Pediatric congenital megacolon consultation guidelines

  Congenital megacolon is a condition in which the distal (anal side of the) intestinal canal ganglion cells are absent or functionally abnormal and are in a spastic stenosis with poor patency, resulting in compensatory enlargement of the proximal (distal anal side of the) intestinal canal and wall thickening, forming a giant colon, called congenital megacolon.
  According to the length of the diseased intestinal canal (spastic stenosis section), it is clinically divided into the following types.
  1, common type (common type): no ganglion cell area intestinal canal extending upward from the anus to the distal end of the sigmoid colon, accounting for 75%.
  2, short segment type: no ganglion cell segment confined to the lower rectum, the proximal rectum began to hypertrophy and dilatation, accounting for 8%.
  3, ultra-short type: the lesion is limited to the internal sphincter part, also known as internal sphincter failure, accounting for 2%.
  4.Long segment type: the segment without nerve cells up to the descending colon accounted for 14%, the splenic flexure accounted for 10%, up to the transverse colon accounted for 4%.
  5.Whole colon type: In addition to the whole colon, the lesion also involves the end of the ileum 30 cm, and some reach 70 cm, accounting for 3% to 10%.
  6.Whole intestine type: the lesion affects all the colon and the end of the ileum for more than 30 cm, and even involves the duodenum.
  The longer the length of the intestinal canal, the more serious the disease is and the earlier the symptoms appear; conversely, the shorter the length of the intestinal canal, the later the symptoms appear.
  Neonatal manifestations.
  Impaired or delayed expulsion of fetal make
  Infrequent or progressive constipation
  distended abdomen and thinning of the abdominal wall
  obstruction of the digestive tract
  Anal fingering of gas and loose stool
  Infantile presentation.
  Incomplete low bowel obstruction
  malnutrition, growth retardation
  alternating diarrhea and constipation
  distended abdomen, frog-shaped abdomen, dilated abdominal wall veins
  large bowel pattern and peristaltic waves, hyperactive bowel sounds
  Flat and protruding umbilicus
  palpable fecal masses or stones in the left lower abdomen
  Diagnostic tests.
  (l) Upright abdominal plain film: pneumatization of the intestinal canal
  (2) Barium enema: intestinal stricture and dilatation
  (3) Anal rectal manometry: lack of rectal-anal reflex
  (4) Intestinal biopsy: no ganglion cells
  If your baby has some of the above manifestations, go to the pediatric surgery department of the hospital in time for early detection and early treatment to avoid serious consequences that may affect your baby’s health for a lifetime.