Hirschsprung's Lecture

7/29/2019 Hirschsprung's Lecture

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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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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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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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Hirschsprung's Lecture