COLON Anatomy / Physiology:

Location, blood supply &

venous drainage,
lymphatic drainage and
nerve supply
Function:
absorption of fluid
and electrolyte
Transport and
temporary storage of
feces

Megacolon

Congenital Megacolon
(Hirschprungs disease)

Megacolon = larger colon

Dolicocolon = longer colon

HD is characterized by the
absence of myenteric and
submucosal ganglion cells in the
distal alimentary tract;
decreased motility in the affected
bowel segment

Frequency
approximately 1 per 5000 live births.

Sex: 4 times more common in males than females.

Age:
Nearly all children with Hirschsprung disease are diagnosed
during the first 2 years of life.
one half are diagnosed before they are aged 1 year.
Minority not recognized until later in childhood or
adulthood.

Mortality/Morbidity:
The overall mortality of Hirschsprung enterocolitis is 2530%, which accounts for almost all of the mortality from
Hirschsprung disease.

Pathophysiology
Absence of parasympathetic ganglion cells in the myenteric
and submucosal plexus of the rectum and/or colon.
Failure of migration of neuroblasts into the gut from vagal
nerve trunks at the end of the first trimester of fetal life.
(proximal colon by 8 weeks and in the rectum by 12 weeks)
Arrest in migration leads to an aganglionic segment.
Loss of ganglion cells extends for a variable distance above
the anorectal junction.

Pathophysiology
This absence of normal parasympathatic innervation
prevents gut peristalsis, leading to functional constipation.
The proximal colon hypertrophied by trying to overcome
functional obstruction.
Transitional zone exists between normal and abnormal
aganglionic intestine.
Failure of the internal sphincter to relax with rectal
distention is pathognomonic.

Acetylcholine concentrations in aganglionic segments are

threefold lower than in ganglionic segments.

Transitional zone

Gross photograph of a distal colonic segment resected for Hirschsprung

disease. Note the dilated, proximal portion separated from the constricted
distal portion by a transition zone

HD can be classified by the extension of the

aganglionosis as follows:

Classical HD (75% of cases): Rectosigmoid

Long segment HD (20% of cases)
Total colonic aganglionosis (3-12% of cases)
rare variants include the following:
Total intestinal aganglionosis
Ultra-short-segment HD (involving the distal rectum below
the pelvic floor and the anus)

Clinical presentation:
Newborns :
Failure to pass meconium within the first 48 hours of life
Abdominal distension that is relieved by rectal stimulation or
enemas
Vomiting
Neonatal enterocolitis
Symptoms in older children and adults include the following:
Severe constipation
Abdominal distension
Bilious vomiting
Failure to thrive
Rectal exam : rectal vault is found devoid of stool and the anal
canal feels narrow with increased tone.

Complications
intestinal perforation (particularly at the appendix)
enterocolitis,
water intoxication, malnutrition, failure to thrive, and
anemia.
Water intoxication results from the use of tap water
enemas.
or there may be hypertonic dehydration from saline
enemas.

Investigations

a.
b.
c.

Plain abdominal radiography

Dilated bowel
Air-fluid levels.
Empty rectum

Investigations
Contrast enema
a. Transition zone
b. Abnormal, irregular
contractions of
aganglionic segment
c. Delayed evacuation of
barium

Investigations
Manometry
- Absence of normal relaxation of the internal
sphincter when the rectum is distended with a
balloon.
Rectal biopsy specimen are usually taken from
just above the anorectal junction.
absence of ganglion cells
hypertrophy and hyperplasia of nerve fibers,
increase in acetylcholinesterase-positive nerve fibers in the lamina propria
and muscularis mucosa.

Treatment
The treatment is surgical removal or bypass of the
aganglionic bowel,
This can be performed by means of:
preliminary colostomy (for a few months till the child weighs 10 kg. the
child will be between 10 months and 1 year of age and toilet training can usually
start soon after the operation) followed by a definitive pull-through

procedure or,
primary definitive procedure.

Examples include:
Soave pull-through procedure,
Duhamel procedure,
Swenson procedure.

Duhamel operation
aganglionic segment is resected
colon is brought behind the rectum and anastomosed to it by a stapler

Swensons procedure (proctocolectomy)

Coloanal anastomosis (Soaves operation)
pull ganglionated bowel through sleeve of rectum
reserved for older children, teenagers and adults

Post operative complications

Early :
Anastomotic leak
Infection (pelvic abscess, wound infection)
Late
:
anastomotic stricture
enterocolitis
incontinence

Hirscprungs disease

Acquired megacolon

Chagas disease (trypanosoma cruzi) leads to

denervation of hollow viscera and consequent
dysfunction (megacolon, megaesophagus, gastric
dilatation)
Neurologic disorders / psychotic patients
Clinic : constipation, abdominal distension
Investigations : BE
Treatment :
- medical (diet w fibres, prokinetiks, laxatives,
enemas),
- surgery in obstructions (segmental colectomies)