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更新于：2025-05-07

Bladder Exstrophy and Epispadias Complex

膀胱外翻和外翻复合体

更新于：2025-05-07

基本信息

别名

BEEC、BEEC - bladder exstrophy, epispadias, cloacal exstrophy complex、BLADDER EXSTROPHY AND EPISPADIAS COMPLEX

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简介

A spectrum of genitourinary malformations ranging in severity from epispadias and classical bladder exstrophy to exstrophy of the cloaca as the most severe form. Depending on severity, the disorder may involve the urinary system, the musculoskeletal system, the pelvis, the pelvic floor, the abdominal wall, the genitalia and sometimes the spine and the anus. The underlying cause remains unknown, rare cases of familial occurrence have been reported and both genetic and environmental factors are likely to play a role.

关联

项与膀胱外翻和外翻复合体相关的药物

Satori-01

靶点-

作用机制-

在研机构

MyoPax GmbH

原研机构

MyoPax GmbH

在研适应症

膀胱外翻和外翻复合体

非在研适应症-

最高研发阶段临床前

首次获批国家/地区-

首次获批日期1800-01-20

项与膀胱外翻和外翻复合体相关的临床试验

NCT03685955

/ WithdrawnN/AIIT

The Effect of Amniotic Membranes on Complex Genitourinary Reconstruction Outcomes in Pediatric and Adult Populations

Urinary fistulas are unfortunately one of the most common post operative complications in hypospadias repairs and bladder neck transections on pediatric and adult populations, an estimated 20%, resulting in multiple repeat operations with prolonged hospitalization. The negative sequelae of these fistulas have significant financial and psychosocial impact and the aim of this study is to evaluate if the fresh frozen preserved amniotic membrane tissue studied, which have live cells and intact naturally occurring growth hormones and peptides that augment healing that have been shown in early studies to be effective facilitating previous failed fistula repairs and leg ulcer healing, respectively, will help reduce the incidence of urinary fistulas in subject populations.

开始日期2020-03-01

申办/合作机构

The Johns Hopkins University

NCT01011777

/ Terminated临床1期IIT

Phase I Study of Endoscopically Injected Muscle Derived Cells in Participants With Exstrophy-epispadias Complex Related Urinary Incontinence

The aim of this study is to study safety, tolerability and efficacy of muscle derived cell (MDC) therapy in children with bladder exstrophy epispadias induced urinary incontinence.

开始日期2017-02-01

申办/合作机构

The Johns Hopkins University

100 项与膀胱外翻和外翻复合体相关的临床结果

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100 项与膀胱外翻和外翻复合体相关的转化医学

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0 项与膀胱外翻和外翻复合体相关的专利（医药）

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196

项与膀胱外翻和外翻复合体相关的文献（医药）

2025-04-01·Journal of Pediatric Orthopaedics

Subperiosteal Tunneled Allograft Reconstruction of the Symphyseal Ligaments (STARS) in Bladder Exstrophy Epispadias Complex

Article

作者: Kenawey, Mohamed ; Keene, David ; Mariotto, Ariana ; Cervellione, Raimondo ; Morakis, Emmanouil

Background:Because of the lack of symphyseal ligaments, pubic symphysis re-diastasis is the rule after iliac osteotomies for bladder exstrophy reconstruction. Progressive symphyseal diastasis may cause pelvic organ prolapse in females and penile retraction in males. This study assesses the results of tendon allograft symphyseal reconstruction for maintaining pubic approximation with iliac osteotomies in exstrophy repair.Methods:Eleven consecutive patients had symphyseal reconstruction with delayed exstrophy repair, 7 classic bladder exstrophy, 2 cloacal exstrophy, and 2 exstrophy variants. There were 4 males and the average age at surgery was 14 months (9 to 20 mo). The average preoperative diastasis was 52 mm (43 to 79 mm). After pubic approximation with modified oblique iliac osteotomies, tendon allografts were looped subperiosteally around the pubic bones and through the obturator foramina, and reinforced by suturing anterior chondro-periosteal flaps. With the legs in a mermaid dressing, external fixators were kept for 3 to 4 weeks. All patients had preoperative pelvic computed tomography scans and pelvic x-rays before fixator removal and at the latest follow-up.Results:All patients had successful urologic closure. The mean postoperative inter-pubic distance was 20.5 mm (8 to 29 mm). The mean postoperative follow-up was 10 months (4 to 19 mo). The mean inter-pubic distance at the latest follow-up was 21.6 mm (3 to 35 mm). We had maintained or even decreased inter-pubic distance in 9 out of 11 cases with the average postoperative interpubic distance of 19.3 mm compared with 18.9 mm at an average 11 months follow-up. In 2 patients, the inter-pubic distance increased from 25 and 26 mm postoperatively to 33 and 35 mm at 4 months follow-up.Conclusion:The interpubic distance was stable or decreasing in 9 out of 11 patients with average follow-up of 11 months. Further reduction in the interpubic distance was observed due to bone formation medial to the pubic bones caused by the subperiosteal dissection. Longer-term follow-up is required to confirm stable pubic approximation.Level of Evidence:Level III.

2025-04-01·Journal of Pediatric Urology

Bladder exstrophy in adulthood: A narrative review of transitional health

Review

作者: Weiss, Dana ; DiCarlo, Heather ; Hampson, Lindsay A ; Lopez, Alejandro D ; Shaw, Nathan M

BACKGROUNDExstrophy-epispadias complex (EEC) classically presents as bladder exstrophy (BE) and requires lifetime urologic care. As men and women with BE age, there is an often difficult period of transition to adulthood in terms of addressing urologic and general health challenges. BE can lead to many urinary and sexual health issues as these patients age, which is often made more complex given their past surgical history and anatomy.OBJECTIVEGiven the relative paucity of research involving adult BE patients, we aim to provide a review of evidence-based best practice management for these patients to guide treating urologists and identify gaps in knowledge.METHODOLOGYAll recommendations are based on peer-reviewed research from trusted academic search engines to the degree that relevant research is available. Where evidence is scant, we rely on expert opinion and comparisons with other congenital urologic conditions where relevant. We also offer common clinical situations that arise as men and women with BE and other conditions age into adulthood to better understand the management of this complex population.CONCLUSIONSAs patients with BE age, they experience a unique set of sexual, urinary, and general health challenges. Like other congenital urological conditions, long term care involving multidisciplinary teams at centers of excellence with a system for transition from pediatric to adult urology can lead to improved outcomes. Further research is needed regarding health outcomes in adults with BE as well as barriers limiting healthcare utilization.

2025-02-01·Journal of Pediatric Urology

Vaginoplasty in female bladder exstrophy–epispadias complex: Analysis of operative technique, outcomes, and complications

Article

作者: Gabrielson, Andrew T ; Crigger, Chad B ; Cheaib, Joseph ; Haffar, Ahmad ; Maxon, Victoria ; Galansky, Logan ; Gearhart, John P ; Di Carlo, Heather N ; Hirsch, Alex

INTRODUCTION AND OBJECTIVEBladder exstrophy-epispadias complex (BEEC) is a rare, but serious congenital malformation. After achieving urinary continence and upper tract preservation, genital reconstruction is performed for function and cosmesis. We evaluated our institutional experience with vaginoplasty, including technical considerations and post-operative outcomes.PATIENTS AND METHODSAn IRB-approved database was used to conduct an institutional retrospective review of BEEC patients undergoing vaginoplasty from 2000 to 2024. Patients with XY karyotype, primary vaginoplasty during BEEC closure or at another institution, or no urologic follow-up visits at least six months post-vaginoplasty were excluded. Data on age, reconstructive method, and suture type was collected. Post-operative complications were analyzed with the primary surgical outcome of vaginal stenosis requiring re-operation (VS) and secondary surgical outcomes including urinary tract infection (UTI), surgical site infection (SSI), dehiscence, rectal injury, fistula, and symptomatic pelvic organ prolapse (POP) after vaginoplasty.RESULTSA total of 240 female BEEC patients were identified with 54 (22.5 %) undergoing vaginoplasty. Median age at vaginoplasty was 15.6 years old. The majority of cases were perineal flap vaginoplasty (88.9 %). Nylon suture was most commonly used (46.3 %), with Vicryl and PDS used in 31.5 % and 22.2 % of cases, respectively. Median follow-up time was 55.2 months. Post-operative complication rates were VS 14.8 %, UTI 3.7 %, SSI 7.4 %, dehiscence 3.7 %, rectal injuries 0 %, fistula 0 %, and symptomatic POP 12.9 % (Table 1). The perioperative complication rate per-patient from post-operative day 1-90 was 20.4 % and the overall lifetime complication rate per-patient was 33.3 %. We found no significant association between reconstructive method and VS (p = 0.2). Among VS events, PDS was used in 50 % of cases (p = 0.041). On univariable analysis, PDS use was significantly associated with VS (OR 4.75, 95 % CI [1.02-23.1], p = 0.042). This finding remained significant when adjusting for reconstructive method on multivariable analysis (OR 5.83, 95 % CI [1.06-32.3], p = 0.043) (Table 2).CONCLUSIONSAs children with BEEC mature into adulthood, optimizing surgical outcomes for genital reconstruction is critical. In this large retrospective cohort of BEEC patients undergoing vaginoplasty, we observed that VS was the most common post-operative complication. Symptomatic POP was the second most common post-operative complication, but of these patients, the majority were managed conservatively. Use of PDS during vaginoplasty was significantly associated with VS, suggesting that other suture types may be more advantageous for successful surgical outcomes, but further investigation into the clinical significance of this finding is warranted.