Bladder Management and Continence in Girls With Cloacal Malformation After 3 Years of Age.

BACKGROUND: Multiple factors impact ability to achieve urinary continence in cloacal malformation including common channel (CC) and urethral length and presence of spinal cord abnormalities. Few publications describe continence rates and bladder management in this population. We evaluated our cohort of patients with cloacal malformation to describe the bladder management and continence outcomes. METHODS: We reviewed a prospectively collected database of patients with cloacal malformation managed at our institution. We included girls ≥3 years (y) of age and evaluated their bladder management methods and continence. Dryness was defined as <1 daytime accident per week. Incontinent diversions with both vesicostomy and enterovesicostomy were considered wet. RESULTS: A total of 152 patients were included. Overall, 93 (61.2%) are dry. Nearly half (47%) voided via urethra, 65% of whom were dry. Twenty patients (13.1%) had incontinent diversions. Over 40% of the cohort performed clean intermittent catheterization (CIC), approximately half via urethra and half via abdominal channel. Over 80% of those performing CIC were dry. In total, 12.5% (n = 19) required bladder augmentation (BA). CC length was not associated with dryness (p = 0.076), need for CIC (p = 0.253), or need for abdominal channel (p = 0.497). The presence of a spinal cord abnormality was associated with need for CIC (p = 0.0117) and normal spine associated with ability to void and be dry (p = 0.004) CONCLUSIONS: In girls ≥ 3 y of age with cloacal malformation, 61.2% are dry, 65% by voiding via urethra and 82% with CIC. 12.5% require BA. Further investigation is needed to determine anatomic findings associated with urinary outcomes. LEVEL OF EVIDENCE: IV.

Operative duration as independent predictor of increased postoperative healthcare utilization.

PURPOSE: Few studies have examined the risk factors for postoperative healthcare resource utilization (HRU) among minimally invasive partial nephrectomy (MIPN), minimally invasive prostatectomy (MIP), and cystectomy (Cx). The aim of this study is to assess if operative duration (OD) is a predictor of HRU in this population. METHODS: The ACS-NSQIP database was filtered for MIPN, MIP, and Cx. Patient characteristics and intraoperative variables were examined. HRU was defined as prolonged length of stay (LOS), unplanned readmission within 30 days, and discharge to continued care facility. Multivariate regression analysis was used to identify independent predictors of HRU. RESULTS: 18,904 MIPN, 50,807 MIP, and 12,451 Cx were included. For MIPN, HRU was seen in 13.9% of cases < 1.75 h, increasing to 36.2% in OD > 4.5 h (p < 0.001). For MIP, HRU was seen in 10.6% of OD < 2 h, increasing to 32.2% for OD > 4.9 h (p < 0.001). For Cx, 57% of those with OD > 8.5 h required HRU compared to 42.1% for OD < 3.3 h (p < 0.001). On multivariate analyses, OD was an independent predictor for increased HRU for all procedures regardless of patient characteristics or comorbidities. For MIPN, OD > 4.5 h had 3.5-fold increased use of HRU (p < 0.001). For MIP, OD > 5 h had 3.7-fold increased use of HRU (p < 0.001). For Cx, OD > 8.5 h demonstrated a twofold increased use of HRU (p < 0.001). CONCLUSIONS: OD during MIPN, MIP, and Cx is an independent predictor of increased HRU irrespective of patient comorbidities. Patients with OD > 4.5 h for MIPN, > 5 h for MIP, and > 8.5 h for Cx have 3.5-fold, 3.7-fold, and twofold increased risk of HRU, respectively.

Novel pelvic immobilization method following bladder exstrophy repair.

We introduce a novel method of pelvic immobilization following bladder exstrophy repair involving a custom-made brace that offers adequate pelvic immobilization with the added benefits of no added operative time, easy evaluation of surgical sites, simplified wound care including sponge bathing, and overall less cumbersome management for the family given its more streamlined size.

Gonadal tumors in a contemporary cohort of patients with differences in sex development undergoing surgery - A multi-site study from the Pediatric Urologic Oncology Working Group of the societies for pediatric urology.

BACKGROUND: Disturbances in gonadal development lead to increased risk of gonadal malignancy in some but not all patients with differences in sex development (DSD). However, the natural history of these tumors is poorly described, and the literature remains sparse. OBJECTIVE: The objective of this study was to describe the incidence of germ cell neoplasia in situ (GCNIS) and germ cell tumor (GCT) in a contemporary cohort of patients with DSD undergoing surgery and to provide long-term oncologic outcomes for these patients. STUDY DESIGN: Patients with DSD who have undergone gonadectomy or gonadal biopsy were identified at four institutions. Clinical characteristics, pathology, and treatment details were obtained retrospectively. Patients were stratified into risk categories based on DSD diagnosis. Oncologic treatment and outcomes were recorded. Descriptive statistics are reported using parametric methods. RESULTS: 83 patients were identified. Distribution of diagnoses is summarized in the summary table. 14 (16.9%) patients underwent gonadal biopsy, and 71 (85.5%) patients underwent gonadectomy (50/71 gonadectomies were bilateral). 8/83 (9.6%) patients had GCNIS or GCT (7 GCNIS, 1 GCT). Median age at surgery was 2.95 years (y) (interquartile range [IQR] 0.6-12.2) and 14y (IQR 0.85-16.9) in patients without and with GCNIS/GCT, respectively. All 8 patients with GCNIS/GCT had high or intermediate risk DSD diagnoses (4 mixed gonadal dysgenesis, 3 Turner with Y, 1 partial gonadal dysgenesis). Of the patients with high-risk diagnoses, 8/54 (15%) had GCNIS/GCT. No patient received adjuvant therapy, no patient had a recurrence, and all patients were living with mean follow up 6.4y. DISCUSSION: The risk of gonadal malignancy is heterogeneous in the DSD population and can vary based on DSD diagnosis as well as maturation, testicularization, and location of the gonads. The most recent consensus recommendations on gonadal management emphasize risk stratification and consideration of gonadal surveillance based on gender of rearing, but supporting literature remains sparse. In this contemporary cohort of DSD patients who underwent gonadal surgery, most patients did not have evidence of adverse pathology, all patients with malignant or premalignant pathology had a high/intermediate risk DSD diagnosis, and all patients with GCNIS/GCT were treated with surgery alone without recurrence. CONCLUSIONS: The distribution of patients with premalignant and malignant gonadal pathology and DSD in this cohort aligns with prior literature, and oncologic outcomes were excellent. These data add valuable information to the current literature and highlight the necessity to develop appropriate screening regimens for retained gonads.

Unusual Renal Mass in a Pediatric Patient.

Renal cell carcinoma (RCC) is rare in the pediatric population, comprising about 5% of renal neoplasms in children.1 Out of all childhood cases of RCC, translocation RCCs (tRCC) is the most common.2 It is well described in the literature that exposure to alkylating agents such as cyclophosphamide and/or topoisomerase II inhibitors such as doxorubicin and etoposide, is a risk factor for the development of Xp11 (or TFE3) tRCC.3 Herein is a case of tRCC development in a patient with history of exposure to topoisomerase II inhibitors and alkylating agents to treat a common childhood malignancy.

Recurrence rates after surgical management of labial adhesions.

INTRODUCTION: Labial adhesions (LA) involves fusion of the labia minora in the midline. LA can range from involving just a small portion of the labia minora or the entire length, covering the urethra and vaginal opening. Treatment for labial adhesions includes the use of topical creams, manual lysis or surgical separation. The recurrence rate has been reported as 55%, 33% and 0% respectively. Only one study with a cohort of 9 patients examines recurrence rates after surgical release (0%). OBJECTIVE: To verify the previous findings that surgical repair has low recurrence rate. We hypothesize the recurrence rate of LA after surgical repair to be lower than more conservative methods but that the recurrence rate is not 0%. STUDY DESIGN: A retrospective review was performed of consecutive cases of LA that were treated surgically from August 2018 to October 2020. The surgical technique used involved opening the LA bluntly then oversewing each labia with fine absorbable suture. All patients were scheduled for clinic or telephone follow up 3 months postoperatively. Patient demographics, indication for procedure, history of prior treatment, operative time, complications, recurrence and length of follow up were recorded and are described using non-parametric methods. RESULTS: Thirty-two female patients underwent surgical treatment of LA using the above-described technique. Median age at surgery was 3.7 y (IQR 2.7-5.3). Most patients (78.1%) had failed prior initial treatment, most commonly topical estrogen creams. Median follow up was 4.3 mos with 10 patients being lost to follow up. Only 2 patients experienced a recurrence at a median of 3.5 months. CONCLUSIONS: This series verifies prior findings that, in the short-term, surgical repair has a lower recurrence rate than topical treatment or manual lysis. However, the recurrence rate is 9%, higher than previously reported. Further studies into longer-term follow up are underway.

Gonadal malignancy in patients with differences of sex development.

Differences of sexual development (DSD) are known to be associated with an elevated risk of malignant and pre-malignant tumors. However, given the rarity of DSD and tumors in patients with DSD, more robust, large scale, prospective literature is required to truly determine the extent of this association, long-term outcomes and the nuances associated with the wide variety of DSD diagnoses. In addition, the spectrum of diagnoses and nomenclature has been ever-changing, limiting assessment of long-term patient outcomes. This review aims to provide an overview of the pathogenesis of DSD conditions, potential malignancies associated with the diagnoses, the available screening for malignancy, and the most recent data on stratification for each DSD diagnosis and association with malignancy.

Oncologic outcomes of pre-malignant and invasive germ cell tumors in patients with differences in sex development - A systematic review.

OBJECTIVE: To describe the rates of GCNIS-free and GCT-free pathology based on age at gonadal surgery and to describe long-term oncologic outcomes in patients with DSD who have GCNIS or GCT at the time of gonadal surgery. STUDY DESIGN: A systematic review was conducted using MEDLINE to identify patients with DSD who underwent gonadal surgery. DSD diagnoses were stratified based on malignancy risk. GCNIS/GCT and GCT-free survival by age of gonadal surgery, RFS and OS were calculated using the Kaplan-Meier method, with groups compared using log-rank testing. RESULTS: 386 articles from 1951 to 2017 were included (2037 patients). Median age at gonadal surgery was 17 years (y) (IQR 11-20), median follow-up was 60 months (m) (IQR 30-68.1). GCNIS/GCT- and GCT-free survival at the time of gonadal surgery was lowest for those in the high/intermediate risk group (p < 0.001) but decreased sharply around age 15y, regardless of risk category. 5y RFS and OS was similar for those with no GCNIS/GCT and GCNIS and was worse for those with GCT (p < 0.001). DISCUSSION: When patients undergo gonadal surgery, regardless of indication (i.e. prophylactic vs. tumor), it appears that GCTs are more commonly found when surgery is done around age 15 y or older, despite risk category. This is similar to ovarian and testicular GCTs. Patients with GCNIS can be reassured that long-term oncologic outcomes are excellent. While RFS and OS for GCTs are not as good as for ovarian and testicular GCTs (95%), they are still >80%. This similar trend was found in a COG review of 9 patients with DSD and ovarian GCT. There were several limitations to this study. This is a retrospective analysis that included aa wide time frame of publications. The indication for surgical intervention was not addressed in the majority of publications. Thus these data provide pathologic outcomes based on age at gonadal surgery rather than the age at which GCNIS/GCT develops over a lifetime, if at all. CONCLUSIONS: The risk of GCNIS or GCT at the time of gonadal surgery appears to increase with age, accelerating between 15 and 20y regardless of risk category. 5y RFS and OS for those with GCNIS is equivalent to those without GCNIS/GCT but is worse for those with GCT. These data may be used when counseling families on timing of gonadal surgery and quantification of outcomes should GCNIS or malignancy be identified.

Racial disparities in bladder management in veterans with spinal cord injury and disorders.

AIMS: Neurogenic lower urinary tract dysfunction is common in individuals with spinal cord injury and disorders (SCI/D). The purpose of this study was to identify specific demographic, neurologic, and urologic factors associated with different bladder management methods (BMMs) in individuals with SCI/D. METHODS: A retrospective review of BMMs at a large Veterans Affairs SCI/D center was performed to identify associated risk factors including demographics, neurologic factors, and urologic factors. Bivariate analysis was performed to identify factors associated with specific BMMs. Then, a propensity-matched racial group analysis was performed to identify independent factors associated with differences in BMM. RESULTS: Data from 833 patients with SCI/D were reviewed and included 52.1% Caucasians and 39.6% African Americans. On bivariate analysis, current age, years since the injury, the severity of functional impairment, nonmedical mechanism of injury, and Caucasian race were associated with increased rates of indwelling catheter use. In an analysis of propensity-matched racial groups, African-American race was found to be an independent risk factor for not using indwelling catheters on multivariate analysis (odds ratio = 0.55). This finding was not related to access to care, as the rate of urodynamic testing was similar between races ( P = 0.174). CONCLUSIONS: Caucasians were more likely to use indwelling catheters and less likely to use conservative BMMs despite proper urodynamic evaluation. The racial discrepancy suggests a need for future research aimed at identifying unknown psychosocial factors associated with the use of indwelling catheters in individuals with SCI/D.

Assessment of communication technology and post-operative telephone surveillance during global urology mission.

OBJECTIVE: Compliance with post-operative follow-up in the context of international surgical trips is often poor. The etiology of this problem is multifactorial and includes lack of local physician involvement, transportation costs, and work responsibilities. We aimed to better understand availability of communication technologies within Belize and use this information to improve follow-up after visiting surgical trips to a public hospital in Belize City. Accordingly, a 6-item questionnaire assessing access to communication technologies was completed by all patients undergoing evaluation by a visiting surgical team in 2014. Based on this data, a pilot program for patients undergoing surgery was instituted for subsequent missions (2015-2016) that included a 6-week post-operative telephone interview with a visiting physician located in the United States. RESULTS: Fifty-four (n = 54) patients were assessed via survey with 89% responding that they had a mobile phone. Patients reported less access to home internet (59%), local internet (52%), and email (48%). Of 35 surgical patients undergoing surgery during 2 subsequent surgical trips, 18 (51%) were compliant with telephone interview at 6-week follow-up. Issues were identified in 3 (17%) patients that allowed for physician assistance. The cost per patient interview was $10 USD.

Prostate Cancer in Transgender Women: Incidence, Etiopathogenesis, and Management Challenges.

OBJECTIVE: To critically analyze the available evidence regarding the incidence, etiopathogenesis, and management of prostate cancer (CaP) in transgender women. In addition, this article aims to present a recent case report of a transgender woman with a unique presentation at the author's institution. MATERIALS AND METHODS: An electronic nonsystematic literature search was performed to identify pertinent studies. PubMed search engine was queried by using the following search terms: "prostate cancer," "male to female transsexual," "transgender patient," "androgen + prostate cancer," "estrogen therapy + prostate cancer," and "health care barrier." In addition, a clinical case managed at our institution was reviewed and critically discussed. RESULTS: Including our case, there have been only 10 documented cases of CaP in transgender women. Additionally, an emerging body of literature has questioned the role of androgens in the development of CaP and suggested that estrogen therapy may not be as protective as initially thought. Therefore, the current evidence suggests that the transgender woman should be screened for CaP the same as a nontransgender men. Barriers to care in the transgender female population include accessing resources, medical knowledge deficits, ethics of transition-related medical care, diagnosing vs pathologizing transgender patients, financial restrictions of the patient, and health system determinants. CONCLUSION: Although rare, CaP in transgender women has been documented. Both the mechanism and the impact of receiving a bilateral orchiectomy on disease development are unclear. Future study is needed to examine these factors, and to further shape the treatment and screening regimen for these patients.