Hirschsprung's disease in children: symptoms, treatment, surgery

Hirschsprung's disease refers to a rare disease. It manifests itself most often in 95% of cases in infants, but can occur in older children and adults.

With this illness the patient has a pathology of the nervous system of the colon. In a healthy colon are located on the walls of the special nerve plexus, which provide the ability of the colon to push in the right direction contents. Such nerve plexus located in the muscle layer of the intestine and its podslizitoy portion.

Hirschsprung's disease is manifested in male patients on Statistics 4 times more often than girls. This disease is not inherited, but the risk of manifestation of its symptoms in children with parents over an average of three per cent.

What it is?

Hirschsprung's disease - an anomaly of the colon of congenital etiology, leading to disruption of innervation fragment intestine (congenital aganglioz) - manifests persistent constipation.

In neonates the clinical picture is distinctive and varied - is related to the length and height of the location (relative to the anus) aganglioza zone. The incidence of Hirschsprung's disease: 1: 5000 newborns.

Pathogenesis

Ganglion (a ganglion) cells - the common name of several species of large neurons in the gut that are responsible for communication between nerve structures. During ontogeny ganglion cells migrate from the upper intestine to its distal department.

Violation migration leads to the formation of ulcers aganglionarnogo portion (functional constriction zone) with reduced or completely absent innervation (the ability to make a progressive-contractile movement). In some cases, aganglionarny portion can expand abnormally, forming a megacolon, which is one of the most serious complications of Hirschsprung disease.

Spot localization aganglionarnogo portion is usually distal colon separated. In rare cases, there are forms of Hirschsprung's disease, in which aganglionarny segment is formed at the site of the splenic pole or developing aganglioz entire colon. In this form of the disease in patients observed a total paralysis of motility.

Symptoms of Hirschsprung's disease

Severity of symptoms of Hirschsprung's disease depends on age, length of lesion and history.

Characteristic symptoms:

1. Periodically there is diarrhea.
2. There is flatulence. In the intestine accumulate gases.
3. Constipation. This is the main feature of the disease. In some children, the chair can not be seen for several weeks. In severe course of disease defecation process is generally impossible without an enema.
4. In newborn babies meconium (first bowel movement) departs late (to 2-3 day) or no leaves.
5. The kid is in pain in the gut. He is capricious and cries, pulls his knees to his tummy.
6. The abdomen becomes bloated form. Navel flattened or turned out. The abdomen becomes asymmetrical shape.
7. Intoxication. The child refuses to eat, not gaining weight. There may be a rise in temperature.
8. When defecation depart "fecal stones" (thick solid stool). Complete emptying occurs. Bowel movements differ unpleasant odor. In appearance they resemble a thin tape.

If the anomaly does not cover the entire intestine, the symptoms in infants are blurred. The child breastfed stool different semi-liquid consistency. They are easily able to be displayed by the body.

The first problems arise at a time when the diet crumbs begin to introduce solid foods. Adult food leads to compaction of feces. There is intestinal obstruction, observed symptoms of intoxication. At first, such a clinic can deal enema. In the future, this event does not bring relief.

Hirschsprung disease stage

Given the dynamics of the disease and symptoms mutating doctors distinguish such disease stages:

• Compensated - constipation celebrate childhood, cleansing enemas can easily remove it for a long time
• Subcompensated - enema gradually become less and less effective, the patient's condition worsens: body mass decreases, and worried about the severity of abdominal pain, shortness of breath; The expressed anemia, metabolic disorders. Subcompensation condition occurs in patients with decompensation on background of conservative treatment
• Decompensated - cleansing enemas and laxatives rarely lead to a complete bowel movement. There is a feeling of heaviness in the lower abdomen, bloating. Under the influence of various factors (sharp change of power, heavy exercise) in patients developing acute intestinal obstruction. Children decompensation often observed with subtotal and total forms of destruction.
• The acute form of Hirschsprung disease is manifested in neonates as low congenital bowel obstruction.

For Hirschsprung's disease, unlike other causes of constipation (tumor, atonic constipation older, in ulcerative colitis), characterized by the appearance of constipation since birth or early childhood.

Often parents notice the presence of endocrine, psychiatric and neurologic abnormalities. The disease may be associated with other congenital anomalies: Down syndrome, cleft soft palate.

complications

Congenital malformation of Hirschsprung often results and the development of various complications. These include the group:

1. Anemia.
2. "Fecal" intoxication. This complication is the result poisoning organism that accumulate toxic substances.
3. Development of acute enterocolitis. With the rapid advance of such inflammation may lead to death.
4. "Fecal stones' education. Thus formed solid mass such that they can compromise the integrity of the intestinal wall.

You can not assume that an innate malformation of the intestine itself will take place. Further the quality of life of a child and his lack of change in the whole body depends on the timely treatment of disease.

Treatment of Hirschsprung's disease

Treatment of the disease is ineffective conservative methods such remedial measures is usually prescribed as a preparation for surgery. The only effective method of treatment Hirschsprung's disease - surgery. In each case, an individual approach and considered three options for the treatment of:

1. The use of conservative methods of treatment of chronic constipation for as long as not resolved the issue of expediency of surgical intervention.
2. Delaying operations, which use the method of imposing a temporary colostomy
3. Emergency surgery

Based on years of experience, the surgeons believe that such an operation is optimum age of 12-18 months. But if conservative treatment there is proof the compensation process, the intervention can be delayed up to 2 -4 years of age.

It is also worth considering if transfer operation and to delay its implementation, in the patient's body begins adverse secondary changes: increasing the threat of enterocolitis, dysbiosis, disorders of protein exchange. Therefore, it is important to the doctor to grasp the precise point when it is the optimal age limit for surgery, and yet striking manifestations of secondary changes.

Conservative treatment

The main purpose of this therapy - is a constant cleansing of the bowel. To do this, doctors recommend taking the following actions:

• Diet. She had just appointed a doctor. Selecting stop on products that have a laxative effect: vegetables (carrots, beets), fruits (plums, apples), cereals (oatmeal, buckwheat), dairy products (fermented baked milk, yogurt).
• Physical exercise. The doctor will recommend a special gymnastics. It stimulates peristalsis and helps to strengthen the press.
• Massage. Daily carried lower abdomen massage. It stimulates the natural peristalsis and bowel cleansing.
• Vitamins. Strengthen the body and protect against the negative effects of toxic substances.
• Cleansing enemas. A child needs constant cleansing of the bowel.
• Probiotics. Medications to stimulate bowel function and saturate it useful microflora.
• Protein preparations. Electrolyte solutions are administered intravenously. They are recommended for increased symptoms of eating disorders.

In the case of conservative treatment methods do not give the desired effect or the disease is in an advanced stage, the patient is prescribed surgery.

Surgery

After a series of surveys surgeon decides, in what time frame and how to do the operation. It should be conducted in the most favorable period, but not during exacerbations. What is the surgical technique? It eliminates the affected segment, and then connect healthy sections of the colon. The operation can be of two types:

1. Simultaneous. Aganglionarnaya truncated area of ​​the colon. A healthy portion of the colon immediately connects to the rectum.
2. Dvuhmomentnaya. First there is a truncation, then colostomy (colon portion is displayed on the surface of the abdomen). Fecal diverted into a special container - Colostomy bags. After the adaptation period, the second portion is carried out the operation: connect healthy parts truncated intestine.

All patients undergoing surgery must undergo medical check-up, to assess functional outcome of treatment and detection and correction of late complications of surgery. Recommended every week for a month after surgery to undergo a medical examination and consultation. Then, throughout the year examinations can be carried out every 3 months.

Hirschsprung's disease in children is quite favorable prognosis. Most of the operations to remove the defective portion of the colon are no further complications, but Only if the parents or the child himself will monitor compliance with the recommendations of the doctor and undergo periodic inspections.