Imperforate Anus Repair

Imperforate anus is a malformation of the anorectal region that may occur in several forms. The rectum may end in a blind pouch that does not connect with the colon. Or, it may have openings to the urethra, bladder, base of penis or scrotum in boys, or vagina in girls. A condition of stenosis (narrowing) of the anus or absence of the anus may be present.
The malformations are caused by abnormal development of the fetus, and many are associated with other birth defects. Imperforate anus is a relatively common congenital malformation that occurs in about 1 out of 5,000 infants.
  • Absence of anal opening
  • Misplaced anal opening
  • Anal opening very near the vaginal opening in the female
  • No passage of first stool within 24 - 48 hours after birth
  • Stool passed by way of vagina, base of penis or scrotum, or urethra
  • Abdominal distention

Signs and tests:
A physical examination with check for anal patency in the newborn will show the malformation.
A lower abdominal x-ray or contrast enema may be recommended.
Definition of Imperforate Anus Repair:
Imperforate anus repair is surgery to correct birth defects involving the rectum and anus. An imperforate anus defect prevents little or no stool from passing out of the rectum. Alternative names are Anorectal malformation repair; Perineal anoplasty.
This surgery repairs the defect so that stool can move thru the rectum. If the baby has no anal opening at all, emergency surgery is needed.
The procedure is done under general anesthesia, which means the patient is asleep and feels no pain during the procedure.
Surgery depends on the type of imperforate anus defect.
For a high-type imperforate anus defect, the surgery usually creates a temporary opening for the colon in the abdomen. This is called a colostomy. The baby is allowed to grow for several months before more complex repairs are attempted.
Surgery for a low-type imperforate anus involves closing any small tube-like openings (fistulas), creating an anal opening, and putting the rectal pouch into the anal opening.
For other related anal repairs, the surgery makes a cut in the abdomen and loosens the colon from nearby structures. This allows the colon to be re-positioned. A cut in the anal area is made to pull the rectal pouch down into place and create an anal opening. A colostomy may be closed during this procedure, or may be left in place for a few more months and closed at a later stage.
A major challenge for these repairs is finding, using, or creating nearby nerve and muscle structures so that the child can move the bowels normally.
Expectations after surgery:
Most anorectal malformations can be corrected successfully with surgery. In most cases, patients recover with normal bowel function. The long-term outlook is excellent if the patient has no other pelvic problems.
The child may spend several days in the hospital. The health care provider will use an instrument to dilate (stretch) the new anus to improve muscle tone and prevent narrowing -- this must be continued for several months. Use of stool softeners and a high-fiber diet are recommended throughout childhood.
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