Hirschsprung’s disease

What is Hirschsprung’s disease?

Hirschsprung’s disease (or agangliosis) is a congenital absence of intestinal ganglia (nerve clusters) in the rectum, sigmoid, and large intestine. Ganglia are responsible for peristalsis, the movement of the intestines, which propels its contents downward towards the anal canal. Agangliosis leads to chronic constipation.

Forms of the disease:

  • Rectal: affects the rectum
  • Rectosigmoid: with involvement of the sigmoid colon
  • Segmental: with one or two affected segments separated by a healthy portion
  • Subtotal: involving the large intestine
  • Total: involving the entire large intestine and part of the small intestine

Why does Hirschsprung’s disease occur?
The exact causes of the disease are not fully understood. There are two theories regarding its onset, both related to fetal development:

  1. Abnormal migration of cells that later become ganglia.
  2. Ganglia die due to inadequate blood supply.

How to recognize Hirschsprung’s disease in a child:

  • Delayed passage of meconium (the first stool), which should normally occur within the first 24-48 hours of life.
  • Abdominal distension and green vomiting in newborns.
  • Constipation from birth: defecation only with an enema, laxatives are ineffective.
  • Enlarged abdomen, poor gas passage, absence of stool staining during constipation.

How is Hirschsprung’s disease diagnosed in children?

  • Irrigography: X-ray examination with contrast material.
  • Biopsy: taking a sample of the intestine and examining it under a microscope.
  • Anorectal manometry: measuring pressure in the rectum and anal canal.

When diagnosing intestinal diseases in children, we rely on advanced international recommendations and never prescribe unnecessary tests.

What happens if Hirschsprung’s disease is left untreated?

The most dangerous complication of the disease is Hirschsprung-associated enterocolitis – inflammation of the intestine. Its symptoms include:

  • High fever
  • Lethargy
  • Liquid, green, foul-smelling stool

Enterocolitis: Risk groups include boys (four times more likely than girls), children with Down syndrome, and children who have previously had enterocolitis.

Is there conservative treatment available?

The disease can only be cured through surgery. However, before the operation, the doctor may provide recommendations on how to alleviate the child’s condition with Hirschsprung’s disease: prescribe a softening diet, enemas, and symptomatic therapy.

What type of surgery is needed for Hirschsprung’s disease?
The general principle of all surgeries is the removal of the section of the intestine without ganglia. Sometimes, to facilitate intestinal function, temporary colostomy may be required – the diversion of the rectum through the abdominal wall.

How does rehabilitation and the postoperative period proceed?
Healing of internal stitches takes 6-7 days. By the 10th day, a digital rectal examination can already be performed.

Possible postoperative complications:

  • Enterocolitis
  • Constipation
  • Fecal incontinencevery.