ANORECTAL MALFORMATION

Introduction:
ARMs are a spectrum of different congenital anomalies in males and females, that varies from fairly
minor lesions to complex anomalies. There are many possible abnormalities as follows:
- The absence of an anal opening.
- The anal opening in the wrong place.
- A connection, or fistula, joining the intestine and urinary system.
- A connection joining the intestine and vagina.
- In females, the intestine can join with the urinary system and vagina in a single opening, known as a
cloaca.

Definition:
An imperforate anusor anorectal malformations (ARMs) are birth defects in which the rectum is
malformed.
Incidences:
Imperforate anus has an estimated incidence of 1 in 5000 births. It will present 90% of the time in
females and 50% of the time in males.
Risk factors:
Although most babies with anorectal malformations have no known previous family history, there
are cases where known inheritance patterns exist.
Pathophysiology:

• The embryogenesis of these malformations remains unclear. The rectum and anus are believed to
develop from the dorsal potion of the hindgut or cloacal cavity when lateral ingrowth of the
mesenchyme forms the urorectal septum in the midline. This septum separates the rectum and anal
canal dorsally from the bladder and urethra. The cloacal duct is a small communication between the
2 portions of the hindgut. Downgrowth of the urorectal septum is believed to close this duct by 7
weeks' gestation. During this time, the ventral urogenital portion acquires an external opening; the
dorsal anal membrane opens later. The anus develops by a fusion of the anal tubercles and an
external invagination, known as the proctodeum, which deepens toward the rectum but is separated
from it by the anal membrane. This separating membrane should disintegrate at 8 weeks' gestation.
• Interference with anorectal structure development at varying stages leads to various anomalies,
ranging from anal stenosis, incomplete rupture of the anal membrane, or anal agenesis to complete
failure of the upper portion of the cloaca to descend and failure of the proctodeum to invaginate.
Continued communication between the urogenital tract and rectal portions of the cloacal plate causes
rectourethral fistulas or rectovestibular fistulas.
• The external anal sphincter, derived from exterior mesoderm, is usually present but has varying
degrees of formation, ranging from robust muscle (perineal or vestibular fistula) to virtually no
muscle (complex long–common-channel cloaca, prostatic or bladder-neck fistula).
Etiology:
The cause of ARMs is unknown. In 8% of patients genetic factors are clearly associated with ARMs.
Some causes are-
1. The breakdown of the upper gastrointestinal tract with the anal area so that babies born without anus.
2. Failure of growth when the baby was 12 weeks / 3 months.
3. The disruption or cessation of embryological development in the intestine, the distal rectum and
urogenital tract, which occurs between the fourth to the sixth week of gestation.

Clinical manifestation:
 If there is a fistula (opening) to the skin, urethra or vagina, a newborn will pass meconium (a baby's
first stool) and, unless a careful exam is done, the imperforate anus may not be suspected. However, if there
is no anal opening and there is no fistula, the baby will not be able to pass stool after birth and this will lead
to a swollen or "enlarged" abdomen and vomiting.Meconium did not come out after 24-48 hours. Neonates
vomit green.
Classifications:
There are several forms of imperforate anus and anorectal malformations.
• A low lesion, in which the colon remains close to the skin. In this case, there may be a stenosis
(narrowing) of the anus, or the anus may be missing altogether, with the rectum ending in a blind
pouch.
• A high lesion, in which the colon is higher up in the pelvis and there is a fistula connecting the
rectum and the bladder, urethra or the vagina.
• A persistent cloaca is a complex anorectal malformation in which the rectum, vagina and urinary
tract are joined into a common single channel that opens onto the perineum via the usual urethral
site.

Other Associated anomalies:

Imperforate anus is associated with an increased incidence of some other specific anomalies as well,
together being called the VACTERL association:
• V – Vertebral anomalies
• A – Anal atresia
• C – Cardiovascular anomalies
• T – Tracheoesophageal fistula
• E – Esophageal atresia
• R – Renal (kidney) and/or radial anomalies
 • L – Limb defects
Diagnostic evaluation:
 Physical examination- 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. 
• An x-ray film of the abdomen is obtained 12-24 hr after birth, with the baby being kept in an inverted
position. A lateral picture of the pelvis should be obtained to define whether the rectal pouch is
above or below a line drawn from the pubis to the coccyx.
• Abdominal ultrasound is performed to determine the existence of other malformations.
• An IV pyelogram and voiding cystourethrogram are recommended for an infant with a high
malformation to identify anomalies of the urinary tract
MEDICAL MANAGEMENT:
In general, the child will need 3 operations to correct the anorectal malformation, as determined by
the anatomy: The first is to create a stoma, the second is to place the rectum through the center of the anal
sphincter and the third to close the stoma. Some children will only need one operation, to place the rectum
through the center of the anal sphincter. As the anatomy varies, the surgical plan will be determined after the
child is carefully evaluated by the surgeon.
 Skin opening in wrong location
If there is an opening for stool to drain, but it is in the wrong location, it will be placed into the correct
location, within the anal sphincter. This is usually done in one operation. 
 No skin opening with low rectum
If there is no skin opening for stool to drain and the rectum is low and near the anal sphincter, an anal
opening will be made in the correct location, within the anal sphincter. This can be done in one operation.
If the rectum ends high in the pelvis and is too far from the skin, surgical repair will be done in stages .
First a stoma, or connection of the colon to the skin on the abdominal wall will be created. 
An opening, called the colostomy, will allow stool to pass into a bag. The second opening is called the
mucous fistula and allows the closed end of the rectum to drain fluid or mucous as needed. After this
operation, the baby can go home to recover and grow.

Before the second operation, where an anal opening will be made in the correct location, within the anal
sphincter, the child may need to have a radiographic study called a distal colostogram to help prepare for
surgery. The study helps to determine the distance from the blind end of the rectum to the skin and if there is
a fistula.
If a fistula is found to the urogenital system, it will be closed at the time of the second operation. The
stoma will be left intact, and not closed, to allow the new anus to heal. After this operation, the baby can go
home to recover. 
Two weeks after the operation to create the new anus, anal dilations will need to begin. About a couple
of months after the new anus is created, the stoma will be closed in a third operation. The baby will then
pass stool through the new anus. It is very important to continue the anal dilations after the stoma is closed,
as directed by the surgeon.
Children can go home once they are feeding normally, having bowel movements per stoma, or anus, are
comfortable on pain medication by mouth, and do not have a fever. If there is a fistula to the urinary system,
a Foley catheter will be placed in the bladder during the time of the operation. This allows time for the
fistula closure to heal. The Foley will be removed before the child goes home. 

Home Care
Babies with anorectal malformations are special babies who need special parents. They will need colon care
throughout life but with personalized care, they can become very independent.
Home care immediately after surgery
 Pain management
Prescription pain medication is not routinely required after hospital discharge. Most children are
comfortable using acetaminophen (Tylenol) or ibuprofen (Motrin) at home. Care for incisions and dressings
Any incision on the abdominal wall may be covered with pieces of white tape called Steri-strips.
These strips fall off on their own or can be removed when they become loose. The dissolvable stitches
underneath the incision may come through the incision and may be associated with a little local redness and
pus. This is normal and is best treated by gently cleansing the area with soap and water and waiting. The
sutures around the stoma and the new anus will also dissolve over the next several weeks. If the child has
worsening redness, swelling pain of the incision or a fever within 1–2 weeks of the operation, visit doctor. 
 Healing Ridge
After the incisions are healed a firm ridge will be able to feel just underneath the incision. This is called
a healing ridge and it is normal to find this under an incision after an operation. The healing ridge usually
lasts for several months before it softens and disappears.
 Bathing Restrictions
The child may bath or shower between 2 and 5 days after the operation, as directed by the surgeon.
Bathing may be done without restriction. The incision and the anus can be cleaned (washed and patted dry)
normally. If the child has a stoma, the pouch can be removed and the child, with their stoma, can be
immersed in the bath. This will not harm the stoma and is recommended. The stoma does not feel any pain
and can be cleaned (washed and patted dry) and the skin around it wiped normally.
 Activity Restrictions
There are no specific activity restrictions following surgery. Babies with stomas can be carried and
cuddled normally.
 Diaper Skin Care
Once the child starts passing stool through the new anus, very frequent bowel movements will occur and
can cause severe diaper rash. Begin using a protective skin product as early as the day after surgery. A
diaper rash can occur rapidly and take days or weeks to heal. Do not stop using the skin protective products
until the number of bowel movements becomes less, usually after many weeks.
 - Remove all butt balm, cream, paste or ointment daily with mineral oil applied gently with cotton
balls. 
- Soak the child's bottom in a tub of warm water after applying the mineral oil. 
- Clean gently with soap and water, do not scrub skin. Avoid making the skin bleed. 
- At the end of the bath, pat at the skin dry. 
- Apply Cavilon 3M No Sting Barrier Film by swab or spray to all affected skin in the diaper area.
- Let dry for 60 seconds 
- Apply butt balm or other recommended barrier paste over the 3M product. 
- After each bowel movement, clean the poop off the butt balm and do not rub the butt balm from the
skin. 
- Reapply more butt balm to the diaper area. 
- Repeat after each bowel movement. 
- Repeat mineral oil and bath routine each day.

 Colostomy care
A colostomy requires routine care to be taken when pouching (placing a bag around the stoma to collect
the stool). A non-pouched colostomy requires additional instructions for management.
- Assess the location of the stoma and the type of colostomy performed. Stoma location is an
indicator of the section of bowel in which it is located and a predictor of the type of fecal
drainage to expect.
- Assess stoma appearance and surrounding skin condition frequently. Assessment of stoma and skin
condition is particularly important in the early postoperative period, when complications are most
likely to occur and most treatable.
- Position a collection bag or drainable pouch over the stoma. Initial drainage may contain more
mucus and serosanguineous fluid than fecal material. As the bowel starts to resume function,
drainage becomes fecal in nature. The consistency of drainage depends on the stoma location in the
bowel.
- In ordered irrigate the colostomy, instilling water into the colon similar to an enema procedure. The
water stimulates the colon to empty.
- When a colostomy irrigation is ordered for a client with a double-barrel or loop colostomy, irrigate
the proximal stoma. Digital assessment of the bowel direction from the stoma can assist in
determining which is the proximal stoma. The distal bowel carries no fecal contents and does not
need irrigation. It may be irrigated for cleansing just prior to reanastomosis.
- Empty a drainable pouch or replace the colostomy bag as needed or when it is no more than one-
third full. If the pouch is allowed to over fill, its weight may impair the seal and cause leakage.
- Provide stomal and skin care for the client with a colostomy as for the client with an ileostomy.
Good skin and stoma care is important to maintain skin integrity and function as the first line of
defense against infection.
- Use caulking agents, such as Stomahesive or karayapaste,and a skin barrier wafer as needed to
maintain a secure ostomy pouch.This may be particularly important for the client with a loop
colostomy.The main challenge for a client with a transverse loop colostomy is to maintain a secure
ostomy pouch over the plastic bridge.
- A small needle hole high on the colostomy pouch will allow flatus to escape. This hole may be
closed with a Band-Aid and opened only while the client is in the bathroom for odor control. Ostomy
bags may “balloon” out, disrupting the skin seal, if excess gas collects.

 Anal dilation
 An anal incision will heal by contracting and becoming tight. If this happens, passing bowel movements
will be very difficult. Stretching the anal incision gently with anal dilators is required and will begin at the
child’s first postoperative visit with the surgeon. Parents will need to continue the dilations at home.
- Anal dilation supplies- Dilator, lubricant, clean diaper, wipes and someone to help, if needed.
- Directions for anal dilations at home
i. Position the child on his/her back, holding the feet toward the head with knees flexed.
i. Lubricate the dilator and gently insert into the anus.
ii. Withdraw the dilator. Clean with soap and water (a very small amount of bleeding may occur,
this can be normal).
iii. Use twice daily, for one week.
iv. Every week, change to a dilator that is one size larger.
v. Use twice daily for one week.
vi. Stop increasing the size once you get to the maximum size as directed by surgeon.
vii. Continue twice daily dilations for 2 weeks using dilator size as directed by surgeon.
viii. Decrease frequency to once daily for 2 weeks.
ix. Decrease to every Monday, Wednesday and Friday for 2 weeks.
x. Decease to once a week for 2 weeks.
xi. Stop dilations.
xii. Do not discard dilators. They are to be cleaned with soap and water and saved in the event they
need to be used again.
xiii. Regular follow up is recommended for bowel management of children with anorectal
malformation and Hirschsprung's Disease.
Guidelines for sizing anal dilators:
1-4 Months of age: 12
4-8 Months of age: 13
8-12 Months of age: 14
1-3 Years of age: 15
3-12 Years of age: 16
> 12 years of age: 17–18

CLIENT AND FAMILY TEACHING

• Prior to discharge, provide written, verbal, and psychomotor instruction on colostomy care, pouch
management, skin care, and irrigation for the client. Whether the colostomy is temporary or permanent, the
client will be responsible for its management. Good understanding of procedures and care enhances the
ability to provide self-care, as well as self-esteem and control.
•Allow ample time for the client (and family, if necessary) to practice changing the pouch, either on the
client or a model. Practice of psychomotor skills improves learning and confidence.
• If an abdominoperineal resection has been performed, emphasize the importance of using no rectal
suppositories, rectal temperatures, or enemas. These, measures are important to prevent trauma to the tissues
when the rectum has been removed.
• The diet for a client with a colostomy is individualized and may require no alteration from that consumed
preoperatively. Dietary teaching should, however, include information on foods that cause stool odor and
gas and foods that thicken and loosen stools. Foods that cause these effects on ostomy output are listed
below.
Foods That Increase Stool Odor: • Asparagus • Fish • Beans • Gar lic• Cabbage • Onions • Eggs • Some
spices
Foods That Increase Intestinal Gas: • Cucumbers • Broccoli • Dairy products • Brussels sprouts • Dried
beans • Cabbage • Peas • Carbonated drinks • Radishes • Cauliflower • Spinach • Corn

Foods That Thicken Stools: •Applesauce • Pasta • Bananas • Pretzels • Bread • Rice • Cheese • Tapioca •
Yogurt • Creamy peanut butter
Foods That Loosen Stools: • Chocolate • Highly spiced foods • Dried beans • Le afy green vegetables •
Fried foods • Raw fruits and juices • Greasy foods • Raw vegetables
Foods That Color Stools: •Beets • Red gelatin
NURSING MANAGEMENT
Assessment
 Health history:
- History of absence of anal opening.
 Physical examination- 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. 
 Family knowledge:
- The family members don’t have knowledge about ARM.

NURSING DIAGNOSIS
- Fluid volume deficit related to excessive vomiting as evidenced bydecreased skin turgor, increased pulse.
- Risk for impaired skin integrity related to irritation from fecal drainage around peristomal area, irritation of appliance.
- Disturbed body image related to presence of ostomy and malodor as evidenced by refusal to look at or touch the stoma.
NURSING INTERVENTIONS
To maintain fluid volume-
- Observe for indications of dehydration (e.g. poor skin turgor, delayed capillary refill, dry mucous
membranes, decreased urine output).
- Monitor fluid status including intake and output.
- Maintain steady IV infusion flow rate.
- Monitor for abnormal serum electrolyte levels.
To improve skin integrity-
- Monitor stoma/surrounding tissue healing and adaptation to ostomy equipment.
- Apply appropriately fitting ostomy appliance.
- Use mild soap on skin while cleaning.
To improve body image-
- Help patient determine the extent of actual changes in the body or its level of functioning.
- Assist patient to separate physical appearance from feelings of personal worth.
 - Facilitate contact with individuals with similar changes in body image.

BIBLIOGRAPHY:
1. Ghai OP. Essential pediatrics. New Delhi, CBS publishers & distributors Pvt. Ltd. 2016 (8th edition)
2. Wong’s. Essentials of Pediatric Nursing. New Delhi, Elsevier India Pvt. Ltd. 2012(8th edition)