Imperforate Anus

Supervisor : Prof.,Dr., Yasser Saad El-Din

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.

Investigations

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 discharge through an orifice on perineum

No meconium Examine urine for meconium or mucus

Meconium/mucus present Low anomaly Fistula present High Anomaly No meconium Inversion radiology

Diagnostic Approach to female anorectal Anomalies

Careful examination of vulva and perinum

Common to urethera, vagina, rectum

Separate urethra ,common rectum& vagina

One each urethera ,vagina, Rectum.

High Anomaly

High Anomaly

Ectopic Anus

Inversion Radiology or needle aspiration of meconium and injection of contrast media High or Low

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 Perineal(cutaneous)fistula Rectourethral fistula Bulbar Prostatic females Perineal(cutaneous)fistula Vestibular fistula

Rectovesical fistula
I

Persistant cloaca Imperforate anus without fistula

mperforate anus without fistula

Rectal atresia

Rectal atresia

Krickenbeck classification (2005):

Major clinical group

Rare /regional variants

Perineal (cutaneous) fistula Rectourethral fistula Prostatic Bulbar Rectovesical fistula Vestibular fistula Cloaca No fistula Anal stenosis

Pouch colon Rectal atresia/stenosis Rectovaginal fistula H fistula Others

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. -Pull through operation. -Closure of colostomy. -Continence work up.

- Cut back in case of membrane. - Perineal anoplasty. - Regular post operative anal dilatation

Initial Pelvic Colostomy:

Descending Colostomy
- whats 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