Imperforate Anus

Imperforate anus

• is a congenital abnormality that occurs when the

opening of the anus is either absent or improperly
formed in newborns. This condition can range from a
partial obstruction to a complete absence of the anal
opening, leading to difficulties in passing stool.
What is Imperforate Anus?
 What is Imperforate Anus?
• When a malformation of the anus is present, the muscles
and nerves associated with the anus often have a similar degree
of malformation.
• The position and nature of these malformations repaired
difficult for early surgeons; the affected organs are located deep
in the pelvis and are not well visualized through abdominal
incisions.
 Pathophysiology

• The embryogenesis of these malformations remains

unclear.
• The rectum and anus are believed to develop from the
dorsal portion of the hindgut or cloacal cavity when
lateral ingrowth of the mesenchyme forms the urorectal
septum in the midline.
 Pathophysiology
• 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.
• Down growth 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.
 Pathophysiology

• 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.
 Statistics and Incidences
• Anorectal malformations occur in approximately 1 newborn per
5000 live births.
• Anorectal and urogenital malformations are rarely fatal,
although some associated anomalies (cardiac, renal) can be life-
threatening.
• Intestinal perforation or postoperative septic complications in a
newborn with imperforate anus can result in mortality or severe
morbidity.
 Statistics and Incidences
• Malformation-related morbidity relates to associated
malformations of rectal motility, anorectal innervation, and
sphincteric musculature; the most common morbidity in this
category is constipation; most children have mild malformations
that commonly result in constipation for reasons that remain
unclear.
• The most severe forms of malformation-associated morbidity are
fecal and urinary incontinence.
 Statistics and Incidences

• No known racial predilection has been reported.

• No known sex predilection has been reported.
• Most children with an anorectal malformation are
identified upon routine newborn physical examination.
 Clinical Manifestations
• Newborns with imperforate anus are usually identified upon the
first physical examination.
• Absence of stool
• Passing of stools in other openings
• Swollen belly
• Absence of anal opening
 Clinical Manifestations

• Absence of stool. There is no passage of stool within a

day or two of birth.
• Passing of stools in other openings. The infant may
pass stools through another opening like the urethra in
boys or vagina in girls.
 Clinical Manifestations

• Swollen belly. The newborn could not pass out stools,

resulting in a swollen belly.
• Absence of anal opening. The opening of the anus is
missing or not in its usual place; in girls, this may be
near the vagina.
Assessment and Diagnostic Findings
• An X-ray showing imperforate anus
 Assessment and Diagnostic Findings
• In the diagnosing imperforate anus the following are suggested:
• Laboratory studies. CBC count, blood typing and screening,
and serum electrolyte levels should be measured in all children
with imperforate anus who require operation; urinalysis should
be performed to determine the presence of a rectourinary fistula
in all cases in which the diagnosis cannot be made based solely
on the physical examination findings.
 Assessment and Diagnostic Findings
• Sacral radiography. Two views of the sacrum, posteroanterior and
lateral, should be obtained to measure sacral ratios and to look for sacral
defects, hemivertebrae, and presacral masses; this should be performed
before surgery.
• Abdominal ultrasonography. This study is specifically used to examine
the genitourinary tract and to look for any other masses; hydronephrosis,
hydrocolpos, presacral mass, abdominal mass, or any similar finding can
profoundly affect management.
 Assessment and Diagnostic Findings
• A laparoscopically assisted pull-through for (high) imperforate
anus
 Assessment and Diagnostic Findings

• Spinal ultrasonography or MRI. All children with

any form of anorectal malformation, even those
considered minor, should undergo screening for spinal
malformations; these lesions can be diagnosed using
ultrasonography prior to the ossification of the spine.
 Assessment and Diagnostic Findings
• Lateral pelvic radiography at 24 hours. Children who could not be
diagnosed based solely on physical examination findings traditionally
underwent invertography, which consisted of holding the baby upside
down and using lateral radiography to observe the level of gas in the
distal rectum; a similar, but more humane, approach is to wait 24 hours
after birth to observe for possible maximal pelvic pouch distension and
then to use cross-table lateral pelvic radiography with a radio-opaque
marker on the anal dimple with the child in the prone position and the
hips slightly raised.
 Assessment and Diagnostic Findings
• MRI. All children who have sacral defects on plain radiographs
should undergo spine ultrasonography to rule out associated
malformations, such as meningocele or meningomyelocele,
teratoma, or mixed lesions.
• CT scanning. CT scanning presently plays no role in the
routine evaluation of children with anorectal malformations.
 Medical Management
• Management of an infant with an imperforate anus includes:
• Nothing per orem
• Neonatal colostomy
• Primary neonatal pull-through without colostomy
• Posterior sagittal pull-through with a colostomy
• Colostomy closure
• Diet
• Activity
 Medical Management
• Nothing per orem. Newborns with imperforate anus should not be fed
and should receive intravenous hydration.
• Neonatal colostomy. 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.
 Medical Management
• Primary neonatal pull-through without colostomy. 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.
• Posterior sagittal pull-through with a colostomy. 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.
 Medical Management

• Colostomy closure. Once the wound has completely

healed and postoperative dilations have achieved their
goal (i.e., the neoanus is at the desired size), the
colostomy may be closed in traditional surgical fashion.
 Medical Management
• Diet. After the obstruction is relieved using colostomy, primary pull-through,
or dilation, children do not require special diet; 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 bread,
dairy, fruits, vegetables, greasy foods, spicy foods) should be encouraged.
• Activity. Children with anorectal malformations are often otherwise healthy;
activity limitations are usually related only to the period around their surgical
procedures.
 Pharmacologic Management
• Many children with anorectal malformations require
medications for various reasons.
• Antibiotic prophylaxis
• Laxatives
 Pharmacologic Management
• Antibiotic prophylaxis. 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.
• Laxatives. Common laxatives include senna products, milk of
magnesia, and propylene glycol solutions (eg, MiraLax,
GlycoLax).
 Nursing Management
• Nursing care for an infant with an imperforate anus includes:
• Nursing Assessment
• Nursing Diagnoses
• Nursing Care Planning and Goals
• Nursing Interventions
• Evaluation
 Nursing Management

• Nursing Assessment
• Assessment of an infant with an imperforate anus includes the
following:
• History. Prenatal ultrasonography examination findings are
often normal, although the polyhydramnios or intraabdominal
cysts may suggest an imperforate anus with associated
hydrocolpos or hydronephrosis.
 Nursing Management
• Nursing Assessment
• Physical exam. Newborns with imperforate anus are usually
identified upon the first physical examination; malformations in
newborns that are missed upon initial examination are often
discovered within 24 hours when the newborn is observed to
have distention and has failed to pass meconium and a more
thorough examination is performed.
 Nursing Management
• Nursing Diagnoses
• Based on the assessment data the major nursing diagnoses are:
• Fluid volume deficit
• Impaired skin integrity
• Risk for infection
 Nursing Management
• Nursing Diagnoses

• Fluid volume deficit related to excessive loss

through vomiting.
• Impaired skin integrity related to the colostomy.
• Risk for infection related to surgical procedures.
 Nursing Management
• Nursing Care Planning and Goals

• The major nursing care planning goals for patients with

imperforate anus:
• Maintaining adequate hydration with moist mucous
membranes, good skin turgor, and adequate capillary refill.
 Nursing Management
• Nursing Care Planning and Goals

• Maintaining stable vital signs.

• Achieving adequate output of urine.
• Achieving dry and damage-free skin around the colostomy.
• Absence of infection.
 Nursing Management

• Nursing Interventions
• Nursing interventions for a child with imperforate anus are:
• Avoid infection
• Protect skin integrity
• Restore balanced fluid volume
 Nursing Management

• Nursing Interventions

• Avoid infection. Teach the caregivers to keep the area around

the colostomy clean with soap and water and to diaper the baby
in the usual way; monitor white blood cell (WBC) count; and
wash hands and teach patient and SO to wash hands before
contact with patients and between procedures with the patient.
 Nursing Management

• Nursing Interventions

• Protect skin integrity. A protective ointment is useful to

protect the skin around the colostomy; monitor site of impaired
tissue integrity at least once daily for color changes,
redness, swelling, warmth, pain, or other signs of infection; and
keep a sterile dressing technique during wound care.
 Nursing Management

• Nursing Interventions

• Restore balanced fluid volume. Administer parenteral fluids as

prescribed; consider the need for an IV fluid challenge with an
immediate infusion of fluids for patients with abnormal vital
signs; teach family members how to monitor output in the home;
instruct them to monitor both intake and output.
 Evaluation
• Goals are met as evidenced by:
• Maintained adequate hydration with moist mucous membranes, skin
turgor and capillary refill good.
• Maintained stable vital signs.
• Achieved adequate output of urine.
• Achieved dry and damage-free skin around the colostomy.
• Absence of infection.
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