ANORECTAL MALFORMATIONS

Synonyms y imperforate anus y anorectal malformations y anorectal anomaly

Frequency
y Anorectal malformations occur in

approximately 1 per 4000 live births

TYPES
HIGH TYPE
y Males
Rectourethral fistula Rectovesical fistula Rectal atresia Persistent cloaca Rectovaginal fistula Perineal fistula Imperforate anus Without Fistula

LOW TYPE

y Females
Perineal fistula Vestibular fistula

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

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

MALE LOW TYPE

MALE HIGH TYPE

FEMALE ARM
LOW HIGH

A flat perineum

A flat perineum-GIRL

Perineal fistula

bucket-handle malformation

Associated malformations genitourinary

y Absent, dysplastic, or horseshoe

kidneys y Vesicoureteral reflux y Hydronephrosis y Hypospadias y Bifid scrotum

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

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

Gastrointestinal and Cardiovascular Systems

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

Two important questions must be answered ?????

y 1. Does the neonate suffer from a serious

associated defect that endangers life and requires urgent treatment? y 2. Can the neonate be treated with an anoplasty or pull-through without a colostomy or is a colostomy needed?

Surgical therapy
yColostomy yDefinitive repair

COLOSTOMY

Colostomy Newborn boys

y Rectobulbar urethral fistula y Rectoprostatic urethral fistula y Rectovesical fistula y Imperforate anus without fistula y Rectal atresia

Newborn girls Colostomy

y Rectovestibular fistula y Imperforate anus without fistula y Persistent cloaca y Rectal atresia y Rectovaginal fistula

The prone cross-table lateral radiograph vs Invertogram

y Better information compared to the

invertogram y Easy positioning, y Better cooperation of the patient, y Elimination of the effect of gravity y Better delineation of the rectal gas shadow

INVERTOGRAM

PCTLR

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

Definitive repair
y Posterior Sagittal Anorectoplasty (PSARP) y Laparotomy

PSARP

Long-term functional outcome

y Few or perhaps none of these children have completely

normal bowel habits after operation. y About half of the infants have acceptable to good results with few episodes of accidental soilage y The remaining children require major adjustments in lifestyle secondary to fecal incontinence, chronic constipation, and odor.