Aganglionosis, total intestinal

Aganglionosis, total intestinal

Definition

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 — per MONDO

Also identified as

Affected Organs

Organ Involvement Source
Alimentary part of gastrointestinal system Disease Has Associated Anatomic Site NCIT · CC BY 4.0
Alimentary part of gastrointestinal system Disease Has Primary Anatomic Site NCIT · CC BY 4.0
Intestine Disease Has Associated Anatomic Site NCIT · CC BY 4.0
Intestine Disease Has Primary Anatomic Site NCIT · CC BY 4.0