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Congenital Anorectal Anomalies: DR Jose Antonio Hernandez Liven
Congenital Anorectal Anomalies: DR Jose Antonio Hernandez Liven
ANOMALIES
Dr Jose Antonio Hernandez Liven
4/11/2016
OUTLINE
• EMBRYOLOGY
• EPIDEMIOLOGY
• TYPES
• CLINICAL FEATURES
• INVESTIGATIONS
• TREATMENT
• COMPLICATIONS
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Embryology
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Cont’d
• The rectal portion of the blind end of the cloaca is the anal
membrane separating the proximal portion of the anus from the
ectodermal depression, the proctoderm, which later becomes the
distal portion of the anus.
• The anal membrane lies at the level of the anal valves and breaks
down by the 8th week to establish continuity of the lumen.
• The anal tubercles around the proctodeum fuse with the lateral
genital folds
• The proctodeum in its development “migrates” posteriorly along
the perineum.
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EPIDEMIOLOGY
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CLASSIFICATION OF CONGENITAL ANOMALIES
OF THE ANORECTUM
Female Male
• High • High
Anorectal agenesis with or without Anorectal agenesis with or without
rectovaginal fistula
Rectal atresia rectoprostatic urethral fistula
Rectal atresia
• Intermediate
Anorectal agenesis with or without • Intermediate
rectovaginal fistula Anorectal agenesis with or without
Anal agenesis rectobulbar urethral fistula
• Low Anal agenesis
Anovestibular or anocutaneous
fistula (anteriorly displaced anus) • Low
Anal stenosis Anocutaneous fistula (anteriorly
Cloaca displaced anus)
——- Anal stenosis
10/10/2018
Types of Anomalies
• Low anomaly (Infra-levator): in this type the bowel passes through
the pelvic floor before it becomes abnormal and so has the
puborectalis sling and internal and external sphincters. Continence is
therefore achieved in at least 90%.
• High anomaly (Supra-levator): The bowel ends above the levator ani
which is contracted around the urethra in the male and vagina in the
female. The internal sphincter is not developed and the external
sphincter is hypoplastic. Continence is, therefore, achieved in small
proportion of patients after a long period of time.
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Low Anomalies-(60%)
Types
•Covered Anus
•Ectopic Anus
•Persistent Anal Membrane
•Anal Stenosis
•Anal Agenesis
CAAPE
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Covered Anus
• In this type, the anal canal and sphincters are normally developed,
but the anal orifice is covered by skin arising from the excessive
fusion of the anal tubercles and lateral genital folds.
• The covering skin may have a small opening either in the centre or
by the side through which meconium or flatus may pass.
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Ectopic Anus Persistent Anal Membrane
• The anus opens in an ectopic • Is caused by the complete or
site usually in the vulva, incomplete failure of the anal
vestibule, lower vagina or the membrane to break down and
perineum. It is caused by failure establish continuity between the
of the anal opening to rectum and the anal pit.
“migrate” posteriorly to its
proper position.
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Anal Stenosis ANAL AGENESIS
• It is narrowing of the anus and • It results from non-
is rare. development of the anal pit and
excessive fusion of the anal
tubercles.
• Symptoms will occur early only
if narrowing is severe.
6/2/2011
4/11/2016
Associated Anomalies
1. Gastrointestinal abnormalities
• Oesophageal atresia
• Intestinal atresia, malrotation of the gut.
2. Skeletal abnormalities
• Sacral agenesis occurs in about a third of the patients with high
anomaly.
• A sacrum of 3 segments or less may be associated with neurological
anomalies of the bladder, pelvic floor and sphincters.
3. Urinary Anomalies
• About 30% of all patients have urinary tract anomalies.
• Megaureter, hydronephrosis
• Malformation of the kidneys occurs in about a third of patients
with a high anomaly
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Cont’d
4. Genital Anomalies
Septate vagina or bicornuate uterus due to failure of fusion of the
Mullerian ducts may occur.
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HIGH ANOMALIES (SUPRA- LEVATOR)
• The bowel ends above the lavetor ani muscle which is contracted
around the urethra in the male and vagina in the female.
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HIGH ANOMALIES (SUPRA- LEVATOR)
They include:
• Anorectal agenesis
• Rectal atresia with normal anal canal
• Cloaca
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ANORECTAL AGENESIS RECTAL ATRESIA WITH NORMAL ANAL
CANAL
• The lower part of the rectum • In this type ,the anal canal is
and the anus are absent and the normally developed but the
gut may end in a dilated pouch. rectum ends blindly above the
• Failure of complete division of pelvic floor and is not connected
the cloaca into urogenital sinus to the urethra or vagina
and rectum may result in a
fistulous tract between the blind
pouch and the posterior urethra
in the male or posterior vaginal
fornix in female , so the
meconium and gas may appear
in the urethra in male or vagina.
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CLOACA
• This occurs only in females and here the bowels , urinary and genital
tracts all open into a common wide cavity.
• Commonly severe malformations of the areas are associated with
other developmental abnormalities.
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CLINICAL FEATURES
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Patient usually present in two groups:
1.Those without obstruction:
• They have an adequate external opening
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2. Those with obstructive symptoms:
i. No meconium is passed from birth, vomiting is late and
abdomen is distended. Such patients may have anorectal
agenesis without fistula, complete anal membrane or
rectal atresia.
ii. Small amounts of meconium are passed, but the baby is
distended and may vomit .The cause may be anorectal
agenesis with a narrow fistula, a covered anus or ectopic
anus with a narrow opening ,or anorectal stenosis.
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Investigations
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Investigations
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Treatment
• Covered anus: Skin folds such as median bands are excised. Where a
track exists it is laid open . Dilation of the anus is continued for 3
months.
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Treatment
High Anomalies
•Regardless of obstruction a sigmoid colostomy is done first.
•At 3-6 months / 9kg baby the definitive surgery is done (posterior sagittal
anorectoplasty)
•Fistulous tracts are divided
•NB: To achieve continence ,its important to place the neorectum within
the puborectalis sling without damaging the muscle fibers or the nerve
supply
•The poorly developed external sphincter is also preserved.
•The colostomy is closed later
•Daily dilation for 3 months
•The level of continence achieved is poor because of the lack of
sphincteric control. Other factors are poor rectal sensation and disorders
of bowel motility.
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Anorectal stenosis:
• Daily dilation is required
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Complications
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THANK YOU
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