Download - imperforate Anus.pptx
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Imperforate Anus Supervisor :
Prof.,Dr., Yasser Saad El-Din
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Embryology
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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. )
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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)
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• proliferation of ectoderm closes the caudalmost region of the anal canal. During the ninth week, this region recanalizes.
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rectoanal atresias and fistulas :are due to ectopic positioning of the anal opening.
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imperforate anus:there is no anal opening. This defect occurs because of a lack of recanalization of the lower portion of the anal canal
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Causes, incidence, and risk factors
• 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.
It occurs in about 1 out of 5,000 infants.
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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
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Signs and tests
• A doctor can diagnose this condition during a physical exam. Imaging tests may be recommended.
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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
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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.
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Investigations
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1-Invertogram
2- Perineal U/S or MRI
3- X-ray
4- Colostogram
5-Abdominal U/S
Evaluation for other anomalies
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Invertogram
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- 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 .
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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.
Perineal U/S or MRI
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X-ray
Abdominal radiograph performed at day one of life, shows multiple air filled distended bowel loops suggestive of bowel obstruction.
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Colostogram Should be done under pressure to illustrate any fistula
Distal colostogram showing the colon ending in a long, narrow rectourethral fistula
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Abdominal U/S
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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
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Evaluation for other anomalies
1-Chest x-ray 2-Lumbosacral x-ray3-Abdominal pelvic ultrasonography4-Kidney Ureter Bladder KUB x-ray5-IVU6-Echocardiography and ECG7-Passage of nasogastric tube
Ivu showingRectovesical fistula
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EXAMINE THE PERINEUM FOR THE MECONIUM
Diagnostic approach to male anorectal anomaly
Meconium discharge
through an orifice on perineum
Low anomaly
No meconium Examine urine for meconium
or mucus
Meconium/mucus present
Fistula present
High Anomaly
No meconium Inversion radiology
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Careful examination of vulva and perinum
Diagnostic Approach to female anorectal Anomalies
Meconium present
Common to
urethera, vagina, rectum
High Anomaly
Separate urethra ,co
mmon rectum&
vagina
High Anomaly
One each urethera ,v
agina,Rectum.
Ectopic Anus
No meconium
Inversion Radiology or needle aspiration of
meconium and injection of contrast media
High or Low
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Classifications of anorectal malformation
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Wingspread classification (1984)
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High Intermediate
Rectovaginal fistula
RectoVestibular fistula Persistant cloaca
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Rectourethral fistula
Rectal atresia
Bulbar Prostatic
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Bucket handle fistula
rectovesical
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Vestibular fistula
Perineal fistula or an anterior ectopic anus
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Males females
Perineal(cutaneous)fistula Perineal(cutaneous)fistula
Rectourethral fistula
Bulbar
Prostatic
Vestibular fistula
Rectovesical fistula Persistant cloaca
Imperforate anus without fistula Imperforate anus without fistula
Rectal atresia Rectal atresia
Pena classification (Therapeutic classification) 1995
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Krickenbeck classification (2005):
Major clinical group Rare /regional variants
Perineal (cutaneous) fistulaRectourethral fistulaProstaticBulbarRectovesical fistulaVestibular fistulaCloacaNo fistulaAnal stenosis
Pouch colonRectal atresia/stenosisRectovaginal fistulaH fistulaOthers
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Treatment of imperforate Anus
• Preoperative Therapy• NICU admission
• IV fluids …NPO & NG tube to exclude TOF.• Antibiotics
• Treatment of associated anomalies.
Vertebral anomalies Cardiovascular a
nomalies
Tracheoesophageal fistula
Renal
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• Surgical treatment :
High and intermediate anomaly Low anomaly
-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
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Initial Pelvic Colostomy:
•Descending Colostomy- what’s colostomy ?- Advantages over other types - Errors of colostomy
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Algorism of Management
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Algorism of Management
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• Posterior sagittal anorectoplasty(PSARP) = THE BEST• Anterior sagittal anorectoplasty(ASARP) • Followed by post operative dilatation.
Pull through operation
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Algorism of Management
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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.
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Thank You