Lightning Learning: Hirschsprung's Disease

Lightning Learning: Hirschsprung's Disease

Hirschsprung’s Disease is a fairly rare (1:5000) congenital condition which (normally) affects the large intestine.
— advice is based on local guidelines & procedures

What?

Hirschsprung’s Disease is a congenital condition which (normally) affects the large intestine (colon). It is fairly rare, affecting approximately 1 in every 5000 births.

Normally affects the rectum or sigmoid colon. Rarely it can affect entire colon. Causes problems with constipation which is not responsive to stool softeners/laxatives.

In extreme cases can lead to intestinal obstruction.

Why?

In utero the foetus does not develop the required ganglion cells (Aganglionosis) in the affected section of bowel. It is not fully understood why this occurs. There are certain genes that have been linked to Hirschsprung’s disease but not all children will express these mutations.

Symptoms include:

  • Constipation

  • Abdominal distention

  • Bilious vomiting

  • Not passing meconium in Day 1 of life

How?

  1. Diagnosis is made through a combination of thorough voiding history and ultimately a biopsy to confirm aganglionosis

  2. Consult with Surgeons

  3. Order abdominal x-ray. Sections of bowel will look distended and subsequent sections will look narrower than normal

  4. Symptomatic relief can be given via bowel washout

  5. Ultimately will require surgical correction with a Duhamel pull-through procedure

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