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Summary Introduction Widespread utilization of telemedicine in our practice to date has been limited to the evaluation of certain post-surgical patients. The COVID-19 pandemic acutely stressed our established system and required us to enhance our utilization of telemedicine. We hypothesized that expansion of telemedicine to new and follow up patient visits for pediatric urology could be done effectively in a way that satisfied patient and parental expectations. Materials and methods Using a pre-COVID-19 established telemedicine program based in our electronic medical record (EMR), patients and providers transitioned to primarily virtual visits when clinically appropriate. Guidelines were formulated to direct patient scheduling, provider and staff education was provided, including a process map designed for multiple providers to complete video visits (VV), and the EMR was redesigned to incorporate telehealth terminology. The number of VV per provider was recorded using the electronic medical record, and patient reported outcomes (PRO) were measured using a standardized questionnaire. Results A total of 631 VV met inclusion criteria during the period of May 2018–April 2020. This included 334 follow up, 172 new, and 125 postoperative visits. The median age of patients at time of visit was 7 years (IQR 2–12 years), median visit time was 20 min (IQR 15–30 min), and the median travel distance saved by performing a VV was 12.2 miles (IQR 6.3–26.8 miles). Diagnoses were varied and included the entire breadth of a standard pediatric urology practice. The PRO questionnaire was completed for 325 of those patient visits. Families reported a high overall satisfaction with the video visits (median score of 10 out of 10) and felt that the visit met their child's medical needs. 90% stated that they would strongly recommend a telehealth visit to other families. Patients and parents reported benefits of VV including decreased travel costs and less time taken off from work and school. Conclusion The EMR enabled nimble redirection of clinical care in the setting of a global pandemic. The enhanced use of telemedicine has proved to be an alternative method to provide care for pediatric urology patients. Families indicate a high degree of satisfaction with this technology in addition to significant time and cost savings. Telemedicine should remain a key aspect of medical care and expanded from post-operative visits to new patient and follow up visits, even as we return to our normal practices as the pandemic restrictions soften. Download : Download high-res image (855KB) Download : Download full-size image Summary figure . Program map depicting the sequence of a video visit (VV). The process begins with our scheduling team which provides the necessary information to the family and ensures that they can be scheduled properly. The family is encouraged to conduct the visit in a quiet place with at least one parent and the child present. Relevant records, imaging, and laboratory studies should be uploaded prior to the visit. In addition, pictures and/or videos uploaded by the family prior to the visit can facilitate the examination portion of the visit. VV = video visit; AVS = after visit summary.

Purpose Gonadal damage is a common consequence of treatment for pediatric malignancies. Nononcologic conditions may also utilize treatments with potential impact on fertility. Models for oncology fertility preservation programs have emerged and demonstrate that a multidisciplinary team approach can have a positive impact on referral patterns, appropriate risk counseling, and access to fertility preservation options. Expansion of programmatic breadth is needed, providing improved care to nonmalignant conditions where the disease itself may impact reproductive health or treatment modalities. Methods With support from the Department of Pediatrics Chair's Initiative, a multidisciplinary, hospital-wide Fertility Preservation Service was created at the Children's Hospital of Philadelphia. A centralized team provides fertility consults across the institution, allowing for risk-based counseling and facilitation of fertility preservation options (both standard care and experimental). Results Team structure, consult process, and available fertility options for prepubertal and pubertal males and females are described. Preinitiative and postinitiative referral patterns were analyzed. Postinitiative referrals from divisions outside oncology more than doubled (34% vs. 15% at baseline). Conclusions A comprehensive model for fertility counseling provides accessible, high-value fertility preservation care to pediatric and young adult patients with a wide variety of diagnoses. A centralized point of contact ensures timely referrals and risk-based counseling and streamlines access to fertility preservation procedures.

We present 2 patients found to have ovotesticular disorder of sexual development (otDSD) in late adolescence. Two 15-year-old phenotypically male patients presented to a large pediatric hospital with different complaints: 1 with concern for testicular rupture after a straddle injury; 1 with gynecomastia. Further workup, including imaging and laboratory tests, was performed before surgical exploration. The first patient had unilateral ovotestis, contralateral testis, and SRY-negative 46,XX karyotype. The second patient with gynecomastia had unilateral ovotestis with hemi-uterus and fallopian tube, contralateral ovarian tissue, and 46,XX/47,XXY Klinefelter mosaic karyotype. Although rare, phenotypically normal male patients may present later with ovotesticular disorder of sexual development.

OBJECTIVE: To assess the prevalence of therapy-related kidney outcomes in survivors of Wilms tumor (WT). STUDY DESIGN: This prospective cohort study included survivors of WT who were ≥5 years old and ≥1 year from completing therapy, excluding those with pre-existing hypertension or prior dialysis or kidney transplant. Participants completed 24-hour ambulatory blood pressure monitoring (ABPM). Abnormal blood pressure (BP) was defined as ≥90(th) percentile. Masked hypertension was defined as having normal office BP and abnormal ABPM findings. Urine was analyzed for KIM-1, IL-18, EGF, albumin, and creatinine. Estimated glomerular filtration rate (eGFR) was calculated using the bedside CKiD equation. Recent kidney ultrasounds and echocardiograms were reviewed for contralateral kidney size and left ventricular hypertrophy (LVH), respectively. Clinical follow-up data was collected for approximately 2 years following study enrollment. RESULTS: Thirty-two participants (median age 13.6 [IQR: 10.5-16.3] years; 75% ≥Stage 3 WT) were evaluated at a median of 8.7 years (IQR: 6.5-10.8) post-therapy. Twenty-nine participants underwent unilateral radical nephrectomy, 2 bilateral partial nephrectomy, and 1 radical and contralateral partial nephrectomy. 72% received kidney radiotherapy and 75% received doxorubicin. Recent median eGFR was 95.6 ml/min/1.73m(2) (IQR: 84.6-114.0; 11 (34%) had an eGFR

Purpose Partial nephrectomy is increasingly used in children with bilateral Wilms' tumor (BWT) or contralateral recurrence. Nephron-sparing surgery seeks to achieve complete tumor removal while preserving functional renal parenchyma. Previous series have documented high rates of complications, recurrence, and mortality. Methods Twelve patients (4 boys and 8 girls aged 9-42 months) with BWT or contralateral recurrence were treated at our institution with unilateral or bilateral partial nephrectomy. Preoperative imaging, operative notes, and pathology reports were reviewed. Outcomes analyzed included complications, recurrence, readmission rate, postoperative glomerular filtration rate (GFR), and survival. Results All patients underwent successful nephron-sparing resection using standard techniques, with only 2 patients requiring unilateral nephrectomy. Median length of stay was 3 days. There were no major complications or urine leaks. Two patients were lost to follow-up. The remaining 10 were followed up for a median of 36 months (range, 3-79 months). There have been no recurrences or unplanned readmissions. Mean GFR is 107.7 (±32.8) mL/min per 1.73 m 2 , with no patient having a GFR below the lower limit of normal for age. Conclusion Nephron-sparing resection is a safe and effective approach for children with BWT or contralateral recurrence and should be part of the multimodality therapeutic approach to this disease.

Summary Introduction Re-operative penile reconstruction is challenging and requires tension-free skin closure. The repair popularized by Cecil and Culp in the 1940s, using the scrotum to provide a temporary vascularized bed for complex hypospadias repairs, fell out of favor due to temporal trends towards single-stage repairs and concern for utilizing hair-bearing skin on the penile shaft. Objective It was hypothesized that a modified Cecil-Culp (CC) concept of penile scrotalization, leaving the penis attached to the scrotum for 1 year rather than 6 weeks as originally described, improves outcomes with this reconstruction for ventral skin deficiency or poor vascular support. Methods Institutional Review Board-approved registries were reviewed to identify patients who underwent a CC repair during 1987–2016 at two institutions. Cecil-Culp technique was utilized in multi-stage hypospadias complication repairs or for insufficient ventral penile shaft skin coverage. Anatomic abnormality, number and type of prior surgeries, and complications before and after CC were recorded. Results Thirty-nine patients underwent CC: 23 failed hypospadias repairs, three hypospadias after bladder exstrophy, 10 penile curvature following circumcision, and three with skin loss from trauma. Mean age at CC was 61.8 months (hypospadias), and 59.8 months (non-hypospadias). Hypospadias patients underwent a mean of 3.6 surgeries (range 1–9) prior to CC. Four of the 39 patients (10.3%) had perioperative complications after CC, including scrotal abscess, skin infections, and difficulty removing the urethral stent. Eight of 37 (21.6%) patients had longer-term complications related to their hypospadias repair, including fistulae, diverticula, dehiscence, and stricture. Mean time from CC placement to release was 345 and 473 days for hypospadias and non-hypospadias cases, respectively. There was no apparent scrotal skin transferred to the penile shaft at the final take-down. Mean follow-up was 22.3 months. Discussion Embedding the penis into the scrotum for added vascularity and ventral skin coverage has been used effectively in cases of the most tenacious fistulas and for significant skin loss and trauma. Limitations of this study were its retrospective approach at two institutions over an extended period of time by multiple surgeons, so patient selection and procedure may have varied. Conclusions Modification of CC repair by delaying 9–12 months before CC take-down enhanced the benefits of a robust vascular bed for wound healing, and helped to avoid transfer of hair-bearing scrotal skin to the penile shaft. The CC technique is an important tool for penile reconstructive surgery of complex hypospadias repairs with inadequate skin, and for traumatic injuries.

Cryptorchidism is a common genital anomaly diagnosed at birth or during childhood. Genetic and/or environmental factors that alter expression or function of hormones crucial for testicular descent, insulin-like 3, and testosterone, may contribute to cryptorchidism. When identified at birth, surgical treatment is indicated by 6 months of age if testes fail to descend, or at the time of diagnosis in older children. A laparoscopic approach is preferred for abdominal testes. Early surgical therapy may reduce the risk of subfertility and/or malignancy.

The evaluation and management of neonates with ambiguous genitalia requires sensitivity, efficiency, and accuracy. The approach to these neonates is facilitated by a multidisciplinary team including urology, endocrinology, genetics, and psychiatry or psychology. Disorders of sex development (DSD) encompass chromosomal DSD, 46,XX DSD, and 46,XY DSD. The 46,XX DSD is the most common DSD and in the majority of these children congenital adrenal hyperplasia is the underlying etiology. The 46,XY DSD is a heterogeneous disorder that often results from a disruption in the production or response to testosterone, dihydrotestosterone, or Mullerian inhibitory substance. Chromosomal DSD includes conditions resulting from abnormal meiosis, including Klinefelter syndrome (47, XXY) and Turner syndrome. The evaluation of children with DSD demands a thorough physical examination, medical history, karyotype, metabolic panel, 17-OH progesterone, testosterone, luteinizing hormone, follicle stimulation hormone, and urinalysis. A radiographic evaluation should begin with an abdominal and pelvic ultrasound but may include magnetic resonance imaging, endoscopy, or laparoscopy.

Cryptorchidism is a common anomaly treated by every pediatric urologist. The etiology is multifactorial and includes hormonal and molecular factors. Diagnostic abilities may be enhanced by radiologic advances. Hormonal and surgical treatment modalities are discussed herein, as well as the identification and prevention of long-term sequelae.

Sexual determination is a complex process that occurs in an organized sequential manner. When chromosomal, gonadal, or phenotypic sex determination goes awry,intersexuality develops. Advances in molecular biology have made it easier to understand the various phenotypes that are encountered. It is easy to be overwhelmed when reviewing the testosterone synthesis pathway and the intersex differential diagnosis. This article presents a useful approach to the evaluation of the newborn with ambiguous genitalia.

Concordant and discordant presentations of posterior urethral valves (PUV) in twins have been described. Twin gestation may complicate the diagnosis of PUV based on prenatal evaluation. A case series of 2 sets of twin births is presented, each of which was discordant for the diagnosis of PUV. A delay in diagnosis occurred in both cases, despite prenatal ultrasound abnormalities. This delay could result from failed sensitivity of prenatal ultrasound or from postnatal evaluation of the incorrect twin. Caution must be exercised during follow-up of abnormalities identified on prenatal ultrasound in diseases in which there are no external distinguishing characteristics.

Summary Introduction Adolescent males with varicoceles present a dilemma for surveillance and treatment. Testicular volumetrics have not been shown to predict SA outcomes. Serial SAs are thus recommended in asymptomatic adolescent males with varicoceles and normal testicular development, but the natural history of semen parameters is unknown. Objective To explore the natural history of semen parameters in adolescent boys with a left varicocele under active surveillance. Study design Adolescents with an asymptomatic unilateral left varicocele, Tanner V development, normal testicular volumes, and an initial SA were retrospectively reviewed in a single-institution prospectively followed cohort. Total motile count (TMC) was calculated. A cutoff of TMC Z 20 million was used to dichotomize SA results into “normal” or “poor.” Those with poor SA were offered repeat SA. Cumulative probabilities of normal TMC over successive rounds of SA were calculated. Bivariate models were used to explore associations of a second consecutive poor TMC with age and varicocele grade. Results A total of 216 patients provided an initial SA between 1992 and 2015. We excluded 17 for a history of cryptorchidism or incomplete SA data for a final cohort of 199 patients with median follow-up of 3.3 years (interquartile range 1.5–5.6 years). The mean age at initial SA was 17.9 years (range 14.8–21.8 years). One hundred and nine out of 199 had an initial normal TMC. Of the 90 out of 199 with an initially poor TMC, 51 had repeat SA and 24 of the 51 patients improved to normal TMC. Of the 27 patients with two consecutive poor TMCs, 15 had a third SA and five out of 15 improved to normal TMC. Thus, cumulatively, 55%, 67%, and 69% of all patients had a normal TMC after an initial, second, and third SA, respectively. However, fewer patients in each round of SA normalized their TMC ( Figure ). Neither age nor varicocele grade was associated with a second consecutive poor TMC. Discussion Two-thirds of Tanner V boys with an uncorrected varicocele and normal testicular volumes achieve a normal TMC regardless of varicocele grade or age. Despite Tanner V development, 47% with an initial “poor” SA will improve to normal status without surgery. However, a small subgroup of patients will have persistently poor TMC and thus should be targeted in future research for timely intervention. Conclusion Semen parameters improve over time. SA should be followed and repeated at least once in symptomatic Tanner V boys with varicoceles. Download : Download high-res image (98KB) Download : Download full-size image Figure . Semen analysis outcomes.

Summary Purpose The main indications for adolescent varicocelectomy are testicular hypotrophy or pain. However, we have previously shown that both serial total testicular volume and volume differential are weakly associated with semen quality. The ultimate patient goal is paternity, but semen analysis is critical to appropriate management of varicocele. We hypothesize that varicocelectomy improves total motile count (TMC) among patients who only have abnormal semen analysis (SA) parameters, but not among those with potential hormonal dysfunction such as Klinefelter syndrome or cryptorchidism. Methods We retrospectively reviewed our registry of adolescent males followed with a clinical left varicocele. For this study, subjects without sustained testicular asymmetry, who were Tanner V, and gave at least one preoperative SA were included. Subjects were excluded if they had embolization for their varicocele or no postoperative SA. Primary outcome was change in TMC before and after surgery, compared using the Wilcoxon signed rank test after stratifying by surgical indication. Secondary outcomes included rates of improved TMC and normalized TMC (>20 million) after surgery, compared across covariates using the Fisher exact test. Results Seventeen patients met the eligibility criteria, 11 of whom underwent repair for only abnormal preoperative TMC. Overall, median age (interquartile range [IQR]) at first preoperative SA was 17.6 (15.9–17.9) years. The median preoperative TMC across all SA was 2.8 (0.7–7.4) million. The median age at surgery was 18.2 (16.8–18.9) years. Postoperatively, the median TMC across all SA increased to 18.2 (3.6–18.2) million (Wilcoxon signed rank test, p Conclusions Adolescent varicocele patients should undergo SA after development of Tanner V. Varicocelectomy has a high success rate for improving TMC in adolescent or young adult males who only have abnormal TMC and no history of cryptorchidism. Download : Download high-res image (153KB) Download : Download full-size image Figure . Change in total motile count after varicocelectomy by patient.

Objective To discuss a case of asynchronous bilateral renal venous thrombosis with a review of current literature. Case A premature fraternal twin male was delivered via Cesarean section to a mother with a history of pre-eclampsia. He was initially diagnosed with a left renal venous thrombosis (RVT) but subsequently developed right RVT. Only intravenous fluids and supportive care were provided. Anticoagulation was withheld because of MRI findings of multiple areas of intracerebral bleeding. His serum creatinine concentration stabilized at 0.7 mg/dL, but follow-up renal ultrasound studies showed bilateral anechoic corticomedullary cysts. He also developed hypertension. Discussion Renal venous thrombosis usually occurs in neonates. Multiple clinical conditions are associated with RVT, two of which exist in this case. Recent work has focused on genetic prothrombotic risk factors, one of which our patient exhibits. Controversy exists over management, i.e. whether or not to initiate anticoagulation or antithrombotic therapy to decrease the development of renal atrophy, renal failure and hypertension. Conclusions This unusual case of asynchronous bilateral renal venous thrombosis recovered in the short term without antithrombotic therapy. Substantial insult early in life is reflected in the progressive worsening of his renal function, ultrasound findings and hypertension, highlighting the need for long-term vigilant observation of these patients.

Microscopically extracted ductal sperm can be successfully cryopreserved using motility as the outcomes measure. The optimal regimen for cryopreservation of microscopically extracted ductal sperm awaits further determination. Pentoxifylline treatment improves initial post-thaw motility of cryopreserved microscopically extracted ductal sperm. The clinical efficacy of this practice awaits further determination. However, the best potential use for cryopreserved microscopically extracted ductal sperm may be IVF with subzonal or intracytoplasmic microinjection. If intracytoplasmic sperm injection becomes more widely available, then enhancement of motility with pentoxifylline may not be necessary. This approach allows for potential fertility insurance at the time of microsurgical bypass of obstruction, and it may preclude, the need for the andrologic surgeon to accurately time his sperm retrieval procedures with IVF. The latter potential advantage would significantly aid current logistical difficulties in the operating room.

Reactive oxygen species production has been demonstrated to impair sperm function. We have noted the potential for the cytokines IL-1 alpha, IL-1 beta, and TNF alpha to stimulate reactive oxygen species production by fertile donor sperm at levels that are consistent with the levels of IL-1 occurring in human seminal plasma. Reactive oxygen species-related sperm membrane peroxidation may be one mechanism by which cytokines can exert a detrimental effect on male fertility. This study suggests a new mechanism by which cell-mediated immunological male infertility may occur.