10. Definition Of ARMs
are birth defects in which the rectum and anus are
malformed, do not develop properly.
ARMs
happen as a fetus is developing during pregnancy.
• During a bowel movement, stool passes from the
large intestine to the rectum and then to the anus
11. Incidence
One/5000
Slightly more common in males.
Rectourethral Fistula: is the most frequent type in male
Rectovestibular: is the most frequent type in females
followed by perineal fistulas & then a cloaca
Imperforate anus without fistula occurs in about 5% and
is associated with Down syndrome.
12. Etiology:
Exact etiology is unknown
It may be occur due to arrest in embronic
development of the anus, lower rectum and uro
genital tract at the 8th week of embryonic life
It may be associated with congenital abnomalies
like down syndrome, CHD, undescended
testes,renal abnormalities and neural tube
defect.
13. Higher ARM = more chance of urologic abnormalities
{90% cloacas or recto-bladder but 10% in perineal fistulas)
Hydronephrosis, urosepsis, and metabolic acidosis from poor
renal function represent the main sources of morbidity in infants
with anorectal malformations.
Genitourinary
14. 1. Absent one vertebrae: mostly has no effect.
2. Absent More than two: poor prognostic for continence
3. Hemi sacrum is usually associated with a presacral mass and poor
bowel control.(s2&s5)
4. Tethered cord: may associated with bad prognosis
Sacrum & Spinal
The most frequently association
Normal sacral ratio is 0.77.
Never seen good bowel control
with ratio < 0.3
17. 1.Rectoperineal Fistulas
• Rectoperineal fistula is what traditionally was known as a “low
defect.”
• It is found as small orfice in the perinium, usually anterior to
the centre of the external sphinctor.In male babies it is found
close to the scrotum.
• This diagnosis is established by perineal inspection. No further
investigations are required.
18. 2.Rectourethral fistula
Imperforate anus with a recto urethral fistula is
the most frequent defect in male patients.
A recto-urethral is a hole between the urethra and
the rectum. This hole leads to leakage of the
urine into the rectum and feces into the bladder.
19. 3.Recto-Bladder Neck Fistulas
• In this defect, the rectum opens into
the bladder neck.
• Not passing feces the first day or two
after birth.
• Passing feces through another
opening such as urethera.
• Repeated UTI.
• About 10% of males with anorectic
atresia fall into this category.
20. 4.Rectal Atresia
Rectal atresia is an
abnormality
mostly occurs in
newborn male
presenting
abdominal
distension and
failure of passing
meconium
21. FEMALE ANORECTAL DEFECTS
1.Rectoperineal Fistulas
• The rectum is well positioned within the sphincter mechanism,
except for its lower portion, which is anteriorly located.
• The rectum and vagina are well separated .
•It is found as small orfice in the
perinium, usually anterior to the centre of
the external sphinctor.In female babies it
is found close to the vulva.
22. 2.Rectovestibular Fistulas
• Rectovestibular fistula is the most common defect in
girls and has an excellent functional prognosis.
• It is an anorectal congenital disdorder where an
abnormal connection between the rectum and the
vulval vestibule of the female4 genitalia.
• The diagnosis is based on clinical examination.
24. Rectal atresia is a rare abnormality in newborn
female presenting with an abdominal distension
and failure of passing meconium
3.Rectal atresia
25. 4.Persistent Cloaca
• This group of defects represents the extreme in the spectrum of
complexity of female malformations.
• A cloaca is defined as a defect in which the rectum, vagina, and
urinary tract meet and fuse, creating a single common channel.
• The diagnosis of persistent cloaca is a clinical one.
• This defect should be suspected in a female born with imperforate
anus and small-looking genitalia.
The length of the common channel varies from 1 to 7 cm.
This distance has technical and prognostic implications.
1. Short common channel less than 3 cm
2. Long common channel more than 3 cm
26. ARMs classification based on levator ani
mucsle(muscle of fecal control)
Supralevator or high ARMs: When rectum terminates above
the levator ani muscle. Eg:rectal atresia,rectovaginal fistula. About
30 % of children achieve bowel continence due to high ARMs.
Translevator or low ARMs: When rectum terminates below
the levator ani muscle. Eg: anovestibular fistula. About 90 % of
children achieve bowel continence due to low ARMs.
27. Clinical features
New born do not pass stool even after 24 -48 hrs.
of birth.
Opening to the anus is either absent or is displaced
to different location.
Stool may pass out, either through vagina, base of
penis, scrotum or urethera.
Abdominal distension and vomiting may . be
present.
Presence of fistula bw the rectum, reproductive
organ and urethera is observed.
Presence of meconium in urine
30. Diagnostic findings
• Invertogram( x-ray with inverted infant):Useful
to locate rectal pouch which can be performed only after the
infant is 24 of age
Your child's doctor will perform a physical examination when your
baby is born, and will look at the anus to see if it is open. Diagnostic
imaging tests may be done to further evaluate the problem, such as
•Abdominal X-rays. Diagnostic test which uses
invisible electromagnetic energy beams to produce
images of internal tissues, bones, and organs onto film.
31. • Abdominal ultrasound (also called sonography). A
diagnostic imaging technique which uses high-
frequency sound waves and a computer to create
images of blood vessels, tissues, and organs.
Ultrasounds are used to view internal organs and to
assess blood flow through various vessels.
• Computed tomography scan (also called a CT or CAT
scan). A diagnostic imaging procedure that uses a
combination of X-rays and computer technology to
produce horizontal, or axial, images (often called
slices) of the body. A CT scan shows detailed images of
any part of the body, including the bones, muscles, fat,
and organs. CT scans are more detailed than general X-
rays.
32. • Magnetic resonance imaging (also called a MRI). A diagnostic procedure
that uses a combination of large magnets, radiofrequencies, and a
computer to produce detailed images of organs and structures within the
body.
• Lower GI (gastrointestinal) series (also called barium
enema). A lower GI series is a procedure that examines the rectum, the
large intestine, and the lower part of the small intestine. A fluid called
barium (a metallic, chalky, liquid used to coat the inside of organs so that
they will show up on an X-ray) is given into the rectum as an enema. An
X-ray of the abdomen shows strictures (narrowed areas), obstructions
(blockages), and other problems.
• Upper GI (gastrointestinal) series (also called barium
swallow). Upper GI series is a diagnostic test that examines the organs
of the upper part of the digestive system: the esophagus, stomach, and
duodenum (the first section of the small intestine). A fluid called barium
(a metallic, chalky, liquid used to coat the inside of organs so that they
will show up on an X-ray) is swallowed. X-rays are then taken to evaluate
the digestive organs.
36. • IVF & antibiotics & Evaluate Associated defects
1. ECHO for Cardiac evaluation
2. US Abdomen to evaluate Hydronephrosis.
3. US Spinal to evaluate tethered cord.
• Important not to make decision about a colostomy or a primary
operation before 24 hours of life.
• Radiologically may not show the correct anatomy before 24
hours because the rectum is collapsed.
38. MANAGEMENT
Surgical interventionis the only method to
correct this abnormality.
A temporary colostomy may be performed.
Medications may be administer to prevent UTI.
A posterior sagittal anorectoplasty can then be
performed between 2 and 12months
47. NURSING MANAGEMENT
During the first 24 hours,
the neonate should:
• Take nothing orally.
• Be examined carefully (perineal examinaton)
• receive intravenous fluids
• and antibiotics
• Lab investigations may be done to detect abnormalities.
• Maintenance of warmth,fiuid and eletrolyte balance.
• Maintain general stability of infant.
• Routine post operative care to be provided after colostomy and
definitive surgery.
48. Pre-operative care:
• Maintanence of warmth
• Maintain fluid and electrolyte
balance
• Measurement of abdominal girth is
important before surgery
• Special care to be provided for
colostomy
49. Post-operative care:
• Prevention of skin breakdown around colostomy
wound
• Prevention of infection
• Maintain fluid and electrolyte balance
• Emotional support for family copping and
demonstration of colostomy care are essential
aspects in nsg.management.
• Health edu. to be given about continuation of care
at home, diet modification prevention of fecal
impaction, bowel habbit training and need for
medical help.
50. 1. Wound infection: in the immediate postoperative period
2. Anal strictures: 2ry to failure of dilatations protocol or
devascularization during the rectal mobilization.
3. Rectal prolapse: more in higher ARM
4. Constipation is the most common functional disorder.
5.Fecal Incontinence: is a very common sequelae
6. Neurogenic bladder: in male patients, either technical or due
to very abnormal sacrum
Post operative complications
51. What is the Best Type of Colostomy for ARM?
Divided Descending Colostomy
As to leave enough redundant, distal rectosigmoid colon to allow for
the subsequent pull through
Others
56. involves a midline incision from the fistula to the putative
site of the anus.
division of the muscles in the midline,separation of the
rectum from the vagina under vision
placement of the rectum within sphincteric complex and
reconstruction of the perineal body.
Indications:
All low and intermediate type of abnormality in females
Revision surgery following cutback operations
ASARP
56
59. Advantages:
LAARP allows the surgeon to treat a high lesion like
a low lesion.
No need to divide the muscle complex from below.
Immediately after the procedure strong and
symmetric contraction of the sphincter around the
neoanus can be seen.
Laparoscopically assisted anorectal
pull through (LAARP) for high ARM
60. It also avoids the,complication and multiple
procedures associated with colostomy.
More rapid return of bowel function
Improved cosmetic appearance
Shorter postoperative recovery
Decreased postoperative complications
63. Levator, muscle complex, and the external sphincter
Normally: used only for brief periods when the rectal fecal mass,
pushed by the involuntary peristaltic contraction of the
rectosigmoid, reaches the anorectal area. This contraction occurs
only in the minutes prior to defecation.
Patients with ARM has different degrees of underdevelopment
Voluntary muscles can be used only when the patient feels that it
is necessary to use them. For that sensation, the patient needs
information that can only be derived from an intact sensory
mechanism, a mechanism that many patients with anorectal
malformations lack
1- Voluntary muscle structures
64. 1- Not or rudimentary Anal canal sensation: Because the Child with
ARM born without an anal canal {Except rectal atresia}
2- Rectum distention can be felt in many of these patients, provided
the rectum has been located accurately within the muscle
structures. This sensation (proprioception) seems to be a
consequence of stretching of the voluntary muscle.
• So liquid stool or soft fecal material may not be felt by the patient
with ARM as the rectum is not distended
• Thus, to achieve some degree of sensation and bowel control, the
patient must be able to (or helped to) form solid stool
2- Sensation in anal canal
65. Is the most important factor in fecal continence
However, impact of motility has largely underestimated
In a normal individual, the rectosigmoid remains quiet for
variable periods of time (one to several days), depending on an
individual’s defecation habits. During that time, anorectal
sensation and voluntary muscle structures are almost
unnecessary { stool in the rectosigmoid if it is solid}.
The patient normally feels the rectosigmoid’s peristaltic
contraction.
3- Bowel Motility
66. Voluntarily, the normal individual can relax the striated muscles,
which allows the rectal contents to migrate down into the highly
sensitive area of the anal canal.
There, accurate information is provided concerning the
consistency and quality of the stool.
The voluntary muscles are used to push the rectal contents back
up into the rectosigmoid and hold them if desired, until the
appropriate time for evacuation.
At the time of defecation, the voluntary muscle structures relax,
allowing the fecal mass to pass into and through the anorectum
The main factor that initiates emptying of the rectosigmoid is a
massive involuntary peristaltic contraction that is helped
sometimes by a Valsalva maneuver.
Normal Bowel control