IN THE NAME OF MERCIFUL GOD

Hirschsprungs disease Primary Megacolon

Khaled Ashour JR Hospital Oxford

Hirschsprungs 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

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%).

Hirschsprungs 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

Pathology (Cont.)
Microscopically:

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

Hirschsprungs 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