Imperforate Anus
Supervisor : Prof.,Dr., Yasser Saad El-Din
Formation of cloaca at 3rd week: •
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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 7 th week: •
Cloaca division into rectum and urogenital tract is initiated by the caudal movement of ( the urorectal septum )
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proliferation of ectoderm proliferation ectoderm closes the caudalmost 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.
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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) (stenosis) of the anus or no anus.
Sym S ymp ptoms •
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Anal opening very near the vagina opening in girls Baby does not pass first stool 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
Siigns and tests S •
A doctor can diagnose this condition during a physical exam. Imaging tests may be recommended.
Asssociated anomalies A • • • • • • •
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 •
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It is usually detected quickly as it is a very obvious defect. defect. It is important to determine the presence of any associated defects during the the newborn newborn period in order to treat them early and avoid further sequelae sequelae.. Sonography can be used to determine the type of imperforate imperforate anus. The decision to open a colostom colostomy y is usually taken within the first 24 hours of birth.
1-Invertogram 2- Perineal U/S or MRI
3- X-ray 4- Colostogram
5-Abdominal U/S Evaluation Ev aluation for other anomalies
- 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.. 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 .
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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 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
Ab A bdomi min nal U/S
During the first 24 hrs. of life,
All these patients need abdominal ultrasound evaluation evaluat ion To identify an obstructive uropathy especially in patients with;
Rectovesical fistula
Rectoprostatic Recto prostatic urethral fistula
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 showingRectov showingRectovesical esical 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
High Anomaly
Separate urethra ,common rectum& vagina
High Anomaly
One each urethera ,vagina, Rectum.
Ectopic Anus
Inversion Radiology or needle aspiration of meconium and injection of contrast media High or Low
Wingspread classification (1984)
Rectovaginal fistula Intermediate
RectoVestibular fistula
High
Persistant cloaca
Rectourethral
fistula
Rectal atresia
Bulbar
Prostatic
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
mperforate anus without fistula
Imperforate anus without fistula
Rectal atresia
Rectal atresia
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Krickenbeck Krick enbeck classification (2005):
Major clinical group
Rare /regional variants
Perineal (cutaneous) fistula
Pouch colon
Rectourethral fistula
Rectal atresia/stenosis
Prostatic
Rectovaginal Rectov aginal fistula
Bulbar
H fistula
Rectovesical fistula
Others
Vestibular fistula Cloaca No fistula Anal stenosis
Treatment of imperforate Anus •
Preoperative Therapy
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NICU admission
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IV fluids …NPO & NG tube to exclude TOF.
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Antibiotics
Treatment of associated anomalies.
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Surgical treatment :
-Initial pelvic colostomy. -Pull through operation. -Closure of o f colostomy. -Continence work up.
- Cut back in case of membrane. - Perineal anoplasty. - Regular post operative anal 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
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Posterior sagittal anorectoplasty(PSARP) = THE BEST
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Anterior sagittal anorectoplasty(ASARP)
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Followed by post operative dilatation.
Algorism of Management
Postoperative Postoperati ve Management Management •
Rectouetheral fistula
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Nutrition??
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Dilatation ??
Postoperativ Post operative e functional 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
