ANORECTAL MALFORMATION

 Anorectal malformation
◦ Result of abnormal development of the
urorectal septum
◦ Incidence 1 in 5000 life birth
◦ Male to female ratio 60% : 40%
◦ Occurs in association with many syndromes
(Down’s syndrome, cat-eye syndrome, Kauf
man-McKusick syndrome)
◦ Heredity plays a minor or insignificant role in
aetiology (5% autosomal dominan)
 Embryology
◦ The exact mechanism of anorectal formation has
remain controversial and hypothetical
◦ Cloaca : cavity into wich open hindgut, tailgut,
allantois and the mesonephric duct.
◦ Septum in the middle of cloaca (urorectal septum,
Tourneux fold) grows downward and fusing with lateral
folds (Rathke’s plicae) divide cloaca into urogenital sinus
and rectum ( 4 – 7 weeks gestation)
◦ A wide variety of the defects are caused by defective
formation of the urorectal septum
Division of the cloaca
Division of the cloaca
Division of the cloaca
 Classification
( Wingspread, 1984 )
 Associated malformation
◦ Most babies with ARM have one or more abnormalities of
other system (50 – 60%)
◦ Cardiovascular anomalies
 12 – 22%
 The most common lesion: Tetralogy of Fallot and VSD
 High lesion : more likely cardiac anomaly
◦ Gastrointestinal anomalies
 Tracheoesophageal abnormality (10%)
 Duodenal obstruction ( atresia, malrotation) (1 – 2 %)
 Aganglionosis ( Hirschsprung ) (2,8%)
◦ Vertebral anomalies
 Lumbosacral anomalies: Spinal dysraphysm (17% low type,
34% high type, 46% cloaca)
◦ Genitourinary system anomalies
 9% in low type, 30% in high type
 The most common : vesicoureteric reflux (59% in low type),
renal agenesis, dysplasia, cryptorchidism, hypospadia: less
frequently
 Uterine and vaginal abnormality

◦ VACTERL (Vertebrae, Anorectal, Cardiac,

TracheoEsophageal fistula, Renal, Limb)
 Diagnosis
◦ High type, Low type anomaly
◦ Investigation
 Inspection of the perineum
 X-Ray (invertogram, lateral X-ray with buttocks rised)
 16 – 24 hours after birth
High type : - skin-bowel distance > 1 cm
- blind ending pouch above PC line
Low type : - skin-bowel distance < 1 cm
- blind ending pouch below PC line
 USG and CT-Scan
 Urinalysis ( male )
Meconeum stained urine
Covered anus
Anovestibular fistula
Perineal fistula
Persistent Cloaca
 High type (fistula):
Male
 Rectovesical fistula
 Rectourethral fistula
 Rectal atresia
(Invertogram):
 ARM without fistula >1cm
_ Above Pubococcygeal Line
 Low type (fistula) :
 Perineal fistula
(Invertogram):
 ARM without fistula < 1 cm
 Below Ischial Line
Female
- Rectovaginal fistula
- Rectovestibular fistula
- Rectal atresia
- Persistent cloaca
- ARM without fistula
>1 cm
- Above Pubococcygeal
Line
- Perineal fistula
- ARM without fistula<1 cm
- Below Ischial Line
Pubococcygeal line ( PC Line )
Pubococcygeal line ( PC Line )
High type anomaly
Low type anomaly
Diagnosis and Management ( Algorithm )
Diagnosis and Management ( Algorithm )
Anoplasty
Colostomy
Posterosagittal Anorectoplasty ( PSARP )
Post Posterosagital Anorectoplasty ( PSARP )