Aganglionosis, total intestinal

MONDO:0008738

A complete lack of ganglia in the intestine. This is an extremely severe form of aganglionosis distinct from Hirschsprung Disease. This is an n-of-1 use case where only one patient or family has been described with this disorder.

Also known as: aganglionosis, total intestinal, total intestinal aganglionosis

1 clinical trial for this condition and its sub-types, 1 tagged with Aganglionosis, total intestinal itself.

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Where it sits in the disease tree

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↑ Hereditary disease (18235)

Including sub-types (1) Tagged with Aganglionosis, total intestinal (1)

Not yet recruiting 1

Robot-Assisted surgery showdown: which fix works best for rare bowel disease?

Disease control Not yet recruiting

This study compares two surgical techniques—Duhamel and Soave—for treating total colonic Hirschsprung disease, a severe birth defect where nerve cells are missing from the colon. Fifty children who had a first surgery as newborns will receive one of the two procedures. Researcher…

Sponsor: Zunyi Medical College • Aim: Disease control

Last updated Jun 27, 2026 08:11 UTC

Aganglionosis, total intestinal

Where it sits in the disease tree

Robot-Assisted surgery showdown: which fix works best for rare bowel disease?