Yilmaz Esra Karabag, Hakalmaz Ali Ekber, Saygılı Seha, Agbas Ayse, Karatas Kubra, Cebi Memnune Nur, Ozcan Rahsan, Kurugoglu Sebuh, Elicevik Mehmet, Emir Haluk, Canpolat Nur
Department of Paediatric Nephrology, Istanbul University-Cerrahpasa, Cerrahpasa Faculty of Medicine, Istanbul, Turkey.
Department of Paediatric Surgery, Istanbul University-Cerrahpasa, Cerrahpasa Faculty of Medicine, Istanbul, Turkey.
J Pediatr Urol. 2025 Aug;21(4):908-914. doi: 10.1016/j.jpurol.2025.03.015. Epub 2025 Mar 24.
Anorectal malformations (ARM) are often accompanied by urological anomalies, which can contribute to the development of chronic kidney disease (CKD).
This study aimed to investigate the prevalence of kidney and urinary tract anomalies, as well as bladder dysfunction, and their potential impact on the development of CKD in children with ARM.
This single-center, retrospective study included 175 children with ARM, after excluding 75 children with missing data. Clinical and radiological findings and serum creatinine levels were obtained from medical records. Anorectal malformations were classified as "high" and "low" type based on the Wingspread classification, and also further classified by fistula type and sex distribution according to the Krickenbeck International Classification. CKD was defined and staged according to the KDIGO (Kidney Disease Improving Global Outcomes) guidelines.
The median age of the patients was 9.7 years, with a male-to-female ratio of 1.13:1. Among the 175 patients, 97 (55 %) had intermediate or low-type ARM, and 78 (45 %) had high-type ARM. Kidney and/or urinary tract anomalies were identified in 85 patients (48.5 %), with 63 having kidney anomalies and 71 having urinary tract anomalies. The most common urinary tract anomaly was vesicoureteral reflux (n = 58). Bladder dysfunction was observed in 56 patients (32 %). CKD was diagnosed in 30 patients (17 %); six of them were in CKD stage 5, and the remaining 24 were in stages 2-4. The development of CKD was more common in both females and males with kidney anomalies (p < 0.001), urinary tract anomalies (p < 0.001 and p = 0.002, respectively), bladder dysfunction (p < 0.001), urinary tract infection (p < 0.001), in addition females with persistent cloaca (p = 0.023) and spinal anomalies (p = 0.013). Kidney anomalies and bladder dysfunction were independently associated with the development of CKD (p < 0.001 and p = 0.046, respectively).
This study highlights that approximately half of the children with ARM had kidney and urinary tract anomalies, and one-third had bladder dysfunction. Additionally, almost one-fifth of the patients developed CKD, mostly in the early stages. Our findings also suggest that both kidney anomalies and bladder dysfunction are significant risk factors for CKD development in children with ARM.
This study emphasizes the presence of kidney anomalies, along with bladder dysfunction as risk factors for CKD. Physicians should take care to monitor and manage these risks to minimize the long-term impacts on kidney health in children with ARM.
肛门直肠畸形（ARM）常伴有泌尿系统异常，这可能导致慢性肾脏病（CKD）的发生。
本研究旨在调查ARM患儿肾脏和尿路异常以及膀胱功能障碍的患病率，及其对CKD发生发展的潜在影响。
本单中心回顾性研究纳入了175例ARM患儿，排除了75例数据缺失的患儿。从病历中获取临床和影像学检查结果以及血清肌酐水平。根据Wingspread分类法将肛门直肠畸形分为“高位”和“低位”类型，并根据Krickenbeck国际分类法进一步按瘘管类型和性别分布进行分类。根据KDIGO（改善全球肾脏病预后组织）指南对CKD进行定义和分期。
患者的中位年龄为9.7岁，男女比例为1.13:1。在175例患者中，97例（55%）为中低位ARM，78例（45%）为高位ARM。85例患者（48.5%）被发现有肾脏和/或尿路异常，其中63例有肾脏异常，71例有尿路异常。最常见的尿路异常是膀胱输尿管反流（n = 58）。56例患者（32%）观察到膀胱功能障碍。30例患者（17%）被诊断为CKD；其中6例处于CKD 5期，其余24例处于2 - 4期。CKD的发生在有肾脏异常的女性和男性中更常见（p < 0.001），在有尿路异常的女性和男性中也更常见（分别为p < 0.001和p = 0.002），在有膀胱功能障碍的患者中更常见（p < 0.001），在有尿路感染的患者中更常见（p < 0.001），此外在有持续性泄殖腔的女性患者中也更常见（p = 0.023）以及有脊柱异常的患者中更常见（p = 0.013）。肾脏异常和膀胱功能障碍与CKD的发生独立相关（分别为p < 0.001和p = 0.046）。
本研究强调，约一半的ARM患儿有肾脏和尿路异常，三分之一有膀胱功能障碍。此外，近五分之一的患者发生了CKD，大多处于早期阶段。我们的研究结果还表明，肾脏异常和膀胱功能障碍都是ARM患儿发生CKD的重要危险因素。
本研究强调肾脏异常以及膀胱功能障碍是CKD的危险因素。医生应注意监测和管理这些风险，以尽量减少对ARM患儿肾脏健康的长期影响。
