ANORECTAL MALFORMATIONS

Question 1

DEFINITION

Answer 1

Errors in the embryonic development of the anus, lower rectum and urogenital tract

ARM - is a term used to designate a series of congenital anomalies of the anus and rectum, characterized by the absence of an external anal opening.
The rectum is connected to the perineum or to the urogenital tract in 95% of the cases “

Question 2

Cloaca

Answer 2

cavity into which opens allantois, mesonephric duct and
hindgut
21 days’ gestation

Question 3

Urorectal septum

Answer 3

6 weeks
 urogenital cavity anteriorly
 an anorectal cavity posteriorly
7 weeks
 the urogenital and
 the anal opening

Question 4

INCIDENCE

Answer 4

1/5000

Most frequent:

• girls – rectovestibular fistula
• boys – rectobulbar fistula

No fistula – 5%
 Down Syndrome (95%)

Question 5

FIRST CLASSIFICATION

1835 – CLINICAL CLASSIFICATION

Answer 5

 ANAL STENOSIS
 ANAL MEMBRANE
 RECTAL MEMBRANE
 IMPERFORATE ANUS
 + RECTAL FISTULA

Question 6

LADD & GROSS CLASSIFICATION

Answer 6

 ANAL & ANORECTAL STENOSIS
 IMPERFORATE ANUS
 IMPEFORATE ANUS WITH FISTULA
 RECTAL ATRESIA

Question 7

GROSS CLASSIFICATION

Answer 7

simple differentiation based on the levator muscle

• supralevator – for those above the levator ani
• infralevator anomalies, for those below the levator ani.

Question 8

Boys

Answer 8

Most-frequent:
Recto-urethral/rectobulbar fistula

Most-simple:
Rectoperineal fistula

Most-complex:
Rectobladder fistula

Question 9

Girls

Answer 9

Most-frequent:
Recto-vestibular fistula

Most-simple
Rectoperineal fistula

Most-complex:
Cloaca

Question 10

ASOCIATED MALFORMATIONS

Answer 10

50-60 %
- Higher abnormalities are associated with more malformations
“50% - urologic abnormality,
30% - a vertebral one,
25% - a spinal cord anomaly,
10% - a cardiac condition that requires treatment, and 8% - esophageal atresia “

Question 11

VERTEBRAL ANOMALIES

Answer 11

30-50% pacients
Diverse forme de malformatii vertebra-spinale
- hemivertebre, scolioza, hemisacru
- lipoame, mielomeningocel
The most frequent spinal problem - tethered cord

Question 12

Sdr Curarino SOS

Answer 12

• Sacral defect
• Anorectal malformation
• Presacral mass
valuable prognostic tool - the degree of sacral hypodevelopment.
Sacral ratio

Question 13

GENITOURINARY ANOMALIES

GYNECOLOGIC ANOMALIES

Answer 13

30-50%
- Vesicoureteric reflux
hydronephrosis
- renal agenesis 
- dysplasia
Cryptorchidism
hypospadias
uterine anomalies
vaginal anomalies
hydrocolpos

Question 14

Revolution of the surgical treatment – 1980 – PSARP
 Correct identification of anatomy an local anomalies
The aim is treating the anomalies , while preserving

Answer 14

fecal continence
 urinary continence
 Sexual function close to normal

Question 15

P-SARP

Answer 15

“Posterior sagital anorectoplasty’’

Question 16

“What are the diagnostic and therapeutic priorities in the management of
a newborn with anorectal malformation?
(initial magement)

Answer 16

First 24 hours:
 Do not operate; rule out important associated malformations
 ECHO cardiogram, babygram (esophageal atresia? Duodenal atresia? Spinal abnormalities? Sacral anomalies?)
 Kidney ultrasound: Renal anomalies, absent kidney
 Pelvic ultrasound (females with cloaca): Rule out hydrocolpos

After 24 hours:
• “Make a decision to operate (colostomy or anoplasty). Depending on the
experience of the surgeon: Colostomy to be done in the majority of malformations. Anoplasty in cases of perineal fistulae, in some cases of vestibular fistula

Question 17

DESCENDING COLOSTOMY

Answer 17

• Initial management - most of the cases
• Descending colostomy
• Distal bowel irigations
Morbidity – it is vital to construct it meticulously

• Not indicated:
• Transvers Colostomy
• Sigmoidian colostomy
• Loop colostomy

Question 18

COLOSTOMY

AIM

Answer 18

 Resume bowel movement eficiently.
 Contrast imaging – distal colostogram
 Irigations of the distal colon
 Protects the anoplasty

Question 19

COLOSTOMY

ADVANTAGES

Answer 19

 Reduced segment of defunctionalized colon
 Does not limit the pull through
 Reduces risk of urinary contamination and UTI
 Reduces incidence of colic prolapse

Question 20

AFTER COLOSTOMY MANAGEMENT

Answer 20

6 months – PSARP
10% boys (rectobladder neck fistula) si 
40% girls (cloaca)
 abdominal approach
 Abdominal approach:
 classical surgery
 Laparoscopic surgery

Question 21

P-SARP

Answer 21

 The patient is placed in a prone position with the pelvis elevated.
 A midline incision is made, and the sphincteric mechanism is divided exactly in the midline,
with equal amounts of muscle on each side –
 the lower portion of the sacrum through the center of the anal dimple and sometimes
extends to the perineal body.
Identification of the bowel (rectum), which will be incised and exposure of the rectal lumen
Exposure of the recto urinary fistula, which will be dissected and excised
surgeon must be extremely careful while separating the rectum from the urinary tract/vaginal
Suturing the rectal defect, circumferential perirectal dissection to gain enough rectal length to
reach the perineum
Suture of the sphincter muscles
Circumferential anoplasty

Question 22

CLOACA

Answer 22

common channel < 3 CM
A) rectal removal
B) Urogenital mobilisation

Question 23

COMPLICATIONS

Answer 23

• Wound infection
• rectal stenosis (stenoza vaginala, stenoza ureterala)
• fistula rectovaginala/rectouretrala
• rectal prolaps (<5%)
• diverticul uretral posterior
• ITU cronic
• urogenic bladder

Question 24

most common complications

Answer 24

CONSTIPATION

SOILING – FECAL INCONTINENCE