Presented By

Anu George
MSc Nsg(Peadiatrics) Student
Supervisor:
Dr.(Mrs.) Raman Kalia
Lecturer

Imperforate anus
Includes agenesis and atresia of the rectum and anus
Etiology: unknown
Incidence: 1 in 4,500
SEX: 60% male

ARM: Embryology
The cloaca

(which is a common chamber into the hindgut and allantois)

Separated into:

The bladder

post-allantoic gut
(rectum)
Downward growth
of a septum

Proctadeum
(epiplastic bud)

Fusion during the 3rd month of intrauterine life

when there is imperfect fusion

imperforate anus

Imperforate anus
Low abnormalities

High abnormalities

Termination of bowel below

the pelvic floor

Termination of bowel above

the pelvic floor

Easy to Diagnose
Simple to R

Often have a fistula into the

urinary tract with
deficient pelvic floor
Difficult to R

Outlook is good
1)Covered anus
2)Ectopic anus
3)Stenosed anus
4)Membranous stenosis

1)Anorectal agenesis
2)Rectal atresia
3)Cloaca

1)Covered anus
Anal canal covered by a bar of skin with a
track running forwards to the perineal
raphe. This track is called Bucket Handle
midline Raphe fistula.

2)Ectopic anus

Anus situated anteriorly

in , it opens in perineum
in , it opens in vulva
ectopic anus

or vagina

(more commonly)>>

(rare)

vulval

3)Stenosed anus
i.e. microscopic anus
There is a minute opening that can be seen if
examined carefuly

4)Membranous stenosis
Rare
Anus is

normally sited
covered with a thin membrane that
bulges with retained meconium

Could be associated with:

a fistulas connection between the blind rectal

stump and the bladder
or other pelvic structures abnormally

1)Anorectal Agenesis
Rare
Blind rectal pouch lies just above the pelvic

floor
In
anterior aspect attached to the
bladder & often there is a rectovesical fistula
or rectourethral fistula (manifested by passage
of gas or meconium in the urine) or perineal
fistula
In
fistula to the posterior fornix >>
rectovestibular (or rectovaginal)
low RVF Easy to correct
Post-op. Function is good
high RVF
Difficult to correct
Post-op function is poor

2)Rectal atresia
Rare
Anal canal is normal but ends blindly at the level

of pelvic floor & the rectum also ends blindly

above the pelvic floor without a fistulas opening.

3)Cloaca
Occurs only in
Bowel, urinary & genital tracts all open into a

common wide cavity

Commonly associated with other
developmental abnormalities e.g.
tracheoesophagial fistula

Approach
History >>> the systems involved
Examination >>> DDx
Investigation >>> Dx
Management

History
Failure to pass meconium within the 1st 24

hours of life.

Patients with anterior ectopic anus may

present in childhood or in adulthood. These

patients have lifelong histories of constipation
and painful defecation and usually have
required laxatives or enemas for management.

Examination
Inspection
alone is sufficient for management plan in 90% of patients.
?
Low

Bucket handle defect

Gas/meconium from perineum
Perineal fistula if skin bridge
between vestibule and fistula.
Anterior ectopic anus
i.e. normal sized anus
surrounded by normal muscle.
Urine free of meconium

(NB average of 20 hrs for meconium to appear in urine).

High

Flat bottom
Short sacrum
Little muscle contraction
Meconium per urethra
Rectovestibular fistula if within mucosal
margin of the vestibule.
Sphincter located close to the scrotum
or bifid scrotum often associated
with a Prostatic Fistula.
Cloacae are often missed if urethral
opening is not identified in female
with a recto-vaginal fistula.
Furthermore, cloacae often have small
openings and may be a cause of
referral for ambiguous genitalia.

Investigation
Very Important to:

1. Exclude other anomalies!!

2.Determine whether abnormality is high or low!!

1. Exclude other Anomalies!!!

Anal atresia may occur as a part of the VACTERL

group of anomalies
V
Vertebral body segmentation defect
A
Anal atresia
C
Cardiovascular (PDA, VSD)
TE Tracheo esophagial fistula
R
unilateral Renal agenesis
L
Limb anomaly (radial ray hypoplasia)

So, very careful examination of the baby must be made

to exclude these anomalies

Exclude other Anomalies!!!

(contd)

Associated abnormality

Investigation

"V" Vertebral Abnormality )butterfly

vertebrae, hemi-vertebrae(

Spinal ultrasound, Spinal x-ray

"C" Cardiac, Heart Abnormality

Cardiac ECHO )VSD, ASD, PDA(

Cardiac ECHO

"R" Renal, Kidney abnormality )solitary Renal ultrasound, Voiding cystokidney, horse shoe kidney(
urethra-gram )VCUG(
"TE" tracheoesophogeal abnormality
)TEF(

Physical examination

"L" Limb deformity

Physical examination, x-rays

2.Determine whether abnormality is

high or low!!
1)Invertogram:

with a metal button or a coin strapped to the site of the anus or

a metal bougie inserted into the blind anal canal

Infant is held upside down for 3-4 minutes
Then radiograph in the inverted lateral position ( both the
greater trochanters should be on the same line)
The gas in the rectum will rise to the top indicates the distance
between the site of the metal indicator and the blind end of the
rectum >>> if the distance > 2.5 cm, the abnormality is high!!
if the rectum ends above the PC line (pubococcygeal line = from
the symphysis pubis to the last vertebra), the abnormality is Low
& vise versa or according to ischeal line (between ant.-sup. Iliac
spines).
When to be done?
Although it is a useful method, sometimes vitiated by a plug of
meconium in the rectum causing an apparent gap far in excess of
that actually present. So, it may be necessary to wait until the
baby is 24 hrs old before rectal gas appears

Metal button

PC line
gas in the rectum

2)Urine culture:
Presence of meconium
Presence of proteus or pseudomonas usually signifies that a fistula is present

3)US:
To evaluate the lesion type

4)MRI:
If we suspected complex malformation

5)Micturating cystourethrogram:
By injecting a dye in the urethra

Treatment of Low abnormalities:

1)Covered anus:
The track should be opened by scissors
Followed by routine dilatation of the anus
2)Ectopic anus:
Plastic cut-back operation
3)Stenosed anus:
Regular dilatation

Treatment of High Abnormalities:

Very difficult problem & each case must be considered

on its merits
The possibilities are:
Two-stage operation:
1st stage laparotomy
Division of rectourethral fistula
Transverse colostomy
2nd stage rectal pull-through operation
One-stage operation:
laparotomy
Division of fistula
pull-through operation
Division of fistula & rectal pull-down operation
through the perinum ( now rarely used)

Treatment of High abnormalities

(contd)
The new technique is post-sagittal anorectoplasty

(PSARP)

Colostomy only ( for cloaca)

Pull-through Operation
Lower bowel is mobilized
New passage is created through the

pelvic floor by passing a pair of curved

forceps & through it, keeping close to
the urethra, to the site of the future
anus.
This is dilated by Hegars dilator so
that the bowel can be pulled down and
its mucosa stitched to the skin of the
newly formed anus.
Daily dilatation will be required for at
least 3 months after the operation and
it may be necessary for years.

PSARP

The PSARP dissection is along the midline providing

excellent exposure with minimal trauma to
nerves and musculature.
It involves:
Stimulation of muscles to demonstrate the midline
and sphincter
Posterior sagittal incision - length depends on
severity of abnormality and required extent of
dissection
Rectum identified. Abdominal approach may be
required in addition in 10% of males and 40% of
cloacae
Rectum dissected
Separation from genitourinary tract
Repositioning the neoanus within the sphincteric
mechanism

Position for PSARP

Incision in PSARP

Posterior sagittal repair of a rectovestibular

fistula.

Prognosis
In high % of cases, imperforate anus is associated
with other congenital abnormalities especially of
the urinary organs & nearly 50% of deaths in
cases of imperforate anus are due to other
malformations

Nursing Management
Identification of ARM at birth and prompt

management/ Referral
Prepare for colostomy
Demonstrate to the parents the care of
colostomy
Ensure competent home care and regular follow
up
Preparation for surgery
Post-Op Nursing Management

Nursing Management
(contd)
Post-Op
Immediate post-op care
Prevent Infection and promote healing: Care of

the suture line, antibiotics

Nutrition
Systematic Dilation
Prevention of constipation
Bowel training
Care of colostomy

In a Summary
1 opening >>> Cloaca
2 openings >>> anorectal agenesis with

rectovaginal fistula
3 openings >>> ectopic anus, stenosed anus,
membranous anus, rectal atresia or even
normal anus!!!
The most important investigation is the
invertogram
It is very important to rule out other
anomalies
The best & the newest operation is PSARP

Bibliography
1. Marlow D.R and Redding B.A. Textbook of

Pedatric Nursing. 6th Edition. p:

.
2. Donna L Wong. Essentials of Pediatric
Nursing. 5th Edition. p: 845-847
3. Kliegman and et al(2007); Nelson Textbook of
Pediatrics; 18th edition; pages: 1635-39.

THANKYOU