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Hirschsprung Disease
Medical Questions » Hirschsprung Disease
| Name: Hirschsprung Disease |
| Also known as: |
| A congenital disease of the large intestine which is far more common in boys than girls. |
Causes of Hirschsprung Disease
Caused by a failure of the nerves supplying the large intestine (colon) to develop correctly. Without these nerves, the intestine cannot contract to move along the faeces, and it collects to dilate the colon to an enormous size. There is a tendency for the disease to occur in successive generations. |
Symptoms of Hirschsprung Disease
Usually diagnosed soon after birth as the baby is severely constipated, has a distended belly, refuses to feed, is lethargic, small in size, and is very irritable. Foul smelling diarrhea may develop as a late symptom. |
Tests for Hirschsprung Disease
Diagnosis confirmed by an X-ray of the gut, and taking a biopsy of the colon. |
Treatment for Hirschsprung Disease
Initially, excess faeces is removed by a tube placed up through the anus, but in due course an operation to remove the affected section of gut is necessary.
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Complications of Hirschsprung Disease of its treatment
Massive overdilation of the large intestine (megacolon). |
Likely Outcome of Hirschsprung Disease
Without treatment most affected babies will die. After the operation, these children progress very well and have only minor long-term problems. |
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