Anorectal malformations

Hartanto

Moderator:
Giovani Fatrio
Odo
 ● In 1835  1st actual anoplasty by Amussat :
suturing rectal wall to the skin edges
● In 1953  Stephens proposed an initial
History sacral approach followed by an
abdominoperineal operation, if necessary
● Posterior sagittal approach  first performed
in 1980
 1 : 4000-5000 newborns
Incidence rate

Male > female

Introduction Slightly more common

Approximately 1%
Risk of having a second child with anorectal malformationj
 Vertebral, Spinal, Sacral anomalies

Anorectal Malformation
Associated Congenital Cardiac defects
congenital Tracheo-Esophageal anomalies
anomaly
Renal anomalis

Limb defects
 ● The more complex the anorectal anomaly -> the more
likely is the presence of an associated spinal and
vertebral anomaly

Vertebral, ● Sacral defect

○Hemisacrum, in association with imperforate
sacral, and anus
○Presacral mass known as Currarino triad
spinal ○Sacral ratio is a valuable prognostic tool
associated Lumbosacral anomalies

anomalies • Hemivertebrae, scoliosis, butterfly vertebrae, and

hemisacrum

Spinal

• Tethered cord ( most frequent ), spinal lipomas,

syringomyelia, myelomeningocele
 Genitourinary Anomalies
• Urologic abnormalities predominate
• Incidence range from 1/3 to ½ of patients and
increases with increasing complexity of
anorectal defect
Associated • Vesicoureteric reflux is the most common
anomalies
Gastrointestinal Anomalies
• Tracheoesophageal abnormalities occur in
about 10% of cases
• Duodenal atresia or malrotation 1-2%
• Hirschprung disease
 Cardiovascular Anomalies
• Cardiovascular anomalies are present in approximately 1/3
patients but only 10% of these require treatment
• The most common lesions are atrial septal defect and
patent ductus arteriosus , TOF, VSD
Associated
anomalies Gynecologic Anomalies
• Hydrocolpos can lead to urinary
obstruction or can cause pylocolpos
• Mullerian anomalies
• Uterine malformations (predominantly
bicornuate uterus and uterus didelphys)
 Type of defects
Rectoperineal

Male Rectourethral

Rectobladder neck

Imperforate anus without fistula

Rectal atresia/ rectal stenosis

Rectoperineal

Female Rectovestibular
Cloaca
Complex malformations
Imperforate anus without fistula
Rectal atresia / rectal stenosis
 Male Anorectal Defects
Rectoperineal

01 Rectourethral

Rectobladder neck

Imperforate anus without fistula

Rectal atresia / rectal stenosis

 ● Also called as “anal membrane,
covered anus, bucket-handle
malformations”
● Usually the anal fistula opening
is stenosis
Rectoperineal
● Lowest defect
fistula ● Rectum is located within most
of the sphincter mechanism
● Lowest part of rectum is
anteriorly mislocated
 ● Most common defect in male
○ Lower part (bulbar)
○ Higher part (prostatic)
● Above the level of fistula, rectum and urethra
share a common wall
Rectourethral ● Lower urethral fistula  usually associated
with good-quality muscles, well developed
fistula sacrum, prominent midline groove, and
prominent anal dimple
● Higher urethral fistula  poor-quality
muscle, abnormally developed sacrum, flat
perineum, barely visible anal dimple
 ● Affects about 10% of male patients
● Poor prognosis for bowel control
Rectobladder ● Sacrum often deformed and short
● Entire pelvis seems to be underdeveloped
neck fistula ● Perineum is often flat
 ● Most patients have a well developed sacrum
Imperforate and good muscles
● Good prognosis of bowel function
Anus without ● Rectum usually terminates approximately
2cm above perineal skin
fistula ● About half of patients without fistula is
associated with Down syndrome
 ● Less than 1% of all anorectal malformations
● Lumen of rectum is atresia or stenosis
● Upper pouch : dilated rectum
Rectal atresia / ● Lower portion : small anal canal in normal
rectal stenosis location, 1-2cm in length
● The repair involves primary anastomosis
between upper and lower part
● Excellent functional prognosis
 Female Anorectal Defects
Rectoperineal

02 Rectovestibular

Cloaca

Imperforate anus without fistula

Rectal atresia / rectal stenosis

 ● Most common defect in females
● Excellent functional prognosis
Rectovestibular ● Diagnosis is based on clinical examination
● Normal urethra meatus and normal vagina +
fistula third hole in vestibule  rectovestibular
fistula
 ● Extreme complexity of malformations
● Distal portions of rectum, vagina, and
urinary tract fuse  creating a single
common channel
● Length of common channel varies; 1-
7cm
Persistent ○ <3cm : defect can be repaired with
cloaca posterior sagittal approach
○ > 3cm : abdominal operation is
usually needed
● Shoul be suspected in female newborn
with imperforate anus and small-looking
genitalia
● Single perineal orifice
Anorectal malformation
Diagnostic
 ● Absence of meconium
● No anal opening
● Unable to perform RT or insertion of a
rectal thermometer into the rectum
● Abdominal distention
Clinical ● Greenish bulging membrane behind anus
manifestation (in case of imperforate anal membrane)
● Intestinal obstruction (without fistula
type)
● Passage of meconium through vagina,
perineal orifice, or with urine in case of
fistula
 ● Physical examination
○ By passing the gloved little finger through the anus
and by observing the passage through which
meconium was passed
● Radiology evaluation
○ Evaluation do not show real anatomy before 24
Diagnostic hours because the rectum is collapsed by the muscle
tone of the sphincters that surround its lower part.
○ Cross table lateral X-Ray film with the baby in
prone position
○ Evaluate the gas in the rectum
■ Above the coccyx
■ Below the coccyx
Algorithm for the
treatment for
male patients
with anorectal
malformation
Algorithm for
the treatment
for female
patients with
anorectal
malformation
 Treatment during
first 24 hours
Intravenous fluids
Antibiotics
Nasogastric decompression to prevent aspiration
Evaluate associated defect
Echocardiogram
Plain radiograph of the lumbar spine and sacrum
Ultrasonography of abdomen
May be indicated to divert colonic contents temporarily
from a pathologic process in the distal part of colon or
rectum

Common indications for colostomy in anorectal

malformation :
• Flat perineum Colostomy
• Meconium in the urine
• Distal gas on the invertogram taken after 24 h
of live above the coccyx
• Cloaca
 Indications Anatomy
• Temporary • Ascending
• Permanent • Transverse
• Descending

Classification
Surgical technique

• Loop
• Double barrel
• Divided Double Barrel
Anatomy
 Indications

• After abdominoperineal resection of

the rectum and Hartmann’s procedure
• Need for complete temporary or
End permanent fecal diversion for
nonobstructive pathology
Colostomy
Contraindication

• Not acceptable solution for a distal

obstruction -> closed loop between the
staple line and the obstruction -> may
eventually result in perforation at the
staple line
 Generally preferable to use the
descending colon rather than sigmoid

End
Descending
Colostomy
 Indications

• Distal obstruction requiring temporary or

palliative fecal diversion
• Trauma to the extraperitoneal rectum or
perineum
• Complicated soft tissue infection of the
Loop perineum requiring significant debriement
Colostomy
Type

• Loop colostomy ( double barrelled

colostomy )
• Divided loop colostomy ( divided double-
barrel colostomy) -> more definitive fecal
diversion
Loop colostomy
( operation
technique )
Stomal
Complication