Alberto Peña, MD, Colorectal Center

  • Total Colonic Aganglionosis

    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.

    Timing of Surgery

    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 diaper rash. 

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