Treating Hirschsprung disease
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The development of Hirschsprung's disease in children ( intestinal aganglionosis) associated with congenital absence of nerve ganglia in the distal parts of the digestive tract - rectum, sigmoid and colon. Moreover, it was noted that boys are five times more likely to suffer than girls are. The disease is hereditary and is associated with a mutation in chromosome 10.
What are the symptoms of Hirschsprung's disease?
When Hirschsprung's disease there is the inability to relax the affected area. It is characterized by a constant expression of muscle spasm. In addition, it lacks peristalsis, making it impossible to advance the intestinal contents through the intestine. Bowel above the affected area expands due to accumulation of feces and atrophy of muscle fibers.
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The forms of the disease
The acute form of Hirschsprung's disease occurs when the total and subtotal lesion of the colon. It manifested in the first days of a child's life. It is characterized by low intestinal obstruction - no discharge of meconium, bloating, vomiting.
Sub-acute form of the disease occurs when the shorter zone aganglionosis. It is characterized by persistent constipation, stool only after the enema, abdominal enlargement, anxiety, retarded neuropsychological and physical development.
The chronic form develops only in lesions of the rectum. Constipation occurs more frequently in children at an older age. At the beginning of constipation short-lived and after cleansing enemas acts of defecation can be realized autonomously. With the progression of the disease fecal stones can be formed and obstructive ileus appears . Such children have reduced immunity, they are significantly more likely to fall ill then their peers. A characteristic feature of the disease is increasing of abdominal size due to the increase of colon size.
Diagnosis of the disease
Diagnosis of Hirschsprung's disease is based on:
- Clinical data;
- X-ray examination (hold lower gastrointestinal series);
- sphincterometry (research tone rectal sphincter);
- morphoimmunohistochemical method (determining the presence or absence of nerve ganglia in the submucosa of all sections of the colon).
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Methods of treatment.
Treating of Hirschsprung's disease is only operative.
In the acute form of the disease surgery is performed immediately after diagnosis. The operation is divided into two stages - the first stage, removing part of the colon (stoma) in the abdominal wall above the affected area, and the affected area is sewn up tight. The second phase of the operation is performed in 1-1.5 years.
In Belarus, several methods are used when performing surgical interventions in Hirschsprung's disease:
According to Duhamel, Soave, Svenson. These operations are reduced to the removal of the aganglionosis zone and anastomosis between a healthy bowel and anal sphincter. The disadvantage of these operations is their high traumatism and long duration of operations. They also leave behind scars on the anterior abdominal wall. The least traumatic and cosmetically justified is surgery to correct a congenital malformation - one-stage transanal endorectal resection of rectosigmoid intestine.
The course of operations is as follows: stretches the anal sphincter, and then a little bit above the dentate line (above the projection of the external anal sphincter) stands straight and sigmoid colon up to the healthy portion of the intestine above suprastenotic expansion. Then clipping of the selected portion of the intestine is made, followed by anastomosis between the mucosa of the rectum and sigmoid colon wall. This type of surgical treatment of Hirschsprung's disease in children has more advantages: the operation is much shorter, does not remain scars on the abdominal wall, the postoperative period passes more smoothly, the children are getting better.
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The Republican Scientific Practical Center of Pediatric Surgery - leading medical centers of Belarus, - surgery is professionally performed for Hirschsprung's disease. You will find not only high-quality medical care, but also the attention and care on the part of employees.
Republican Scientific Practical Center of Pediatric Surgery specialists have extensive experience in carrying out successful operations for Hirschsprung's disease. Leave your request in the form below or call +375 (44) 596-6-596: we will answer your questions, help you to apply for treatment at the clinic, and, if necessary, help with transfer, finding temporary housing and other issues associated with the visit to Belarus.