Borg H, Sillén U, Doroszkiewicz M, Holmdahl G, Sjöström S, Abrahamsson K
Department of Paediatric Surgery, Queen Silvia Children's Hospital, The Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden.
Department of Paediatric Surgery, Queen Silvia Children's Hospital, The Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden; The Paediatric UroNephrologic Centre, Queen Silvia Children's Hospital, The Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden.
J Pediatr Urol. 2021 Feb;17(1):76.e1-76.e9. doi: 10.1016/j.jpurol.2020.10.016. Epub 2020 Oct 16.
Neurogenic lower urinary tract dysfunction (LUTD) has been reported in 20-50% of children with anorectal malformations (ARM). As neurogenic LUTD represents an inherent risk of renal deterioration and urinary tract infections, an early diagnosis is important. The gold standard for evaluating neurogenic LUTD involves invasive urodynamic testing but a useful addition should be an easy-to-perform, non-invasive method of screening.
In this study, we evaluate non-invasive 4 h voiding observations as a screening method for neurogenic LUTD in ARM children.
Thirty-four patients with ARM, excluding those with perineal fistulas, were evaluated using both 4 h voiding observation and urodynamic testing before and after posterior sagittal anorectoplasty (PSARP) at median ages of 0.3 and 1.1 years. In the urodynamic assessment, the gold standard for neurogenic LUTD, nine children received the diagnosis, eight innate and one post-surgery.
Five boys with a high urethral fistula and anomalies of the spinal cord had urodynamically diagnosed neurogenic LUTD, a dysfunction also identified in the 4 h voiding observations. The pattern was characterised both by an increase in the number of voiding and the number of interrupted voiding, urinary leakage and elevated residual urine (Figure). In three girls with a vestibular fistula and tethered cord, an urodynamic investigation identified suspected mild neurogenic LUTD. In the voiding observations, an abnormal voiding pattern was not as obvious in the girls as in the five males. One girl with cloaca showed signs of postsurgical denervation damage, which was easily identified in the 4 h voiding observations (high capacity and elevated residual urine).
In the present study, gender differences in the severity of dysfunction reflected in the free voiding pattern in infants with ARM and neurogenic LUTD is probably the result of the different underlying causes of neurogenic LUTD in boys and girls. Boys with the condition have a congenital malformation of the caudal part of the spinal cord and girls a tethering of the cord. The most obvious limitation of the study was the low number of patients. Despite this, we consider the results worth reporting, since we found that results in the free voiding observations effectively confirmed what was established in the urodynamic investigations.
In pre-PSARP patients, 4 h voiding observations can be used to screen for severe neurogenic LUTD requiring attention and treatment. When post-surgical denervation is suspected, the voiding observation is also a good method for indicating the diagnosis.
据报道，20%至50%的肛门直肠畸形（ARM）患儿存在神经源性下尿路功能障碍（LUTD）。由于神经源性LUTD存在肾脏恶化和尿路感染的内在风险，早期诊断至关重要。评估神经源性LUTD的金标准涉及侵入性尿动力学检测，但一种易于实施的非侵入性筛查方法应是有益的补充。
在本研究中，我们评估4小时排尿观察作为ARM患儿神经源性LUTD筛查方法的效果。
34例ARM患儿（不包括会阴瘘患儿）在中位年龄0.3岁和1.1岁时，于后矢状位肛门直肠成形术（PSARP）前后分别接受了4小时排尿观察和尿动力学检测。在尿动力学评估（神经源性LUTD的金标准）中，9名儿童被诊断为神经源性LUTD，其中8名是先天性的，1名是术后的。
5名患有高位尿道瘘和脊髓异常的男孩经尿动力学诊断为神经源性LUTD，4小时排尿观察中也发现了这种功能障碍。其模式的特征是排尿次数增加、间断排尿次数增加、尿失禁和残余尿量增加（图）。3名患有前庭瘘和脊髓栓系的女孩经尿动力学检查发现疑似轻度神经源性LUTD。在排尿观察中，女孩异常的排尿模式不如5名男孩明显。1名泄殖腔畸形女孩表现出术后去神经损伤的迹象，这在4小时排尿观察中很容易识别（膀胱容量大且残余尿量增加）。
在本研究中，ARM合并神经源性LUTD婴儿的自由排尿模式所反映的功能障碍严重程度的性别差异，可能是由于男孩和女孩神经源性LUTD的潜在病因不同。患有这种疾病的男孩存在脊髓尾部先天性畸形，女孩则存在脊髓栓系。本研究最明显的局限性是患者数量少。尽管如此，我们认为结果值得报告，因为我们发现自由排尿观察的结果有效地证实了尿动力学检查所确定的情况。
在PSARP术前患者中，4小时排尿观察可用于筛查需要关注和治疗的严重神经源性LUTD。当怀疑术后存在去神经损伤时，排尿观察也是一种很好的诊断方法。
