Harold

Hirschprung (1886)

described 2 children with the classic clinical &

anatomic characteristics of the disease

the disease congenital dilatation of the colon
Fenwick

(1900) Three basic theories

Malfunction theory
Obstructive theory

Spastic theory

Ehrenpreis (1946)
the colon became secondarily dilated due to
more distal obstruction

Whitehouse&Kernohan and
Zuelzer&Wilson (1948)
the absence of ganglion cells of the myenteric
plexus

Swenson&Bill (1948)
the first corrective surgery

in 4400 to 1 in 7000 live births

The male : female ratio = 4 : 1
In long segment disease the ratio
approach 1 : 1
The racial distribution is similar.

 Clinical

Presentation
Radiographic studies
Anorectal manometry
Rectal biopsy

 History

of constipation dating back to the

newborn period
History of delayed passege of fecal matter
within the first 48 hours of life
Other presenting sign & symptom :
Constipation
Abdominal distention
Poor feeding

 Physical

examination
Assesment of the anal position
Rectal examination

abdominal radiographs reveal

distended loops of intestine.
A contrast enema :
Plain

Water soluble-contrast
Barium
The

point of caliber change (transition

zone) is the key radiographic finding

 Another

methode of diagnosing HD.

The parameters are assessed :
Absence of a relaxation reflex after a distensing

bolus in the rectal lumen.

Thr resting anal sphincter pressure (elevated with
HD).

 Have

some limitations
The accuracy rates vary widely.
The rate of false-negative results varies
from 0-24%
The rate of false-positive results varies
from 0-62%

Rectal biopsy the gold standard for the

diagnosis of HD
Swenson et al (1959) first described the
method of full-thickness rectal biopsy
Shandling reported the method of
suction rectal biopsy
Gherardi (1960) demonstrated the level
of aganglionosis was identical in the
submucosa and myenteric plexus.

 The

diagnostic accuracy is reportedly as

high as 99,7%
The most common problem :
An inadequate specimen with an insufficient

amount of mucosa
Performance of biopsy too close to anal sphincter

Other mechanical obstruction

Meconium ileus
Distal ileal or colonic atresia
Small intestine stenosis
Low imperforate anus

Functional obstruction of the intestinal tract

Prematurity
Small left colon syndrome
Meconium plug syndrome
Sepsis and electrolyte imbalance
Cretinism and myedema
Functional constipation

 Decompression

Nasogastric tube
Repeated emptying of the rectum with rectal tube

and irrigation

leveling end colostomy with

frozen-section confirmation of the level of
ganglion cells.

Colostomy

 Swensons

technique
Duhamel procedure
Soave procedure
Rehbein procedure
Myectomi
Martin procedure
Kimura aganglionic patch

 Early

complication

Anastomotic leaks
Anastomotic stricture
Wound infections

Disturbance of micturition
Late

complication

Chronic constipation

enterocolitis