Recognizing congenital megacolon

  If the ganglion cells that innervate the intestine, especially the colon, do not develop during embryonic development, the intestine is unable to move and empty properly, resulting in fecal retention and partial or complete intestinal obstruction, a condition we call congenital megacolon. This disease is known as congenital megacolon. This lack of ganglion cells can lead to bacterial colonization of the digestive tract and infection, causing serious problems such as intestinal perforation and sepsis.  All children with megacolon require surgical treatment. The incidence of megacolon is about 1:5,000, and is about four times more common in boys than in girls. Children with Down syndrome also have a high incidence of this disease. Some cases of megacolon may be genetically linked. If the parents, especially the mother, have a history of megacolon, the risk of the child developing the disease is greatly increased. If one child in a family has the disease, the chance that one of his or her siblings will develop the disease is 3-12%, which is much higher than the average incidence. 80% of children with the disease will show symptoms in the first 6 weeks of life. About 90% of children with megacolon have delayed fecal excretion, and the rest have a short segment of ganglion cell-free intestine, which may not be particularly symptomatic for months or years.  Common early symptoms of megacolon include: 1. absence of fetal feces for more than 24 hours after birth 2. progressive abdominal distention 3. progressive vomiting 4. fever If early symptoms are not obvious or are ignored, the child may be seen with: 1. sepsis 2. progressive constipation 3. lack of appetite 4. delayed growth The diagnosis of megacolon includes a detailed physical examination and a series of tests including: abdominal x-ray ray: often suggests the presence of intestinal obstruction.  Barium enema: contrast is injected into the rectum and the morphology of the colon is observed on x-ray. It is the most valuable diagnostic imaging tool for megacolon.  Rectal biopsy: the tissue of rectal wall is taken and the presence or absence of ganglion cells is directly observed under the microscope.  Rectal manometry: usually selected only in older children.  All types of megacolon require surgical correction. The diseased ganglion cell-free segment is excised and the normal intestine is pulled down to the anus for anastomosis. For megacolon diagnosed clearly in the neonatal period, the current preference is for early one-stage radical treatment. The scarless procedure has been widely used in the surgical treatment of common megacolon. The results are satisfactory. However, in many cases such as children with severe intestinal obstruction due to poor infection status and too much dilatation of the intestinal canal, a fistula followed by radical treatment may be a better approach. Minimally invasive surgery with laparoscopic assistance for long segmental megacolon has also achieved more satisfactory results. However, regardless of the procedure, postoperative constipation may also persist due to the need to preserve the anal canal and sphincter.