ARM Publications

Inter-rater Reliability of Sacral Ratio Measurements in Patients with Anorectal Malformations.

Metzger G, Cooper JN, Kabre RS, Mak GZ, Halleran DR, Boyd K, Chan S, Corea D, Dydynski P, Gill K, Kraus S, Sanchez R, Afrazi A, Calkins C, Linden AF, Downard C, Ehrlich PF, Fraser JD, Landman M, Leys CM, Rymeski B, Wood RJ, Levitt MA, Deans KJ, Bates DG, Minneci PC; Midwest Pediatric Surgery Consortium. Inter-rater Reliability of Sacral Ratio Measurements in Patients with Anorectal Malformations. J Surg Res.. 2020 Jul 23;256:272-281. doi: 10.1016/j.jss.2020.06.040. Online ahead of print.PMID: 32712441

  • Abstract

    Background: Anorectal malformations (ARMs) are a spectrum of congenital anomalies with varying prognosis for fecal continence. The sacral ratio (SR) is a measure of sacral development that has been proposed as a method to predict future fecal continence in children with ARM. The aim of this study was to quantify the inter-rater reliability (IRR) of SR calculations by radiologists at different institutions.

    Materials and methods: x-Rays in the anteroposterior (AP) and lateral planes were reviewed by a pediatric radiologist at each of six different institutions. Subsequently, images were reviewed by a single, central radiologist. The IRR was assessed by calculating Pearson correlation coefficients and intraclass correlation coefficients from linear mixed models with patient and rater-level random intercepts.

    Results: Imaging from 263 patients was included in the study. The mean inter-rater absolute difference in the AP SR was 0.05 (interquartile range, 0.02-0.10), and in the lateral SR was 0.16 (interquartile range, 0.06-0.25). Overall, the IRR was excellent for AP SRs (intraclass correlation coefficient [ICC], 81.5%; 95% confidence interval, 75.1%-86.0%) and poor for lateral SRs (ICC, 44.0%; 95% CI, 29.5%-59.2%). For both AP and lateral SRs, ICCs were similar when examined by the type of radiograph used for calculation, severity of the ARM, presence of sacral or spinal anomalies, and age at imaging.

    Conclusions: Across radiologists, the reliability of SR calculations was excellent for the AP plane but poor for the lateral plane. These results suggest that better standardization of lateral SR measurements is needed if they are going to be used to counsel families of children with ARM.

    Cite

    Metzger G, Cooper JN, Kabre RS, Mak GZ, Halleran DR, Boyd K, Chan S, Corea D, Dydynski P, Gill K, Kraus S, Sanchez R, Afrazi A, Calkins C, Linden AF, Downard C, Ehrlich PF, Fraser JD, Landman M, Leys CM, Rymeski B, Wood RJ, Levitt MA, Deans KJ, Bates DG, Minneci PC; Midwest Pediatric Surgery Consortium. Inter-rater Reliability of Sacral Ratio Measurements in Patients with Anorectal Malformations. J Surg Res.. 2020 Jul 23;256:272-281. doi: 10.1016/j.jss.2020.06.040. Online ahead of print.PMID: 32712441

Can fecal continence be predicted in patients born with anorectal malformations? 

Minneci PC, Kabre RS, Mak GZ, Halleran DR, Cooper JN, Afrazi A, Calkins CM, Corkum K, Downard CD, Ehrlich P, Fraser JD, Gadepalli SK, Helmrath MA, Kohler JE, Landisch R, Landman MP, Lee C, Leys CM, Lodwick DL, McLeod J, Mon R, McClure B, Rymeski B, Saito JM, Sato TT, St Peter SD, Wood R, Levitt MA, Deans KJ; Midwest Pediatric Surgery Consortium. Can fecal continence be predicted in patients born with anorectal malformations? J Pediatr Surg. 2019 Jun;54(6):1159-1163. doi: 10.1016/j.jpedsurg.2019.02.035. Epub 2019 Mar 1 PMID: 30898398 

  • Abstract

    Purpose: The purpose of this study was to identify factors associated with attaining fecal continence in children with anorectal malformations (ARM).

    Methods: We performed a multi-institutional cohort study of children born with ARM in 2007-2011 who had spinal and sacral imaging. Questions from the Baylor Social Continence Scale were used to assess fecal continence at the age of ≥4 years. Factors present at birth that predicted continence were identified using multivariable logistic regression.

    Results: Among 144 ARM patients with a median age of 7 years (IQR 6-8), 58 (40%) were continent. The rate of fecal continence varied by ARM subtype (p = 0.002) with the highest rate of continence in patients with perineal fistula (60%). Spinal anomalies and the lateral sacral ratio were not associated with continence. On multivariable analysis, patients with less severe ARM subtypes (perineal fistula, recto-bulbar fistula, recto-vestibular fistula, no fistula, rectal stenosis) were more likely to be continent (OR = 7.4, p = 0.001).

    Conclusion: Type of ARM was the only factor that predicted fecal continence in children with ARM. The high degree of incontinence, even in the least severe subtypes, highlights that predicting fecal continence is difficult at birth and supports the need for long-term follow-up and bowel management programs for children with ARM.

    Type of study: Prospective Cohort Study.

    Level of evidence: II.

    Cite

    Minneci PC, Kabre RS, Mak GZ, Halleran DR, Cooper JN, Afrazi A, Calkins CM, Corkum K, Downard CD, Ehrlich P, Fraser JD, Gadepalli SK, Helmrath MA, Kohler JE, Landisch R, Landman MP, Lee C, Leys CM, Lodwick DL, McLeod J, Mon R, McClure B, Rymeski B, Saito JM, Sato TT, St Peter SD, Wood R, Levitt MA, Deans KJ; Midwest Pediatric Surgery Consortium. Can fecal continence be predicted in patients born with anorectal malformations? J Pediatr Surg. 2019 Jun;54(6):1159-1163. doi: 10.1016/j.jpedsurg.2019.02.035. Epub 2019 Mar 1 PMID: 30898398 

Screening Practices and Associated Anomalies in Infants with Anorectal Malformations: Results from the Midwest Pediatric Surgery Consortium.  

Minneci PC, Kabre RS, Mak GZ, Halleran DR, Cooper JN, Afrazi A, Calkins CM, Downard CD, Ehrlich P, Fraser J, Gadepalli SK, Helmrath MA, Kohler JE, Landisch R, Landman MP, Lee C, Leys CM, Lodwick DL, Mon R, McClure B, Rymeski B, Saito JM, Sato TT, St Peter SD, Wood R, Levitt MA, Deans KJ; Midwest Pediatric Surgery Consortium. Screening practices and associated anomalies in infants with anorectal malformations: Results from the Midwest Pediatric Surgery Consortium. J Pediatr Surg. 2018 Jun;53(6):1163-1167. doi: 10.1016/j.jpedsurg.2018.02.079. Epub 2018 Mar 7. PMID: 29602552

  • 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.

    Cite

    Minneci PC, Kabre RS, Mak GZ, Halleran DR, Cooper JN, Afrazi A, Calkins CM, Downard CD, Ehrlich P, Fraser J, Gadepalli SK, Helmrath MA, Kohler JE, Landisch R, Landman MP, Lee C, Leys CM, Lodwick DL, Mon R, McClure B, Rymeski B, Saito JM, Sato TT, St Peter SD, Wood R, Levitt MA, Deans KJ; Midwest Pediatric Surgery Consortium. Screening practices and associated anomalies in infants with anorectal malformations: Results from the Midwest Pediatric Surgery Consortium. J Pediatr Surg. 2018 Jun;53(6):1163-1167. doi: 10.1016/j.jpedsurg.2018.02.079. Epub 2018 Mar 7. PMID: 29602552