Evaluation of anorectal

malformation( ARM) in
children
Dr. Md. Tosaddeque Hossain Siddiqui
MBBS, MS (pediatric surgery) ,
Associate Professor,
Department of pediatric surgery
BSMMU
Contents
Introduction
Historical background
Brief embryology
Classification
Clinical evaluation
Management
Follow up
Complications & their management
Outcome
Conclusion
Introduction
Anorectal malformation(ARM) is a
common problem in paediatric
surgical practice.
Incidence is about 1 in 4000-5000
live births.
Timely diagnosis and management
is very important to get satisfactory
result.
Historical background

Throughout the history surgeons have attempted to treat

infants and children with ARM but the written accounts
are scanty probably most of the patient died without
treatment
1st inguinal colostomy for ARM was performed in 1783
but most infants died
Amussat in 1835 mobilized the bowel through a perineal
incision and sutured to the skin
In 1700-1800, several author suggested that the perineum
should be opened if the bowel was not encountered from
below
Cont.
In the mid 1900, single staged abdomino perineal
procedure became popular & usually involved
resection of the recto sigmoid
Shortly after that, Stephens described a procedure that
emphasizied passage of the rectum within the pubo-
rectalis sling
Until early 1980 this surgery plus its modification was
the standard approach, but it involved blind dissection
near the posterior urethra
After 1980, PSARP proposed by Penna was adopted
the procedure of choice because it allows full
visualization of the sphincter complex and urogenital
structures.
Brief embryology
At the end of 7th week of gestation cloacal membrane
ruptures, creating the anal opening for the hind gut,
after that, rapid proliferation of ectoderm closes the
caudal portion of the anal canal and at 9th week of
gestation this region recanalizes
Recto anal atresia and fistulas are due to ectopic
positioning of the anal opening and not due to defects
in the uro-rectal septum
Imperforate anus occurs due to lack of recanalization
of the lower portion of the anal canal
Classification by Penna
Male defects

Perineal fistula
Recto-bulbar-urethral fistula
Recto-prostatic-urethral fistula
Recto-vesical fistula
Imperforate anus without fistula
Rectal atresia
Cont.
Female defects
Perineal fistula
Recto-vestibular fistula
Imperforate anus without fistula
Persistent cloaca
Rectal atresia
Complex malformations
-Cloacal malformation with a short common channel(<3cm)
-Cloacal malformation with a long common channel (>3cm)
-H- shaped fistula(recto-vaginal)
-Rectal duplication
-Associated with presacral mass
Clinical evaluation
History:
-Antenatal history of polyhydramnios on USG during
routine visit.
-Perinatal history: date and time of delivary, meconium
staining of the urine and clothings.
Clinical examination:
-General physical examination, JACCOL
-Examination of the perineum
-Examination of the back
-Examination for VACTERL association
Perineal examination

Common to both sex

Site and size of the anus
Presence of anal dimple
Perineal profile
-Good looking perineum
-Flat bottom
Meconium staining of the perinium, thigh,
clothings and urine
Perineal examination in male

Sub epithelial tract

Bucket handle skin tag
Urethral opening
Figure: perineal
cutaneous fistula and
bucket handle skin
tag
Fig. 79.5. Low imperforate anus
in a male. Bucket handle
shown at site of covered anus
with small amount of meconium
extruding (arrow).
Fig. 79.4. Low imperforate
anus in a male. Well developed
raphe that will probably
demonstrate fistula
with meconium extrusion
over first 1-2 days of life.
Fig. 79.1. High imperforate anus in a male. Median raphe is flat and without any
signs of meconium extrusion
Figure 11.2 The featureless perineum of a male baby with a
rectourethral fistula
Anocutaneous fistula
Perineal examination in female

Site of the anal opening

Urethral opening
Fig. 79.3. Low
imperforate
anus in a female
with fistula
visible at the
posterior
fourchette
(vestibular
fistula).
Fig. 79.2. Young
female with anterior
ectopic anus.
Arrows mark the
posterior edge of
the vaginal opening
and the anus.
These two structures
are too
close, and the
anal opening lies
outside the anal
dimple.
Figure 11.4 A rectovestibular fi stula in a female with the
thermometer passing cranially up the fistula behind the vagina.
Figure 11.1 A schema of the more common
varieties of anorectal malformations. (A1)
Male perineal fi stula (A2) Female perineal
fi stula (B1) Rectoprostatic fi stula (B2)
Rectobulbar fi stula (C1) Rectovesical fi stula
(C2) Rectovestibular fi stula (D1) Cloacal
anomaly (D2) No fi stula (E) Anal stenosis.
Examination of the back

Any swelling at the back

Stigmata of spina bifida
Investigations
Urine RME
USG of abdomen especially of KUB
region
USG of pelvis through perineum
USG of lumbosacral spine
Crosstable lateral prone X-ray
Figure 11.6 A micturating cystourethrogram showing contrast
in a rectourethral fistula outlining the distal colon as well as the
bladder.
Fig. 79.6. High imperforate anus in
a male undergoing colostogram.
Arrows trace
the path of the colourethral fistula
Examination under anaesthesia

Panendoscopy
Contrast study of common channel
Contrast study of
common channel of
cloacal
malformation
Figure 11.6 A
micturating
cystourethrogram
showing contrast
in a rectourethral
fi stula outlining
the distal colon as
well as the
bladder.
Pictures
Cont.
Cont.
Cont.
Cont.
Cont.
Cont.
Clinical presentation
Acute presentation: Presents like intestinal
obstruction, emergency management.

Late presentation: Elective procedures.

Emergency management
(For acute presentations)
Nil by mouth
Nasogastric suction
I/V fluid
I/V antibiotic
Inj. Vitamin K
Measures to prevent heat loss
Transportation: Hypothermia is the main risk during
transport.
 Management
Decision making algorithm for male newborn with
ARM
Perineal inspection

No perineal fistula + No
Perineal fistula No perineal meconium in the urine
fistula +
Mecomeum
in the urine

Anoplasty Cross table lateral X-ray in

prone position

Colostomy
-Rectal gas above coccyx
- Rectal gas extends
-Associated defects present
below coccyx
-Abnormal sacrum
-Flat bottom perineum -No associated defects

Colostomy
PSARP with or
without colostomy
Decision making algorithm for female baby
Perineal inspection
Single
perineal Perineal No visible
Vestibular fistula
opening fistula fistula
<10%
(cutaneous)

Cloaca
Colostomy or Cross table
-urological evaluation Primary lateral prone
-rule out hydrocolpos Anoplasty PSARP X-ray
or
Dilatation

Colostomy +/-
High Rectum
Colpostomy
rectum below
coccyx
Urinary diversion
if necessary

Colostomy
Colostomy or Primary
PSARP
PSARP
Posterior sagital ano recto plasty (PSARP)
Perineal muscle fibres are arranged in different
alignment.
- Parasagital fibres: which forms the external anal
sphincter .
- Vertical fibres: Known as muscle complex
- Levator ani muscles.
Figure:
Perineal
muscle
arrangement
Figure: Levator
ani muscles
Specific management
Ano-cutaneous fistulas: Anoplasty or minimal PSARP
Recto-vestibular fistula: (Initial colostomy then)
Limited PSARP
Recto-urethral fistulas and low cloacal malformation:
Initial colostomy (and colpostomy +/-vesicostomy)
then Full PSARP/PSARVP
Recto-bladder neck fistula and high cloacal
malformation: Initial colostomy then PSARP +
Abdominal approach
Colostomy
Principles of PSARP
Dissection through strict midline of the perineum
Equal display of parasagital and vertical fibres
Incision may extend upward upto the middle of the
sacrum and anteriorly through the perineal body,
encircling around a fistulous opening if present.

Advantages: Minimum post operative pain, No or little

chance of damage to the muscles and structures like,
urethra, vas deference , seminal vesicles etc, proper
anal position is maintained.
Follow up after colostomy

Colostomy care
Distal colostogram
Advise to come after one month for PSARP
Management of the complication of colostomy (if
present)
Complications of colostomy
Bleeding
Prolapse
Retraction
Stenosis
Skin excoriaton
Colostomy diarrhoea
Paracolostomy hernia
Fecal impaction in distal loop
Urinary tract infection
Post operative care after PSARP
Foleys catheter should be kept for 5-14 days
Antibiotic ointment on the wound site
I/V antibiotics for one week
If abdomen is not opened oral feeding can be started
on the day of surgery
Anal dilatation: Dilatation with Hegars dilator set,
started two weeks after PSARP. Calibration to
determine the size which goes easily but fits snuggly,
then increasing the one size weekly to attain desired
size. Then gradually tappered over 4-5 months
Hegars dilators size

1-4 months no. 12 size

4-8 months no. 13 size
8-12 months no. 14 size
1-3 years no. 15 size
3-12 years no. 16 size
> 12 years no. 17 size
Cont.
When the desired size is reached then the colostomy
can be closed.

But the dilatation should be continued with gradual

tapering of the frequency as follows:
Once daily for one month, every third day for one
month, twice a week for one month, once a week for
one month and once a month for one month.
Follow up after PSARP

Anal dilatation
After attaining desired size the parents should be
advised to come for closure of the colostomy of their
baby
Management of complications if occurs.
Complications of PSARP
During operation: Hemorrhage, injury to the muscles,
urethra, vas deference, seminal vesicles, vagina (in
female baby)
Early complications: Catheter displacement, blockage,
and retention, pain, wound infection, wound
dehiscence
Late complications: Anal or ano-rectal stricture
formation, Urethro vaginal fistula, recurrent recto-
urethral fistula, fecal incontinence, post operative
neurogenic bladder etc.
Outcome
Outcome of low defects are good but for high defects,
very different prognosis for bowel function.
Patients should be evaluated without any medical
treatment.
With medical treatment most patients are clean for 24
hours and allows them to live a normal social life. This
does not mean, they all have normal bowel function.
Without medical help many of these patient suffer
from fecal incontinence.
Cont.
Medical helps:
Not trainable group, for constipation laxatives and
enema. For diarrhoea dietary modification and drugs.
Trainable group: With low defects, toilet training +/-
laxatives or suppositories
Conclusion
Proper diagnostic evaluation of anorectal
malformation is necessary to manage the
problem satisfactorily
T H A N K Y O U A L L