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Hirschsprung's disease

Barium enema performed in a newborn with Hirschsprung's disease. Often, classical changes are not obvious in the neonatal period

Corman ML. Colon and rectal surgery. 5th ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2005:555-603; used with permission

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Hirschsprung's disease

Contrast enema may demonstrate mucosal irregularity in the aganglionic distal colon and may show a transition zone between smaller caliber aganglionic distal colon and dilated proximal ganglionic colon

From the personal collection of Lily Cheng, MD; used with permission

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3

Hirschsprung's disease

Abdominal x-ray image showing gaseous distension of the large bowel with air absent from the rectum (typical of Hirschsprung’s disease)

BMJ Case Reports 2012; doi:10.1136/bmj.e5521

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1

Hirschsprung's disease

Histological section, including mucosa with submucosa of the rectum showing clusters of ganglion cells in the submucosal plexus. This excludes Hirschsprung's disease at this level

Corman ML. Colon and rectal surgery. 5th ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2005:555; used with permission

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2

Hirschsprung's disease

Histological section, including mucosa and submucosa of the rectum showing tortuous and hypertrophic nerve trunks of the submucosal plexus. There is no evidence of any ganglion cell present. This establishes the diagnosis of Hirschsprung's disease

Corman ML. Colon and rectal surgery. 5th ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2005:555; used with permission

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5

Hirschsprung's disease

Contrast enema showing an abnormal rectosigmoid ratio (sigmoid diameter larger than rectal diameter)

From the personal collection of Lily Cheng, MD; used with permission

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7

Hirschsprung's disease

Hematoxylin and eosin showing ganglion cells in the myenteric plexus

From the personal collection of Lily Cheng, MD; used with permission

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8

Hirschsprung's disease

Contrast enema demonstrates the typical proximal dilation, transition zone, and non-distended, aganglionic portion

Corman ML. Colon and rectal surgery. 5th ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2005:555-603; used with permission

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