Revascularized Pyelo-Uretero-Cystoplasty in Patients with Chronic Bladder Outlet Obstruction Due to Ectopic Ureterocele: A Safe Surgical Technique with Superior Continence Outcomes.

PURPOSE: To present the outcomes of revascularized pyeloureterocystoplasty with ureterocele unroofing in end stage bladder patients with duplex system and ureterocele. METHODS: Thirteen patients with obstruction of intrauterine outlet from an ectopic obstructive ureterocele were included. Fourteen units of duplex systems underwent upper pole partial nephrectomy in conjunction with augmentation revascularized pyeloureterocystoplaty and ureterocele unroofing. The anterior and lateral walls of the ureterocele were excised before cystoplasty, and the resultant edges of the posterior wall were sutured to the bladder epithelium. Anastomosis of the upper pole vein and artery to the inferior iliac artery and the common iliac vein was performed. Detubularization of the whole ureter was performed with exception of the intramural ureteric part that kept tubularized for 'jet/turbulent' occurrence. Five patients (control group) underwent pyeloureterocystoplasty without revascularization. Patients underwent several evaluations in long-term follow-up. RESULTS: Patients were all dry by day and night in our long-term follow-up. Urinary incontinence improved in patients with no need for re-augmentation technique. Vesicoureteral reflux subsided in all patients postoperatively except one, who was asymptomatic. After five years, median bladder capacity rose from 128.5 ml to 395 ml and bladder compliance showed significant improvement from 15 ml/cm H2O to 29 ml/cm H2O, in experimental group and remained stable for 24-36 months. Median bladder capacity did not rise significantly in control group. CONCLUSION: Pyeloureterocystoplasty is an efficient choice in this type of patients, which may prevent the recurrence of hypocompliant bladders and prevent ischemia and subsequent fibrosis.

Correction to: Clinical and genetic analysis of two wolfram syndrome families with high occurrence of wolfram syndrome and diabetes type II: a case report.

Following publication of the original article [1], the authors flagged that the name of 'Asal Hojjat' was misspelled; the name had been spelled as 'Asal Hojat'.

Clinical and genetic analysis of two wolfram syndrome families with high occurrence of wolfram syndrome and diabetes type II: a case report.

BACKGROUND: Mutations of the WFS1 gene are responsible for most cases of Wolfram syndrome (WS), a rare, recessively inherited neurodegenerative disorder characterized by juvenile-onset non-autoimmune diabetes mellitus and optic atrophy. Variants of WFS1 are also associated with non-syndromic hearing loss and type-2 diabetes mellitus (T2DM). Our study adds to literature significant associations between WS and T2DM. CASE PRESENTATION: In this study, we analyzed the clinical and genetic data of two families with high prevalence of WS and T2DM. Genetic linkage analysis and DNA sequencing were exploited to identify pathogenic variants. One novel pathogenic variant (c.2243-2244insC) and one known pathogenic (c.1232_1233delCT) (frameshift) variant were identified in exon eight of WFS1 gene. CONCLUSIONS: The mutational and phenotypic spectrum of WS is broadened by our report of novel WFS1 mutation. Our results reveal the value of molecular analysis of WFS1 in the improvement of clinical diagnostics for WS. This study also confirms the role of WFS1 in T2DM.

Value of urinary carbohydrate antigen 19-9 to predict failure of conservative management in children with ureteropelvic junction obstruction.

OBJECTIVE: To assess the role of urinary carbohydrate antigen 19-9 (CA19-9) measurement in determining optimal management of ureteropelvic junction obstruction (UPJO) and predicting failure of conservative management. PATIENTS AND METHODS: Children with UPJO diagnosed between December 2012 and April 2015 were included. Depending on clinical and para-clinical findings, patients were divided into three groups: Group 1 consisted of patients who were considered for non-operative management with improvement of the condition during the course of follow-up. Group 2 were suitable for observation; however due to deterioration of condition pyeloplasty was indicated after a period of observation. Group 3 patients required immediate pyeloplasty. Urinary CA19-9 was measured in all patients at baseline and compared between the study groups. RESULTS: A total of 112 children (115 affected kidneys) with UPJO and mean age of 18.6 ±â€¯3.3 months were assessed. Group 1, 2, and 3 consisted of 54(48.2%), 24(21.4%), and 34(30.4%) patients, respectively. Mean baseline urinary CA19-9 was 37.83 ±â€¯5.20, 145.45 ±â€¯18.38 and 244.62 ±â€¯41.42 in groups 1, 2 and 3, respectively. Multivariate analysis showed that both CA19-9 and APD are independent predictors of need for surgery in patients on observation. ROC curve analysis revealed that urinary CA19-9 level at cut off value of 52.6 U/mL had sensitivity of 92.0% and specificity of 70.9% in predicting failure of non-operative management. CONCLUSION: Higher urinary CA19-9 level is associated with failure of non-operative management in patients with UPJO. Such patients may require close follow-up and assessments to prevent irreversible damage to the kidney. TYPE OF STUDY: Study of Diagnostic Test. LEVEL OF EVIDENCE: Level II.

Bladder Rupture as a Complication of Circumcision following Total Subcoronal Urethral Ligation.

Circumcision is one of the most commonly performed procedures and generally is considered a safe and minor surgery. However, serious and catastrophic complications may sometime occur if adequate attention is not paid to all aspects of this procedure. Bleeding is the most common early complication of circumcision and sometimes is controlled by applying sutures. We hereby report a case of urethral obstruction after deep suturing of the bleeding site performed at a district center that led to bladder rupture as an exceedingly rare complication following circumcision.

Comparison Characteristics of Family and Demographic of Children with Antenatal Hydronephrosis between 2nd and 3rd Trimesters of Gestation.

BACKGROUND: The aim of this study was comparison characteristics of family and demographics of children with antenatal hydronephrosis, in 2nd and 3rd trimester of pregnancy, in order to the need for postnatal management. METHODS: This cross-sectional study described some information from family of children with antenatal hydronephrosis, at the Pediatric Urology Research Center of Children's Hospital Medical Center of Tehran University of Medical Sciences. Data for 193 children, admitted in 2012-2013, were collected retrospectively. They were allocated to two groups of 2nd and 3rd trimester, based on the time of diagnosis antenatal hydronephrosis. Data analysis was done using SPSS. Chi-Square, Fisher-exact and independent t-test also Mantel-Hanzel test were used. P-value<0.05 was considered as statistically significant. RESULTS: Of 193 infants (36 female, 157male), the antenatal hydronephrosis of 76 cases (39.4%) have been diagnosed in the 2nd trimester. In addition, 110 cases (57%) were bilateral and 33 cases (17.1%) had severe antenatal hydronephrosis. Consanguinity marriage, being unilateral or bilateral and the severity of antenatal hydronephrosis were significant with the specific trimester that it has been diagnosed (P<0.05). CONCLUSION: Infants with bilateral and severe grade of antenatal hydronephrosis also with the history of consanguinity marriage among their parent will diagnose in the second trimester more than the third trimester and will refer for some surgical correction, relating to other kidney diseases such as vesicoureteral reflux, more than others. Thus, this is a good sign for caring infants.

Stone growth patterns and risk for surgery among children presenting with hypercalciuria, hypocitraturia and cystinuria as underlying metabolic causes of urolithiasis.

INTRODUCTION: Hypercalciuria, hypocitraturia and cystinuria are the most common underlying metabolic stone abnormalities in children. The present study compared stone growth patterns, stone burden, and the risk of stone-related surgery among these underlying metabolic conditions. METHODS: A retrospective cohort of 356 children with renal stones, followed from 2000 to 2015, was studied. Differences among metabolic groups were determined using Kruskal-Wallis test; the Scheffé-test was used for multiple comparisons to determine differences among single groups. Independent sample t-test was used when adequate, given the sample size, and Chi-squared test was used for categorical variables. Stone growth rates were calculated as differences in diameter divided by time elapsed between U/Ss (mm/year). Logistic regression was performed to assess the effect of initial stone size on the likelihood of surgery. RESULTS: Median stone size at presentation was significantly different among groups, with cystinuria being the group with the largest proportion of stones >10 mm, while patients with stones <5 mm were likely to have a normal metabolic workup (P < 0.05). Stones with a higher growth rate were found in the operative group, while slower growing stones were mostly managed conservatively (3.4 mm/year vs 0.8 mm/year, respectively; P = 0.014). However, stone growth rates were not significantly different among metabolic groups. On the other hand, the rate of new stone formation in cystinuric patients at their first follow-up was 30.4%, which was significantly higher than in patients with hypercalciuria (16.3%) or with a normal metabolic workup (17.2%; P < 0.05). Compared with stones <5 mm, stones measuring 5-10 mm were more than four times more likely to result in surgery, whereas the likelihood of surgery for 10-20 mm or >20 mm stones was almost 16 or 34 times, respectively (P < 0.001). CONCLUSIONS: It is believed that this is the first study to evaluate stone growth patterns, stone burden and surgical risk among children with hypercalciuria, hypocitraturia and cystinuria. Cystinuric patients presented with larger stones at the time of diagnosis, higher new stone formation rates, and were at higher risk of surgery. While no significant difference of growth rate was found among metabolic groups, stones with a higher growth rate were significantly more likely to result in surgical treatment than slower growing stones. Initial stone size, location of largest stone, previous urinary tract infection, and patient's metabolic type significantly influenced the likelihood of a surgical intervention. Better understanding of the natural history ultimately helps surgeons and clinicians defining prognosis, treatment, and prevention plans for pediatric urolithiasis.

Maternal Urinary Carbohydrate Antigen 19-9 as a Novel Biomarker for Evaluating Fetal Hydronephrosis: A Pilot Study.

OBJECTIVE: To evaluate maternal urinary CA19-9 as a potential marker to diagnose severe antenatal hydronephrosis (ANH) during pregnancy and to compare the values with those in normal pregnancies as controls. PATIENTS AND METHODS: A total of 20 women in their third pregnancy trimester were enrolled. An anteroposterior pelvic diameter (APD) of ≥15 was considered as severe ANH. Case group consisted of 10 women with a diagnosis of severe ANH. Ten women with similar age, gestational age, fetal sex, normal ultrasonography, and no history of any congenital anomalies were chosen as controls. Urine samples were collected and maternal urinary CA19-9 was measured. The levels in case and control groups were compared using Mann-Whitney U test. RESULTS: Each group consisted of nine mothers with male fetuses and one with female fetus. The APD in the ANH group ranged from 17 to 40 mm. Five of 10 children in the ANH group also had contralateral APD of ≥4 mm (bilateral ANH). The mean age and gestational age of pregnant women in the two groups were comparable. The mean maternal CA19-9 was significantly higher in the ANH group compared with the controls (mean: 134.5 U/mL vs 22.2 U/mL, P < .001). CONCLUSION: To our best knowledge, this is the first time that maternal urinary CA19-9 has been used as a marker for ANH. Based on these pilot data, CA19-9 levels are significantly higher in the urine of pregnant women carrying fetuses with severe ANH, and it may have the potential to serve as a noninvasive and useful biomarker to diagnose ANH.

The role of serum and urinary carbohydrate antigen 19-9 in predicting renal injury associated with ureteral stone.

This study was designed to evaluate the role of urinary and serum carbohydrate antigen 19-9 (CA19-9) as a biomarker in the assessment of patients with ureteral stone. A total of 38 patients with ureteral stone and hydronephrosis who underwent transurethral lithotripsy (TUL) (Group A) and 24 age-matched healthy controls (Group B) were evaluated in this study. Urinary and serum CA19-9 concentrations were measured in group A patients before TUL as well as 4 and 8 weeks following the operation. Urinary and serum CA19-9 concentrations were also measured in group B participants. Median concentration of urinary and serum CA19-9 was 34.0 and 15.0 kU/L in group A patients and 16.1 and 5.3 kU/L in group B, respectively (p < 0.001). Medians of CA19-9 concentration in urine and serum reduced to 12.5 and 4.5 kU/L 8 weeks after TUL (p < 0.001). Following successful TUL and hydronephrosis resolution, a significant decline was detected in serum and urinary CA19-9. We also noted that duration of ureteral obstruction was associated with serum and urinary CA19-9 concentrations, suggesting the potential role of this marker in predicting renal damage associated with urinary tract obstruction and determining the appropriate timing of interventions.

Intermittent voiding per urethra as an indicator of cutaneous vesicostomy malfunction.

PURPOSE: To present a new approach for management of cutaneous vesicostomy (CV) prolapse, with special emphasis on normal appearing vesicostomy may be malfunctioning. To introduce the application of temporary stoma-free drainage as a diagnostic and therapeutic tool. MATERIALS AND METHODS: From December 2000 to September 2006, 66 children (61 males and 5 females) with CV were studied. The mean age at vesicostomy was 7 months (range 1-30), and the main underlying disease was posterior urethral valves (in 45 children, 68%). Indications for CV included significant hydroureteronephrosis (HUN) and recurrent urinary tract infection. Patients were followed up for complications and were treated based on our institutional approach. All patients with persistent upper tract dilatation and micturition per urethra underwent temporary bladder (via stoma) free drainage. Patients with stomal stenosis were managed either by a revision surgery or by simple dilatation and intermittent catheterization. Purse string suturing was applied in mucosal prolapses as the first choice. RESULTS: The complications were observed in 21 patients (31%), including twelve stomal stenosis, nine severe mucosal prolapses, and two recurrent urinary infections. HUN and significant voiding per urethra persisted following initial CV in 19 out of 66 patients (29%), eleven of which having normal appearing CVs. Seventeen of these patients were managed by temporary stoma-free drainage (accompanied by purse string suturing in mucosal prolapse), and two patients with severe stenosis underwent surgical revision. Temporary stoma-free drainage improved HUN in 94% of patients (16 of 17). CONCLUSIONS: Voiding per urethra is an indicator of CV malfunction, and temporary stoma-free drainage can be a diagnostic and therapeutic option in such children. A seemingly open CV may still be malfunctioning, and ureterovesical or intravesical obstructions should be considered if HUN does not improve following temporary stoma-free drainage.

The timing of established detrusor hyperreflexia in a rat model of neuropathic bladder.

BACKGROUND: Proper timing of catheter insertion and the use of a suitable surgical method are essential parts of producing rat models to evaluate neuropathic bladder following spinal cord injury (SCI). METHODS: Thirty-two female Sprague-Dawley rats were randomly allocated into four groups. Group 1 underwent surgical laminectomy using the classic method. Group 2 underwent SCI 7 d following insertion of the catheter, and group 3 underwent sham operation. For bladder catheterization, a 4.5 Fr catheter was fixed into the bladder and tunneled beneath the skin to reach out at the nape of the neck. Group 4 underwent urodynamic study via bladder catheter prior to surgery and every 10 d following the operation to determine the exact time of establishing neuropathic bladder following spinal shock. The animals' survival rate and bladder wall's histopathologic changes were assessed 30 d following the operation. RESULTS: Simultaneous suprapubic catheter placement raised the mortality rate in group 1 in comparison with group 2. Repeated urodynamic study in group 4 showed hypertonic behavior in the bladder 10 d after SCI, with significantly increased leak point pressure and bladder capacity; however, the end filling pressure and constant neuropathic bladder on cystometric indices are attained from 20 d after the operation. CONCLUSIONS: Insertion of a bladder catheter 1 wk prior to SCI provides an applicable route for repeated cystometric studies in rats. The results demonstrate that sustained bladder overactivity is established in rats 20 d after SCI and animals are ready for further experiments on neuropathic bladder dysfunction following this period.

Teapot ureterocystoplasty and ureteral Mitrofanoff channel for bilateral megaureters: technical points and surgical results of neurogenic bladder.

PURPOSE: We present the long-term results of simultaneous "teapot" ureterocystoplasty and ureteral Mitrofanoff in patients with bilateral megaureters due to neurogenic bladder, and compare urodynamic results before and after the procedure. MATERIALS AND METHODS: We treated 13 children (mean age 7.3 years) with end stage neurogenic bladder and refluxing megaureters (mean diameter 5.5 cm) with simultaneous teapot ureterocystoplasty and Mitrofanoff appendicovesicostomy between April 1995 and May 2001. The larger ureter was used for teapot bladder augmentation while keeping its distal 2 cm tubularized. The Mitrofanoff channel was then created using the opposite ureter. RESULTS: Followup ranged from 109 to 169 months (median 121). At the end of the followup period all patients were dry with clean intermittent catheterization and/or voiding. No repeat augmentation was needed and there were no bladder calculi during followup. Median postoperative bladder capacity was 430 ml (IQR 380 to 477), which was increased significantly compared to preoperative evaluations (210 ml, IQR 181 to 230, p = 0.001). During followup bladder compliance also improved significantly (p = 0.001) and serum creatinine level decreased (p = 0.021). CONCLUSIONS: Although neurogenic bladder and high grade reflux are poor prognostic factors for ureterocystoplasty, the present modification resulted in enduring bladder augmentation with no calculus formation. Bladders remained compliant with good capacity, presumably because sufficient tissue and blood supply were provided for the augmented flap.