Renal-Protective Urinoma Formation in a Newborn Boy With Posterior Urethral Valves.

Posterior urethral valves (PUV) are an uncommon urologic congenital anomaly in males often discovered antenatally and more rarely after birth. PUV can lead to obstructive nephropathy and voiding dysfunction, putting patients at increased risk for irreversible renal damage and subsequent progression to end-stage renal disease. Much of the renal damage caused by PUV is proportional to the amount of time that the kidney has been experiencing retrograde pressure. Although much debate exists within the field, spontaneous decompression within the collecting system (e.g., "pop-off" valve) such as urinoma formation or spontaneous ascites has been found to relieve pressure on and thus protect the kidney, decreasing the risk of progression to advanced stages of chronic kidney disease. Despite the significant mass effect on the renal parenchyma, the pressure-relieving function of urinoma formation is a net protective factor allowing renal function to be preserved. We report a unique case of antenatal detection of PUV in a male with postnatal complicated urinoma formation secondary to forniceal rupture. Remarkably, despite significant external compression of the kidney and the development of urosepsis from infection of the urinoma with a multidrug-resistant organism that required percutaneous drainage, renal function was preserved throughout the disease course. After ablation of the PUV and drainage of the septic urinoma, the patient recovered rapidly after intervention and was ultimately discharged in stable condition.

Application of Tunica Vaginalis Flap for Epispadias Repair in the Epispadias-Exstrophy Complex.

OBJECTIVE: To describe long-term outcomes and experience with the tunica vaginalis flap (TVF) as a tissue augment for complex epispadias repair. METHODS: A prospectively maintained institutional database was reviewed for patients who underwent epispadias repair with TVF from 2010 to 2021. Evaluation of patient characteristics between those who developed dehiscence or UCF and those who did not was performed via Mann-Whitney U, Kruskal-Wallis, or Fisher's exact test, as appropriate. RESULTS: A total of 47 male patients were identified, of whom, 1 (2.1%) had cloacal exstrophy, 38 (80.9%) had classic bladder exstrophy or a variant, and 8 (17.0%) had epispadias as their primary exstrophy-epispadias complex diagnosis. The median age at epispadias repair was 13 months (IQR 11 - 19). The overall rate of fistula or dehiscence development was 19.1% (9 patients), of whom, 7 developed urethrocutaneous fistulae, 1 dehiscence, and 1 both. Notably, 0 of the 5 patients who had undergone prior epispadias repair and 0 of the 8 patients with a diagnosis of isolated epispadias developed a UCF or dehiscence after repair with TVF. Differences in age at repair, primary EEC diagnosis, prior epispadias repair, pre-repair testosterone, fibrin sealant utilization, closure layers, stent removal time, and bladder capacity at repair were not statistically significant (P>.05) CONCLUSION: Our expanded evaluation indicates that utilization of the tunica vaginalis as an adjunct to epispadias repair may provide durable protection against fistula development in EEC patients who have undergone prior epispadias repair and in primary repair of patients with isolated epispadias.

Systematic review of telemedicine in pediatric urology.

INTRODUCTION: Telemedicine has bridged the distance gap between patients and pediatric urologists for over a decade, yet many pediatric urologists have not embraced it as a major part of their practice. The purpose of this systematic review is to evaluate and clarify the optimal role of telemedicine in pediatric urology, as well as the benefits, barriers, risks, and other important considerations that must be accounted for in its optimal adoption. METHODS: Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, this systematic review utilized Medical Subject Headings (MeSH) to search PubMed through April 29, 2021 as follows: (Humans [MeSH]) AND ((Child [MeSH]) OR (Infant [MeSH])) AND ((Remote Consultation [MAJR]) OR (Telemedicine [MAJR]) OR (Videoconferencing [MAJR]) OR (Health Services Accessibility [MAJR])) AND ((Urology [MAJR]) OR (Postoperative Care [MAJR]) OR (Urologic Surgical Procedures [MAJR])). 73 resulting articles, plus 21 from manual searches (e.g. papers too recent to have MeSH terms), were screened. 17 met inclusion criteria by discussing telemedicine in context of pediatric urology in a full manuscript. Non-complete manuscripts and papers not specifically considering pediatric urology, or in which telemedicine was not a major focus, were excluded. RESULTS: 17 papers met inclusion criteria. Varied approaches to the topic included surveys, controlled studies, retrospective studies, and descriptive opinion pieces. Quality of evidence varied, representing at least 1851 virtual encounters, 409 in-person encounters, and 68 clinician opinions. Four papers included a comparison or control group, and none utilized randomization. All 17 papers support expanded application of telemedicine in pediatric urology with varied evidence that telemedicine improves patient access to pediatric urologic care, satisfies both patient families and clinicians, is safe, provides equivalent outcomes, and is practicable. CONCLUSIONS: Implementation of telemedicine in pediatric urology should be expanded as it can practicably and safely improve patient access to pediatric urologic care, satisfy both patient families and clinicians, and maintain outcomes.

Practice patterns in classic bladder exstrophy: A global perspective.

INTRODUCTION: While evaluation and management options for classic bladder exstrophy (CBE) patients are numerous and varied, little is known regarding the relative utilization of these different methods throughout the world. A large group of exstrophy surgeons practicing globally was surveyed, seeking to document their methods of care. METHODS: A list of international exstrophy surgeons' email addresses was compiled using professional contacts and referral networking. An online survey was sent to each email address. Surgeons who had not performed a CBE closure within the previous 5 years were excluded. Survey questions queried the respondents' surgical practice type, years since training, and their preferred methods of preoperative evaluation, operative management, and postoperative management. Survey invitations were sent out starting in December 2014 and responses were collected for approximately 6 months. RESULTS: A total of 1152 valid email addresses were invited, resulting in 293 respondents (25%) from 39 countries and every American Urological Association (AUA) section. Seventy-six were excluded, leaving 217 respondents (Table). Respondents reported a median of 17 years since finishing their surgical training (IQR 8-25 years). Practice types included pediatric urology (n = 209), general urology (n = 9), pediatric surgery (n = 59), and other practice makeup (n = 3). On subgroup analyses, there were no significant regional practice differences, with the exception of complete primary repair of exstrophy (CPRE) and oral opioid prescribing being significantly higher in North America compared to other regions. DISCUSSION: Findings indicate that there may be diversity in CBE practice patterns globally. While most responding surgeons from regions outside of North America indicated modern staged repair of exstrophy (MSRE) as their preferred closure technique, a relatively equal distribution of respondents from North America selected CPRE and MSRE. A majority of North American surgeons chose performing osteotomies for both newborn and delayed closures, while an appreciable number of respondents from other regions selected never using osteotomies in their closures. Limitations to this study include a low survey response rate, particularly from surgeons outside of the United States, which may have significantly impacted the ability to draw meaningful global comparisons. CONCLUSIONS: Global variation among practices of surgeons performing CBE closures may exist. The wide range of methods demonstrated by this survey suggests the need for more conclusive comparative studies to elucidate whether an optimal standard exists. Local social factors, access to surgical expertise and transportation to referral centers, and finances play a role in what constitutes the best operative approach.

Long-term sexual health outcomes in men with classic bladder exstrophy.

OBJECTIVES: To identify the long-term sexual health outcomes and relationships in men born with classic bladder exstrophy (CBE). MATERIALS AND METHODS: A prospectively maintained institutional database comprising 1248 patients with exstrophy-epispadias was used. Men aged ≥18 years with CBE were included in the study. A 42-question survey was designed using a combination of demographic information and previously validated questionnaires. RESULTS: A total of 215 men met the inclusion criteria, of whom 113 (53%) completed the questionnaire. The mean age of the respondents was 32 years. Ninety-six (85%) of the respondents had been sexually active in their lifetime, and 66 of these (58%) were moderately to very satisfied with their sex life. The average Sexual Health Inventory for Men score was 19.8. All aspects of assessment using the Penile Perception Score questionnaire were on average between 'very dissatisfied' and 'satisfied'. Thirty-two respondents (28%) had attempted to conceive with their partner. Twenty-three (20%) were successful in conceiving, while 31 (27%) reported a confirmed fertility problem. A total of 31 respondents (27%) reported undergoing a semen analysis or post-ejaculatory urine analysis. Of these, only four respondents reported azoospermia. CONCLUSION: Patients with CBE have many of the same sexual and relationship successes and concerns as the general population. This is invaluable information to give to both the parents of boys with CBE, and to the boys themselves as they transition to adulthood.

Patient-Reported Impact of Pelvic Organ Prolapse on Continence and Sexual Function in Women With Exstrophy-Epispadias Complex.

OBJECTIVE: This study aimed to characterize long-term urogynecologic issues of women with a history of bladder exstrophy and pelvic organ prolapse (POP) and to assess the impact of POP repair on continence and sexual function. DESIGN: Patient demographics and surgical history related to exstrophy and POP were collected through chart review. Patient perceptions regarding sexual function, urinary continence, and quality of life were assessed through Web-based administration of validated questionnaires: International Consultation on Incontinence Questionnaire-Urinary Incontinence Short Form and POP-Urinary Incontinence Sexual Questionnaire. SETTING: Maryland, United States. PARTICIPANTS: Review of a single-institution exstrophy-epispadias complex database resulted in 25 adult female patients with a history of POP treated at the authors' institution. Eleven patients participated and were included in the analysis. MAIN OUTCOME MEASURES: Urinary continence and sexual function. RESULTS: All participants underwent surgical repair for prolapse, with 7 (63.6%) experiencing unsuccessful initial repair and subsequent recurrence. Median total number of POP repairs was 2.5 (1-4). After correction of POP, patients reported a median improvement in International Consultation on Incontinence Questionnaire-Urinary Incontinence Short Form scores of 11 (21 to -1) of 21 and in POP-Urinary Incontinence Sexual Questionnaire scores of 9.5 (6.5-33.0) of 48.0. With regard to urinary continence, 6 (54.5%) patients presently reported no incontinence, 3 (27.3%) reported mild incontinence, and 2 (18.2%) reported continuous incontinence. CONCLUSIONS: Pelvic organ prolapse poses significant reductions in quality of life for women born with exstrophy, with effects on urinary continence and sexual function. Identification and correction of prolapse seems to result in notable improvements in the lives of these patients.

Sexual Function and Quality of Life Before and After Penile Prosthesis Implantation Following Radial Forearm Flap Phalloplasty.

OBJECTIVE: To provide sexual function and quality of life outcomes in patients with severe penile deficiency who underwent radial forearm flap phalloplasty with and without penile prosthesis implantation. PATIENTS AND METHODS: Patients with history of severe penile deficiency who underwent microsurgical radial forearm flap phalloplasty with and without penile prosthesis implantation between 2007 and 2014 were identified. They completed a set of web-based validated questionnaires including the International Index of Erectile Function, the Pediatric Penile Perception Score, the Sexual Quality of Life for Men, and several items addressing general quality of life. Outcomes were compared between groups. RESULTS: Nine of the 12 identified patients who had prosthesis after phalloplasty and 4 out of the 7 phalloplasty-only patients completed the survey, resulting in an overall response rate of 68%. Among the phalloplasty-prosthesis patients, 66% reported current sexual activity and 78% reported regular masturbation, whereas 1 of the 4 phalloplasty-only patients reported both. Prosthesis patients scored notably higher in all domains of the International Index of Erectile Function except for sexual desire. In contrast, they demonstrated similar scores of penile perception, as well as general and sexual quality of life. CONCLUSION: Among patients who have undergone flap phalloplasty, the subsequent placement of penile prosthesis appears to effectively allow for both intercourse and masturbation, resulting in measurable improvements in orgasmic function, intercourse satisfaction, and overall sexual satisfaction. Despite these important benefits, prosthesis placement does not appear to result in improvements in penile perception scores, or general or sexual quality of life.

Profound Hematuria in a Toddler Yields an Unusual Diagnosis.

Herein we present a rare case of profound recurrent gross hematuria in a young child with no known predisposing event. She was eventually diagnosed with a large lymphovascular malformation of the bladder. She underwent multiple unsuccessful attempts at embolization before eventual curative partial cystectomy.

Continence in the cloacal exstrophy patient: What does it cost?

BACKGROUND: Surgical advancements have made cloacal exstrophy (CE) a survivable condition, though management remains complex. Urologic, orthopedic, colorectal and gynecologic interventions are not standardized, and the cost of this care is high. While the importance of a successful primary closure in terms of outcomes is known, the economic consequences of failure remain uncharacterized. METHODS: A prospectively maintained institutional database of epispadias-exstrophy complex patients was reviewed for continent CE patients. Hospital charges for all inpatient admissions prior to achieving urinary continence were inflation-adjusted to year 2013 values using Consumer Price Index for medical care published by the United States Bureau of Labor Statistics. Records for which charge data were incomplete were completed by using single mean imputation, also inflation-adjusted. Descriptive data are presented as mean±standard deviation (SD). RESULTS: Of 102 CE patients, 35 had available hospital charge data: 15 who underwent successful primary closure at the authors' institution and 20 who presented after previously failed primary closures at referring institutions. The mean±SD hospital charges for primary closure in the success group were $136,201±$48,920. These patients then underwent subsequent additional surgeries that accrued charges of $59,549±$25,189 in order to achieve continence. Overall, successful primary closures accumulated hospital charges of $200,366±$40,071. In comparison, patients referred after prior failure required significantly more hospital admissions and additional charges of $207,674±$65,820 were required to achieve continence (p<0.001). Patients who failed primary closure are estimated to accumulate 70% more total health care charges compared to the group following successful primary closure. CONCLUSION: The cost of CE management until urinary continence is high, averaging more than $200,000 in inpatient hospital charges alone. Initial success is desirable from both an outcomes and economic perspective, as the cost of salvaging a failed primary closure at our institution is similar to the overall costs of a successful closure; this is in addition to the cost of any previous failed closures. Further studies will be required to determine the optimal timing of surgical management in terms of both patient outcomes and financial consequences.

Newborn exstrophy closure without osteotomy: Is there a role?

INTRODUCTION: Recent articles document successful classic bladder exstrophy (CBE) closure without osteotomy. Still, many patients require osteotomy if they have a large bladder template and pubic diastasis, or non-malleable pelvis. OBJECTIVE: To understand the indications and outcomes of bladder closure with and without pelvic osteotomy in patients younger than 1 month of age. METHODS: An institutional database of 1217 exstrophy-epispadias patients was reviewed for CBE patients closed at the authors' institution within the first month of life. Patient demographics, closure history, pubic diastasis distance, bladder capacity, and outcomes were recorded and compared using chi-square tests between osteotomy and non-osteotomy patients. Failure was defined as bladder dehiscence, prolapse, vesicocutaneous fistula, or bladder outlet obstruction requiring reoperation. Bladder capacity >100 mL was deemed sufficient for bladder neck reconstruction (BNR). RESULTS: One hundred CBE patients were included for analysis: 38 closed with osteotomy (26 male, 12 female), and 62 closed without osteotomy (42 male, 20 female). There were four failed closures in the osteotomy group (2 dehiscence, 2 prolapse) and four failed closures in the non-osteotomy group (2 dehiscence, 2 prolapse). This corresponded to statistically equivalent rates of failure between the osteotomy and non-osteotomy groups (10.5% vs. 6.5%, p = 0.466). There was no statistically significant difference between the groups' ability to achieve bladder capacity sufficient for BNR (82% vs. 71%, p = 0.234). DISCUSSION: A successful primary bladder closure, regardless of the use of osteotomy, has been shown to be the single most important predictor of eventual continence. Because of the complexity of exstrophy manifestations, a multidisciplinary team approach is of the utmost importance. Based on our institutional experience, closure without osteotomy is considered when patients are <72 h of life, have a pubic diastasis <4 cm, malleable pelvis, and pubic apposition without difficulty. Rates of successful closure and attaining sufficient capacity for BNR were both statistically equivalent across groups. This retrospective study is limited by selection bias and the significant difference in follow-up time between groups. Nevertheless, as a high-volume exstrophy center this study draws from one of the largest cohorts available. CONCLUSIONS: Regardless of the type of closure undertaken, there clearly is a role for newborn CBE closure without pelvic osteotomy in patients considered suitable for closure by both the pediatric urologist and orthopedic consultant. However, if there is any doubt concerning pubic diastasis width, pelvic malleability, or ease of pubic apposition, an osteotomy is highly recommended.

Pediatric and adolescent renal cell carcinoma.

OBJECTIVE: Renal cell carcinoma (RCC) is an uncommon malignancy among children and adolescents. Because of this, there has been relatively sparse research and evidence on the topic. As the body of research regarding pediatric and adolescent RCC has developed in recent years, it has become increasingly clear that it demonstrates important differences from the much more common adult-type RCC. This review aims to examine and summarize the current literature, with a focus on the ways that pediatric and adolescent RCC differ from the adult disease, and to make recommendations for evaluation and management based on this evidence whenever possible. METHODS AND MATERIALS: A thorough search of all articles relating to pediatric and adolescent RCC has been undertaken using PubMed. The reference lists from all relevant articles have been further reviewed, to ensure inclusion of all pertinent literature. CONCLUSION: The most significant development in recent years has been the realization that most of the pediatric and adolescent RCC cases, but only a very small fraction of adult RCC cases, demonstrate "translocation tumor" pathology. It is likely that such differences may eventually explain many of the previous observations regarding differences in behavior of RCC among children and teenagers. At this point, however, the relevance of translocation pathology to clinical management remains unclear, and so most continue to treat these patients in much the same way as those with the more conventional tumor subtypes.

The cost of failure: The economic impact of failed primary closure in classic bladder exstrophy.

PURPOSE: Failure of primary closure in classic bladder exstrophy (CBE) is a significant cause of morbidity, and yet its relative economic impact has not been well characterized. The authors aim to determine whether CBE patients who underwent failed primary closure incur greater economic burden in the year following their successful closure than those patients who underwent a successful primary closure. MATERIALS AND METHODS: After institutional review board approval CBE patients who were successfully closed between 1993 and 2013 were identified in an institutional exstrophy-epispadias database. Patients who were never closed at the study institution and those who had no documented successful closure were excluded. Inpatient hospital charges, hospital costs, and professional fees were collected for the year following successful closure. RESULTS: 162 patients met the inclusion and exclusion criteria and accounted for 312 inpatient admissions in the year following and including their respective successful bladder closures. 62 of the patients failed their primary closure and the remaining 100 succeeded. Adjusting for covariates, patients who underwent successful primary closure experienced a reduction in inpatient hospital charges of $8497, hospital costs of $9046 and professional fees of $11,180 in the year following their successful closure compared to those patients who failed their primary closure. CONCLUSION: Apart from the self-evident financial advantages of a successful primary closure, namely the avoidance of reclosure, there appears to be a lasting negative financial impact of failed primary closure even after these patients undergo successful reclosure at the study institution.

The Tunica Vaginalis Flap as an Adjunct to Epispadias Repair: A Preliminary Report.

OBJECTIVE: To report our preliminary institutional experience of incorporating a tunica vaginalis flap (TVF) as an adjunct into primary or secondary epispadias repair. PATIENTS AND METHODS: A prospectively maintained institutionally approved database of exstrophy-epispadias complex patients was used to identify and retrospectively review male patients who underwent epispadias repair from September 2010 to October 2014 at the authors' institution. Patients who underwent epispadias repair with TVF were identified and their clinical outcomes were measured. RESULTS: A total of 49 male patients were identified as meeting inclusion criteria, of which 15 (2 isolated epispadias, 13 classic bladder exstrophy) underwent epispadias repair incorporating a TVF. Median age at time of repair was 12 months (interquartile range [IQR] 10-15.5). A median of 4 layers (IQR 4-5) was incorporated into each repair closure, applying EVICEL Fibrin Sealant as an additional layer in all patients. All patients received preoperative testosterone injection therapy of 2 mg/kg 5 and 2 weeks before surgery for penile growth. There were no intraoperative complications. Median follow-up of 19 months (IQR 12-23) revealed 5 patients who underwent epispadias revision, 3 (20%) of which developed a urethrocutaneous fistula. All patients had a successful repair without recurrence. CONCLUSION: The authors have found the utilization of a TVF with epispadias repairs to be beneficial but no better than the repair with our routine soft-tissue coverage in primary epispadias repair. However, in patients presenting with complex anatomy and limited tissue reserves, a TVF is an important adjunct to epispadias repair and/or revision and may lead to improved outcomes.

Secondary reclosure in classic bladder exstrophy: challenges and outcomes.

OBJECTIVE: To analyze the outcomes of failed classic bladder exstrophy (CBE) reclosure with and without pelvic osteotomy. Each failed CBE closure decreases the chance of eventual continence. To minimize secondary failures, most institutions use pelvic osteotomy with reclosure. Reclosure with and without osteotomy can still fail. METHODS: An institutional database of 1210 exstrophy-epispadias complex patients was reviewed for CBE patients who had 2 prior failed closures with the third closure at the authors' institution. Patient demographics, closure history, diastasis distance, bladder capacity, and outcomes were examined by chi-square tests comparing osteotomy status with first reclosure. RESULTS: Of 848 CBE patients, 17 met inclusion criteria: 12 with osteotomy at reclosure (group 1) and 5 without (group 2). Median time between initial closure and reclosure in the 2 groups were 6.5 months (range, 0-42 months) and 3 months (range, 0-59 months), respectively. There was no significant difference in the rate of attaining sufficient bladder capacity for bladder neck reconstruction (BNR; 100 cc) between groups 1 and 2 (42% vs 40%; P = .490). Within group 1, patients receiving proper immobilization with external fixation (n = 5) demonstrated a significantly greater rate of attaining sufficient bladder capacity for BNR compared with patients who did not (80% vs 14%; P = .023). There were no differences in the rates of attaining dryness per urethra. CONCLUSION: CBE outcomes worsen with each successive failed closure. Reclosure should be performed with osteotomy and proper immobilization to maximize the chance of sufficient capacity for BNR or augmentation cystoplasty.

Ureteral reimplantation before bladder neck reconstruction in modern staged repair of exstrophy patients: indications and outcomes.

OBJECTIVE: To evaluate a single referral center's experience with vesicoureteral reflux (VUR) and its role in management before bladder neck reconstruction (BNR) in exstrophy patients after modern staged repair of exstrophy (MSRE). VUR after primary closure of bladder exstrophy can usually be managed conservatively until ureteral reimplantation (UR) at the time of BNR. However, VUR in these patients is occasionally associated with febrile urinary tract infection and worsening renal function necessitating UR before BNR. MATERIALS AND METHODS: A total of 199 patients who underwent MSRE were identified from an institutional review board-approved database. Patient demographics, closure history, postprimary closure VUR, history of urinary tract infection, cystography outcomes, renal function, history of UR before BNR, and UR outcomes were recorded. Univariate and multivariate analyses were performed to determine factors predicting high-grade VUR and the need for pre-BNR UR. RESULTS: After primary closure, VUR was observed in 150 patients (75.4%) of whom 31 (15.58%) had high-grade VUR. Closure without osteotomy and postclosure outlet obstruction were independently associated with postprimary closure high-grade VUR (P <.05). Twenty-two patients (11.06%) required pre-BNR UR. Female gender and those with high-grade VUR were more likely to require UR before BNR (P <.05). Early UR did not affect the continence rate. CONCLUSION: VUR after exstrophy closure can generally be managed with conservative treatment and periodic monitoring of the upper urinary tracts. About 11% of patients undergoing MSRE will require UR before BNR. Closure without osteotomy and patients who develop outlet obstruction after closure are at increased risk for developing high-grade VUR.

Modern management of the exstrophy-epispadias complex.

The exstrophy-epispadias complex is a rare spectrum of malformations affecting the genitourinary system, anterior abdominal wall, and pelvis. Historically, surgical outcomes were poor in patients with classic bladder exstrophy and cloacal exstrophy, the two more severe presentations. However, modern techniques to repair epispadias, classic bladder exstrophy, and cloacal exstrophy have increased the success of achieving urinary continence, satisfactory cosmesis, and quality of life. Unfortunately, these procedures are not without their own complications. This review provides readers with an overview of the management of the exstrophy-epispadias complex and potential surgical complications.

Safety and efficacy of staged pelvic osteotomies in the modern treatment of cloacal exstrophy.

INTRODUCTION AND OBJECTIVE: Staged pelvic osteotomy (SPO) prior to bladder closure has been shown to be a safe and effective method for achieving pubic approximation in cloacal exstrophy (CE) patients with extreme diastasis. However, SPO outcomes have never been compared to those for combined pelvic osteotomy (CPO) at the time of closure in CE patients. METHODS: A prospectively maintained database of 1208 exstrophy-epispadias complex patients was reviewed for CE patients treated with pelvic osteotomies. Inclusion criteria were osteotomy at the authors' institution and closure within two months of osteotomy. After inclusion, patients were separated into four groups depending on osteotomy procedure (SPO vs. CPO) and whether their osteotomy occurred with primary closure or re-closure. Patient demographics, closure history, pre-operative diastasis measurement, most recent post-operative diastasis measurement, and outcomes were recorded and compared by chi-squared tests and ANOVA. RESULTS: Among 116 CE patients reviewed, 46 met inclusion criteria. With primary closure or re-closure, 27 had SPO and 19 had CPO. No SPO re-closure patients had previous osteotomy; 4 CPO re-closure patients had a previous osteotomy with closure. Median time between osteotomy and closure in SPO patients was 14 days. Median follow-up after SPO and CPO were 4 and 11 years, respectively. SPO significantly reduced the pre-operative diastasis compared to CPO on most recent diastasis measurement (3.5 cm vs. 0.4 cm, p=0.003). There were no significant differences in the overall complication rate, or the rates of each specific complication, between the SPO and CPO groups. No patients had wound dehiscence or prolapse. One CPO patient was able to intermittently catheterize per urethra while all other patients required continent urinary diversion to achieve continence. CONCLUSIONS: To the authors' knowledge, this is the first study comparing SPO and CPO outcomes in CE patients. SPO reduces pre-operative diastasis more than CPO, and does not appear to incur increased rates of complication, closure failure, or incontinence. Due to its apparent safety and greater efficacy, SPO should be considered in all CE patients with extreme diastases undergoing primary closure or re-closure.

New advances in the pathophysiologic and radiologic basis of the exstrophy spectrum.

The exstrophy-epispadias complex is a rare spectrum of anomalies affecting the genitourinary system, anterior abdominal wall, and pelvis. Recent advances in the repair of classic bladder exstrophy (CBE) and cloacal exstrophy (CE) have resulted in significant changes in outcomes of surgical management (including higher continence rate, fewer surgical complications, and better cosmesis) and health-related quality of life in these patients. These noteworthy changes resulted from advances in the pathophysiological and genetic backgrounds of this disease and better radiologic assessment of the three-dimensional anatomy of the bony pelvis and its musculature. A PubMed search was performed with the keyword exstrophy. The resulting literature pertaining to genetics, stem cells, imaging, tissue engineering, epidemiology, and endocrinology was reviewed. The following represents an overview of the advances in basic science understanding and imaging of the exstrophy-epispadias spectrum and discusses their possible and future effects on the management of CBE and CE.

Renal pelvis rupture in a kidney with ureteropelvic junction obstruction and extrarenal calyces.

The extrarenal calyx (ERC) is a rare congenital anomaly, associated with various other abnormalities of the urogenital system. We report a unique case of ERC in a solitary functioning kidney with a massively dilated pelvis that developed spontaneous rupture. A dismembered pyeloplasty was performed at the time of rupture. The patient did well post-operatively with a stable creatinine and stable SFU grade 2 hydronephrosis.