Peter C Minneci1, Rashmi S Kabre2, Grace Z Mak3, Devin R Halleran4, Jennifer N Cooper4, Amin Afrazi5, Casey M Calkins6, Cynthia D Downard7, Peter Ehrlich8, Jason Fraser9, Samir K Gadepalli8, Michael A Helmrath10, Jonathan E Kohler5, Rachel Landisch6, Matthew P Landman11, Constance Lee12, Charles M Leys5, Daniel L Lodwick4, Rodrigo Mon8, Beth McClure7, Beth Rymeski10, Jacqueline M Saito12, Thomas T Sato6, Shawn D St Peter9, Richard Wood4, Marc A Levitt4, Katherine J Deans4. 1. Department of Pediatric Surgery and the Research Institute, Nationwide Children's Hospital, Columbus, OH. Electronic address: Peter.Minneci@nationwidechildrens.org. 2. Division of Pediatric Surgery, Department of Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL. 3. Section of Pediatric Surgery, Department of Surgery, The University of Chicago Medicine and Biologic Sciences, Chicago, IL. 4. Department of Pediatric Surgery and the Research Institute, Nationwide Children's Hospital, Columbus, OH. 5. Division of Pediatric Surgery, Department of Surgery, University of Wisconsin, Madison, WI. 6. Division of Pediatric Surgery, Department of Surgery, Medical College of Wisconsin, Milwaukee, WI. 7. Division of Pediatric Surgery, Hiram C. Polk, Jr., M.D. Department of Surgery, University of Louisville, Louisville, KY. 8. Section of Pediatric Surgery, Department of Surgery, University of Michigan, Ann Arbor, MI. 9. Division of Pediatric Surgery, Department of Surgery, Children's Mercy Hospital, Kansas City, MO. 10. Division of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH. 11. Division of Pediatric Surgery, Department of Surgery, Indiana University School of Medicine, Indianapolis, IN. 12. Division of Pediatric Surgery, Department of Surgery, Washington University School of Medicine, St. Louis, MO.
Abstract
BACKGROUND: This study evaluates screening practices and the incidence of associated anomalies in infants with anorectal malformations (ARM). METHODS: We performed a multi-institutional retrospective cohort study of children born between 2007 and 2011 who underwent surgery for ARM at 10 children's hospitals. ARM type was classified based on the location of the distal rectum, and all screening studies were reviewed. RESULTS: Among 506 patients, the most common ARM subtypes were perineal fistula (40.7%), no fistula (11.5%), and vestibular fistula (10.1%). At least 1 screening test was performed in 96.6% of patients, and 11.3% of patients underwent all. The proportion of patients with ≥1 abnormal finding on any screening test varied by type of ARM (p<0.001). Screening rates varied from 15.2% for limb anomalies to 89.7% for renal anomalies. The most commonly identified anomalies by screening category were: spinal: tethered cord (20.6%); vertebral: sacral dysplasia/hemisacrum (17.8%); cardiac: patent foramen ovale (58.0%); renal: hydronephrosis (22.7%); limb: absent radius (7.9%). CONCLUSION: Screening practices and the incidence of associated anomalies varied by type of ARM. The rate of identifying at least one associated anomaly was high across all ARM subtypes. Screening for associated anomalies should be considered standard of care for all ARM patients. TYPE OF STUDY: Multi-institutional retrospective cohort study. LEVEL OF EVIDENCE: III.
BACKGROUND: This study evaluates screening practices and the incidence of associated anomalies in infants with anorectal malformations (ARM). METHODS: We performed a multi-institutional retrospective cohort study of children born between 2007 and 2011 who underwent surgery for ARM at 10 children's hospitals. ARM type was classified based on the location of the distal rectum, and all screening studies were reviewed. RESULTS: Among 506 patients, the most common ARM subtypes were perineal fistula (40.7%), no fistula (11.5%), and vestibular fistula (10.1%). At least 1 screening test was performed in 96.6% of patients, and 11.3% of patients underwent all. The proportion of patients with ≥1 abnormal finding on any screening test varied by type of ARM (p<0.001). Screening rates varied from 15.2% for limb anomalies to 89.7% for renal anomalies. The most commonly identified anomalies by screening category were: spinal: tethered cord (20.6%); vertebral: sacral dysplasia/hemisacrum (17.8%); cardiac: patent foramen ovale (58.0%); renal: hydronephrosis (22.7%); limb: absent radius (7.9%). CONCLUSION: Screening practices and the incidence of associated anomalies varied by type of ARM. The rate of identifying at least one associated anomaly was high across all ARM subtypes. Screening for associated anomalies should be considered standard of care for all ARM patients. TYPE OF STUDY: Multi-institutional retrospective cohort study. LEVEL OF EVIDENCE: III.