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Hirschsprung's disease
Hirschsprung's disease involves an enlargement of the colon, caused by
bowel obstruction resulting from an aganglionic section of bowel (the
normal enteric nerves are absent) that starts at the anus and progresses
upwards. The length of bowel that is affected varies but seldom
stretches for more than a foot or so.
This disease is named for Harald Hirschsprung, the Danish physician who
first described the disease in 1886.
Symptoms and signs
The disease is congenital, but occasionally symptoms do not appear until
early adulthood. In infants, the primary symptom is not passing meconium
within the first 24 to 48 hours of life.
Diagnosis
Definitive diagnosis is via rectal biopsy.
Treatment
Treatment is always surgical,
with resection of the offending area, often with a colostomy performed
before a second stage procedure to reanastomose the colon with the anus.
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