ANORECTAL MALFORMATIONS

Synonyms
imperforate anus anorectal malformations anorectal anomaly

Frequency
Anorectal malformations occur in approximately 1 per 5000 live births

TYPES
HIGH TYPE LOW TYPE

Clinical Findings
findings are associated with a high malformation
A flat perineum, as evidenced by the lack of a midline gluteal fold absence of an anal dimple, indicates that the patient has poor muscles in the perineum.

Perineal signs found in patients with low malformations include

the presence of meconium at the perineum, a bucket-handle malformation anal membrane (through which meconium is visible).

RADIOGRAPHY-INVERTOGRAM RADIOGRAPHY1616-24 hours

A flat perineum

A flat perineum-GIRL perineum-

Perineal fistula

bucketbucket-handle malformation

Associated malformations genitourinary Absent, dysplastic, or horseshoe kidneys Vesicoureteral reflux Hydronephrosis Hypospadias Bifid scrotum

Skeletal System
Partial or complete lumbosacral agenesis Hemivertebrae Agenesis of thoracic vertebrae Scoliosis Hemisacrum or scimitar sacrum Asymmetric sacrum Posterior protruding sacrum Agenesis of the coccyx

spinal anomalies
Tethered cord Dural sac stenosis Narrow spinal canal Myelomeningocele, meningocele Intraspinal teratoma Neurogenic bladder

Gastrointestinal and Cardiovascular Systems

The following associated anomalies can occur separately or as VATER and VACTERL associations:
Esophageal atresia Duodenal atresia Ventricular or atrioseptal defects Tetrology of Fallot Hirschsprung's disease

Calculation of the sacral ratio

Surgical therapy
Colostomy Definitive repair

Colostomy Newborn boys

Rectobulbar urethral fistula rectoprostatic urethral fistula rectovesical fistula imperforate anus without fistula rectal atresia

Newborn girls Colostomy Rectovestibular fistula imperforate anus without fistula persistent cloaca rectal atresia rectovaginal fistula

COLOSTOMY

Definitive repair
Anoplasty :
Rectoperineal fistula - girls Rectoperineal fistula boys Covered anus Bucket-handle malformation

Definitive repair
posterior sagittal ARP LAPARATOMY

PSARP

LongLong-term functional outcome

Few or perhaps none of these children have completely normal bowel habits after operation. About half of the infants have acceptable to good results with few episodes of accidental soilage The remaining children require major adjustments in lifestyle secondary to fecal incontinence, chronic constipation, and odor.