Dr.Dinesh.M.G Professor of Surgery J.J.M.M.C Davangere

1. Dr. Dinesh. M.G
Professor of Surgery
J.J.M.M.C.
Davangere

2. Anatomy: Blood supply

3. Venous drainage

4. Lymphatic drainage

5. Congenital megacolon

6. Hirschsprung’s disease
 Congenital disorder characterised by absence of ganglion
cells in the Meissner’s and Aurebach’s plexus of distal
colon resulting in functional obstruction

7. Hirschsprung’s disease
Pathology
 Absence of ganglion cells ( Auerbach’s & Meissner’s
plexus) in anorectum extending variably upwards
 Contracted non peristaltic segment with dilated
hypertrophied normal colon above
 Three zones
 Distal spastic segment zone(aganglionic)
 Proximal middle transitional cone shaped zone (sparse
ganglion cells)
 Hypertrophied dilated proximal zone(normal ganglionic)

8. Hirschsprung’s disease

9. Hirschsprung’s disease
Clinical features
 Occurs one in 4500 live births
 Familial tendency(10%)
 More common in males
 Hirschsprung’s disease should be considered in any
newborn who fails to pass meconium in first 24-48 hours
 Currently 90% of cases are diagnosed in newborn period

10. Hirschsprung’s disease
Clinical features
 Abdominal distension with
features of intestinal obstruction
 Enterocolitis in neonate with
perforation and septicemia
 Older children have chronic
constipation since birth with
stunted growth
 Rectal examination
 Rectum is empty and grips
examining finger
 No perianal soiling

11. Hirschsprung’s disease
Investigations
 Plain x-ray : distended bowel loops
 Barium enema: narrow distal colon with proximal dilation
 Full thickness rectal biopsy: Confirmatory test
 Absence of ganglion cells
 Hypertrophic nerve fibres in nerve plexus
 Anorectal manometry: absence of rectosphincteric
inhibitory reflex

12. Hirschsprung’s disease
 Plain x-ray
Dilated bowel loops

13. Hirschsprung’s disease
 Barium enema

14. Hirschsprung’s disease
Treatment principle
 Excision of entire aganglionic segment and anastomosis of
healthy innervated colon to the distal anorectum(with or
without an initial diverting colostomy)

15. Hirschsprung’s disease
Treatment
 Traditionally : diverting colostomy followed by definitive
surgery once child attains 10 kgs
 Operations
 Modified Duhamel operation
 Swenson’s operation
 Soave’s mucosectomy and pull through operation
 Total proctocolectomy with ileoanal anastomosis for total
colonic Hirschsprung’s disease
 Anorectal myomectomy for ultra short segment disease

16. Hirschsprung’s disease

17. Hirschsprung’s disease

18. Acquired megacolon
 Hard faecal mass in rectum
 Anus is patulous with perianal soiling
 Poor toilet training in infancy may be the cause
 Full thickness biopsy of rectal wall show normal ganglion
cells
 Barium enema shows gross faecal loading of enlarged
rectum without cone formation
 Treated usually by repeated enemas, manual evacuation,
toilet training and laxatives

19. Points to remember
 What is the other name of Hirschsprung’s disease
 Congenital megacolon
 What is the characteristic pathologic feature of Hirschsprung’s
disease
 Absence of ganglion cells in colonic wall
 What should be suspected if a newborn fails to pass meconeum in
first 24-48 hours
 Hirschsprung’s disease
 Which is the important confirmatory test in the diagnosis of
congenital megacolon
 Full thickness rectal biopsy
 Surgical treatment of congenital megacolon includes following
operations
 Modified Duhamel operation
 Swenson’s operation
 Soave’s operation
 Anorectal myomectomy

20. Thank you