Dear Lactnetters, Hirschsprung's disease was mentioned recently and because
the son of one of my colleagues has this unfortunate problem, I wanted to
pass along some more information about it in regards to our fascinating poop
thread.

If the baby has Hirschsprung's disease (lack of innervation to the
colo-rectal part of the intestine), this can be a big problem from the
beginning, long before the baby starts solids. The intestine is a big
muscle. It is the muscular activity of the intestine that moves the poop
along through the intestine. Because of the lack of nerves to the intestine
with Hirschsprung's, the fecal material is not moved along. Hence, the baby
doesn't poop. Often there is a delay in the passage of meconium, followed by
*constipation*. The severity of the *constipation* may depend on the amount
of intestine that is lacking the proper innervation. It may be just a
centimeter of the rectal area or the entire large intestine.

This problem is *fixed* by surgical removal of the part of the intestine
which is lacking the needed innervation. Obviously, if the entire large
intestine is involved (very rare, but happens; the son of one of my
colleagues, unfortunately, has involvement of the entire colon), you still
have some problems after the *fixing*.

If the involved part of the intestine is not removed, the poop doesn't move
along and can back-up in back of the involved segment. This can result in a
situation called *toxic megacolon*. The colon basically gets huge just in
back of the involved segement from all of the poop that can't move along.
The bacteria that normally don't cause a problem in the gut are now in a
abnormal environment and cause enterocolitis.

Because of all of this, it is very important to consider this rare disorder
when dealing with *constipation* in babies. One of the key questions to ask,
is about the inital passage of meconium. Did it come out with 24 hours? If
so, Hirschsprung's is unlikely. If the baby is having infrequent copious
soft (normal) breastfed stools (the common pattern after the first couple
weeks; and apparently normal for some of our *outliers* from the first
week!), I would not be considering this diagnosis.

Mari Douma, DO
Dept Peds MSU

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