Contrast-enhanced voiding urosonography (CEVUS) as a safe alternate means of assessing vesicoureteral reflux in pediatric kidney transplant patients.

BACKGROUND: Although voiding cystourethrogram (VCUG) is currently the gold standard in VUR evaluation, there is ionizing radiation exposure. Contrast-enhanced voiding urosonography (CEVUS) uses ultrasound contrast agents to visualize the urinary tract and has been reported to be safe and effective in VUR evaluation in children. CEVUS application has yet to be specifically described in VUR evaluation in the pediatric kidney transplant population. The purpose of this study was to report the use of CEVUS and VCUG in evaluating and managing VUR in pediatric renal transplant patients. METHODS: Retrospective review was conducted for pediatric kidney transplant patients (18 years and younger) who underwent VCUG or CEVUS to assess for transplant VUR from July 2019 through June 2021. Demographic information, reason for VUR evaluation, fluoroscopy time, and postimaging complications were evaluated. Costs of imaging modalities were also considered. RESULTS: Eight patients were evaluated for transplant VUR during the study period. Of the 3 patients who underwent VCUG, all 3 had VUR (median grade 3). Median fluoroscopy time was 18 s and dose-area product was 18.7 uGy*m2 . Of the 5 patients who underwent CEVUS, 4 had VUR (median grade 4). There were no complications for either modality. Based on clinical and radiographic findings, patients were recommended no intervention, behavioral modification, or ureteral reimplantation. The total cost of CEVUS was $800 less than that of VCUG. CONCLUSION: CEVUS can provide an alternate means of safely evaluating VUR in kidney transplant patients with similar outcomes, potentially lower costs, and no exposure to ionizing radiation.

Detection and Management of Tethered Cord in Anorectal Malformation: A Survey of Pediatric Neurosurgeons in the United States.

INTRODUCTION: The reported prevalence of tethered spinal cord in patients with anorectal malformations (ARMs) ranges from 9% to 64%. Practice patterns surrounding the diagnosis and management of tethered cord (TC) are suspected to vary, with consideration to the type of spine imaging, adjunct imaging modalities, what patients are offered surgical intervention, and how patients are followed after detethering. We sought to determine what consensus, if any, exists among pediatric neurosurgeons in the USA in terms of diagnosis and management of TC and, specifically, patients with TC and ARM. METHODS: A survey was sent to members of the American Society of Pediatric Neurosurgeons (ASPN). Members of the ASPN received an email with a link to an anonymous REDCap survey that asked about their experience with detethering procedures, indications for surgery, diagnostic tools used, and follow-up protocols. RESULTS: The survey was completed by 93 of the 192 ASPN members (48%). When respondents were asked about the total number of all simple filum detetherings they performed annually, 61% (N = 57) indicated they perform less than 10 for all TC patients. Ninety-three percent (N = 87) of neurosurgeons performed these procedures in patients with simple filum TC and ARM patients (TC + ARM) specifically. When asked about prophylactic detethering in those with a confirmed diagnosis of low-lying conus and with a filum fatty terminale, 59.1% (N = 55) indicated they would offer this to TC + ARM patients regardless of their age. Regarding preoperative workup for simple filum detethering, all respondents indicated they would order an MRI in both TC and TC + ARM patients, with a minority also requiring additional testing such as urodynamics, neurodevelopmental assessments, and anorectal manometry for both groups. When following patients postoperatively, almost all respondents indicated they would require clinical neurosurgical follow-up with a clinic visit (100% in all simple filum TC patients, 98.9% in fatty filum/low-lying conus TC + ARM patients), but there was wide variation in the use of other tools such as urological testing, neurodevelopmental assessment, and anorectal manometry. DISCUSSION/CONCLUSIONS: A wide variety of diagnostic criteria and indication for procedural intervention exists for management of TC patients with and without ARM. Further studies are needed to determine outcomes. Prospective protocols need to be developed and evaluated to standardize care for this patient population and determine best practices.

Integration of child life services in the delivery of multi-disciplinary differences in Sexual Development (DSD) and Congenital Adrenal Hyperplasia (CAH) care.

INTRODUCTION: Multiple studies have demonstrated the benefit of incorporating certified child life specialist (CCLS) services in various aspects of pediatric care. Although the significance of psychosocial support of patients with Disorders of Sexual Development (DSD) and Congenital Adrenal Hyperplasia (CAH) is increasingly recognized, the involvement of CCLS services into the DSD and CAH multidisciplinary care model has yet to be described. OBJECTIVE: To evaluate the feasibility, acceptability, and patient and family experience of routinely incorporating CCLS services into the multidisciplinary DSD and CAH care model. STUDY DESIGN: As part of a quality improvement initiative, CCLS services were routinely incorporated in the multidisciplinary DSD and CAH clinics at our institution. Encounters for patients seen in clinic between July 2018 through October 2019 were reviewed for demographic information, DSD diagnosis classification, CCLS documentation, and whether an exam under anesthesia (EUA) was required due to an incomplete clinical exam. CCLS documentation was reviewed for assessments, interventions, whether patients tolerated their physical exams, time of CCLS services, and additional CCLS support beyond the physical exam. All patients were limited to one physical exam per clinic visit. RESULTS: Out of the 45 encounters with CCLS involvement, 42 (93.3%) exams were well-tolerated. CCLS assessments considered patient development, communication considerations, temperament, medical stressors, coping preferences, and patient preferences for activities and distractions. Interventions included preparing patients for their physical exams, encouragement before and during exams, addressing patient stressors, distractions and coping mechanisms, and advocating for the patient. No patients required an EUA. DISCUSSION: The CCLS aimed to provide families with a sense of control during clinic visits and teach them to advocate for themselves. The CCLS helped prepare and distract patients for their clinic visit and addressed the sensitive nature of the physical exam by focusing on the emotional and development needs of patients. CCLS contributions to a positive patient experience are consistent with multiple studies demonstrating the benefit of CCLS services for pediatric care. This quality improvement initiative ultimately helped to create a positive experience for patients and families. CONCLUSION: This study demonstrates the feasibility, acceptability, and positive impact of CCLS services in the delivery of patient and family-centered care for patients with DSD and CAH as part of the multidisciplinary team model.

Digital photography in the evaluation and management of female patients with congenital adrenal hyperplasia: A standardized protocol for quality improvement.

INTRODUCTION: Digital photography can be securely stored in the medical record and enhance documentation of physical exam findings and monitor wound healing. A standardized protocol that respects the dignity of the patient and maintains the fidelity of objective documentation is needed for patients with differences in sexual development (DSD) and congenital adrenal hyperplasia (CAH). OBJECTIVE: The purpose of this study was to evaluate the feasibility, acceptability, and applications of a HIPAA-compliant digital photography protocol in the care of female patients with CAH. STUDY DESIGN: A protocol for standardized digital imaging including consent, permission, data capture, and storage in the electronic medical record (EMR) was implemented. Patients undergoing physical examination during multidisciplinary CAH clinic visits, preoperative evaluation, and postoperative follow-up from October 2020 through May 2021 were included. Male patients with CAH, patients with clitoromegaly or urogenital sinus not from CAH, and patients seen through telehealth were excluded. Consent was obtained from caregivers and permission from patients. Images of the exam were taken during clinic visits or at the time of surgery with no identifying features included. Images were directly uploaded into the patient's chart in the HIPAA-protected EMR separate from other clinical documentation and not stored on personal devices. RESULTS: There were 17 patients with CAH seen with median age 6 years (range 2 weeks-18 years). There was a median of 3 photos per patient during the study period with cooperation from both the patient and their caregiver. Amongst the patients seen, 6 patients underwent reconstruction with a median of 10 photos per patient. Images were available and used for preoperative planning and counseling. Patients with previous images did not require repeat examinations and were subjected to fewer genital examinations. Fewer providers were present during exams. Images taken by providers and caregivers during the postoperative period were used to monitor wound healing and surgical outcomes. DISCUSSION: Protocol implementation improved patient care by reducing the number of exams and number of providers present, enhancing clinical documentation, and providing a means of tracking the physical exam over time. This was in concordance with guidelines for limiting exams for patients with DSD and CAH. Implementation of best practices for medical photography was important in respecting patient dignity and confidentiality. CONCLUSION: Implementation of standardized digital photography was feasible and acceptable to patients and caregivers. Digital images reduced the need for repeat physical examination and provided a visual means of enhancing clinical documentation.

Bedside Intervention for Neonatal Hydrometrocolpos and Imperforate Hymen.

Low complexity cases of neonatal hydrometrocolpos from imperforate hymen are typically treated with hymenotomy or hymenectomy. Although this is commonly performed in the operating room, bedside management is also safe and effective. We present a case of prenatally diagnosed, simple hydrometrocolpos treated via bedside hymenotomy on the first day of life. The patient received periprocedural antibiotics prior to the procedure and had full resolution of hydrometrocolpos without any complication or need for further intervention. Early intervention and administration of periprocedural antibiotics allowed for safe management of this condition without the risks and resources of treatment in the operating room.