1. Hirschsprung’s disease, the past and the present
Dr.Dávidovics Sándor
Petz A.County Teaching Hospital Department of Paediatric Surgery
Győr

2. Hirschsprung’s disease
One in 5000 live births
Male-to-female ratio of 4:1
In 90 % are mature newborns
In 1-6 % are familial
In 10%-30% are associated anomalies

3. Hirschsprung’s disease
To rectosigmoid in 75%
Short segment (rectum)
Ultra short (part of the rectum)
Long segment
Total aganglionosis (large intestine, sometimes a part of small intestine) up to 10%
Segmental

4. Hirschsprung’s disease
Embriology
Migration of neuroblasts on the 5-7 g.week
On the 12. g.week they reach the rectum
Myenteric plexus
Submucous plexus
Ganglions
The process of maturation continues after the birth

5. Hirschsprung’s disease
Innervation
adrenergic
cholinergic
Nonadrenergic and noncholinergic
-pepdidergic
-nitrergic
-nerve-supporting cells
-interstitial cells in Cajal

6. Hirschsprung’s disease
Histopathology
Absence of ganglionic cells
Hypertrophic cholinergic nerve trunks
Limited number of adrenergic fiber

7. Hirschsprung’s disease
Clinical symptoms
The disease can considered to be incomplete intestinal obstruction
The lenght of the aganglionic segment is variable
The symptoms are variable too
The symtoms appears in different ages

8. Hirschsprung’s disease
Symptoms in newborn age
Fail to pass meconium (in 24 hours of life)
Abdominal distension, but the abdomen is palpable
Vomiting
The rectal tube can’t be put easily
After irrigation the signs and symptoms return again in a few days

9. Hirschsprung’s disease
Symptoms in newborn age(enterocolitis)
Life-threatening condition
Diarrhea: it can be an early sign
Toxic megacolon
Abdominal distension
Bile-stained vomiting
Fiver and signs of dehydration
Rectal tube:explosive expulsion of gas and foul-smelling stools

10. Hirschsprung’s disease
Symptoms in infants
Constipation
Meteorism
Palpable faecaloma
Sometimes putrescent diarrhea
Ulceration, bleeding
Hypoproteinaemia, anaemia
Electrolyt disorders

11. Hirschsprung’s disease
Symptoms in childhood
Gracile limbs
Dilated drumlike belly
Long history of constipation
Defecation in 7-10 days
Multiple fecal masses
The stimulus of defecation is missing
Rectum is empty and narrow

12. Hirschsprung’s disease

13. Hirschsprung’s disease
Examinations
Plain abdominal radiographs
Contrast enema
Anorectal manometry
Rectal biopsy,histopathology

14. Hirschsprung’s disease
Plain X ray: air/fluid levels,free air
Contrast enema:
-Narrow distal segment
-Funnel-shaped transition zone
-Dilation of proximal colon
-After hours the contrast material
is in the bowel
-Mucosal irregularity (enterocolitis)

15. Hirschsprung’s disease

16. Hirschsprung’s disease

17. Hirschsprung’s disease
Anorectal manometry
Anorectal pressure is elevated
No relaxation of the internal sphincter
It can be put through safely in newborn age as well

18. Hirschsprung’s disease

19. Hirschsprung’s disease
Biopsy and Histology
Full-thickness strip-biopsy
Suction biopsy

20. Hirschsprung’s disease

21. Hirschsprung’s disease

22. Nitrergic innervation pattern (light microscopy, x100)
Normal colon Hirschsprung’s disease

23. Nitrergic innervation pattern (light microscopy, x100)
Hirschsprung's disease Total intestinal aganglionosis

24. Hirschsprung’s disease
Treatment:
Decompression: introduce a rectal tube and irrigation
Colostomy
Definitive procedures
Closing of the stoma

25. Hirschsprung’s disease

26. Hirschsprung’s disease

27. Hirschsprung’s disease

28. Hirschsprung’s disease
Treatment
Transanal Endorectal Pull-Trough
It can be performed safely in infant as well
Generally one-stage surgery
No abdominal phase
The anastomosis is happening in a „safe” place at the pectinate line

29. Hirschsprung’s disease

30. Hirschsprung’s disease

31. Hirschsprung’s disease

32. Hirschsprung’s disease

33. Hirschsprung’s disease
Long-term outcome
If started in time, under adequate treatment is good.

34. Summary Past Operation in 2 or 3 steps
Pull-through on the afunctional bowel
Strip-biopsy
Stoma-wearing
Nursing,dilatations
Higher possibility of complcations
Present
Operation in 1 step
- Pull-through the functional gut
Suction biopsy
No stoma
- No dilatation
Smaller possibility of complications

35. Summary Past Long hospitalisation High input /operations,medicines/
Free stoma tools
Travelling support
Long-term financial support of goverment
Harder social integration /kidergarten,school/
Present
Short hospitalisation
Small input
No stoma
Less controll examination
The support can be extinguished earlier
Easy social integration

36. Thank you for your attention !