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IN THE NAME OF MERCIFULGOD
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Hirschsprungs disease
Primary Megacolon
Khaled Ashour
JR Hospital
Oxford
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Hirschsprungs Disease
Definition
It is a primary gastrointestinal disease
caused by congenital absence of theintestinal ganglion cells, namely, the
submucosal Meissners, and the
intermuscular Aurbachs
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Hirschsprungs Disease
Incidence: -
1 : 4400 to 1 : 7000 live birth.
In Classic H.D. male : female = 4 : 1. In long segment H.D. M : F = 1 : 1.
No racial difference.
Increased incidence in familial cases (2-18%).
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Hirschsprungs Disease
Incidence: -The affected part of the intestine:
Rectosigmoid area : 77%Long segment colonic : 14%
Total colonic : 7%
Total GIT : 2%
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EtiologyTheories: -
- Failure of migration of the neuroenteric cellsdistally along the alimentary canal.
- Presence of hostile environment (lack of neural
cell adhesion molecule NCAM).
- Immunologic theory: increased expression of class
II antigen.
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PathophysiologyDue to the absence of ganglia, the affected
segment loses its receptive relaxation ability.
Thus, it becomes functionally contracted.
Proximally, the normal segment overcontracts to
pass the stool distally, which results in gradual
dilatation and hypertrophy.
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Pathology So, the gross pathology will show 3 distinct
regions: -
1) the narrow segment affected.
2) A transitional zone hypoganglionic
3) dilated hypertrophied segment normal
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1
2 3
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Pathology (Cont.)Microscopically: -
1) Absence ofMeissners
andAurbachs
ganglia.2) Abundant nerve fibers.
This might be evident either by Hematoxylin &
Eosin stain, or better, using Acetyl Choline estrasestain.
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Hirschsprungs disease Presentation:
1) Neonatal: Onset -> during neonatal period.Clinical picture:
*Delayed passage of meconium.
*Abdominal distension.* Constipation.
* +\- bilious vomiting.
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H.D. Presentation2) Infantile type:
* Chronic constipation.
* Abdominal distension.
* Bouts of abdominal colics
* Very infrequently vomiting.* mild growth retardation.
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H.D Clinical pictureO/E:
- Abdominal distension, lax abdomen if
uncomplicated.
- visible intestinal loops.
- P/R: Passage of gush of stool and gases.
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H.D. Investigations1) Plain X ray abdomen standing.
2) Ba enema
3) rectal biopsy.
4) Rectal manometry.
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H.D. InvestigationsPlain X ray
abdomen
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H.D. Investigations
Plain X ray abdomen
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H.D. Investigations
Plain X ray abdomen
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H.D. Investigations
Ba enema lateral view
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H.D. Investigations
Ba enema A-P view
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H.D. Investigations
Ba enema A-P view
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H.D. Investigations
Ba enema A-P view.
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Classical managementPerforming defunctioning colstomy.
Followed later on by the definite pull-through operation.
Finally, closure of colostomy
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H.D. Management Pull through techniques:
1) Soave endorectal pull-through.
2) Swenson pull-through.
3) Duhamel pull-through.
4) Rhebein anterior resection.
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H.D. Surgical treatment
Child with Rt. TV.
Colostomy
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Duhamel Pull-through
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Swenson
Pull-through
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Soave pull-through Identification of the
pathological segment.
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Soave pull-through
Development of the
seromuscular cuff.
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Soave pull-through
The healthy colon is
ready to be pulledthrough the
seromuscular cuff.
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Soave pull-through
The colon after being
pulled through the cuff
to outside the body.
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New trends in management Two-stages modality: First leveling
pelvic colostomy, followed by definitepull-through.
Performing the one stage pull-through
technique without preliminary
colostomy (in older age group).
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More recentThe introduction of one stage transanal pull-
through technique by De la Torrein 1998.
Yet, few reports are available about its application
in the neonatal period.
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Technique for transanal pull-through
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Technique of TAPTPerforming anal
dilatation.
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Technique of TAPTRetraction is effected
using Langenbeck
retractor instead of theclassical Lone-Starretractor
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Technique of TAPTTension sutures
application.
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Technique of TAPTSecond layer of
tension sutures.
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Technique of TAPTDissection of the
mucosa leavingthe seromuscular
cuff.
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Technique of TAPTProceeding
dissection till
peritoneal reflection.
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Technique of TAPTThe cuff is opened,
and full thickness
dilated colon is
now pulled with
mesentericdevascularization.
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Technique of TAPTThe excised colorectal
segment, showing the
coning of H.D.
relatively long
segment H.D.
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Technique of TAPTAfter the pulled
segment is cut, the
cut edge is sutured
to the anal mucosa.
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Technique of TAPTRectal tube +/-
drain is left for one
day.
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Postoperative barium enemaBa enema was
done in thecourse of thefollow-up toevaluate thecolonpostoperatively
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Thank you
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