Borer J G, Vasquez E, Canning D A, Kryger J V, Bellows A, Weiss D, Groth T, Shukla A, Kurtz M P, Mitchell M E
Department of Urology, Boston Children's Hospital, Boston, MA, USA.
Department of Urology, Boston Children's Hospital, Boston, MA, USA.
J Pediatr Urol. 2017 Jun;13(3):275.e1-275.e6. doi: 10.1016/j.jpurol.2017.01.006. Epub 2017 Feb 20.
INTRODUCTION/BACKGROUND: Bladder exstrophy is a rare diagnosis that presents major reconstructive challenges. To increase experience and proficiency in the care of bladder exstrophy (BE), the Multi-Institutional BE Consortium (MIBEC) was formed, with a focus on refining technical aspects of complete primary repair of bladder exstrophy (CPRE) and subsequent care.
Outcome measures included successful CPRE (absence of dehiscence), complications, and integrated points of technique and care over the short-term.
Boston Children's Hospital, Children's Hospital of Philadelphia and Children's Hospital of Wisconsin alternately served as the host, with observation, commentary and critique by visiting collaborating surgeons. CPRE with bilateral iliac osteotomy was performed at 1-3 months of age. High-definition video capture of the surgery allowed local and distant broadcast to facilitate real-time observation and teaching, and recording of all procedures.
From February 2013 to February 2015, MIBEC participating surgeons performed CPRE on 27 consecutive patients (22 classic BE, five epispadias). There were no dehiscences in 27 patients (0%, 95% CI 0-12.5%). Thirteen girls and 14 boys underwent CPRE at a median age of 2.3 months (range 0.1-51.6). One boy had a hypospadiac urethral meatus at CPRE completion. Hydronephrosis of mild or moderate grade was present postoperatively in eight girls and two boys. Additional results, per gender, are presented in the Summary table below.
Absence of dehiscence in this cohort was comparable or compared favorably with the literature. However, several girls had significant obstructive complications following CPRE. The rate of bladder outlet obstruction (BOO) in girls was increased compared with published reports. A low complication rate was noted in the boys following CPRE, which was comparable to reports in the literature, and early signs of continence and spontaneous voiding were noted in some boys and girls. Limitations included variation in patient age at presentation, thereby introducing a wide age range at CPRE. Outcome data were limited by short follow-up regarding voiding with continence.
This collaborative effort proved beneficial regarding significantly increased surgeon exposure to CPRE, refinement of CPRE technique, surgeon learning and expertise. Technical refinement of CPRE is ongoing.
引言/背景：膀胱外翻是一种罕见的诊断，带来了重大的重建挑战。为了增加在膀胱外翻（BE）护理方面的经验和熟练度，成立了多机构膀胱外翻联盟（MIBEC），重点是完善膀胱外翻完全一期修复（CPRE）的技术方面及后续护理。
结果指标包括成功的CPRE（无裂开）、并发症以及短期内技术与护理的综合要点。
波士顿儿童医院、费城儿童医院和威斯康星儿童医院轮流担任主办单位，来访的合作外科医生进行观察、评论和批评。在1至3个月大时进行CPRE并双侧髂骨截骨术。手术的高清视频捕捉允许本地和远程播放，以促进实时观察和教学，并记录所有手术过程。
2013年2月至2015年2月，MIBEC参与手术的外科医生连续为27例患者（22例典型BE，5例尿道上裂）进行了CPRE。27例患者均无裂开（0%，95%可信区间0 - 12.5%）。13名女孩和14名男孩接受了CPRE，中位年龄为2.3个月（范围0.1 - 51.6个月）。一名男孩在CPRE完成时存在尿道下裂尿道口。8名女孩和2名男孩术后出现轻度或中度肾积水。按性别分类的其他结果见下表总结。
该队列中无裂开情况与文献报道相当或更优。然而，几名女孩在CPRE后出现了严重的梗阻性并发症。与已发表报告相比，女孩膀胱出口梗阻（BOO）的发生率有所增加。男孩CPRE后的并发症发生率较低，与文献报道相当，并且在一些男孩和女孩中注意到了控尿和自主排尿的早期迹象。局限性包括就诊时患者年龄的差异，从而导致CPRE时年龄范围较广。结果数据因关于控尿排尿的随访时间短而受限。
这项合作努力证明是有益的，显著增加了外科医生对CPRE的接触，完善了CPRE技术，促进了外科医生的学习和专业技能提升。CPRE的技术完善工作正在进行。
