关键词: Anorectal malformation Congenital Renal anomalies Southwestern Uganda

来  源:   DOI:10.1016/j.jpurol.2024.07.027

Abstract:
BACKGROUND: Anorectal malformations (ARMs) may be associated with congenital anomalies affecting other body parts namely vertebral, anorectal, cardiac, tracheoesophageal, renal, and limb (VACTERL) with varying incidences of 7%-60% . Genitourinary defects might occur approximately in 50% of all patients with anorectal malformations hence patients should be evaluated from birth to rule out these defects.
OBJECTIVE: To identify urological anomalies associated with anorectal malformation in southwestern Uganda.
METHODS: This was a descriptive retrospective cohort study conducted at our regional referral hospital in Southwestern Uganda involving patients who have undergone surgical correction of ARMs between June 2021 and July 2023.
RESULTS: The overall prevalence of renal anomalies in our study patient population was 18.05%. Of those with ARM-associated renal anomalies, Specific anomalies included; renal agenesis (6.8%), hydronephrosis, (4.5%), duplex collecting system (3.8%), crossed fused kidney (1.5%), and ectopic kidney (0.75%). (Table) DISCUSSION: We found that the prevalence of ARM-associated renal anomalies was 18.05%, and the commonest anomaly was unilateral agenesis (6.8%) similar to other studies. Previous data have shown renal anomalies are common anomalies in ARM. While the exact values vary across studies, they all concluded that the rate of associated anomalies is extremely high in ARMs and warrants a thorough preoperative investigation once the ARMs are detected. This finding therefore underscores the importance of thorough evaluation and a multidisciplinary approach of care and follow-up system for ARM management including urologists even when the children are asymptomatic now. The main limitation of our study was missing information on patients\' charts, we were not able to get the diagnosis since most patients didn\'t have their discharge forms at the time of evaluation.
CONCLUSIONS: ARM associated with renal anomalies may remain undiagnosed and asymptomatic. Those identified as asymptomatic need to be followed in a multidisciplinary fashion including pediatric urologists.
摘要:
背景:肛门直肠畸形(ARM)可能与影响其他身体部位的先天性异常有关,即椎骨,肛门直肠,心脏,气管食管,肾,和肢体(VACTERL),发生率为7%-60%。在所有肛门直肠畸形患者中,约有50%可能发生泌尿生殖系统缺陷,因此应从出生时对患者进行评估以排除这些缺陷。
目的:在乌干达西南部发现与肛门直肠畸形相关的泌尿系统异常。
方法:这是一项描述性回顾性队列研究,在我们位于乌干达西南部的地区转诊医院进行,研究对象为2021年6月至2023年7月期间接受ARM手术矫正的患者。
结果:我们研究的患者人群中肾脏异常的总患病率为18.05%。在手臂相关的肾脏异常患者中,特定异常包括;肾发育不全(6.8%),肾积水,(4.5%),复式收集系统(3.8%),交叉融合肾(1.5%),和异位肾(0.75%)。(表)讨论:我们发现ARM相关肾脏异常的患病率为18.05%,与其他研究相似,最常见的异常是单侧发育不全(6.8%)。先前的数据表明,肾脏异常是ARM的常见异常。虽然确切的值因研究而异,他们都得出结论,ARM中相关异常的发生率极高,一旦发现ARM,就需要进行彻底的术前调查.因此,这一发现强调了对包括泌尿科医师在内的ARM管理进行彻底评估以及多学科护理和随访系统的重要性,即使现在儿童无症状。我们研究的主要局限性是缺少患者图表上的信息,我们无法得到诊断,因为大多数患者在评估时没有出院表。
结论:与肾脏异常相关的ARM可能仍未诊断且无症状。那些被确定为无症状的患者需要以多学科的方式进行随访,包括儿科泌尿科医师。
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