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Guidelines for synoptic reporting of surgery and pathology in Hirschsprung
disease, Journal of Pediatric Surgery
Adapted from: L.V. Veras, M. Arnold, J.R. Avansino, et al., Guidelines for synoptic reporting of surgery and pathology in Hirschsprung
disease, Journal of Pediatric Surgery, https://doi.org/10.1016/j.jpedsurg.2019.03.010
Some useful references and figures for Hirschsprung pull-throughs
A note about fibromuscular adventitial dysplasia:
Reference: Thaker, Ameet I; Kapur, Raj P. Pediatric and Developmental Pathology, 07/2018, Volume 21, Issue 4
Abnormal submuosal vessels characterized by excess adventitial smooth muscle (mature type AFD) or excess myofibroblasts and myxoid matrix (immature type AFD).
A note about eosinophilic (myenteric) ganglionitis:
Reference: Lowichik A, Weinberg AG. Ped Pathol Lab Med 1997;17:885-91
Reference: Kapur, R. Am J Surg Pathol. 40(12):1637-1646, December 2016.
Excess eosinophils within the myenteric plexus can be seen in association with changes in the aganglionic and transition zone of Hirschsprung disease.
When present, it is most often encountered in infants.
Rare cases of intestinal pseudo-obstruction have been ascribed to isolated eosinophilic ganglionitis and improved with immunosuppressive therapy raising the possibility that this pattern of inflammation may affect motility.
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Type of Pull-through procedure
Total length of resected Distal Aganglionic Segment
Total Length of Resected Proximal Ganglionic Bowel (if applicable)