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直肠膀胱颈瘘型肛门直肠畸形患儿的临床及尿动力学结果

Clinical and urodynamic outcomes in children with anorectal malformation subtype of recto-bladder neck fistula.

作者信息

Strine A C, VanderBrink B A, Alam Z, Schulte M, Noh P H, DeFoor W R, Minevich E, Sheldon C A, Frischer J S, Reddy P P

机构信息

Division of Pediatric Urology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

Division of Pediatric Urology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

出版信息

J Pediatr Urol. 2017 Aug;13(4):376.e1-376.e6. doi: 10.1016/j.jpurol.2017.06.008. Epub 2017 Jul 10.

Abstract

INTRODUCTION

Patients with anorectal malformations (ARMs) have a high incidence of genitourinary anomalies. Those with a recto-bladder neck fistula may represent a high-risk group, but their long-term urologic outcomes are poorly described.

OBJECTIVE

To evaluate the clinical and urodynamic outcomes in a large cohort of patients with an ARM subtype of recto-bladder neck fistula.

MATERIALS AND METHODS

A retrospective cohort study was performed of patients who had been treated for a recto-bladder neck fistula at the present institution since 2007. The primary outcomes were the ability to achieve urinary continence after 4 years of age, and development of a mildly decreased glomerular filtration rate (GFR) or worse (<89 ml/min/1.73 m). Continence was defined as the ability to store urine for 3-4 h during the day and 8 h overnight without leakage.

RESULTS

Demographic and clinical data are provided in the Summary Table. The most recent urodynamic findings included the presence of detrusor overactivity in 30 (75%) patients, median leak point pressure of 56.0 cmHO (range, 14-140), median functional cystometric capacity at 40 cmHO of 125.5% age-expected capacity (range, 36-473%), and median maximum cystometric capacity of 131.0% age-expected capacity (range, 44-473%). A mildly decreased GFR or worse developed in 13 (24%) patients. Of the 52 (78%) patients who were followed by pediatric urology at the present institution with a median follow-up of 30.9 months (range, 0.0-86.8), 35 (67%) were at least 4 years of age and could be assessed for continence. Continence was achieved in five (14%) patients voiding spontaneously and 15 (43%) performing CIC. Recurrent urinary tract infections (UTI) (OR 0.70, P = 0.006) were an independent predictor of incontinence, while urethral anomalies (OR 1.40, P = 0.03) were an independent predictor of chronic kidney disease (CKD) on multiple logistic regression analysis.

DISCUSSION

The findings favorably compared with other studies, but were more robust due to the size of the cohort and breadth of urologic evaluation. Limitations included the retrospective design at a single institution. Incomplete clinical data and misclassification of continence may have lead to bias.

CONCLUSIONS

This large cohort of patients with an ARM subtype of recto-bladder neck fistula had a high incidence of genitourinary anomalies. They were rarely able to achieve continence with spontaneous voiding alone and were at risk of developing CKD, both of which were likely multifocal in origin. Long-term urologic follow-up is warranted for patients with a recto-bladder neck fistula.

摘要

引言

肛门直肠畸形(ARM)患者的泌尿生殖系统异常发生率很高。那些患有直肠膀胱颈瘘的患者可能是高危人群,但他们的长期泌尿系统结局描述甚少。

目的

评估一大群患有直肠膀胱颈瘘这种ARM亚型患者的临床和尿动力学结局。

材料与方法

对自2007年以来在本机构接受直肠膀胱颈瘘治疗的患者进行了一项回顾性队列研究。主要结局是4岁后实现尿失禁的能力,以及出现轻度降低的肾小球滤过率(GFR)或更差情况(<89 ml/min/1.73 m²)。尿失禁定义为白天能够储存尿液3 - 4小时且夜间8小时无渗漏。

结果

汇总表中提供了人口统计学和临床数据。最近的尿动力学检查结果包括30例(75%)患者存在逼尿肌过度活动,中位漏点压力为56.0 cmH₂O(范围为14 - 140),在40 cmH₂O时中位功能性膀胱容量为预期年龄容量的125.5%(范围为36 - 473%),中位最大膀胱容量为预期年龄容量的131.0%(范围为44 - 473%)。13例(24%)患者出现轻度降低的GFR或更差情况。在本机构接受小儿泌尿外科随访的52例(78%)患者中,中位随访时间为30.9个月(范围为0.0 - 86.8),35例(67%)年龄至少4岁且可评估尿失禁情况。5例(14%)自主排尿患者和15例(43%)进行清洁间歇导尿(CIC)患者实现了尿失禁。复发性尿路感染(UTI)(比值比0.70,P = 0.006)是尿失禁的独立预测因素,而尿道异常(比值比1.40,P = 0.03)是多因素逻辑回归分析中慢性肾脏病(CKD)的独立预测因素。

讨论

研究结果与其他研究相比情况较好,但由于队列规模和泌尿外科评估的广度,结果更为可靠。局限性包括单一机构的回顾性设计。不完整的临床数据和尿失禁的错误分类可能导致偏差。

结论

这一大群患有直肠膀胱颈瘘这种ARM亚型的患者泌尿生殖系统异常发生率很高。他们很少仅通过自主排尿实现尿失禁,且有发生CKD的风险,这两者可能都源于多方面原因。对于直肠膀胱颈瘘患者,有必要进行长期的泌尿外科随访。

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