Suction Rectal and Full Thickness Biopsies
RECTUM, *** CM, BIOPSY:
Normal:
– Ganglionated colonic mucosa/submucosa with no significant abnormality
Hirschprung Disease:
– Aganglionic colonic mucosa/submucosa with **** neural hypertrophy
COMMENT: The rectal biopsy from *** cm shows aganglionic colonic mucosa and submucosa. There is associated neural hypertrophy. A calretinin immunohistochemical stain does not demonstrate any neural elements within the mucosa. Overall, these findings are consistent with Hirschsprung’s disease. Clinical correlation is recommended.
Transition Zone:
Although it is transitioning from aganglionic to ganglionated tissue, the surgical anastomosis should be performed proximal to this region.
– Ganglionated colonic mucosa/submucosa with neural hypertrophy, consistent with transition zone
COMMENT: The rectal biopsy from *** cm shows ganglionated colonic mucosa and submucosa. However, there is associated neural hypertrophy consistent with sampling of the transition zone. A calretinin immunohistochemical stain demonstrates neural twigs within the mucosa. Clinical correlation is recommended.
Physiologic Aganglionic/Hypoganglionic Zone (Distal 2 cm of rectum):
– Aganglionic anorectal squamocolumnar junctional mucosa, consistent with physiologic hypoganglionic zone (see comment)
COMMENT: The presence of squamocolumnar junctional mucosa indicates biopsy of the physiologic hypoganglionic zone located within 2 cm of the dentate line. This specimen is inadequate for the evaluation of Hirschsprung disease.
Inadequate specimen:
– Specimen insufficient for evaluation (see comment)
COMMENT: Insufficient submucosal tissue is present to evaluate for the presence of ganglion cells. Suggest consideration for repeat biopsy if cinical concern persists.
Colitis:
– Aganglionic colonic mucosa with acute inflammation and mucosal necrosis (see comment)
COMMENT: In addition to the absence of ganglion cells, acute colitis with cryptitis and mucosal necrosis are seen. These features raise concern for Hirschsprung-associated enterocolitis.
– Ganglionated colonic mucosa with increased eosinophils (see comment)
COMMENT: Scattered crypts are disrupted by numerous intraepithelial eosinophils (with up to *** eosinophils per single high-power field). This feature raises concern for an allergic proctocolitis. Clinical correlation is recommended.
“Pull-through” Resection for Hirschsprung Disease:
Here is a good video on how to gross these specimens
COLON, RECTO-SIGMOID (PULL-THROUGH RESECTION):
– Aganglionosis of distal *** cm of specimen, consistent with Hirschsprung disease
– Hypoganglionated colonic segment with neural hypertrophy, consistent with Transition Zone, in middle *** cm the specimen
– Well-formed submucosal (Meisner) and myenteric (Auerbach) plexi with ganglion cells in proximal *** cm of specimen (see comment)
COMMENT: Histologic sections of the entirely submitted rectosigmoid segment demonstrate distal aganglionosis consistent with Hirschsprung disease in the distal approximately *** cm of the specimen. Proximal to that, there is an approximately *** cm segment with hypoganglionosis and neural hypertrophy, consistent with the transition zone. The most proximal *** cm of the specimen contains well-formed ganglion cells within both neural plexi. The frozen section diagnoses have been confirmed.
Last updated: June 30, 2020