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Most of the constipation in the pediatric age belong to the category of functional disorders. That is, they are not secondary to any organic problem. But there is a small percentage of cases that are secondary to a narrowing in the most distal part of the colon. It is called Hirschprung's disease (HD).
HD (Hirschprung's disease) is a disorder is due to a neurological problem. Specifically, some cells present in the neural crest of the embryo called neuroblasts do not migrate correctly in this disease. They should be positioned close to the intestine, but they don't. Consequently, part of the large intestine does not develop properly, but instead becomes small. Or atrophic, as we doctors say.
This problem affects 1 / 2500-5000 newborns and sometimes is associated with the presence of more complex syndromes. Clinically, it causes delayed meconium evacuation, constipation at the expense of very small stools, marked abdominal distension, and poor weight and length gain.
For its diagnosis, several techniques can be used. As an imaging technique, it is of choice the opaque enema, in which we are going to identify a zone of narrowing in the colon, proximally to which we will see a dilation.
The functional test of choice is called manometry (in which we will detect an absence in the relaxation of the internal anal sphincter when pressing on that area). We will make the diagnosis of certainty through a colonic biopsy, where we will appreciate the already mentioned absence of nerve cells.
The treatment of HE is surgical, and consists of resecting the affected section and joining the remaining healthy sections.
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