A 10-day-old boy was brought to the hospital by his parents due to severe green biliary vomiting. The parents thought it was strange that the child vomited frequently right after birth, but in the end, it felt like her stomach was swelling badly, so they went to see a doctor. The child was diagnosed with suspected congenital megacolon, an unfamiliar name.
Inability to pass stool, causing constipation and vomiting
Congenital megacolon, also called Hirschsprung’s disease, is a disease that causes symptoms such as constipation, vomiting, and abdominal distension by not being able to push stool toward the anus due to congenital inability to move the intestines smoothly.
The intestine moves food through contraction and relaxation movements. It is the intestinal ganglion cells that are involved in these intestinal movements. In the prenatal period, intestinal ganglion cells are created from the vicinity of the mouth through the small intestine and large intestine to the anus, and enteric ganglion cells are created from the vicinity of the mouth to the end of the intestine according to the order of each organ. This results in aganglia without enteric ganglion cells. About 80% of these aganglia occur mainly in the colon, the tip of the large intestine.
In the part where the ganglion cells are normally distributed, food moves well, but when it reaches the aganglia, where it does not have a bowel movement, it cannot move any more, and it stagnates and accumulates. As it accumulates and cannot be discharged, the upper intestine in the affected area is abnormally stretched, causing the symptoms of the megacolon.
If it is dismissed as simple constipation, it increases the disease and threatens life.
Congenital megacolon affects about 1 in 5000 people. Before birth, no specific problem is found, but symptoms appear as the bowel movement begins after birth. Usually, if the meconium is not discharged within 24 hours or the abdomen expands with vomiting, congenital megacolon is suspected. In addition, even if symptoms do not appear immediately after birth, symptoms such as persistent constipation, green vomiting, and abdominal discomfort may appear around 3 months after birth.
If the lesion area without intestinal ganglion cells is short, it does not show any special symptoms in newborns, but it may appear only after 2 or 3 years of age. can grow So, if the child’s constipation is severe, it is necessary to suspect congenital megacolon at least once.
If the treatment time is missed, gas and feces may continuously accumulate in the intestine, leading to bacterial growth and enteritis. In severe cases, it can progress to sepsis and be life-threatening, so early detection and treatment are essential.
Surgery required, prognosis is good
When congenital megacolon is suspected, the condition of the small intestine and large intestine is checked through abdominal X-ray examination and colonography. If an abnormally stretched area is identified, a rectal biopsy is performed. This is a test method to check the presence of ganglion cells by collecting some tissue from the rectal area through the anus. If there are no ganglion cells in the area, congenital megacolon can be confirmed.
Surgery is essential for treatment. Surgery is performed considering the location and length of the lesion without intestinal ganglion cells and the condition of the patient. Most cases where the aganglia are located in the colon can be cured with a single operation. However, if the aganglia are located above the colon rather than the colon, the operation may be more complicated, and in some cases, more than one operation may be required.
Surgery is important for congenital megacolon, but management of bowel obstruction after surgery is very important. This is because even children who have undergone surgery may experience constipation or constipation more easily than normal children. Therefore, it is necessary to regularly consult with a specialist through outpatient surgery after surgery.
Professor Oh Chae-yeon of the Department of Pediatrics at Korea University Ansan Hospital said, “Constipation is a common bowel disorder, so even if a child shows persistent constipation symptoms, it is often overlooked. If symptoms such as abdominal distension, vomiting, and severe diarrhea are accompanied by constipation, it is necessary to consult a pediatric surgeon. If the meconium discharge is delayed, and there is an infant under 1 year old who is usually very full, or a 2 or 3 year old infant with severe constipation, it is necessary to suspect this disease at least once.”
