Atypical Presentation Delays Treatment of Pediatric Testicular Torsion.

OBJECTIVE: The aim of the study is to identify patient- and care-related factors associated with time to treatment for acute testicular torsion and the likelihood of testicular loss. METHODS: Data were retrospectively collected for patients 18 years and younger who had surgery for acute testicular torsion between April 1, 2005, and September 1, 2021. Atypical symptoms and history were defined as having abdominal, leg, or flank pain, dysuria, urinary frequency, local trauma, or not having testicular pain. The primary outcome was testicular loss. The primary process measure was time from emergency department (ED) triage to surgery. RESULTS: One hundred eleven patients were included in descriptive analysis. The rate of testicular loss was 35%. Forty-one percent of all patients reported atypical symptoms or history. Eighty-four patients had adequate data to calculate time from symptom onset to surgery and time from triage to surgery and were included in analyses of factors affecting risk of testicular loss. Sixty-eight patients had adequate data to evaluate all care-related time points and were included in analyses to determine factors affecting time from ED triage to surgery. On multivariable regression analyses, increased risk of testicular loss was associated with younger age and longer time from symptom onset to ED triage, while longer time from triage to surgery was associated with reporting atypical symptoms or history.The most frequently reported atypical symptom was abdominal pain, in 26% of patients. These patients were more likely to have nausea and/or vomiting and abdominal tenderness but equally likely to report testicular pain and swelling and have testicular findings on examination. CONCLUSIONS: Patients presenting to the ED with acute testicular torsion reporting atypical symptoms or history experience slower transit from arrival in the ED to operative management and may be at greater risk of testicular loss. Increased awareness of atypical presentations of pediatric acute testicular torsion may improve time to treatment.

Hydrometrocolpos: a Contemporary Review of the Last 5 Years.

PURPOSE OF REVIEW: The goal of this review is to provide a comprehensive overview of hydrometrocolpos, covering disease etiology, pathophysiology, clinical presentation, and diagnostic and management techniques, and known outcomes. RECENT FINDINGS: This narrative review presents the literature on hydrometrocolpos in the pediatric population from the past 5 years. We highlight the 69 reported cases of hydrometrocolpos and classify them based on type of obstruction or associated anomaly, discuss new diagnostic algorithms based on imaging, and present novel and underutilized surgical techniques for definitive management. Hydrometrocolpos, a condition characterized by retained fluid causing a distended vagina and uterus in the setting of a distal vaginal outflow obstruction, has a wide range of presentation severity based on the type of obstruction. Whether hydrometrocolpos is due to an isolated condition like imperforate hymen, a complex abnormality like cloacal malformation, or a part of a large congenital syndrome, the mainstay of treatment is decompression of the dilated vagina and surgical correction of the outflow obstruction. Imaging-based diagnostic algorithms and new treatment techniques reported in the literature, as well as longitudinal and patient-reported outcome research, can improve the lives of children affected by this condition.

Rare case of an isolated scaphoid congenital megalourethra: before and after reconstruction.

Congenital megalourethra, first described in 1955, is a rare urethral anomaly resulting from dysgenesis of the penile corpus spongiosum, with or without corpus cavernosum involvement, leading to dilatation of the penile urethra. Presentations come in two forms, scaphoid and fusiform, with the former being more common and resulting from deficient or absent corpus spongiosum. Fusiform types are much rarer, and consist of absence of both the corpus spongiosum and cavernosum.3 Here, we present a case involving the surgical correction of an isolated scaphoid-type congenital megalourethra with significantly improved postoperative cosmetic and functional outcomes.

Virilization of a 46,XX Fetus Following Aromatase Inhibitor Treatment of Breast Cancer.

Virilization of the 46,XX infant may be attributed to maternal or fetoplacental origin. Maternal sources may be endogenous, as with an androgen-producing tumor, or drug-related. Iatrogenic virilization by maternal drug exposure is rarely reported, with individual case reports and case series demonstrating the effects of progesterone and other medications affecting the pituitary-ovarian axis.1-3 The class of medications known as aromatase inhibitors are recognized as effective in treating hormone receptor-positive breast cancer by preventing the conversion of androgens into estrogens by aromatase. In fetal development, placental aromatase plays a critical role in preventing virilization of the XX fetus by maternal and fetal androgens during development. In the setting of placental aromatase deficiency, the XX fetus may be virilized. It is conceivable, therefore, that maternal exposure to aromatase inhibitors early in gestation may lead to in utero virilization, though there have been no known reports of this phenomenon to date. We present a case of virilization of a 46,XX infant attributed to pharmacologic aromatase inhibition. The infant's parents provided informed consent for the reporting of this case.

Distal urethral reconstruction with AlloDerm: a case report and review of the literature.

Reconstruction of large urethral defects is a complex problem for which no standardized surgical guidelines exist due to the lack of clinical trials demonstrating definitively the superiority of one technique over another. AlloDerm has been used in other surgical specialties with success, however, its utility in urologic surgery and, more specifically, for urethral reconstruction has been limited. In this case report, we demonstrate its application in a single-staged closure of a significant anterior urethral defect following distal penile necrosis secondary to an extruded penile prosthesis in a diabetic patient.

Biologic adjuvant urethral coverings for single-stage primary hypospadias repairs: A systematic review and pooled proportional meta-analysis of postoperative urethrocutaneous fistulas.

BACKGROUND: The use of barrier layers between the neourethra and skin is associated with lower rates of post-operative urethrocutaneous fistula (UCF) following hypospadias surgery. Recent studies have evaluated the ability of biologic adjuvant urethral coverings (BAUCs) - namely acellular matrix (AM), tissue adhesives (TAs), and autologous platelet-rich plasma or fibrin (PRP/PRF) - to prevent wound complications following hypospadias surgery. In general, however, these studies are small and conducted at single institutions. OBJECTIVE: To assess the effect of BAUCs on the rate of UCF following single-stage primary hypospadias repair. METHODS: We conducted a systematic review of studies reporting the rate of postoperative UCF in pediatric patients undergoing single-stage, primary hypospadias repairs using either AM, TA, or PRP/PRF as a layer interposed between the neourethra and skin. We then performed a pooled proportional meta-analysis of post-operative UCF. Patients within each study who underwent comparable surgery but did not receive a BAUC were used as controls. RESULTS: 10 studies were included in our review. The meta-analysis included 280 patients from 7 studies who underwent hypospadias repairs with BAUCs. The pooled incidence of UCF was 10% (95% CI 6-14%). Mean follow-up ranged 5-23.5 months in the 5/7 studies reporting specific durations, and ≥6 month and 14-30 months, respectively, in the other two studies. Patients in whom a BAUC was used had significantly lower odds of UCF than control patients (OR 0.39, 95% CI 0.24-0.64, p = 0.0002). In subgroup analyses, significant superiority held for AM and TA; proximal or penoscrotal cases; transverse preputial island flap (TPIF) technique; when both cases and controls had local flaps; and when neither cases nor controls had flaps. DISCUSSION: The use of BAUCs was associated with decreased rates of post-operative UCF in single-stage primary hypospadias repairs and may be most beneficial in more severe cases and when used in addition to local flaps or when using a flap is not possible. In 2/3 studies of PRP/PRF and 2/4 studies of tubularized incised plate (TIP) technique, dartos flaps were used in controls but not BAUC patients, which may explain the lack of benefit demonstrated for these subgroups. This meta-analysis is limited by the quality of evidence in the included studies, which are not uniformly randomized. Furthermore, the follow-up durations and methods for assessing complications are not standardized between included studies. CONCLUSION: The meta-analysis herein suggests that using BAUCs may reduce UCF rates following hypospadias surgery. Rigorous prospective evaluation is needed to validate this benefit.

Press Ganey Ratings in Urology: Who is at Risk of Bias?

OBJECTIVE: To evaluate effect of patient and physician demographics on Press Ganey (PG) survey ratings for urologists. METHODS: PG surveys (02/2020-08/2021) for urologists at a single tertiary care center were analyzed. Univariate and multivariate logistic regression models were used to assess the relationship between patient and physician-level covariates and the primary outcome of a "topbox" Overall Doctor Rating (topbox-ODR) score of 9 or 10 of 10. RESULTS: A total of 4155 surveys of 20 attending urologists (8 female (F)) across 7 subspecialties, were assessed. Mean ODR score for F physicians was 9.2 (SD 1.7) compared to 9.5 (SD 1.3) for males (M), P < .001. Univariate regression demonstrated that F patients are less likely (OR 0.27, P < .001) to give topbox-ODRs than M patients, and F physicians are 58% less likely (OR 0.42, P = .01) to receive topbox-ODRs than M physicians. Oncologists are more likely to receive topbox-ODRs (OR 3.3, P = .009) than all other subspecialists. Multivariate regression demonstrated that M patients are more likely to give M physicians top-box-ODRs (OR 0.32, P = .02), while F patients are less likely to give topbox-ODRs to physicians of both genders (M: OR 0.24, P < .001; F: 0.21, P < .001). Physicians in practice for >10 years are 66% less likely to receive topbox-ODRs (OR 0.33, P = .002). CONCLUSION: Urologists who care for F patients are at risk of being affected by bias in PG physician ratings. M physicians who care for M patients appear to be at the least risk; while F physicians who care for F patients appear to be at the highest risk.

Pediatric Penile Non-Involuting Congenital Hemangioma With an Associated Pyogenic Granuloma: Surgical Management of a Rare Vascular Anomaly.

Vascular anomalies include both tumors and malformations. Infantile hemangiomas are the most common benign vascular tumor of infancy that proliferate after birth and eventually involute. By contrast, congenital hemangiomas are formed at birth and are categorized into three groups: rapidly involuting, partially-involuting, and non-involuting congenital hemangiomas (NICH). NICH do not regress and grow with age. Pyogenic granulomas, another acquired vascular tumor, develop over vascular lesions and cause bleeding. Primary treatment options for NICH and pyogenic granulomas are surgical resection. Here, we report a case of a NICH with a co-existing pyogenic granuloma involving the penile shaft and scrotum treated surgically.

Inappropriate imaging for management of cryptorchidism: Has the choosing Wisely® recommendation reduced occurrence?

INTRODUCTION: Cryptorchidism is one of the most common referral diagnoses to pediatric urologists. It is well recognized in the urologic community that diagnostic imaging is unnecessary in the work-up of these patients, and the Choosing Wisely® recommendation (CWR) on this subject re-emphasized this in 2013. Many boys, however, still are sent for testing prior to referral. OBJECTIVE: The purpose of our study was dual in nature. We pursued, first, to identify any factors that make patients more likely to be sent for imaging prior to referral, and second, to determine if rates of diagnostic imaging for cryptorchidism decreased after the release of the CWR. STUDY DESIGN: We included all boys who had surgery for cryptorchidism by Urology at our institution between January 2007 and August 2018. Demographics and clinical data were collected including height, weight, race, insurance type, pre-referral imaging status, testis location at time of surgery, and distance from our medical center. Chi-squared analysis was utilized to compare imaging use before and after CWR. Influence of other clinical and socioeconomic factors on imaging utilization was also evaluated using chi-squared and two-sample t tests. Those found to be significant at the 0.2 level were analyzed in multivariate logistic regression. Significance was set at 0.05. RESULTS: 1010 boys were available for analysis. Of the 256 patients (25.3%) with pre-referral studies, 7 had axial exams (CT or MRI), and the remainder underwent ultrasounds. Children living closer to the medical center were more likely to undergo imaging (p < 0.01) as were boys with testes not found in the inguinal canal at the time of surgery (p = 0.007). Race, insurance status, age at first visit, and increased body mass index were not found to be influential. Similarly, the release of CWR had no impact on the imaging usage (p = 0.61). CONCLUSION: Utilization of pre-referral diagnostic imaging remains inappropriately high despite evidence demonstrating the ineffectiveness of the studies. Boys living closer to the medical center and those with non-inguinal testes are more likely to undergo these studies, but no other factors were found to have an effect. Further, the Choosing Wisely® recommendation has not improved rates of inappropriate imaging use in boys with cryptorchidism in our referral area. Our findings indicate the need for increased efforts to disseminate this evidence-based guideline more widely to primary care providers in order to promote more cost-effective and timely care of boys with undescended testes.

When Chemotherapy Is Not Enough-Management of Prostatic Embryonal Rhabdomyosarcoma in an Infant.

A baby boy was diagnosed with embryonal rhabdomyosarcoma causing left hydroureteronephrosis. A loop ureterostomy was performed, and the infant was treated per the RMS13 protocol. After 3 months of chemotherapy, the infant's tumor burden increased, and he underwent radical cystoprostatectomy and right-to-left transureteroureterostomy (end-to-end fashion utilizing the distal limb of his ureterostomy). This innovative method was utilized because the infant's tumor burden was too large to be treated effectively and safely with radiation. One year later, the infant has no evidence of disease. This demonstrates that optimal management of rhabdomyosarcoma is still unknown; therefore, each child warrants an individualized approach for optimal outcomes.

Resolution of Diabetes Insipidus After Pyeloplasty: A Case Report and Review of the Literature.

Nephrogenic diabetes insipidus (NDI), a rare cause of polyuria and polydipsia in children, is usually managed with medications and careful monitoring of water intake. We present a child who was incidentally found to have right hydronephrosis secondary to ureteropelvic junction obstruction, and was subsequently also diagnosed with NDI. After being medically managed, he underwent open right pyeloplasty. His polydipsia abated within 1 month of surgery, and he has done well off of medications since that time. NDI resolution after correction of obstructive uropathy in adults has been reported, but this represents a novel case in pediatrics.

Hydronephrosis After Pyeloplasty: "Will It Go Away?"

OBJECTIVE: To identify attributes of pediatric patients with hydronephrosis due to ureteropelvic junction obstruction and of their surgical encounters which are predictive of resolution of dilatation in order to provide more effective counseling about expected outcomes. This study was inspired by the suggestion in recent literature that greater than 20% improvement in anteroposterior diameter (APD) of the renal pelvis after pyeloplasty is indicative of resolution of obstruction. The remaining dilatation, however, is often distressing to caregivers, and there are no data to guide clinicians in counseling about its likelihood to resolve. METHODS: We retrospectively reviewed children who underwent surgery at our institution for ureteropelvic junction obstruction between 1/01/2010 and 6/30/2017. APD of the pre- (preAPD) and postoperative (postAPD) renal pelves were documented. In children with more than 1 postoperative ultrasound, lastAPD was the measurement on their most recent study. Appropriate statistical tests examined the effects of clinical and surgical variables on hydronephrosis resolution. RESULTS: PostAPD and lastAPD were obtained at medians of 3 months and 1.9 years after surgery, and were 0 cm in 12 of 105 (11.5%) and 9 of 65 (13.8%) patients, respectively. None of the variables analyzed significantly impacted complete resolution at either time point. Of those that did not resolve, 80.6% (75/93) showed greater than 20% improvement in APD; however, 3 of these children required reoperation due to secondary obstruction. In our study, no one with APD reduction greater than 43% required reintervention. CONCLUSION: Complete resolution of hydronephrosis is uncommon and unpredictable. All caregivers should be counseled to expect dilatation to persist after obstruction is corrected.

Enterocystoplasty: The long-term effects on bone mineral density.

BACKGROUND: Studies show that enterocystoplasty has a negative effect on bone mineral density (BMD). The aim of this study was to investigate the long-term impact of enterocystoplasty on BMD. We used dual energy x-ray absorptiometry (DEXA) scans to determine BMD and identify patients with osteopenia and osteoporosis who are at potential long-term risk for fracture. MATERIALS AND METHODS: We reviewed our database of >200 individuals, who had undergone enterocystoplasty or continent diversion for both neurogenic and non-neurogenic reasons during childhood. We chose to study the non-neurogenic group first for a number of technical reasons, and identified 24 individuals who had undergone the procedure for non-neurogenic reasons, and had more than 15 years of follow-up. In addition we had a control group of 10 individuals born with bladder exstrophy, who had undergone primary closure before the year 2000, without enterocystoplasty. We used DEXA scan T- and Z-scores to identify patients with osteopenia and osteoporosis. RESULTS: Eleven of 24 patients had normal DEXA scans with normal T- and Z-scores; seven had identifiable osteopenia and increased long-term risk for fracture. Six had osteoporosis; three of whom had reduced glomerular filtration rate (GFR). Eight of the 10 individuals in the control group had a normal DEXA scan. CONCLUSIONS: Enterocystoplasty during childhood can lead to loss of BMD. This does not seem to be related to the enterocystoplasty alone. It is more pronounced in individuals who have other risk factors, such as reduced GFR. The identification of BMD loss makes it possible to intervene before osteoporosis occurs and leads to pathologic fracture.

Fluid Management in Pediatric Urology: A Review of the Literature and Call for a Change in Practice.

INTRODUCTION: We investigate the intravenous fluid ordering practices of pediatric urologists. We also provide evidence for the use of isotonic fluids and, thus, establish a new standard for the field. METHODS: An online questionnaire was distributed via the listserve of the American Academy of Pediatrics Section on Urology. For 3 patient scenarios the respondents were asked to select which intravenous fluid they would prescribe and which infusion rate they would choose. Fluid choices were listed as lactated Ringer solution, normal saline, 0.45% normal saline, 5% dextrose in lactated Ringer solution, 5% dextrose in 0.45% normal saline, none and other (with write-in option). Infusion rate choices were listed as maintenance (defined according to the Holliday-Segar 4-2-1 nomogram), ½x maintenance, 1.5x maintenance, 2x maintenance and other (with write-in option). A final question probed physician reason(s) for selections. RESULTS: The survey had a 35% response rate. The majority of respondents use 5% dextrose in 0.45% normal saline, and the most common infusion rate is 1.5x maintenance. Additionally, choices for fluid use and infusion rate were based on a combination of physician training and personal experience. Only 13% of respondents stated that they routinely avoid isotonic fluids. A PubMed® literature search demonstrated that the general pediatric and surgical specialty literature discourages hypotonic fluids and favors the judicious use of isotonic hydration and dextrose. CONCLUSIONS: A change in pediatric urology needs to occur such that isotonic fluids at maintenance rate become the standard, with the addition of dextrose and/or increasing of rate only for carefully selected patients.