for undergraduate medical students

1. Hirschsprung Disease
MAGDI AHMED LOULAH
PROFESSOR OF PEDIATRIC SURGERY, MENOUFIA UNIVERSITY

2. Hirschsprung disease
 HD is a developmental disorder characterized by absence of ganglion cells in the
distal rectum leading to functional intestinal obstruction and megacolon.
 Incidence: 1: 1500-5000

3. Hirschsprung’s
disease
pathophysiology
- Absent ganglion cells in
the distal colon
(Rectum).
- Over activity of the
adrenergic receptors
- Spasticity of the rectum
and failure of relaxation
- Accumulation of
meconium in the
proximal colon and
progressive dilatation
- Enterocolitis

4. Histopathology
Absence of
ganglion
cells.
Hypertrophy
of nerve
terminals
(arrows).
Immunostaini
ng: L-
calretinin
and

5. Clinical features
 Delayed passage of meconium
 Abdominal distention
 Vomiting
 Examination
 Dry anus
 P/R empty rectum with passage of a gush of meconium on extraction of your finger

6. Investigations
 Plain erect X-Ray
 Contrast enema in AP ant Lateral position
 Rectal biopsy
 Anorectal manometry : absent RAIR reflex

8. Treatment
 Multistage surgery
 First stage: colostomy
 Second stage: Pullthrough operation
 Swenson pullthrough
 Duhamel Pullthrough
 Soave pullthrough
 One stage transanal pullthrough operation.
 Soave pullthrough
 Swenson pullthrough