TY - JOUR
T1 - Determining the correct resection level in patients with Hirschsprung disease using contrast enema and full thickness biopsies
T2 - Can the diagnostic accuracy be improved by examining submucosal nerve fiber thickness?
AU - Beltman, Lieke
AU - Shirinskiy, Igor
AU - Donner, Naomi
AU - Backes, Manouk
AU - Benninga, Marc
AU - Roelofs, Joris
AU - van der Voorn, Patrick
AU - van Schuppen, Joost
AU - Oosterlaan, Jaap
AU - van Heurn, Ernst
AU - Derikx, Joep
N1 - Funding Information: This study was supported with a grant from the Dutch patient association for Hirschsprung disease. Publisher Copyright: © 2022 The Author(s)
PY - 2023/8
Y1 - 2023/8
N2 - Background: Intraoperative resection level in patients with Hirschsprung disease (HD) is determined by contrast enema, surgeon's intraoperative judgement and full thickness biopsy (FTB) identifying ganglia. This study aims to evaluate diagnostic accuracy of contrast enema and FTB in determination of resection level and whether this can be improved by measuring submucosal nerve fiber diameter. Methods: We retrospectively analyzed contrast enema and intraoperative FTBs obtained in our center, determining diagnostic accuracy for level of resection. Gold standard was pathological examination of resection specimen. Secondly, we matched transition zone pull-through (TZPT) patients with non-TZPT patients, based on age and length of resected bowel, to blindly compare nerve fibers diameters between two groups using group comparison. Results: From 2000–2021, 209 patients underwent HD surgery of whom 180 patients (138 males; median age at surgery: 13 weeks) with 18 TZPTs (10%) were included. Positive predictive value of contrast enema was 65.1%. No caliber change was found in patients with total colon aganglionosis (TCA). Negative predictive value of surgeon's intraoperative judgement and FTB in determining resection level was 79.0% and 90.0% (91.2% single-stage, 84.4% two-stage surgery) respectively. Mean nerve fiber diameter in TZPT was 25.01 µm (SD= 5.63) and in non-TZPT 24.35 µm (SD= 6.75) (p = 0.813). Conclusion: Determination of resection level with combination of contrast enema, surgeon's intraoperative judgement and FTB results in sufficient diagnostic accuracy in patients with HD. If no caliber change is seen with contrast enema, TCA should be considered. Resection level or transition zone cannot be determined by assessment of submucosal nerve fiber diameter in FTB. Type of Study: clinical research paper.
AB - Background: Intraoperative resection level in patients with Hirschsprung disease (HD) is determined by contrast enema, surgeon's intraoperative judgement and full thickness biopsy (FTB) identifying ganglia. This study aims to evaluate diagnostic accuracy of contrast enema and FTB in determination of resection level and whether this can be improved by measuring submucosal nerve fiber diameter. Methods: We retrospectively analyzed contrast enema and intraoperative FTBs obtained in our center, determining diagnostic accuracy for level of resection. Gold standard was pathological examination of resection specimen. Secondly, we matched transition zone pull-through (TZPT) patients with non-TZPT patients, based on age and length of resected bowel, to blindly compare nerve fibers diameters between two groups using group comparison. Results: From 2000–2021, 209 patients underwent HD surgery of whom 180 patients (138 males; median age at surgery: 13 weeks) with 18 TZPTs (10%) were included. Positive predictive value of contrast enema was 65.1%. No caliber change was found in patients with total colon aganglionosis (TCA). Negative predictive value of surgeon's intraoperative judgement and FTB in determining resection level was 79.0% and 90.0% (91.2% single-stage, 84.4% two-stage surgery) respectively. Mean nerve fiber diameter in TZPT was 25.01 µm (SD= 5.63) and in non-TZPT 24.35 µm (SD= 6.75) (p = 0.813). Conclusion: Determination of resection level with combination of contrast enema, surgeon's intraoperative judgement and FTB results in sufficient diagnostic accuracy in patients with HD. If no caliber change is seen with contrast enema, TCA should be considered. Resection level or transition zone cannot be determined by assessment of submucosal nerve fiber diameter in FTB. Type of Study: clinical research paper.
KW - Contrast enema
KW - Diagnostic accuracy
KW - Full thickness biopsy
KW - Hirschsprung disease
KW - Nerve fibers
KW - Pathologist
UR - http://www.scopus.com/inward/record.url?scp=85146930793&partnerID=8YFLogxK
U2 - https://doi.org/10.1016/j.jpedsurg.2022.08.019
DO - https://doi.org/10.1016/j.jpedsurg.2022.08.019
M3 - Article
C2 - 36180266
SN - 0022-3468
VL - 58
SP - 1463
EP - 1470
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
IS - 8
ER -