Case Pres - Imperforate Anus

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Alternative Names

Anorectal malformation; Anal atresia

Definition of Imperforate anus:

Imperforate anus is congenital (present from birth) defect in which


the opening to the anus is missing or blocked. The anus is the opening
to the rectum through which stools leave the body.

Causes, incidence, and risk factors:

Imperforate anus 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 problem is caused by abnormal development of the fetus, and many


forms of imperforate anus are associated with other birth defects. It
is a relatively common condition that occurs in about 1 out of 5,000
infants.

Imperforate anus

Symptoms:

 Anal opening very near the vaginal opening in girls

 Missing or misplaced opening to the anus


 No passage of first stool within 24 - 48 hours after birth

 Stool passes out of the vagina, base of penis, scrotum, or urethra

 Swollen belly area

Signs and tests:

A doctor can diagnose this condition during a physical exam. Imaging


tests may be recommended.

Imperforate anus

Treatment:

The infant should be checked for other problems, especially those


affecting the genitals, urinary tract, and spine.

Surgical reconstruction of the anus is needed. If the rectum connects


with other organs, repair of these organs will also be necessary. A
temporary colostomy is often required.

Expectations (prognosis):

With treatment, the outcome is usually good. However, it depends on


the exact problem. Some infants may never develop adequate bowel
control.

Complications:

 Bowel incontinence
 Constipation

 Intestinal blockage

Calling your health care provider:

This disorder is usually discovered when the newborn infant is first


examined. Call your health care provider if a child that was treated for
imperforate anus has abdominal pain or fails to develop any bowel
control by the age of 3.

what is an imperforate anus?


An imperforate anus is a defect (something which is abnormal) that
happens to an unborn baby while it is still growing inside its mother.
The baby develops this defect or abnormality during the fifth to
seventh weeks of the mother’s pregnancy. With these defects, the
anus (opening at the end of the large intestine where stool passes) and
the rectum (area of the large intestine just above the anus) do not
develop properly.

Imperforate Anus Incidence


Imperforate anus affects 1 in 5,000 babies and is a little more common
in males. 

No one knows the exact reason some baby’s have imperforate anus. The
exact cause of imperforate anus is unknown. In some cases,
environmental factors or drug use during pregnancy may play a role, but
no one is completely sure. 

During a bowel movement, stool passes from the large intestine to the
rectum and then to the anus. Nerves in the anus help us feel the need
for a bowel movement and start muscle activity. Muscles in this area
help control when we have a bowel movement. 
With an imperforate anus, any of the following can happen:

 The anal opening may be narrow or misplaced in front of where it


should be located
 A membrane (covering) may be present over the anal opening
 The rectum may not connect to the anus
 The rectum may connect to part of the urinary tract or the
reproductive system though an opening called a fistula, and an anal
opening is not present
Return to top
Why worry about an imperforate anus?
A number of problems can happen depending on the type and how
severe the imperforate anus is.

 When the anal opening is narrow or misplaced in front of the


correct location, a child may have a hard time passing a bowel
movement, causing constipation and discomfort.
 If there is a membrane covering the anal opening, the baby may
be unable to have a bowel movement until the membrane is opened
with surgery.
 If the rectum is not connected to the anus and no fistula
(abnormal connection between the rectum and urinary tract or
vagina) is present, there is no way for stool to leave the intestine.
The baby will be unable to have a bowel movement. This will cause a
bowel obstruction (stool becomes trapped inside the baby).
 When the rectum is not connected to the anus but a fistula is
present, stool may pass through the fistula instead of the anus.
This can cause urinary tract infections.
Return to top
Who is at risk for developing an imperforate anus?
Although most babies who have imperforate anus are the only ones in
their family to have it, there are some cases where other family
members have had it as well.

Are other disorders associated with imperforate anus?


Almost 50% of babies with imperforate anus have other abnormal
defects along with the imperforate anus. These commonly include:

 Spinal abnormalities, such as hemivertebra, absent vertebra


and tethered spinal cord
 Kidney and urinary tract malformations, such as horseshoe kidney
and duplication of parts of the urinary tract
 Congenital heart defects
 Tracheal and esophageal defects and disorders
 Limb (particularly forearm) defects
Down syndrome, Hirschsprung's disease and duodenal atresia can also
happen with an imperforate anus.
Return to top
How is imperforate anus diagnosed?
When a baby is born, the doctor will check the baby for any problems.
While the doctor is checking the baby, he or she will check to make
sure the baby’s anus is open and in the correct position. If an
imperforate anus is found, a number of tests may be done to better
understand the problem and to see if any other problems are present.

 X-rays of the stomach will give the doctor a picture showing the
general location of the imperforate anus. X-rays also let doctors
know if there are problems with the spine and sacrum (a triangle-
shaped bone just below the lumbar vertebrae).
 Abdominal ultrasound and spinal ultrasound -- These tests are
used to look at the urinary tract and spinal column. They also help
doctors decide if a tethered spinal cord (an abnormality where the
end of the spinal cord is abnormally anchored) is present. 
       A tethered spinal cord may cause neurological problems, such as
incontinence (toileting without control) and leg weakness as the child
grows.

 Echocardiogram -- This test is done to find heart defects.


 Magnetic resonance imaging / MRI --Magnetic resonance
imaging / MRI -- In some cases, this test is needed to make a
definite diagnosis of tethered cord or other spinal problems.
How is imperforate anus treated or repaired?
Treatment recommendations will depend on the type of imperforate
anus, the presence and type of associated abnormalities and the child's
overall health. However, most infants with an imperforate anus will
need surgery

Rectoperineal Malformation
Infants with a rectoperineal malformation will need an operation called
an anoplasty, which involves moving the anus to an appropriate place
within the muscles that control continence (controlled toileting, in this
case bowel movements)

Return to top
Colostomy for Infants with Imperforate Anus Without a Fistula
Newborn boys and girls who have an imperforate anus without a fistula
will need one or more operations to correct it. An operation to make a
colostomy is usually done early. 

With a colostomy, the large intestine is divided into two sections, and
the ends of intestine are brought through small openings in the
abdominal wall (small opening will be on your child’s belly). 

The upper section allows stool to pass through the opening, called a
stoma, and into a collection bag. Mucus from the intestine exits
through the opening of the lower section of intestine. 
By performing this surgery, digestion will not be harmed and growth
can continue to happen before the next operation is needed. By
changing the original route of the stool, the risk of infection will be
smaller when the next operation is done. 

Nurses and other health care workers who work with your child's
doctor will help you learn how to take care of the colostomy, and they
will help you, as you prepare to take care of your child at home. Local
and national support groups may also be very helpful during this time. 

The next operation creates a connection between the rectum and the
newly created anal opening. This procedure is usually performed from
the child’s bottom. 

In some cases where the rectum ends within the abdomen (high
lesions), laparoscopic surgery (surgery through small holes, usually
without an incision) or traditional open surgery (surgery with an incision
or cut opening) can be used to bring the rectum down to the anal
opening. 

After the rectum is brought down to the anal opening the colostomy
will stay in place for six to eight weeks. Stool will continue to leave the
body through the colostomy until it is closed with surgery. The
colostomy will have to stay in place to let the new anal opening heal
without being infected by stool and let the child undergo the dilation
process (schedule that includes slowly stretching the anus to the
correct size for the child’s age).

Return to top
Anal Dilatation After Surgery to Repair Imperforate Anus
A few weeks after surgery, parents are taught to perform anal
dilatations to make sure the anal opening is large enough to allow
normal passage of stool. 

The colostomy is closed in another operation at least six to eight weeks


later. Several days after surgery, the child will begin passing stools
through the rectum (anal opening). Shortly after surgery, stools may
be loose and happen more often than usual. Diaper rash and skin
irritation can also be a problem. Your doctor and nurse will help you
with the care needed to keep the diaper area healthy. 

Within a few weeks after surgery, stools will happen less often and
become firmer. Anal dilatations should continue for several weeks or
months. 

Some infants may become constipated. To avoid this, we encourage


following a high-fiber diet. Laxatives may be required prior to the age
of potty training. 

In cases of severe constipation, a bowel management program may be


developed according to the specific needs of the child. The program
may include child and parent education in the use of laxatives, stool
softeners, enemas, bowel training techniques.

Return to top
What should be done about toilet training kids who've had an
imperforate anus?
Toilet training should be started at the usual age, usually when the
child is around 3 years old. Children who have had imperforate anus
generally gain bowel control more slowly, and depending on the type of
malformation and the operations done to repair it, some children may
not be able to gain good bowel control. Each child’s situation will be
slightly different and will be determined with the help of your doctor.

What is the long-term outlook for children with imperforate anus?


Children who have had an imperforate anus that have a rectoperineal
fistula are usually able to gain good control over their bowel movements
after surgical repair. 

However, those with more difficult types of anorectal malformation


may need to participate in a bowel management program to help them
achieve control over their bowel movements and prevent constipation. 

Nurses and other health care professionals who work with your child's
doctors can outline a program made for your child's individual needs.

mperforate anus
From Wikipedia, the free encyclopedia

Imperforate anus

Classification and external resources

An X-ray showing Imperforate anus

10

OMIM 301800 207500
MedlinePlus001147

eMedicine ped/1171 ped/2923

D001006

An imperforate anus or anal atresia is a birth defect in which


the rectum is malformed. Its cause is unknown.

Contents
]

 1 Diagnosis
 2 Treatment
 3 Features
 4 Prognosis
 5 Epidemiology
 6 Associated
anomalies
 7 References
 8 External links
[edit]Diagnosis

When an infant is born with an anorectal malformation, it is usually


detected quickly as it is a very obvious defect. Doctors will then
determine the type of birth defect the child was born with and
whether or not there are any associated malformations. It is important
to determine the presence of any associated defects during the
newborn period in order to treat them early and avoid furthersequelae.
There are two main categories of anorectal malformations: those that
require a protective colostomy and those that do not. The decision to
open a colostomy is usually taken within the first 24 hours of birth.
[edit]Treatment

Imperforate anus usually requires immediate surgery to open a passage


for faeces. Depending on the severity of the imperforate, it is treated
either with a perineal anoplasty[1] or with a colostomy.
[edit]Features

There are several forms of imperforate anus:

 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 (from the term cloaca, an analogous orifice
in reptiles and amphibians), in which the rectum, vagina and urinary
tract are joined into a single channel.
Imperforate anus is usually present along with other birth defects—
spinal problems, heart problems, tracheoesophageal fistula, esophageal
atresia, renal anomalies, and limb anomalies are among the possibilities.
[2]

Sonography can be used to determine the type of imperforate anus.[3]


[edit]Prognosis

This section does not cite any references or sources.


Please help improve this article by adding citations to reliable
sources. Unsourced material may
challenged and removed. (February 2010)
With a high lesion, many children have problems controlling bowel
function and most also become constipated. With a low lesion, children
generally have good bowel control, but they may still become
constipated.
For children who have a poor outcome for continence and constipation
from the initial surgery, further surgery to better establish the angle
between the anus and the rectum may improve continence and, for
those with a large rectum, surgery to remove that dilated segment may
significantly improve the bowel control for the patient. An antegrade
enema mechanism can be established by joining the appendix to the
skin (Malone stoma); however, establishing more normal anatomy is the
priority.
[edit]Epidemiology

Imperforate anus has an estimated incidence of 1 in 5000 births. [4][5] It


affects boys and girls with similar frequency. [6] However, imperforate
anus will present as the low version 90% of the time in females and
50% of the time in males.
Imperforate anus is an occasional complication of sacrococcygeal
teratoma.[7]
[edit]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

mperforate anus: Introduction


Imperforate anus: A congenital disorder where the anus is missing or
located in the wrong position. More detailed information about
the symptoms, causes, and treatments of Imperforate anus is
available below.

Symptoms of Imperforate anus


Click to Check

 Abnormal anus
 Absent anus
 Rectal opening into vagina
 Rectal opening near scrotum
 more symptoms...»
Read more about symptoms of Imperforate anus

Home Diagnostic Testing


Home medical testing related to Imperforate anus:

 Food Allergies & Intolerances: Home Testing:


o Home Food Allergy Tests
o Home Food Intolerance Testing
o Home Water Testing
 more...»

Wrongly Diagnosed with Imperforate anus?

 Misdiagnosis of Imperforate anus


 Hidden causes of Imperforate anus (possibly wrongly diagnosed)

Surya Mega: Colostomy Operation

Surya Mega, the sixth child of a widow from an impoverished village in


Lombok, was born with an imperforate anus and survived for three
years, when a colostomy operation was performed. In July 2003, at 7
years of age, she was brought to the Foundation for assistance and
underwent an operation in Bali to restore her bowel function to normal.

Surya Mega with her family in Lombok. Through donations, the


Foundation is also assisting the family so that the three school-aged
children can continue in school.
   
Abdominal and Digestive Conditions / Diagnoses
Early Diagnosis of Anorectal Malformations / Imperforate Anus
Related Services
 Colorectal Center
Early diagnosis and prompt treatment can help change the outcome for
children born with anorectal malformations / imperforate anus.

An emerging area of interest is the prenatal diagnosis of anorectal


malformations. Much is being learned in this area as technology
improves, and perinatologists are learning to recognize certain clues of
the prenatal ultrasound.

Immediate Postnatal Diagnosis


Certainly the vast majority of anorectal malformations diagnoses are
made in the delivery room on examination of the infant after birth or
in the newborn nursery when the nurse attempts to take a rectal
temperature and no anus is visible.

Inspection of the perineum, the area surrounding the external genitals


and where the anus would normally be located, should be performed on
all newborns and may result in an immediate postnatal diagnosis of an
anorectal malformation / imperforate anus.
An infant who is diagnosed with an anorectal malformation /
imperforate anus requires a more comprehensive examination that may
include evaluation of the following conditions:

 Does the abdomen appear distended (enlarged or stretched out)?


 Is the infant vomiting?
 Does the infant display a voiding (urination) pattern that would
indicate involvement of the urinary tract?
 Is meconium present in the perineum of a male infant or in the
genitalia of a female infant? Meconium, a greenish mass of cells
that accumulate in the bowel of the fetus, is usually discharged
after birth.
 Is meconium present in the urine of a male baby? Meconium can
be detected by filtering the urine through a gauze pad placed at
the tip of the penis or by laboratory examination of the urine
(urinalysis).
Infant Care Following Anorectal Malformation / Imperforate Anus
Diagnosis
Once the infant has been diagnosed with an anorectal malformation /
imperforate anus, the goals of care are:

 Determining whether the infant needs a


temporary colostomy (explained below) or whether the defect can
be treated primarily without a colostomy
 Evaluating other defects or medical problems that require
immediate attention
 Providing information and emotional support to the parents and
other family members
 Providing general medical support to the child
Decision to Perform a Colostomy Versus an Anoplasty
Some anorectal malformations / imperforate anus can be treated with
a one-stage surgical repair. In infants with more extensive defects,
however, a colostomy is needed to surgically create an alternate route
for feces to exit the infant's body while the infant awaits more
extensive surgery to correct the anorectal malformation.

A colostomy decompresses the bowel, diverting feces outside the body.


It also helps prevent infection during the reconstructive surgery to
correct the anorectal malformation / imperforate anus. The decision to
perform a colostomy is generally made after 24 hours of observation.

Associated Defects Requiring Immediate Attention


The most frequently associated defects of anorectal malformations /
imperforate anus that require immediate attention are those of the
urinary tract.

Infants with anorectal malformations require an ultrasound


examination of the abdomen to detect a urinary obstruction. If the
ultrasound examination is abnormal, a more detailed urologic evaluation
is indicated.

Associated defects of the digestive tract include an obstruction of the


esophagus (tube that carries food and liquids from the throat to the
stomach).

Known as esophageal atresia, this condition can also cause leakage from
the digestive tract into the lungs, and requires surgical correction so
the child can eat.

Children born with anorectal malformations / imperforate anus may


also have abnormalities in the bones, particularly the sacrum bone and
the lumbosacral spine, and heart.

All infants diagnosed with an anorectal malformation should have a


thorough cardiac evaluation with an echocardiogrambefore undergoing
surgery.
An ultrasound of the spine will help to rule out the presence of a
condition called tethered cord, which includes the final outcome.

Care of the Infant Awaiting Treatment


Once an infant has been diagnosed with an anorectal malformations and
is being evaluated for treatment, several steps are recommended:

 Intravenous nourishment while taking nothing by mouth (NPO)


 Inserting a nasogastric (NG) tube to avoid vomiting and risk of
aspiration
 Intravenous fluids
 Antibiotics
 Vitamin K to correct clotting deficiencies of a newborn prior to
surgery
 Strict monitoring of intake and output
We believe that it is now possible to establish an accurate early
diagnosis, which allows us to tell the parents what the future of their
child is going to be in terms of bowel control.

Treatment

Medical Care
Newborns with imperforate anus should not be fed and should receive
intravenous hydration. Life-threatening comorbidities take precedence
and must be treated first.

If a urinary fistula is suspected, broad-spectrum antibiotics can be


administered, although anaerobic coverage is unnecessary within the
first 48 hours of life. Any cardiac murmurs identified upon physical
examination should be evaluated using echocardiography prior to
surgical intervention. The remainder of treatment includes diagnostics
and surgical evaluation and management.

Surgical Care
The decision-making process aims to determine which children should
undergo primary repair in the neonatal period and which children
require colostomy and definitive repair in a staged fashion. Children
with anorectal malformations may undergo one or several of the
following surgical procedures based on the child's presentation,
physical examination findings, and imaging study findings.

 Neonatal colostomy

o A colostomy is performed in children who are not amenable


to primary pull-through either because of malformation
complexity (any urinary fistula in boys, vestibular fistula and
cloaca in girls, no fistula in either sex >1 cm from perineal
skin) or associated comorbidity.

o The colostomy is usually fashioned through a left lower


quadrant incision. The colon is divided at the point where
the descending colon meets the sigmoid colon, and both ends
are brought to the abdominal wall. By fashioning the
colostomy at this location, the entire sigmoid colon is kept in
place; thus, when the pull-through is eventually performed, a
large portion of the colon is available for the surgeon to
bring down to the perineal skin.

o The mucous fistula (the downstream segment) should be


very small, flush with the skin, and far enough from the
proximal end to be outside the colostomy appliance (or
under the flange) to avoid continued urinary soiling with
feces.

o During this operation, the distal segment of the colon must


be exhaustively irrigated to clean out the impacted
meconium, which is always significant. This prevents
postdiversion urinary sepsis and allows for effective distal
colostography.

 Primary neonatal pull-through without colostomy

o Many pediatric surgeons opt for primary pull-through in


children with perineal fistulas (or no fistulas) and close (<1
cm) rectal pouches on 24-hour lateral pelvic radiography.
Some pediatric surgeons who specialize in colorectal
problems often offer the same procedure for girls with
vestibular fistulas.

o Cystoscopy is usually performed to rule out associated


malformation. This is performed immediately prior to the
pull-through operation. A Foley catheter is inserted
following the cystoscopy.

o The preferred surgical approach is the posterior sagittal


approach developed by Peña et al.

 The child is placed in the prone position with generous


padding under the face and chest and a large bolster
under the hips to elevate the area of interest.

 A muscle stimulator is used to show the precise


position of the rectal muscle complex to enable exact
division at the midline. The midline for this operation
is defined by the line that precisely divides the muscle
complex in half.

 Dissection proceeds until the rectal pouch is


identified. The pouch is then mobilized until adequate
length is obtained and the rectum is fully separated
from its attachment to the genitourinary tract. Even
if the structures do not communicate, they remain
intimately associated until fully mobilized. This step
ensures that the surgeon does not miss a fistula that
was missed on urinalysis findings.

 Once the rectum has been mobilized, the muscle


stimulator is used to mark the anterior and posterior
limits of the muscle complex. The perineal body is then
reconstructed, and the rectum is tacked down in the
middle of the muscle complex. The posterior wound is
closed and anoplasty is performed.

 Posterior sagittal pull-through with colostomy

o This approach is used in boys with rectourinary fistula


(bulbar, prostatic, or bladder-neck fistula), in girls with
cloaca or vestibular fistula, and in patients of either sex
who do not have a fistula when the rectal pouch is further
than 1 cm on 24-hour lateral prone abdominal radiography.

o The approach is also used in children who may have


malformations that were amenable to primary neonatal pull-
through but were unable to undergo such a procedure
because of extreme prematurity or other comorbidity.

o Colostomy is performed after 24 hours (or immediately if


one of the above diagnoses is made based on either physical
examination findings or meconium in the urine).

o Several weeks following colostomy, distal colostography is


performed, and the specifics of the malformation are
clarified.

o Cystoscopy is usually performed to clarify anatomy and to


rule out associated malformation. This is performed
immediately prior to the pull-through operation. A Foley
catheter is inserted following the cystoscopy, except in
girls with cloaca.

o The reconstructive procedure varies based on the


malformation, but the essential concepts include identifying
and separating the rectum from other structures, dividing
and ligating any fistulas, and fully reconstructing the pelvic
anatomy with placement of the rectum within the confines
of the muscle complex. Procedures for specific
malformations are as follows:

 Vestibular fistulas are directly visible but have the


longest common wall between the rectum and vagina
and require significant delicate mobilization to avoid
holes in either structure.

 The posterior sagittal approach is used in boys with


bulbar or prostatic urethral fistulas. The rectum is
isolated and opened, and the fistula is identified
through progressive distal opening. Once the fistula is
identified, the rectum proximal to it may be mobilized,
and the fistula is then ligated. Reconstruction then
proceeds with primary pull-through, as described
above.

 Abdominal (open or laparoscopic) and posterior sagittal


approaches are best in boys with bladder-neck fistulas
because the fistula is best identified in the abdomen.

 Cloaca procedures are complex. A short – common-


channel cloaca can be repaired using total urogenital
mobilization. The posterior sagittal wound is opened
into the cloaca, which is then further proximally
opened until the urethral orifice is identified and
catheterized. The rectum is then sought. In girls with
2 hemivaginas (50%), the rectum opens in the vaginal
septum, although significant asymmetry may be
present. After identification, the rectum is separated
from the urogenital tract and completely mobilized.
The urogenital tract is then mobilized as a solitary
structure until the urethral orifice reaches the
perineum. This is then reconstructed, and the muscle
is marked to enable creation of an adequate vaginal
opening and perineal body without impinging on rectal
space.

 A long – common-channel cloaca repair often


necessitates formal separation of the bladder and
vagina, which requires laparotomy and ureteral
catheterization. Vaginal replacement is sometimes
necessary if the vaginal length is insufficient for
reconstruction.

 Colostomy closure: Once the wound has completely healed and


postoperative dilations have achieved their goal (ie, the neoanus
is at the desired size), the colostomy may be closed in traditional
surgical fashion.

Consultations

 Pediatric surgeon: Early consultation with a pediatric surgeon


experienced with these anomalies is essential. Ill-conceived
procedures during the newborn period may have lifelong
consequences for the patient.

 Neurosurgeon: Consultation with a neurosurgeon is warranted if a


tethered spinal cord is present (25% of all cases).
 Urologist: The need for consultation with a urologist depends on
the malformation and the individual pediatric surgeon.

Diet

 After the obstruction is relieved using colostomy, primary pull-


through, or dilation, children do not require special diet.

 The most common complication of imperforate anus repair is


constipation or anal incontinence; therefore, diet can be a crucial
part of management. Many patients may require laxatives,
enemas, or other medications or irrigations in addition to dietary
manipulations.3 Children should avoid constipating foods, such as
those included in the bananas, rice, applesauce, and toast (BRAT)
diet. High-fiber and laxative foods (whole-grain foods and
breads, dairy, fruits, vegetables, greasy foods, spicy foods)
should be encouraged. Unfortunately, dietary manipulation is
often of limited effectiveness because of the fussy nature of
most children regarding diets. Fiber supplements and laxatives
can be critically important in avoiding constipation, which can
significantly affect prognosis.

Activity

 Children with anorectal malformations are often otherwise


healthy.

 Activity limitations are usually related only to the period around


their surgical procedures.

Medication

 Many children with anorectal malformations require medications


for various reasons. Beyond perioperative medications,
maintenance medications often include urinary antibiotic
prophylaxis or treatment and/or laxatives.

 Urinary prophylaxis is used to mitigate the risk of urinary


infection and urosepsis in children with risk factors for urinary
infection such as urinary fistula, vesicoureteral reflux, or
continent diversion. Common agents include oral amoxicillin, oral
trimethoprim/sulfamethoxazole, and gentamicin bladder
irrigations. Comprehensive information on all these medications
and others is available in the eMedicine pediatric topicUrinary
Tract Infection.

 Common laxatives include senna products, milk of magnesia, and


propylene glycol solutions (eg, MiraLax, GlycoLax).

Multimedia

Media file 1: Distal colostogram, posteroanterior view. The initial


phase of augmented-pressure distal colostography aims to determine
where the colostomy was placed in the colon and how much colon is
available for pull-through, without taking down the colostomy.

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Media file 2: Distal colostogram, lateral view. This image shows the
second phase of distal colostography, in which the patient is placed in
the lateral position. A radio-opaque marker is clearly visible in the
lower right side of the image, marking the muscle complex on the skin.
This image shows that the rectal pouch joins the urinary tract at the
level of the bulbar urethra, a relatively common malformation in boys.

(Enlarge Image)

Media file 3: Bucket-handle malformation. The appearance of a


band of skin overlying the sphincteric muscle complex is a
common sign in a child born with imperforate anus and perineal
fistula.

(Enlarge Image)

Media file 4: String-of-pearls malformation. This image shows white


mucoid material within a perineal fistula. The fistula frequently
extends anteriorly up the scrotum's median raphe.

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Media file 5: Cloaca. This is the classic appearance of a girl with
a cloacal malformation with a single perineal orifice. The genitals
appear quite short, which is a finding consistent with cloaca.

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Media file 6: Fourchette fistula. This malformation is somewhere


halfway between perineal fistula and vestibular fistula. The fistula
has a wet vestibular mucosal lining on its anterior half, but the
posterior half is 

(Enlarge Image)

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