Orchio-Septopexy: A new technique to cover and fix detorsed testis undergoing fasciotomy of tunica albuginea.

PURPOSE: Compartment Syndrome (CS) has been recognized as a potential factor that worsens testicular viability after detorsion, especially in borderline cases of prolonged ischemia. Fasciotomy of the testicular tunica albuginea to relieve the pressure associated with CS has been proposed to accommodate edema after detorsion, embracing the raw fasciotomy area with tunica vaginalis flap (TVF) or graft. Fashioning the TVF can be tedious in cases of severe scrotal edema. Herein we present a technique that facilitates and expedites the procedure, maintaining the fasciotomy area decompressed. MATERIALS AND METHODS: In testicular torsion, where the testis remains with dark coloration and questionable viability after detorsion a longitudinal releasing incision is made in the tunica albuginea (fasciotomy) to decrease compartmental pressure. If signs of parenchymal recovery (bleeding points, better color) are seen an orchio-septopexy is performed, suturing the incised albuginea's edges to the septum with a running suture, avoiding CS as well as re-torsion. RESULTS: Orchio-septopexy was performed in 11 cases with a mean age of 11.9 years (3-17). All cases had clinic follow-up and testicular Doppler US with a mean of 9.5 months (6-24). 6/11 cases (54%) were salvaged, with good vascularity in the Doppler US and maintained more than 50% testicular volume compared to the contralateral side. CONCLUSION: Orchio-septopexy after testicular fasciotomy is a simple and fast technique that can be utilized in cases of prolonged testicular ischemia and questionable viability. More than half of the testes recovered, encouraging us to propose its utilization as well as its validation by other surgeons.

Measurement of Differential Renal Function by Scintigraphy in Hydronephrotic Kidneys: Importance of Conjugate Views for Accurate Evaluation.

PURPOSE: Traditionally a single posterior view is used to measure differential renal function during nuclear renal scintigraphy. Nevertheless, experimental data show important variation in this measurement in the setting of significant hydronephrosis. To date, the impact of degree of hydronephrosis on the accuracy of differential renal function determination has not been addressed. We evaluated the discrepancy between function measured by anterior and posterior views, and the relationship to varying degrees of hydronephrosis. MATERIALS AND METHODS: We retrospectively reviewed consecutive mercaptoacetyltriglycine renal scans from 2009 to 2011. Ultrasounds were reviewed and degree of hydronephrosis was recorded using anteroposterior pelvic diameter. Absolute percent difference in differential renal function between each view (anterior minus posterior) was calculated and correlated to anteroposterior pelvic diameter. Patients were stratified into 4 groups according to anteroposterior pelvic diameter, ie less than 10 mm, greater than 10 mm, greater than 15 mm and greater than 25 mm. RESULTS: A total of 519 scans with corresponding ultrasounds were analyzed. Median patient age was 2.26 years. Kidneys with a larger anteroposterior pelvic diameter had a greater discrepancy in function on anterior and posterior views. There was a meaningful discrepancy for anteroposterior pelvic diameter greater than 10 mm (p = 0.034) and greater than 25 mm (p = 0.032). Several statistical models were used to identify a meaningful cut point of 15 mm, where the discrepancy in anterior and posterior views became significant (p = 0.001). CONCLUSIONS: The use of single views during nuclear renography for grossly hydronephrotic kidneys is often inaccurate. The discrepancy in differential renal function obtained on the anterior and posterior views is adversely impacted especially as anteroposterior pelvic diameter increases beyond 15 mm. Therefore, we suggest incorporation of conjugate views for estimating differential renal function in patients with hydronephrosis.

Reduced flow after tubularized incised plate urethroplasty--increased fibrogenesis, elastin fiber loss or neither?

PURPOSE: Low urinary flow rates are common after tubularized incised plate urethroplasty but the etiology remains unclear and may be related to low urethral compliance due to abnormal collagen concentrations and/or fewer elastic fibers in the healed urethral plate. We hypothesized that inserting a preputial mucosal graft over the dorsal raw area after the midline incision may avoid scarring and improve urethral compliance. MATERIALS AND METHODS: Adult rabbits were submitted to tubularized incised plate urethroplasty with or without inlay preputial graft according to a previously described protocol. Tissular concentrations of collagens I, III, IV, VI, VIII and XIII were measured. Histomorphometric analysis was used to quantify elastic fibers in the urethra. Tubularized incised plate urethroplasty with and without inlay preputial graft was compared to normal rabbit urethras (controls). RESULTS: mRNA concentrations for collagens I, II and XIII were similar between controls and operated rabbits. The proportions between collagens I and III were 1.05, 0.87 and 1.21, respectively, in controls and animals undergoing tubularized incised plate urethroplasty with and without inlay preputial graft. mRNA concentrations for collagen IV and collagens VI/VIII tended to be higher and lower, respectively, in the operated urethras, despite showing statistical significance only for collagen VIII in animals undergoing tubularized incised plate urethroplasty with inlay preputial graft vs controls (p=0.02). The operated animals did not demonstrate a reduced number of elastic fibers in the urethral tissues compared to controls. CONCLUSIONS: Elastic fiber number and distribution were similar between tubularized incised plate urethroplasty cases and controls, suggesting that decreased concentrations of elastic fibers do not explain the reduced urethral compliance after tubularized incised plate urethroplasty. The raw area determined by the dorsal urethral incision regenerated after standard tubularized incised plate urethroplasty, while cicatrization with fibrosis occurred in correspondence to the grafted areas after tubularized incised plate urethroplasty with inlay preputial graft.

Decisional regret after distal hypospadias repair: single institution prospective analysis of factors associated with subsequent parental remorse or distress.

PURPOSE: Hypospadias repair is a commonly performed procedure. Little is known about decisional regret in parents who agree to proceed with this surgical reconstruction. We present data on this previously underexplored issue. MATERIALS AND METHODS: We performed followup analysis of 100 couples prospectively evaluated after counseling for surgical correction of distal hypospadias in their son with assessment of complications and decisional regret 1 year after surgery. Findings were contrasted with baseline demographics, hypospadias knowledge and decisional conflict at the time of counseling. RESULTS: Decisional regret was found in 116 parents, including mild regret in 41.4% and moderate to severe regret in 8.6%. There was no statistically significant difference in paired regret analysis between mothers and fathers. Complications were strongly associated with decisional regret (p <0.001). On regression analysis postoperative complications (OR 14.7, 95% CI 1.6-131.6), parental desire to avoid circumcision (OR 7.4, 95% CI 1.1-49.4) and initial decisional conflict level (OR 1.06, 95% CI 1.02-1.09) were statistically significant predictors of moderate to strong decisional regret. These findings remained robust after imputation strategies to address missing data. The impact of decisional conflict and preference for circumcision were significant even after excluding families who experienced complications. CONCLUSIONS: To our knowledge this is the first study demonstrating parental decisional regret after providing consent for surgical correction of distal hypospadias in their son. Based on the described risk factors efforts aimed at minimizing complications and counseling about foreskin preservation techniques may be prudent to ameliorate decisional regret. The novel association between decisional conflict and regret suggests that conflict assessment during counseling may help screen families at risk for postoperative regret.

Urinary tract abnormalities in boys with recurrent urinary tract infections after hypospadias repair.

OBJECTIVE: To examine the development of recurrent urinary tract infections (UTIs) in boys who have undergone hypospadias repair. MATERIALS AND METHODS: We retrospectively reviewed the records of all boys who had recurrent UTIs after primary or redo tubularized incised plate (TIP) or transverse island flap (TVIF) repairs, between 1998 and 2009. Data on age, operating details, postoperative complications and imaging studies were collected. We attempted to identify risk factors for recurrent UTIs after hypospadias repair. RESULTS: During the study period, 43/2249 boys (1.91%) were diagnosed with recurrent UTIs after hypospadias repair. The boys' mean (range) age at repair was 14 (6-24) months and the median (range) follow-up was 6.5 (1.5-11) years. Primary TIP and TVIF were performed in 47% (20/43) and 35% (15/43) of the boys, respectively. Redo surgeries were performed in 18% of the boys (8/43). The initial meatal location was proximal in all TVIF and redo repairs, and in one of the TIP repairs. Postoperative voiding cysto-urethrography, ultrasonography and dimercapto-succinic acid (DMSA) scans were performed in 58% (25/43), 90% (39/43) and 19% (8/43) of the boys, respectively. Abnormalities were noted. Of those boys who underwent a TVIF repair, urethral diverticula were seen in 47% (7/15) and urethral fistulae were also seen in 47% (7/15). Conversely, in those who had a TIP repair, an elevated PVR and vesico-ureteric reflux were more common; they were found in 40% (8/20) and 50% (10/20) of patients, respectively. CONCLUSIONS: The pathophysiology of recurrent UTI is multifactorial, but postoperative complications seem to vary with type of procedure. Recurrent UTIs after hypospadias surgery should prompt a specific assessment for potentially functionally relevant and correctable anatomical abnormalities.

Redo surgery to improve urinary function, sexual function and cosmesis in male patients with exstrophy-epispadias complex complications. Technical principles and pearls based on case scenarios.

The bladder exstrophy-epispadias complex includes some of the most challenging conditions treated by pediatric urologists. They are associated with the need for multiple intricate reconstructive procedures, aimed at restoring the anatomy and function of the bladder, urethra and external genitalia. These patients often endure multiple redo reconstructive procedures to improve urinary function, sexual function and cosmesis throughout the first two decades of life. In this article, we present the 30-year experience of a single surgeon performing redo surgery for males born with epispadias and bladder exstrophy. Through detailed documentation of 6 clinical cases, we highlight technical aspects that may contribute to a successful surgical reconstruction in these patients. The article is focused specifically on patients undergoing redo epispadias repair with or without concomitant continence procedures. We make the case for complete penile disassembly with external rotation of the corpora to correct recurrent dorsal curvature; this approach also allows the surgeon to have access to the proximal urethra and bladder neck after opening the intersymphiseal scar/band. This is useful when additional procedures on the bladder, such as bladder neck tailoring, are necessary. We also highlight the importance of avoiding reverse Byars' flaps when performing skin closure, due to the resulting midline scar. Besides being associated with a poor cosmetic outcome, it can also contribute to recurrent dorsal curvature. The authors advocate for rotational skin flaps to cover the penile shaft. Correction of dorsal curvature and improved cosmesis obtained with complete penile disassembly sometimes comes at the expense of the urethra being left as a hypospadias (figure). This will require further surgeries (usually a 2-stage buccal mucosa graft), much like the treatment of proximal hypospadias. Redo epispadias surgery in males remains a challenge. The systematic approach offered by the case scenarios may help guide surgeons dealing with this difficult condition. Patient with complications after repair of classic bladder exstrophy. A) Stone retrieved from posterior urethra after complete penile disassembly. B) After opening the inter-symphiseal scar, the bladder has been opened and the bladder neck tailored. C) Complete penile disassembly has been completed with corporal bodies and urethra individualized. D,E,F) Final appearance of the repair; abdominal wall was closed with anterior rectus sheath flaps, penile skin was closed with rotational flaps and urethra ended up as a hypospadias.

Permanent cutaneous vesicostomy: a pragmatic approach to safely manage lower urinary tract dysfunction in pediatric patients with chronic and life-limiting conditions and neuropathic bladders.

Introduction: Lower urinary tract dysfunction (LUTD) in cerebral palsy (CP) and other neuromuscular diseases can present with chronic retention that leads to hydronephrosis, recurrent urinary tract infections (UTI), and stone formation. Whenever the conservative treatment of LUTD fails for any reason, it is considered to be complicated LUTD, in which a surgical approach is warranted. Cutaneous vesicostomy (CV) is a simple, well-tolerated, and potentially reversible procedure that protects the upper tracts. We describe our experience using CV for this complex population. Materials and methods: Children with CP and other neuromuscular diseases admitted to pediatric long-term care units for palliative care between 2015 and 2019 were included in the study. They present multi-system involvement, polypharmacy, and Gross Motor Function Classification System levels of 4 or 5. We retrospectively studied this population's indications and results of CV. Results: Of the 52 admitted patients, 18 presented LUTD with UTI (n:18; 100%), stones (n:5; 28%), progressive hydroureteronephrosis (n:3; 17%), or stones (n:2; 11%). Conservative initial management (catheterizations, prophylaxis antibiotics) was effective in half the cases. The remaining nine were defined as complicated LUTD and underwent CV. After a mean follow-up of 11.3 months, the follow-up showed improved hydronephrosis in all nine (100%) patients. Recurrent UTIs were no longer seen in eight of nine patients, although three patients required bladder irrigations; bladder stones did not recur after CV; the kidney stones needed further intervention. Revision of the CV was required in two (11%) cases at 12 and 24 months postoperatively due to stoma stenosis. Conclusion: CV is a relatively simple and effective procedure representing a pragmatic solution for managing complicated LUTD in complex long-term institutionalized pediatric palliative care patients with neuropathic bladders.

Kidney function outcomes in patients after complete primary repair of bladder exstrophy and penopubic epispadias: Results from the international bladder exstrophy consortium.

INTRODUCTION: Historically, repair of bladder exstrophy (BE) is associated with compromise to the upper tracts; the single stage repair of BE was considered to exacerbate risks of kidney impairment. OBJECTIVE: We aim to evaluate the risk of upper urinary tract deterioration or chronic kidney disease after the complete primary repair of exstrophy (CPRE). STUDY DESIGN: As part of the U.S.-India Multi-institutional Bladder Exstrophy Collaboration, we prospectively performed data collection on all patients managed at the Civil Hospital, Ahmedabad from 2010 to 2020. All patients who underwent primary or redo BE or primary penopubic epispadias (PE) repair using CPRE were included. Data on annual VCUG and DMSA, serum creatinine and cystatin-C, urinary albumin, and creatinine were aggregated. RESULTS: 72/104 patients who underwent CPRE at a median age of 1.7 years (IQR: 1.1-4.6) were included: 43(60%) patients with primary BE, 17(24%) with redo BE, and 12(17%) with primary PE. At a median follow-up of 4 years (IQR: 3-6), the overall median eGFR was 105 for BE, and 128 ml/min for PE. 14(19%) patients had eGFR<90, and 22(31%) had microalbuminuria. 21(30%) patients had kidney scarring in DMSA and 31(44%) had VUR. Multivariate analysis showed that neither kidney scarring nor VUR could predict the presence of eGFR<90 or microalbuminuria. Of 72 patients, 2 (3%) patients had dry intervals >3 h, 9 (13%) patients have dry intervals of 1-3 h and 44 (61%) patients had dry intervals <1 h during follow-up. We found that kidney function outcomes (i.e., eGFR and microalbuminuria) were not associated with continence status (p = 0.3). DISCUSSION: In this series, we report a 5% incidence of CKD stage 2 or above that was not impacted by continence status. Furthermore, a 40% incidence of VUR and a 30% incidence of kidney scarring during follow-up was observed within this cohort, neither of which had a significant impact on renal function deterioration (i.e, decline in eGFR), but underscores the need for close kidney surveillance in children that have undergone bladder exstrophy repair. CONCLUSIONS: Modern CPRE technique for the repair of BE may increase the risk of kidney scarring in the intermediate-term follow-up, however, this finding does not correlate with low eGFR and presence of albuminuria inpatients. Therefore, close follow-up with serial kidney function measurements is warranted and necessary after CPRE.

Do infants with mild prenatal hydronephrosis benefit from screening for vesicoureteral reflux?

PURPOSE: Screening for vesicoureteral reflux in asymptomatic infants with low grade hydronephrosis is unjustified if this condition is benign. We present a cohort with prenatally detected hydronephrosis, some observed without voiding cystourethrography, and compare outcomes to a classic, screened group. MATERIALS AND METHODS: A total of 206 consecutive children presenting with postnatally confirmed prenatally detected hydronephrosis (47% bilateral) were included in the study. Cases with associated renal or bladder anomalies were excluded. Children with low grade hydronephrosis either underwent screening voiding cystourethrography or did not, largely based on whether care was managed by a urologist or a nephrologist. Patients with high grade hydronephrosis routinely underwent voiding cystourethrography. Primary outcome was a symptomatic or febrile urinary tract infection. We used Mantel-Haenszel analysis to determine urinary tract infection risk factors during the first 2 years of life. RESULTS: No urinary tract infection was observed in patients with grade I hydronephrosis. Urinary tract infections in low grade hydronephrosis were only seen in the voiding cystourethrogram group (7 patients), including 1 infection following voiding cystourethrogram. Urinary tract infection rate was 3.52 infections per 100 patient-years in children with low grade hydronephrosis and 11.1 infections per 100 patient-years in those with high grade hydronephrosis (p = 0.02). This increased risk of urinary tract infection in high grade hydronephrosis persisted after correcting for gender and circumcision status (IRR 3.17, p = 0.01). The association remained strong (IRR 2.48, 95% CI 0.96-6.44) but was not statistically significant (p = 0.053) after additionally correcting for vesicoureteral reflux status. CONCLUSIONS: Our data suggest that children with low grade hydronephrosis and otherwise normal kidneys and bladder do not benefit from voiding cystourethrographic screening. Interestingly high grade hydronephrosis appears to carry a threefold greater risk of urinary tract infection compared to low grade hydronephrosis.

Analysis of decisional conflict among parents who consent to hypospadias repair: single institution prospective study of 100 couples.

PURPOSE: Although obtaining informed consent for distal hypospadias repair is common practice, little is known about the uncertainty or conflict between consenting parents faced with this decision. We systematically evaluated decisional conflict between parents who elected to have their child undergo hypospadias surgery. MATERIALS AND METHODS: A total of 100 couples who were counseled about treatment options agreed to participate. Using a validated questionnaire, the Decisional Conflict Scale, we prospectively collected data on decisional conflict demographics, preference for circumcision, education level and prior knowledge about hypospadias. RESULTS: All parents elected surgical repair. Evidence of decisional conflict was encountered in 28% of participants (score less than 25 in 72%, 25 to 37.5 in 23.5%, greater than 37.5 in 4.5%). No statistically significant differences among parents were noted for total score (mean ± SD 16.1 ± 12 in mothers and 18.3 ± 12.6 in fathers) or subscales, except the informed subscale (mean ± SD 16.7 ± 14.3 in mothers and 21.1 ± 16.6 in fathers). Parental self-report of prior knowledge about hypospadias and preference for neonatal circumcision correlated with lower Decisional Conflict Scale scores (p = 0.02 and p <0.01, respectively). No statistical association was found between score and parental education level (p = 0.7) or expertise of the counselor (staff vs pediatric urology fellow, p = 0.4). CONCLUSIONS: These data describe the level of decisional conflict in couples agreeing to proceed with hypospadias repair, with no evidence of significant discrepancy between them. The novel description of factors related to decreased decisional conflict might help focus efforts aimed at minimizing difficulties encountered during the decision making process.

Comparative analysis of detorsion alone versus detorsion and tunica albuginea decompression (fasciotomy) with tunica vaginalis flap coverage in the surgical management of prolonged testicular ischemia.

PURPOSE: Recent data suggest that testicular torsion may include an element of the compartment syndrome that improves with decompression. In 2009 we instituted tunica albuginea incision with tunica vaginalis flap coverage as an alternative in cases in which the torsed testis continued to appear ischemic after detorsion. MATERIALS AND METHODS: The medical records of 65 boys who underwent scrotal exploration for testicular torsion between 2000 and 2010 were reviewed. There were 6 patients excluded from study due to lack of followup. Of the remaining 59 patients 31 (52.5%) showed improvement in testicular appearance after detorsion and underwent orchiopexy, whereas 28 (47.5%) did not show evidence of recovery after detorsion. Of these patients 11 underwent tunica albuginea incision with tunica vaginalis flap coverage and 17 underwent orchiectomy. Demographic data, duration of symptoms and rate of testicular salvage were analyzed. RESULTS: Mean patient age was 11.8 years (detorsion plus orchiopexy), 10.1 years (tunica albuginea incision plus tunica vaginalis flap coverage) and 10.1 years (detorsion plus orchiectomy). Average followup was greater than 6 months in all groups. Mean duration of torsion was 13.4 hours (detorsion plus orchiopexy), 31.2 hours (tunica albuginea incision plus tunica vaginalis flap coverage) and 67.5 hours (detorsion plus orchiectomy). Before tunica albuginea incision with tunica vaginalis flap coverage was offered, the rate of orchiectomy was 35.9% (14 of 39) vs 15% (3 of 20) after this technique was introduced (p <0.05). The rates of testicular salvage were 62.5% (detorsion plus orchiopexy), 54.6% (tunica albuginea incision plus tunica vaginalis flap coverage) and 0% (detorsion plus orchiectomy). Although the numbers are limited, it is likely that without tunica albuginea incision with tunica vaginalis flap coverage 6 of 11 testes would have been removed. CONCLUSIONS: This preliminary experience suggests that tunica albuginea incision with tunica vaginalis flap coverage is a promising option for the management of clinically marginal torsed testes, enhancing salvageability after prolonged ischemia. We recommend considering this maneuver before performing orchiectomy in selected cases of testicular torsion.

Early postoperative ultrasound after open pyeloplasty in children with prenatal hydronephrosis helps identify low risk of recurrent obstruction.

PURPOSE: Prediction of successful pyeloplasty can be challenging, particularly in asymptomatic children treated for worsening prenatally diagnosed hydronephrosis. We evaluated early noninvasive objective predictors of success in this population. MATERIALS AND METHODS: We reviewed patients who underwent pyeloplasty for asymptomatic, prenatally detected, worsening hydronephrosis treated between 2000 and 2010 with followup greater than 1 year. For all patients renal pelvis anteroposterior diameter in a mid transverse view and Society for Fetal Urology hydronephrosis grade were evaluated preoperatively and 3 to 4 months postoperatively. Aside from subjective evaluation based on change in hydronephrosis grade, we estimated the percentage of improvement in anteroposterior diameter (preoperative diameter--postoperative diameter/preoperative diameter). Repeat intervention after pyeloplasty arbitrarily defined failure. Patients were categorized into 3 groups, ie no failure on followup ultrasound (group 1), no failure with postoperative nuclear scan to document success (group 2) and failed pyeloplasty (group 3). ROC curves were plotted to correlate 4 variables with failure, namely preoperative anteroposterior diameter, postoperative anteroposterior diameter, percent improvement in anteroposterior diameter and subjective change in grade. RESULTS: Of 229 patients 192 (84%) who met inclusion criteria had ultrasound at 3 to 4 months postoperatively available. Mean age at surgery was 19 months and mean ± SD followup was 49.9 ± 27.7 months. Percent improvement in anteroposterior diameter and postoperative anteroposterior diameter measure were the most reliable variables to predict failure (AUC 0.88 and 0.86, respectively, p <0.0001), whereas preoperative anteroposterior diameter and subjective changes in grade were not good predictors (AUC 0.52, p >0.05). A percent improvement in anteroposterior diameter of 38% or greater or postoperative anteroposterior diameter of 11.5 mm or less was associated with success, with sensitivity of 100% and specificity of 61% and 55%, respectively. CONCLUSIONS: Percent improvement in anteroposterior diameter and postoperative pelvic anteroposterior diameter measure can provide objective guidance as to which patients need closer monitoring during followup, and can help select children at low risk for repeat intervention after pyeloplasty.

Nephron sparing surgery for unilateral Wilms tumor in children with predisposing syndromes: single center experience over 10 years.

PURPOSE: Unilateral Wilms tumors associated with predisposing syndromes are treated with preoperative chemotherapy followed by surgical resection. We describe our experience with nephron sparing surgery for Wilms tumor in this population at risk for metachronous lesions. MATERIALS AND METHODS: We conducted a retrospective review of all children with a predisposing syndrome who underwent nephrectomy for malignancy during a 10-year period (2000 to 2010). Data collected included age, mode of detection, tumor size, treatment, pathology results, followup time and recurrence episodes. RESULTS: From 2000 to 2010, 13 of 75 (19%) patients treated for Wilms tumor were diagnosed with predisposing syndrome(s). Eight patients with unilateral tumors were treated and had a mean age at diagnosis of 27 months (range 7 months to 9 years). Beckwith-Wiedemann syndrome, isolated hemihyperplasia, WAGR (Wilms tumor, Aniridia, Genitourinary abnormalities, mental Retardation) syndrome and isolated 11p13 deletion were the underlying diagnoses in 3, 2, 2 and 1 patient, respectively. All but 2 patients were diagnosed by screening ultrasound and 5 underwent preoperative chemotherapy. Median tumor size at surgery was 2.5 cm (range 1 to 13). Nephron sparing surgery was performed in 6 of 8 patients. Pathological study showed favorable histology Wilms tumor and nephrogenic rests in 6 and 2 patients, respectively. After a mean followup of 36 months (range 6 to 72) no recurrences were documented and all children had normal creatinine levels. CONCLUSIONS: Nephron sparing surgery appears safe for patients with unilateral Wilms tumor associated with predisposing syndrome(s), allowing for the preservation of renal function and good oncologic outcomes for the available followup time. If more studies confirm our observation, current recommendations for the surgical treatment of Wilms tumor may need to reemphasize the value of attempting nephron sparing surgery in this patient population.

Bladder exstrophy: We need to improve. A lot.

INTRODUCTION: Bladder exstrophy (BE) affects continence and sexual function, impacting on social life and mental health. Long-term data from the patients' point of view are needed to get a real-life perspective on the problem. STUDY DESIGN: A self-developed questionnaire concerning sexual, psychosexual and psychosocial outcomes was sent to the adult members of the Brazilian Exstrophy Group. RESULTS: Fifty out of 67 adults from the group (74.5%) responded to the questionnaire. Failure of initial bladder closure attained 62%. Almost ¾ of the patients had augmentation cystoplasty. Bladder lithiasis was common. Esthetic procedures were frequently done. Repetitive UTI (n = 32, 64%) and kidney scars/disease (n = 20, 40%) were frequent. Most (88%) patients either depend on CIC or remain incontinent. Sexual problems predominated in males. Surgery for continence often failed, requiring re-operations, but the prognosis without these procedures was comparatively worse. Continent patients underwent more surgeries (mean 18, 13 and 9 procedures in continent, imperfectly continent and incontinent patients, respectively). Augmented patients more frequently achieved dryness (p = 0.0035). Two-thirds of the women underwent vaginoplasties, but dyspareunia/feeling of "tight" vagina still affected a quarter of them. Four women (15.4%) delivered healthy children. 91.7% of the males reported "normal" erections, but sexual inhibition was common due to feeling of having a small penis (n = 18, 75%). Persistent dorsal curvature and abnormal ejaculation were common (58.3% and 77.1%, respectively). Patients' comments related mainly to mental health issues/need for specialized care, limitations of medicine to cure/treat their disease, unavailability of experts, especially adult specialists, embarrassment over deformities and insufficient information about disease/treatment/prognosis. DISCUSSION: Most BE patients are well-integrated into society, but feelings of sadness and low self-esteem are common. Most welcome procedures to become dry, despite self-catheterization. The results of bladder neck reconstruction are far from perfect, despite multiple attempts and bladder augmentation was often necessary. Volitional voiding is uncommon. Sexual problems are worse for males, and sexual avoidance is common. Sexual function and self-image are inter-related. It seems reasonable to offer selective esthetic procedures to improve social/sexual interaction. Obstetric complications are common, especially UTI, need for ureteral and/or conduit stenting, abnormal fetal positioning, uterine prolapse, technical problems during surgical deliveries and prematurity. CONCLUSION: Continence/dryness in BE was mostly eventually achieved, usually depending on multiple interventions, bladder augmentation and self-catheterization. Despite multiple surgeries many adults remain incontinent. Sexual problems and avoidance are the rule in males, due to the feelings of penile inadequacy. Pregnant females deserve expert obstetric care.

Caregiver distress: A mixed methods evaluation of the mental health burden of caring for children with bladder exstrophy.

Introduction: Caring for children with bladder exstrophy-epispadias complex (BEEC) exacts a long-term emotional toll on caregivers. Previous studies leave a gap in understanding the impact that caring for a child with BEEC has on caregivers in low- and middle-income countries (LMIC). We hypothesize that families and caregivers experience psychological distress that has long gone unaddressed. Materials and methods: From 2018 to 2020, researchers conducted a multi-method evaluation of caregiver distress with participants recruited as part of the annual International Bladder Exstrophy Collaboration based in Ahmedabad, Gujarat, India. In 2018, pilot data was collected through cognitive interviews. In 2019, researchers conducted structured interviews predicated on themes from the previous year, which subsequently prompted formal mental health screenings in 2020. Caregivers who reported suicidal thoughts were immediately referred for intervention. Results: In 2018, caregivers described the primary source of stigma arose from their village (n = 9, 26.5%). Caregivers also identified long-term concerns (n = 18, 52.9%), including future fertility and marital prospects, as sources of anxiety. In 2019, caregivers substantiated preliminary findings with the primary source of anticipated (n = 9, 31%) and experienced (n = 19, 65.5%) stigma again stemming from their communities. Both cohorts identified the collaboration as a positive source of support (n = 23, 36.5%). In 2020, caregivers stated decreased emotional wellbeing as number of subsequent repairs increased (n = 54, 75%, p = 0.002). Caregivers of children who underwent initial surgery within 5 years of screening reported higher anxiety (n = 46, 63.8%) and this was exacerbated as the number of subsequent repairs increased (p = 0.043). Conclusion: Complex, long-term course of care, including additional surgeries, significantly impacts caregiver distress in the LMIC setting. Screening for caregivers of children with complex congenital anomalies, like BEEC, should be an essential element of any comprehensive effort to alleviate the global burden of disease.

Critical outcome analysis of staged buccal mucosa graft urethroplasty for prior failed hypospadias repair in children.

PURPOSE: Although staged buccal mucosa graft urethroplasty is a well accepted technique for salvage urethroplasty, there are few reports on this procedure for redo hypospadias repair in children. MATERIALS AND METHODS: We reviewed patients who underwent staged buccal mucosa graft urethroplasty for redo hypospadias repair. Age, quality of graft before tubularization, meatal position, presence of balanitis xerotica obliterans and complications were recorded. RESULTS: A total of 30 patients underwent 32 repairs during a 5-year period. Mean age at first stage was 7 years (range 1 to 17) and mean interval between stages was 9.3 months (5 to 13). Mean followup after second stage was 25 months (range 10 to 46). Meatal position before first stage was proximal in 44% of patients, mid shaft in 39% and distal in 16%. Nine patients had biopsy proved balanitis xerotica obliterans. There were no donor site complications. Four patients underwent a redo grafting procedure. Complications after second stage occurred in 11 of 32 repairs (34%), consisting of urethral stenosis in 5, glanular dehiscence in 3 and urethrocutaneous fistula in 3. A third of the patients had some degree of graft fibrosis/induration after the first stage. These patients were prone to more complications at second stage (9 of 11, 82%), compared to patients without these unfavorable findings (4 of 21, 19%; p<0.001). Presence of balanitis xerotica obliterans and meatal position were not significant factors associated with adverse outcomes. CONCLUSIONS: Staged buccal mucosa graft urethroplasty is a suitable technique for salvage urethroplasty. Complications after second stage were seen in approximately a third of patients, mainly those with fibrotic/indurated grafts.

Hydronephrosis in children with abdominal and pelvic neoplasms: outcome and survival analysis of a single center pediatric oncology series.

PURPOSE: We ascertained the incidence and outcome of hydronephrosis related to abdominal or pelvic neoplasms and survival in pediatric patients. MATERIALS AND METHODS: We retrospectively reviewed our institutional oncology database between January 1995 and November 2009. We reviewed the charts of all children with intra-abdominal (nonrenal) and pelvic neoplasms. RESULTS: Of the 366 patients whose charts were reviewed 66 (18%) had hydronephrosis at some point during treatment, including 12 with hydronephrosis that was not caused by the neoplasm and 1 who was lost to followup, leaving 53 with malignant obstruction. Of the remaining patients hydronephrosis resolved in 34 (64%) with treatment for the primary neoplasm alone while in 19 (36%) hydronephrosis persisted after primary oncological treatment. Univariate analysis revealed that patients with persistent hydronephrosis (p = 0.025), those with urological intervention (p = 0.05) and those with high stage disease (p <0.001) had statistically significantly worse overall survival. On Cox multivariate analysis only disease stage remained statistically significant (p = 0.004). CONCLUSIONS: Analysis of this group revealed that pediatric nonrenal abdominal and pelvic tumors are associated with hydronephrosis in about 20% of cases. Approximately 60% of these cases resolved with treatment for the primary tumor alone while 13% required specific urological intervention for urinary tract involvement or compression. Patients with pediatric malignant ureteral obstruction had a 20% 5-year mortality rate. The main predictive factor was primary disease stage.

Long-term followup and time to event outcome analysis of continent catheterizable channels.

PURPOSE: Creation of a continent catheterizable channel has facilitated the treatment of patients undergoing lower urinary tract reconstruction. We present outcomes and complications of a single center series of continent catheterizable channels followed out to 15 years. MATERIALS AND METHODS: We retrospectively reviewed medical records of all children who underwent continent catheterizable channel (Mitrofanoff and Monti) between 1992 and 2007. Collected data included age, underlying diagnosis, associated procedures, stoma site, conduit type (appendix or reconfigured bowel), time to complications and need for subsequent surgical revisions. RESULTS: We identified 71 girls and 98 boys who underwent surgery at a mean age of 7.5 years (range 6 months to 22 years) and were subsequently followed for a mean of 5.8 years (8 months to 15 years). Underlying diagnoses included neurogenic bladder (36% of patients), bladder exstrophy (25%), epispadias (6%) and posterior urethral valves (6%). Concurrent procedures were conducted in 71% of cases, including augmentation (35%) and bladder neck plasty (22%) or closure (8%). Surgical revision was performed in 39% of patients, including stomal revision (18%), redo operation (8%), bulking agent injection (8%) and prolapse correction (4%). Although an initial peak was followed by a relatively stable complication-free period, delayed problems were detected on long-term followup. No statistically significant differences in complication rates were noted when comparing use of appendix and reconfigured bowel or different stoma locations. CONCLUSIONS: Despite an initial decrease in complications soon after continent catheterizable channel creation, late problems appeared on long-term evaluation. In our experience no specific factor predicted the likelihood of complications. Nevertheless, despite the need for surgical revision, good functional outcomes were evidenced in this series.

Clinical evolution of vesicoureteral reflux following endoscopic puncture in children with duplex system ureteroceles.

PURPOSE: We studied the clinical evolution of vesicoureteral reflux after endoscopic puncture of ureterocele in pediatric duplex systems. MATERIALS AND METHODS: We retrospectively reviewed charts of children with duplex system ureteroceles treated between 1992 and 2007. We analyzed patient age, prenatal diagnosis, urinary tract infection at presentation, differential renal function and preoperative vesicoureteral reflux. The fate of associated vesicoureteral reflux after endoscopic puncture of ureterocele was specifically addressed. RESULTS: We analyzed 60 patients with a mean age of 12 months, of whom 32 (52%) were diagnosed prenatally and 40 (66%) presented with a urinary tract infection. The majority of ureteroceles were ectopic, and pre-puncture vesicoureteral reflux was seen in 40 patients (67%). Postoperative voiding cystourethrogram was performed in 50 cases. New onset of vesicoureteral reflux occurred postoperatively in 24 patients (40%), mainly ipsilateral to the lower pole or contralaterally. Spontaneous vesicoureteral reflux resolution or improvement occurred in 16 of 22 patients (72%) observed after primary endoscopic puncture of ureterocele. Surgical intervention after endoscopic puncture of ureterocele was performed in 25 patients (42%), of whom 9 underwent more than 1 reparative procedure. CONCLUSIONS: Vesicoureteral reflux after endoscopic puncture of ureterocele may occur in the ipsilateral upper or lower poles, or in the contralateral renal segments. Therefore, the reflux is not necessarily related to the puncture itself. Vesicoureteral reflux after endoscopic puncture of ureterocele can resolve spontaneously in a significant number of patients. Therefore, initial management by close surveillance is warranted. Considering the simplicity of the procedure, our findings support that endoscopic puncture of ureterocele is an attractive alternative for the initial management of pediatric duplex system ureteroceles.

Comparative histological and functional controlled analysis of tubularized incised plate urethroplasty with and without dorsal inlay graft: a preliminary experimental study in rabbits.

PURPOSE: We analyzed the histological and functional characteristics of the tubularized incised plate vs dorsal inlay graft urethroplasty in an experimental rabbit model. MATERIALS AND METHODS: A total of 24 New Zealand male rabbits were randomly allocated into 4 groups, including sham operation, urethroplasty, tubularized incised plate urethroplasty and dorsal inlay graft urethroplasty. In the urethroplasty group the anterior urethral wall was half excised and the dorsal aspect was tubularized. In the tubularized incised plate group the same steps were followed but tubularization followed a longitudinal midline incision in the dorsal wall. In the dorsal inlay graft group the defect created by the dorsal incision was covered with an inner preputial graft. The animals were sacrificed at 4 and 8 weeks, respectively. The penis was immediately harvested for standardized passive flowmetry and subsequently fixed for histological staining. RESULTS: The grafts took in all animals. The tubularized incised plate defect was bridged by urothelium while in the dorsal inlay graft group the preputial graft kept its original histological characteristics. There was a significant decrease in average flow in the urethroplasty group (1.6 ml per second) compared to that in the sham operated group (3.4 ml per second) and to the other groups (p <0.05). However, no significant difference in average flow was found for the tubularized incised plate and dorsal inlay graft groups (2.4 and 2.2 ml per second, respectively, p = 0.7). CONCLUSIONS: In this short-term rabbit model dorsal inlay graft urethroplasty was feasible with good graft take and integration. Simple tubularization of a reduced urethral plate led to significantly decreased flow. Incision of the reduced plate with or without grafting improved the average flow. Findings in this experimental model do not support the superiority of dorsal inlay graft urethroplasty over tubularized incised plate urethroplasty in terms of urethral flow dynamics.