A Contemporary Review of Cryptorchidism Management in Adults: A Rare Presentation of Bilateral Cryptorchidism Presenting as Pelvic Pain in an Adult Patient.

This case report presents a rare case of adult cryptorchidism, found incidentally in a 25-year-old gentleman who initially presented with abdominal and suprapubic pain and was successfully treated with staged orchidopexy. To our knowledge, to date, our case is the first published instance of bilateral cryptorchidism in an adult presenting with nonspecific suprapubic pain. Cryptorchidism is the most common genital abnormality in newborn boys, and due to its association with an increased risk of infertility and malignancy, current management involves surgical correction with orchidopexy by 12 to 18 months of life. Adult presentation of cryptorchidism is very unusual due to early intervention; therefore, bilateral cryptorchidism is even more rare. As a result, current guidelines do not address proper management for adult cryptorchidism. Therefore, after performing a thorough review of the literature on contemporary guidelines for cryptorchidism management, we aim to highlight our approach to management in this rare case of adult bilateral cryptorchidism. We suggest bilateral orchiectomy as the safest option, if the patient is amendable, or bilateral orchiopexy with long-term follow-up for testicular cancer. Although the American Urological Association guidelines recommend orchiectomy for postpubertal cryptorchid children, currently, no explicit guidelines exist for the preferred method of managing adult cryptorchidism. Due to the increased risk of infertility and testicular cancer with cryptorchidism, orchiectomy instead of orchiopexy may be the preferred surgical approach in some instances. Still, in the case of bilateral cryptorchidism, orchiectomy may not always be the most viable solution, making orchiopexy with long-term follow-up for testicular cancer the best option, such as in our case.

Surge in testicular torsion in pediatric patients during the COVID-19 pandemic.

BACKGROUND: Testicular torsion is a pediatric surgical emergency, and prompt diagnosis and treatment is imperative. During the COVID-19 pandemic, pediatric patients with symptoms of testicular torsion may be reluctant to seek medical care which increases the likelihood of delayed presentation and the need for an orchiectomy. This observational study sought to determine whether there was a higher number of testicular torsion cases during COVID-19. METHODS: As the first patient with COVID-19 was admitted to our facility on March 6, 2020, we identified male children ages 1-18 years with testicular torsion between March 1-December 31, 2020 (during COVID-19) compared to the same time period between 2015 and 2019 (prior to COVID-19). All patients were evaluated at our Institution's Emergency Department by a pediatric urologist. RESULTS: There were 38 cases of testicular torsion between March 1-December 31, 2020 compared to 15.8 cases on average during the same 10-month period between 2015 and 2019 (a total of 79 cases). There was a statistically significant increase in testicular torsion cases during the COVID-19 pandemic compared to equivalent time periods in 2015-2019 (38 vs. 15.8, p = 0.05). Patients with testicular torsion during the COVID-19 pandemic were younger, had a longer duration of symptoms, and had a higher number of orchiectomies (although not statistically significant). CONCLUSION: During the COVID-19 pandemic, an escalation in testicular torsion cases was observed. Timely assessment, diagnosis, and surgery are crucial to prevent testicular loss and potential infertility in the future. Further evaluation is needed to elucidate the surge in testicular torsion and possible mechanisms.

Case Report: Novel Copy Number Variant 16p11.2 Duplication Associated With Prune Belly Syndrome.

Prune belly syndrome (PBS) is a rare congenital disease that predominantly occurs in males and is identified by its classic triad of abdominal wall musculature deficiencies, cryptorchidism, and urinary tract abnormalities. However, numerous anomalies involving the kidneys, heart, lungs, and muscles have also been reported. A multitude of chromosomal abnormalities have been implicated in its pathogenesis. PBS can occur in association with trisomy 18 and 21. Gene duplications and deletions have also been reported; however, a definite cause of PBS is still unknown. We report the first PBS patient with a copy number variant in 16p11.2.

Case Report: Rare Presentation of Mixed Germ Cell Tumor in an Infant.

The estimated incidence of pediatric testis tumor is 0.5-2.0 per 100,000 children, accounting for 1-2% of all pediatric tumors. Mixed germ cell tumors (MGCT) in prepubertal males are exceedingly rare, with only one previous case report found in the literature. We report a case of a MGCT in an infant. For prepubertal males, GCTs typically present with a painless scrotal mass, though trauma, testis torsion and hydrocele are also common presentations. Similar to such tumors in postpubertal males, ultrasonography, computed tomography, and tumor markers are integral to determine the best treatment. The patient described in this report presented with a painless scrotal mass. Following orchiectomy, the patient was found to have MGCT that was limited to the testis. With prudent management, these patients tend to have favorable prognoses.

Conservative Management for Urethral Foreign Body: A Case Report of an Adolescent Boy With Repeated Events.

Placing foreign bodies into the urethra is not a common occurrence in the general population. Patients self-insert foreign bodies for a multitude of reasons such as sexual gratification, secondary gain, and psychiatric illness. From our own experience and what has been reported in the literature, there is a wide variability in the type of objects that patients place into the urethra. We report a unique case of a 17-year-old adolescent boy with repeated foreign body insertions into the urethra over a 1-year period. This patient suffers from significant psychiatric illness. Due to the number of events in this past year, we initiated a conservative observational approach that contrasts the traditional invasive protocol to treat with endoscopic removal. This management has proven to be successful in his case and can be replicated in other scenarios after careful consideration of the clinical presentation.

Long-term urological and gynecological outcomes following complete primary repair in females with bladder exstrophy.

INTRODUCTION: Long-term continence outcomes in patients undergoing complete primary repair of exstrophy (CPRE) have shown that a subset of patients do not achieve continence until after puberty. We aim to update the continence outcomes as well as describe gynecological outcomes for females having undergone CPRE. MATERIALS AND METHODS: This was a retrospective review between 1989 and 2019 at a single institution. Inclusion criteria were females with classic bladder exstrophy who underwent CPRE. Exclusion criteria were variant diagnoses and age <4 years. Continence was defined as dry for ≥3 h (volitional voiding or clean intermittent catheterization (CIC)). Partial continence was defined as minor stress incontinence or enuresis. Vaginal stenosis was evaluated in post-pubertal patients. RESULTS: Eighteen patients met inclusion criteria. Median age at last follow-up was 15.9 years (IQR 13.1, 18.4). All patients underwent primary closure <30 days of life (n = 18). Subsequent procedures included bladder augmentation (n = 4), continent catheterizable channel (n = 7), bladder neck injections (n = 12) and bladder neck reconstruction (n = 12). Continence and partial continence were achieved in 6/18 (33.3%) and 9/18 (50.0%), respectively, with mean 3.2 ± 2.5 continence procedures at 9.6 years (IQR 7.3, 15.2). Volitional voiding was seen in 11/18 (61.1%) and 7/18 (38.9%) performed CIC, with no significant difference in continence. Mean bladder capacity was 199 ml ± 96 versus 90 ml ± 29 in the volitional voiding versus CIC group (P = 0.0047). Eleven women with median age of 18.0 years (IQR 15.2, 21.4) had recorded menarche: 6/11 (54.5%) patients reported painful/irregular menses, controlled with hormonal therapy. Six of 11 (54.5%) women had vaginal stenosis managed with vaginal dilation (n = 2) or vaginoplasty (n = 4). Three (27.3%) reported tampon use and penetrative intercourse. CONCLUSION: Overall, the majority of women who have undergone CPRE achieved complete or partial continence, though most required additional procedures and time to attain it. Additionally, volitional voiding was achievable. Bladder capacity was significantly lower in patients dependent on CIC. Most required medical or surgical interventions for gynecologic concerns post menarche. This study underscores the unique needs of girls and young women with bladder exstrophy and further supports the importance of close long-term urologic and gynecologic management throughout development.

A non-surgical approach to 46,XY differences in sex development through hormonal suppression at puberty: a single-center case series study.

PURPOSE: We aim to report outcomes and safety with hormonal suppression to facilitate gonadal preservation in a select group of patients with 46,XY differences in sex development (DSD) who are raised and identify as female yet have diagnoses with potential for androgenization at puberty. METHODS: We performed a retrospective review of the past 10 years of DSD patients treated by a multidisciplinary program. Inclusion criteria were 46,XY DSD, female sex of rearing, risk of androgenization at puberty, and plan for hormonal suppression at puberty. Patients on hormonal suppression had at least 6 months of follow-up from initiation. We excluded those with complete gonadal dysgenesis or complete androgen insensitivity. RESULTS: Four patients met inclusion criteria. Initial evaluation by DSD team was at a mean age of 6.6 years (3 weeks-16 years). All patients were evaluated in a coordinated multidisciplinary clinic. The diagnoses are listed in Table 1. Mean follow-up was 5.7 years (1.2-10.9 years). One patient presented as an infant, and is being monitored until Tanner stage 2 and/or serum hormonal evidence to initiate hormonal suppression. Three patients have been receiving hormonal suppression for 1.4 years (1.1-1.9 years) without side effects or complication. Three patients were initiated with estrogen replacement to promote desired breast development. At last follow-up, all patients had retained their gonads, all have female gender identity with no reported gender dysphoria, and no progression of androgenization. CONCLUSIONS: In our initial experience, gonadal preservation with hormonal suppression is a tool in multidisciplinary management of select DSD patients with female gender identity with conditions associated with androgenization at puberty. Patients' growth, bone health, and overall psychosocial well-being will need to be monitored closely.

Patient-reported outcomes in adult females with bladder exstrophy: A study of long-term sexual, reproductive and urinary outcomes using social media.

INTRODUCTION: Limited data exist on patient-reported outcomes in adults with bladder exstrophy (BE). We partnered with the Association for the Bladder Exstrophy Community (A-BE-C) using social media to survey adult females with BE. The aim of the study was to assess long-term patient-reported sexual, reproductive and continence outcomes. MATERIALS AND METHODS: Between December 3, 2018 and January 18, 2019, A-BE-C promoted an anonymous survey of adult females with BE on social media. The survey included the Female Sexual Function Index (FSFI) and International Consultation on Incontinence Questionnaire - Short Form (ICIQ-SF) in addition to questions on demographics, reproductive and gynecological outcomes. RESULTS: A total of 130 women with a median age of 30 years (IQR 26, 41) completed the survey. The majority of women were born in the United States (N = 86, 66.2%). Women reported a median of 10 (IQR 5, 17) surgeries performed for their condition. The mean ICIQ-SF score was 6.2 ± 6.2 (moderate). Only 19.2% (N = 25) reported volitionally voiding and the majority reported intermittent catheterization through a catheterizeable channel or the urethra (Summary Table). The mean FSFI score was (20.1 ± 9.0), indicating risk for sexual dysfunction (FSFI < 26.55). Of the respondents, 28.5% (N = 37) were treated for uterine prolapse, 56.9% (N = 74) required surgery in order to use tampons or have penetrative intercourse, and over half (55.4%) reported some degree of dissatisfaction with the appearance of their external genitalia. Forty-seven women (36.2%) reported pregnancies, and of these 32 (68.1%) reported complications with pregnancy. Outcomes of 100 pregnancies included miscarriage/abortion (41.0%), preterm vaginal (3.0%), preterm cesarean section (19.0%), term vaginal (2.0%), and term cesarean section (35.0%). Seven (15.2%) women identified having children with a medical diagnosis, none of which included BE. Infertility was reported in 17.3% (22/127) women. Women identified sexual function, fertility, and body image as areas that need future research. CONCLUSION: This is the largest study of patient-reported outcomes in females with BE achieved through partnership with an advocacy group and social media. We found that continence and sexual function were both impaired in adult women with BE. Respondents reported poor obstetric and gynecological outcomes including infertility, uterine prolapse, vaginal stenosis, and need for multiple surgeries. BE surgeons, through multi-institutional collaboration, should continue to reassess surgical techniques at initial repair to better address these functional issues. These results should be a part of counseling parents of newborns with BE.

Simulation Training in Urology: State of the Art and Future Directions.

There has been a major shift from the old paradigm of 'see one, do one, teach one' in medical training due in large part to resident work-hour restrictions and required oversight in the operating room. In response to this, advancements in technology have allowed for the introduction of more objective measures to assess the skill competency and proficiency of surgical trainees. Patient safety and trainee well-being are important drivers for this new model, and so surgical training programs are adopting simulation into their curriculum. Urology is uniquely positioned at the forefront of new emerging technologies in surgery, because of the field's commitment to safe and efficient minimally invasive surgery and endourological procedures. Due to these technically challenging procedures, urological training must incorporate these educational technologies to allow for objective skills assessment, skills transfer, and ultimately providing optimal patient care with the production of proficient and competent urological trainees.

Osseous Metaplasia in a Bladder Diverticulum in a Patient with Mosaic Menkes Disease.

Menkes disease, or Kinky Hair Syndrome, is a rare disorder of copper metabolism that causes fatal neurodegenerative disease in infancy. This X-linked disorder results from mutations in the ATP7A gene. Along with neurological decline, characteristic coarse appearance of the hair is seen. Urological issues are prevalent in this patient population, with bladder diverticula being the most common. Herein, we describe a unique male patient with genetic mosaicism and osseous metaplasia found in a ruptured bladder diverticulum.

Surgery and hormonal treatment for prostate cancer and sexual function.

Prostate cancer (PC) is one of the most common cancers effecting men today. With earlier detection and improvements in available treatment modalities, there still remains significant morbidity associated with the treatment of PC. Male sexual health and erectile function are greatly impacted by these therapies and remain a concern to PC survivors. This article reviews the current literature on male sexual health following radical prostatectomy (RP) or androgen ablation therapy for PC. Each treatment modality affects male sexual function to an appreciable level, although certain patients have better outcomes if they have preoperative potency, are younger, or have nerve-sparing surgery. There is a delayed recovery up to 2 years seen in erectile function following RP. With androgen deprivation therapy (ADT), attempts can be made at different administration strategies and exercise may possibly play a role in maintaining erectile function. Penile rehabilitation protocols attempt to protect erectile function immediately following therapy through different modalities, although no one approach has been agreed upon.