Abstract：
UNASSIGNED: Bladder exstrophy (BE) poses challenges both during the surgical repair and throughout follow-up. In 2013, a multi-institutional BE consortium was initiated, which included utilization of unified surgical principles for the complete primary repair of exstrophy (CPRE), real-time coaching, ongoing video capture and review of video footage, prospective data collection, and routine patient data analysis, with the goal of optimizing the surgical procedure to minimize devastating complications such as glans ischemia and bladder dehiscence while maximizing the rate of volitional voiding with continence and long-term protection of the upper tracts. This study reports on our short-term complications and intermediate-term continence outcomes.
UNASSIGNED: A single prospective database for all patients undergoing surgery with a BE epispadias complex diagnosis at 3 institutions since February 2013 was used. For this study, data for children with a diagnosis of classic BE who underwent primary CPRE from February 2013 to February 2021 were collected. Data recorded included sex, age at CPRE, adjunct surgeries including ureteral reimplantations and hernia repairs at the time of CPRE, osteotomies, and immobilization techniques, and subsequent surgeries. Data on short-term postoperative outcomes, defined as those occurring within the first 90 days after surgery, were abstracted. In addition, intermediate-term outcomes were obtained for patients operated on between February 2013 and February 2017 to maintain a minimum follow-up of 4 years. Outcomes included upper tract dilation on renal and bladder ultrasound, presence of vesicoureteral reflux, cortical defects on nuclear scintigraphy, and continence status. Bladder emptying was assessed with respect to spontaneous voiding ability, need for clean intermittent catheterization, and duration of dry intervals. All operating room encounters that occurred subsequent to initial CPRE were recorded.
UNASSIGNED: CPRE was performed in 92 classic BE patients in the first 8 years of the collaboration (62 boys), including 46 (29 boys) during the first 4 years. In the complete cohort, the median (interquartile range) age at CPRE was 79 (50.3) days. Bilateral iliac osteotomies were performed in 89 (97%) patients (42 anterior and 47 posterior). Of those undergoing osteotomies 84 were immobilized in a spica cast (including the 3 patients who did not have an osteotomy), 6 in modified Bryant\'s traction, and 2 in external fixation with Buck\'s traction. Sixteen (17%) patients underwent bilateral ureteral reimplantations at the time of CPRE. Nineteen (21%) underwent hernia repair at the time of CPRE, 6 of which were associated with orchiopexy. Short-term complications within 90 days occurred in 31 (34%), and there were 13 subsequent surgeries within the first 90 days. Intermediate-term outcomes were available for 40 of the 46 patients, who have between 4 and 8 years of follow-up, at a median of 5.7 year old. Thirty-three patients void volitionally, with variable dry intervals.
UNASSIGNED: Cumulative efforts of prospective data collection have provided granular data for evaluation. Short-term outcomes demonstrate no devastating complications, that is, penile injury or bladder dehiscence, but there were other significant complications requiring further surgeries. Intermediate-term data show that boys in particular show encouraging spontaneous voiding and continence status post CPRE, while girls have required modification of the surgical technique over time to address concerns with urinary retention. Overall, 40% of children with at least 4 years of follow-up are voiding with dry intervals of > 1 hour.
UNASSIGNED: A single prospective database for all patients undergoing surgery with a BE epispadias complex diagnosis at 3 institutions since February 2013 was used. For this study, data for children with a diagnosis of classic BE who underwent primary CPRE from February 2013 to February 2021 were collected. Data recorded included sex, age at CPRE, adjunct surgeries including ureteral reimplantations and hernia repairs at the time of CPRE, osteotomies, and immobilization techniques, and subsequent surgeries. Data on short-term postoperative outcomes, defined as those occurring within the first 90 days after surgery, were abstracted. In addition, intermediate-term outcomes were obtained for patients operated on between February 2013 and February 2017 to maintain a minimum follow-up of 4 years. Outcomes included upper tract dilation on renal and bladder ultrasound, presence of vesicoureteral reflux, cortical defects on nuclear scintigraphy, and continence status. Bladder emptying was assessed with respect to spontaneous voiding ability, need for clean intermittent catheterization, and duration of dry intervals. All operating room encounters that occurred subsequent to initial CPRE were recorded.
UNASSIGNED: CPRE was performed in 92 classic BE patients in the first 8 years of the collaboration (62 boys), including 46 (29 boys) during the first 4 years. In the complete cohort, the median (interquartile range) age at CPRE was 79 (50.3) days. Bilateral iliac osteotomies were performed in 89 (97%) patients (42 anterior and 47 posterior). Of those undergoing osteotomies 84 were immobilized in a spica cast (including the 3 patients who did not have an osteotomy), 6 in modified Bryant\'s traction, and 2 in external fixation with Buck\'s traction. Sixteen (17%) patients underwent bilateral ureteral reimplantations at the time of CPRE. Nineteen (21%) underwent hernia repair at the time of CPRE, 6 of which were associated with orchiopexy. Short-term complications within 90 days occurred in 31 (34%), and there were 13 subsequent surgeries within the first 90 days. Intermediate-term outcomes were available for 40 of the 46 patients, who have between 4 and 8 years of follow-up, at a median of 5.7 year old. Thirty-three patients void volitionally, with variable dry intervals.
UNASSIGNED: Cumulative efforts of prospective data collection have provided granular data for evaluation. Short-term outcomes demonstrate no devastating complications, that is, penile injury or bladder dehiscence, but there were other significant complications requiring further surgeries. Intermediate-term data show that boys in particular show encouraging spontaneous voiding and continence status post CPRE, while girls have required modification of the surgical technique over time to address concerns with urinary retention. Overall, 40% of children with at least 4 years of follow-up are voiding with dry intervals of > 1 hour.
摘要：
■膀胱外翻（BE）在手术修复期间和整个随访期间都面临挑战。2013年，启动了一个多机构的BE联盟，其中包括使用统一的手术原则进行完整的一期外翻修复（CPRE），实时辅导,正在进行的视频捕获和视频片段的审查，前瞻性数据收集，和常规的患者数据分析，目的是优化外科手术程序，以最大程度地减少破坏性并发症，例如龟头缺血和膀胱裂开，同时最大程度地提高自愿排尿率，并对上消化道进行长期保护。这项研究报告了我们的短期并发症和中期失禁结果。
■使用了自2013年2月以来在三个机构进行膀胱外翻外翻复杂诊断的所有手术患者的单一前瞻性数据库。对于这项研究，收集了2013年2月至2021年2月接受原发性CPRE的经典BE诊断儿童的数据.记录的数据包括性别，CPRE的年龄，辅助手术,包括在CPRE时进行输尿管再植入和疝修补术，截骨和固定技术，以及随后的手术。短期术后结果的数据，定义为在手术后的前90天内发生的事件，是抽象的。此外,在2013年2月至2017年2月期间接受手术的患者获得了中期结局,以维持最少4年的随访时间.结果包括肾脏和膀胱超声（RBUS）的上尿路扩张，膀胱输尿管反流（VUR）的存在，核闪烁显像（DMSA）上的皮质缺陷，和失禁状态。膀胱排空根据自发性排尿能力进行评估，需要清洁间歇导管插入术（CIC），和干燥间隔的持续时间。记录了最初CPRE之后发生的所有手术室遭遇。
■在合作的前8年中，对92名经典BE患者进行了CPRE（62名男孩），前4年包括46名（29名男孩）。在完整的队列中，CPRE时的中位年龄(IQR)为79(50.3)天.89例（97％）患者（前42例，后47例）进行了双侧髂截骨术。在接受截骨术的患者中，84人固定在脊柱石膏中（包括3人没有截骨术），6在修改后的科比的牵引力，和2在带雄鹿牵引的外部固定中）。16例（17％）患者在CPRE时接受了双侧输尿管重植。19例（21%）在CPRE时接受了疝修补术，其中6例与睾丸固定术有关。90天内出现短期并发症31例（34%），在最初的90天内有13次手术。46例随访4至8年的患者中有40例获得了中期结局，中位数为5.7岁。33名患者自愿无效，具有可变的干燥间隔，在汇总表中演示。
■前瞻性数据收集的累积努力为评估提供了细粒度数据。短期结果显示没有毁灭性的并发症，也就是说,阴茎损伤或膀胱裂开，但还有其他重大并发症需要进一步手术.中期数据显示，特别是男孩在CPRE后表现出令人鼓舞的自发性排尿和失禁状态，而随着时间的推移，女孩需要修改手术技术，以解决尿潴留的问题。总的来说,至少4年随访的儿童中有40％排尿，干燥间隔>1小时。
■使用了自2013年2月以来在三个机构进行膀胱外翻外翻复杂诊断的所有手术患者的单一前瞻性数据库。对于这项研究，收集了2013年2月至2021年2月接受原发性CPRE的经典BE诊断儿童的数据.记录的数据包括性别，CPRE的年龄，辅助手术,包括在CPRE时进行输尿管再植入和疝修补术，截骨和固定技术，以及随后的手术。短期术后结果的数据，定义为在手术后的前90天内发生的事件，是抽象的。此外,在2013年2月至2017年2月期间接受手术的患者获得了中期结局,以维持最少4年的随访时间.结果包括肾脏和膀胱超声（RBUS）的上尿路扩张，膀胱输尿管反流（VUR）的存在，核闪烁显像（DMSA）上的皮质缺陷，和失禁状态。膀胱排空根据自发性排尿能力进行评估，需要清洁间歇导管插入术（CIC），和干燥间隔的持续时间。记录了最初CPRE之后发生的所有手术室遭遇。
■在合作的前8年中，对92名经典BE患者进行了CPRE（62名男孩），前4年包括46名（29名男孩）。在完整的队列中，CPRE时的中位年龄(IQR)为79(50.3)天.89例（97％）患者（前42例，后47例）进行了双侧髂截骨术。在接受截骨术的患者中，84人固定在脊柱石膏中（包括3人没有截骨术），6在修改后的科比的牵引力，和2在带雄鹿牵引的外部固定中）。16例（17％）患者在CPRE时接受了双侧输尿管重植。19例（21%）在CPRE时接受了疝修补术，其中6例与睾丸固定术有关。90天内出现短期并发症31例（34%），在最初的90天内有13次手术。46例随访4至8年的患者中有40例获得了中期结局，中位数为5.7岁。33名患者自愿无效，具有可变的干燥间隔，在汇总表中演示。
■前瞻性数据收集的累积努力为评估提供了细粒度数据。短期结果显示没有毁灭性的并发症，也就是说,阴茎损伤或膀胱裂开，但还有其他重大并发症需要进一步手术.中期数据显示，特别是男孩在CPRE后表现出令人鼓舞的自发性排尿和失禁状态，而随着时间的推移，女孩需要修改手术技术，以解决尿潴留的问题。总的来说,至少4年随访的儿童中有40％排尿，干燥间隔>1小时。
