Hirschsprung disease is a congenital aganglion of the large intestine. The patient does not have nerve cells in the submucous plexus of Meissner and the muscle plexus of Auerbach. Due to the absence of any contractions in the affected area and prolonged stagnation of stool in other departments, there is a significant lengthening and widening of the gut.
Symptoms of Hirschsprung's disease
The earliest symptoms of Hirschsprung's disease are flatulence, constipation and an increase in the circumference of the abdomen. If the patient does not consult a doctor, late signs begin to appear. These include:
- anemia ;
- hypotrophy;
- fecal stones.
In some cases, patients experience pain in the abdomen, whose strength may increase as the duration of constipation increases.
Stages of Hirschsprung disease
The syndrome of Hirschsprung develops in several stages. The very first stage of the disease is compensated: the patient has constipation, but for a long time, various cleansing enemas very easily eliminate it.
After this, a subcompensated stage occurs, during which the patient's condition worsens and enemas become less effective. At this stage in the development of Hirschsprung's disease in adults, the body weight decreases, they are troubled by the heaviness in the abdomen and shortness of breath. In some cases, severe anemia and metabolic disorders are noted.
The next stage of the disease is decompensated. Patients are no longer helped by cleansing enemas and various laxatives. He still has a feeling of heaviness in the lower abdomen, and also intestinal obstruction quickly develops.
Diagnosis of Hirschsprung's disease
In case of suspicion of Hirschsprung's disease, rectal examination is carried out first. In the presence of the disease, an empty ampoule of the rectum is found in the patient. The tone of the sphincter is increased. In this case, it is necessary to undergo a survey radiograph of all the organs of the abdominal cavity. With Hirschsprung's disease, the colon loops are enlarged and inflated, sometimes detecting fluid levels.
The patient also needs to undergo a sigmoidoscopy, irrigography, colonoscopy and histochemical diagnostics.
Treatment of Hirschsprung's disease
The only way to treat Hirschsprung's disease is surgery. The main objectives of the operation are:
- the liquidation of all extended departments;
- removal of the aganglionic zone;
- preservation of the part of the large intestine that functions.
For children, Swanson's, Duhamel's and Soave's radical operations have been developed. Adults their performance in the usual form is contraindicated because of anatomical features and severe sclerosis in the muscular or submucous membranes of the intestine. Most often, with Hirschsprung's disease, Duhamel's operation is modified, in which the aganglionary zone is removed with the creation of a short stump of the rectum. In most cases, it is possible to prevent damage to the sphincter of the anus and form a colorectal anastomosis.
Before surgery, the patient needs
The overall prognosis for Hirschsprung's disease after surgery is relatively favorable. But in some cases, repeated surgery may be required. Almost always the essence of such an operation is reduced to the reconstruction of the anastomosis and it is carried out through the peritoneal or perineal access.