Surgical indications for operative management of vesicoureteral reflux in children.

PURPOSE OF REVIEW: Vesicoureteral reflux (VUR) management has been steadily evolving over the last several years. There is not a definitive algorithm for operative intervention, but there are some recognized patterns to follow when caring for this patient base. It is extremely relevant to review the rationale behind practice patterns as both literature and clinical practice are dynamic. RECENT FINDINGS: VUR is a common malady that is emotionally, physically, and financially draining for families. As new treatment options emerge with minimally invasive techniques and older methods are re-explored, it is imperative to re-evaluate care strategies. This article reviews the mainstays of treatment in addition to newer therapeutic modalities. SUMMARY: The decision to operate on any patient, particularly pediatric patients, must be preceded by sound clinical judgment. Thoughtful planning must be utilized to ensure every patient receives individualized and up-to-date VUR management. This article reviews indications for surgical intervention to consider when managing these patients.

Early Vs. Expectant Management of Spina Bifida Patients-Are We All Talking About a Risk Stratified Approach?

PURPOSE OF REVIEW: Advancements in the care of patients affected by myelomeningocele have flourished in recent years especially with respect to renal preservation and continence. Involvement of urologists both prenatally and early in life has driven many developments in preventative care and early intervention. As of yet, however, the ideal management algorithm that offers these patients the least invasive diagnostic testing and interventions while still preserving renal and bladder function remains ill defined. RECENT FINDINGS: In a shift from prior years where the use of surgical intervention and intermittent catheterization were more liberally employed, some providers have more recently advocated for monitoring patients in a more conservative manner with a variety of diagnostic tests until radiographic or clinical changes are discovered. The criteria used to define the need for catheterization and the timing to initiate CIC or more invasive interventions is disparate across pediatric urology and there is published data to support several approaches. This review presents some of these criteria for use of CIC and some newer evidence to support different approaches along with supporting the trend toward individualized medicine and use of risk stratification in developing clinical treatment algorithms.

Transscrotal Near Infrared Spectroscopy as a Diagnostic Test for Testis Torsion in Pediatric Acute Scrotum: A Prospective Comparison to Gold Standard Diagnostic Test Study.

PURPOSE: A rapid test for testicular torsion in children may obviate the delay for testicular ultrasound. In this study we assessed testicular tissue percent oxygen saturation (%StO2) measured by transscrotal near infrared spectroscopy as a diagnostic test for pediatric testicular torsion. MATERIALS AND METHODS: This was a prospective comparison to a gold standard diagnostic test study that evaluated near infrared spectroscopy %StO2 readings to diagnose testicular torsion. The gold standard for torsion diagnosis was standard clinical care. From 2013 to 2015 males with acute scrotum for more than 1 month and who were less than 18 years old were recruited. Near infrared spectroscopy %StO2 readings were obtained for affected and unaffected testes. Near infrared spectroscopy Δ%StO2 was calculated as unaffected minus affected reading. The utility of near infrared spectroscopy Δ%StO2 to diagnose testis torsion was described with ROC curves. RESULTS: Of 154 eligible patients 121 had near infrared spectroscopy readings. Median near infrared spectroscopy Δ%StO2 in the 36 patients with torsion was 2.0 (IQR -4.2 to 9.8) vs -1.7 (IQR -8.7 to 2.0) in the 85 without torsion (p=0.004). AUC for near infrared spectroscopy as a diagnostic test was 0.66 (95% CI 0.55-0.78). Near infrared spectroscopy Δ%StO2 of 20 or greater had a positive predictive value of 100% and a sensitivity of 22.2%. Tanner stage 3-5 cases without scrotal edema or with pain for 12 hours or less had an AUC of 0.91 (95% CI 0.86-1.0) and 0.80 (95% CI 0.62-0.99), respectively. CONCLUSIONS: In all children near infrared spectroscopy readings had limited utility in diagnosing torsion. However, in Tanner 3-5 cases without scrotal edema or with pain 12 hours or less, near infrared spectroscopy discriminated well between torsion and nontorsion.

Bowel preparation prior to reconstructive urologic surgery in pediatric myelomeningocele patients.

INTRODUCTION: Mechanical bowel preparation (MBP) has historically been the standard of care for patients undergoing reconstructive urologic surgery, including urinary diversion. To date, several studies have examined the role of mechanical bowel preparation in postoperative outcomes in pediatric patients undergoing augmentation cystoplasty. However, these patient populations have been heterogeneous in nature, with no studies dedicated to examining the role of MBP prior to reconstructive urologic surgery in pediatric patients with myelomenginoceles. Thus, our objective was to retrospectively assess perioperative measures and postoperative complications after reconstructive urologic surgery with or without mechanical bowel preparation in pediatric myelomeningocele patients. MATERIALS AND METHODS: From 2008 to 2013, 80 patients with myelomeningocele underwent reconstructive urologic surgery involving the use of bowel. Seventy patients underwent a preoperative MBP while 10 did not. Perioperative measures and postoperative complications for these two cohorts were assessed. RESULTS: Eighty patients with myelomeningocele were identified; 70 patients underwent MBP while 10 patients did not. There were no statistically significant differences in demographics or operative time. There were no statistically significant differences in postoperative outcomes including time to first bowel movement and time to tolerating diet. There was also no significant difference in overall complication rate; patients with MBP had 31/70 (44%) complications while 2/10 (20%) of those without MBP had complications (p = 0.18). CONCLUSION: There was no significant difference in perioperative measures and postoperative complications for patients who did not receive a mechanical bowel preparation. Our findings indicate that it is safe and warranted to perform a prospective, randomized study to better characterize the risks and benefits of preoperative bowel preparation for patients with myelomeningocele.

Perioperative and Short-Term Outcomes of Robotic vs Open Bladder Neck Procedures for Neurogenic Incontinence.

PURPOSE: Complex urological reconstruction may be facilitated by the improved magnification and dexterity provided by a robotic approach. Minimally invasive surgery also has the potential advantages of decreased length of stay and improved convalescence. We reviewed perioperative and short-term outcomes between robot-assisted and open bladder neck sling/repair with catheterizable channel in patients with neurogenic bladder. MATERIALS AND METHODS: We performed an institutional review board approved retrospective chart review of all patients who underwent open or robotic bladder neck reconstruction without augmentation cystoplasty for refractory urinary incontinence between 2010 and 2014. Age at surgery, operative time, length of stay, complications within 30 days of surgery and future continence procedures (injection of bladder neck/catheterizable channel, additional bladder neck surgery, botulinum toxin A injection) were compared between the groups. RESULTS: A total of 45 patients underwent bladder neck reconstruction (open in 26, robotic in 19) with a mean follow up of 2.8 years. There was no difference in preoperative urodynamics, age at surgery or length of stay (median 4 days in each group, p >0.9). Operative time was significantly longer in the robotic group (8.2 vs 4.5 hours, p <0.001). Three patients (16%) undergoing robotic and 3 (12%) undergoing open surgery had a complication within 30 days (p >0.9). Of patients undergoing open repair 14 (56%) underwent 23 subsequent surgeries for incontinence. By comparison, 8 patients undergoing robotic repair (42%) underwent 12 additional procedures (p = 0.5). CONCLUSIONS: Although a robotic approach may take longer to perform, it does not result in increased complications or length of stay, or worsened continence outcomes.

Long-Term Outcomes of Bladder Neck Reconstruction without Augmentation Cystoplasty in Children.

PURPOSE: Bladder outlet procedures without augmentation cystoplasty remain controversial. We hypothesized that bladder outlet procedures without augmentation cystoplasty may lead to unfavorable bladder dynamics, upper tract changes and/or continued incontinence. We reviewed long-term urodynamic, upper tract and continence outcomes following bladder outlet procedures without augmentation cystoplasty. MATERIALS AND METHODS: We retrospectively reviewed all patients who underwent bladder neck reconstruction/closure/sling without augmentation cystoplasty between 2000 and 2014. Because of variation in length of followup, we calculated the cumulative incidence and proportion of cases of upper tract and urodynamic changes, augmentation cystoplasty and subsequent continence procedures. Preoperative factors were compared between patients with and without adverse outcomes. RESULTS: A total of 109 patients underwent bladder outlet procedures without augmentation cystoplasty at a mean age of 8.5 years. At a mean of 4.9 years of followup 59 patients (54%) had undergone additional continence surgery, 20 (18%) had undergone augmentation cystoplasty, 50 (46%) manifested vesicoureteral reflux or hydronephrosis and 23 (21%) had newly diagnosed or worsening renal scarring. At augmentation cystoplasty 13 of 18 patients (72%) had upper tract changes, 15 (83%) had continued incontinence and 11 (61%) had an end fill pressure of greater than 40 cm H2O. All patients had resolution of these changes after augmentation cystoplasty. Patients who had previously undergone vesicostomy or surgery for vesicoureteral reflux were significantly more likely to undergo a subsequent augmentation cystoplasty or to show upper tract changes. CONCLUSIONS: Following bladder outlet procedures without augmentation cystoplasty the estimated 10-year cumulative incidence of augmentation cystoplasty is 30%, continence procedures 70%, upper tract changes greater than 50% and chronic kidney disease 20%. Because of these risks, careful patient selection and close followup are essential if considering a bladder outlet procedure without augmentation cystoplasty.

Diagnosing Testicular Torsion before Urological Consultation and Imaging: Validation of the TWIST Score.

PURPOSE: The TWIST (Testicular Workup for Ischemia and Suspected Torsion) score uses urological history and physical examination to assess risk of testis torsion. Parameters include testis swelling (2 points), hard testis (2), absent cremasteric reflex (1), nausea/vomiting (1) and high riding testis (1). While TWIST has been validated when scored by urologists, its diagnostic accuracy among nonurological providers is unknown. We assessed the usefulness of the TWIST score when determined by nonurological nonphysician providers, mirroring emergency room evaluation of acute scrotal pain. MATERIALS AND METHODS: Children with unilateral acute scrotum were prospectively enrolled in a National Institutes of Health clinical trial. After undergoing basic history and physical examination training, emergency medical technicians calculated TWIST score and determined Tanner stage per pictorial diagram. Clinical torsion was confirmed by surgical exploration. All data were captured into REDCap™ and ROC curves were used to evaluate the diagnostic usefulness of TWIST. RESULTS: Of 128 patients (mean age 11.3 years) 44 (13.0 years) had torsion. TWIST score cutoff values of 0 and 6 derived from ROC analysis identified 31 high, 57 intermediate and 40 low risk cases (positive predictive value 93.5%, negative predictive value 100%). CONCLUSIONS: TWIST score assessed by nonurologists, such as emergency medical technicians, is accurate. Low risk patients do not require ultrasound to rule out torsion. High risk patients can proceed directly to surgery, with more than 50% avoiding ultrasound. In the future emergency medical technicians and/or emergency room triage personnel may be able to calculate TWIST score to guide radiological evaluation and immediate surgical intervention at initial assessment long before urological consultation.

Comparison of Complications of Robot-Assisted Laparoscopic and Open Appendicovesicostomy in Children.

PURPOSE: Robot-assisted laparoscopic appendicovesicostomy in children has become increasingly popular. However, the literature on this technique mainly consists of small case series with only 1 small comparison to an open cohort. We compared the number of complications and surgical revisions required with open and robotic surgery in children undergoing appendicovesicostomy at our institution. MATERIALS AND METHODS: We retrospectively reviewed the charts of all patients who underwent appendicovesicostomy by 3 surgeons between July 2002 and September 2013. Acute complications and surgical revisions were recorded and compared between groups with t-tests for continuous variables and Fisher exact test for categorical variables. RESULTS: A total of 28 open and 39 robotic appendicovesicostomies were included. At a mean followup of 2.7 years there was no difference in number of complications or reoperations (p = 0.788 and p = 0.791, respectively) between groups. Time to first reoperation was shorter in the robotic group. However, there was no significant difference between groups regarding number of patients who underwent reoperation within the first 12 months postoperatively (p = 0.346). CONCLUSIONS: Comparison of robotic and open appendicovesicostomy revealed no significant difference in the number of acute complications or reoperations between groups. However, the nature and timing of complications differed between groups.

Multi-institutional review of outcomes of robot-assisted laparoscopic extravesical ureteral reimplantation.

PURPOSE: We performed a multi-institutional assessment of the outcomes and complications of robot-assisted laparoscopic extravesical ureteral reimplantation for vesicoureteral reflux in children. MATERIALS AND METHODS: We retrospectively reviewed the records of patients who underwent robot-assisted laparoscopic extravesical ureteral reimplantation as done by 1 of 5 surgeons at Children's Medical Center, Dallas, Texas, or Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania, from 2010 to 2013. Procedure failure was defined as persistent vesicoureteral reflux on postoperative voiding cystourethrogram or radionuclide cystogram and/or the need for reoperation. Multivariate logistic regression was done to identify possible risk factors for failure using STATA®, version 11. RESULTS: A total of 61 patients (93 ureters) with a mean age of 6.7 years (range 0.6 to 18.0) underwent a procedure, of which 32 (52%) were bilateral. Ten patients (16%) underwent previous subureteral injection for vesicoureteral reflux. At a mean followup of 11.7 months the procedure was successful in 44 of 61 patients (72%). There were 14 cases of persistent vesicoureteral reflux (23%), 6 complications (10%) and 9 reoperations (11%). Multivariate logistic regression identified no factor that increased the risk of failure (p = 0.737). CONCLUSIONS: Compared to the literature we found a notably lower success rate for robot-assisted laparoscopic extravesical ureteral reimplantation in the hands of 5 fellowship trained, robotically experienced pediatric urologists. More than 10% of patients required at least 1 reoperation for persistent vesicoureteral reflux or a surgical complication. Our experience suggests a higher complication rate and a lower success rate for robot-assisted laparoscopic ureteral reimplantation compared to the gold standard of open reimplantation.

Painless pediatric priapism and cough.

Pediatric priapism can be a medical emergency but is not a common complaint seen in pediatric emergency department. Priapism in a previously healthy child is also rare. We report a case of painless stuttering priapism associated with an acute Mycoplasma pneumoniae infection in a previously healthy boy.

Demographics of pediatric renal trauma.

PURPOSE: There is a lack of national data describing the demographics and nature of pediatric renal trauma. We used the National Trauma Data Bank to analyze mechanisms and grades of injury, demographics and treatment characteristics of pediatric renal trauma cases. MATERIALS AND METHODS: Renal injuries were identified by Abbreviated Injury Scale codes and converted to American Association for the Surgery of Trauma renal injury grades. Patients were stratified by age (0 to 1, 2 to 4, 5 to 14 and 15 to 18 years) for more specific analyses of mechanisms and grades of injury. Data reviewed included mechanisms and grades of renal injury, demographics, and setting and type of treatment. RESULTS: A total of 2,213 pediatric renal injuries were converted to American Association for the Surgery of Trauma grade. Mean ± SD age at injury was 13.7 ± 4.4 years, with 2,089 patients (94%) being 5 to 18 years old. Of the injuries 79% were grade I, II or III. Penetrating injury accounted for less than 10% of all pediatric renal injuries. A majority of patients (57%) were admitted to university hospitals with a dedicated trauma service (73%) and only 12% of patients were admitted to a pediatric hospital. A total of 122 nephrectomies (5.5%) were performed. CONCLUSIONS: Most renal trauma in children is low grade, is blunt in nature and occurs after age 5 years. The majority of these cases are managed at adult hospitals. Although most patients are treated conservatively, the rate of nephrectomy is 3 times higher at adult hospitals than at pediatric centers.

Emergent robot-to-open conversion - Multidisciplinary simulation training in crisis management.

Anticipating and addressing unexpected intraoperative events and anatomies are some of the most challenging aspects of pediatric urologic practice; uncontrolled hemorrhage is one of the most anxiety provoking and precarious. The increasing application of the robotic platform in pediatric urology adds another layer of complexity as surgeons are not immediately at the patient's bedside. Should hemorrhage occur in robotic cases, clear communication and seamless coordination between members of the operating room team are paramount to optimize patient safety and minimize errors. This is especially important in pediatric cases for which the margin of error is narrow. Non-technical skills, including leadership, decision-making, situational awareness, stress management, and team-communication, become increasingly critical. While many programs have focused on robotic training, few prepare the operating room team and surgical trainees to manage these unforeseen, emergent intraoperative scenarios. This review discusses the role of a multidisciplinary, in situ robot-to-open conversion simulation program in addressing this educational gap, ways to approach establishing these programs, and potential barriers.

Renal Medullary Carcinoma: The Zebra Amongst the Horses.

A 13-year old Latino male presented with recurrent gross hematuria, 5cm right-sided poorly defined heterogeneous mass, enlarged retrocaval lymph nodes, and 1.2 cm paratracheal lymph node. Given the need for multiple blood transfusions, robot-assisted radical nephrectomy with lymph node dissection was performed. Pathology revealed pT3a high-grade tumor, clear margins, and positive lymph node. Additionally, with multiple sickled RBCs and loss of staining of SMARCB1 in tumor specimen, and hemoglobin electrophoresis suggesting sickle cell trait, diagnosis of metastatic renal medullary carcinoma was confirmed. The patient was enrolled into COG AREN 03B2 trial, and has completed 10 cycles of carboplatin/gemcitabine/bortezomib alternating with cisplatin/gemcitabine/paclitaxel, with no evidence of recurrent disease 9 months post-surgery.

Reinnervating the penis in spina bifida patients in the United States: ilioinguinal-to-dorsal-penile neurorrhaphy in two cases.

INTRODUCTION: Penile sensation is absent in some patients with myelomeningocele owing to the dysfunction of the pudendal nerve. Here, we describe the introduction of penile sensation via ilioinguinal-to-dorsal-penile neurorrhaphy in two patients with penile anesthesia due to neural tube defects. AIM: To establish penile sensation via ilioinguinal-to-dorsal-penile-nerve neurorrhaphy. METHODS: A 20-year-old and a 35-year-old male with L5/S1 myelomeningocele were both highly functioning and ambulatory, with intact ilioinguinal nerve distribution sensation but anesthesia of the penis and glans. They were sexually active and able to ejaculate antegrade. Both had high International Index of Erectile Function scores for confidence to achieve erection sufficient for intercourse. An incision was made from anterior superior iliac crest to the glans penis to expose the inguinal canal and ilioinguinal nerve. The ilioinguinal and dorsal penile nerve were transected and anastomosed. The anastomotic site was then wrapped in a hemostatic agent and a drain was left in place. For penile rehabilitation, both patients were instructed to stimulate the penis while looking at the genitalia to encourage redistribution of perceived sensation. MAIN OUTCOME MEASURES: Presence of erogenous penile sensation was tested by neurologic examination and patient feedback, and patients completed sexual health questionnaires. RESULTS: Both patients reported paresthesias of the groin with penile stimulation 1 month after surgery. Both patients are now 24 months postoperative and have erogenous sensation on the ipsilateral glans and shaft during intercourse. Neither patient has difficulty achieving or maintaining erections. CONCLUSIONS: We present two patients with dorsal penile reinnervation via the ilioinguinal nerve. Although nerve reinnervation has been used in urological procedures, this is the first description of an attempt to resupply penile sensation via the dorsal penile nerve in the United States with a minimum of 18 months follow-up. Early follow-up suggests successful neuronal remapping and regained sensation of the penis.

Testicular relapse of acute lymphoblastic leukemia.

Contemporary chemotherapy regimens have led to improved survival and decreased incidence of testicular relapse for pediatric patients with acute lymphoblastic leukemia (ALL). Local therapies to the testes, such as radiotherapy and orchiectomy, are often not necessary given that high-dose chemotherapy agents can overcome the relative blood-testis barrier. However, urologists should be aware of clinical scenarios involving ALL which still warrant testicular biopsy to guide management. Here, we present a case of a 12-year-old boy with high-risk pre-B cell ALL presenting with a testicular relapse and a clinical presentation overlapping with non-infectious epididymo-orchitis.

Karyotype Anomalies in Patients with Disorders of Sexual Development.

OBJECTIVES: Objectives Disorders of sex development(DSD)can result in discordance between the chromosomal and anatomicand/orphenotypic sex of a patient. Reporting patients with uncommon karyotypes associated with DSD is important for clinical comparison of developmental outcomes, and management. Methods We describe three female patients with karyotypes resulting in DSD and the use of a combination of chromosomes and FISH techniques to identify potential causes. Results The first patient was mosaic for idic(Y) that was negative for SRY by FISH. The second patient had idic(Y) that was positive for SRY by FISH. The third patient had an unbalanced translocation between the X chromosome and chromosome 2 [der(2)(X;2)] and XY. These three patients illustrate three different genetic mechanisms underlying DSD. Conclusion Our findings expand the list of abnormal karyotypes that can be associated with DSD and highlight the importance of SRY and DAX1 in phenotypic and functional sexual development.

Delayed Surgical Management of an Unusual Classic Bladder Exstrophy Variant.

Anterior abdominal wall defects are rare anomalies that can affect multiple organ systems including gastrointestinal, genitourinary, musculoskeletal, and the neurospinal axis. The highly varied, complex anatomy in this patient population creates a challenging reconstruction scenario that merits careful surgical planning. We present an unusual female variant with an anorectal malformation as well as musculoskeletal and genital abnormalities consistent with classic bladder exstrophy in which the urinary bladder, sphincter, and urethra were largely uninvolved.

Ureteral stent extraction strings in children: Stratifying the risk of post operative urinary tract infection.

INTRODUCTION: Ureteral stents facilitate recovery and avoid external drains in pediatric ureteral reconstruction. Extraction strings avoid the need for a secondary cystoscopy and anesthetic. Due to concerns regarding febrile UTIs in children with extraction strings, we retrospectively assessed the relative risk of UTI in children with extraction strings. OBJECTIVE: Our hypothesis was that stents with extraction strings do not increase the risk of UTI after pediatric ureteral reconstruction. METHODS: Records of all children undergoing pyeloplasty and ureteroureterostomy (UU) from 2014 to 2021 were reviewed. The incidences of UTI, fever, and hospitalization were recorded. RESULTS: 245 patients mean age 6.4 years (163M:82F) underwent pyeloplasty (n = 221) or UU (n = 24). 42% (n = 103) received prophylaxis. Of these, 15% developed UTI versus 5% of those not receiving prophylaxis (p < 0.05). 42 females had prior history of UTI, compared to 20 males (p < 0.05). 49 patients had an extraction string. Stents with extraction strings were removed on average 0.6 months post-op while others underwent cystoscopic removal on average 1.26 months post-op (p < 0.05). 9 (18.4%) required hospitalization for febrile UTI while the stent with extraction string was in place, while only 13 (6.6%) of those without extraction string did (p < 0.02). Of the 9 children with a febrile UTI in the extraction string group, 6 had history of prior UTI (46.1%), compared to only 3 (8.3%) without a prior UTI (p < 0.05). With no prior UTI, there was no difference in UTI risk between those with (3, 8.3%) and without (8, 6.4%) extraction string (p = 0.71). Females with prior UTI and extraction string were more likely to develop UTI than those with prior UTI and no extraction string (p = 0.01). There were not enough males with history of UTI to analyze alone. There were 5 (10%) stent dislodgements in the extraction string group, 2 required further intervention with cystoscopy or percutaneous drainage. DISCUSSION: Extraction strings provide the assurance of drainage while avoiding the need for a second general anesthetic procedure. There is not an increased risk of UTI with extraction string in those without prior history of UTI, but we no longer routinely leave extraction strings if there is history of UTI. CONCLUSION: Children, particularly females, with prior history of UTI have a significantly increased risk of febrile UTIs associated with the use of extraction strings. Prophylaxis does not seem to reduce this risk. Patients with no prior UTI had no higher risk of UTI with extraction string use for pyeloplasty or UU.

Clinical utility and interrater reliability of video urodynamics in children with isolated fibrolipoma of filum terminale.

INTRODUCTION: Children with an isolated fibrolipoma of filum terminale (IFFT) but otherwise normal spinal cord are often evaluated with video urodynamics (VUDS). VUDS interpretation is subjective and can be difficult in young children. These patients may undergo detethering surgery if there is concern for current or future symptomatic tethered cord. OBJECTIVE: We hypothesized that VUDS in children with IFFT would have limited clinical utility regarding decision for or against detethering surgery and VUDS interpretation would have poor interrater reliability. METHODS: Patients with IFFT who underwent VUDS for from 2009 to 2021 were retrospectively reviewed to evaluate clinical utility of VUDS. 6 pediatric urologists who were blinded to patient clinical characteristics reviewed the VUDS. Gwet's first order agreement coefficient (AC1) with 95% CI was used to assess interrater reliability. RESULTS: 47 patients (24F:23M) were identified. Median age at initial evaluation was 2.8yrs (IQR:1.5-6.8). 24 (51%) patients underwent detethering surgery (Table). VUDS at initial evaluation were interpreted by treating urologist as normal in 4 (8%), reassuring for normal in 39 (81%), or concerning for abnormal in 4 (9%). Based on neurosurgery clinic and operative notes for the 47 patients, VUDS made no change in management in 37 patients (79%), prompted detethering in 3 (6%), was given as reason for observation in 7 (15%), and was normal or reassuring for normal but not documented as a reason for observation in 16 (34%) (Table). Interrater reliability for VUDS interpretation had fair agreement (AC1 = 0.27) for overall categorization of VUDS and EMG interpretation (AC1 = 0.34). Moderate agreement was seen for detrusor overactivity interpretation (AC1 = 0.54) and bladder neck appearance (AC1 = 0.46). DISCUSSION: In our cohort, 90% of patients had a normal or reassuring for normal interpretation of VUDS. VUDS interpretation affected clinical course in a minority of patients. There was fair interrater reliability for overall VUDS interpretation and therefore clinical course regarding detethering surgery could vary depending upon interpreting urologist. This fair interrater variability appeared to be related to variability in EMG, bladder neck appearance, and detrusor overactivity interpretation. CONCLUSION: VUDS affected clinical management in about 20% of our cohort and supported the choice for observation in around 50% of patients. This suggests VUDS does have clinical utility in pediatric patients with IFFT. The overall VUDS interpretation had fair interrater reliability. This suggest VUDS interpretation has limitations in determining normal versus abnormal bladder function in children with IFFT. Neurosurgeons and urologists should be aware of VUDS limitations in this patient population.

Causes of death among people with myelomeningocele: A multi-institutional 47-year retrospective study.

PURPOSE: This study aimed to analyze organ system-based causes and non-organ system-based mechanisms of death (COD, MOD) in people with myelomeningocele (MMC), comparing urological to other COD. METHODS: A retrospective review was performed of 16 institutions in Canada/United States of non-random convenience sample of people with MMC (born > = 1972) using non-parametric statistics. RESULTS: Of 293 deaths (89% shunted hydrocephalus), 12% occurred in infancy, 35% in childhood, and 53% in adulthood (documented COD: 74%). For 261 shunted individuals, leading COD were neurological (21%) and pulmonary (17%), and leading MOD were infections (34%, including shunt infections: 4%) and non-infectious shunt malfunctions (14%). For 32 unshunted individuals, leading COD were pulmonary (34%) and cardiovascular (13%), and leading MOD were infections (38%) and non-infectious pulmonary (16%). COD and MOD varied by shunt status and age (p < = 0.04), not ambulation or birthyear (p > = 0.16). Urology-related deaths (urosepsis, renal failure, hematuria, bladder perforation/cancer: 10%) were more likely in females (p = 0.01), independent of age, shunt, or ambulatory status (p > = 0.40). COD/MOD were independent of bladder augmentation (p = >0.11). Unexplained deaths while asleep (4%) were independent of age, shunt status, and epilepsy (p >= 0.47). CONCLUSION: COD varied by shunt status. Leading MOD were infectious. Urology-related deaths (10%) were independent of shunt status; 26% of COD were unknown. Life-long multidisciplinary care and accurate mortality documentation are needed.