Anorectal Malformations

ANORECTAL
MALFORMATIONS
• Anorectal malformations are birth defects of a baby’s anus or rectum
that interfere with the normal passage of stool.
• When the anus is completely blocked, the condition is called
imperforate anus.
• In children with anorectal malformations, the anus may be missing,
blocked by a thin or thick layer of tissue, or narrower than normal.
• In rare cases, the anus may be normal while the rectum is blocked or
narrowed.
CLASSIFICATION
• In both females and males
• In females
• In males
Malformations found in both males and
females:
• Anorectal malformation without fistula – the anal opening is
missing or in the wrong place
• Rectal atresia and stenosis – the anus or rectum is too small to
allow stool to pass
• Rectoperineal fistula – the rectum connects to the perineum, an
area of skin between the anus and genitals
Malformations found in males:
• Rectobulbar urethral fistula and rectoprostatic urethral

fistula – the rectum connects directly into the urethra (the tube
that carries urine out of the body through the genitals)
• Recto-bladder neck fistula – the rectum connects to the bottom

of the bladder, where the urethra begins
Malformations found in females:
• Rectovestibular fistula – the rectum connects to just outside of

the vagina
• Rectovaginal fistula – rare malformation with a connection
between the rectum and the vagina
• Cloaca – the vagina, rectum and urinary tract are combined into
a single channel
Risk Factors
Anorectal malformation may be seen with some genetic syndromes or
congenital problems that are present at birth. These include:
• VACTERL association. This disorder includes problems with the spine, anus,
heart, trachea, esophagus, kidneys, and arms and legs.
• Digestive system problems
• Urinary tract problems
• Spinal problems
• Down syndrome
• Townes-Brocks syndrome. This syndrome includes problems with the anus,
kidneys, ears, and arms and legs.
DIAGNOSTIC EVALUATION
• The diagnosis is made by physical examination.
• If the anal opening is absent or in the wrong spot, it can be seen on
examination.
• If there is stool coming out of the urethra, or vagina, instead of the anus,
it will be visible.
• In females with anorectal malformation, careful examination of the
vestibule (area between the labia) must be made to ensure separate
openings of the urethra and vagina.
• In the males with imperforate anus, careful examination of the perineum
is necessary to identify any abnormal passage of stool.
Treatment
• Narrow anal passage - Surgery may not be needed. A procedure
known as anal dilation may be done from time to time. This helps to
stretch the anal muscles so stool can pass through.
• Anal membrane - Surgery is done to remove the membrane. Anal
dilations may need to be done afterwards to help with any narrowing
of the anal passage.
• Surgery - the surgeon must decide in the newborn period whether

the child requires fecal diversion with a colostomy, or can undergo a
primary repair procedure.
Colostomy
• The preferred colostomy is a descending colostomy, i.e. made from
the descending portion of the colon located in the lower-left quadrant
of the abdomen, with separated stomas.
• The proximal stoma is connected to the upper gastrointestinal tract
and drains stool.
• The distal stoma, also called a mucous fistula, is connected to the
rectum and will drain small amounts of mucus material.
Attaching the rectum to the anus
• This surgery is often done in the first few months of life.
• The colostomies stay in place for a few months after this surgery.
• This is so the area can heal without being infected by stool.
• The rectum and anus are now joined. But stool will leave the body
through the colostomies until they are closed with surgery.
• A few weeks after surgery, you may be doing anal dilations to help
your child get ready for the next phase.
Closing the colostomies
• This surgery is done about 2 to 3 months later.
• child will not be able to eat anything for a few days after surgery. This
lets the intestine heal.
• Several days after surgery, child will start passing stool through the
rectum. At first, stool will pass often and they will be loose. Diaper
rash and skin irritation can be a problem at this stage.
• A few weeks after surgery, the frequency of stool passage reduces and
are more solid. This often causes constipation. high-fiber diet is
recommended to help with constipation. This includes fruits,
vegetables, juices, whole-wheat grains and cereals, and beans.
Posterior sagittal approach
• The repair of an anorectal malformation requires a meticulous and delicate
technique and a surgeon with experience in the management of these defects.
The posterior sagittal approach is an ideal method of defining and repairing
anorectal anomalies. If the baby growing well, the repair can be performed at 1–
2 months of age.
• Ninety percent of male patients can be approached with a posterior sagittal

approach alone, while 10% require an abdominal component (with laparotomy
or laparoscopically) to mobilize a very high rectum. All female malformations,
with the exception of about 30% of cloacas can be repaired with this approach.
In 30% of cloacas, the rectum or vagina is so high as to require an abdominal
approach as well
(a) The pull-through can be performed via the abdominal, anterior perineal (anterior sagittal),
or sacroperineal (posterior sagittal) approach. Posterior sagittal anorectoplasty or the
sacroperineal approach comprises incising the posterior area of the anal site (dotted yellow
line) and performing pull-through (yellow curved arrow). Anterior sagittal anorectoplasty
involves incising the anterior area of the anal site (dotted blue line) and performing pull-
through (blue curved arrow). The abdominal approach involves performing pull-through after
abdominal incision (red arrow). Laparoscopy may be used instead of abdominal incision.
• (b) Low-type anorectal malformation with anovestibular fistula. In
female patients with low-type anovestibular fistula, anterior sagittal
anorectoplasty is usually selected as the surgical approach. Anterior
sagittal anorectoplasty involves incising the anterior area of the anal site
(dotted blue line), performing pull-through (blue curved arrow), and
separating the anovestibular fistula (green arrow) from the vestibule.
• (c) Intermediate-type anorectal malformation with rectourethral
fistula. In male patients with intermediate-type anorectal
malformation with rectourethral fistula, the posterior sagittal
anorectoplasty or abdominal approach (including laparoscopically
assisted anorectoplasty) is usually selected as the surgical approach.
Posterior sagittal anorectoplasty or the sacroperineal approach
involves incising the posterior area of the anal site (dotted yellow
line), performing pull-through (yellow curved arrow), and separating
the rectourethral fistula (green arrow) from the rectum.
• (d) High-type anorectal malformation with rectovesical fistula. In male
patients with high-type anorectal malformation with rectovesical
fistula, the abdominal approach (including laparoscopically assisted
anorectoplasty) is usually selected as the surgical approach. The
abdominal approach or laparoscopically assisted anorectoplasty
involves performing pull-through from the abdominal side (red arrow)
and separating the rectovesical fistula (green arrow) from the rectum.
Surgical repair of anorectal malformation is divided into two steps:
anoplasty and the pull-through step. Anoplasty involves creating a new
anus at the correct site. This step is performed at the perineum. The
pull-through step entails moving the distal rectal pouch to the correct
new anal site and anastomosing it with the distal anus. During pull-
through, the fistula is separated from the anorectal tract.
Nursing Interventions and Rationales
1. Perform complete physical assessment
• This condition is usually diagnosed within the first 24 hours of life. Note
if the patient has anal opening and if it appears to be located in the
anatomically correct location.
2. Observe for passage of meconium stools

• Patients with imperforate anus are either unable to pass stool
(meconium) at all, or it is passed from an inappropriate location. For
female infants, carefully note if the meconium is passed from anus,
vagina or urethra. For male patients, the anal opening may be located
below the penis or the scrotum.
3. Monitor vital signs
• Patients will often have other congenital malformations. Monitor
heart rate and rhythm, respiratory effort and rate and
temperature. Axillary temperature may be more appropriate
instead of rectal.
4. Prepare patient for diagnostic tests and surgery

• Patient will likely undergo several tests including imaging and labs
prior to the determination for surgery. Ultimately, the patient may
require a colostomy placed.
5. Monitor skin integrity and provide wound care following surgery
• The passage of stool from incorrect locations can lead to rapid skin
breakdown. Following surgery, assess incision site and provide
wound care per facility protocol. Observe for signs of infection.
6. Provide colostomy care if appropriate

• A colostomy may be required. Provide colostomy care per facility
protocol and as needed. Educate parents to care for patient with
colostomy. Assess the stoma for bleeding or signs of infection.
7. Provide education for parents
• Parents will often be very anxious about their baby’s condition.
Help by providing resources and information or contacts for
support groups.
• Educate parents on how to care for the patient post-surgery and
at home.
• As the child grows, constipation may be an issue and a longer
period may be required for toilet training.
• Encourage good nutrition with high fiber foods.

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ANORECTAL

• Rectobulbar urethral fistula and rectoprostatic urethral

• Recto-bladder neck fistula – the rectum connects to the bottom

• Rectovestibular fistula – the rectum connects to just outside of

• Surgery - the surgeon must decide in the newborn period whether

• Ninety percent of male patients can be approached with a posterior sagittal

2. Observe for passage of meconium stools

4. Prepare patient for diagnostic tests and surgery

6. Provide colostomy care if appropriate

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