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Total colonic aganglionosis is a type of Hirschsprung disease in
which nerve cells known as ganglion cells are lacking in the entire large
intestine and sometimes the small bowel. As a result, the large intestine
cannot move stool through and becomes blocked, causing the abdomen to swell. This is sometimes referred to as long segment Hirschsprung disease.
Treating total colonic aganglionosis involves surgically
removing the colon and reconnecting the lower end of the small intestine to the
beginning of the anal canal. Initially an anal ileostomy is performed so stool
empties into a bag.
A patch of aganglionic colon is used, but the patch can
cause a significant number of problems. These include irritation and
inflammation of the intestines, retention of secretions and stool in the patch,
and diarrhea from the secretions in the patch itself and in the rest of the
intestines. In some cases, the diarrhea is so severe that the patch has to be
removed. We have had to do this for several patients who had received the
patches in operations performed at other hospitals. This included patients who
lost a large volume of fluid, so much that it required liters of electrolyte
solution given intravenously every day to compensate for the losses. The moment
we resect the patch, the patients improve dramatically.
Because of these complications, we currently use a
straightforward anastomosis between the end of the small intestine and
beginning of the anal canal to treat patients with total colonic aganglionosis.
The optimal timing for the surgery is controversial. While
surgeons have demonstrated that the operation can be performed very early in
life, it may not be the best option for the patient in the long run. In our
opinion, the surgery should be performed when the patient is totally continent
for urine (able to exercise total control over urination). Performing the
surgery when the patient is continent will allow the patient to become toilet
trained for feces much faster and therefore the patient will suffer much less
For more information or to
request an appointment for the Peña Colorectal Center, contact us.
Get more information or request an appointment for the Peña Colorectal Center.
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