Anorectal malformation ppt 5

my own ppts

1. Power point presentation
on
ANORECTAL MALFORMATION
(ARMs)

2. Anorectal
Malformation
( Imperforate anus)

3. Learning objectives:
•Anal Anatomy
•Definition
•Etiology
•Classification
•Clinical manifestations
•Diagnostic studies
•Complications
•Management
•Nursing management

4. Anal Anatomy

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 rectumand 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:
Exactetiologyisunknown
Itmaybeoccurduetoarrestinembronic
developmentoftheanus,lowerrectumanduro
genitaltractatthe8
th
weekofembryoniclife
Itmaybeassociatedwithcongenitalabnomalies
likedownsyndrome,CHD,undescended
testes,renalabnormalitiesandneuraltube
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

15. Classification
Anorectal malformations represent a wide
spectrum of defects
Newborn girl with imperforate anus.
Newborn boy with imperforate anus.

16. Classification:
Males
1. Rectoperineal fistula
2. Rectourethral fistula
3. Recto–bladder neck fistula
4. Rectal atresia
Females
1.Rectoperineal fistula
2.Vestibular fistula
3.Rectal atresia
4.Persistent Cloaca

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
Rectalatresiaisan
abnormality
mostlyoccursin
newbornmale
presenting
abdominal
distensionand
failureofpassing
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 atresiais 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 cloacais defined as a defect in which the rectum, vagina, and
urinary tract meet and fuse, creating a single common channel.
•The diagnosis of persistent cloacais 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 b\w 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 examinationwhen 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. •Abdominalultrasound(alsocalledsonography).A
diagnosticimagingtechniquewhichuseshigh-
frequencysoundwavesandacomputertocreate
imagesofbloodvessels,tissues,andorgans.
Ultrasoundsareusedtoviewinternalorgansandto
assessbloodflowthroughvariousvessels.
•Computedtomographyscan(alsocalledaCTorCAT
scan).Adiagnosticimagingprocedurethatusesa
combinationofX-raysandcomputertechnologyto
producehorizontal,oraxial,images(oftencalled
slices)ofthebody.ACTscanshowsdetailedimagesof
anypartofthebody,includingthebones,muscles,fat,
andorgans.CTscansaremoredetailedthangeneralX-
rays.

32. •Magneticresonanceimaging(alsocalledaMRI).Adiagnosticprocedure
thatusesacombinationoflargemagnets,radiofrequencies,anda
computertoproducedetailedimagesoforgansandstructureswithinthe
body.
•LowerGI(gastrointestinal)series(alsocalledbarium
enema).AlowerGIseriesisaprocedurethatexaminestherectum,the
largeintestine,andthelowerpartofthesmallintestine.Afluidcalled
barium(ametallic,chalky,liquidusedtocoattheinsideoforganssothat
theywillshowuponanX-ray)isgivenintotherectumasanenema.An
X-rayoftheabdomenshowsstrictures(narrowedareas),obstructions
(blockages),andotherproblems.
•UpperGI(gastrointestinal)series(alsocalledbarium
swallow).UpperGIseriesisadiagnostictestthatexaminestheorgans
oftheupperpartofthedigestivesystem:theesophagus,stomach,and
duodenum(thefirstsectionofthesmallintestine).Afluidcalledbarium
(ametallic,chalky,liquidusedtocoattheinsideoforganssothatthey
willshowuponanX-ray)isswallowed.X-raysarethentakentoevaluate
thedigestiveorgans.

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.

37. Complications
•Wound infection
•Anal stenosis
•Constipation
•UTI
•Intestinal obstruction
•Reoccurance of fistula
•Fecal impaction

38. MANAGEMENT
Surgical interventionisthe only method to
correct this abnormality.
A temporary colostomy may be performed.
Medications may be administer to prevent UTI.
A posterior sagittalanorectoplastycan then be
performed between 2 and 12months

47. NURSING MANAGEMENT
During the first 24 hours,
the neonate should:
•Take nothing orally.
•Be examined carefully (perinealexaminaton)
•receive intravenous fluids
•and antibiotics
•Lab investigations may be done to detect abnormalities.
•Maintenance of warmth,fiuidand eletrolytebalance.
•Maintain general stability of infant.
•Routine post operative care to be provided after colostomy and
definitive surgery.

48. Pre-operative care:
•Maintanenceof 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:
•Preventionofskinbreakdownaroundcolostomy
wound
•Preventionofinfection
•Maintainfluidandelectrolytebalance
•Emotionalsupportforfamilycoppingand
demonstrationofcolostomycareareessential
aspectsinnsg.management.
•Healthedu.tobegivenaboutcontinuationofcare
athome,dietmodificationpreventionoffecal
impaction,bowelhabbittrainingandneedfor
medicalhelp.

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

52. PSARP Position

53. PSARP PROCEDURE
STAY SUTURES RECTAL POUCH

54. PSARP PROCEDURE
AFTER MOBILIZATION AFTER CLOSURE
FINAL PICTURE FOLLOW UP PICTURE

55. Abdominoperinealpull through

56. involves a midline incision from the fistula to the putative
site of the anus.
division of the muscles in the midline,separationof the
rectum from the vagina under vision
placement of the rectum within sphinctericcomplex and
reconstruction of the perinealbody.
Indications:
All low and intermediate type of abnormality in females
Revision surgery following cutback operations
ASARP
56

57. ASARP-procedure
57

58. 58

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

62. 1.Voluntary muscle structures.
2.Sensation.
3.Bowel motility
Fecal continence:
depend on 3 factors

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

69. Management protocol of Congenital
Anorectal Malformation in Boys

70. Management protocol of Congenital
Anorectal Malformation in Girls