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Published on: January 7, 2019
Screening for VACTERL Anomalies in Children with Anorectal Malformations: Outcomes of a Standardized Approach
Hannah M E Evans-Barns1, Liesel Porrett1, Penelope L Hartmann2
1Department 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.
Insights
Standardized screening protocols significantly improved the detection of VACTERL association in infants with anorectal malformations (ARM). Routine screening is crucial for all ARM patients, irrespective of malformation complexity, to identify associated congenital anomalies.
Area of Science:
- Pediatric Surgery
- Congenital Anomalies
- Medical Imaging
Background:
- Anorectal malformation (ARM) frequently co-occurs with other congenital anomalies.
- Systematic screening, including renal, spinal, and cardiac imaging, is recommended for all ARM patients.
- Standardized protocols aim to enhance the completeness and consistency of VACTERL screening.
Purpose of the Study:
- To evaluate the effectiveness of standardized protocols in VACTERL screening for ARM patients.
- To assess the completeness of screening investigations post-protocol implementation.
- To compare current screening findings with historical data.
Main Methods:
- Retrospective cohort study of ARM patients managed between January 2016 and December 2021.
- Analysis of cohort demographics, medical characteristics, and screening investigations.
- Comparison of data with a pre-protocol implementation cohort (2000-2015).
Main Results:
- Complete screening was achieved in 84.3% of 127 included children.
- Associated anomalies were diagnosed in 79.4%, with VACTERL association in 53.3%.
- Complete screening rates significantly increased post-protocol implementation (RR 0.43), though less complex ARM types were less likely to be fully screened.
Conclusions:
- Standardized protocols significantly enhanced VACTERL anomaly screening in ARM patients.
- The high prevalence of associated anomalies underscores the importance of routine VACTERL screening for all ARM cases.
- Screening effectiveness is improved regardless of ARM complexity.
Purpose:
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.
Methods:
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.
Results:
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.
Conclusion:
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.
Level Of Evidence:
II.
