Hirschsprung’s disease or “congenital aganglionic megacolon” is a disease that occurs with the absence of nerve cells that allow the intestines to relax. The intestines move by contracting and relaxing. Since there are no cells that provide relaxation in this disease, the affected part of the intestine remains contracted. The resulting blockage prevents stool from passing downward. Intestinal obstruction that occurs in this way can only be treated surgically by removing the intestine without nerve cells and replacing it with the normal intestine. In 70-75% of patients, the “aganglionic segment” includes the 5-15 cm portion of the intestine just above the anus. In the remaining patients, the aganglionic area may extend to the upper parts of the large intestine. Rarely, the entire large intestine may be aganlionic. The absence of ganglion cells in all of the small and large intestines is incompatible with life.
The main principle in treatment is to disable the aganglionic part without cells and replace it with the ganglionic part with cells. In other words, the aganglionic section is removed in the surgery and the ganglionic section is replaced with the upper ganglionic section and connected to the anus or the intestine just above it (pull-through process).
Surgical procedure; It is performed in one, two or three stages, depending on the age of the child, the length of the aganglionic segment and the findings. Today, with the changing technological possibilities and intensive care conditions, these surgeries are usually performed in one or two stages. Treatment options in Hirschsprung’s disease:
Three-stage method: It is a method that was first described and still used today. Accordingly, in the first stage, the part of the large intestine with ganglion cells is temporarily attached to the abdominal wall. With this method, called colostomy, it is aimed to rest the enlarged intestinal segment and allow the child to easily remove the stool. In the second phase; By applying different methods, the aganglionic intestine without cells is removed or disabled and the ganglionic intestine with cells is withdrawn instead. In the third stage, the colostomy is closed and the treatment is completed.
Two-stage surgeries: In the first stage, the part of the large intestine with ganglion cells is temporarily attached to the abdominal wall. In the second phase; By applying different methods, the aganglionic intestine without cells is removed or disabled and the colostomy tip, which has cells inside, is pulled down.
Single-stage surgeries: It can be performed as an open surgical method or closed (laparoscopically). In both methods, the intestinal tissue with ganglion cells is identified by a rapid pathological examination (frozen) during the operation, and this area is pulled down to the anus, and the operation is completed.
Primary Transanal Pull_Through (Primary TEP): It is a new method that has been widely used in recent years. It is in the form of entering directly through the anus without opening the abdomen of the patient, pulling the aganglionic intestine out from here and connecting the ganglionic intestine to the anus. Compared with other methods, the fact that the abdomen is not entered and there is no trace on the abdominal wall is considered an advantage, but the long-term results of this method are not yet known.
The success of the outcome in these patients depends on the patient’s anatomical structure, comorbidities, if any, and family-patient compatibility (colostomy washing, compliance with the expansion program).
The duration of the operation is between 2-5 hours under normal conditions, and the chance of success is approximately 90%, depending on the patient, the length of the aganlionic bowel, and the presence and severity of additional anomalies seen in the patient. After the surgery, the patient is followed up in the clinic for approximately 2-7 days. During this period, the patient may not be fed orally and total parenteral nutrition (TPN) can be applied.
Problems that may be encountered in children with Hirschsprung’s Disease during recovery and in the long term:
Chronic constipation:
Chronic constipation is a troubling problem in approximately 6-10% of operated patients. it can continue. In addition, toilet training may take longer as these children do not know how to manage the muscles used during defecation. As the years pass, these problems become less and less. In these patients, both oral stool softeners and rectal enemas can be used for many years.
The most common cause of constipation in these patients is anal sphincter achalasia (the contraction of the defecation muscle), which benefits from dilation and sphincterotomy. If it persists despite this, a second surgery may be required.
Enterocolitis:
It occurs in approximately 12-58% of patients.
Children are particularly at risk of developing an intestinal infection (enterocolitis) within one year of surgery.
In its treatment, intravenous fluid and antibiotic therapy and rectal enemas are applied.
Be aware of the signs and symptoms of enterocolitis and consult your doctor if any of the following are observed:
Diarrhea
Bloody stools
Fever
Abdominal swelling
Vomiting with or without bile
Encopresis (weight loss of stool, weight soiling):
It occurs in approximately 12% of patients. It is due to chronic constipation and the accumulation of stool in the intestines. Particularly specific to Duhamel surgery, petrified stool fragments called fecaloma may be found in the rectum.
Its treatment is the same as in chronic constipation.
Other long-term problems:
Primary infertility, erectile dysfunction, azoospermia and psychosexual problems can be seen in approximately 10% of male patients operated for Hirschsprung’s disease.
