Department of Neurosurgery, Children's Hospital Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
J Pediatr Urol. 2012 Apr;8(2):181-6. doi: 10.1016/j.jpurol.2011.02.004. Epub 2011 Mar 12.
To evaluate urologic manifestations of sacral agenesis (SA) and their association with bony defects.
Urological manifestations of SA were investigated in 50 patients referred to the urology or neurosurgery department. Urologenital signs and symptoms were assessed and a complete history of previous surgical procedures was attained. Plain lumbosacral radiography, abdominal/pelvic ultrasound, voiding cystourethrogram and urodynamic study were evaluated if available.
The most common urologic complaints were urinary incontinence and/or constant dribbling, seen in 30 (85%) of 35 children aged 4 years and over. Recurrent urinary tract infection, the second most common, was seen in 37 (74%). Vesicoureteral reflux was identified in 32 (65.3%) patients, 19 (59.3%) were found to have high maximal voiding pressures and post-voiding residual urine was notable in 42 (85.7%). Abnormal urodynamic parameters were found to be consistent with a neurogenic bladder in all patients. Cases were divided into upper motor lesions (in 34) and lower motor disorders (in 15). There was no statistically significant correlation for any GU finding with type of bony aplasia or motor neuron lesion (P = 0.338).
Voiding impairment and VUR together with recurrent UTI, especially in children with associated renal anomalies, contribute to renal damage. Urinary incontinence with associated social problems frequently occurs in patients with SA. Considering the devastating consequences of this disease in the urinary tract, timely diagnosis, thorough evaluation and appropriate intervention are essential.
评估骶骨发育不全(SA)的泌尿系统表现及其与骨缺陷的关系。
对泌尿科或神经外科就诊的 50 例 SA 患者的泌尿系统表现进行了研究。评估了泌尿生殖系统的体征和症状,并获得了既往手术史的详细信息。如果有条件,还评估了腰骶部平片、腹部/盆腔超声、排尿性膀胱尿道造影和尿动力学研究。
35 名 4 岁及以上儿童中,最常见的泌尿系统主诉为尿失禁和/或持续滴尿,有 30 例(85%);其次是复发性尿路感染,共 37 例(74%)。32 例(65.3%)患者存在输尿管反流,19 例(59.3%)发现最大排尿压高,42 例(85.7%)存在排尿后残余尿。所有患者的尿动力学参数均提示为神经性膀胱。病例分为上运动神经元病变(34 例)和下运动神经元障碍(15 例)。任何 GU 发现与骨发育不全类型或运动神经元病变均无统计学显著相关性(P=0.338)。
排尿障碍和 VUR 以及复发性 UTI,尤其是伴有肾异常的儿童,会导致肾脏损害。遗尿伴有相关的社会问题,在 SA 患者中经常发生。鉴于这种疾病对泌尿道的破坏性后果,及时诊断、彻底评估和适当干预至关重要。