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Hirschprung
Hirschprung
congenital megacolon
st nerve 1
th week gestation 5 arrive in the
esophagus
anatomi
Two basic theories exist regarding the
embryologic defect in hirschprungs desease
Blokade of Nonsupportive
normal neural environment
crest cell Abnormal distribution
fibronectin and laminin
migration
Vascular defect
Diagnosis
History
failure to pass meconium, painless abdomenal distension & constipation)
Physical examinations
Distended abdomen with Multiple fecal masses on abdominal examination
on DREcharacteristically there is
Anal sphincter is hypertonic
Rectum is typically empty.
Hard fecal mass.
Radiology
1. Plain x-rays of the abdomen :Erect & supine
2. Contrast Enema.
Shows narrow distal segment,funnel-shaped dilatation at level of transition zone with
marked dilatation of the proximal colon.
24-hrs delayed films is important in diagnosis; it shows poor emptying with barium
throughout the colon, as opposed to the child with psychogenic stool holding in
whom the barium generally collects in the distal rectosigmoid.
contrast enema should be done with out preparation of bowel,
4. Electromanometry :
not useful in neonate
excellent screening tool in infant & children .
The classic finding is the absence of the recto anal inhibitory
reflex when the rectum is distended.
5. Rectal biopsy :
Rectal biopsy is the definitive diagnostic test and demonstrates
absence of ganglion cells, nerve hypertrophy and stains indicating
increased acetylcholinesterase activity.
suction mucosal biopsy (at different levels ). Can be done without
anesthesia
full thickness biopsy is done under general anesthesia.
1. Open surgery :
There are many surgical options for Pull-through operation. All aiming at
resection of aganglionic segment and anastomosing the two normal
ganglionic ends. They give excellent result in 90%.
a.swenson.
b.soave.
c.Rehbein.
d. Duhamel.
e. Boley's.
2. LAPAROSCOPY .
3. TEPT transanal endorectal pullthrough (without laparotomy )
COMPLICATIONS of Pullthrough
1. anastomotic leak.
2. stricture .
3. retraction of the colon.
4. fecal incontinence (soiling or encopresis ).
5. persistant constipation.
Distinguishing features between childhood functional
constipation and Hirschsprungs disease