Department of Paediatric Surgery, The Royal Children's Hospital, Melbourne, Victoria, Australia; Surgical Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Victoria, Australia.
Department of Paediatric Surgery, The Royal Children's Hospital, Melbourne, Victoria, Australia; Surgical Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, Australia.
J Pediatr Surg. 2023 Jul;58(7):1263-1268. doi: 10.1016/j.jpedsurg.2023.01.051. Epub 2023 Feb 15.
The majority of patients with an anorectal malformation (ARM) have associated congenital anomalies. It is well established that all patients diagnosed with an ARM should undergo systematic screening, including renal, spinal, and cardiac imaging. This study aimed to evaluate the findings and completeness of screening, following local implementation of standardized protocols.
A retrospective cohort study was performed assessing all patients with an ARM managed at our tertiary pediatric surgical center, following a standardized protocol implementation for VACTERL screening (January 2016-December 2021). Cohort demographics, medical characteristics, and screening investigations were analyzed. Findings were compared with our previously published data (2000-2015), conducted prior to protocol implementation.
One hundred twenty-seven (64 male, 50.4%) children were eligible for inclusion. Complete screening was performed in 107/127 (84.3%) children. Of these, one or more associated anomalies were diagnosed in 85/107 (79.4%), whilst the VACTERL association was demonstrated in 57/107 (53.3%). The proportion of children that underwent complete screening increased significantly in comparison with those assessed prior to protocol implementation (RR 0.43 [CI 0.27-0.66]; p < 0.001). Children with less complex ARM types were significantly less likely to receive complete screening (p = 0.028). Neither presence of an associated anomaly, nor prevalence of the VACTERL association, differed significantly by ARM type complexity.
Screening for associated VACTERL anomalies in children with ARM was significantly improved following standardized protocol implementation. The prevalence of associated anomalies in our cohort supports the value of routine VACTERL screening in all children with ARM, regardless of malformation type.
II.
大多数肛门直肠畸形（ARM）患者都存在先天性畸形。已明确，所有诊断为 ARM 的患者均应进行系统筛查，包括肾脏、脊柱和心脏影像学检查。本研究旨在评估在实施标准化方案后，筛查的发现和完成情况。
本研究采用回顾性队列研究，评估了在我们的三级儿科外科中心接受管理的所有 ARM 患者，在实施 VACTERL 筛查的标准化方案后（2016 年 1 月至 2021 年 12 月）。分析了队列的人口统计学、医学特征和筛查调查结果。将这些发现与我们之前发表的数据（2000-2015 年）进行了比较，该数据是在方案实施之前进行的。
共有 127 名（64 名男性，50.4%）患儿符合纳入标准。在 127 名患儿中，有 107 名（84.3%）进行了完整筛查。在这些患儿中，有 85 名（79.4%）诊断出一种或多种相关畸形，而 57 名（53.3%）患儿显示 VACTERL 相关畸形。与方案实施前相比，接受完整筛查的患儿比例显著增加（RR 0.43 [CI 0.27-0.66]；p<0.001）。ARM 类型越简单的患儿，进行完整筛查的可能性越低（p=0.028）。ARM 类型的复杂性与相关畸形的存在或 VACTERL 关联的发生率无显著差异。
在实施标准化方案后，对 ARM 患儿进行相关 VACTERL 异常的筛查得到了显著改善。本研究队列中的相关畸形发生率支持对所有 ARM 患儿进行常规 VACTERL 筛查的价值，而与畸形类型无关。
II 级。
