Anorectal Malformation – Pediatric Surgery

You are called to see a newborn male infant (2 hours old) with an anorectal malformation and there is no apparent opening on the perineum.

Wait 24 hours (as in all anorectal malformation patients) to perform a cross table radiograph to establish the position on the rectum.

Perform a colostomy within the first 24 hours

Wait for 24 hours and perform the cross table radiograph and then perform a colostomy in 72 hours

Perform an ileostomy within 24 hours.

Wait 5 days and then perform a colostomy

What is the incidence of ARM per live births – 1 in….?

5,000

10,000

1,000

3,000

20,000

According to which classification are anorectal malformations classified by the precise anatomical abnormality of the distal rectum and the location of the fistula in relationship to the perineum and urogenital system?

Into low, intermediate, or high, depending on the termination of the rectum relative to the puborectalis

Wingspread classification

Krickenbeck classification

Montreal classification

Pena classification

A patient with an anorectal malformation without a fistula defect (blind ending rectum) on high pressure distal colostogram, is most commonly associated with which syndrome?

Trisomy 21

Trisomy 18

Trisomy 13

Beckwith–Wiedemann syndrome

Currarino-Syndrome

A child with a rectal atresia and anal stenosis requires which workup

Trisomy 21

Trisomy 18

Frey syndrome

Beckwith–Wiedemann syndrome

Currarino-Syndrome

What is the most common gynecologic associated issue in a female with an anorectal malformation- rectovestibular fistula?

Vesico-ureteral-reflux (VUR)

Rectal atresia

Vaginal septum

Urethral atresia

Absent os sacrum

A female patient with a normal anus, urogenital sinus and hypertrophy of the clitoris requires evaluation for:

Congenital adrenal hyperplasia (CAH)

Congenital hyperinsulinism (HI)

Cloaca

ARM with recto-vestibular fistula

Klinefelter-Syndrome

In a male ARM patient, the “no fstula” defect is almost always located at the level of:

Bladderneck

Bulbar urethra

Prostatic urethra

Vagina

Symphysis

A previously repaired ARM patient is found to have mislocated anus with rectal prolapse. MRI of the pelvis reveals a mass behind the urethra. Reoperation is considered. The posterior urethral mass most likely represents:

Rectal prolapse

Rectocele

R.O.O.F. Remnant Of Original Fistula

Urethral diverticulum

A 13 years old previously repaired cloacal patient, presents with cyclical, monthly left lower quadrant abdominal pain. What is the most likely diagnosis?

Ureterocele

Rectocele

Ureteropelvic junction (UPJ) obstruction

Hematometrocolpos associated with an obstructed Mullerian structure

Cloacal extrophy