What is Hirschsprung's disease in children?

Children are the future of the motherland and the hope of the family. Every parent hopes to have a smart and healthy baby. The health of the child is of concern to the parents. Now that living standards have improved and nutrition is abundant, many babies will have gastrointestinal problems such as constipation and diarrhea. Many parents worry that their children will suffer from Hirschsprung's disease. So what exactly is Hirschsprung's disease? How to treat it?

Hirschsprung's discase is also known as aganglionosis of the intestinal tract. Because Hirschsprung described it in detail, it is usually called Hirschsprung's Disease. It is caused by continuous spasm of the intestinal tract at the distal end of the rectum or colon and fecal stasis in the proximal colon, causing hypertrophy and expansion of the intestine. It is a common congenital intestinal malformation in children. Hirschsprung's disease Hirschsprung's disease is a relatively common congenital gastrointestinal malformation with a certain familial genetic tendency.

Etiology and pathophysiology Currently, it is generally believed that congenital megacolon is caused by genetic and environmental factors, which is caused by temporary ischemia and hypoxia in the embryonic intestine under the condition of multi-gene genetic factors. The basic pathological changes are the lack of ganglion cells in the intestinal wall myenteric and submucosal nerve plexuses, and the increase in the number and thickening of unmyelinated parasympathetic nerve fibers, which is a developmental arrest.

Due to the absence of ganglion cells, abnormal function of sympathetic and parasympathetic nerves and lack of peptidergic nerves, the smooth surface of the pathogenic intestinal segment continues to contract and become spastic. Normal propulsive intestinal peristalsis disappears, forming functional intestinal obstruction and making it difficult to pass stool. The proximal end of the spastic intestinal segment gradually expands and thickens due to long-term accumulation of stool, forming megacolon. Another pathological change is the disappearance of the defecation reflex, which is due to defects in the visceral sensory motor nervous system of the intestinal wall, which cannot produce normal reflex rectal contraction and relaxation of the internal anal sphincter, resulting in the inability to excrete feces. In the neonatal period, the colon wall is very thin, the distal intestine and colon are dilated, and the whole abdomen is bulging. With age, the intestinal wall muscles strengthen due to long-term adaptation to the spastic intestinal segments.

The proximal colon close to the spasm segment encounters the greatest resistance and gradually expands and thickens to form a more localized megacolon. According to the length of the narrowed segment of the intestine, this disease can be divided into 5 types. In the neonatal period, the colon wall is very thin, the distal intestine and colon are dilated, and the entire abdomen is bulging. With age, the intestinal wall muscles strengthen. Due to long-term adaptation to the spastic tract segment, the proximal colon near the spastic segment is subject to the greatest resistance, gradually expanding and hypertrophying, forming a more localized megacolon.

Delayed meconium excretion, stubborn constipation, and abdominal distension: More than 90% of infants have no meconium or small amounts of meconium within 36-48 hours after birth, and need anal dilation or anal opening to defecate. They will then have stubborn constipation, and in severe cases, they will develop the condition of not needing enema or defecation. The longer the spasm period, the earlier constipation occurs and the more severe it is. Abdominal distension worsens day by day, the abdominal wall becomes tense, there is venous dilation, intestinal patterns and peristaltic waves are visible, bowel sounds increase, and the diaphragm rises, causing difficulty breathing. 2. Vomiting, malnutrition, and developmental delay: Due to functional intestinal obstruction, vomiting may occur, but the amount is not large. The vomitus contains a small amount of bile. In severe cases, fecal fluid may be seen. In addition, long-term abdominal distension and constipation will cause the child to have a decreased appetite, which affects nutrient absorption and leads to developmental delay, consumption, anemia, or hypoproteinemia with edema.

Conservative treatment: Oral laxatives and lubricants, such as honey, to help with bowel movements. Use chlorhexidine, glycerin suppositories, anal dilators, etc. to stimulate the sphincter and induce defecation. For enema, the concentration of the anal tube insertion should exceed the narrow section. Use normal saline for enema once a day, with an injection volume of 50-100 ml each time. The enema water and feces water will be discharged from the back of the abdomen. Repeat several times to gradually discharge the accumulated feces. Note that the injection volume should be basically equal. The vital signs of the child should be closely observed during the enema process. 2. Colostomy: Patients with megacolon and uncontrolled enterocolitis, malnutrition, high fever, anemia, abdominal distension, who cannot tolerate surgery, or those who do not respond to conservative treatment, and whose abdominal distension significantly affects their breathing, should undergo colostomy in a timely manner, and then undergo radical surgery when they are a little older. 3. Radical surgery: In recent years, many scholars have advocated early radical surgery, believing that patients with a body weight of more than 3 kg and in good general condition can undergo radical surgery. Radical treatment should be performed in children. Before surgery, cleansing enema should be performed for 10-14 consecutive days to correct dehydration, electrolyte imbalance and acid-base imbalance. Strengthen supportive therapy and improve general condition.

What is Hirschsprung's disease in children? After reading the above content, parents should understand what congenital megacolon is in children. If a newborn has abnormal bowel movements, it must be taken seriously. Don’t worry too much about simple constipation in children. Constipation in children is mainly caused by weak spleen and stomach and slow gastrointestinal motility. If other symptoms occur, go to the hospital for a careful examination, consult a doctor for a diagnosis, and follow the doctor's advice for treatment.