Imperforate Anus
Formation of cloaca at 3rd week:
• The hindgut forms the posterior portion of the cloaca( the
future anorectal canal)
• the allantois forms the anterior portion ( the future urogenital
sinus. )
Development of urogenital sinus and rectum at
7th week:
• Cloaca division into rectum and urogenital tract is initiated
by the caudal movement of ( the urorectal septum)
• proliferation of ectoderm closes the caudalmost region of the
anal canal. During the ninth week, this region recanalizes.
rectoanal atresias and fistulas :
are due to ectopic positioning of the anal opening.
imperforate anus:
there is no anal opening. This defect occurs because of a
lack of recanalization of the lower portion of the anal canal
Causes, incidence, and risk factors
It occurs in about 1 out of 5,000 infants.
• The rectum may end in a blind pouch that does not connect with the
colon.
• The rectum may have openings to the urethra, bladder, and base of the
penis or scrotum in boys, or vagina in girls.
• There may be narrowing (stenosis) of the anus or no anus.
Symptoms
• Anal opening very near the vagina opening in
girls
• Baby does not pass first stool within 24 - 48
hours after birth
• Missing or moved opening to the anus
• Stool passes out of the vagina, base of penis,
scrotum, or urethra
• Swollen belly area
Signs and tests
• A doctor can diagnose this condition during a physical exam.
Imaging tests may be recommended.
Associated anomalies
• V - Vertebral anomalies
• A - Anal atresia
• C - Cardiovascular anomalies
• T - Tracheoesophageal fistula
• E - Esophageal atresia
• R - Renal (Kidney) and/or radial anomalies
• L - Limb defects
Diagnosis
• It is usually detected quickly as it is a very obvious defect.
• It is important to determine the presence of any associated defects
during the newborn period in order to treat them early and avoid
further sequelae.
• Sonography can be used to determine the type of imperforate anus.
• The decision to open a colostomy is usually taken within the first 24
hours of birth.
Investigation
s
1-Invertogram
2- Perineal U/S or MRI
3- X-ray
4- Colostogram
5-Abdominal U/S
Evaluation for other anomalies
Invertogram
- X-ray with head down hips flexed at 90 degrees and legs
flexed at 90 degrees
Baby held for several minutes to allow air to pass into the rectal
pouch.
To find out the level of the rectal atresia by viewing how far the gas
has reached in relation to area where sphincter should be (Put a coin)
High lesions are above the levator if the distance between level of the
air and coin more than 2cm.
Intermediate lesions are characterized by the rectal pouch ending
within the levator,
Low lesions, the rectal pouch has completely traversed the levator
musculature, the distance between level of the air and coin less than
2cm .
• If required, the level of the rectal pouch can be delineated more
definitively by ultrasonography or magnetic resonance imaging.
• Perineal ultrasonography may be useful in determining the distance
between the rectal pouch and the anal skin.
Abdominal radiograph performed at day one
of life, shows multiple air filled distended
bowel loops suggestive of bowel obstruction.
Colostogram
Should be done under pressure to illustrate any fistula
Distal colostogram showing
the colon ending in a long,
narrow rectourethral fistula
Abdominal U/S
During the first 24 hrs. of life,
All these patients need abdominal ultrasound
evaluation To identify an obstructive uropathy
especially in patients with;
Rectovesical fistula
Rectoprostatic urethral fistula
Evaluation for other anomalies
1-Chest x-ray
2-Lumbosacral x-ray
3-Abdominal pelvic ultrasonography
4-Kidney Ureter Bladder KUB x-ray
5-IVU
6-Echocardiography and ECG
7-Passage of nasogastric tube
Ivu showingRectovesical
fistula
Diagnostic approach to male anorectal anomaly
EXAMINE THE PERINEUM FOR THE MECONIUM
Meconium No meconium
discharge Examine urine for meconium
through an or mucus
orifice on
perineum
Meconium/mucus present
Low anomaly No meconium
Fistula present
Inversion
radiology
High Anomaly
Diagnostic Approach to female anorectal Anomalies
Careful examination of vulva and perinum
Common Separate One each
to urethra urethera
urethera, ,common ,vagina,
rectum& Inversion Radiology or
vagina, Rectum.
rectum vagina needle aspiration of
meconium and injection
of contrast media
Ectopic
High High High or Low
Anus
Anomaly Anomaly
Wingspread classification (1984)
Rectovaginal fistula
Intermediate High
RectoVestibular fistula Persistant cloaca
Rectourethral
fistula Bulbar Prostatic
Rectal atresia
Bucket handle fistula
rectovesical
Vestibular fistula
Perineal fistula or an
anterior ectopic anus
Pena classification (Therapeutic classification) 1995
Males females
Perineal(cutaneous)fistula Perineal(cutaneous)fistula
Rectourethral fistula Vestibular fistula
Bulbar
Prostatic
Rectovesical fistula Persistant cloaca
I mperforate anus without fistula Imperforate anus without fistula
Rectal atresia Rectal atresia
Krickenbeck classification (2005):
Major clinical group Rare /regional variants
Perineal (cutaneous) fistula Pouch colon
Rectourethral fistula Rectal atresia/stenosis
Prostatic Rectovaginal fistula
Bulbar H fistula
Rectovesical fistula Others
Vestibular fistula
Cloaca
No fistula
Anal stenosis
Treatment of imperforate Anus
• Preoperative Therapy
• NICU admission
• IV fluids …NPO & NG tube to exclude TOF.
• Antibiotics
• Treatment of associated anomalies.
• Surgical treatment :
-Initial pelvic colostomy.
- Cut back in case of membrane.
-Pull through operation.
- Perineal anoplasty.
-Closure of colostomy.
- Regular post operative anal
-Continence work up.
dilatation
Initial Pelvic Colostomy:
• Descending Colostomy
- what’s colostomy ?
- Advantages over other
types
- Errors of colostomy
Algorism of Management
Algorism of Management
Pull through operation
• Posterior sagittal anorectoplasty(PSARP) = THE
BEST
• Anterior sagittal anorectoplasty(ASARP)
• Followed by post operative dilatation.
Algorism of Management
Postoperative Management
• Rectouetheral fistula
• Nutrition??
• Dilatation ??
Postoperative functional disorders
• Constipation is the most common problem.
• Intestinal Obstruction.
• Bowel incontinence.
• Urinary tract infection.
• Fecal impaction.
• Colostomy related problems.( Like inflammation & colostomy Prolapse)
• Recurrence of fistula & anal stenosis.
Thank You
