Headlines

 Anal Anatomy
 Embryology of the imperforate anus
 Definition
 Incidence
 Classification
 Approach
 Prognosis
 Photos!!☺☼☻
Anal Anatomy
Anorectal
The cloaca
malformations
(which is a common chamber into the hindgut and allantois)

Separated into:

post-allantoic gut
The bladder (rectum)

Proctadeum
Downward growth (epiplastic bud)
of a septum

Fusion during the 3rd month of intrauterine life

when there is imperfect fusion imperforate anus
Imperforate anus
 Includes agenesis and atresia of the rectum and anus
 Etiology: unknown
 Incidence: 1 in 4,500
 SEX: 60% male
Imperforate
Low abnormalities
anus High abnormalities
Termination of bowel below the Termination of bowel above the
pelvic floor pelvic floor

Easy to Dx Often have a fistula into the

Simple to ttt urinary tract with deficient
Outlook is good pelvic floor
Difficult to ttt
1)Covered anus
2)Ectopic anus 1)Anorectal agenesis
3)Stenosed anus 2)Rectal atresia
4)Membranous stenosis 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 (more commonly)>> vulval ectopic anus

or vagina (rare)
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 abnormalitie
1)Anorectal agenesis
 Rare
 Blind rectal pouch
 lies just above the pelvic floor
 In males
 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
postop. Function is good
high RVF difficult to correct
postop 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.
Inspection
alone is sufficient for management plan in 90% of patients.

Examination
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.
Differential Diagnosis of Failure to Pass Meconium in the
Newborn

 Hirschsprung's disease >>Tight anus, empty rectum, transition zone

 Meconium plug syndrome >> Meconium plugs

 Meconium ileus >> Abdominal distention at birth, cystic fibrosis

 Anorectal malformation >> Absent anus, tight anus or fistula

 Small left colon syndrome >>Transition zone* at splenic flexure

 Hypoganglionosis >> Transition zone*

 Neuronal intestinal dysplasia type A >>Transition zone*, mucosal inflammation

 Neuronal intestinal dysplasia type B >> Megacolon

 Megacystis-microcolon-intestinal hypoperistalsis syndrome >> Microcolon,

megacystis
*--Transition zone (from small- to large-diameter bowel) refers to radiographic visualization on contrast study.
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
 Associated abnormality Investigation

"V" Vertebral Abnormality (butterfly Spinal ultrasound, Spinal x-ray

vertebrae, hemi-vertebrae)

"C" Cardiac, Heart Abnormality Cardiac ECHO

Cardiac ECHO (VSD, ASD, PDA)

"R" Renal, Kidney abnormality (solitary Renal ultrasound, Voiding cysto-

kidney, horse shoe kidney) urethra-gram (VCUG)

"TE" tracheoesophogeal abnormality Physical examination

(TEF)

"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
 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)

 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?
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:
Cont. Treatment of High abnormalities:

 The new technique is post-sagittal rectoplasty

(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 Hegar’s 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
Published by Dr. Albeto Peña >> It is assumed that the
innervation to the sphincter mechanism is paired and meets in the
midline. 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
IN Summary
 1 common single opening in girls>>> 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
Cloaca
Anorectal agenesis and rectovestibular fistula.
Anourethral fistula
imperforate anus and a rectoperineal fistula .
imperforate anus and a bucket-handle malformation
Covered anus
hank you!!

References:
 Bailey & Love’s short practice of surgery (24th edition)
 The mcinatti children’s hospital medical center (2007)
 University of Michigan, Section of Pediatric Surgery
 Clinical picture from Emedicine website & Cinncinatti
CHMC.