Cecostomy tubes improve bowel continence for pediatric patients with spina bifida: A retrospective analysis of outcomes from a single clinic.

PURPOSE: Pediatric patients with spina bifida often experience neurogenic bowel dysfunction. Although cecostomy tubes could improve bowel continence, their effectiveness is not well established in this population. The aims of this study were to better understand the effectiveness of cecostomy tubes relative to other management strategies (between-subject) and to explore their effectiveness among patients who received these placements (within-subject). METHODS: Retrospective analysis of data from pediatric patients enrolled in a national spina bifida patient registry (n = 297) at a single multidisciplinary clinic was performed, covering visits between January 2014 -December 2021. Linear and ordinal mixed effect models (fixed and random effects) tested the influence of cecostomy status (no placement vs placement) and time (visits) on bowel continence while controlling for demographic and condition-specific covariates. RESULTS: Patients with cecostomy tubes had higher bowel continence compared to patients without placements (B = 0.695, 95% CI [0.333, 1.050]; AOR = 2.043, p = .007). Patients with cecostomy tubes had higher bowel continence after their placements compared to before (B = 0.834, 95% CI [0.142, 1.540]; AOR = 3.259, p = 0.011). CONCLUSION: Results indicate cecostomy tubes are effective for improving bowel continence in this pediatric population. Future research is needed to conduct risk analyses and determine the clinical significance of these effects.

Identifying variability in surgical practices and instrumentation for hypospadias repair across the Western Pediatric Urology Consortium (WPUC) network.

BACKGROUND: Although hypospadias outcomes studies typically report a level or type of repair performed, these studies often lack applicability to each surgical practice due to technical variability that is not fully delineated. An example is the tubularized incised plate (TIP) urethroplasty procedure, for which modifications have been associated with significantly decreased complication rates in single center series. However, many studies fail to report specificity in techniques utilized, thereby limiting comparison between series. OBJECTIVE: With the goal of developing a surgical atlas of hypospadias repair techniques, this study examined 1) current techniques used by surgeons in our network for recording operative notes and 2) operative technical details by surgeon for two common procedures, tubularized incised plate (TIP) distal and proximal hypospadias repairs across a multi-institutional surgical network. STUDY DESIGN: A two-part study was completed. First, a survey was distributed to the network to assess surgeon volume and methods of recording hypospadias repair operative notes. Subsequently, an operative template or a representative de-identified operative note describing a TIP and/or proximal repair with urethroplasty was obtained from participating surgeons. Each was analyzed by at least two individuals for natural language that signified specified portions of the procedure. Procedural details from each note were tabulated and confirmed with each surgeon, clarifying that the recorded findings reflected their current practice techniques and instrumentation. RESULTS: Twenty-five surgeons from 12 institutions completed the survey. The number of primary distal hypospadias repairs performed per surgeon in the past year ranged from 1-10 to >50, with 40% performing 1-20. Primary proximal hypospadias repairs performed in the past year ranged from 1-30, with 60% performing 1-10. 96% of surgeons maintain operative notes within an electronic health record. Of these, 66.7% edited a template as their primary method of note entry; 76.5% of these surgeons reported that the template captures their operative techniques very or moderately well. Operative notes or templates from 16 surgeons at 10 institutions were analyzed. In 7 proximal and 14 distal repairs, parameters for chordee correction, urethroplasty suture selection and technique, tissue utilized, and catheter selection varied widely across surgeons. CONCLUSION: Wide variability in technical surgical details of categorically similar hypospadias repairs was demonstrated across a large surgical network. Surgeon-specific modifications of commonly described procedures are common, and further evaluation of short- and long-term outcomes accounting for these technical variations is needed to determine their relative influence.

Pediatric cystectomy for refractory cystitis post-bone marrow transplant in dyskeratosis congenita: A case report.

We present a case of refractory cystitis after BK-virus associated hemorrhagic cystitis following bone marrow transplantation requiring cystectomy in a 17-year-old female with genetically confirmed dyskeratosis congenita, a telomere disorder characterized by early bone marrow failure. She presented with a contracted, small bladder with intense urinary symptoms non-responsive to conservative therapy and requiring opioids for pain control. Cystectomy is a rare, final surgical treatment for benign bladder conditions, especially among younger patients, and she experienced successful resolution of symptoms and cessation of chronic opioids post-intervention.

Robotic assisted retrovesical approach to prostatic utricle excision and other complex pelvic pathology in children is safe and feasible.

INTRODUCTION: Excision of the prostatic utricle has been a challenging surgical problem due to the location deep in the pelvis between the rectum and bladder. Robotic-assisted laparoscopic surgery allows minimally invasive access to this location. OBJECTIVE: To describe the robotic surgical outcomes and important techniques associated with robotic excision of the prostatic utricle and explain how these techniques apply to similar pediatric pelvic pathology. STUDY DESIGN: Retrospective chart evaluation of patients treated at a single institution with robotic excision of a prostatic utricle as well as two other patients in which the similar approach was employed. RESULTS: 4 patients underwent robotic excision of a prostatic utricle. A fifth patient underwent excision of urethral diverticulum that was the remnant of an ectopic ureter. The sixth patient had a high urogenital sinus that required laparoscopic assisted vaginoplasty approached in a similar manner to the above listed cases. Mean age at surgery was 35.5 months for the utricle patients. Mean follow-up duration for the utricle patients was 30.75 months. Average hospital stay for the utricle patients was one day. In the utricle patients one patient developed transient urinary retention and one had a postoperative urinary tract infection. Concomitant cystoscopy used in the two non-utricle patients was a key step in defining the proper location of dissection. DISCUSSION: Robotic-assisted laparoscopic retrovesical excision of the prostatic utricle is a safe operation that requires a skill set that can be readily applied to other complex pelvic pathology such as the ectopic ureter, urethral diverticulum, and urogenital sinus. Concomitant cystoscopy can aid significantly in defining the location and limits of dissection.

Translating pediatric urodynamics from clinic into collaborative research: Lessons and recommendations from the UMPIRE study group.

Pediatric patients present unique challenges in the performance and interpretation of urodynamic studies. Interpretation of urodynamics to guide clinical management at an institutional level is accepted as reliable. Challenges arise however when multi-site collaborations incorporate urodynamics into study design to determine primary or secondary outcomes or to direct decision-making. Although standardized terminology has been established by ICCS, the application of this shared language to performance and interpretation of pediatric urodynamics to across multiple sites may not be intuitive or reliable. With a primary goal of defining the care necessary to protect future renal function, the UMPIRE protocol (Urologic Management to Preserve Initial REnal function) utilizes a urodynamics-based risk stratification to determine medical management for infants with myelomeningocele. Iterative changes in the protocol are based upon the clinical progress of the enrolled children. Despite a team experienced in subtleties of urodynamics and despite efforts to minimize variability across sites, the UMPIRE study group identified several areas in which the language of urodynamics required additional clarification or creation of more explicit definitions to standardize performance and interpretation across sites. This article reviews the foundations of current urodynamics practice, describes limitations and challenges unique to pediatric studies, and the shares the humble lessons learned by the UMPIRE study group on their journey toward standardized urodynamic language for management of infants and children with myelomeningocele.

Urodynamic characteristics of neurogenic bladder in newborns with myelomeningocele and refinement of the definition of bladder hostility: Findings from the UMPIRE multi-center study.

INTRODUCTION: Infants with myelomeningocele are at risk for chronic kidney disease caused by neurogenic bladder dysfunction. Urodynamic evaluation plays a key role to risk stratify individuals for renal deterioration. OBJECTIVE: To present baseline urodynamic findings from the Urologic Management to Preserve Initial Renal function for young children with spina bifida (UMPIRE) protocol, to present the process that showed inadequacies of our original classification scheme, and to propose a refined definition of bladder hostility and categorization. STUDY DESIGN: The UMPIRE protocol follows a cohort of newborns with myelomeningocele at nine children's hospitals in the United States. Infants are started on clean intermittent catheterization shortly after birth. If residual volumes are low and there is no or mild hydronephrosis, catheterization is discontinued. Baseline urodynamics are obtained at or before 3 months of age to determine further management. Based on protocol-specific definitions, urodynamic studies were reviewed by the clinical site in addition to a central review team; and if necessary, by all site urologists to achieve 100% concurrence. RESULTS: We reviewed 157 newborn urodynamic studies performed between May 2015 and September 2017. Of these 157 infants, 54.8% were boys (86/157). Myelomeningocele closure was performed in-utero in 18.4% (29/157) and postnatally in 81.5% (128/157) of newborns. After primary review, reviewers agreed on overall bladder categorization in 50% (79/157) of studies. Concurrence ultimately reached 100% with further standardization of interpretation. We found that it was not possible to reliably differentiate a bladder contraction due to detrusor overactivity from a volitional voiding contraction in an infant. We revised our categorization system to group the "normal" and "safe" categories together as "low risk". Additionally, diagnosis of detrusor sphincter dyssynergia (DSD) with surface patch electrodes could not be supported by other elements of the urodynamics study. We excluded DSD from our revised high risk category. The final categorizations were high risk in 15% (23/157); intermediate risk in 61% (96/157); and low risk in 24% (38/157). CONCLUSION: We found pitfalls with our original categorization for bladder hostility. Notably, DSD could not be reliably measured with surface patch of electrodes. The effect of this change on future renal outcomes remains to be defined.

Relative value units do not adequately account for operative time in pediatric urology.

BACKGROUND: Relative value units (RVUs) are the measure of value used in United States Medicare and Medicaid reimbursement. The Relative Update Committee (RUC) determines physician work RVU (wRVUs) based on operative time, technical skill and effort, mental effort and judgment, and stress. The primary aim of this study was to assess whether operative time is adequately accounted for in the wRVU system in pediatric urology. METHODS: The American College of Surgeons National Surgical Quality Improvement Program Pediatric Participant User File (ACS-NSQIPP-PUF) was reviewed from 2012 to 2017. Most common single pediatric urology current procedural terminology (CPT) codes were included. The primary variable was wRVU per hour of operative time (wRVU/h). Linear regression analysis was used to assess the relative influence that operative time had on wRVU/h. RESULTS: 25,432 cases were included in the final study population from 45 unique CPT codes. The median operative time was 79 min, and the median RVU/h was 12.2. Procedures with operative time less than 79 min had higher wRVU/h compared with procedures longer than 79 min (14.5 vs 10.5, p < 0.001). Procedures with higher than average incidence of any complications had a lower wRVU/h (9.0 vs. 14.6 p < 0.001). Linear regression analysis revealed that each additional hour of operative time was expected to decrease wRVU/h by 4.2 (-0.70 per 10 min, 95% CI: -0.71 to -0.69, p < 0.001; R2 = 0.39). CONCLUSION: This analysis of contemporary large pediatric population national-level data suggests that the wRVU system significantly favors shorter and less complex procedures in Pediatric Urology. Pediatric urologists performing longer and more complex procedures are not adequately compensated for the increase in complexity. EVIDENCE LEVEL III: Retrospective comparative study.

Work relative value units do not account for complexity and operative time in hypospadias surgery.

INTRODUCTION: Relative value units (RVU) are the measure of value used in United States Medicare and Medicaid reimbursement. The Relative Update Committee (RUC) determine physician work RVU (wRVU) based on operative time, technical skill and effort, mental effort and judgement, and stress. In theory, wRVU should account for the complexity and operative time involved in a procedure. OBJECTIVE: The primary aim of this study is to assess if operative time and complexity of hypospadias surgery is adequately accounted for by the current wRVU assignments. STUDY DESIGN: The American College of Surgeons National Surgical Quality Improvement Program Participant User File (ACS-NSQIP PUF) database was utilized from 2012 to 2017. Single stage hypospadias current procedural terminology (CPT) codes (including acceptable secondary CPT codes) were extracted. Using total wRVU and total operative time, the primary variable of wRVU per hour was calculated (wRVU/hr). Multivariable linear regression analysis was used to assess the relative influence that wRVU and operative time had on the wRVU/hr variable. RESULTS: 9810 cases were included in the final study population divided into four categories: simple distal (eg. MAGPI, V-Flap), single stage distal, single stage mid, single stage proximal. On analysis of variance, there was statistically significant different wRVU/hr for the four different types of hypospadias repairs with simple distal having the highest mean wRVU/hr of 19.5 and the lowest being proximal hypospadias repairs at 13.2. Simple distal, distal and midshaft hypospadias had statistically significantly higher wRVU/hr compared to proximal hypospadias (16.2, 95% CI: 15.8-16.5 vs. 13.2, 95% CI 10.9-15.5; p<0.001). Multivariable linear regression revealed that each additional hour of operative time was expected to decrease wRVU/hr by 10.5 (-10.5, 95% CI: -11.0 to -10.1, p < 0.001); total work wRVU had a statistically significant independent association with wRVU/hr (0.6, 95%CI: 0.5-0.7, p <0.001). DISCUSSION: This the first objective assessment of the current wRVU assignments with regards to one stage hypospadias repairs. More complex and longer hypospadias procedures are not adequately compensated by wRVU. Most notably, simple distal procedures are reimbursed at a mean of 19.5 wRVU/hr compared to 13.2 wRVU/hr for one stage proximal repairs. CONCLUSION: This analysis of national-level data suggests that the current wRVU assignments significantly favor shorter and simpler procedures in hypospadias surgery.

Perioperative complications within 30 days of hypospadias surgery: Results from NSQIP-Pediatrics.

INTRODUCTION: There are no large multi-institutional studies reporting on perioperative complications of hypospadias repairs. We sought to determine perioperative complications of hypospadias repairs from the National Surgical Quality Improvement Program Pediatrics (NSQIP-P) to aid in patient counseling. STUDY DESIGN: This cohort study from 2012 to 2017 was conducted using NSQIP-P database. Pediatric patients undergoing hypospadias surgery were identified and compared based on 4 major categories: distal/midshaft repair, one-stage repair proximal, stage one repair, and stage two repair. Baseline demographics between the four groups and perioperative parameters were compared. Multivariable logistic regression analysis models including type of repair was used to determine associations with overall complications, infectious complications, and dehiscence. DISCUSSION: There were 11,292 patients identified in the study population. Overall, 78% underwent distal/midshaft hypospadias repair, 12% underwent one-stage proximal repair, 1.4% underwent proximal first stage repair and 9% underwent proximal second stage repair. Multivariable logistic regression analysis revealed that proximal first stage procedures had similar overall complications to distal/mid repairs but proximal one-stage and proximal second stage procedures were associated with significantly more overall complications, local infectious complications, and dehiscence. Age, race, operative time, prematurity were also independently associated with increased overall complications. As expected, complication rates are higher in those with proximal hypospadias. In staged hypospadias, first stage has a lower complication rate compared to second stage. All complications, especially of infectious and dehiscence are the highest in the one-stage proximal and proximal second stage repairs. CONCLUSION: We report large multi-institutional analysis of 30-day peri-operative hypospadias repair complications; this information is useful for patient counseling and education.

Congenital virilization of female infants recognized after pregnancies with retained levonorgestrel intrauterine devices.

The Mirena intrauterine device (IUD) is a hormone-secreting contraceptive device. Pregnancy with the Mirena is rare and effects to the fetus are unknown. Here we present four females with genital virilization after pregnancy with persistent Mirena IUD. All patients had a 46, XX karyotype and normal hormone evaluation. All underwent exam under anesthesia, demonstrating posterior labial fusion and short urogenital sinus with normal bladder, urethra, vagina, and cervix. Three of four patients underwent flap vaginoplasty without complications and good cosmetic outcomes. This series suggests that persistent levonorgestrel-secreting IUD during pregnancy is associated with genital virilization in female fetuses.

Invasive High-grade Upper Tract Urothelial Carcinoma in a 14-Year-Old Girl.

We present an unusual pediatric case of invasive upper tract urothelial carcinoma with an associated genetic predisposition. A 14-year-old female presented with intermittent right flank pain, and was found to have a poorly functioning hydronephrotic right kidney. Laparoscopic nephrectomy was performed. Pathology demonstrated upper tract urothelial carcinoma, and she subsequently underwent completion ureterectomy. Genetic studies demonstrated a double-hit constitutional deletion of a DNA mismatch repair protein, revealing a rare Lynch syndrome variant known as Constitutional Mismatch Repair Deficiency Syndrome. This disease places her at high risk for multiple malignancies, including upper tract urothelial carcinoma.

Design and Methodological Considerations of the Centers for Disease Control and Prevention Urologic and Renal Protocol for the Newborn and Young Child with Spina Bifida.

PURPOSE: Care of children with spina bifida has significantly advanced in the last half century, resulting in gains in longevity and quality of life for affected children and caregivers. Bladder dysfunction is the norm in patients with spina bifida and may result in infection, renal scarring and chronic kidney disease. However, the optimal urological management for spina bifida related bladder dysfunction is unknown. MATERIALS AND METHODS: In 2012 the Centers for Disease Control and Prevention convened a working group composed of pediatric urologists, nephrologists, epidemiologists, methodologists, community advocates and Centers for Disease Control and Prevention personnel to develop a protocol to optimize urological care of children with spina bifida from the newborn period through age 5 years. RESULTS: An iterative quality improvement protocol was selected. In this model participating institutions agree to prospectively treat all newborns with spina bifida using a single consensus based protocol. During the 5-year study period outcomes will be routinely assessed and the protocol adjusted as needed to optimize patient and process outcomes. Primary study outcomes include urinary tract infections, renal scarring, renal function and bladder characteristics. The protocol specifies the timing and use of testing (eg ultrasonography, urodynamics) and interventions (eg intermittent catheterization, prophylactic antibiotics, antimuscarinic medications). Starting in 2014 the Centers for Disease Control and Prevention began funding 9 study sites to implement and evaluate the protocol. CONCLUSIONS: The Centers for Disease Control and Prevention Urologic and Renal Protocol for the Newborn and Young Child with Spina Bifida began accruing patients in 2015. Assessment in the first 5 years will focus on urinary tract infections, renal function, renal scarring and clinical process improvements.

Distal ureteral diameter measurement objectively predicts vesicoureteral reflux outcome.

OBJECTIVE: Vesicoureteral reflux (VUR) grading may be difficult when discrepancies exist between the degree of dilation of the pyelocalyceal system and the ureter. Resolution may be more accurately predicted by the appearance of the distal ureter. We analyzed a novel, objective method of evaluating VUR based on the diameter of the distal ureter. METHODS: Seventy-nine voiding cystourethrograms were reviewed (18 boys; 61 girls; aged 1 month to 7.5 years). The largest ureteral diameter within the false pelvis was measured and normalized by dividing by the distance from the L1-L3 vertebral body to give the distal ureteral diameter: L1-L3 ratio (UDR). Clinical outcome was defined as spontaneous resolution or surgical correction. RESULTS: A significant association between grade and UDR existed (p < 0.0001). Mean UDR was significantly greater in those who underwent surgical correction (0.34 ± 0.02 vs 0.18 ± 0.02; p < 0.0001). Logistic regression analysis demonstrated a significant association of UDR with outcome controlling for grade (p = 0.001). Grade effect on outcome when controlling for UDR was not significant (p = 0.76). Odds ratio for surgical correction corresponding to a 0.1 increase in UDR equaled 2.25 (95% CI: 1.39, 3.64). CONCLUSION: UDR provides an objective measurement of VUR and appears more predictive of clinical outcome than grade in this series.

Description of a novel murine model for ileocystoplasty and early histologic changes.

There is concern that bladder augmentation with bowel segments predisposes toward the development of carcinoma. Additionally, patients with neurogenic bladder and bladder cancer often present with advanced stage and have poor survival. Cellular hyperproliferation at the urointestinal junction (UIJ) has been implicated in this scenario. We aimed to develop a reproducible murine model of ileocystoplasty (ICP). We also performed preliminary analysis of any early histologic changes with focus on cellular proliferation at the UIJ. Fifteen 6- to 8-week-old female C57BL/6 mice underwent ICP, where a 1-cm ileal segment was used for bladder augmentation. Four sham mice underwent cystotomy and closure, and four mice did not undergo surgery. The mice were euthanized at 12 weeks postsurgery, and paraffin sections were stained for hematoxylin and eosin (H&E). Cellular proliferation was investigated using Ki-67. A novel model of ICP in mice was developed and demonstrated to be technically feasible in approximately 60 min under the operating microscope. Twelve-week postsurgical survival rates were 80% (12 of 15). The surviving mice had a similar weight gain as the sham mice. H&E sections showed thickened urothelium (six to 10 cell layers) at the UIJ, but sparse mitotic figures and no dysplastic changes. Ki-67 staining was rare in the urothelium, and showed no differences between the sham and ICP mice in the bladder or at the UIJ. We here demonstrate the first murine model of ICP. Preliminary studies did not show evidence of early hyperproliferation at the UIJ or in the bladder, but further long-term studies as well as studies with transgenic mice are warranted.

The prognostic impact of an abnormal initial renal ultrasound on early reflux resolution.

OBJECTIVE: In a group of children diagnosed with vesicoureteral reflux (VUR) we evaluated renal ultrasound findings, associated findings on renal scan, and prognostic impact on VUR resolution. METHODS: Medical records were reviewed for children with primary reflux and no history of antenatal hydronephrosis who underwent an initial renal ultrasound. Abnormal renal ultrasound was defined as hydronephrosis or relative difference in renal size ≥1 cm. Reflux resolution was evaluated at 2 years post diagnosis. RESULTS: In 129 children with VUR (111 girls, 18 boys), 39 (30%) had an abnormal renal ultrasound. Two-year VUR resolution in the abnormal renal ultrasound group was 21% versus 46% in the group with normal renal ultrasound (P = 0.01). Combining grade II and III reflux, an abnormal ultrasound was associated with a statistically significantly lower resolution rate (grade II-III 23% vs 47%, P = 0.049). For children with moderate hydronephrosis, 8/9 (89%) had abnormal initial renal scans and all failed to achieve resolution of reflux at 2 years. CONCLUSIONS: In this cohort of children with VUR, 30% had abnormalities on renal ultrasound. The presence of moderate hydronephrosis on ultrasound may indicate a high likelihood of abnormality on renal scan and failure to achieve early resolution of VUR.

Safety and efficacy of spica casts for immobilization following initial bladder closure in classic bladder exstrophy.

OBJECTIVES: Pelvic immobilization constitutes a necessary component of successful bladder exstrophy closure. The efficacy of spica cast immobilization has been reported as markedly inferior to external fixation, with success rates below 25%. We reviewed our experience with spica cast immobilization following bladder closure. PATIENTS AND METHODS: We retrospectively reviewed classic bladder exstrophy patients undergoing bladder closure with spica cast immobilization. Success of bladder closure and complications related to immobilization were noted, as were age, type of closure, use of osteotomy, duration of immobilization, and number of cast changes. RESULTS: Fifteen patients underwent bladder closure (10 staged, 5 complete repair) at a median age of 4 days (range 1-6) and 14 were immobilized with spica casts. Initial closures were successful in 11 (73%). Success rates were higher in patients undergoing osteotomies (6/7, 86%) compared to those without osteotomies (5/8, 63%). No patients immobilized with spica casts developed serious complications related to their immobilization. Minor skin breakdown occurred in 3/14 patients (21%). Median time of immobilization was 39 days (range 22-48). CONCLUSIONS: Spica casts are a safe, effective method of postoperative immobilization and are associated with a low risk of cast-related complications.

Vesicoureteral reflux: who benefits from correction.

There has been an emergence of a therapeutic nihilistic attitude about the surgical treatment of vesicoureteral reflux (VUR). Evidence-based reviews have questioned whether surgical treatment is beneficial for children with VUR. Even the use of prophylactic antibiotics, which have traditionally been the first-line therapy recommended for virtually all patients with VUR, has come under scrutiny after several randomized controlled trials found them to have no effect on decreasing the risk of urinary tract infections (UTIs) in children with VUR. Grade is the strongest predictor of VUR resolution, with high-grade VUR being much less likely to resolve. Other factors that negatively influence resolution include lower bladder volume or pressure at onset of reflux, older age, female sex, bilateral VUR, ureteral duplication, abnormal or scarred kidneys, and bladder dysfunction. These factors can be used, along with grade, in computer models or nomograms to improve the ability to predict spontaneous resolution.

Supernumerary kidney in a child with OEIS complex.

Supernumerary kidney and cloacal exstrophy are rare anomalies of the genitourinary tract. We present an exceedingly rare case of a supernumerary third kidney discovered by antegrade nephrostography of a 6-month-old child with OEIS complex (omphalocele, exstrophy, imperforate anus, spinal defects).

Rare variant of bladder exstrophy associated with urethral, bladder, and colonic duplication.

Urethral duplication, bladder duplication, and bladder exstrophy are rare congenital urinary tract anomalies. We present an extremely rare case of complete urethral and bladder duplication in a boy with bladder exstrophy, omphalocele, and colonic duplication. Bladder augmentation was subsequently performed using the left exstrophic bladder as a patch to augment the right duplicated bladder.