Anorectal

Malformations

Moderator: Dr Ajay Kumar Presenter: Dr Bimlesh Thakur
(Director, Professor and Head)

Co- moderator : Dr Gyan Saurabh
Objectives
To review the anatomy of anal canal
To classify anorectal malformations
To discuss clinical presentation of various anorectal
malformations
To discuss management of various anorectal malformations


Anal Canal Anatomy
Sabiston Textbook of Surgery 19
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edition page 1382
Dentate line marks the junction of anoderm and anal
transition zone.

Anorectal ring is formed by fusion of the puborectalis, deep
external sphincter and the internal sphincter.
ANATOMY
Above dentate line Below dentate line
Arteries Superior rectal
artery
Inferior rectal artery
Veins Superior rectal vein Middle and inferior rectal vein into the
internal iliac vein and internal pudendal vein
respectively
Lymphatics Internal iliac nodes Superficial inguinal nodes
Nerves Inferior hypogastric
plexus (L1,2) and
pelvic splanchnic
nerves(S2,3,4
Inferior rectal branch of pudendal nerve.
Embryology
Embryology
Between 4-6 weeks, the cloaca becomes the common
depository for the developing urinary, genital and rectal
systems.
The cloaca is divided into an anterior urogenital sinus and a
posterior intestinal canal by the urorectal septum.
Two lateral folds of cloacal tissue join the urorectal septum
to complete the separation of the urinary and rectal tracts.

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Wingspread Classification of
Anorectal Anomalies (1984)
Level Male Female
High Anorectal agenesis Anorectal agenesis
With rectoprostatitis With rectovaginal fistula
Without fistula Without fistula
Rectal atresia Rectal atresia
Intermediate

Rectobulbar urethral
fistula
Rectovestibular fistula
Rectovaginal fistula
Anal agenesis without a
fistula
Anal agenesis without a fistula

Low Anocutaneous fistula Anovestibular fistula
Anal stenosis Anocutaneous fistula
Anal stenosis
Cloacal malformations
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Low lesion-bowel extends below puborectalis
Primary Perineal Operation
Intermediate or high lesion
Colostomy in neonatal period followed by
Sacroperineal or sacroperineoabdominal approach
Clinical assessment
Weight, gestational age.
Evaluate for other fatal major anomalies e.g. esophageal
atresia
Careful perineal inspection and probing for any visible orifice
Occasionally radiography
Male infants
(I)Perineal opening with meconium exudation
Ectopic anus- Anterior to normal stenotic site Transplant
anoplasty

Anocutaneous fistula
(i)Fistula along median raphe Transplant anoplasty
(ii)Anal dimple without meconium Rectal atresia or covered
anus Initial colostomy
Rob & Smiths Operative Surgery 4
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edition Pediatric Surgery
Page 349

Ectopic anus
http://www.ptolemy.ca/members/current/Newborn%20Anorectal%20Malformations/

Anocutaneous fistula
http://www.ptolemy.ca/members/current/Newborn%20Anorectal%20Malformations/

Covered anus
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Pediatric Surgery Page 349
Rectal atresia
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(II) No perineal opening, No meconium exudation

Urine contains meconium Recto-urethral/vesical fistula
Initial Colostomy
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edition Pediatric Surgery Page 349


Recto-urethral fistula

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(III) No perineal opening, No meconium exudation, No
meconium in urine Radiography when atleast 24 hour old.
Primary perineal repair for low lesions.
Primary colostomy f/b surgery at 8-9mo for high and
intermediate lesions
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edition Pediatric Surgery Page 349



Low- Rectal gas below ischial ossification line
Intermediate- Between ischial line and pubococcygeal line
High- Above pubococcygeal line
Invertogram
Lateral invertogram in a patient
with anorectal agenesis and a
rectourethral fistula
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Prone cross table lateral X ray

Prone cross table lateral X ray
Female infants
(I) 3 perineal orifices
(a) Ectopic anus-Anterior to normal stenotic site
Transplant anoplasty
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edition Pediatric Surgery Page 351



(b) Fistula- Gentle probing Fistula just deep to skin Covered
anus with Anocutaneous, anovulvar, anovestibular fistula
Transplant anoplasty

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edition Pediatric Surgery Page 351


(c) Fistula in vestibule, running upwards Anal agenesis with
rectovestibular fistula Initial colostomy
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(II) 2 orifices anal agenesis with rectovaginal fistula Initial
colostomy


(III) Single orifice CloacaInitial colostomy
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edition Pediatric Surgery Page 351

Covered anus with Anocutaneous
and anovestibular fistula
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Anal agenesis with rectovestibular
fistula
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Cloaca
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Associated Malformations
Cardiovascular anomalies
Gastrointestinal anomalies
Spinal, sacral, and vertebral
Genitourinary anomalies
Gynecologic anomalies


Sacral ratio=BC/AB
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Management
During the first 24 hours
Nil per oral
Intravenous fluids and antibiotics
Evaluation for associated defects that may be life-threatening.
NGT to exclude esophageal atresia
Echocardiogram to exclude cardiac malformations, esophageal
atresia.

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edition
Radiograph of the lumbar spine and the sacrum
Spinal ultrasonogram to evaluate for a tethered cord.
Ultrasonography of the abdomen will evaluate for renal
anomalies.
Urine analysis

Aschcrafts Pediatric Surgery 5
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edition
After 24 hours, Re evaluate
Perineal fistula:- Anoplasty, without a protective
colostomy, can be performed during the first 48 hours of
life.
if there is no meconium on the perineum, do Invertogram
or cross table lateral X ray
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edition Page 475
If air in the rectum is located below the coccyx : a posterior
sagittal operation with or without a protective colostomy
If the rectal gas does not extend beyond the coccyx, or the
patient has meconium in the urine, an abnormal sacrum, or a
flat bottom, a colostomy should be done.
A posterior sagittal anorectoplasty 8 to 9 months later,
provided the neonate is gaining weight appropriately.

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Algorithm for male newborns
with anorectal malformations
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Algorithm for female newborns
with anorectal malformations
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Advantages of single stage procedure:
(i) Potential for acquiring anal sensation
(ii) Less morbidity
Disadvantages of single stage procedure:
(i)More chances of aspiration due to prone position during
PSARP in an infant with obstruction.
(ii)Without a colostogram, failure to recognise urethral or
vesical fistula
Advantages of two stage procedure:
(i) Evaluation of malformations with colostogram
(ii) Avoidance of a major surgery during infancy
Disadvantages of two stage procedure:
(i) Complications of stoma
(ii) Lack of early development of anal sensations
TRANSPLANT ANOPLASTY
Correction of imperforate anus with rectofourchette fistula.
The anal site is selected with the aid of an electrical stimulator
Traction sutures are placed. The fistula is carefully dissected
free with tenotomy scissors .
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The traction sutures are used to guide the opening to a
transplanted anal location within the sphincter complex.
Interrupted 4-0 absorbable sutures are used .
The fistulous site is closed with interrupted 4-0 suture.
Perineal body is preserved.

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Pelvic Loop Colostomy
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Colostogram in a patient with an
ideally placed colostomy
Divided sigmoid colostomy preferred to transverse colostomy
Disadvantages of transverse colostomy :
(i) Fluid and electrolyte imbalances
(ii) Loose stools make skin care more difficult
(iii) Cleaning of defunct distal loop is difficult
(iv) Delineation of downstream fistula more difficult
(v) Prolapse, megarectosigmoid, left microcolon
Disadvantages of sigmoid colostomy :
(i) Repititive trauma to pelvic colostomy in neonates due to
hip flexion as a protective reflex
(ii) A too low colostomy in sigmoid, can limit the pull
through.

Posterior sagittal anorectoplasty.
In the prone position, an incision is made in the midline from
the lower sacrum to the selected anal site .
The levator and sphincter muscles are divided posteriorly in
the midline. The rectal pouch is identified .
The pouch is opened, and the rectourethral fistula is
identified within the rectal lumen .
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Submucosal resection frees the bowel from the fistula, which
is closed with interrupted sutures .
The bowel is tapered to a No. 12 Hegar size .
The muscle complex is reconstituted starting at the deepest
portion of the puborectalis muscle and the deep external
sphincter.
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Levators and superficial external sphincters are then
reapproximated with interrupted sutures .
The tapered anoplasty is sutured to the skin with interrupted
4-0 absorbable suture .

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NEONATAL PULL-THROUGH
PROCEDURES
1. Abdominoperineal pull through procedure
2. Sacroperineal or sacroabdominoperineal pull through
procedures (Stephens procedure)
3. Kiesewetter modification of Stephens technique
4. Pena procedure: Posterior saggital anorectoplasty
5. Laparoscopically assisted anorectal pull through (LAARP)
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Complications
1. Wound infections
2. Anal strictures
3. Constipation
4. Ureteric injury, neurogenic bladder

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Thank you!