This document discusses Hirschsprung's disease, also known as congenital megacolon. It is a congenital disorder characterized by the absence of ganglion cells in the distal colon, resulting in functional obstruction. The pathology involves absent ganglion cells in the anorectum extending variably upwards. Clinical features include failure to pass meconium in newborns and chronic constipation in older children. Diagnosis is confirmed by full thickness rectal biopsy showing absent ganglion cells. Surgical treatment involves excision of the aganglionic segment and anastomosis of the healthy colon to the anorectum.

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