Anorectal Disorders

Question 1

T/F: Congenital anorectal disorders are usually isolated findings.

Answer 1

False. Congenital anorectal disorders are often part of other disorders, including VACTERL.

Question 2

How does perineal fistula present in males?

Answer 2

A perineal fistula presents with a small orifice on the perineum located just anterior to the center of the external orifice, usually it is close to the scrotum. Anal dimples are prominent. Boys have a “bucket handle” malformation or “black ribbon” structure in their perineum that is a subepithelial fistula filled with meconium.

Question 3

How often are other congenital anomalies found in patients with perineal fistulae?

Answer 3

Less than 10% of those affected have other organ anomalies.

Question 4

What is the recommended management of perineal fistulae?

Answer 4

The defect can be surgically repaired without need for colostomy.

Question 5

How does perineal fistula present in females?

Answer 5

It presents with a small orifice on the perineum located close to the vulva, anterior to the center of the external orifice.

Question 6

What is the most common anorectal defect in males?

Answer 6

Rectourethral fistula

Question 7

What is a rectourethral fistula?

Answer 7

A rectourethral fistula occurs in males when the rectum communicates with the lower (bulbar) or upper (prostatic) part of the urethra.

Question 8

How does a rectourethral fistula typically appear on exam?

Answer 8

Most patients have a well-defined midline perineal groove and an anal dimple.

Question 9

What is the recommended management of rectourethral fistulae?

Answer 9

A protective colostomy is required during the newborn period, with complete surgical repair later in life.

Question 10

What is a rectovesical fistula?

Answer 10

A rectovesical fistula occurs when the rectum communicates with the neck of the bladder. The sacrum is frequently absent.

Question 11

How are rectovesical fistulae managed?

Answer 11

Bowel function is poor in these patients, so colostomy is required in the newborn period, followed by corrective surgery later in life.

Question 12

What is the most common anorectal defect in females?

Answer 12

Vestibular fistula

Question 13

What is a vestibular fistula?

Answer 13

The rectum opens into the vestibule of the female genitalia immediately outside the hymenal orifice. The sacrum and sphincter tone are normal and an anal dimple is present.

Question 14

What is the recommended management for vestibular fistulae?

Answer 14

A protective colostomy must be performed in the newborn period before definitive surgery can be done.

Question 15

What is a persistent cloaca?

Answer 15

Persistent cloaca means that the vagina, rectum, and urinary tract meet and fuse at a common channel. There is a single orifice just behind the clitoris.

Question 16

Differentiate between the presentation of females with persistent cloaca < 3 cm and those with persistent cloaca > 3 cm.

Answer 16

Those with short channels (< 3 cm) have a well-developed sacrum and good sphincter tone. Those with long channels (> 3 cm) have a more complex defect and typically have a poorly defined sacrum and sphincter tone.

Question 17

What (2) additional anatomic abnormalities are often present in patients with persistent cloaca?

Answer 17

Most affected females also have an abnormally large vagina filled with mucus (hydrocolpos), and 90% also have urologic abnormalities requiring emergent attention.

Question 18

What is the recommended management of peristent cloaca?

Answer 18

A colostomy must be performed in the newborn period followed by definitive reconstructive surgery.

Question 19

What is imperforate anus?

Answer 19

An imperforate anus occurs when the rectum is completely closed off and does not communicate with the anus or skin.

Question 20

Which chromosomal abnormality has an increased risk of imperforate anus?

Answer 20

Trisomy 21

Question 21

What is the recommended management of imperforate anus?

Answer 21

Initial colostomy in the newborn period with reparative surgery later in life.

Question 22

What is rectal atresia?

Answer 22

Patients have a normal anus and anal canal, with obstruction located approximately 2 cm above the skin level.

Question 23

What is the recommended management of rectal atresia?

Answer 23

Initial colostomy in the newborn period with reparative surgery later in life.

Question 24

What is rectal prolapse?

Answer 24

Rectal prolapse occurs when ≥1 layer of the rectum protrudes through the anus. It presents as a red-purple, circular protrusion from the anus.

Question 25

What are the 3 most common causes of rectal prolapse?

Answer 25

Constipation > infectious diarrhea > cystic fibrosis

Question 26

What is the most common cause of symptomatic hemorrhoids?

Answer 26

Constipation with chronic straining.

Question 27

What is the most common cause of rectal bleeding in children?

Answer 27

Anal fissures

Question 28

List seven potential etiologies of perianal itching.

Answer 28

Candida overgrowth after a course of antibiotics, atopic dermatitis, contact dermatitis, perianal strep infection, anal fissures, pinworm/tapeworm infection, and occasionally UTI in a younger child.

Question 29

How does perianal strep present?

Answer 29

It presents as an angry, bright red, confluent rash around the anal orifice that can spread throughout the entire perineal area. Rectal bleeding can occur, and history may reveal a recent strep exposure.

Question 30

What is the recommended treatment for perianal strep infection?

Answer 30

Treat with oral penicillin. Topical treatments are ineffective.