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Review
. 2019 Oct;54(10):2017-2023.
doi: 10.1016/j.jpedsurg.2019.03.010. Epub 2019 Mar 21.

Guidelines for synoptic reporting of surgery and pathology in Hirschsprung disease

Affiliations
Review

Guidelines for synoptic reporting of surgery and pathology in Hirschsprung disease

Laura V Veras et al. J Pediatr Surg. 2019 Oct.

Abstract

Background/purpose: Synoptic, or standardized, reporting of surgery and pathology reports has been widely adopted in surgical oncology. Patients with Hirschsprung disease may experience morbidity related to surgical factors or underlying pathology and often undergo multiple operations. Our aim is to improve the postoperative outcome and care of patients with Hirschsprung disease by proposing a standardized set of data that should be included in every surgery and pathology report.

Methods: Members of the American Pediatric Surgical Association Hirschsprung Disease Interest Group and experts in pediatric pathology of Hirschsprung disease participated in group discussions, performed literature review and arrived at expert consensus guidelines for surgery and pathology reporting.

Results: The importance of accurate operative and pathologic reports and the implications of inadequate documentation in patients with Hirschsprung disease are discussed and guidelines for standardizing these reports are provided.

Conclusions: Adherence to the principles of reporting for operations and surgical pathology may improve outcomes for Hirschsprung disease patients and will facilitate identification of correlations among morphology, function, genetics and outcomes, which are required to improve the overall management of these patients.

Level of evidence: V.

Keywords: Aganglionosis; Enteric nervous system; Hirschsprung disease; Pathology; Surgery; Synoptic.

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Figures

Figure 1.
Figure 1.. Recommended operative report addendum for surgical reporting of HSCR.
The form provides checkboxes for surgical technique, including open versus minimally-invasive approaches, variations on ostomies, specific types of pull-through operations, and details of prior operations if a re-do surgery has been performed. A diagram to annotate locations of biopsies, placement of ostomies, and site of anastomosis is provided. Space is provided for detailing distances that are important to note, such as length of small bowel disease (relative to either the ileocecal valve or ligament of Treitz), anastomosis distance from the dentate line, and the length of muscular cuff (Soave) or retained aganglionic segment (Duhamel).
Figure 2.
Figure 2.. Recommended surgical pathology work-up for pull-through resection specimens.
Intraoperative frozen section evaluation of the entire circumference of the proximal surgical margin should be performed to exclude histological features of transition zone. An en face section of the margin can be frozen entirely either as a concentric ring (“donut”) or divided into linear sectors (easier to orient with large diameter specimens). The remaining bowel should be opened longitudinally and sampled by some variation of either of the two illustrated methods to include full-circumference samples of the proximal and distal ends, along with either a longitudinal strip or closely spaced (1–2 cm) transverse sections.

References

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