Download MBBS Pediatric Surgery Presentations 5 Anorectal Malformation Lecture Notes

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MALFORMATION

(ARM)

CONTENTS

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vIntroduction
vEmbryology
vGenetics and associations
vIncidence

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vClassification
vApproach to a patient

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ANORECTAL MALFORMATION

Complex group of

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congenital anomalies

involving the anus

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and rectum, as well

as the urinary and

genital tracts in a

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significant number of

cases.

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EMBRYOLOGY

Abnormal development of the urorectal

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septum in early fetal life.

At 3 weeks

EMBRYOLOGY

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At 5,7,8 weeks

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GENETICS

ARM

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SYNDROMIC

NON-SYNDROMIC

SPORADIC

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FAMILIAL


LADD AND GROSS CLASSIFICATION- 1934

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WINGSPREAD

CONFERENCE

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CLASSIFICATION- 1984

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High (Supra-levator)- Male

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High (Supra-levator)- Female

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Intermediate- Male

Intermediate- Female

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Low (Trans-levator)- Male

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Low (Trans-levator)- Female

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Miscellaneous- Female

Miscellaneous- Male

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INCIDENCE & FREQUENCY

2-2.5 per 10,000 live births
M:F ratio= 1.5:1
Supra-levator lesions more in males

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Associated anomalies more in males
Commonest-
- recto-urethral fistula
- ano-vestibular fistula (female)

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RISK FACTORS

Mothers >35 years old
Maternal recreational drug use
Parental consanguinity

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Maternal residence at high altitude
Paternal occupation in vehicle manufacture
Consumption of raw vegetables (pesticides)
Geographical (pouch colon)

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ANATOMY

NORMAL -VS -ARM
APPROACH TO A PATIENT

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OF ARM

Prenatal Diagnosis- USG

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(Low sensitivity and specificity)

Dilated colon
Oligohydramnios and a highly distended vagina (in

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females)

Absence of circular rim of hypoechogenicity (Anus).
Enterolithiasis
Polyhydramnios if associated with UGI obstruction.

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Associated anomalies.


GOALS OF INITIAL ASSESSMENT

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1. To determine the level of the malformation

in relation to the muscular sphincters and the

site of any fistulous communications.

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2. To determine the integrity of sphincters and

their nerve supply.

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3. To document any associated anomalies that

may affect survival.

ALGORITHM FOR MALE ARM

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I. LEVEL OF MALFORMATION

Neonatal examination

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1. Good perineal examination under a good light
2. Position of the anus or its absence; fistula.
3. Perineal shape (midline raphe)- parasagittal

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fibers and gluteal development.

4. Passage of a soft catheter greater >2 cm into the

rectum and the presence of meconium passage

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rules out atresia

vNormal size of the anus- 1.3 + (3 ? birth wt in kg) in

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mm


HIGH ARM-FLAT GLUTEUS

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EVIDENCE OF FISTULA

H/O meconuria
Meconium at the tip of

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meatus

Meconium/squamous

epithelium in urine

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microscopy


ANTERIOR ECTOPIC ANUS

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Anal index of < 0.34 in girls

and < 0.46 in case of boys

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Anal index-
- Ratio of scrotal-anal distance

to

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the

scrotal-coccygeal

distance in males and

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- fourchette-anal distance to

fourchette-coccygeal distance

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in females.


LOW ARM WITH FISTULA IN SCROTUM,

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MECONIUM TRACT IN PERINEUM

PERINEAL FISTULA WITH PROBE IN SITU

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ALGORITHM FOR MALE ARM

AXR

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INVERTOGRAM

* Narsimharao KL et al. Am J Roentgenol 1983; 140: 227-229

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Cross-table Prone lateral x-ray (CTPL)

12?24 hrs after birth
Prone position, hips- slightly flexed-3 mins
Centering on the greater trochanter

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LOW

HIGH

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ALGORITHM FOR MALE ARM


COLOSTOMY

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HIGH DIVIDED SIGMOID COLOSTOMY (HDSC)

Bowel decompression and protection for the final

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repair

Facilitates the distal colostogram
Relatively short segment of defunctionalized distal

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colon- less chances of microcolon

Mechanical cleansing
Large rectourethral fistula- Less metabolic acidosis
Doesn't permit stool distally

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Easy pull through


ANOPLASTY

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Computed Tomography

Demonstrate the level of the terminal colon
Increased detail - osseous structures

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musculature.

Difficulty in distinguishing meconium from the

rectal wall and adjacent musculature- and so

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the site of fistula.

Radiation cost to the patient.

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MRI

No radiation burden
Easy

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differentiation of

meconium from the rectal

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wall

and

levator

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musculature

Level of the terminal bowel,

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and the state of the pelvic

floor musculature

Fistulae

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(difficult

in

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neonates)

II. Assessment of the Sphincter

complex and Nerve supply

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Absent

S4 AND S5-

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Normal variant

Absent S3,4,5 - Variable

incontinence

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Absent S1 AND S2 -

always incontinent

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Hemisacral

defects-

Results unpredictable

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Meningomyeloceles.


? The sacral ratio is a valuable prognostic tool

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? It quantifies degree of sacral agenesis

? Patient with ratio < 0.5 are universally incontinent

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? Ratio that approach 1 usually predict a good prognosis

KRICKENBECK SURGICAL PROCEDURES

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Pressure-augmented colostogram

Posterior sagittal Anorectoplasty (PSARP)

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Jack knife position (Prone)

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PSARP

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Electrical stimulator- sphincter location.

Rectourethral fistulas

Rectourethrobulbar fistula Rectourethroprostatic fistula

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-Most frequent defect in male patients

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-Rectum identified and

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opened posteriorly in

between stay sutures

-Fistula visualized

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-Rectum separated from

the

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urethra,

and

mobilised.

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-Rectum passed in front

of levator and within

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sphincter.

-Anoplasty done.

Recto-bladder neck fistula

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Combined abdomino-perineal approach
Poor prognosis

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ANTERIOR SAGITTAL ANORECTOPLASTY(ASARP)

ARM VARIANTS

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I.PERSISTENT CLOACA

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Defect in which the

rectum, one or two

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vaginas and the urinary

tract converge into one

common channel.

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Cloacal anomalies

Short common

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Long common

Double vagina/uterus

channel

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channel

Hydrocolpus

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Posterior

High rectal

cloaca

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implantation into

vagina

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DEFINITIVE MANAGEMENT

Genitogram
Genitoscopy

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3D MR Genitography

SURGERY

Posterior sagittal approach

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Rectal mobilization (sub

serosally)

Total Uro Genital

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mobilization

Vulval & perineal

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reconstruction

Abdomino- perineal

approach if long common

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channel.


VAGINAL SWITCH

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VAGINAL AUGUMENTATION

From rectum
From colon

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From small intestine

2. CONGENITAL POUCH COLON

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2.5 ? 9 % of ARM in North

India.

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All or part of the colon is

replaced

by a pouch-like dilatation,

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which communicates

distally with the urogenital

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tract via a large fistula.


MODIFIED WAKHLU'S CLASSIFICATION

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Depending on length of available colon

? Wakhlu AK, Tandon RK, Kalra R (1982) Short colon with anorectal

malformation. Indian J Surg 44:621?629.

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? Narsimha Rao KL, Yadav K, Mitra SK, Pathak IG (1984) . Congenital short colon with

imperforate anus (CPC syndrome). Ann Pediatr Surg 1:159

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MANAGEMENT

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3. RECTAL ATRESIA

1-2% of ARM

Common in

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South India

End to end
anastomosis via

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PSARP route.


Postoperative management

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Foleys catheter
IV antibiotics and wound care
Anal dilatation (after 2 weeks) till desired size
Colostomy closure

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COMPLICATIONS


EVALUATION OF OUTCOMES

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FUNCTIONAL OUTCOME DURING CHILDHOOD- LOW ARM

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Functional outcome during childhood ? high

malformations: PSARP

TREATMENT OF FECAL INCONTINENCE

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Proper bowel

management program

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Dietary modifications

Surgical procedures to

improve continence

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OTHER OUTCOMES

Urinary function

Sexual function

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Long-term growth and development