Hirschsprung Disease in Children

What is Hirschsprung Disease in Children?

Hirschsprung disease is a congenital defect of the colon, which is manifested by the genesis of the ganglia of the submucosal and muscle-intestinal nerve plexuses.

A defect arises between the fifth and twelfth weeks of pregnancy. There are 4 options for the disease:

  • total intestinal agangliosis
  • total agangliosis of the large intestine
  • proximal segment agangliosis
  • agangliosis of the ultrashort distal segment

Hirschsprung disease is diagnosed in one newborn out of 5 thousand. Boys are sick more often. The ratio of male and female with this diagnosis is 4: 1.

Causes of Hirschspring Disease in Children

At the heart of Hirschsprung’s disease is a malformation of the distal colon – congenital agangliosis.

Pathogenesis during Hirschsprung Disease in Children

The child has no intramural nerves or is in deficit. Abnormal nerve fibers and ganglia are present. Conductivity in the neuro-reflex arcs is disturbed.

Secondary phenomena – changes in the submucous, muscular and mucous layer in the intestine. But the cause of the abnormal development of the nervous elements of the intestinal wall today is not exactly known.

Symptoms of Hirschsprung Disease in Children

Hirschsprung’s disease in children is manifested by a multitude of symptoms that occur in various combinations. The main symptom is chronic constipation. It can be observed including in newborns. The initial manifestations of constipation depend on the length of the aganglionic segment, the compensatory capabilities of the intestine, the nature of feeding.

With a short aganglionic segment of the feces in newborns is delayed by 1-3 days. You can use cleansing enema to eliminate this symptom. If the affected area is longer, intestinal obstruction occurs. Constipation in all cases becomes more prolonged.

The baby, while breastfeeding, can defecate normally due to the liquid consistency of feces and the functioning of non-affected parts of the large intestine. But during the introduction of lures or the transition to artificial feeding feces becomes less liquid, then increased constipation.

In older children, constipation can last at different times, which depends largely on the treatment. As a rule, constipation lasts 3-7 days. Incomplete emptying of the colon in some cases can lead to compaction and accumulation of feces in the form of fecal stones in the distal section. The appearance of stones can lead to acute intestinal obstruction.

A typical symptom of Hirschsprung’s disease in children is flatulence (gas accumulation). It manifests itself in the first day after birth. Chronic delay of feces and gases causes the expansion of the sigmoid, and sometimes other parts of the colon.

You may notice that the newborn form of the abdomen is not within the normal range. The cause is the above indicated dilation of the colon. At the same time, the navel is lower than usual or turned out. The belly is asymmetrical. Doctors with palpation find a large intestine that is full of feces.

Secondary changes arising from chronic fecal intoxication worsen with the maturation of the child or due to improper care for him. In many cases, hypotrophy and anemia appear. If the treatment is not carried out, the deformation of the chest is manifested: the costal arch is turned, its angle approaches the blunt one. The diaphragm is high. Fix the preload of the lungs, reduced respiratory surface. This sets the stage for recurrent bronchitis and pneumonia.

If there is no stool for a long time, intoxication or intestinal obstruction occurs, which, in turn, lead to the development of such a symptom as vomiting. This symptom is often characteristic of newborns and infants.

In older children, vomiting can talk about obturation or inversion of the intestine. Also, with vomiting in some cases, pain in the abdomen. With the appearance of such symptoms, it is time to take active actions, because they talk about complications – obstruction or peritonitis.

Often in children with Hirschsprung’s disease, diarrhea occurs. Such cases are described by foreign authors, in our country such a symptom was not observed with this disease. If diarrhea occurs in the first weeks after birth, it can be fatal.

Today, the symptoms of Hirschsprung’s disease have become more “mild.” Among the persistent manifestations is chronic constipation. But the shape of the abdomen does not always change.

Forms of Hirschsprung disease:

  • easy
  • average
  • heavy

Symptoms are divided into 3 groups:

  • early
  • late
  • complications

The earliest include flatulence, constipation, an increase in the circumference of the abdomen in a child. Late include hypotrophy, anemia, fecal stones and chest deformity. Symptoms of complications are: abdominal pain, vomiting, paradoxical diarrhea, etc. The group of late symptoms is not expressed in all cases.

Stages of Hirschspring’s disease in children:

  1. Compensated
  2. Subcompensated
  3. Decompensated

Diagnosis of Hirshprung Disease in Children

Diagnosis of this disease is carried out using radiographs. This method allows to reveal signs of dilatation of the proximal part of the large intestine with the formation of an aganglious cone, tapering towards the distal part, as well as violation of the barium evacuation.

Anorectal manometry is also used to prevent the internal sphincter from relaxing. To confirm the diagnosis, use a biopsy of the intestinal wall. Sometimes doctors may also prescribe a laparotomy.

Differential diagnosis should be the difference Hirschsprung’s disease in a newborn child from the meconium ileus with cystic fibrosis, malrotation, atresia or duplication of the intestine.

Treatment of Hirschspring Disease in Children

Only surgical treatment is applied. Otherwise, there may be early or late complications. Early complications include fistula, stenosis, enterocolitis, anastomosis; late – chronic constipation, fecal incontinence.

Treatment of total agangliosis is problematic.