Abstract：
BACKGROUND: Epispadias, which occurs on the more mild end of the Bladder Exstrophy Epispadias Complex (BEEC) spectrum, presents still with a wide range of severity in boys, from mild glanular epispadias to penopubic epispadias with severe urethral and bladder neck defects. Surgical management ranges from isolated epispadias repair to epispadias repair with bladder neck reconstruction (BNR) with or without pelvic osteotomies.
OBJECTIVE: We aimed to evaluate outcomes in epispadias treated at three institutions prior to formation of a formal collaboration. In addition, we sought to delineate outcomes based on anatomic severity at time of diagnosis, and initial procedure performed in cases of penopubic epispadias.
METHODS: IRB approved databases were retrospectively queried at three institutions for patients who underwent repair of epispadias between 1/1993 and 1/2013. Degree of epispadias, age and technique at initial repair, and self-reported continence status at last follow-up were recorded. Continence was categorized as: wet, intermediate (dry 2-3 h), or dry, while also distinguishing those who void and those who require clean intermittent catheterization (CIC). Those not seen since 1/1/2015, younger than 10 years at last follow up, or in whom continence data were not recorded were excluded.
RESULTS: A total of 48 boys were identified; 36 met inclusion criteria. The epispadias cohort consisted of 8 glanular epispadias (GE) (22%); 8 penile epispadias (PE) (22%), and 20 penopubic epispadias (PPE) (56%) with a median follow-up of 11.3 years (3.2-26.2 years). Overall, 33 of 36 (92%) boys void per urethra. Within the group that voids, 19/33 (58%) are completely dry, while 6/33 (18%) are wet. Among patients who underwent initial epispadias repair without concurrent or subsequent bladder neck reconstruction, continence rates were: GE 63% (5/8); PE 75% (6/8); PPE 71% (5/7). Among the 9 boys with PPE who underwent initial epispadias repair with concurrent BNR, 22% (2/9) were dry with no further surgeries. Overall, 8/20 (40%) of boys with PPE void with complete dryness.
CONCLUSIONS: This multi-center retrospective review of continence in epispadias demonstrates that even some boys with glanular and penile epispadias can have challenges with continence, and boys with penopubic epispadias may remain wet despite careful preoperative assessment of bladder neck functionality and concurrent BNR.
CONCLUSIONS: Continence outcomes in boys with all degrees of epispadias can be variable. Even boys with more distal defects may have significant bladder neck deficiency. And those with the most severe form of epispadias may require bladder neck reconstruction to achieve continence.
OBJECTIVE: We aimed to evaluate outcomes in epispadias treated at three institutions prior to formation of a formal collaboration. In addition, we sought to delineate outcomes based on anatomic severity at time of diagnosis, and initial procedure performed in cases of penopubic epispadias.
METHODS: IRB approved databases were retrospectively queried at three institutions for patients who underwent repair of epispadias between 1/1993 and 1/2013. Degree of epispadias, age and technique at initial repair, and self-reported continence status at last follow-up were recorded. Continence was categorized as: wet, intermediate (dry 2-3 h), or dry, while also distinguishing those who void and those who require clean intermittent catheterization (CIC). Those not seen since 1/1/2015, younger than 10 years at last follow up, or in whom continence data were not recorded were excluded.
RESULTS: A total of 48 boys were identified; 36 met inclusion criteria. The epispadias cohort consisted of 8 glanular epispadias (GE) (22%); 8 penile epispadias (PE) (22%), and 20 penopubic epispadias (PPE) (56%) with a median follow-up of 11.3 years (3.2-26.2 years). Overall, 33 of 36 (92%) boys void per urethra. Within the group that voids, 19/33 (58%) are completely dry, while 6/33 (18%) are wet. Among patients who underwent initial epispadias repair without concurrent or subsequent bladder neck reconstruction, continence rates were: GE 63% (5/8); PE 75% (6/8); PPE 71% (5/7). Among the 9 boys with PPE who underwent initial epispadias repair with concurrent BNR, 22% (2/9) were dry with no further surgeries. Overall, 8/20 (40%) of boys with PPE void with complete dryness.
CONCLUSIONS: This multi-center retrospective review of continence in epispadias demonstrates that even some boys with glanular and penile epispadias can have challenges with continence, and boys with penopubic epispadias may remain wet despite careful preoperative assessment of bladder neck functionality and concurrent BNR.
CONCLUSIONS: Continence outcomes in boys with all degrees of epispadias can be variable. Even boys with more distal defects may have significant bladder neck deficiency. And those with the most severe form of epispadias may require bladder neck reconstruction to achieve continence.
摘要：
背景：Epispadias，发生在膀胱exstrophyEpispadias复合物（BEEC）光谱的较温和末端，在男孩中仍然表现出广泛的严重性，从轻度的腺外上下裂到有严重尿道和膀胱颈缺损的耻骨外上裂。手术治疗范围从孤立的上裂修复到有或没有骨盆截骨术的膀胱颈重建（BNR）的上裂修复。
目的：我们的目的是在正式合作之前评估在三个机构治疗的外裂的结果。此外,我们试图根据诊断时的解剖严重程度来描述结局，以及在耻骨外裂病例中进行的初始手术。
方法：在1993年1月至2013年1月期间，在三个机构中回顾性查询了IRB批准的数据库，以获取接受外裂修复的患者。癫痫程度,初始修复时的年龄和技术，并记录上次随访时自我报告的尿失禁状况.Continence被归类为：湿，中间（干2-3小时），或干燥，同时还区分那些无效的人和那些需要清洁间歇性导管插入术（CIC）的人。自2015年1月1日以来从未见过的，在最后一次随访中小于10岁，或未记录尿失禁数据的患者被排除.
结果：共确定了48名男孩；36名符合纳入标准。外联症队列包括8个腺体外联症（GE）（22％）；8个阴茎外联症（PE）（22％），和20例耻骨外外裂（PPE）（56％），中位随访时间为11.3年（3.2-26.2年）。总的来说,36个（92％）男孩中的33个尿道空隙。在空缺的群体中，19/33（58%）完全干燥，而6/33（18％）是湿的。在接受初次外裂修复而未同时或随后进行膀胱颈重建的患者中，尿失禁发生率为:GE63%(5/8);PE75%(6/8);PPE71%(5/7)。在9名患有PPE的男孩中，他们同时接受了BNR的初始外阴修复，22%(2/9)干燥,没有进一步手术。总的来说,8/20(40%)的男生PPE虚空伴完全干燥。
结论：此多中心回顾性研究表明，即使是一些患有腺性和阴茎性外阴的男孩，也可能面临尿失禁的挑战。尽管术前仔细评估了膀胱颈功能并同时进行了BNR，但患有耻骨外的男孩仍可能保持湿润。
结论：患有各种上下裂程度的男孩的延续性结果可能是可变的。即使有更多远端缺陷的男孩也可能有明显的膀胱颈缺陷。而那些患有最严重形式的尿道下裂的人可能需要膀胱颈重建才能实现节制。
目的：我们的目的是在正式合作之前评估在三个机构治疗的外裂的结果。此外,我们试图根据诊断时的解剖严重程度来描述结局，以及在耻骨外裂病例中进行的初始手术。
方法：在1993年1月至2013年1月期间，在三个机构中回顾性查询了IRB批准的数据库，以获取接受外裂修复的患者。癫痫程度,初始修复时的年龄和技术，并记录上次随访时自我报告的尿失禁状况.Continence被归类为：湿，中间（干2-3小时），或干燥，同时还区分那些无效的人和那些需要清洁间歇性导管插入术（CIC）的人。自2015年1月1日以来从未见过的，在最后一次随访中小于10岁，或未记录尿失禁数据的患者被排除.
结果：共确定了48名男孩；36名符合纳入标准。外联症队列包括8个腺体外联症（GE）（22％）；8个阴茎外联症（PE）（22％），和20例耻骨外外裂（PPE）（56％），中位随访时间为11.3年（3.2-26.2年）。总的来说,36个（92％）男孩中的33个尿道空隙。在空缺的群体中，19/33（58%）完全干燥，而6/33（18％）是湿的。在接受初次外裂修复而未同时或随后进行膀胱颈重建的患者中，尿失禁发生率为:GE63%(5/8);PE75%(6/8);PPE71%(5/7)。在9名患有PPE的男孩中，他们同时接受了BNR的初始外阴修复，22%(2/9)干燥,没有进一步手术。总的来说,8/20(40%)的男生PPE虚空伴完全干燥。
结论：此多中心回顾性研究表明，即使是一些患有腺性和阴茎性外阴的男孩，也可能面临尿失禁的挑战。尽管术前仔细评估了膀胱颈功能并同时进行了BNR，但患有耻骨外的男孩仍可能保持湿润。
结论：患有各种上下裂程度的男孩的延续性结果可能是可变的。即使有更多远端缺陷的男孩也可能有明显的膀胱颈缺陷。而那些患有最严重形式的尿道下裂的人可能需要膀胱颈重建才能实现节制。
