Relationship between the size of the bladder template and the subsequent bladder capacity in bladder exstrophy (original) (raw)
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American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 2011
Bladder exstrophy (BE) is a complex congenital anomaly characterized by a defect in the closure of the lower abdominal wall and bladder. We aimed to provide an overview of the literature and conduct an epidemiologic study to describe the prevalence, and maternal and case characteristics of BE. We used data from 22 participating member programs of the International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR). All cases were reviewed and classified as isolated, syndrome, and multiple congenital anomalies. We estimated the total prevalence of BE and calculated the frequency and odds ratios for various maternal and case characteristics. A total of 546 cases with BE were identified among 26,355,094 births. The total prevalence of BE was 2.07 per 100,000 births (95% CI: 1.90-2.25) and varied between 0.52 and 4.63 among surveillance programs participating in the study. BE was nearly twice as common among male as among female cases. The proportion of isolated cases was 71%. Prevalence appeared to increase with increasing categories of maternal age, particularly
Journal of Pediatric Surgery, 1991
Bladder capacity was measured at micturating cystourethrography and normal ranges were established for children up to 1 year of age. Bladder capacity was compared with patient weight and distance from first lumbar to third lumbar vertebrae (L, to L,). The simplified formula-Capacity (mL) = 7 x weight (kg)-was shown to give a reliable estimate of the expected bladder capacity in infants independent of age; this is useful in those infants whose weight lies outside the normal range for their age. Similarly, a formula was deduced relating expected bladder capacity to the measured L, to L, distance on an anteroposterior radiograph, which is of potential use to radiologists.
The Journal of Urology, 2001
Purpose: The surgical repair of bladder exstrophy remains challenging for the pediatric urologist. We present our preliminary experience with complete primary repair of exstrophy in neonates and children with failed initial closure. Materials and Methods: Between November 1998 and April 1999, 9 boys and 2 girls with bladder exstrophy underwent complete repair. This procedure was performed in the first 72 hours of life in 4 boys and at age 3 months in 1 girl. Complete repair with osteotomy was performed after failed initial closure in 5 boys and 1 girl at a mean age of 28 months (range 15 to 36). The bladder and urethra were closed in continuity and epispadias was repaired by total penile disassembly. All patients were kept in a spica cast for 3 weeks. Ureteral stents and suprapubic tube were removed 10 and 14 days, respectively, after surgery. Ultrasound was performed preoperatively and every 3 months postoperatively, voiding cystourethrography was done 6 to 12 months after surgery.
Neurourology and Urodynamics, 2014
Aims: To understand the function development of bladder and its evaluation in neonates and infants less than 2 years old. Methods: Literature on neonatal and infant bladder function development and urodynamic evaluation were collected and reviewed. Results: Normal range of bladder volume, pressure during voiding and other parameters in neonates and infants less than 2 years old is far from set up, making interpretation of UDS findings difficult. This review provides insight into the bladder development process and problems of the lower urinary tract in this age group with special emphasis on the urodynamic evaluation. Conclusions: Further animal and human studies will increase our understanding of bladder development leading toward mature function. UDS are still important in providing information for early bladder dysfunction in newborns and infants. Neurourol. Urodynam.
Journal of Urology, 2014
Purpose-Substantial variability exists in bladder exstrophy (BE) care, and little is known about costs associated with BE. We aim to define the care patterns and first-year cost for BE patients at select free-standing children's hospitals in the United States. Materials and Methods-The Pediatric Health Information System (PHIS) database was used to identify BE patients born between 1/99 and 12/10 whose primary closure occurred in the first 120 days of life (DOL). Demographic, surgical, postoperative, and cost data for all encounters were assessed. A multivariable linear regression was used to examine the association between patient, surgeon, and hospital characteristics and costs. Results-Of the 381 patients who underwent primary closure within the first 120 DOL, 279 (73%) had this done within 3 DOL. 119 (31%) patients received pelvic osteotomies, including 51/279 (18%) of those closed within DOL 3, 38/67 (56%) of those closed between DOL 4-30 and 30/35 (86%) of those closed between DOL 31-120 (p=0.0017). The median inflationadjusted firstyear cost (US$) per patient was $66,577 [IQR: 45,335-102,398]. The presence of non-renal comorbidity and primary closure after 30 DOL were associated with 24% and 53% increased firstyear costs, respectively. Increasing post-closure length of stay (LOS) was associated with increased costs. Conclusions-At select freestanding U.S. children's hospitals, the majority of bladder closures occur within the first 3 DOL. Most, but not all, patients closed after the neonatal period underwent osteotomy. The presence of non-renal comorbidity and increasing postoperative LOS were associated with increased costs.
Primary Bladder Exstrophy Closure in Neonates: Challenging the Traditions
The Journal of Urology, 2014
Purpose: We describe a novel approach to neonatal bladder exstrophy closure that challenges the role of postoperative immobilization and pelvic osteotomy. Materials and Methods: We reviewed the primary management of bladder exstrophy at our institutions between 2007 and 2011. In particular we compared postoperative management in the surgical ward using epidural analgesia to muscle paralysis and ventilation in the intensive care unit. Clinical outcome measures were time to full feed, length of stay, postoperative complications and redo closure. Cost-effectiveness was also evaluated using hospital financial data. Data are expressed as median (range). Significance was explored by Fisher exact test and unpaired t-test. Results: A total of 74 patients underwent primary closure without osteotomy. Successful closure was achieved in 70 patients (95%). A total of 48 cases (65%) were managed on the ward (group A) and 26 (35%) were transferred to the intensive care unit (group B). The 2 groups were homogeneous for gestational age (median 39 weeks, range 27 to 41) and age at closure (3 days, 1 to 152). Complications requiring surgical treatment were noted in 4 patients (8.3%) in group A and 3 (11.5%) in group B (p ¼ 0.609). Length of stay was significantly shorter for the group managed on the ward (11 vs 18 days, p <0.0001). Median costs were 42,732forpatientsadmittedtotheintensivecareunitand42,732 for patients admitted to the intensive care unit and 42,732forpatientsadmittedtotheintensivecareunitand16,214 for those admitted directly to the surgical ward (p <0.0001). Conclusions: Primary closure of bladder exstrophy without lower limb immobilization and osteotomy is feasible. Postoperative care on the surgical ward using epidural analgesia results in shorter hospitalization. Abbreviations and Acronyms BE ¼ bladder exstrophy CBEX ¼ classic bladder exstrophy ICU ¼ intensive care unit
Are we accurately predicting bladder capacity in infants?
Canadian Urological Association Journal, 2014
Introduction: Estimating bladder capacity is an important component in the evaluation of many urological disorders. For estimates to be of clinical value, precise reference ranges are needed. While accepted reference ranges have been established in adults and older children, none have been validated in infants. We endeavour to determine the normal bladder capacity of children less than 1 year of age.Methods: We retrospectively reviewed the charts of children aged 0 to 12 months with cutaneous stigmata of spinal dysraphism who were referred to the urology clinic to rule out tethered cord between October 2004 and July 2011. Patients with normal urologic assessment, who did not have surgery during the time they were followed, were included in the study cohort. Urodynamic studies were performed using the Laborie Medical Technologies UDS-600. Bladder filling occurred via a catheter at a rate of 10% of the expected total bladder capacity/minute. Bladder capacity was defined as the volume ...
Some new insights into bladder function in infancy
British Journal of Urology, 1995
Objectives To evaluate normal bladder function and micturition patterns in infants. Patients, subjects and methods Twenty-one infants (16 boys, five girls: mean age 5.9 months) with no lower urinary tract pathology underwent natural filling cystometry. Micturition patterns were also observed simultaneously with polysomnography in 26 healthy neonates (16 boys, 10 girls: mean age 7.4 days). Results In infants, cystometry showed (95% CI) a capacity of 42-53 mL, a maximum rise in detrusor pressure during voiding of 95-120 cmH,O and a voiding efficiency (voided volume/capacity) of 0.86-0.91. On micturition, urinary flow was discoordinated from peak detrusor pressures in 10 infants. Detrusor instability occurred in one of 21 infants. Micturition was observed only during wakefulness or
Results of Surgical Treatment in Children with Bladder Exstrophy
British Journal of Urology, 1992
Methods We performed a retrospective analysis of 11 patients diagnosed with CM-1 who were treated in our center in the years 2007 to 2016. There were 6 female and 5 male individuals. Short-term evaluation of the outcome was based on comparison of the presenting symptoms and radiological images before and after the surgical treatment. Long-term follow-up was carried out using survey questionnaires based on the Chicago Chiari Outcome Scale (CCOS) devised originally by Aliaga et al. Results Patients, based on their CCOS score were divided into three groups marked as Bimproved,^Bunchanged,^and Bworse,d epending on a range of CCOS score: 13-16, 9-12, 4-8, respectively. The outcome of patients was as follows: 6 patients (55%) were evaluated as improved, and 5 (45%) as unchanged. No patient was classified as worse after surgery. Significant negative Spearman's correlation was found between the CCOS score and patients' age at the time of surgery (R = − 0.85, p = 0.0009). Conclusions The decision of whether to operate pediatric patients with CM-1 should be considered very carefully. In our department, the main indication for surgery was the occurrence of clinical symptoms. Our study revealed that in the symptomatic patients, surgery improves their quality of life measured with CCOS.