Hirschsprung disease entities

Clinic

• HD or HSCR is a birth defect in which nerves are missing from parts of the intestine.
• The most prominent symptom is constipation.
• Other symptoms may include vomiting, abdominal pain, diarrhea and slow growth.
• Symptoms usually become apparent in the first two months of life.
• Complications may include enterocolitis, megacolon, bowel obstruction and intestinal perforation.
• The disorder may occur by itself or in association with other genetic disorders such as Down syndrome or Waardenburg syndrome.
• About half of isolated cases are linked to a specific genetic mutation, and about 20% occur within families. Some of these occur in an autosomal dominant manner. The cause of the remaining cases is unclear.

Hirschsprung vs intussusception

The key difference between intussusception and Hirschsprung disease is that in intussusception disease, a part of the intestine slides into an adjacent part of the intestine and causes an internal obstruction, while in Hirschsprung disease, some nerve cells in the large intestine don’t develop properly.

Intestinal obstruction is a blockage that keeps liquid matter from passing through the small intestine or large intestine to the rectum. Intussusception and Hirschsprung disease are two different types of diseases that are caused due to intestinal blockage. Moreover, they are predominantly observed in children.

CONTENTS

1. Overview and Key Difference

2. What is Intussusception

3. What is Hirschsprung Disease

4. Similarities – Intussusception and Hirschsprung Disease

5. Intussusception vs Hirschsprung Disease in Tabular Form

6. Summary – Intussusception vs Hirschsprung Disease

What is Intussusception?

Intussusception is a serious condition where a part of the intestine slides into an adjacent part of the intestine. In this condition, a segment of the large intestine is telescoped into an adjacent lower segment. This telescoping frequently blocks food or fluid from passing through the intestine. In addition, intussusception cuts off the blood supply to the part of the intestine that is affected. This medical condition can lead to a tear in the bowel, infection, and death of the bowel tissue (necrosis). The symptoms of intussusception may include loud crying due to abdominal pain, a tool mixed with blood and mucus (jelly stool), vomiting, a lump in the abdomen, weakness or lack of energy, and diarrhoea.

Figure 01: Intussusception

The causes of intussusception may include idiopathic reasons, a polyp, tumor, scar-like tissue in the intestine (adhesions), weight loss surgery or other surgery on the intestinal tract, and inflammation due to diseases such as Crohn’s disease. Moreover, intussusception can be diagnosed through medical history, physical examination, and abdominal imaging like ultrasound, X-ray, and CT-scan. Furthermore, intussusception can be treated through a water-soluble contrast or air enema and surgery.

What is Hirschsprung Disease?

Hirschsprung disease is a condition that causes intestinal obstruction due to defects in some nerve cells in the large intestine. This condition is present at birth as a result of missing nerve cells in the muscles of the baby’s colon. Without these nerve cells stimulating the gut muscles, it’s difficult for content to move through the intestine. In Hirschsprung disease, the content of the intestine backs up and causes blockages in the bowel. The sign and symptoms in newborns suffering from Hirschsprung disease include a swollen belly, vomiting, constipation or gas, diarrhoea, and delayed passage of meconium. The sign and symptoms in older children include chronic constipation, gas, and fatigue.

Figure 02: Hirschsprung Disease

Hirschsprung disease runs in families and is most probably caused by a genetic mutation. Moreover, Hirschsprung disease can be diagnosed through abdominal radiograph, colon tissue biopsy, abdominal X-ray by using a contrast dye, and anal manometry. Furthermore, Hirschsprung disease is treated through pull-through surgery, ostomy surgery, and lifestyle and home remedies (serve high fiber foods, increase fluids, encourage physical activities, and laxatives).

What are the Similarities Between Intussusception and Hirschsprung Disease?

• Intussusception and Hirschsprung disease are two different types of diseases that are caused due to intestinal blockage.
• Both conditions are predominantly observed in children.
• They can cause complications.
• Both conditions can be diagnosed through abdominal imaging.
• They are treated through surgeries.

What is the Difference Between Intussusception and Hirschsprung Disease?

In intussusception disease, a part of the intestine slides into an adjacent part of the intestine and causes an internal obstruction, while in Hirschsprung disease, some nerve cells in the large intestine don’t develop properly, causing an obstruction. Thus, this is the key difference between intussusception and Hirschsprung disease. Furthermore, intussusception disease can be caused by polyps, tumor,  scar-like tissue in the intestine (adhesions),  weight loss surgery or other surgery on the intestinal tract, and inflammation due to diseases such as Crohn’s disease. On the other hand, Hirschsprung disease is caused by a genetic mutation that runs in the family.

The below infographic presents the differences between intussusception and Hirschsprung disease in tabular form for side-by-side comparison.