Hirschsprung's disease

Hirschsprung's disease is caused by an aganglionic segment of bowel due to a developmental failure of the parasympathetic Auerbach (myenteric) and Meissner (submucosal) plexuses. Although rare (occurring in 1 in 5,000 births) it is an important differential diagnosis in childhood constipation

Presentations

• symptoms usually present within first two months of life and usually within the first week after birth
• neonatal period: failure or delay to pass meconium, megacolon, abdominal distension
• older children: constipation, abdominal distension

Associations

• 3 times more common in males
• Down syndrome

Treatment

• Resection of affected bowel segment