Download MBBS Pediatric Surgery Presentations 5 Anorectal Malformation Lecture Notes

--- Content provided by⁠ FirstRanker.com ---

MALFORMATION

(ARM)

CONTENTS

--- Content provided by⁠ FirstRanker.com ---

vIntroduction
vEmbryology
vGenetics and associations
vIncidence

--- Content provided by‍ FirstRanker.com ---

vClassification
vApproach to a patient

--- Content provided by⁠ FirstRanker.com ---

ANORECTAL MALFORMATION

Complex group of

--- Content provided by FirstRanker.com ---

congenital anomalies

involving the anus

--- Content provided by⁠ FirstRanker.com ---

and rectum, as well

as the urinary and

genital tracts in a

--- Content provided by​ FirstRanker.com ---

significant number of

cases.

--- Content provided by FirstRanker.com ---

EMBRYOLOGY

Abnormal development of the urorectal

--- Content provided by⁠ FirstRanker.com ---

septum in early fetal life.

At 3 weeks

EMBRYOLOGY

--- Content provided by‌ FirstRanker.com ---

At 5,7,8 weeks

--- Content provided by​ FirstRanker.com ---

GENETICS

ARM

--- Content provided by‌ FirstRanker.com ---

SYNDROMIC

NON-SYNDROMIC

SPORADIC

--- Content provided by​ FirstRanker.com ---

FAMILIAL


LADD AND GROSS CLASSIFICATION- 1934

--- Content provided by​ FirstRanker.com ---

WINGSPREAD

CONFERENCE

--- Content provided by‌ FirstRanker.com ---

CLASSIFICATION- 1984

--- Content provided by‌ FirstRanker.com ---

High (Supra-levator)- Male

--- Content provided by‍ FirstRanker.com ---

High (Supra-levator)- Female

--- Content provided by‍ FirstRanker.com ---

Intermediate- Male

Intermediate- Female

--- Content provided by FirstRanker.com ---

--- Content provided by‍ FirstRanker.com ---

Low (Trans-levator)- Male

--- Content provided by⁠ FirstRanker.com ---

Low (Trans-levator)- Female

--- Content provided by‌ FirstRanker.com ---

Miscellaneous- Female

Miscellaneous- Male

--- Content provided by​ FirstRanker.com ---

INCIDENCE & FREQUENCY

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

--- Content provided by⁠ FirstRanker.com ---

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

--- Content provided by‍ FirstRanker.com ---

RISK FACTORS

Mothers >35 years old
Maternal recreational drug use
Parental consanguinity

--- Content provided by‍ FirstRanker.com ---

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

--- Content provided by FirstRanker.com ---

ANATOMY

NORMAL -VS -ARM
APPROACH TO A PATIENT

--- Content provided by‌ FirstRanker.com ---

OF ARM

Prenatal Diagnosis- USG

--- Content provided by‍ FirstRanker.com ---

(Low sensitivity and specificity)

Dilated colon
Oligohydramnios and a highly distended vagina (in

--- Content provided by​ FirstRanker.com ---

females)

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

--- Content provided by‌ FirstRanker.com ---

Associated anomalies.


GOALS OF INITIAL ASSESSMENT

--- Content provided by FirstRanker.com ---

1. To determine the level of the malformation

in relation to the muscular sphincters and the

site of any fistulous communications.

--- Content provided by FirstRanker.com ---

2. To determine the integrity of sphincters and

their nerve supply.

--- Content provided by‍ FirstRanker.com ---

3. To document any associated anomalies that

may affect survival.

ALGORITHM FOR MALE ARM

--- Content provided by FirstRanker.com ---

I. LEVEL OF MALFORMATION

Neonatal examination

--- Content provided by‍ FirstRanker.com ---

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

--- Content provided by​ FirstRanker.com ---

fibers and gluteal development.

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

rectum and the presence of meconium passage

--- Content provided by FirstRanker.com ---

rules out atresia

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

--- Content provided by⁠ FirstRanker.com ---

mm


HIGH ARM-FLAT GLUTEUS

--- Content provided by‍ FirstRanker.com ---

EVIDENCE OF FISTULA

H/O meconuria
Meconium at the tip of

--- Content provided by​ FirstRanker.com ---

meatus

Meconium/squamous

epithelium in urine

--- Content provided by​ FirstRanker.com ---

microscopy


ANTERIOR ECTOPIC ANUS

--- Content provided by⁠ FirstRanker.com ---

Anal index of < 0.34 in girls

and < 0.46 in case of boys

--- Content provided by FirstRanker.com ---

Anal index-
- Ratio of scrotal-anal distance

to

--- Content provided by‍ FirstRanker.com ---

the

scrotal-coccygeal

distance in males and

--- Content provided by​ FirstRanker.com ---

- fourchette-anal distance to

fourchette-coccygeal distance

--- Content provided by‍ FirstRanker.com ---

in females.


LOW ARM WITH FISTULA IN SCROTUM,

--- Content provided by‌ FirstRanker.com ---

MECONIUM TRACT IN PERINEUM

PERINEAL FISTULA WITH PROBE IN SITU

--- Content provided by‍ FirstRanker.com ---

ALGORITHM FOR MALE ARM

AXR

--- Content provided by‍ FirstRanker.com ---

INVERTOGRAM

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

--- Content provided by‍ FirstRanker.com ---

Cross-table Prone lateral x-ray (CTPL)

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

--- Content provided by​ FirstRanker.com ---

LOW

HIGH

--- Content provided by⁠ FirstRanker.com ---

ALGORITHM FOR MALE ARM


COLOSTOMY

--- Content provided by​ FirstRanker.com ---

HIGH DIVIDED SIGMOID COLOSTOMY (HDSC)

Bowel decompression and protection for the final

--- Content provided by‍ FirstRanker.com ---

repair

Facilitates the distal colostogram
Relatively short segment of defunctionalized distal

--- Content provided by‌ FirstRanker.com ---

colon- less chances of microcolon

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

--- Content provided by‌ FirstRanker.com ---

Easy pull through


ANOPLASTY

--- Content provided by⁠ FirstRanker.com ---

Computed Tomography

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

--- Content provided by FirstRanker.com ---

musculature.

Difficulty in distinguishing meconium from the

rectal wall and adjacent musculature- and so

--- Content provided by‍ FirstRanker.com ---

the site of fistula.

Radiation cost to the patient.

--- Content provided by‌ FirstRanker.com ---

MRI

No radiation burden
Easy

--- Content provided by⁠ FirstRanker.com ---

differentiation of

meconium from the rectal

--- Content provided by⁠ FirstRanker.com ---

wall

and

levator

--- Content provided by‍ FirstRanker.com ---

musculature

Level of the terminal bowel,

--- Content provided by⁠ FirstRanker.com ---

and the state of the pelvic

floor musculature

Fistulae

--- Content provided by​ FirstRanker.com ---

(difficult

in

--- Content provided by FirstRanker.com ---

neonates)

II. Assessment of the Sphincter

complex and Nerve supply

--- Content provided by​ FirstRanker.com ---

Absent

S4 AND S5-

--- Content provided by FirstRanker.com ---

Normal variant

Absent S3,4,5 - Variable

incontinence

--- Content provided by⁠ FirstRanker.com ---

Absent S1 AND S2 -

always incontinent

--- Content provided by‌ FirstRanker.com ---

Hemisacral

defects-

Results unpredictable

--- Content provided by‍ FirstRanker.com ---

Meningomyeloceles.


? The sacral ratio is a valuable prognostic tool

--- Content provided by FirstRanker.com ---

? It quantifies degree of sacral agenesis

? Patient with ratio < 0.5 are universally incontinent

--- Content provided by‌ FirstRanker.com ---

? Ratio that approach 1 usually predict a good prognosis

KRICKENBECK SURGICAL PROCEDURES

--- Content provided by FirstRanker.com ---

Pressure-augmented colostogram

Posterior sagittal Anorectoplasty (PSARP)

--- Content provided by‌ FirstRanker.com ---

Jack knife position (Prone)

--- Content provided by‍ FirstRanker.com ---

PSARP

--- Content provided by‍ FirstRanker.com ---

Electrical stimulator- sphincter location.

Rectourethral fistulas

Rectourethrobulbar fistula Rectourethroprostatic fistula

--- Content provided by⁠ FirstRanker.com ---

-Most frequent defect in male patients

--- Content provided by‍ FirstRanker.com ---

-Rectum identified and

--- Content provided by⁠ FirstRanker.com ---

opened posteriorly in

between stay sutures

-Fistula visualized

--- Content provided by​ FirstRanker.com ---

-Rectum separated from

the

--- Content provided by​ FirstRanker.com ---

urethra,

and

mobilised.

--- Content provided by‌ FirstRanker.com ---

-Rectum passed in front

of levator and within

--- Content provided by​ FirstRanker.com ---

sphincter.

-Anoplasty done.

Recto-bladder neck fistula

--- Content provided by​ FirstRanker.com ---

Combined abdomino-perineal approach
Poor prognosis

--- Content provided by⁠ FirstRanker.com ---

ANTERIOR SAGITTAL ANORECTOPLASTY(ASARP)

ARM VARIANTS

--- Content provided by FirstRanker.com ---

I.PERSISTENT CLOACA

--- Content provided by‌ FirstRanker.com ---

Defect in which the

rectum, one or two

--- Content provided by⁠ FirstRanker.com ---

vaginas and the urinary

tract converge into one

common channel.

--- Content provided by‌ FirstRanker.com ---

Cloacal anomalies

Short common

--- Content provided by​ FirstRanker.com ---

Long common

Double vagina/uterus

channel

--- Content provided by​ FirstRanker.com ---

channel

Hydrocolpus

--- Content provided by‍ FirstRanker.com ---

Posterior

High rectal

cloaca

--- Content provided by⁠ FirstRanker.com ---

implantation into

vagina

--- Content provided by‌ FirstRanker.com ---

DEFINITIVE MANAGEMENT

Genitogram
Genitoscopy

--- Content provided by FirstRanker.com ---

3D MR Genitography

SURGERY

Posterior sagittal approach

--- Content provided by FirstRanker.com ---

Rectal mobilization (sub

serosally)

Total Uro Genital

--- Content provided by‌ FirstRanker.com ---

mobilization

Vulval & perineal

--- Content provided by​ FirstRanker.com ---

reconstruction

Abdomino- perineal

approach if long common

--- Content provided by‍ FirstRanker.com ---

channel.


VAGINAL SWITCH

--- Content provided by‍ FirstRanker.com ---

VAGINAL AUGUMENTATION

From rectum
From colon

--- Content provided by FirstRanker.com ---

From small intestine

2. CONGENITAL POUCH COLON

--- Content provided by‍ FirstRanker.com ---

2.5 ? 9 % of ARM in North

India.

--- Content provided by⁠ FirstRanker.com ---

All or part of the colon is

replaced

by a pouch-like dilatation,

--- Content provided by​ FirstRanker.com ---

which communicates

distally with the urogenital

--- Content provided by FirstRanker.com ---

tract via a large fistula.


MODIFIED WAKHLU'S CLASSIFICATION

--- Content provided by‍ FirstRanker.com ---

Depending on length of available colon

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

malformation. Indian J Surg 44:621?629.

--- Content provided by‍ FirstRanker.com ---

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

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

--- Content provided by‌ FirstRanker.com ---

MANAGEMENT

--- Content provided by⁠ FirstRanker.com ---

3. RECTAL ATRESIA

1-2% of ARM

Common in

--- Content provided by‍ FirstRanker.com ---

South India

End to end
anastomosis via

--- Content provided by⁠ FirstRanker.com ---

PSARP route.


Postoperative management

--- Content provided by​ FirstRanker.com ---

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

--- Content provided by‌ FirstRanker.com ---

COMPLICATIONS


EVALUATION OF OUTCOMES

--- Content provided by​ FirstRanker.com ---

FUNCTIONAL OUTCOME DURING CHILDHOOD- LOW ARM

--- Content provided by⁠ FirstRanker.com ---

Functional outcome during childhood ? high

malformations: PSARP

TREATMENT OF FECAL INCONTINENCE

--- Content provided by‌ FirstRanker.com ---

Proper bowel

management program

--- Content provided by‍ FirstRanker.com ---

Dietary modifications

Surgical procedures to

improve continence

--- Content provided by​ FirstRanker.com ---

OTHER OUTCOMES

Urinary function

Sexual function

--- Content provided by‍ FirstRanker.com ---

Long-term growth and development