorimetric reaction and gas chromatograph.

A high-throughput screening (HTS) reactor for high-pressure oxidative reforming of methane in a direct reaction route was developed. With a combination of catalyst preparation by a split-and-pool method and HTS, Ni-K/alpha-Al(2)O(3) catalyst was found to show high activity under 1 MPa at 650 degrees C with high selectivity even when O(2) conversion is less than 100%. The HTS reactor required a new simple syngas detector operable under high pressure because the number of parallel reactor is limited when equipped with the conventional detection system. The complexity of the pressure reducing unit is the main reason of the limitation. Reduction of metal oxide accompanied with the color change was applied to the detection system. Copper oxide was supported on the filter disk made of alumina, and the filter was placed underneath the catalyst bed. After the methane was oxidatively reformed under 1 MPa at 650 degrees C, color change of spots from dark brown to light brown was observed just under the catalyst which produced hydrogen. Color change of the disk can be used to detect hydrogen formation from the reforming catalyst under pressure.

Scrotal nubbins associated with blind-ending spermatic vessels and a normal vas deferens on laparoscopy.

OBJECTIVES: To determine whether a scrotal nubbin is present in children with unilateral non-palpable testis when diagnostic laparoscopy demonstrates blind-ending vessels and a normal vas deferens entering a closed internal ring. METHODS: Eighty consecutive patients with a unilateral nonpalpable testis were retrospectively reviewed. Patients underwent initial diagnostic laparoscopy, and, if needed, subsequent inguinal exploration was performed. On inguinal exploration, any testicular remnant or nubbin-like tissue was removed and evaluated histologically. Patients with a patent processus vaginalis were excluded from this analysis. RESULTS: Overall, 60 of the 80 patients had neither an abdominal testis nor a patent processus vaginalis. Of these 60, 34 patients had both a vas deferens and spermatic vessels entering a closed internal ring, and all of these underwent inguinal exploration. A total of 17 patients had both a blind-ending vas deferens and blind-ending spermatic vessels; no inguinal exploration was attempted. In nine patients, laparoscopy revealed blind-ending vessels with a normal vas deferens entering the closed internal ring. Of these nine, six underwent inguinal exploration, and a scrotal nubbins was found in three. At histological examination, hemosiderin deposit and calcification were seen in the nubbin tissue. No viable germ cell was detected in these specimens. CONCLUSIONS: A laparoscopic finding of blind-ending vessels above the closed internal ring does not mean intra-abdominal vanished testis, regardless of the appearance of the vas deferens.

Severe in utero under-virilization in a 46,XY patient with Silver-Russell syndrome with 11p15 loss of methylation.

Background Silver-Russell syndrome (SRS) is characterized by growth retardation and variable features including macrocephaly, body asymmetry, and genital manifestations such as cryptorchidism in 46,XY patients. Case presentation The patient was born at 39 weeks with a birth weight of 1344 g. Subtle clitoromegaly warranted a thorough evaluation, which disclosed 46,XY karyotype, bilateral undescended testes, and a rudimentary uterus. Because of severe under-virilization, the patient was assigned as female. Failure to thrive, macrocephaly, and body asymmetry led to the diagnosis of SRS, confirmed by marked hypomethylation of H19/IGF2 intergenic differentially methylated region (IG-DMR). From age 9 years, progressive virilization occurred, which necessitated luteinizing hormone-releasing hormone analog (LHRHa) treatment. Gonadal resection at 15 years revealed immature testes with mostly Sertoli-cell-only tubules. Panel analysis for 46,XY-differences of sex development (DSD) failed to detect any pathogenic variants. Conclusions This is the second reported case of molecularly proven 46,XY SRS accompanied by severe under-virilization. SRS should be included in the differential diagnosis of 46,XY-DSD.

Age-related changes in urinary flow following dorsal inlay graft urethroplasty for hypospadias in early childhood: Potential improvement over 11 years of age.

INTRODUCTION: One of the goals of hypospadias repair is to create a neourethra with normal urinary stream and normal growth. Several studies have reported that dorsal inlay graft urethroplasty (DIG) has wide indications for various clinical phenotypes of hypospadias, with good short-term outcomes and few complications. However, there have been no reports that evaluated both short-term and long-term functional outcomes using uroflowmetry in patients with DIG. OBJECTIVE: The aim was to investigate whether uroflowmetry parameters change with time following DIG for hypospadias in early childhood. MATERIALS AND METHODS: Uroflowmetry parameters after DIG for hypospadias in childhood were retrospectively evaluated and compared between two defined ages at follow-up: 4-6 years and 11-13 years. Maximum urinary flow (Qmax) under the 25th percentile on the Miskolc nomogram was defined as obstruction. To evaluate the shapes of uroflow quantitatively, the flow index (FI) was used. The FI cut-offs for the flow shapes were defined as tower >1.253, plateaus <0.659; bells were between these two values. RESULTS: Thirty-four patients met the inclusion criteria. The median operative age was 1.8 years (range 1.0-4.9 years). The median follow-up time was 10.3 years (range 7.2-12.3 years). The mean ± SD Qmax at the two follow-up times increased with time, from 9.2 ± 3.7 to 18.8 ± 7.8. The mean ± SD FI changed from 0.53 ± 0.19 to 0.85 ± 0.31. Both Qmax and the FI were significantly improved (p < 0.001, p < 0.001, respectively). There were significantly fewer patients with obstruction evaluated by the Miskolc nomogram at 11-13 years of age (n = 11, 32.4%) than at 4-6 years (n = 31, 91.2%) (p < 0.001). Plateau shapes assessed by the FI were seen in 24 (70.6%) patients at 4-6 years and 10 (29.4%) patients at 11-13 years (Table). The number of patients with plateau shapes was significantly decreased (p = 0.001). DISCUSSION: Only one report noted long-term outcomes and spontaneous uroflowmetry resolution after tubularized incised plate urethroplasty. The current report is the first to present the short-term and long-term uroflowmetry outcomes after DIG. In addition, the FI was used for the first time to evaluate uroflowmetry after hypospadias repair. It was found that the uroflowmetry parameters improved spontaneously over 11 years of age following DIG for hypospadias in early childhood. CONCLUSION: Uroflowmetry parameters improved spontaneously over 11 years of age following DIG for hypospadias in early childhood.

Renal scarring on DMSA scan is associated with hypertension and decreased estimated glomerular filtration rate in spina bifida patients in the age of transition to adulthood.

OBJECTIVE: Improved management for spina bifida (SB) has increased the number of patients transitioning to adult care. This trend increases the importance of maintaining renal function concurrently with bladder function in patients with SB. Dimercaptosuccinic acid (DMSA) renal scanning is an optimal tool for investigating renal insufficiency in children with SB; however, the benefits of DMSA scans in adulthood are unclear. The role of DMSA renal scans for patients with SB during the transition to adulthood (15-25 years of age) to reveal their association with current renal function was investigated. MATERIALS AND METHODS: DMSA renal scanning was routinely performed patients with SB aged 15-25 years concurrently with examination of serum creatinine, serum cystatin C, urinalysis, and blood pressure between January 2006 and August 2016. Hypertension was defined as systolic or diastolic pressure above the age-specific normal range. The estimated glomerular filtration rate (eGFR) using serum creatinine or cystatin C was calculated; decreased eGFR was defined as eGFR below 90 mL/min/1.73 m2. Patients were divided into two groups according to the absence or presence of renal scarring on DMSA scan. Factors associated with renal function, including hypertension, decreased eGFR, and proteinuria, were compared between groups. RESULTS: Eighty-seven patients (36 males and 51 females) were analyzed. Median age was 19 years (range 15-24 years); 28 patients (32%) had renal scarring. Patients with renal scarring had significantly higher rates of hypertension (n = 13, 46%) and decreased eGFR (n = 5, 18%). However, there was no difference in proteinuria between the groups (Table). The group with renal scarring had significantly lower eGFR. DISCUSSION: This study showed that DMSA scans in patients with SB aged between 15 and 25 years were useful for assessing renal scarring despite a history of febrile urinary tract infection. DMSA scans could be performed in all patients with SB in the transition to adulthood to detect renal scarring. This study also showed that renal scarring was associated with hypertension and decreased eGFR in this age group. Treatment with antihypertensive drugs should be considered for patients with SB with renal scarring in this age range to protect renal function. CONCLUSION: Spina bifida patients in the age of transition to adulthood with renal scarring already showed signs of chronic kidney disease, suggesting that DMSA renal scans could be useful to identify patients who require close monitoring of renal function.

Retroperitoneoscopic assisted single-site pyeloplasty using EZ access in children: Comparison with open dismembered pyeloplasty.

PURPOSE: The aim of this study was to compare the results of the retroperitoneoscopic assisted pyeloplasty (RASP) using EZ access (silicone rubber cap) with open dismembered pyeloplasty (ODP) in children. METHODS: A retrospective review was performed of patients treated for ureteropelvic junction (UPJ) obstruction with either RASP or ODP from 2010 to 2015. For patients with RASP, two 5-mm trocars were placed in the EZ access. The UPJ was dissected retroperitoneoscopically and dismembered pyeloplasty was performed extracorporeally. Patient demographics and operative outcomes were compared between the groups. RESULTS: A total of 50 children were included, with 25 RASP and 25 ODP. Mean patient age was 49months in the RASP group and 53months in the ODP group. Perioperative outcomes, including operative time (185 vs 188min) and postoperative hospital stay (2.0 vs 2.2days), were similar between the two groups. Mean skin scar length (17 vs 34mm) was significantly smaller in the RASP group. The postoperative success rate (96% vs 100%) was not significantly different between the groups. CONCLUSIONS: The RASP represents a safe and effective single-site procedure in children. This procedure significantly reduces the skin scar length and has equivalent surgical outcomes to ODP. THE TYPE OF STUDY: Retrospective comparative study. LEVELS OF EVIDENCE: III.

Prevention of ventriculoperitoneal shunt complications after intraperitoneal urological surgeries.

PURPOSE: To evaluate perioperative management for the prevention of postoperative shunt infection and malfunction after intraperitoneal urological surgery in patients with myelodysplasia and a ventriculoperitoneal shunt. METHODS: From 2005 to 2015, 20 consecutive patients with myelodysplasia and a ventriculoperitoneal shunt who underwent intraperitoneal urological surgeries were managed with the same perioperative regimen. Intraperitoneal surgeries involved opening gastrointestinal tracts, including bladder augmentation by enterocystoplasty, creating continent catheterizable channels and Malone antegrade continent enema. We compared results with those from seven previous reports regarding postoperative shunt complications, surgical histories of previous shunt revisions, management of bacteriuria before surgery preoperative bowel preparation, antibiotic regimens, and duration of indwelling drain. RESULTS: Of 20 patients, 18 received prior shunt revisions, and 14 had positive urine culture before surgery that was managed with oral antibiotics. Thirteen patients underwent bladder augmentation with ileum, and one underwent augmentation with sigmoid colon. Nineteen patients underwent Malone antegrade continent enema using the appendix. All parenteral antibiotics were stopped on postoperative day 2.5. Mean duration of indwelling peritoneal drain was 2.7days. Mean follow-up period was 59.8months. Neither postoperative shunt infections nor intraperitoneal shunt malfunctions were recognized during follow-up period. CONCLUSIONS: This is the first study to evaluate postoperative ventriculoperitoneal shunt complications in patients with myelodysplasia who underwent intraperitoneal urological surgeries with a specific perioperative regimen. Shunt complications are greatly reduced by rigorous perioperative management, including preoperative control of bacteriuria, appropriate administration of prophylactic antibiotics, and early removal of intraperitoneal drains. LEVELS OF EVIDENCE: The type of study: Case series with no comparison group, IV.