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Updated: Feb 7, 2026

Diagnosis of Hirschsprung's Disease by Immunostaining Rectal Suction Biopsies for Calretinin, S100 Protein and Protein Gene Product 9.5

Diagnosis of Hirschsprung's Disease by Immunostaining Rectal Suction Biopsies for Calretinin, S100 Protein and Protein Gene Product 9.5

Published on: April 26, 2019

近位空腸瘻造設術で管理された腸管全域ヒルシュスプルング病の新生児例

Farhana Ali-Geiger¹, Michael Traynor², Lauren Baumann²

¹Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, University of Utah, USA.

Intestinal Failure (New York, N.Y.)

|February 6, 2026

要旨

Background:

Hirschsprung disease (HD) is a congenital disorder of the enteric nervous system, and its management will differ clinically depending on the degree of aganglionosis. Total intestinal HD (TIHD) is a rare variant, accounting for < 1 % of all cases, and is defined as aganglionosis of nearly the entire intestine with less than 20 cm of ganglionated small bowel past the ligament of Treitz (LOT). TIHD can be challenging to manage with high risk for chronic intestinal failure.

Case Report:

Ours is a neonate confirmed pathologically to have total intestinal aganglionosis, surgically managed with a jejunostomy formed at 40 cm distal to LOT.

Conclusion:

Case of TIHD that has benefitted from a proximal diverting jejunostomy.

キーワード:

ヒルシュスプルング病空腸瘻造設術小児腸管不全全腸管無神経節症

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