Hosting 728 files, 2951 images, and 4 contributors.

Random Case

Osteoid Osteoma
Joseph Junewick, MD FACR
over 11 years ago
Enter A Workflow
Standard2   Academic2

Please choose a workflow. A standard workflow allows you to browse the repository with full case detail; the academic workflow allows you to browse the repository with limited case detail revealed. Double click on the images to launch image viewer.

ARSt Case Repository

Hirschprung Disease

Case Detail

Anatomy: Gastrointestinal
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 9 years ago
Updated: over 9 years ago
Tags: PEDS
Modality/Study Types: CR FL
Adobe32 PDF Imagej32 ImageJA


1 day old with trisomy 21 and emesis.

Case Images


Hirschprung Disease

Clinical Notes

Pathology – No ganglion cells on rectal biopsies.


CR – Moderate diffuse dilation of bowel; no rectal air. Note the left colonic pneumatosis on the photographic magnification of the abdominal radiograph.

FL – Water-soluble contrast enema performed one week after the abdominal radiograph demonstrates transition zone in the sigmoid colon.


Defective distal migration of vagal neural crest cells results in absent parasympathetic innervation (intramuscular Auerbach and submucosal Meissner plexi) and subsequent inability of bowel relaxation. This inability of relaxation creates a functional distal obstruction.

Short segment (distal to rectosigmoid junction) aganglionosis accounts 90% of Hirschprung disease and has a strong male predominance. Long segment disease occurs more often in caucasians although the male predominance is less. Hirschprung disease occurs in 2-16% of patients with trisomy 21; other syndromes with increased incidence of Hirschprung disease include Waardenburg, Goldberg-Shprintzen, Mckusick-Kauffman, Bardet-Biedl, Haddad, and Currarino.

The pneumatosis in this case is a manifestation of enterocolitis and represents a complication of Hirschprung disease. Enterocolitis is an important cause of comorbidity in Hirschprung disease and is related to stasis and bowel dilation, delayed diagnosis, and long segment disease.


Hernanz-Schulman M. The Colon – Normal Anatomy and Congenital Disorders, in Caffey’s Pediatric Diagnostic Imaging, 11th ed (2008).

Corporate Office: 616.363.7272, 3264 North Evergreen Drive, Grand Rapids, MI 49525

Spectrum Health Helen Devos Childrens Hospital GE HealthCare