Body image and psychosocial outcomes in youth and young adults with differences of sex development: a multi-method study.

OBJECTIVE: Differences of sex development (DSD) can affect the physical health, appearance, and psychosocial functioning of affected individuals, but little is known about how subjective appearance perceptions (body image) impact psychosocial outcomes. This study evaluated body image and its associations with psychosocial outcomes including quality of life, resilience, and psychosocial adjustment. METHODS: This cross-sectional, multi-method study assessed body image and psychosocial outcomes including quality of life, adjustment, and resilience in 97 youth and young adults with DSD (mean age = 17 ± 3.7 years; 56% assigned female in infancy) using psychometrically sound instruments. A subsample (n = 40) completed qualitative interviews. RESULTS: Quantitative results indicated that overall, participants were satisfied with their physical appearance, although less so with their primary sex characteristics. Body image dissatisfaction was associated with poorer psychosocial adjustment, quality of life, and resilience. Qualitatively, youth and young adults reported a variety of perceptions, both positive and negative, related to their body image and the impact of living with a DSD condition. Themes identified included appearance management; effects of DSD on body image; diagnostic factors and features; attitudes about diagnosis; and treatment. CONCLUSIONS: Body image is significantly associated with psychosocial outcomes in youth and young adults with DSD, with qualitative findings highlighting both positive and negative body image experiences. Results have implications for clinical care including screening for appearance concerns, normalization of appearance variations, and intervention development to better support healthy body image and psychosocial functioning in youth and young adults with DSD.

Psychosocial Functioning Among Children With and Without Differences of Sex Development.

OBJECTIVE: To assess health-related quality of life (HRQoL), psychosocial adjustment, and family functioning of children with differences of sex development (DSD) or cleft lip and/or palate (CL/P). METHODS: In this cross-sectional study, parents of children with DSD (n = 67), CL/P (n = 121), and a comparison group of unaffected youth (n = 126) completed standardized measures assessing family functioning and their children's HRQoL and psychosocial adjustment. Medical charts were abstracted for youth with either congenital condition. RESULTS: Children with DSD were rated as having significantly lower HRQoL and greater internalizing problems compared to youth with CL/P and unaffected youth. Children in the DSD group were also significantly more likely to fall into the clinical risk categories for total and internalizing problems relative to the CL/P and unaffected groups. Caregivers of children with DSD were significantly more likely to endorse items about child suicidality compared with caregivers in the CL/P and unaffected groups. No significant differences were found between groups for externalizing problems or the expressiveness domain of family functioning; parents of children with DSD reported significantly less family conflict relative to the other groups and greater cohesion relative to the unaffected group. Conclusions Youth with DSD appear to be at greater risk for psychosocial problems relative to children with CL/P and unaffected peers. Results underscore the need for integrated interdisciplinary care and ongoing psychosocial risk monitoring in youth with DSD.

TESTICULAR REGRESSION SYNDROME: PRACTICE VARIATION IN DIAGNOSIS AND MANAGEMENT.

Objective: The purpose of this study was to assess clinical practice patterns with regard to diagnosis and management of testicular regression syndrome (TRS), a condition in 46,XY males with male phenotypic genitalia and bilateral absence of testes. Methods: A retrospective review was conducted at two large pediatric academic centers to examine diagnostic and management approaches for TRS. Results: Records of 57 patients were reviewed. Diagnostic methods varied widely between patients and included hormonal testing, karyotype, imaging, and surgical exploration, with multiple diagnostic methods frequently used in each patient. Of the 30 subjects that had reached adolescence at the time of the study, 17 (57%) had gaps in care of more than 5 years during childhood. Thirty subjects had received testosterone replacement therapy at a mean age of 12.1 ± 1.0 years. Forty-seven percent had a documented discussion of infertility. Eighty-two percent discussed prosthesis placement, with 35% having prostheses placed. Twenty-three percent were seen by a psychosocial provider. The between-site differences were age at fertility discussion, age at and number of prostheses placed, and type/age of testosterone initiation. Conclusion: Our findings highlight the wide variation in diagnostic approaches, follow-up frequency, testosterone initiation, fertility counseling, and psychosocial support for patients with TRS. Developing evidence-based guidelines for the evaluation and management of TRS would help reduce inconsistencies in care and unnecessary testing. Ongoing follow-up and coordination of care, even during the years when no hormonal treatment is being administered, could lead to opportunities for psychosocial support and improved interdisciplinary approach to care. Abbreviations: AMH = antimüllerian hormone; CAH = congenital adrenal hyperplasia; DSD = differences/disorders of sex development; hCG = human chorionic gonadotropin; TRS = testicular regression syndrome.

Spinal anesthesia for surgery longer than 60 min in infants: experience from the first 2 years of a spinal anesthesia program.

PURPOSE: Spinal anesthesia (SA) is being increasingly used in infants to avoid the potential negative neurocognitive effects of general anesthesia (GA). However, SA has been reported to provide a relatively short duration of surgical anesthesia. METHODS: We retrospectively reviewed SA cases for surgical procedures lasting more than 60 min in children up to 3 years old. All patients received bupivacaine 0.5% (1 mg/kg up to 7 mg) with clonidine 1 µg/kg ± epinephrine. The primary outcome was success of SA without subsequent conversion to GA. RESULTS: Thirty-five patients met inclusion criteria (all males, age 7 ± 5 months, weight 8 ± 2 kg). Procedures included male genital, groin and multiple site surgeries. Average surgical duration was 71 ± 12 min (range 60-111 min). SA was successful in 31 of 35 patients (89%; 95% confidence interval 78, 99%). The cause of failure was rarely due to the duration of surgery (1 of 4 patients). Six patients with successful SA required sedation with dexmedetomidine ± fentanyl. Differences in procedure duration and patient characteristics were not statistically significant between successful and failed SA. CONCLUSIONS: SA is a highly successful technique and may offer an alternative to GA in children undergoing appropriate surgery expected to last as long as 60-100 min.

Association of O-Antigen Serotype with the Magnitude of Initial Systemic Cytokine Responses and Persistence in the Urinary Tract.

UNLABELLED: Urinary tract infection (UTI) is one of the most common ailments requiring both short-term and prophylactic antibiotic therapies. Progression of infection from the bladder to the kidney is associated with more severe clinical symptoms (e.g., fever and vomiting) as well as with dangerous disease sequelae (e.g., renal scaring and sepsis). Host-pathogen interactions that promote bacterial ascent to the kidney are not completely understood. Prior studies indicate that the magnitude of proinflammatory cytokine elicitation in vitro by clinical isolates of uropathogenic Escherichia coli (UPEC) inversely correlates with the severity of clinical disease. Therefore, we hypothesize that the magnitude of initial proinflammatory responses during infection defines the course and severity of disease. Clinical UPEC isolates obtained from patients with a nonfebrile UTI elicited high systemic proinflammatory responses early during experimental UTI in a murine model and were attenuated in bladder and kidney persistence. Conversely, UPEC isolates obtained from patients with febrile UTI elicited low systemic proinflammatory responses early during experimental UTI and exhibited prolonged persistence in the bladder and kidney. Soluble factors in the supernatant from saturated cultures as well as the lipopolysaccharide (LPS) serotype correlated with the magnitude of proinflammatory responses in vitro. Our data suggest that the structure of the O-antigen sugar moiety of the LPS may determine the strength of cytokine induction by epithelial cells. Moreover, the course and severity of disease appear to be the consequence of the magnitude of initial cytokines produced by the bladder epithelium during infection. IMPORTANCE: The specific host-pathogen interactions that determine the extent and course of disease are not completely understood. Our studies demonstrate that modest changes in the magnitude of cytokine production observed using in vitro models of infection translate into significant ramifications for bacterial persistence and disease severity. While many studies have demonstrated that modifications of the LPS lipid A moiety modulate the extent of Toll-like receptor 4 (TLR4) activation, our studies implicate the O-antigen sugar moiety as another potential rheostat for the modulation of proinflammatory cytokine production.

Incidence of Hip Dysplasia Associated With Bladder Exstrophy.

BACKGROUND: Exstrophy of the bladder is a rare congenital defect seen in 2.15 children out of every 100,000 live births, with the most severe variant, cloacal exstrophy (CE), only occurring in 1 in 200,000. Developmental dysplasia of the hip (DDH) describes a spectrum of disease ranging from mild hip instability to frank dislocation. Underlying malformations, such as myelomeningocele and arthrogryposis, are often associated with the most severe variant of hip dysplasia, teratologic hip dislocation. The varying degrees of severity in DDH have been encountered in classic bladder exstrophy (CBE) patients, but the exact incidence is unknown. We sought to determine the incidence of DDH in CBE and CE patients. METHODS: We performed a retrospective review of all children with CBE or CE presenting to a single pediatric center between 1994 and 2014. Each chart was reviewed for correct diagnosis of CBE or CE, patient age and demographics, associated medical conditions, pertinent surgeries performed, and the age at operation. Patient imaging was reviewed to determine whether bilateral hip imaging was available. RESULTS: In a 20-year retrospective review, we identified 66 patients who were diagnosed with either CBE or CE and had available hip imaging (38 males and 28 females). Of these, 11 patients were found to have radiographic evidence of DDH, for an incidence of 16.7% (11/66). Five of these patients had CE, whereas 6 presented with CBE. The first radiographic evidence of DDH was noted at a mean age of 5.75 years (range, birth to 22 y). CONCLUSIONS: We advocate the use of routine hip screening ultrasound in all infants born with either CBE or CE. Early identification of DDH in these patients may allow additional treatment options to coincide with frequently used osteotomy and orthopaedic interventions. LEVEL OF EVIDENCE: Level III-retrospective study.

A Single Center's Experience With Spinal Anesthesia for Pediatric Patients Undergoing Surgical Procedures.

PURPOSE: To perform a single institution review of spinal instead of general anesthesia for pediatric patients undergoing surgical procedures. Spinal success rate, intraoperative complications, and postoperative outcomes including unplanned hospital admission and emergency department visits within seven days are reported. METHODS: Retrospective chart review of pediatric patients who underwent spinal anesthesia for surgical procedures from 2016 until 2022. Data collected included patient demographics, procedure and anesthetic characteristics, intraoperative complications, unplanned admissions, and emergency department returns. RESULTS: The study cohort included 1221 patients. Ninety-two percent of the patients tolerated their surgical procedure without requiring conversion to general anesthesia, and 78% of patients that had spinals placed successfully did not receive any sedation following lumbar puncture. The most common intraoperative event was systolic blood pressure below 60 mm Hg (14%), but no cases required administration of vasoactive agents, and no serious intraoperative adverse events were observed. Post-Anesthesia Care Unit Phase I was bypassed in 72% of cases with a median postoperative length of stay of 84 min. Forty-six patients returned to the emergency department following hospital discharge, but no returns were due to anesthetic concerns. CONCLUSIONS: Spinal anesthesia is a viable and versatile option for a diversity of pediatric surgical procedures. We noted a low incidence of intraoperative and postoperative complications. There remain numerous potential advantages of spinal anesthesia over general anesthesia in young pediatric patients particularly in the ambulatory setting. LEVEL OF EVIDENCE: IV. TYPE OF STUDY: Retrospective cohort treatment study.

Surgical treatment of high urogenital sinuses using the anterior sagittal transrectal approach: a useful strategy to optimize exposure and outcomes.

PURPOSE: Surgical management of the high urogenital sinus remains challenging. The anterior sagittal transrectal approach provides optimal exposure, facilitates vaginal dissection and separation from the urethra, and allows reconstruction of the bladder neck musculature. In this study we report our initial experience with this technique. MATERIALS AND METHODS: We performed a retrospective review of a 6-year multi-institutional experience treating patients with a urogenital sinus anomaly using the anterior sagittal transrectal approach without preoperative colostomy or prolonged postoperative fasting. Variables analyzed included patient age, associated malformations, the need for additional procedures and surgical outcomes. RESULTS: A total of 23 children with a mean age of 2.3 years (range 3 months to 17 years) who underwent surgery between 2003 and 2010 were included in the study. Mean followup was 3.4 years (range 14 months to 7 years). All children had a high urogenital sinus with (16) or without (7) congenital adrenal hyperplasia. There were 3 isolated cases treated with additional procedures. Only 1 anterior sagittal transrectal approach related complication was encountered when a perineal infection developed in a child and required temporary diverting colostomy without compromising the repair. There were no postoperative urethrovaginal fistulas. All toilet trained patients were continent for feces and most were voiding normally per urethra (21), except for 2 with associated urological malformations. There were 15 patients who underwent followup examination under anesthesia, and demonstrated separate urethral and vaginal openings. CONCLUSIONS: The anterior sagittal transrectal approach provides excellent exposure for the management of a high urogenital sinus, facilitating the separation of urogenital structures. Good outcomes in terms of urinary/fecal continence as well as the absence of urethrovaginal fistulas were achieved in the majority of cases, supporting its consideration for the surgical management of this congenital abnormality.

Effectiveness of a Practical, At-Home Regimen of Parasacral Transcutaneous Electrical Nerve Stimulation in Pediatric Overactive Bladder.

OBJECTIVE: To describe and evaluate efficacy of a more practical, at-home regimen of parasacral transcutaneous electrical nerve stimulation (TENS) for pediatric overactive bladder (OAB). METHODS: We prospectively enrolled patients with OAB. INCLUSION CRITERIA: age 5-13 years and willingness to try TENS. EXCLUSION CRITERIA: urinary tract anatomic abnormalities, current use of OAB medications, neurologic condition, and elevated post-void residual. Patients were instructed to complete 20-minute sessions 2x/day for 1 month. Patients completed the Vancouver Symptom Score (VSS) and 48-hour frequency-volume chart before/after treatment. Compliance was assessed with a daily log. We recorded patient-reported improvement. Primary outcome was difference in VSS before/after treatment; secondary outcomes included: differences in frequency of voids/24 hours, max voided volume in 48 hours (%EBC), mean voided volume (%EBC), and mean number incontinence episodes/24 hours. RESULTS: We enrolled 21 patients (3 male, 18 female; median age 9.9 years). We had complete VSS data on 17 patients and frequency-volume chart data on 12 patients. Median % of TENS sessions completed was 98%. 8/17 patients reported subjective improvement. There was a significant difference between pre- and post-TENS VSS (median score: 23 pre-TENS and 21 post-TENS, P = .009). There were no differences in secondary outcomes before/after treatment. CONCLUSION: In our cohort of medically-refractory OAB pediatric patients, nearly half reported subjective improvement with our regimen, despite modest objective improvement. Our compliance rates suggest this regimen is practical but may be best used as an adjunct to other therapies.

Correlation of anorectal malformation complexity and associated urologic abnormalities.

BACKGROUND: Patients with anorectal malformations (ARM) commonly have associated urologic anomalies. Few large studies exist to accurately characterize the incidence or associations between severity of malformation and urologic diagnosis. The purpose of our study was to determine the incidence of urologic diagnoses in a large cohort of children with ARM and evaluate for any correlation between severity of ARM and the incidence and number of associated urologic diagnoses. METHODS: A retrospective review was performed of patients with ARM treated at our pediatric colorectal center. All patients underwent protocolized urologic screening. ARM subtypes were ordered with increasing severity as follows in males: perineal, bulbar, prostatic and bladder neck fistulae. Females were similarly categorized as perineal, vestibular and vaginal fistulae followed by cloaca with <3 cm common channel and cloaca with >3 cm common channel. The following urologic diagnoses were assessed to determine whether a correlation existed with the severity of the ARM subtype: hydronephrosis, vesicoureteral reflux (VUR), solitary kidney, renal ascent anomalies (ectopic or pelvic), renal fusion anomalies (horseshoe or cross fused kidney), duplex kidney, hypospadias and undescended testicle. ARM subtypes were defined by distal rectal anatomy. RESULTS: A total of 712 patients were included in our study with a mean age of 4 years and of whom 45% were male. The overall rate of urologic anomalies was greater in males than females (65% vs 56% p < 0.026). In both sexes, the rate of urologic anomalies increased with increasing severity of ARM subtype (p<0.00010) finding that males with bladder neck fistula and females with cloacal malformations, particularly with long common channels, being the highest incidence. In males and females, the rate of hydronephrosis increased as the complexity of ARM increased and this correlated significantly (p < 0.0001 vs p < 0.0003 respectively). Similarly, the incidence of VUR also increased as complexity of ARM increased in both males and females (p = 0.01 and p<0.0001 respectively). The remaining urologic diagnoses were not significantly correlated with severity of ARM. CONCLUSIONS: Urologic anomalies occur at a high rate in children with ARM and appear to increase in frequency with increasing complexity of ARM subtype. These findings stress the importance of proper ARM screening and proactive collaboration with a clinician with expertise in pediatric urology early in the management of such children to improve early recognition of urologic diagnoses. LEVEL OF EVIDENCE: Level III.

Surgical outcomes are equivalent after pure laparoscopic and robotic-assisted pyeloplasty for ureteropelvic junction obstruction.

INTRODUCTION: Minimally invasive (robotic and pure laparoscopic) pyeloplasty has been increasingly used for treatment of ureteropelvic junction obstruction (UPJO). However, few large-scale studies have compared these two modalities directly. METHODS: We performed a retrospective single-center review of all patients who underwent pure laparoscopic (LP) or robotic pyeloplasty (RALP) between 2007 and 2018. Patients were excluded if the initial surgery at our institution was a redo pyeloplasty or if they lacked follow-up information. Outcomes of interest included operative time, length of stay, and complication rates, including rates of secondary procedures. We compared these outcomes between groups using Student's t test for continuous variables and a Chi-square for categorical variables. RESULTS: A total of 282 patients were identified. Forty-eight were excluded based on study criteria; therefore, our total study cohort was 234 patients: 119 RALP and 115 LP cases. Overall mean postoperative follow-up time was 20.8 months, with no significant differences between groups. Mean operative time was shorter in the LP group when compared the RALP group (3 h 7 min vs. 3 h 41 min, p < 0.001). There were no significant differences between groups in length of stay (1.22 days vs 1.50 days, p = 0.095). Complications occurred in 52 patients (22.2% of overall cohort) with no difference in incidence between groups. Twenty-five patients (14 in the RALP group and 11 in the LP group) underwent unplanned secondary procedures; 19 of these patients (9 in the RALP group and 10 in the LP group) needed a procedure to address secondary obstruction. CONCLUSION: We demonstrated no significant differences between RALP and LP in regards to complication rates. Surgeons performing RALP and LP have the potential to offer the same level of care for the surgical management of UPJO, especially in countries where robotic technology may not be readily available.

Impact of successful pediatric ureteropelvic junction obstruction surgery on urinary HIP/PAP and BD-1 levels.

INTRODUCTION: In the pediatric patient whose ureteropelvic junction obstruction (UPJO) is not always symptomatic, imaging is the most common means of detecting surgical success. There is interest, however, in other means of post-operative monitoring. A panel of antimicrobial peptides (AMPs) has been previously found to be elevated in UPJO, but the impact of surgical correction on these AMPs is unknown. OBJECTIVE: To determine if elevated levels of candidate urinary AMP biomarkers of urinary tract obstruction decrease following UPJO repair. STUDY DESIGN: Pediatric patients undergoing surgical correction of an UPJO were recruited for participation. Bladder urine from uninfected consenting/assenting patients was collected immediately prior to surgery and then at least 6 months afterward. Based on prior studies demonstrating significant elevation of beta defensin 1 (BD-1), hepatocarcinoma-intestine-pancreas/pancreatitis-associated protein (HIP/PAP), cathelicidin (LL-37), and neutrophil gelatinase-associated lipocalin (NGAL) in patients with UPJO versus control patients, we performed enzyme-linked immunosorbent assays on these four AMPs to compare their expression before and after surgical intervention. If found to significantly decrease, AMP levels were compared to healthy controls. AMP levels were normalized to urine creatinine. Results were analyzed with paired t test or Wilcoxon test using Graphpad software. Correlation was calculated using Pearson or Spearman correlation. A p-value of <0.05 was considered significant. RESULTS: 13 UPJO patients were included in this study; 9 were male (69%). Age at surgery was a median of 4.3 years (average 6.1, range 0.4-18.4 years). Follow-up urine samples were collected a median of 27.4 months after surgery (average 27.4; range 7.8-45.3 months). All 13 patients had clinical improvement and/or signs of improved hydronephrosis on post-operative imaging. HIP/PAP and BD-1 significantly decreased in post-surgical samples compared to pre-surgical samples (p = 0.02 and 0.01, respectively); NGAL and LL-37 did not significantly change. Overall, HIP/PAP decreased in 12 patients (92%) and BD-1 decreased in 11 patients (85%). BD-1 levels after successful repair were not different from healthy controls (p = 0.06). DISCUSSION: Urinary biomarkers of obstruction should detect significant obstructive pathology as well as reflect its resolution. This would enable their use in post-operative monitoring and augment current methods of determining successful surgical outcome through imaging. CONCLUSIONS: The AMPs HIP/PAP and BD-1 are significantly elevated in UPJO but then significantly decrease after pyeloplasty, with BD-1 returning to healthy control levels. As a result, these AMPs could serve as markers of successful surgical intervention.

Urethral triplication with meatuses terminating on the penis: a rare case presenting with bifid urinary stream.

Urethral duplication is an uncommon clinical finding, and classification systems have been described to explain the clinical findings. Urethral triplication is an extremely uncommon diagnosis, with few published case reports. We present the rare case of an infant noted to have a bifid urinary stream and found to have urethral triplication on voiding cystourethrography.

Laparoscopic reconstructive options for obstruction in children with duplex renal anomalies.

OBJECTIVE: To present our initial experience of laparoscopic reconstructive surgery in children with upper urinary tract obstruction associated with duplex anomalies, as although there is much information on ablative procedures such as laparoscopic heminephrectomy, there is little available about minimally invasive reconstructive options for duplex renal anomalies in children. PATIENTS AND METHODS: We retrospectively reviewed four consecutive patients (aged 6-11 years) with duplex anomalies and laparoscopic reconstruction for obstructed, dilated segments treated at our institution. The port placement and surgical exposure were analogous to that for transperitoneal laparoscopic pyeloplasty. A JJ stent was placed retrogradely into the ureter immediately before each procedure. The procedures performed were pyelo-ureterostomy for incomplete duplication and lower pole pelvi-ureteric junction (PUJ) obstruction, lower pole pyeloplasty for lower pole PUJ obstruction and complete duplication, and ipsilateral uretero-ureterostomy and distal ureterectomy for an obstructed, ectopic upper pole. Foley catheters were left indwelling for 36-48 h and stents were removed at 4-6 weeks. Postoperative imaging included either ultrasonography or intravenous urography. RESULTS: Three children presented with intermittent flank pain due to lower pole PUJ obstruction. The other child presented with pyonephrosis and purulent drainage from her vagina due to an ectopic ureter associated with a functioning upper pole segment. All procedures were successfully completed. The only complication was in the first patient (pyelo-ureterostomy) who had transient urinary extravasation that resolved with bladder decompression for 10 days. With a follow-up of 6-18 months, all had resolution of symptoms with improvement in radiographic variables. CONCLUSIONS: This series shows that children with duplex anomalies and obstruction can undergo successful reconstruction using techniques learned with laparoscopic pyeloplasty.

The modified Koyanagi hypospadias repair for the one-stage repair of proximal hypospadias.

Perineal and penoscrotal hypospadias were often managed by two-stage urethroplasty with variable results and significant number of these may need third surgery. Though modified Koyanagi one-stage repair has a learning curve, it has all the advantages of two-stage repair. The aim was to review the results of modified Koyonagi repair from the literature and our own centre experience.

Percutaneous Vesicoscopic Approach for Retrograde Ureteroscopy Following Cohen Cross-trigonal Ureteral Reimplantation.

Obtaining retrograde access into a ureter that has been previously reimplanted in a cross-trigonal fashion can be a challenging procedure. We describe a percutaneous vesicoscopic approach to obtain access into a reimplanted ureter to perform ureteroscopy and laser lithotripsy.

Combination treatment for cicatrix after neonatal circumcision: An office-based solution to a challenging problem.

Cicatrix formation, which can form after neonatal circumcision to entrap the glans penis, presents a therapeutic challenge. Previous studies in the literature have described either using a topical steroid cream or stretching of the scar tissue with an instrument but not a combination of both modalities. In our experience, monotherapy has resulted in significant recurrence and/or need for further treatment. We present our successful experience that combines cicatrix stretching with a hemostat using local anesthesia in the office followed by several weeks of topical steroids with a minority of patients needing any additional therapy.

Normal pelvic ultrasound or MRI does not rule out neoplasm in patients with gonadal dysgenesis and Y chromosome material.

INTRODUCTION: Patients with gonadal dysgenesis (GD) with a Y chromosome have an increased risk of gonadal neoplasm. Few data exist on the ability of imaging to detect malignancy in intra-abdominal gonads in these patients. OBJECTIVE: We aimed to determine the correlation between preoperative imaging findings and gonadal pathology in GD patients with Y chromosome material. METHODS: A retrospective review was performed of patients with XY or XO/XY GD who underwent gonadectomy at our institution from 2003 to 2017. Patients were assessed preoperatively with ultrasonography; some additionally underwent MRI. RESULTS: The series consisted of 10 patients, all with female gender and non-palpable gonads. Median age was 13.1 years (range 2.4-18.3 years). Overall, four of the ten patients (40%) had a tumor (gonadoblastoma or dysgerminoma) on final pathology. Four patients had a gonad or gonads that were definitively seen on ultrasonography. All visualized gonads were described as "normal" or "small" with the exception of one patient, who had a normal MRI. Three of the four patients in this group had a tumor on final pathology. The remaining six patients had a gonad or gonads that were not definitively visualized on ultrasound; one patient in this group had a tumor on final pathology. Overall, five of seven gonads (71%) definitively visualized on ultrasound had tumor on final pathology, and two of thirteen gonads (15%) not visualized on ultrasound had tumor on final pathology; this difference was statistically significant (p = 0.012). Three patients were imaged with MRI. Of the gonads that could be visualized on MRI, no definitive abnormalities were seen. All patients imaged with MRI had tumors on final pathology. DISCUSSION: Both ultrasound and MRI are relatively poor at identifying and characterizing intra-abdominal gonads in GD patients. The majority of patients who had a neoplasm had normal imaging findings. Gonads that were definitively visualized on ultrasound were more likely to contain neoplasms that could not be visualized, which perhaps because of tumor growth. No other consistent imaging findings of malignancy were found. Our study included ultrasound evaluations that were completed over 10 years ago and not performed by pediatric ultrasonographers, which may have biased the results. However, results suggest that when discussing gonadectomy with GD patients, one should not be reassured by "normal" imaging findings. Neither ultrasound nor MRI should be relied on for surveillance in GD patients who decide against gonadectomy. CONCLUSION: A normal ultrasound or MRI does not rule out neoplasm in GD patients with intra-abdominal gonads.

Simultaneous Robotic-Assisted Laparoscopy for Bladder and Bowel Reconstruction.

INTRODUCTION: Patients with neurogenic bladder frequently also have bowel dysfunction and a simultaneous urologic and colorectal reconstruction is possible. We present our experience with combined reconstructive procedures using robot-assisted laparoscopy, and demonstrate the utility of a minimally invasive approach that considers both the bowel and bladder management of these patients. METHODS: We retrospectively reviewed all patients who underwent combined bowel and urologic reconstruction at our institution since the start of our multidisciplinary robotic program. RESULTS: Seven patients were identified in our cohort with a mean age of 6.4 years (3.8-10.1 years). Six patients had myelomeningocele and 1 had caudal regression. Malone appendicostomies were placed in all 7 patients. A split appendix technique was used as a conduit in 5 patients, in situ appendix in 1, and neoappendicostomy with cecal flap in 1. Six patients had a Mitrofanoff appendiceal conduit created, while 1 patient had a sigmoid colovesicostomy for urinary diversion. Five patients required bladder neck repair. One patient had stenosis of the Mitrofanoff and one patient had an anastomotic leak of the sigmoid anastomosis. The average operating time was 526 minutes (313-724 minutes). The median length of stay (LOS) was 5 days (4-7 days), excluding one outlier who suffered an anastomotic leak and had an extended LOS (50 days). All patients who underwent continent bladder reconstruction are dry on their current catheterizing regimen, 6/7 are clean with antegrade flushes. CONCLUSION: Patients with neurogenic bladder often have coexisting bowel dysfunction, which provides an opportunity to reconstruct both organ systems simultaneously and achieve social urinary and bowel continence. Before committing to any intervention, the surgeon should consider both the urologic and gastrointestinal needs of the patient, and perform the needed procedures simultaneously. We describe a number of combined operations aimed at bowel and bladder management that can be performed safely using robot-assisted laparoscopy.