Early urodynamic findings after complete primary repair of exstrophy.

INTRODUCTION: Detrusor contraction in bladder exstrophy (BE) patients following reconstruction is poorly understood as there are few published studies assessing urodynamic findings in this population. Understanding the ability of the detrusor to contract in BE patients early after closure may be able to inform the longer-term management and potential for the development of future continence in this population. OBJECTIVE: We sought to evaluate early detrusor contraction using urodynamic studies (UDS) in children who had previously undergone complete primary repair of bladder exstrophy (CPRE). We hypothesized that a majority of children with BE would display the presence of normal detrusor contractile function after CPRE. STUDY DESIGN: A retrospective review of our prospectively collected database was performed for all patients with a diagnosis of classic BE who underwent primary CPRE between 2013 and 2017. From this cohort we identified patients with at least one post-operative UDS at 3 years of age or older who had undergone an initial CPRE. Our primary outcome was the presence of a detrusor contraction demonstrated on UDS. RESULTS: There were 50 children (31 male, 19 female) with CBE who underwent CPRE between 2013 and 2017.There were 26 (13 male, 13 female) who met inclusion criteria. Median age was 3.5 (IQR: 3.2-4.7) years at the time of UDS Sixteen of the 26 (61.5%) generated a sustained detrusor contraction generating a void, with a median peak voiding pressure of 38 cm H20 (IQR: 28-51). The median bladder capacity reached was 48 ml, which represented a median of 30% of expected bladder capacity. The median post void residual (PVR) for the entire cohort was 26 ml (IQR: 9, 47) or 51% (IQR: 20%-98%) of their actual bladder capacity, while the median PVR for those children with a sustained detrusor contraction was 18 ml (IQR: 5, 46) or 33% (IQR: 27%, 98%) of their actual bladder capacity. Intraoperative bladder width and bladder dome to bladder neck length did not correlate with the presence of voiding via a detrusor contraction (p = 0.64). DISCUSSION: We present the first study assessing early UDS finding of detrusor contraction in BE patients after CPRE. In our cohort, 61.5% of patients were able to generate a sustained detrusor contraction on UDS which is a higher percentage than has been reported in previous series. A difference in initial surgical management may account for these findings. CONCLUSION: At short term follow up, the majority of children in our cohort were able to produce sustained detrusor contractions sufficient to generate a void per urethra with a modest post void residual volume. Long-term follow-up and repeated UDS will be needed to track detrusor contractility rates, bladder capacities, compliance, post void residuals and ultimately continence rates over time.

A comparison of clinical outcomes of acute testicular torsion between prepubertal and postpubertal males.

SHORT INTRODUCTION/BACKGROUND: Surgical intervention for acute testicular torsion can require either orchiopexy or orchiectomy. The decision of which surgery to perform is dependant on the amount of time that the testicle experienced ischemia and the viability of the testicle after reperfusion. OBJECTIVE: It is hypothesized that (1) there is a difference in orchiectomy and orchiopexy rates between prepubertal and postpubertal males with acute testicular torsion and (2) presenting symptoms may vary between the two age groups as prepubertal males may present with atypical symptoms, which could result in delayed presentation and diagnosis. STUDY DESIGN: A retrospective chart review was conducted on pediatric patients who were diagnosed with acute testicular torsion between June 2010 and August 2017. Demographic and clinical characteristics were extracted: age, ethnicity, referral pattern, primary insurance status, symptoms at presentation, prior history of ipsilateral testicular pain or intermittent torsion, recent trauma to genitalia, duration of symptoms (hours), gradual vs. acute onset of symptoms, time/weekday/season at presentation, and time interval from arrival at the study institution to surgical intervention (minutes). Patients were categorized into two groups: prepubertal group (age 1-12 years) and postpubertal group (age 13-18 years). Statistical analyses were performed using R, version 3.3.1. RESULTS: Ninety-one patients were included in the study. The overall orchiectomy rate was 30.8%. More prepubertal males underwent orchiectomy than postpubertal males (42.4% vs. 24.1%, respectively). Prepubertal males were more likely to present with abdominal pain than postpubertal males (27.3% vs. 10.3%, respectively). Those who underwent orchiectomy were more likely to present with longer duration of symptoms, testicular swelling, and abdominal pain than those who underwent orchiopexy. The risk of orchiectomy decreased by 14% per 1-year increase in age (odds ratio [OR]: 0.86, 95% confidence interval [CI]: 0.94-1.00, p = .009). A steady decline in the proportion of patients undergoing orchiectomy was seen from 1 to 12 years of age. DISCUSSION: This study found that prepubertal males are at higher risk for orchiectomy than postpubertal males. The risk of orchiectomy decreases by 14-16% per 1-year increase in age. Prepubertal males are more likely to present with atypical symptoms and delayed presentation and diagnosis, leading to delayed surgical intervention. It is important for providers to perform a genital examination in prepubertal males who present with abdominal pain to rule out acute testicular torsion. Patients presenting with longer duration of symptoms, testicular swelling, and abdominal pain are at higher risk for orchiectomy. No correlation was found between orchiectomy rate and ethnicity, referral status, primary insurance status, and time/weekday/season at presentation. CONCLUSION: Among patients presenting to a tertiary pediatric hospital with acute testicular torsion, prepubertal males (younger than 12 years) are at higher risk for orchiectomy than postpubertal males. Prepubertal males are more likely to present with atypical symptoms which results in delayed presentation and diagnosis, leading to delayed in surgical intervention.

Short-term outcomes of the multi-institutional bladder exstrophy consortium: Successes and complications in the first two years of collaboration.

INTRODUCTION/BACKGROUND: Bladder exstrophy is a rare diagnosis that presents major reconstructive challenges. To increase experience and proficiency in the care of bladder exstrophy (BE), the Multi-Institutional BE Consortium (MIBEC) was formed, with a focus on refining technical aspects of complete primary repair of bladder exstrophy (CPRE) and subsequent care. OBJECTIVE: Outcome measures included successful CPRE (absence of dehiscence), complications, and integrated points of technique and care over the short-term. STUDY DESIGN: Boston Children's Hospital, Children's Hospital of Philadelphia and Children's Hospital of Wisconsin alternately served as the host, with observation, commentary and critique by visiting collaborating surgeons. CPRE with bilateral iliac osteotomy was performed at 1-3 months of age. High-definition video capture of the surgery allowed local and distant broadcast to facilitate real-time observation and teaching, and recording of all procedures. RESULTS: From February 2013 to February 2015, MIBEC participating surgeons performed CPRE on 27 consecutive patients (22 classic BE, five epispadias). There were no dehiscences in 27 patients (0%, 95% CI 0-12.5%). Thirteen girls and 14 boys underwent CPRE at a median age of 2.3 months (range 0.1-51.6). One boy had a hypospadiac urethral meatus at CPRE completion. Hydronephrosis of mild or moderate grade was present postoperatively in eight girls and two boys. Additional results, per gender, are presented in the Summary table below. DISCUSSION: Absence of dehiscence in this cohort was comparable or compared favorably with the literature. However, several girls had significant obstructive complications following CPRE. The rate of bladder outlet obstruction (BOO) in girls was increased compared with published reports. A low complication rate was noted in the boys following CPRE, which was comparable to reports in the literature, and early signs of continence and spontaneous voiding were noted in some boys and girls. Limitations included variation in patient age at presentation, thereby introducing a wide age range at CPRE. Outcome data were limited by short follow-up regarding voiding with continence. CONCLUSION: This collaborative effort proved beneficial regarding significantly increased surgeon exposure to CPRE, refinement of CPRE technique, surgeon learning and expertise. Technical refinement of CPRE is ongoing.

Technique for Resonance® stent exchange in patients with extrinsic obstruction: description of a novel approach and literature review.

Malignant ureteral obstruction is a morbid diagnosis and can be difficult to manage. Ureteral stents often fail as the disease progresses. Metallic stents have provided a longer-term option for decompression as they inherently resist compression and require changes left often. However, changing metallic stents is challenging as it cannot be done over a wire as is done with plastic stents. We present a novel technique for safe and efficient exchange of the Resonance(®) metallic stent. We also review the literature on metallic stents.

Delayed onset ureteral obstruction following Deflux® injection for vesicoureteral reflux.

Endoscopic injection treatment of vesicoureteral reflux is an increasingly common and successful option. Obstruction is an infrequent postoperative complication, occurring in 1% of patients; delayed onset of obstruction is even rarer. There is a paucity of literature describing possible treatments. We present a novel approach by excision of the implanted material.

Effects of an environmental endocrine disruptor on fetal development, estrogen receptor(alpha) and epidermal growth factor receptor expression in the porcine male genital tract.

PURPOSE: We studied the effect of a potent reproductive tract toxin, 2,3,7,8-tetrachlorodibenzo-rho-dioxin, on fetal development and expression of estrogen receptor alpha and epidermal growth factor receptor (EGFR) in male swine. MATERIALS AND METHODS: Fetal domestic swine and miniswine were injected with 1 microg./kg. dioxin on day 50 of gestation and removed near term (114 days). Germ cell counts were performed on sections of formalin fixed testes. Estrogen receptor a protein, and messenger ribonucleic acid (mRNA) and EGFR mRNA expression were analyzed in frozen tissue using Western blotting and semiquantitative reverse transcriptase polymerase chain reaction. RESULTS: Of 15 dioxin exposed male offspring 8 (53%) had genital anomalies, including cryptorchidism in 4, epididymal detachment in 1, epididymal atresia in 1 and vasal dilatation in 3, while 3 of 17 control male swine (18%) had incompletely descended testes (p = 0.06). High intra-abdominal testes were found in 3 of 4 cryptorchid dioxin exposed but no control male swine. Mean germ cell number per tubule was 4.0+/-1.1 and 2.7+/-0.7 in control and dioxin groups, respectively (p = 0.01). Estrogen receptor a protein and mRNA were identified in fetal uterus, testis, gubernaculum and epididymis. Protein levels were 2 to 3-fold higher in dioxin exposed testis, and mRNA levels were significantly lower in gubernaculum and epididymis. EGFR mRNA expression was similar in treated and control testis and epididymis. CONCLUSIONS: Preliminary data suggest that dioxin produces cryptorchidism and wolffian duct anomalies in male swine exposed just before mid gestation. Germ cell counts and estrogen receptor alpha mRNA expression in gubernaculum and epididymis were significantly reduced, and estrogen receptor a protein expression in testis appeared to be increased by dioxin exposure. Aberrant regulation of estrogen receptor a expression by dioxin may contribute to reproductive tract anomalies in male fetuses.

Quantitative nuclear cystography does not predict outcome in patients with primary vesicoureteral reflux.

PURPOSE: Quantitative nuclear cystography has been advocated as a tool for determining the prognosis in children with primary vesicoureteral reflux. We reviewed our data on this technique to assess its usefulness for predicting the outcome in this population. MATERIALS AND METHODS: We retrospectively reviewed the records of all patients with primary reflux in whom findings were positive on at least 2 nuclear cystograms at our institution between 1992 and 1997. Patients followed at least 3 years were stratified according to outcome. Unfavorable prognostic criteria included bladder volume at reflux onset 60% or less of total bladder capacity and calculated volume of reflux 2% or greater of bladder capacity. RESULTS: Of the 107 patients in our study 63 were followed for 3 years or longer, and reflux resolved in 17, was repaired in 24 and persisted in 22. Mean patient age at latest followup, duration of followup and number of cystograms did not significantly differ among groups. Intermittent reflux in 33% of the patients followed 3 years or longer was not associated with outcome or detrusor instability. Bladder and reflux volume varied and was nonpredictive in individuals. CONCLUSIONS: Quantitative nuclear cystography did not predict the outcome in patients followed for primary vesicoureteral reflux at a single institution for 3 years or longer. Intermittent reflux was common. These data suggest that nuclear cystography cannot be used to assess reliably the prognosis in individuals. Strong consideration should be given to using negative findings on 2 cystograms to confirm reflux resolution in patients at high risk.

The outcome of artificial urinary sphincter placement after a mean 15-year follow-up in a paediatric population.

OBJECTIVE: To evaluate the long-term outcome in children who had an artificial urinary sphincter (AUS) placed, after a minimum of 10 years of follow-up. PATIENTS AND METHODS: The medical records of patients who had an AUS placed at the Children's Hospital of Michigan were reviewed and a telephone questionnaire was then completed by all patients with an AUS currently in place. RESULTS: Forty-seven children initially had an AUS placed between October 1978 and August 1986; medical records and follow-up were available for 32. After a mean follow-up of 15.4 years, 13 patients had had the AUS removed and 19 currently have an intact AUS. Erosion or infection was responsible for all AUS removals. Possible risk factors for AUS removal were prior AUS erosion, prior bladder neck surgery and a balloon pressure of >70 cmH2O. Eighteen of 19 patients with an intact AUS are dry and seven void volitionally. Revision was the most common reason for additional surgery, but the revision rate has decreased with the most current AS-800 model, to 0.03 revisions per patient-year. Of the 13 patients with an AS-800 model placed after 1987, nine have not required revision. Upper tract changes were mild and uncommon. CONCLUSION: The AUS is a durable and effective surgical option in the management of neurogenic urinary incontinence, and is the only reliable technique that can preserve volitional voiding. With technical improvements to the AUS and a longer follow-up, the revision rate has decreased. Causes of AUS removal may be preventable with improvements in surgical technique and patient selection. AUS placement should be considered as a first choice for the surgical management of neurogenic sphincteric incompetence.

Bladder cancer screening.

Transitional cell carcinoma of the bladder possesses several features that make it an ideal candidate for screening: metastases rarely occur before tumors invade the muscularis propria; superficial disease is treated very successfully by relatively inexpensive and nonmorbid means; this malignancy is almost never found incidentally at autopsy so that early detection cannot harm a patient; and almost all tumors cause hematuria if one tests frequently enough. When compared with a contemporary age, geography, and gender-matched unscreened population, bladder cancer screening in healthy men age 50 years and older by repeated home hematuria testing using chemical reagent strips significantly decreases bladder cancer morbidity and mortality and is cost-effective. A randomized prospective trial of bladder cancer screening in this population is recommended. Additionally, other potential means of bladder cancer screening and other target populations are discussed in this article.