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Item Age-based risk of end-stage kidney disease in patients with myelomeningocele(Elsevier, 2023-04) Adams, Cyrus M.; Misseri, Rosalia; Roth, Joshua D.; Whittam, Benjamin M.; Guckien, Zoe E.; King, Shelly J.; Kaefer, Martin; Rink, Richard C.; Szymanski, Konrad M.; Urology, School of MedicineObjective We aimed to quantify end-stage kidney disease (ESKD) risk after infancy in individuals with myelomeningocele (MMC) followed by urology in the modern medical era and to assess if ESKD risk was higher after surgery related to a hostile bladder. Methods We retrospectively reviewed patients with MMC followed by urology at our institution born ≥ 1972 (when clean intermittent catheterization was introduced) past 1 year of age (when mortality is highest, sometimes before establishing urology care). ESKD was defined as requiring permanent peritoneal/hemodialysis or renal transplantation. Early surgery related to hostile bladder included incontinent vesicostomy, bladder augmentation, detrusor Botulinum A toxin injection, ureteral reimplantation, or nephrectomy for recurrent urinary tract infections. Survival analysis and proportional hazards regression were used. Sensitivity analyses included: risk factor analysis with only vesicostomy, timing of surgery, including the entire population without minimal follow-up (n = 1054) and only patients with ≥ 5 years of follow-up (n = 925). Results Overall, 1029 patients with MMC were followed for a median of 17.0 years (49% female, 76% shunted). Seven patients (0.7%) developed ESKD at a median 24.3 years old (5 hemodialysis, 1 peritoneal dialysis, 1 transplantation). On survival analysis, the ESKD risk was 0.3% at 20 years old and 2.1% at 30 years old (Figure). This was ∼100 times higher than the general population (0.003% by 21 years old, p < 0.001). Patients who underwent early surgery for hostile bladder had higher ESKD risk (HR 8.3, p = 0.001, 6% vs. 1.5% at 30 years). On exploratory analyses, gender, birth year, shunt status and wheelchair use were not associated with ESKD risk (p ≥ 0.16). Thirty-year ESKD risk was 10% after early vesicostomy vs. 1.4% among children without one (p = 0.001). Children undergoing bladder surgery between 1.5 and 5 years old had a higher risk of ESKD. No other statistically/clinically significant differences were noted. Comment Patients with MMC remain at risk of progressive renal damage throughout life. We relied on the final binary ESKD outcome to quantify this risk, rather than imprecise glomerular filtration rate formulas. Analysis was limited by few people developing ESKD, inconsistent documentation of early urodynamic findings and indications for bladder-related surgery. Conclusions While ESKD is relatively uncommon in the MMC population receiving routine urological care, affecting 2.1% of individuals in the first 3 decades, it is significantly higher than the general population. Children with poor bladder function are likely at high risk, underlining the need for routine urological care, particularly in adulthood.Item Characteristics of Female Genital Restoration Surgery for Congenital Adrenal Hyperplasia Using a Large-scale Administrative Database(Elsevier, 2018) Roth, Joshua D.; Casey, Jessica T.; Whittam, Benjamin M.; Bennett, William E., Jr.; Szymanski, Konrad M.; Cain, Mark P.; Rink, Richard C.; Urology, School of MedicineObjective To analyze nationwide information on the timing of surgical procedures, cost of surgery, hospital length of stay following surgery, and surgical complications of female genital restoration surgery (FGRS) in females with congenital adrenal hyperplasia (CAH). Materials and Methods We used the Pediatric Health Information System database to identify patients with CAH who underwent their initial FGRS in 2004-2014. These patients were identified by an International Classification of Diseases, Ninth Revision (ICD-9) diagnosis code for adrenogenital disorders (255.2) in addition to a vaginal ICD-9 procedure code (70.x, excluding vaginoscopy only) or perineal ICD-9 procedure code (71.x), which includes clitoral operations (71.4). Results A total of 544 (11.8%) females underwent FGRS between 2004 and 2014. Median age at initial surgery was 9.9 months (interquartile range 6.8-19.1 months). Ninety-two percent underwent a vaginal procedure, 48% underwent a clitoral procedure, and 85% underwent a perineal procedure (non-clitoral). The mean length of stay was 2.5 days (standard deviation 2.5 days). The mean cost of care was $12,258 (median $9,558). Thirty-day readmission rate was 13.8%. Two percent underwent reoperation before discharge, and 1 (0.2%) was readmitted for a reoperation within 30 days. Four percent had a perioperative surgical complication. Conclusion Overall, 12% of girls with CAH underwent FGRS at one of a national collaborative of freestanding children's hospitals. The majority underwent a vaginoplasty as a part of their initial FGRS for CAH. Clitoroplasty was performed on less than half the patients. Overall, FGRS for CAH is performed at a median age of 10 months and has low 30-day complication and immediate reoperation rates.Item Epidural Analgesia Decreases Narcotic Requirements in Low Level Spina Bifida Patients Undergoing Urologic Laparotomy for Neurogenic Bladder and Bowel(Elsevier, 2018) Roth, Joshua D.; Misseri, Rosalia; Whittaker, Stephanie C.; Monn, M. Francesca; Horn, Nicole D.; Cain, Mark P.; Green, Morton C.; Urology, School of MedicinePurpose Concern of anatomical anomalies and worsening neurologic symptoms has prevented widespread use of epidural catheters in patients with low level spina bifida (LLSB). We hypothesize that thoracic epidural placement in the T9-T10 interspace is safe and decreases narcotic requirements in LLSB patients following major open lower urinary tract reconstruction (LUTR). Materials and Methods We reviewed consecutive LLSB patients who had LUTR and epidurals for post-operative pain control. Controls were LLSB patients who received single shot transversus abdominis plane (TAP) blocks with similar procedures. Complications from epidural placement, including changes in motor and sensory status were recorded. Opioid consumption was calculated utilizing equivalent IV morphine doses. Mean and maximum pain scores on post-operative day (POD) 0-3 were calculated. Results 10 LLSB patients who had lower urinary tract reconstruction and epidurals were matched to 10 LLSB patients who had lower urinary tract reconstruction and transverse abdominis plane blocks. Groups were demographically similar. All had full abdominal sensation and functional levels at or below L3. No epidural complications or changes in neurological status were noted. The epidural group had decreased opioid consumption on POD 0-3 (0.75 mg/kg vs. 1.29 mg/kg, p=0.04). Pain scores were similar or improved in the epidural group. Conclusions Thoracic epidural analgesia appears to be a safe and effective opioid sparing option to assist with post-operative pain management following lower urinary tract reconstruction in LLSB patients.Item Incidence of pathologic postobstructive diuresis after resolution of ureteropelvic junction obstruction with a normal contralateral kidney(Elsevier, 2018) Roth, Joshua D.; Lesier, Jeffrey D.; Casey, Jessica T.; Szymanski, Konrad M.; Whittam, Benjamin M.; Misseri, Rosalia; Rink, Richard C.; Cain, Mark P.; Pediatrics, School of MedicineIntroduction Postobstructive diuresis (POD) after unilateral pyeloplasty or percutaneous nephrostomy (PCN) tube insertion for ureteropelvic junction obstruction (UPJO) in patients with a normal contralateral kidney is not well described. Objective The objective of this study was to determine the incidence and characteristics of POD after relief of unilateral UPJO in patients with a normal contralateral kidney. Study design Children who underwent a unilateral pyeloplasty or PCN for UPJO from 2010 to 2017 with a normal contralateral kidney were retrospectively reviewed. Postobstructive diuresis was defined as urine output (UO) of >300% of expected UO. Patients with a solitary kidney or those who underwent bilateral pyeloplasty or bilateral PCN tube placement were excluded. Results Out of 396 children meeting inclusion criteria, seven (1.8%) developed POD (4 after pyeloplasty and 3 after PCN tube placement). Median age at intervention was 1.7 years (range 11 days–18 years); median weight was 11.4 kg (range 3.7–54.2 kg). Postobstructive diuresis was more likely to occur in patients with grade 4 hydronephrosis (3.0%) and larger kidneys and if a PCN tube was placed before pyeloplasty. There was no significant difference in age, gender, kidney laterality, or function between those who developed POD and those who did not. Postobstructive diuresis was managed with additional intravenous fluids and electrolyte monitoring. Median initial postprocedure UO was 5.9 mg/kg/hr (range 3.2–10.0 mg/kg/hr). In five children who underwent PCN in whom UO could be differentiated between kidneys, median initial postprocedure UO was 6.1 mg/kg/hr (range 2.5–9.1 mg/kg/hr) from the affected side and 0.8 mg/kg/hr (range 0.4–0.9 mg/kg/hr) from the unaffected side. The median length of time to resolution of POD was 3 days (range 2–4 days). One patient developed significant acidosis and lethargy that improved with intravenous fluid management. Mild hyponatremia developed in two, hypokalemia in one, hypophosphatemia in one, acidosis in one, and hypoglycemia in 1 patient. Discussion A low but clinically significant risk of POD occurring after relief of unilateral UPJO in children with a normal contralateral kidney is described. Limitations include retrospective analysis and small sample size due to the rarity of the condition. Conclusion Postobstructive diuresis after decompression of UPJO in patients with a normal contralateral kidney is a rare event (1.8%). However, POD does occur, and patients should be carefully monitored after these procedures given the potential for significant dehydration and electrolyte disturbances.Item A novel technique for direct visualization of reservoir placement for penoscrotal inflatable penile prostheses using a single incision(Wolters Kluwer, 2018-10) Roth, Joshua D.; Monn, M. Francesca; Shelton, Thomas M.; Mellon, Matthew J.; Urology, School of MedicineIntroduction: We aim to present a modified technique and outcomes of a novel method allowing for direct visualization of the reservoir placement during a penoscrotal inflatable penile prosthesis (IPP). Methods: Out of165 patients who underwent IPP placement from August 2012 to March 2015, 157 underwent a modified technique and comprised the cohort of this study. A Deaver's retractor was placed lateral to the penis and over the pubic bone to allow for direct visualization of the tissues overlying the lower abdomen. After dissecting through the superficial layers, the Deaver's was slowly advanced, allowing for visualization of the fascia, which was incised. Using blunt dissection, a space for the reservoir was created between the bladder and the pubic bone. The reservoir was then placed safely into this space and the Deaver's retractor was removed. Results: The causes of ED in the study cohort included postprostatectomy ED (n = 107), organic impotence (n = 40), Peyronie's disease (n = 3), ED following cystoprostatectomy (n = 2), ED due to spinal cord injury (n = 2), ED resulting from priapism (n = 2), and ED after pelvic injury (n = 1); all of which were refractory to medical management. The median age of study population was 66 years and the mean (standard deviation) operative time was 72.8 (14.7) min. Eighty percent of the procedures were performed on outpatient basis. Complication rates were low (<5%), with four infections, one proximal pump migration, one scrotal hematoma, and one urinary tract infection. Conclusion: The modified technique for placement of the IPP's spherical reservoir under direct visualization through a penoscrotal incision is quick, safe, and effective.Item An ovulating follicle presenting as a testicular mass in a teenage patient with ovotesticular DSD(Elsevier, 2018-01-31) Roth, Joshua D.; Haddad, Nadine G.; Albright, Eric A.; Cheng, Liang; Rink, Richard C.; Kaefer, Martin; Urology, School of MedicineItem Radiographic abnormalities, bladder interventions, and bladder surgery in the first decade of life in children with spina bifida(Springer, 2019-07) Rensing, Adam J.; Szymanski, Konrad M.; Misseri, Rosalia; Roth, Joshua D.; King, Shelly; Chan, Katherine; Whittam, Benjamin M.; Kaefer, Martin; Rink, Richard C.; Cain, Mark P.; Urology, School of MedicineBackground Spina bifida (SB) patients are at increased risk for hydronephrosis, bladder storage and emptying problems, and renal failure that may require multiple bladder surgeries. Methods We retrospectively reviewed patients born with SB 2005–2009, presenting to our institution within 1 year of birth. Outcomes at 8–11 years old included final renal/bladder ultrasound (RBUS) results, clean intermittent catheterization (CIC) use, anticholinergic use, surgical interventions, and final renal function. We excluded those without follow-up past age 8 and/or no RBUS or fluoroscopic urodynamic images (FUI) within the first year of life. Imaging was independently reviewed by four pediatric urologists blinded to radiologists’ interpretation and initial findings compared with final outcomes. Results Of 98 children, 62 met inclusion criteria (48% male, 76% shunted). Median age at last follow-up was 9.6 years. Upon initial imaging, 74% had hydronephrosis (≥ SFU grade 1), decreasing to 5% at 10 years (p < 0.0001). Initially, 9% had ≥ SFU grade 3 hydronephrosis, decreasing to 2% (p = 0.13). CIC and anticholinergic use increased from 61% and 37% to 87% and 86%, respectively (p = 0.001 and p < 0.0001, respectively). With follow-up, 55% had surgical intervention and 23% had an augmentation. Of children with a serum creatinine/cystatin-C at 8–11 years old, one had confirmed chronic kidney disease (stage 2). Conclusions Despite initial high incidence of hydronephrosis, this was low grade and resolved in the first decade of life. Additionally, the 8–11-year incidence of kidney disease and upper tract changes was low due to aggressive medical management.Item Sexual activity and function of adult men with spina bifida(Elsevier, 2023-06) Szymanski, Konrad M.; Roth, Joshua D.; Hensel, Devon J.; Wiener, John S.; Younsi, Nina; Stein, Raimund; Whittam, Benjamin; Kaefer, Martin; Rink, Richard C.; Cain, Mark P.; Misseri, Rosalia; Urology, School of MedicineObjective Data on sexual function of men with spina bifida (SB) is limited. We aimed to assess sexual activity and erectile dysfunction (ED) in a large international sample of men with SB. Methods Men with SB (≥18yo) were recruited in an international online survey via clinics and social media. We collected data on demographics, ambulation (Hoffer classification), penile rigidity (Erection Hardness Score), sexual activity and ED (International Index of Erectile Function). Non-parametric tests were used. Results A total of 162 men (median age 35, 62% shunted, 38% community ambulators) reported sexual desire similar to the general population (p = 0.82), but 55% were dissatisfied with their sex life (Summary Table). Overall, 36% reported full penile rigidity with erections, more commonly with better ambulation (p = 0.01), 69% had ever experienced orgasm and 84% ejaculated. In 44 men (27%) attempting sexual intercourse in the last 4 weeks, 59% had ED (11% severe, 7% moderate, 14% mild-moderate, 27% mild). In this group, 91% of men reporting less than full penile rigidity had ED, compared to 30% with full penile rigidity (p = 0.001). Overall, partnered non-genital contact in the last 3 months was reported by 56%, solo masturbation: 62%, partnered intercourse: 48% (31% vaginal). Of 54 men who used phosphodiesterase type 5 inhibitors (PDE5I), 80% reported improved erections, 56% improved intercourse. Overall, 40% reported non-genital erogenous zones as most pleasurable, especially with poorer ambulation (p = 0.002, chest/nipples: 73%). Comment Strengths of this study include anonymous, voluntary, online participation maximizing participation of a heterogenous, international population. Whenever available, we compared findings to published values for the general population. Since romantic and sexual activity is a complex intersection of interest, opportunity and ability, a more comprehensive assessment was beyond the study's scope. Future work will focus on the interplay with issues like incontinence. Conclusions ED was frequent among men with SB, especially in men with poorer ambulation. PDE5 inhibitors may be beneficial. Partnered sexual activity was reported by half of the men, although it may not involve penetrative intercourse.Item Solo and Partnered Sexual Behavior Among an International Sample of Adults with Spina Bifida(Oxford, 2022-12) Hensel, Devon J.; Misseri, Rosalia; Wiener, John S.; Roth, Joshua D.; Whittam, Benjamin M.; Cain, Mark P.; Szymanski, Konrad M.; Pediatrics, School of MedicineBackground Spina bifida (SB) may differentially impact adults’ participation in solo and partnered sexual behaviors, but little research investigates this topic. Aim Describe solo and partnered sexual behaviors among an international sample of adult men and women with SB. Main Outcome Measures Ever participated (no/yes) and recent participation (>1 year ago/within last year) in solo masturbation, cuddled with a partner, held hands with a partner, kissed a partner, touched a partner's genital, had genitals touched by a partner, gave a partner oral sex, received oral sex from a partner, vaginal sex, anal sex, and sex toy use. Methods Data were drawn from a larger cross-sectional, internet-based survey assessing the sexual behaviors of an international sample of men and women with SB. We used logistic regression to examine the impact of background (gender, age, independent living, and relationship status) and health (shunt status, ambulation, and genital sensation) factors on each outcome. Results The sample consisted of 345 respondents aged 18–73 years from 26 nations. Very few (<3%) had no lifetime experience with any solo or partnered behaviors; 25.0% reported participating in all behaviors at some point in their lives. The median number of past year sexual behaviors (of 16 total) was 7. Lifetime and recent participation were associated with demographic and health factors. Clinical Implications Despite impairment, adults with spina bifida do participate in solo and partnered sexual behaviors. Medical personnel who work with this population should include discussions about sexuality as part of routine care. Strengths & Limitations Although this research measured solo and partnered sexual behavior in large international sample of adults with spina bifida, it is limited by its cross-sectional retrospective design and non-clinical convenience sample. Conclusion Despite disability, many adults with SB participate in solo and partnered sexual behavior. Medical and psychosocial supports are needed to help adults in this population enjoy sexuality in a healthy and safe manner.Item Transitioning Young Adults with Neurogenic Bladder – Are We Asking Too Much?(Elsevier, 2019) Roth, Joshua D.; Szymanski, Konrad M.; Ferguson, Ethan J.; Cain, Mark P.; Misseri, Rosalia; Pediatrics, School of MedicineIntroduction Significant numbers of young adults with chronic health conditions fail to transition. Objective We aimed to evaluate how ready urologic patients who have transitioned were actually prepared for that process. Due to the cognitive impairments frequently seen with spina bifida (SB), we hypothesize that these individuals will be less prepared to transition medical care to adult providers compared to their healthy counterparts. Methods Participants included consecutive patients in the transitional SB clinic at our institution and controls (college student without obvious physical disability or interest in healthcare related fields aged 18-25). Both groups were administered the Transition Readiness Assessment Questionnaire (TRAQ) over a nine-month period. Five TRAQ domains assess 20 skills necessary to transition. Likert scale responses range from 1 “No, I do not know how” to 5 “Yes, I always do this when I need to” (which we considered appropriate for transitioned patients). Demographics and the number of daily medications taken were collected. Patients and healthy controls were compared using 1) total and domain TRAQ scores, 2) the proportion of non-transitioned skills (“1”) and 3) fully transitioned skills (“5”). Non-parametric statistics were used. Results 43 unique SB patients (30.8% shunted, 46.5% female) and 100 controls were enrolled. SB patients were older than controls (21 vs 20 years, p<0.001). There was no gender difference between groups (p=0.33). Transitioned patients and college students were fully transitioned only in the “Talking with Providers” domain ( Figure ). College students performed significantly better than patients in the domains of “Appointment Keeping” (p=0.04) and “Tracking Health Issues” (p=0.02). Transitioned patients were less likely to be interested in learning how to perform skills in the domains of “Appointment Keeping” and “Tracking Health Issues” (p<0.001 for both domains). Discussion We describe the transition readiness of young adults with SB compared to healthy controls and other youths with chronic health conditions. Limitations include the small sample size, potentially limiting generalizability, as well as cross-sectional nature. Conclusion “Transitioned” patients with SB had lower TRAQ scores in some domains compared to healthy college students, who themselves had scores indicating that they were not fully ready for transition. Increased attention to transition readiness in people with SB is necessary, as even healthy young adults struggle with these tasks and are poorly prepared for transition.