Spinal dysraphism in exstrophy: a single-center study of a 39-year prospective database.

OBJECTIVE: The two main objectives of this study were to explore the rate of spinal dysraphism within bladder and cloacal exstrophy and to analyze the relationship between spinal dysraphism surgery, including timing of spinal dysraphism surgery, with urological and neurological outcomes. METHODS: A prospectively maintained IRB-approved database of pediatric exstrophy patients treated from 1982 to 2021 was retrospectively reviewed for patients with spinal dysraphism. Spinal dysraphism was categorized into the following 7 subtypes: lipoma-based closed defect, myelomeningocele, meningocele, diastematomyelia, myelocystocele, low-lying conus with tethered cord/fatty filum, and sacral bony defect. Other factors assessed included patient demographic characteristics, type of spinal dysraphism procedure, reoperation, complication, presence of other neurological problems (e.g., hydrocephalus, Chiari malformation), neurological status, and urological function. RESULTS: Analysis revealed that 114/1401 patients had coexisting spinal dysraphism. Of these 114, sufficient records including type of dysraphism were available for 54. Spinal dysraphism was most common within cloacal exstrophy (83.3% [45/54 patients]), followed by cloacal exstrophy variants (9.3% [5/54]), classic bladder exstrophy (3.7% [2/54]), and classic bladder exstrophy variants (3.7% [2/54]). Within spinal dysraphism, lipoma-based closed defects (63.0% [34/54]) and low-lying conus with tethered cord/fatty filum (11.1% [6/54]) were most common. Hydrocephalus and Chiari malformation occurred in 24.1% (13/54) and 11.1% (6/54) of patients. All 13 patients with hydrocephalus underwent shunt placement. Among those who underwent neurosurgical intervention, the complication rate for spinal dysraphism was 14.6% (7/48). Motor function data were available for 41 patients and revealed that motor function declined for 2/41 (4.8%) patients and improved for 6/41 (14.6%) after neurosurgery. There was no statistical difference in lower-extremity motor outcome related to timing of neurosurgery and exstrophy closure. CONCLUSIONS: The authors have reported the surgical management and outcomes of patients with exstrophy and coexisting spinal dysraphism (n = 54). In 54 patients, spinal dysraphism was most common in the subset of patients with cloacal exstrophy (83.3%). Lipoma-based closed defects (63.0%) and low-lying conus with tethered cord/fatty filum (11.1%) were the most common, and the rates of hydrocephalus and Chiari malformation were 24.1% and 11.1%, respectively. There was no difference in lower-extremity motor outcome related to timing of neurosurgery and exstrophy closure.

New Insights on the Basic Science of Bladder Exstrophy-epispadias Complex.

The exstrophy-epispadias complex is a rare congenital anomaly presenting as a wide spectrum of disorders. The complex nature of this malformation leads to continuous investigations of the basic science concepts behind it. Elucidating these concepts allows one to fully understand the mechanisms behind the disease in order to improve diagnosis, management, and treatment ultimately leading to improvement in patient quality of life. Multiple technological advancements within the last 10 years have been made allowing for new studies to be conducted. Herein, the authors conduct a literature review of studies from 2009 to 2019, considering novel theories regarding the genetics, embryology, bladder, bony pelvis, prostate, and genitalia of patients with bladder exstrophy-epispadias complex.

Attitudes towards child life specialists and their utilization within pediatric urology.

INTRODUCTION: The American Academy of Pediatrics views Certified Child Life Specialists (CCLS) as "an important component of pediatric hospital based care to address the psychosocial concerns that accompany hospitalization." CCLSs help patients and parents navigate the complex medical system in order to minimize psychosocial and emotional stress by implementing age appropriate coping skills. This survey explores the perceptions towards CCLS and their utilization with pediatric urology. MATERIALS AND METHODS: A Survey Monkey questionnaire was developed and distributed to Society for Pediatric Urology members (SPU) (n = 314). Providers were queried about CCLS implementation and their perception regarding CCLS's role in improving health related quality of life (HRQOL). RESULTS: There was a 34.1% response rate (n = 107). Ninety-four providers (87.9%) reported CCLS interaction with their patients and greater than 95% of providers felt CCLS imparted some degree of benefit to their patients' HRQOL. Only 4.7% felt CCLS offered no benefit to the patients. CCLS were consistently used in a minority of inpatient and outpatient settings and never in the radiological setting. They were used at least 50% of the time by the most responders in inpatient and radiological setting and nearly the same in the ambulatory surgery setting. CONCLUSIONS: This survey illuminates that the majority of providers interact with CCLS in clinical settings and believe their involvement is beneficial. However, CCLSs are under-utilized during invasive urological procedures where patient anxiety is high. By understanding perceptions of providers and their practice patterns we can overcome barriers to CCLS use and improve their quality of life.

Influential Factors in Pursuit of Pediatric Urology Fellowship Training.

INTRODUCTION: We investigated factors that helped recent fellows achieve a successful match and sought to understand the influences in pursuing a career in pediatric urology. Selecting to continue training after urology residency entails much consideration. However, there are few tangible resources available to help residents understand the process. METHODS: A 20-item web based survey was e-mailed to second year fellows and recent graduates of pediatric urology fellowships (103) from the graduating classes of 2013 to 2015. The survey explored factors that affected the decision to pursue this field along with the influence of mentors, fellows and other residents who applied to pediatric urology. We assessed the key factors that helped applicants achieve a successful match. Descriptive statistics and univariate logistic regression analysis were used to determine relationships between program characteristics and respondents' decisions to apply and interview at various programs. RESULTS: In total, 59 of 103 individuals surveyed (57.3%) responded to the survey. Faculty, program reputation and clinical volume had key roles in ranking programs, while satisfaction with the match result was contingent upon accurately assessing programs based on key factors such as family considerations, location, program reputation, faculty, clinical year volume and gut feeling. In addition, our survey demonstrated that having a pediatric urology fellow at one's institution had little impact on a resident's decision to pursue pediatric urology. CONCLUSIONS: As the subspecialty of pediatric urology continues to grow, we identified key elements that help shape and influence choices to participate in the future of this ever evolving field.

Prediction of surgical complications in the elderly: Can we improve outcomes?

As the number of Americans aged 65 years and older continues to rise, there is projected to be a corresponding increase in demand for major surgeries within this population. Consequently, it is important to utilize accurate preoperative risk stratification techniques that are applicable to elderly individuals. Currently, commonly used preoperative risk assessments are subjective and often do not account for elderly-specific syndromes that may pose a hazard for geriatric patients if not addressed. Failure to accurately risk-stratify these patients may increase the risk of postoperative complications, morbidity, and mortality. Therefore, we aimed to identify and discuss the more objective and better-validated measurements indicative of poor surgical outcomes in the elderly with special focus on frailty, patient optimization, functional status, and cognitive ability.