INTRODUCTION:Complete primary repair of bladder exstrophy (CPRE) is widely used for classic bladder exstrophy (CBE) closure. Long-term renal function with or without dilation in these patients is poorly characterized and may be impacted by bladder storage parameters or recurrent urinary tract infections (UTIs). OBJECTIVE:We sought to assess our long-term experience with CPRE and investigate factors that may influence upper-tract deterioration. STUDY DESIGN:A retrospective review of patients at our institution with CBE undergoing CPRE from 1990 to 2015 was performed. Patients were considered included if age at the last renal ultrasound was at least 5 years. Renal imaging and renal function were reviewed. The Society of Fetal Urology (SFU) and Upper Tract Dilation (UTD) grades were retrospectively assigned to all available ultrasounds with hydronephrosis (HN). Additionally, outcomes related to vesicoureteral reflux and lower urinary tract function were assessed. Descriptive and comparative statistical analyses were performed to assess factors influencing HN and renal function at follow-up. RESULTS:Thirty patients (57% male) had a median follow-up of 9.7 (3.9-22.3) years. The table shows the HN status and grade for the entire group, stratified by gender, continence, and surgical reconstruction in. The mean creatinine was 0.50 mg/dL (0.2-1.0) and the mean estimated glomerular filtration rate (eGFR) was 106.8 mL/min/1.73 m2. No patient had greater than stage 2 chronic kidney disease. Male gender was associated with worse renal outcomes, including overall rate of HN (p < 0.001), severity of HN (p = 0.004) and worse eGFR (p = 0.05). Lower tract reconstruction, urodynamic parameters, and continence were not associated with differences in upper-tract outcomes. Ureteral reimplantation was performed in 22 patients (73%) at a mean age of 22 months for indications of persistent VUR (10), worsening HN (1), or recurrent UTI (11). DISCUSSION:HN is common following CPRE, although severe HN is seen infrequently. Overall long-term renal outcomes are similar to those of other techniques presented in the literature. However, male gender portends a higher risk for long-term upper-tract deterioration as measured by HN and eGFR. Ureteral reimplantation and the status of the lower urinary tract were not associated with differences in upper-tract outcomes. CONCLUSIONS:Prospective, standardized approaches to characterize upper-tract outcomes are needed to follow children with bladder exstrophy into adulthood. We believe higher-risk patients should be assessed early and managed more aggressively with attention paid towards accurately assessing renal function and lower tract anatomy.

译文

简介:完全的膀胱外翻修复(CPRE)被广泛用于经典的膀胱外翻(CBE)闭合术。这些患者伴或不伴扩张的长期肾功能的特征较差,可能会受到膀胱存储参数或复发性尿路感染(UTI)的影响。
目的:我们试图评估我们在CPRE方面的长期经验,并调查可能影响上呼吸道退化的因素。
研究设计:回顾性分析1990年至2015年本院接受CPRE的CBE患者。如果最后一次肾脏超声检查的年龄至少为5岁,则认为患者包括在内。肾脏成像和肾功能进行了审查。胎儿泌尿外科学会(SFU)和上呼吸道扩张(UTD)等级被回顾性地分配给所有可用的肾积水(HN)超声。此外,评估了与膀胱输尿管反流和下尿路功能有关的结局。进行描述性和比较性统计分析,以评估随访时影响HN和肾功能的因素。
结果:30名患者(男性占57%)的中位随访时间为9.7年(3.9-22.3年)。该表显示了整个组的HN状态和等级,按性别,节制和手术方式分层。平均肌酐为0.50 mg / dL(0.2-1.0),平均估计肾小球滤过率(eGFR)为106.8 mL /min/1.73平方米。没有患者的慢性肾脏病超过2期。男性性别与肾结局恶化相关,包括HN总体发生率(p <0.001),HN严重程度(p = 0.004)和eGFR恶化(p = 0.05)。下尿道重建,尿动力学参数和节制与上尿道结局的差异无关。平均年龄为22个月的22例患者(73%)进行了输尿管再植术,提示有持续性VUR(10),HN恶化(1)或UTI复发(11)。
讨论:CPRE后HN很常见,尽管很少见到严重的HN。总体长期肾脏结局与文献中介绍的其他技术相似。然而,根据HN和eGFR的测量,男性性别预示着长期上呼吸道退化的更高风险。输尿管再植和下尿路状况与上尿路结局的差异无关。
结论:需要有前瞻性,标准化的方法来表征上位结局,才能使患有膀胱萎缩症的儿童进入成年期。我们认为高危患者应及早评估并更积极地处理,并应注意准确评估肾功能和下尿道解剖结构。

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