Hirschsprung's disease

Description of Hirschsprung's disease

Болезнь Гиршпрунга – редкое врожденное заболевание. It affects nearly every 5000th neonate. Bowel obstruction disease ends and prevents normal bowel movements. Hirschsprung's disease occurs most often separately, but it can also be part of a syndrome.

Causes Hirschsprung's disease

Hirschsprung's disease is caused by the absence of certain nerve cells. These cells, called ganglia, They are found in the intestinal wall. They help relax the bowel wall, allowing feces to move through the colon. In children with this disease of the colon is nerasslablennoy, causing obstruction. The disease usually affects the past 30-50 centimeters of the colon, that ends with the rectum.

The absence of ganglia is due to a genetic defect. In some cases, Hirschsprung's disease is hereditary. It means, that parents can pass on to their children. This can occur, even if the parents do not have the disease.

Risk factors for Hirschsprung's disease

Factors, which increases the risk of Hirschsprung's disease:

• The presence of family members with Hirschsprung's disease;
• It is more common in men;
• The emergence of Hirschsprung's disease may be associated with other birth defects.

Symptoms of Hirschsprung's disease

Hirschsprung's disease is usually diagnosed in childhood, but it can also be detected later. Symptoms may vary with age.

Newborns

• Failure to pass meconium within the first 48 hours of life (Меконий – темное липкое вещество, which is the first bowel movement);
• Vomiting after eating;
• Abdominal distention.

In young children,

• Severe constipation;
• Diarrhea;
• Anemia;
• Stunting.

Adolescents

• Severe constipation for most of life.

Diagnosis of Hirschsprung's disease

In most cases, Hirschsprung's disease are diagnosed in infancy, although some cases were discovered during adolescence or young adulthood.

Tests for diagnosis may include:

• Бариевая клизма – впрыск раствора соли бария в прямую кишку. At the same time the intestines become visible on x-ray, that allows us to consider possible defects;
• Биопсия – удаление образца ткани кишечника, to check for ganglia (or lack thereof);
• Аноректальная манометрия – измерение давления внутреннего и наружного сфинктера с помощью ректального баллона или специального датчика.

Treatment of Hirschsprung's disease

Основное метод лечение для болезни Гиршпрунга – хирургическая операция, which removes the damaged part of the colon. There are three phases of surgery, but sometimes they do not need.

Phase surgery for Hirschsprung's disease:

• Колостомия – эта фаза предполагает создание хирургическим путем отверстия в толстой кишке, which is brought to the abdominal surface. Feces and intestinal contents are allocated through the hole.
• Удаление – пораженный участок толстой кишки удаляется и здоровая часть присоединяется к прямой кишке.
• Закрытие колостомы – колостома зашивается, and bowel function gradually returns to normal.

Results

Symptoms are eliminated in 90% children after surgery. The best result is connected with the beginning of early treatment.

Complications

Complications may include:

• Enterobrosia;
• Enterocolitis;
• Short gut syndrome.

Prevention of Hirschsprung's disease

Methods of preventing this disease does not exist.