Learning Objective

(Jessica Philbertha / 405100065)

1. Anatomy and histology of lower GI

2. Physiology and biochemistry of
defecation
3. Lower GI disorder
(child :
- organic/structural constipation :
hirschprungs disease, obstructive
ileus (fecolith), hypotyroid ;
- functional constipation ;
- anorectal anomaly : atresia ani)

Colon

Tunika mukosa

m Tun
u
- l - S sku ika
on irk la
gi ul ris
tu ar
di i s
na
lis

Tunika
submukosa

Lymphonodulus
solitarius
Taenia Coli

Appendiks

Epitel mukosa
(rusak)

Tunica
submucosa

Lymphonodulus
aggregatti

Kelenjar
Lieberkhun
ka
Tuni aris
cul
mus

Rectum - Anus

Kelenjar
sebasea
Folikel rambut

Kelenjar
circumanalis

Kriptus
lieberkuhn

Epitel selapis torak bersel goblet epitel

berlapis

 Combination of both voluntary and involuntary processes with force.

Unneeded material ampulla recti rectal walls expand stretch
receptors trigger the contraction of rectal muscles, relaxation of
the internal anal sphincter and an initial contraction of the skeletal
muscle of the external sphincter
Relaxation of the internal anal sphincter signal to brain indicating
an urge to defecate
Urge is not acted material in the rectum is returned to the colon by
reverse peristalsis more water is absorbed fecal matter may
harden constipation
Voluntary signal to defecate brain abdominal muscles contract
increase of intra abdominal pressure perineal wall is lowered
ano rectal angle to decrease from 90 degrees to less than 15 degrees
(almost straight) and the external anal sphincter relaxes rectum
now contracts and shortens in peristaltic waves forcing fecal
material out of the rectum and out through the anal canal
The internal and external anal sphincters along with the puborectalis
muscle allow the feces to be passed by pulling the anus up over the
exiting feces in shortening and contracting actions.

CONSTIPATION
Constipation is decreased frequency of
bowel movements usually associated with
a hard stool consistency passed with
difficulty every 3rd day.
Functional constipation implies that there
is

no

identifiable

causative

organic

condition

Functional constipation typically starts

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PHYSIOLOGY OF CONSTIPATION

Defecation Reflexes.

when the rectum is loaded due to the entry of feces by mass

movement, sensory nerve endings are stimulated . The impulse from
the nerve endings are transmitted via afferent fibres of pelvic nerve
to the defecation center situated in sacral segments of spinal cord.
The center, in turn sends motor impulses to the descending colon,
sigmoid colon, and rectum via efferent nerve fibres of pelvic pain.
The motor impulses cause strong contraction of descending colon,
sigmoid colon and rectum and relaxation of internal sphincter.
Simultaneously, voluntary relaxation of external sphintcers occurs. It is
due to the inhibition of pudendal nerve by impulses arising from
cerebral cortex.

Failure to voiding of feces is called

constipation

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Causes
Non organic
Bad dietary) habits
(Functional
lack of fibre in diet.
Improper toilet
training
Psychogenic ( poor
child relationship,
maladjustment in
children)
Fear of defecation
Stool withholding
habits and fecal
soiling.
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organic
Intestinal anatomic
Hirschsprungs disease.
Ultra short segment HD
Anal / rectal stenosis
Anteriorly displaced anal
opening
Stricture
Metabolic / endocrine
Cystic fibrosis
hypokalemia
Hyper calcemia
Diabetes mellitus
Hypothyroidism
Panhypopituitarism
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Causes organic
Intestinal disorder
Celiac disease
Cows milk protein
intolerance
Cystic fibrosis
Inflammatory bowel
disease
Tumor
Connective tissue
disorders
SLE
scleroderma
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Neuro muscular
Cerebal palsy
Psychomotor
retardation
Spinal cord lesion
Myotonic dystrophy
Psychiatric disorder
Anorexia nervosa
Drugs
Anti depressants
Lead
Viatmin D intoxication
15

Assessing constipation
Red flag symptoms include:
> 48 hours before passing meconium as a neonate
Abdominal distension esp if failing to thrive
Infrequent small or ribbon stools
Constant leaking especially if linked with urinary
leaking too
Failed management with appropriate standard
intervention (with compliance)

Hirschprungs Disease

Definition
A disorder of the gut caused by the failure of the
neural crest cells to migrate completely during
fetal development of the intestine, eventually
forming Auerbach's plexus.
The affected segment of the colon fails to relax,
causing an obstruction.
Disorder affects the short segment of the distal
colon. (5 percent of cases the entire colon is
affected).
Also called congenital aganglionic
megacolon.

Etiology
Defect in the craniocaudal migration
of neuroblasts originating from the
neural crest during the first 12 weeks
of gestation.
Defects in the differentiation of
neuroblasts into ganglion cells and
accelerated ganglion cell destruction
within the intestine may also
contribute to the disorder.

Pathophysiology
Absence of the ganglionic cell no
peristalsis distention of the
abdomen, constipation,
malabsorption, and dehydration.

Epidemiology
Appears on 18.6 per 100,000 live
births.
Male : female = 4.32:1
White > non-white
Nine percent of the Hirschsprung
cases were also diagnosed as having
Down's Syndrome.

Clinical features

Delayed passage of meconium

Abdominal distension
Constipation
Chronic Enterocolitis
The enlarged section of the bowel is
found proximally, while the
narrowed, aganglionic section is
found distally.

Diagnosis

Suspected in baby who:

Has not passed meconium within 48 hours of delivery
Green or brown vomit
Explosive stools after a doctor inserts a finger into the
rectum
Swelling of the abdomen, lots of gas and bloody diarrhea

Definitive diagnosis :
Suction rectal biopsy (gold standard)
Anorectal manometry
Barium enema
Radiologic findings may also assist with diagnosis.

Variable

Onset of constipation

Functional
(Acquired)
after 2 yrs of age

Hirschsprung
Disease
At birth

Forced bowel training

Usual

None

Abdominal distension

Uncommon

Common

Poor weight gain

Rare

Uncommon

Anal tone

Normal

Normal

Rectal examination

Stool in ampulla

Ampulla is empty

Malnutrition

None

Possible

Anorectal manometry

Distension of the
rectum causes
relaxation of the
internal sphincter

No sphincter
relaxation or
paradoxic increase
of pressure

Rectal biopsy

Normal

No ganglion cells

Barium enema

Massive amounts of
stool, no transition
zone

Transition zone,
delayed evacuation

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Treatment
1. Surgical removal (resection) & reanastomosis.
. Colostomy

The large intestine is cut and an opening is made through the

abdomen. This allows bowel contents to be discharged into a bag.
Later, when the childs weight, age, and condition is right, a pullthrough procedure is performed.

. Swenson, Soave, Duhamel, and Boley procedures

Swenson : leaves a small portion of the diseased bowel.

Soave-Boley : leaves the outer wall of the colon unaltered.
Duhamel : uses a surgical stapler to connect the good and bad bowel.

2. Symptomatic : laxatives
3. Nutrition : high fiber diet

Obstructive Ileus (Fecolith)

Definition
an obstruction proximal to the
appendicitis that causes an increase
in pressure and mucosal injury which
predisposes to bacterial invasionusually e. coli and b. fragilis

Hypothyroidism

Hypothyroidism
Endocrine disorder error in the
thyroid hormone synthesis.
The thyroid is a gland that is located
in the neck. Its job is to keep putting
out hormones that relate to
metabolism and many of the day to
day organ functions.

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Clinical Manifestation

Tired
Depressed
Constipation
Seeing weight gain

Pathophysiology
The slower the digestive process
goes the more chance there is that
the items that are in the digestive
tract will start to dry out and become
harder to move through. Eventually
the waste may stop moving.

Effect
Just like food that is left in the garbage can
will begin to rot after a few days, so will the
food that is in your body. That is why the body
tries to process what it needs and get rid of
the waste quickly. If it can't, and you suffer
from constipation, that rotting will begin
happening in your system instead. That
means toxins given off during the rotting
process are also stuck inside your body and
can seep through the walls of your digestive
tract and into your blood stream.

Functional Constipation

Definition
chronic idiopathic constipation
(CIC)
constipation that does not have a
physical (anatomical) or physiological
(hormonal or other body chemistry)
cause.

Causes
Improper toilet training
Psychogenic cause
1. poor parent child
relationship
2. maladjustment in school
3. Fear / anxiety
4. Precipitating family stress
Stool with holding habits and feacel
soiling.
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Rome III Criteria

C3. Functional Constipation
Diagnostic criteria*
1. Must include two or more of the following:
a. Straining during at least 25% of defecations
b. Lumpy or hard stools in at least 25% of defecations
c. Sensation of incomplete evacuation for at least 25% of
defecations
d. Sensation of anorectal obstruction/blockage for at least 25% of
defecations
e. Manual maneuvers to facilitate at least 25% of defecations (e.g.,
digital evacuation, support of the pelvic floor)
f. Fewer than three defecations per week
2. Loose stools are rarely present without the use of laxatives
3. Insufficient criteria for irritable bowel syndrome
* Criteria fulfilled for the last 3 months with symptom onset at least 6
months prior to diagnosis

Treatment

Lactulose
Milk of magnesia (Magnesium hydroxide)
Polyethylene glycol 3350
Polyethylene glycol-electrolyte solution (lavage)
Mineral oil
Senna
Bisacodyl
Docusate sodium
Glycerin suppositories
Phosphate enemas

Education
Fecal disimpaction
Behavioural modification
Dietary modification

Anorectal Malformation

Imperforate anus:
Includes agenesis and atresia of the
rectum and anus
Etiology: unknown
Incidence: 1 in 4,500 SEX: 60% male

Imperforate anus:
Low
Termination of bowel
below the pelvic floor
Easy to Dx
Simple to ttt
Outlook is good
1)Covered anus
2)Ectopic anus
3)Stenosed anus
4)Membranous stenosis
Imperforate anus

High
Termination of bowel
above the pelvic floor
Often have a fistula
into the urinary tract
with deficient pelvic
floor
Difficult to ttt
1)Anorectal agenesis
2)Rectal atresia
3)cloaca

Low abnormalities:
1)Covered anus:
Anal canal covered by a bar of skin with a track running forwards
to the perineal raphe.
This track is called Bucket Handle midline Raphe fistula.

2)Ectopic anus:
Anus situated anteriorly in , it opens in perineum in , it opens
in vulva (more commonly)>> vulval ectopic anus or vagina (rare)

3)Stenosed anus:
i.e. microscopic anus
There is a minute opening that can be seen if examined carefuly

4)Membranous stenosis:
Rare Anus is normally sited covered with a thin membrane that
bulges with retained meconium

High abnormalities:

Could be associated with: a fistulas connection between the blind rectal

stump and the bladder or other pelvic structures abnormalities

1)Anorectal agenesis:
Rare Blind rectal pouch lies just above the pelvic floor
In anterior aspect attached to the bladder & often there is a
rectovesical fistula or rectourethral fistula (manifested by passage of gas
or meconium in the urine) or perineal fistula
In fistula to the posterior fornix >> rectovestibular (or rectovaginal) low
RVF easy to correct postop.
Function is good high RVF difficult to correct postop function is poor

2)Rectal atresia:
Rare Anal canal is normal but ends blindly at the level of pelvic floor &
the rectum also ends blindly above the pelvic floor without a fistulas
opening.

3)Cloaca:
Occurs only in Bowel , urinary & genital tracts all open into a common
wide cavity Commonly associated with other developmental
abnormalities e.g. tracheoesophagial fistula

Approach:
Approach History >>> the systems
involved
Examination >>> DDx
Investigation >>> Dx
Management

History:
Failure to pass meconium within the 1
st 24 hours of life.
Patients with anterior ectopic anus
may present in childhood or in
adulthood. These patients have
lifelong histories of constipation and
painful defecation and usually have
required laxatives or enemas for
management .

Examination
Low
Bucket handle defect
Gas/meconium from
perineum
Perineal fistula if skin bridge
between vestibule and fistula.
Anterior ectopic anus i.e.
normal sized anus surrounded
by normal muscle.
Urine free of meconium (NB
average of 20 hrs for
meconium to appear in urine)

High

Flat bottom
Short sacrum
Little muscle contraction
Meconium per urethra
Rectovestibular fistula if within
mucosal margin of the vestibule.
Sphincter located close to the
scrotum or bifid scrotum often
associated with a Prostatic Fistula.
Cloacae are often missed if
urethral opening is not identified
in female with a recto-vaginal
fistula.
Furthermore, cloacae often have
small openings and may be a
cause of referral for ambiguous
genitalia.

Investigation:
Very Important to:
1. Exclude other anomalies!!
2.Determine whether abnormality is
high or low!!

1. Exclude other anomalies!!:

Anal atresia may occur as a part of the VACTERL
group of anomalies
V V ertebral body segmentation defect
A A nal atresia
C C ardiovascular (PDA, VSD)
TE Tracheo Esophagial fistula
R unilateral R enal agenesis
L Limb anomaly (radial ray hypoplasia)

So, very careful examination of the baby must be

made to exclude these anomalies

2.Determine whether
abnormality is high or low!!:
1)Invertogram:
2)Urine culture: Presence of meconium
Presence of proteus or pseudomonas
usually signifies that a fistula is
present
3)US: To evaluate the lesion type
4)MRI: If we suspected complex
malformation
5)Micturating cystourethrogram: By
injecting a dye in the urethra

Treatment of Low
abnormalities: :
1)Covered anus:
The track should be opened by
scissors
Followed by routine dilatation of the
anus
2)Ectopic anus: Plastic cut-back
operation
3)Stenosed anus: Regular dilatation

Treatment of High abnormalities: :

Very difficult problem & each case must be considered on its
merits

The possibilities are:

Two-stage operation:
1 st stage : laparotomy, division of rectourethral fistula,
transverse colostomy
2 nd stage : rectal pull-through operation

One-stage operation: laparotomy, division of fistula, pullthrough operation

Division of fistula & rectal pull-down operation through the

perinum ( know rarely used)

The new technique is post-sagittal rectoplasty ( PSARP )

Colostomy only ( for cloaca)

In a Summary:
1 opening >>> Cloaca
2 openings >>> anorectal agenesis with
rectovaginal fistula
3 openings >>> ectopic anus, stenosed anus,
membranous anus, rectal atresia or even
normal anus!!!
The most important investigation is the
invertogram
It is very important to rule out other anomalies
The best & the newest operation is PSARP

Atresia Ani

Definition
Disruption passage of stool due to
the blockage / constriction in the
anus / rectum because the anus is
not formed / stenosis.
The etiology is still unknown.

Pathophysiology
Absence of anus then mekoneum and air will be captured in
a gut lumen. Due to increased intraluminal pressure, will
reduce drainage water and sodium from the lumen of the
intestine into the blood. Because every day a lot of fluid
secreted into the lumen of the intestine, the absence of
absorption would lead to intraluminal filling quickly. Vomiting
and discharge through the NGT will add to the loss of fluids
and electrolytes. The effect of this loss is shrinking
extracellular fluid space resulting in shock - hypotension,
reduced cardiac output, decreased tissue perfusion and
metabolic acidosis. Stretching intestine resulting in a vicious
cycle of continuous decline in the fluid absorption and
increased secretion of fluid into the intestine. Local Effects of
ischemia due to stretching of the intestine is distended and
increased permeability due to necrosis, with bacterial toxins
toxin absorption into the peritoneal cavity and systemic
circulation.

Clinical Manifestation

Anus looks red

Dilated bowel image
Sometimes seem ileus obstruction
Auscultation sounds hiperperistaltik
Baby vomiting that began at the age of
24-48 hours
No defecation mekoneum
Thermometers can not enter from the
anus, as held by the network.

Classification
1. Stenosis of the lower rectum or at the anus
2. Membrane anus settled
3. Imperforate anus and rectum ends deadlocked
located at various distances from the peritoneum.
4. Separate anus with the tip of the clogged
rectum.
In the third ad is often accompanied rectovaginal
fistula, rektoperitoneal, or rektourinarius.

Investigation
Abdomen photo: plain, with contrasting or
sistouretrogram. (to know until where the
blockage is)
1. The abdomen showed air in the intestine of
neonates with interrupted suddenly. This
indicates that there is obstruction in the area.
2. The absence of air shadow in the pelvic
cavity of the newborn needs to suspected or
anal atresia ani imperforatus.

Management
Surgery
1. Excision of the anal membrane
2. Operation, the temporary colostomy and repair performed
after 3 months at a time.

Preoperative and post-surgical care:

-

Attach infusion
Aspiration of gastric fluid
Observations of vital signs
Parental Support.
Give the sense that the operation was carried out two stages.
First create an artificial anus and after 3 months the second
surgery reconnection. Parents are expected to maintain the
cleanliness of the operating area and children's health.
- Routine care