Hirschsprung Disease

Hirschsprung Disease

• The rectosigmoid region of the colon is most commonly affected in Hirschsprung disease.

Clinical Presentation

• The first sign of Hirschsprung disease in newborns is often a failure to pass meconium in the first 24-48 hours after birth.

Total Colonic Hirschsprung Disease

• In total colonic Hirschsprung disease, the entire colon can be affected.

Medical Terminology

• Hirschsprung disease is also known as congenital aganglionic megacolon.

Types of Hirschsprung Disease

• The long-segment subtype of Hirschsprung disease is associated with an early and more severe onset of symptoms.

Characteristics

• A characteristic of Hirschsprung disease is the absence of ganglion cells in the myenteric plexus of the intestinal wall.

Symptoms

• The main symptom of Hirschsprung disease in newborns is a delay in passing meconium.

Pathophysiology

• The length of the aganglionic segment is directly correlated with the onset of symptoms in Hirschsprung disease, with longer segments associated with earlier and more severe symptoms.