IN THE NAME OF MERCIFUL GOD

Hirschsprung’s disease “Primary Megacolon”
Khaled Ashour JR Hospital Oxford

Hirschsprung’s Disease Definition
It

is a primary gastrointestinal disease caused by congenital absence of the intestinal ganglion cells, namely, the submucosal “Meissners”, and the intermuscular “Aurbachs”

Hirschsprung’s 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%).

Hirschsprung’s Disease Incidence:  The

affected part of the intestine: Rectosigmoid area : 77% Long segment colonic : 14% Total colonic : 7% Total GIT : 2%

Etiology
 Theories:

- Failure of migration of the neuroenteric cells distally along the alimentary canal. - Presence of hostile environment (lack of neural cell adhesion molecule NCAM). - Immunologic theory: increased expression of class II antigen.

Pathophysiology
 Due

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.

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”

1

2

3

Pathology (Cont.)
 Microscopically:

1) Absence of Meissners and Aurbach’s ganglia. 2) Abundant nerve fibers. This might be evident either by Hematoxylin & Eosin stain, or better, using Acetyl Choline estrase stain.

Hirschsprung’s disease

Presentation:

1) Neonatal: Onset -> during neonatal period.  Clinical picture: *Delayed passage of meconium. *Abdominal distension. * Constipation. * +\- bilious vomiting.

H.D. Presentation
2) Infantile type: * Chronic constipation. * Abdominal distension. * Bouts of abdominal colics * Very infrequently vomiting. * mild growth retardation.

H.D Clinical picture
 O/E:

- Abdominal distension, lax abdomen if uncomplicated. - visible intestinal loops. - P/R: Passage of gush of stool and gases.

H.D. Investigations
1) Plain X ray abdomen standing. 2) Ba enema 3) rectal biopsy. 4) Rectal manometry.

H.D. Investigations
 Plain

X ray abdomen

H.D. Investigations

 Plain

X ray abdomen

H.D. Investigations

 Plain

X ray abdomen

H.D. Investigations

 Ba

enema lateral view

H.D. Investigations

 Ba

enema A-P view

H.D. Investigations

 Ba

enema A-P view

H.D. Investigations

 Ba

enema A-P view.

Classical management
 Performing

defunctioning colstomy.  Followed later on by the definite pullthrough operation.  Finally, closure of colostomy

H.D. Management
 Pull

through techniques: 1) Soave endorectal pull-through. 2) Swenson pull-through. 3) Duhamel pull-through. 4) Rhebein anterior resection.

H.D. Surgical treatment

 Child

with Rt. TV. Colostomy

Duhamel Pullthrough

Swenson Pull-through

Soave pull-through

Identification of the pathological segment.

Soave pull-through

Development of the seromuscular cuff.

Soave pull-through

The healthy colon is ready to be pulled through the seromuscular cuff.

Soave pull-through

The colon after being pulled through the cuff to outside the body.

New trends in management
Two-stages modality: First leveling pelvic colostomy, followed by definite pull-through. Performing the one stage pull-through technique without preliminary colostomy (in older age group).

More recent
 The

introduction of one stage transanal pullthrough technique by De la Torre in 1998.  Yet, few reports are available about its application in the neonatal period.

Technique for transanal pull-through

Technique of TAPT
 Performing

anal

dilatation.

Technique of TAPT
 Retraction

is effected using “Langenbeck” retractor instead of the classical “Lone-Star” retractor

Technique of TAPT
Tension

sutures application.

Technique of TAPT
 Second

layer of tension sutures.

Technique of TAPT
Dissection

of the mucosa leaving the seromuscular cuff.

Technique of TAPT
 Proceeding

dissection till peritoneal reflection.

Technique of TAPT
 The

cuff is opened, and full thickness dilated colon is now pulled with mesenteric devascularization.

Technique of TAPT
 The

excised colorectal segment, showing the coning of H.D.

 relatively

long segment H.D.

Technique of TAPT
 After

the pulled segment is cut, the cut edge is sutured to the anal mucosa.

Technique of TAPT
 Rectal

tube +/drain is left for one day.

Postoperative barium enema
Ba

enema was done in the course of the follow-up to evaluate the colon postoperatively

Thank you

Sign up to vote on this title
UsefulNot useful