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.

Digital photographic measurement in hypospadias: validation and comparison to intraoperative measurement.

BACKGROUND: We propose a standardized method of photographing the hypospadias penis to capture penile dimensions that may be relevant for surgery. We also validate the use of digital imaging software for calculating penile dimensions as a substitute for intraoperative caliper-based measurements. METHODS: Photographs were taken of hypospadias penises in four different views after placement of a traction stitch and retraction of the preputial hood. Intraoperative measurements were obtained with a caliper. Digital measurements were taken of the same parameters postoperatively. All measurements were obtained in triplicate by multiple participants, and averages were tested for equivalency by determining the correlation coefficient for each parameter. Inter-observer correlation was also calculated for each parameter. RESULTS: 180 intraoperative and 180 digital measurements were taken from 60 dimensions on 10 hypospadiac penises. Strong correlation existed between most digital and intraoperative measurements. Average inter-observer correlations ranged from 0.91 to 0.99 for each of the intraoperative measurements, and 0.90 to 1.00 for each of the digital measurements. CONCLUSIONS: Standardized imaging is effective in capturing penile dimensions and measurements during hypospadias surgery. When compared with intraoperative measurements, digital measurements are reliable and precise; digital photography has the potential to both aid in surgical planning and improve documentation.

Classic bladder exstrophy: orthopaedic surgical considerations.

Classic bladder exstrophy is an embryologic malformation that results in complex deficiency of the anterior midline, with urogenital and skeletal manifestations. Urogenital reconstruction is a challenging procedure that can be facilitated by closure of the bony pelvic ring by an orthopaedic team. Surgical options include a multiyear staged approach and the single-stage complete repair for exstrophy. The goals of urologic surgery include closure of the bladder and abdominal wall with eventual bladder continence, preservation of renal function, and cosmetic and functional reconstruction of the genitalia. Pelvic osteotomy is done at the time of bladder closure in the patient in whom the anterior pelvis cannot be approximated without tension. Traction or spica casting is used postoperatively. Good outcomes are probable with appropriate management at specialized treatment centers.

Comparison of the learning curve and outcomes of robotic assisted pediatric pyeloplasty.

PURPOSE: We compared the learning curve and outcomes in children undergoing robotic assisted laparoscopic pyeloplasty during the initiation of a robotic surgery program compared to the benchmark of open pyeloplasty. MATERIALS AND METHODS: The records of our first consecutive 33 children undergoing robotic assisted laparoscopic pyeloplasty from 2006 to 2009 were retrospectively reviewed and compared to those of age and gender matched children who underwent open repair done by senior faculty surgeons before the initiation of our robotic surgery program. We compared operative time, complications, postoperative pain, length of stay and surgical success for 2 surgeons who adopted the robotic approach at an academic teaching institution. RESULTS: We found no significant differences in length of stay, pain score or surgical success at a median followup of 16 months. The number of complications was similar and they tended to be early and technical in the robotic assisted laparoscopic pyeloplasty group. Overall average operative time was 90 minutes longer (38%) for robotic assisted laparoscopic pyeloplasty (p <0.004). When evaluated chronologically, there was evidence of a learning curve. After 15 to 20 robotic cases overall operative times for robotic assisted laparoscopic cases was consistently within 1 SD of our average open pyeloplasty time with no significant difference in overall operative time (p = 0.23). Of the decrease in overall operative time 70% was due to decreased pyeloplasty time rather than peripheral time. CONCLUSIONS: There was similar safety and efficacy with robotic assisted laparoscopic pyeloplasty, although complications tended to be technical and early in our initial experience. Operative time decreased with experience and after 15 to 20 cases it was similar to that of open pyeloplasty with similar outcomes and surgical success.

Can we rely on the presence of dextranomer-hyaluronic acid copolymer mounds on ultrasound to predict vesicoureteral reflux resolution after injection therapy?

PURPOSE: We determined whether the presence or absence of dextranomer-hyaluronic acid copolymer mounds on bladder ultrasound coincides with voiding cystourethrogram results after injection therapy in children with primary vesicoureteral reflux. MATERIALS AND METHODS: We retrospectively reviewed consecutive cases of dextranomer-hyaluronic acid copolymer injection for primary vesicoureteral reflux. The primary outcome investigated was the appearance of dextranomer-hyaluronic acid copolymer mounds on ultrasound and their association with voiding cystourethrogram results postoperatively. An intramural dextranomer-hyaluronic acid copolymer mound on ultrasound was considered a negative test result for vesicoureteral reflux. RESULTS: A total of 187 cases were identified, of which 132 had imaging available for evaluation. Intramural mounds were seen on ultrasound in 86 cases postoperatively, of which 34 (40%) had a positive voiding cystourethrogram. Of 46 cases in which no mound was identified 21 (46%) had a positive voiding cystourethrogram. The sensitivity of ultrasound to determine the presence or absence of vesicoureteral reflux was 38%, specificity was 67%, accuracy was 55%, the positive predictive value was 46% and the negative predictive value was 60%. CONCLUSIONS: Our results indicate poor correlation of dextranomer-hyaluronic acid copolymer mound appearance on ultrasound with voiding cystourethrogram results after injection. To adequately evaluate for the resolution of vesicoureteral reflux a voiding cystogram or nuclear medicine cystogram needs to be performed after injection therapy.

Spica casting compared to Bryant's traction after complete primary repair of exstrophy: safe and effective in a longitudinal cohort study.

PURPOSE: Bryant's traction is the most commonly used method for immobilization after bladder exstrophy repair. We hypothesized that spica casting is a safe and effective alternative to Bryant's traction after complete primary repair of exstrophy. MATERIALS AND METHODS: Complete primary repair of exstrophy was performed for initial repair in 39 consecutive children by all surgeons at Seattle Children's Hospital since 1998. Three sequential cohorts were evaluated-Bryant's traction without osteotomy (13 patients), spica casting without osteotomy (14) and spica casting with osteotomy. These 3 sequential cohorts represent eras of care and an evolution of practice. Primary outcomes included major complications related to immobilization, dehiscence, urinary incontinence and length of stay. We defined complications of immobilization as nonunion of pelvic osteotomy, femoral nerve palsy, revision of spica cast requiring return to the operating room, infection at the osteotomy site and activity limiting pain at the osteotomy site. Fisher's exact test or t test was used to determine statistical significance. RESULTS: There was no difference in urinary continence (p = 0.09). Use of Bryant's traction was associated with double the length of stay (p >0.001). There was no correlation of major complications to the type of immobilization used. CONCLUSIONS: Spica casting compared to Bryant's traction is associated with shorter hospitalization following complete primary repair of exstrophy and does not have a significant difference in the rate of complications. In our longitudinal cohort study with long-term followup spica cast was safe and effective for patients with bladder exstrophy, and should be considered an acceptable method of immobilization.

Initiation of a pediatric robotic surgery program: institutional challenges and realistic outcomes.

BACKGROUND: Few institutions have reported their experience initiating a pediatric robot-assisted laparoscopic (RAL) program, and results vary regarding the outcomes for robotic surgery in children. We present the initiation of our pediatric robotic surgery program, provide suggestions for overcoming institutional challenges, and perform a comparative analysis to illustrate realistic outcomes during the learning curve. METHODS: Outcomes from consecutive children who underwent RAL surgery since the 2006 acquisition of the da Vinci® surgical system were retrospectively reviewed. To evaluate the safety and outcomes during the introduction of this new technology, we performed an outcome analysis of ureteral reimplantations comparing RAL cases to matched open controls. RESULTS: The first 50 RAL cases were performed over 20 months by two general and two urologic surgeons. Fourteen different procedures were performed successfully. The average patient age was 8.6 ± 5.7 years with 10 patients weighing less than 10 kg (20%). Three urologic cases were converted to traditional laparoscopy and two general surgery cases were converted to open. There were five mechanical failures. Initial outcomes comparing RAL and open ureteral reimplantations revealed similar length of stay, complications, and success with lower estimated blood loss in the RAL group. Overall OR time was 53% longer in the RAL reimplant group (361 ± 80 vs. 236 ± 58 min, p < 0.0001). CONCLUSION: Robotic surgery appears to be safe in pediatric patients for many procedures. Proper instruction and training precedes technological proficiency. The institutional learning curve may be magnified when there are multiple participating surgeons. Operative times for initial RAL cases can be expected to be greater than their open correlates.

Effects of cranberry juice on pharmacokinetics of beta-lactam antibiotics following oral administration.

Cranberry juice consumption is often recommended along with low-dose oral antibiotics for prophylaxis for recurrent urinary tract infection (UTI). Because multiple membrane transporters are involved in the intestinal absorption and renal excretion of beta-lactam antibiotics, we evaluated the potential risk of pharmacokinetic interactions between cranberry juice and the beta-lactams amoxicillin (amoxicilline) and cefaclor. The amoxicillin-cranberry juice interaction was investigated in 18 healthy women who received on four separate occasions a single oral test dose of amoxicillin at 500 mg and 2 g with or without cranberry juice cocktail (8 oz) according to a crossover design. A parallel cefaclor-cranberry juice interaction study was also conducted in which 500 mg cefaclor was administered with or without cranberry juice cocktail (12 oz). Data were analyzed by noncompartmental methods and nonlinear mixed-effects compartmental modeling. We conclude that the concurrent use of cranberry juice has no significant effect on the extent of oral absorption or the renal clearance of amoxicillin and cefaclor. However, delays in the absorption of amoxicillin and cefaclor were observed. These results suggest that the use of cranberry juice at usual quantities as prophylaxis for UTI is not likely to alter the pharmacokinetics of these two oral antibiotics.

Surgical management of congenital ureteropelvic junction obstruction: a Pediatric Health Information System database study.

PURPOSE: Although laparoscopic pyeloplasty has gained popularity, to our knowledge no multi-institutional study has evaluated the prevalence of this approach in children. We used a multicenter database to determine trends in the treatment of congenital ureteropelvic junction obstruction. MATERIALS AND METHODS: The Pediatric Health Information System database contains data on 37 freestanding hospitals for children across the United States. We extracted data on 0 to 19-year-old patients from 2001 to 2006 with the ICD-9 diagnosis code for congenital ureteropelvic junction obstruction and the procedure code for the correction of ureteropelvic junction obstruction. We identified laparoscopic cases based on hospital charges for 1) laparoscope, 2) trocar, 3) insufflating needle or 4) insufflator and tubing. Data were then analyzed using the chi-square and Student t tests to determine management trends. RESULTS: We identified 2,353 patients, of whom 2,177 (92.5%) underwent open pyeloplasty and 176 (7.5%) underwent laparoscopic pyeloplasty. The percent of pediatric pyeloplasties performed laparoscopically increased from 2001 to 2003 (2.53% to 9.73%) and has since remained stable. Patients undergoing laparoscopic pyeloplasty were significantly older than those in the open group (age 8.2 vs 3.3 years, p <0.0001). Average hospital charges were significantly higher in the laparoscopic group than in the open group ($23,295.71 vs $16,467.49, p <0.05). There was no significant difference in terms of race, gender or length of stay. CONCLUSIONS: The percent of pediatric pyeloplasties performed laparoscopically has increased with time. However, laparoscopic pyeloplasty is associated with higher hospital charges than open surgery without a significant decrease in length of stay.

Long-term followup of complete primary repair of exstrophy: the Seattle experience.

PURPOSE: Since 1989, we have used the complete primary repair of exstrophy surgical technique to reconstruct the genitourinary system of children born with the exstrophy-epispadias complex based on the assumption that this complex represents a malformation. We initially reported using this technique in 1999. We now report a longer term followup of this initial group as well as surgical outcomes in a larger group of children who have undergone this repair for classic bladder exstrophy. MATERIALS AND METHODS: Since 1989, we have prospectively followed 39 children who underwent the complete primary repair of exstrophy technique to construct classic bladder exstrophy. Median followup in the original group of patients that we reported in 1999 is 106 months. Median followup in the entire series is 58 months. RESULTS: Of boys and girls 4 years or older 74% have achieved daytime continence with volitional voiding. Of boys and girls 20% and 43%, respectively, have achieved primary urinary continence without the need for bladder neck reconstruction. An additional 18% of boys and girls achieved continence with only bladder neck injection following complete primary repair of exstrophy. Complications developed in 7 of the 39 children (18%) in the entire series. CONCLUSIONS: Urinary continence has been consistently achieved with this form of exstrophy repair. Primary continence without the need for further reconstruction is possible. The results of this patient series have caused us to modify the complete primary repair of exstrophy technique in an effort to improve the rate of primary urinary continence.

Robotic-assisted laparoscopic mitrofanoff appendicovesicostomy and antegrade continent enema colon tube creation in a pediatric spina bifida patient.

Robotic-assisted laparoscopic surgery has been applied to pediatric surgery, especially for technically challenging reconstructive procedures owing to the improved suturing capabilities over pure laparoscopic techniques when using fine suture material. In this paper, we report the techniques of creating both an appendicovesicostomy and an antegrade continent enema colon tube in a 9-year-old female with a neurogenic bladder and bowel secondary to myelomeningocele.

Metastatic adenocarcinoma after augmentation gastrocystoplasty.

PURPOSE: Augmentation gastrocystoplasty has been proposed as an alternative to enterocystoplasty because of potential benefits, including decreased risk of mucus production, stone formation and urinary tract infections. Although cancer has rarely been reported in this patient population, it is a well recognized potential risk of all augmentation cystoplasties. To define better the risk of malignancy associated with gastric augmentation and the appropriate surveillance protocol for these patients, we describe our experience in 2 patients with metastatic adenocarcinoma following gastrocystoplasty. MATERIALS AND METHODS: We retrospectively reviewed the charts of all patients who had undergone augmentation gastrocystoplasty between 1990 and 1994. Of the 72 patients identified 2 were diagnosed with a primary malignancy arising from the augmented bladder. Charts were reviewed for medical history, clinical outcomes and pathology. RESULTS: Two patients were identified with a primary bladder malignancy after gastrocystoplasty. Both patients had metastatic disease at initial presentation. Neither patient had a history of gross hematuria, recurrent urinary tract infections or pain before initial presentation. Mean patient age at augmentation was 5.5 years. Mean age at diagnosis of malignancy was 19.5 years, with a mean time from augmentation of 14 years. CONCLUSIONS: Although the risk of bladder cancer is low after gastric augmentation, the effects may be life threatening. Therefore, we advocate routine annual surveillance with cystoscopy, bladder biopsy and upper tract imaging in all patients who have undergone augmentation gastrocystoplasty.

Recurrent nephrogenic adenoma in a 10-year-old boy with prune belly syndrome : a case presentation.

Nephrogenic adenoma is a rare benign lesion of the urinary tract that is associated with a history of irritation or injury of the urothelium. Predisposing factors include infection, calculi, surgery, trauma, and renal transplantation. Nephrogenic adenoma commonly presents with lower urinary tract symptoms or hematuria. We present the case of recurrent nephrogenic adenoma in a 10-year-old boy with a history of prune belly syndrome and discuss management of this disease in the pediatric population. To our knowledge this represents the first reported case of recurrent nephrogenic adenoma associated with prune belly syndrome.

Augmentation cystoplasty rates at children's hospitals in the United States: a pediatric health information system database study.

PURPOSE: We identified augmentation cystoplasty rates in children with spina bifida at children's hospitals enrolled in the Pediatric Health Information System database. MATERIALS AND METHODS: The Pediatric Health Information System database tabulates demographic and diagnostic patient data from 35 children's hospital centers in the United States. Between October 1999 and September 2004 we extracted data on 0 to 19-year-old patients with International Classification of Diseases-9 diagnosis codes for spina bifida. The International Classification of Diseases-9 procedure code for augmentation cystoplasty was cross-referenced with these patients to determine the total number of patients with augmentation, total population augmentation rates and individual institution rates of bladder augmentation. RESULTS: Staff at enrolled pediatric medical centers submitted inpatient data accounting for 9,059 beds servicing an aggregate metropolitan population of 82 million individuals. In the 5-year period 12,925 unique spina bifida patient encounters were identified, including 665 patients who underwent augmentation cystoplasty. The mean 5-year institutional number of augmentations performed in children with spina bifida was 20 (range 1 to 121) and the mean annual number of augmentations performed per institution was 4. The overall augmentation rate at 33 hospitals contributing data for the full years 2000 to 2003 was 5.4% (range 0.5% to 16.3%, p <0.0001). The male-to-female ratio of those who underwent augmentation was 1:1.2. Median length of stay in children with augmentation was 7 days (mean 9). The median age of children with augmentation was 10.4 years, that is 11.3 years in boys and 9.8 years in girls. The difference in mean age was statistically significant (p <0.003). At institutions where 10 or more augmentations were performed in 5 years (mean 27) mean patient age at operation was 10.1 years. This was significantly younger than the mean patient age of 12.3 years at hospitals where fewer than 10 augmentations (mean 5) were done in 5 years (p <0.05). CONCLUSIONS: Clinical management for neurogenic bladder conditions has evolved to emphasize nonoperative management. Several studies suggest that aggressive early intervention improves bladder compliance and may protect renal function. However, results from the Pediatric Health Information System database demonstrate no change in augmentation rates during this time and they demonstrate significant interinstitutional variability. To our knowledge this represents the largest series of augmentation cystoplasty in children with spina bifida to date.