Surgeon, and Institution Characteristics Associated Surgical Preferences in the Pediatric KIDney Stone Care Improvement Network.

OBJECTIVE: To reveal barriers and opportunities to implement evidence for the management of pediatric kidney stone disease, we determined surgeon and institutional factors associated with preferences for the type of surgical intervention for kidney and ureteral stones. METHODS: We conducted a cross-sectional study of urologists participating in the Pediatric KIDney Stone Care Improvement Network (PKIDS) trial. Questionnaires ascertained strengths of urologists' preferences for types of surgery as well as characteristics of participating urologists and institutions. The outcome was the strength of preferences for ureteroscopy, shockwave lithotripsy, and percutaneous nephrolithotomy for four scenarios for which two alternative procedures are recommended by the AUA guidelines: (1) 2 cm kidney stone, (2) 9 mm proximal ureteral stone, (3) 1.5 cm lower pole kidney stone, (4) 1 cm nonlower pole kidney stone. Principal component analysis was performed to identify unique clusters of factors that explain surgical preferences. RESULTS: One hundred forty-eight urologists at 29 sites completed surveys. Stated preferences were highly skewed except for the choice between ureteroscopy and percutaneous nephrolithotomy for a 1.5 cm kidney stone. Shockwave lithotripsy ownership and local practice patterns most frequently associated with the strength of surgeons' preferences for the type of surgery. Principal component analysis revealed that three clusters of stone, patient, and heterogenous characteristics explained 30% of the variance in preferences. CONCLUSION: There is wide variation in the strengths of preferences for surgical interventions supported by current guidelines that are partially explained by surgeon and institutional characteristics. These results reveal opportunities to develop strategies for guidelines that consider real-world drivers of care.

Impact of Social Disparities on 5-Year Survival Rates in Pediatric Hematologic Malignancies.

BACKGROUND: Socioeconomic disparities exist in pediatric patients with hematologic malignancies, leading to suboptimal survival rates. Social determinants of health impact health outcomes, and in children, they may not only lead to worse survival outcomes but carry over into late effects in adult life. The social deprivation index (SDI) is a composite score using geographic county data to measure social determinants of health. Using the SDI, the purpose of the present study is to stratify survival outcomes in pediatric patients with hematologic malignancies based on area deprivation. METHODS: A retrospective cohort study was performed using the national Surveillance, Epidemiology, and End Results oncology registry in the USA from 1975 to 2016 based on county-level data. Pediatric patients (≤18 y old) with a diagnosis of leukemia or lymphoma based on the International Classification for Oncology, third edition (ICD-O-3) were used for inclusion criteria. Patients were grouped by cancer subtype for leukemia into acute lymphoblastic leukemia (ALL) and acute myeloid leukemia while for lymphoma into non-Hodgkin's lymphoma and Hodgkin's lymphoma. SDI scores were calculated for each patient and divided into quartiles, with Q1 being the lowest area of deprivation and Q4 being the highest, respectively. RESULTS: A total of 38,318 leukemia and lymphoma patients were included. Quartile data demonstrated stratification in survival based on area deprivation for ALL, with no survival differences in the other cancer subtypes. Patients with ALL from the most deprived area had a roughly 3% difference in both overall and cancer-specific morality at 5 years compared with the least deprived area. CONCLUSION: Disparities in pediatric patients with ALL represent a significant area for quality improvement. Social programs may have value in improving survival outcomes and could rely on metrics such as SDI.

Impact of social disparities on 10 year survival rates in paediatric cancers: a cohort study.

Background: Studies reporting on the impact of social determinants of health on childhood cancer are limited. The current study aimed to examine the relationship between health disparities, as measured by the social deprivation index, and mortality in paediatric oncology patients using a population-based national database. Methods: In this cohort study of children across all paediatric cancers, survival rates were determined using the Surveillance, Epidemiology, and End Results (SEER) database from 1975 to 2016. The social deprivation index was used to measure and assess healthcare disparities and specifically the impact on both overall and cancer-specific survival. Hazard ratios were used to assess the association of area deprivation. Findings: The study cohort was composed of 99,542 patients with paediatric cancer. Patients had a median age of 10 years old (IQR: 3-16) with 46,109 (46.3%) of female sex. Based on race, 79,984 (80.4%) of patients were identified as white while 10,801 (10.9%) were identified as Black. Patients from socially deprived areas had significantly higher hazard of death overall for both non-metastatic [1.27 (95% CI: 1.19-1.36)] and metastatic presentations [1.09 (95% CI: 1.05-1.15)] compared to in more socially affluent areas. Interpretation: Patients from the most socially deprived areas had lower rates of overall and cancer-specific survival compared to patients from socially affluent areas. With an increase in childhood cancer survivors, implementation of social determinant indices, such as the social deprivation index, might aid improvement in healthcare outcomes for the most vulnerable patients. Funding: There was no study sponsor or extramural funding.

Open versus minimally invasive ureteroneocystostomy: trends and outcomes in a NSQIP-P cohort.

Although surgical intervention has commonly been performed using an open approach for vesicoureteral reflux (VUR), this is rapidly changing due to adoption of minimally invasive surgery (MIS). Success rates with MIS are similar to open for re-implantation (> 90%); however, open ureteral re-implantation is still widely considered the gold standard. Using national surgical quality improvement program-pediatric (NSQIP-P) data, this manuscript evaluates recent large population trends of open versus robotic-assisted and laparoscopic ureteroneocystostomy for complications and factors associated with worse outcomes. Cases were identified in the 2012-2019 NSQIP-P database using the ureteroneocystostomy operative codes and vesicoureteral reflux post-operative diagnosis codes. A 1:1 propensity score match (PSM) analysis was performed comparing surgical outcomes while matching patients with similar characteristics to reduce bias. A total of 4183 patients were included; 621 patients with MIS and 3562 with open approach. Patients in the MIS approach tended to be older (67 months vs. 53 months) and non-Caucasian (12.9% vs. 6.3%) with no differences in other demographics. After 1:1 PSM, 30-day complications after ureteroneocystostomy showed no significant differences in readmission, reoperation, or extended hospital stay. A multivariate analysis found patients with CNS structural abnormalities (such as spina bifida) had 4.5 times greater odds of experiencing a reoperation (p value < 0.05). Similarly, patients with an ASA above two had 2.0 times greater odds of an UTI (p value < 0.05). The cohorts undergoing open and MIS approaches are well matched overall, without profound differences in outcomes overall.

Effect of social disparities on 10 year survival in pediatric patients with Wilms' tumor.

BACKGROUND: To stratify 10-year survival outcomes by degree of social disparities in pediatric Wilms' tumor patients. We applied the Social Deprivation Index (SDI) to survival outcomes from the national SEER database to elucidate the effects of lower socioeconomics on cancer survival. METHODS: A retrospective cohort study was performed using the national Surveillance, Epidemiology, and End Results (SEER) oncology registry from 1975 to 2016 based on county-level data. Pediatric patients (<18 years old) with a diagnosis of WT (C64.9) and confirmed based on histology codes (8960/8963) were included. SDI scores were calculated for each patient and initially divided into quintiles. Patients were delineated into high-risk (>60th percentile/more deprived) or low-risk (<60th percentile/less deprived) groups. Statistics were assessed using Fisher's exact test, Student's t-test, and Kaplan-Meier assessed survival differences with log-rank test for trend. RESULTS: A total of 3406 patients were included with 1366 patients reported in the high-risk group and 2040 patients in the low-risk group. Quintile data demonstrated a stratification in survival based on socioeconomic status. Patients in more socially deprived counties were significantly (p = 0.035) more likely to have worse overall survival compared with those living in less deprived areas at 10-year (87.3% vs 89.3%) follow-up. CONCLUSIONS: 10-year overall and cancer-specific survival data for patients with Wilms' tumor stratify by socioeconomic lines. This represents an area that needs to be addressed in this pediatric oncologic population. Patients from more socially deprived areas have significantly worse 10-year overall survival rates and noticeably different 10-year cancer-specific survival rates.

Volumetric and acetabular changes in the bony pelvis associated with primary closure of classic bladder exstrophy.

INTRODUCTION: Repair of classic bladder exstrophy (CBE) is known to alter dimensions of the bony pelvic ring. Pelvic volume and acetabular configuration are additional metrics which merit analysis in the reconstruction process. Advances in magnetic resonance imaging (MRI) allow for precise elucidation of such anatomy in pediatric patients, providing enhanced knowledge of how primary reconstruction may impact factors in pelvic health. METHODS: An IRB-approved exstrophy-epispadias database of 1337 patients was reviewed for patients with CBE who had pelvic MRI performed before and after repair. Pelvic MRIs were analyzed by a pediatric radiologist, and three-dimensional volumetric renderings of the true pelvis were calculated. Pre- and post-closure imaging were compared, in addition to imaging from age-matched controls without pelvic pathology. Cartilaginous acetabular index and version angles were also calculated and compared between groups. RESULTS: Eighteen patients with post-closure imaging, 14 of whom also had pre-closure imaging, and 23 control patients (ages 0-365 days) were included. The median ages at pre- and post-closure scan were 2 and 178 days, respectively. Osteotomy was performed in 6 (33%) of the closures. The median segmented pelvic volumes were 89 cm3 in the pre-closure group, 105 cm3 in the post-closure group, and 72 cm3 in the control group. At a given age, patients with CBE pre-closure had the largest pelvic volume, and those without bladder exstrophy demonstrated the smallest pelvic volumes (Summary Figure). CBE patients' pelvic volumes were overall lower following repair, relative to age (p = 0.007). Cartilaginous acetabular version angle increased following closure, with acetabular orientation converting from retroversion to anteversion in all cases. However, only acetabular version angles were significantly different between groups. DISCUSSION: This study found that pelvic volume significantly decreased relative to age following primary repair of CBE, but that it did not correct to control levels. Similarly, acetabular retroversion that is naturally seen in exstrophy patients was converted to anteversion post-closure but did not become completely normal. Knowledge of these persistent anatomical anomalies may be useful in treatment of future concerns related to exstrophy, such as continence achievement, pelvic organ prolapse, and potential gait disturbances. Lack of significant differences between study groups regarding acetabular index angles can give assurance to providers that hip dysplasia is neither a natural concern nor iatrogenically inflicted through reconstruction. CONCLUSIONS: The primary closure of exstrophy results in pelvic volumes and anteverted acetabula that more closely resemble those without bladder exstrophy, compared to pre-closure findings.

Laparoscopic intra-abdominal patent processus vaginalis ligation in pediatric urology practice.

INTRODUCTION AND OBJECTIVE: Inguinal hernias and communicating hydroceles from a patent processus vaginalis (PPV) are common problems in children. This study provides a detailed description of the laparoscopic intra-abdominal patent processus vaginalis ligation (LIPPL) procedure along with its results in pediatric urology patients. METHODS: Prospectively collected data were captured from children (<18 years) who underwent LIPPL from 2012 to 2014. Demographics as well as postoperative characteristics were reviewed and descriptively analyzed. LIPPL is performed using a 5-mm camera through the umbilicus. A loop of polypropylene suture is passed through a spinal needle percutaneously on one side of the internal ring. The needle is reinserted on the opposite side and the tail of the suture is fed through the original loop such that a purse-string is created around the peritoneum of the internal ring above the spermatic vessels and the vas; the suture is tied extracorporeally. RESULTS: 142 patients (3 female) were evaluated with 197 PPV sites repaired using LIPPL at median 24 (1-216) months of age. Median operating time was 35 (20-91) and 43 (27-85) minutes for unilateral and bilateral repairs, respectively. There were no intraoperative complications. During the 14 (1-34) months from surgery, there were no hernia recurrences, one surgical site infection, seven patients with residual small non-communicating hydroceles, and one patient who developed a suture granuloma. CONCLUSIONS: LIPPL is a safe and effective technique with minimal rate of hernia recurrence and few complications. LIPPL allows for easy repair of all sizes of PPV, with patients receiving the benefits of minimally invasive surgery including the ability to evaluate the contralateral inguinal ring.

Open Partial Nephrectomy for Wilms' Tumor in a Cross-fused Pelvic Ectopic Kidney.

Wilms' tumor is the most common pediatric solid renal tumor. Cross-fused renal ectopia is a rare congenital anomaly in which the left and right kidneys become fused and fail to ascend from the pelvis and abdomen. We report a case of a 5-year-old girl that underwent open partial nephrectomy on a cross-fused ectopic kidney, "pancake kidney," after incidental discovery of a solid renal mass found to be a Wilms' tumor. Thorough review of the literature shows that this combination of Wilms' tumor in the setting of cross-fused renal ectopia has only been reported twice previously.

Continence in the cloacal exstrophy patient: What does it cost?

BACKGROUND: Surgical advancements have made cloacal exstrophy (CE) a survivable condition, though management remains complex. Urologic, orthopedic, colorectal and gynecologic interventions are not standardized, and the cost of this care is high. While the importance of a successful primary closure in terms of outcomes is known, the economic consequences of failure remain uncharacterized. METHODS: A prospectively maintained institutional database of epispadias-exstrophy complex patients was reviewed for continent CE patients. Hospital charges for all inpatient admissions prior to achieving urinary continence were inflation-adjusted to year 2013 values using Consumer Price Index for medical care published by the United States Bureau of Labor Statistics. Records for which charge data were incomplete were completed by using single mean imputation, also inflation-adjusted. Descriptive data are presented as mean±standard deviation (SD). RESULTS: Of 102 CE patients, 35 had available hospital charge data: 15 who underwent successful primary closure at the authors' institution and 20 who presented after previously failed primary closures at referring institutions. The mean±SD hospital charges for primary closure in the success group were $136,201±$48,920. These patients then underwent subsequent additional surgeries that accrued charges of $59,549±$25,189 in order to achieve continence. Overall, successful primary closures accumulated hospital charges of $200,366±$40,071. In comparison, patients referred after prior failure required significantly more hospital admissions and additional charges of $207,674±$65,820 were required to achieve continence (p<0.001). Patients who failed primary closure are estimated to accumulate 70% more total health care charges compared to the group following successful primary closure. CONCLUSION: The cost of CE management until urinary continence is high, averaging more than $200,000 in inpatient hospital charges alone. Initial success is desirable from both an outcomes and economic perspective, as the cost of salvaging a failed primary closure at our institution is similar to the overall costs of a successful closure; this is in addition to the cost of any previous failed closures. Further studies will be required to determine the optimal timing of surgical management in terms of both patient outcomes and financial consequences.

Post-surgical infections and perioperative antibiotics usage in pediatric genitourinary procedures.

BACKGROUND: Post-surgical infections (PSIs) are a source of preventable perioperative morbidity. No guidelines exist for the use of perioperative antibiotics in pediatric urologic procedures. OBJECTIVE: This study reports the rate of PSIs in non-endoscopic pediatric genitourinary procedures at our institution. Secondary aims evaluate the association of PSI with other perioperative variables, including wound class (WC) and perioperative antibiotic administration. STUDY DESIGN: Data from consecutive non-endoscopic pediatric urologic procedures performed between August 2011 and April 2014 were examined retrospectively. The primary outcome was the rate of PSIs. PSIs were classified as superficial skin (SS) and deep/organ site (D/OS) according to Centers for Disease Control and Prevention guidelines, and urinary tract infection (UTI). PSIs were further stratified by WC1 and WC2 and perioperative antibiotic usage. A relative risk and chi-square analysis compared PSI rates between WC1 and WC2 procedures. RESULTS: A total of 1185 unique patients with 1384 surgical sites were reviewed; 1192 surgical sites had follow-up for inclusion into the study. Ten total PSIs were identified, for an overall infection rate of 0.83%. Of these, six were SS, one was D/OS, and three were UTIs. The PSI rate for WC1 (885 sites) and WC2 (307 sites) procedures was 0.34% and 2.28%, respectively, p < 0.01. Relative risk of infection in WC2 procedures was 6.7 (CI 1.75-25.85, p = 0.0055). The rate of infections in WC1 procedures was similar between those receiving and not receiving perioperative antibiotics (0.35% vs. 0.33%). All WC2 procedures received antibiotics. DISCUSSION: Post-surgical infections are associated with significant perioperative morbidity. In some studies, PSI can double hospital costs, and contribute to hospital length of stay, admission to intensive care units, and impact patient mortality. Our study demonstrates that the rate of PSI in WC1 operations is low, irrespective of whether the patient received perioperative antibiotics (0.35%) or no antibiotics (0.33%). WC2 operations were the larger source of morbidity with an infection rate of 2.28% and a 6.7 fold higher increase in relative risk. CONCLUSIONS: WC1 procedures have a rate of infection around 0.3%, which is independent of the use of perioperative antibiotics. WC2 procedures have a higher rate of infection, with a relative risk of 6.7 for the development of PSI, and should be the target of guidelines for periprocedural prophylaxis.

Comparison of musculoskeletal anatomic relationships, determined by magnetic resonance imaging, in postpubertal female patients with and without classic bladder exstrophy.

UNLABELLED: WHAT'S KNOWN ON THE SUBJECT? AND WHAT DOES THE STUDY ADD?: Several studies in the paediatric literature have characterized the pelvic musculoskeletal anatomy of infants and children with bladder exstrophy using MRI and three-dimensional CT. The pelvic floor anatomy of female patients with bladder exstrophy who have undergone somatic growth and puberty is less well described. This study uses MRI to characterize comprehensively the pelvic anatomy of postpubertal females with classic bladder exstrophy by measuring 15 pelvic floor variables previously described in younger children with bladder exstrophy. OBJECTIVE: To characterize pelvic musculoskeletal anatomy in postpubertal females with classic bladder exstrophy, and to compare this with females without bladder exstrophy. PATIENTS AND METHODS: The authors reviewed the medical records of all females in our institutional review board-approved bladder exstrophy database of 1078 patients and identified those with classic bladder exstrophy who underwent pelvic magnetic resonance imaging (MRI) after the age of 12 years. Indications for MRI included haematuria, adnexal lesion, perineal fistula, non-pelvic cancer staging, abdominal wall hernia and vaginal stenosis. Age- and race-matched female patients without exstrophy who underwent MRI evaluation for similar indications were included for comparison. The MRI protocol included axial, sagittal and coronal T1- and/or T2-weighted imaging. RESULTS: The study included 30 patients with a median (range) age of 22.5 (12-55) years at time of MRI. Ten patients had bladder exstrophy while 20 control patients did not. A smaller percentage of levator ani was located in the anterior compartment of the pelvis in patients with bladder exstrophy compared with controls. The iliac wing angle, puborectalis angle, ileococcygeous angle, levator ani width, symphyseal diastasis, erectile body diastasis, posterior bladder neck distance and posterior anal distance was greater in patients with bladder exstrophy than in those without. The ischial angle and obturator internus angle were narrower in patients with bladder exstrophy than in those without, and there was no significant difference between levator ani surface area, sacral anal angle, sacral bladder neck angle and bladder neck erectile body distance between the two patient groups. CONCLUSIONS: In postpubertal females with bladder exstrophy, significant deviations from normal pelvimetry exist, including posterior location of the majority of the levator ani muscle, a wider ileococcygeous angle and a wider symphyseal diastasis. These differences are similar to those described in previous comparisons of younger children with bladder exstrophy and control children.

Preservation of renal function in the modern staged repair of classic bladder exstrophy.

OBJECTIVE: To compare the estimated glomerular filtration rate (eGFR) in bladder exstrophy patients with published normative GFR estimates. PATIENTS AND METHODS: eGFR was calculated using the Schwartz formula at three timepoints, with mean eGFR at each timepoint compared to normative values. RESULTS: At primary closure (n = 53) the mean eGFR (ml/min/1.73 m(2)) in exstrophy patients was similar to norms at 0-7 days (exstrophy vs norm: 42.5 vs 40.6, p > 0.05) and after 2 years of age (108.8 vs 133, p > 0.05). However, the mean eGFR in exstrophy patients was significantly lower than norms between 8 days (44.8 vs 65.8, p < 0.0001) and 2 years of life (68 vs 95.7, p = 0.01). At bladder neck reconstruction (n = 13) no statistically significant difference existed between the exstrophy and normative eGFR values (137.1 vs 133, p > 0.05). Similarly, among 27 patients with at least 1 year follow-up after bladder neck reconstruction, the mean exstrophy eGFR was no worse or higher than normative values (2-12 years: 124.5 vs 133, p > 0.05; males ≥13 years 175.6 vs 140, p = 0.04; females ≥13 years 128.8 vs 126, p > 0.05). CONCLUSION: The staged reconstruction of exstrophy does not appear to negatively impact renal function in most patients. As eGFR detects only significant changes, surgical reconstruction may still cause more subtle renal damage.

Using a serosal trough for fashioning a continent catheterizable stoma: technique and outcomes.

UNLABELLED: WHAT'S KNOWN ON THE SUBJECT? AND WHAT DOES THE STUDY ADD?: Continent urinary diversion with bladder augmentation is an established method of providing urinary continence in children with bladder exstrophy, who are not suitable candidates or have a failed bladder neck reconstruction. Sub-mucosal implantation of the tubularized catheterizable stoma (usually the appendix) into the reservoir, with backing typically provided by either the bladder musculature or colonic taenia, is safe and highly effective in these children. In some cases of classic bladder exstrophy and in the majority of patients with cloacal exstrophy, the ileum is used for enterocystoplasty and therefore there is no taenia to back the implanted catheterizable channel. This study describes the steps for providing a reliable flap-valve mechanism for the continent catheterizable channel using the serosal trough technique. OBJECTIVES: To evaluate the efficacy and potential complications of the serosal-trough (ST) technique for the implantation of a continent catheterizable stoma (CCS) during enterocystoplasty. To describe the surgical technique and provide detailed illustrations. PATIENTS AND METHODS: Using an institutional review board-approved departmental database, children with bladder exstrophy, born after 1990, were selected, and patients who had undergone urinary diversion with a CCS created using the ST technique were identified. Demographic and technical characteristics, as well as the eventual clinical outcomes, were retrospectively reviewed. RESULTS: A total of 135 patients with urinary diversion were identified, of whom 26 (13 males) had undergone CCS implantation using the ST technique. Patients included 14 classic exstrophies, 10 cloacal exstrophies, and two epispadias. The appendix and tapered ileum were used for the creation of a CCS in 11 and 15 patients, respectively. The median (range) age at creation of a CCS was 10.7 (4.4-17.4) years. At the time of CCS creation, 21 patients underwent initial enterocystoplasty, four had repeat augmentations, and one had a CCS on a previously augmented bladder. Ileum (mean length 18 cm) was used in 24/25 augmentations and was selected owing to lack of redundant sigmoid in 52% of patients and intraoperative surgeon preference in the remaining cases. In one case of cloacal exstrophy, a hindgut remnant was used. In 24 (92%) cases, initial CCS resulted in complete continence of the catheterizable channel. After a median (range) of 2.5 (0.2-7.5) years' follow-up all patients were dry via intermittent catheterization. The CCS failed at postoperative months 6 and 21 and required complete revision in two cases. CONCLUSIONS: Using a ST to provide a strong backing for a catheterizable channel is an excellent option when a channel must be placed in the ileum, hindgut, or in an area of augmentation where muscular backing is not available. The ST technique provides a reliably catheterizable tunnel, durable continence mechanism and a good success rate when creating a CCS in combination with a urinary diversion.

Is pelvic osteotomy associated with lower risk of pelvic organ prolapse in postpubertal females with classic bladder exstrophy?

PURPOSE: Bladder exstrophy is a major congenital anomaly involving defects in the genitourinary tract and pelvic musculoskeletal system. It appears intuitive that closure of the pelvic ring using osteotomy would be associated with a decreased risk of pelvic organ prolapse. We investigated whether osteotomy is associated with a decreased risk of pelvic organ prolapse in females with classic bladder exstrophy. MATERIALS AND METHODS: We searched our institutional review board approved exstrophy database of 1,078 patients and identified 335 females. We excluded patients who were younger than 13 years, had cloacal exstrophy or epispadias and did not have postpubertal imaging for measurement of pubic diastasis available. Our final study population consisted of 67 females. Univariate analysis was performed using t test or rank sum test for continuous variables and chi-square test for categorical variables. Logistic regression was used for multivariate analysis. RESULTS: Median patient age was 23 years (range 13 to 60). A total of 20 patients (29.9%) had pelvic organ prolapse at a median age of 20 years (range 11 to 43). Of the 67 patients 25 (37.3%) had undergone osteotomy at a median age of 6 months (range birth to 10 years). Seven patients had at least 1 pregnancy (range 1 to 3), and 24 patients had undergone vaginoplasty. On univariate analysis only diastasis was associated with pelvic organ prolapse, with smaller diastasis associated with a decreased risk of prolapse. On multivariate analysis including diastasis and osteotomy only diastasis was statistically significant. CONCLUSIONS: Osteotomy does not decrease the risk of pelvic organ prolapse in patients with classic bladder exstrophy. Rather, degree of diastasis is significantly associated with pelvic organ prolapse.

Delayed primary repair of bladder exstrophy: ultimate effect on growth.

PURPOSE: Late referrals or unsuitable bladder templates often require delayed primary repair of bladder exstrophy. We investigated longitudinal bladder growth rates and eventual outcomes following this approach. MATERIALS AND METHODS: After institutional review board approval, we reviewed the medical records of patients with classic bladder exstrophy who underwent neonatal or delayed (more than 30 days) primary closure at our institution between 1970 and 2006. Clinical characteristics and annual cystographic bladder capacity before the continence procedure were compared. Failed primary exstrophy repairs were excluded. RESULTS: A total of 33 patients with available bladder capacity measurements underwent delayed exstrophy closure due to small bladder template in 18 (88% male) and late referral in 15 (80% male) at respective median ages of 305 days (range 86 to 981) and 172 days (31 to 676). They were compared to 82 patients (71% male) undergoing neonatal closure at a median of 2 days of life (range 0 to 27). Pelvic osteotomy was performed in 32 of 33 delayed closures. Longitudinal analysis of the bladder capacities demonstrated that, compared to neonatally closed cases, bladder capacities were on average 36 ml smaller in those with delayed repair due to small templates (p = 0.01) and 29 ml smaller in those with late referrals (p = 0.13). However, the rate of bladder growth did not differ significantly among the 3 groups. CONCLUSIONS: Delayed primary repair of exstrophy does not compromise the rate of bladder growth. However, children born with smaller templates will have overall smaller capacities and are less likely to undergo bladder neck reconstruction.

The modern staged repair of classic bladder exstrophy: a detailed postoperative management strategy for primary bladder closure.

PURPOSE: Successful primary bladder closure of classic bladder exstrophy sets the stage for development of adequate bladder capacity and eventual voided continence. The postoperative pathway following primary bladder closure at the authors' institution is quantitatively and qualitatively detailed. MATERIALS AND METHODS: Sixty-five consecutive newborns (47 male) undergoing primary closure of classic bladder exstrophy were identified and data were extracted relating to immediate postoperative care. Overall success rate was utilized to validate the pathway. RESULTS: Mean age at time of primary closure was 4.6 days and mean hospital stay was 35.8 days. Osteotomy was performed in 19 patients (mean age 8.8 days), and was not required in 39 infants (mean age 2.9 days). All patients were immobilized for 4 weeks. Tunneled epidural analgesia was employed in 61/65 patients. All patients had ureteral catheters and a suprapubic tube, along with a comprehensive antibiotic regimen. Postoperative total parenteral nutrition was commonly administered, and enteral feedings started around day 4.6. Our success rate of primary closure was 95.4%. CONCLUSIONS: A detailed and regimented plan for bladder drainage, immobilization, pain control, nutrition, antimicrobial prophylaxis, and adequate healing time is a cornerstone for the postoperative management of the primary closure of bladder exstrophy.

Congenital renal anomalies in patients with classic bladder exstrophy.

OBJECTIVE: To determine the proportion and type of renal anomalies that occur in children born with classic bladder exstrophy. MATERIAL AND METHODS: All patients in a database of 1044 patients with the bladder exstrophy-epispadias-cloacal exstrophy complex were reviewed. Those with classic bladder exstrophy had their complete medical records reviewed and children with radiographic evidence of congenital renal anomalies were identified and characterized. RESULTS: A total of 674 patients were identified with classic bladder exstrophy, of whom 462 had renal ultrasounds at the authors' institution. In this population, 13/462 (2.8%) had concomitant renal anomalies. The most common malformation was a duplicated collecting system in 6 patients. Hypoplastic or absent kidneys were present in 3 patients, pelvic kidney in 2, ureteropelvic junction obstruction in 1, and multicystic dysplastic kidney in 1 patient. CONCLUSION: Isolated renal anomalies occur at a rate of 2.8% in children born with classic bladder exstrophy. Although uncommon in this rare birth defect, anatomic understanding of the entire genitourinary system is critical for operative planning and long-term follow-up of patients with bladder exstrophy.

Colorectal anomalies in patients with classic bladder exstrophy.

AIM: This study aims to determine the proportion and type of colorectal anomalies that occur in children born with classic bladder exstrophy (CBE). METHODS: All patients in a database of 1044 patients with the bladder exstrophy-epispadias-cloacal exstrophy complex were reviewed. Those with CBE had their complete medical records reviewed. Children noted to have gastrointestinal malformations were identified, and all aspects of their history were extracted. RESULTS: A total of 676 patients were identified with CBE, of whom 12 patients were identified who had a concomitant colorectal anomaly. In this population, the proportion of colorectal anomalies is 1.8%. The most common gastrointestinal anomaly was imperforate anus in 8 patients. Two patients had severe rectal stenosis requiring serial dilations, and 2 patients were born with congenital rectal prolapse. CONCLUSIONS: Excluding all variants and cloacal patients with exstrophy, isolated colorectal anomalies occur at a rate of 1.8% in children born with CBE, a 72-fold increase compared with the general population. Although uncommon in this rare birth defect, the morbidity is significant and warrants prompt evaluation and treatment of both the genitourinary and colorectal anomalies.