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  • Shaken Baby Syndrome

    Tuesday, 21 July 2015 16:28
  • Amniotic fluid problems

    Thursday, 14 May 2015 12:54
  • Choosing a pre-school

    Friday, 10 April 2015 17:50
  • Newborn reflexes

    Tuesday, 03 March 2015 15:49
  • Mastitis

    Tuesday, 03 March 2015 15:41
  • Pelvic floor exercises

    Wednesday, 11 February 2015 17:20
  • Colic

    Wednesday, 11 February 2015 17:11
  • Antenatal Classes

    Monday, 03 June 2013 09:34
  • Strap-in-the-Future

    Thursday, 30 June 2011 13:52

Imperforate anus

imperforated anusAn Imperforate anus is a defect that develops during the fifth to seventh week of foetal development. With an imperforate anus several abnormalities can occur, as it affects the development of the anus (opening at the end of the large intestine through which stool passes) and the rectum (area of the large intestine just above the anus).

The intestines are long tubes running from the stomach to the anus, consisting of the small intestine, large intestine and rectum. The intestines are responsible for absorbing nutrients and water from what we eat and drink. During the digestive process, food moves from the small intestine into the large intestine (colon). The large bowel removes water from waste to create stool. During a bowel movement, stool passes from the large intestine into the rectum and then into the anus. Nerves in the anal canal help us sense the need for a bowel movement and stimulate muscle activity. Muscles in this area help control when we have a bowel movement. Abnormalities in the anal area due to imperforate anus may include:

  • The anal passage being too narrow or misplaced (in front of where it should be located)
  • The presence of a membrane over the anal opening
  • The rectum not being connected to the anus
  • The absence of an anal opening, with the rectum connecting to part of the urinary tract or the reproductive system, though a passage known as a fistula

Cause:

There is no known cause for the condition. The abnormality affects approximately 1 in 5 000 babies and is slightly more frequent in males than females.

Symptoms:

  • No anal opening
  • Misplaced anal opening
  • In females, the anal opening may be close to the vagina
  • Abdominal distension
  • No bowel movement within 24-48 hours after birth
  • Stool passed through the vagina in girls or urethra in boys

Approximately 50% of babies with imperforate anus have other abnormalities, including:

  • Abnormalities of the spine
  • Windpipe (tracheal) or esophageal defects
  • Defects of the arms and legs
  • Down syndrome
  • Hirschsprung’s disease (blockage of the large intestine)
  • Duodenal atresia (the first part of the small bowel is not properly developed)
  • Congenital heart defects

Problems and concerns:

A number of problems can develop depending on the type and severity of the imperforate anus.

  • If the anal opening is narrow or misplaced in front of the correct location, the child may experience bowel movement difficulties, resulting in discomfort and constipation.
  • If there is a membrane covering the anal opening, the baby will be unable to have a bowel movement until the membrane is opened in a surgical procedure.
  • If the rectum is not connected to the anus and a fistula (abnormal connection between the rectum and urinary tract or vagina) is present, stool may pass through the fistula instead of the anus. This may result in urinary tract infections.
  • If the rectum is not connected to the anus and there is no fistula, there is no way for stool to leave the intestine. The baby will be unable to have a bowel movement, resulting in intestinal obstruction.

Diagnosis:

Babies are given a comprehensive physical examination at birth. This includes checking that the babies anus is open and in the correct position. If an imperforated anus is detected, further diagnostic tests can be performed to assess the problem and determine if other abnormalities are present.

  • X-rays provide information about the anatomical location of the imperforate anus, indicating whether the problem is situated high or low in the imperforate anus area. X-rays also draw attention to any abnormalities in the spine or sacral areas (the sacrum is a triangle-shaped bone just below the lumbar vertebrae).
  • Abdominal and spinal ultrasounds allow for the assessment of the urinary tract and spinal column. These tests are able to detect possible abnormalities, such as a tethered spinal cord (an anatomical abnormality in which the end of the spinal cord is abnormally anchored). A tethered spinal cord may produce neurological difficulties, including incontinence (toileting without control) and leg weakness as the child grows.
  • An echocardiogram may be performed to determine if there are any heart defects.
  • In some cases, an MRI (magnetic resonance imaging) is required to accurately diagnose a tethered cord or other spinal problems.

Treatment:

Infants with imperforate anus, should not be fed and receive intravenous hydration after birth. If a urinary fistula is suspected, antibiotics are prescribed to prevent or treat infection. A nasogastric tube may be inserted to keep the stomach empty (decompressed) to avoid the risk of vomiting or stomach contents being breathed into the respiratory system.

Treatment recommendations depend on the type of imperforate anus, the presence and severity of associated abnormalities, and your child’s overall health. Most infants with imperforate anus require surgical intervention. The exact type of corrective surgery will depend on the specifics of the defect, including how far the infant’s rectum descends, if the nearby muscles are affected, and whether fistulas are present.

There are three primary goals for the surgical repair of imperforate anus:

  • To detach the rectum from other structures (such as the vagina and urethra) and repair these structures, if necessary.
  • To create an anus in the normal place outside the body, in an operation called a perineal anoplasty.
  • To pull the rectum down towards the new anus, and connect it, in what is referred to as a “pull-through” operation.

Anoplasty

Infants with a rectoperineal malformation need an operation called an anoplasty. This procedure involves moving the anus to an appropriate position within the muscles that control continence (anal sphincter muscles).

Colostomy

Infants diagnosed with imperforate anus may require one or more corrective surgeries. Some babies temporarily require a colostomy, a surgery that allows solid waste to leave the body. A colostomy involves dividing the large intestine into two sections. The ends of the intestine are brought through two small openings in the abdominal wall. Each opening is called a stoma. The upper section allows stool to pass through an opening into a pouch situated outside the body (called an ostomy bag). The lower section is brought to the other stoma and provides an opening to clean the lower intestines.

Colostomies allow digestion and growth to continue before the next required operation. The diversion of stool also minimizes the risk of infection, when further reconstructive surgery is performed.

A stomal therapy nurse will teach you how to take care of the colostomy, and assist you in the transition from hospital to home.

The next operation involves connecting the rectum to the newly created anal opening. This procedure is usually performed from a posterior midline approach (between the buttocks).

In cases where the rectum ends within the abdomen (high lesions), surgery is required to connect the rectum to the anal opening.

Post-surgical care

A few weeks post-surgery, parents may be taught how to perform anal dilations. This process ensures that the anal opening is large enough for the passage of stool and is an appropriate size for the child’s age.

Children, who required a colostomy, will undergo another operation 6-8 weeks later, to reconnect and close the area. They will begin passing stool through the rectum several days later. Initially the stool may be loose and frequent. Skin irritation and nappy rash are common during this time. Your surgeon will advise on creams to apply before surgery to prevent the buttock skin becoming torn or worn following closure. Within a few weeks, stool becomes less frequent and firmer. Anal dilations may be required for several weeks or months.

Some infants become constipated. A high-fiber diet is recommended. Laxatives may be prescribed before potty training age.

Severe constipation is treated through the development of a bowel management programme. Both parent and child will be educated in the use of laxatives, stool softeners, enemas, bowel training techniques and biofeedback.

Potty training for children with imperforate anus should be started at the usual age, around 2-3 years. Sitting your child on a potty for no more than 5 minutes after meals may help establish the brain/bowel connection.

Prognosis

Children who have had imperforate anus generally gain bowel control more slowly than their peers. Individual variations largely depend on the type of malformation and its surgical repair. Children who have had an imperforate anus with a fistula are usually able to gain good bowel control following surgery. However, children with more complex anorectal abnormalities may need to participate in a bowel management program or have a Malone Antegrade Continence Enema or MACE procedure, to prevent chronic constipation or incontinence.

Most children who have surgery develop bowel control. The most common long-term problem for children who have had imperforate anus is constipation, even following surgical repair. Stool incontinence is the most troublesome complication. With close follow-ups and management, most problems can be overcome. Your child’s healthcare team will teach you how to manage your child’s care, depending on their individual needs.

 http://www.cincinnatichildrens.org/health/i/imperforate-anus/

 http://www.healthline.com/health/imperforate-anus#Symptoms2

 http://www.seattlechildrens.org/medical-conditions/digestive-gastrointestinal-conditions/imperforate-anus-treatment/

 http://surgery.med.umich.edu/pediatric/patient/conditions/A-M/malone.shtml