Anorectal Malformations

History
1710, Littre's suggested performing an inguinal colostomy for imperforate anus

1783, Dubois acted and other surgeons followed suit, but almost all infants died, so colostomy remained unpopular and only a procedure of last resort
In 1787, Bell suggested using a midline perineal incision to find the bowel 1826 Dieffenbach described anal transposition 1835 Amussat did mobilization of the bowel through a perineal incision with suturing of it to the skin and this technique gained rapid acceptance. Strictures were less common than was observed with earlier procedures

History

1856 Chassaignac used a probe through a stoma to guide the perineal dissection
1872 Leisrink, McLeod (1880), and Hadra (1884) recommended opening the peritoneum if the bowel was not encountered from below 1930 Imaging to delineate the abnormality was first advocated by Wangensteen and Rice

1948-1949 Single-stage abdominoperineal procedures became widely used

1953 Stephens emphasized preservation of the puborectalis muscle (remember Cook from 1676?)

This surgery and its modifications were the standard approach until 1980

History In 1980 ( De Vries and Pena), the surgical approach to repairing anorectal malformations changed dramatically with the introduction of the posterior sagittal approach (PSARP)

Classification of Anorectal Malformation by Pea

Males Perineal fistula Rectourethral fistula Bulbar Prostatic Rectovesical fistula (bladder neck) Imperforate anus without fistula Rectal atresia Females Perineal fistula Vestibular fistula Persistent cloaca <3 cm common channel >3 cm common channel Imporforate anus without fistula Rectal atresia

No Colostomy

Colostomy

No Colostomy

Colostomy

 Bowel control is the main concern for the surgeon correcting these anomalies. Urinary control and sexual function must also be considered. Associated problems, such as a poorly developed sacrum, nerve supply, and spinal cord likely contribute to an inability to achieve continence

Incidence: 1 in 5000 live births.

Most common in females is a rectovestibular fistula 50% of all patients with anorectal malformations have an associated urogenital anomaly

The relationship of the distal rectum to the puborectalis muscle divides the imperforate anus malformations into high (supralevator) and low (infralevator) malformations

Early treatment for neonates born with an anorectal anomaly is crucial. During the first 24-48 hours of life, answer the following 2 questions: 1. Are any associated anomalies present that threaten the

baby's life and need to be addressed immediately?

2. Should the infant undergo a primary procedure with no protective colostomy or a protective colostomy with a definitive repair at a later date?

Trying to determine the location of the distal rectum before 16 hours of life is senseless because of the contracted state of the funnellike sphincter mechanism.

FEMALE

10

MALE

11

CLINICAL EXAMINATION

Perineal inspection may show a normal urethra, normal vagina, and another orifice that is the rectal fistula Meconium beneath the membranous covering typical of a low lesion A flat or rockerbottom perineum indicates poor sphincter or levator muscle development typical of a high anomaly Female malformations, 95% are of the low variety Most male anomalies are high

Newborn boy with imperforate anus.

Newborn girl with imperforate anus.

Imperforate anus and rectovestibular fistula in a newborn.

Newborn with imperforate anus and a rectoperineal fistula.

Newborn with imperforate anus and a bucket-handle malformation (usually associated with a rectoperineal fistula).

CLINICAL EXAMINATION (CLOACA)

The presence of a single perineal orifice in a patient is clinical evidence of persistent cloaca Patients with these anomalies also have small genitalia

examination of the abdomen may reveal an abdominal mass that likely represents a distended vagina (hydrocolpos), which is present in 50% of patients with persistent cloaca

CLOACA

IMAGING STUDIES

In the newborn period

Plain

radiography ( spina bifida, hemivertebrae, hemisacrum ) Crosstable lateral radiography Ultrasonography ( urologic anomalies, distended vagina, spinal anomalies)

Crosstable lateral radiograph of a patient in which the air column in the distal rectum can be observed close to the perineal skin.

AFTER THE NEWBORN PERIOD :

DISTAL COLOSTOGRAPHY
demonstrate

the rectourinary communication and determine the rectum's true height most cases of female anorectal malformations, except for persistent cloaca, distal colostography is not necessary because the fistula is evident clinically

In

DISTAL COLOSTOGRAM

Distal colostogram of a patient with imperforate anus and a rectourethral fistula.

24

ASSOCIATED ANOMALIES
VACTERL V vertebral A Anorectal C Cardiac T Tracheo-esophageal fistula E Esophageal, duodenal atresia R Renal L radial Limb

ASSOCIATED MALFORMATIONS GENITOURINARY

Absent,

dysplastic, or horseshoe

kidneys Vesicoureteral reflux Hydronephrosis Hypospadias

SKELETAL SYSTEM
Partial or complete lumbosacral agenesis Hemivertebrae Agenesis of thoracic vertebrae Scoliosis Hemisacrum or scimitar sacrum Asymmetric sacrum Agenesis of the coccyx

SPINAL ANOMALIES

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

SURGICAL MANAGEMENT
Initial : Colostomy or Anoplasty
Transversum

colon Descending colon ( Recommended ) Definitive : PSARP

COLOSTOMY

PSARP POSITION

Recommended

colostomy with divided stomas, the proximal stoma in the distal descending colon.