1. Hirschsprung’s Disease: an approach to management
Edward Kiely
Great Ormond Street
London

2. Hirschsprung’s disease
aim of surgery
relieve symptoms
no constipation
normal bowel habit
normal control

3. Hirschsprung’s disease
to achieve this
multiple operations devised
many operations = none perfect

4. Hirschsprung’s disease
all operations have specific complications
Soave enterocolitis
dribbling incontinence
Swenson stricture
Duhamel faecaloma

5. Hirschsprung’s Disease
diagnosis by histology/histochemistry rectal suction biopsy at 2.5, 3.0 cms punch biopsy occasional full thickness no reliance on X-ray studies for diagnosis / length

6. Hirschsprung’s Disease
who gets biopsied? distal obstruction in neonates all meconium plug obstruction when constipation begins <1 yr

7. Hirschsprung’s Disease
once the diagnosis is made: washouts or stoma? depends on condition of child

8. Hirschsprung’s Disease
if not unwell
- try washouts
if unwell
perforated, enterocolitis
- stoma
if washouts fail

9. Hirschsprung’s Disease
where to site the stoma? frozen section optimal otherwise distal ileum

10. Hirschsprung’s Disease
what type of stoma? <1yr loop skin bridge >1yr double barrelled remember to biopsy the stoma

11. Hirschsprung’s Disease
washouts usually done with NaCl once/day sufficient in most important to verify that the washouts are succeeding

12. Hirschsprung’s Disease
washouts there are potential problems parent compliance long segment may not work enterocolitis perforation

13. Hirschsprung’s Disease
when to perform the pullthrough? is there an ideal age? no evidence that younger is better

14. Hirschsprung’s Disease
at present surgery in first few months advised improved anaesthesia and supportive care make this a safe approach

15. Hirschsprung’s Disease
we would usually operate <3mths except total colonic disease severe enterocolitis premature

16. Hirschsprung’s Disease
which operation? Swenson Soave (trans-anal) Duhamel Rehbein

17. Hirschsprung’s Disease
choice of operation is surgeon dependent now also driven by patient expectation - no scars

18. Hirschsprung’s Disease
preference - laparoscopic Duhamel - one, two or three stage camera RUQ 2 working ports RIF LUQ 3mms instruments

19. Hirschsprung’s Disease
laparoscopic Duhamel initial sero-muscular biopsies distal, mid, proximal sigmoid splenic flexure mid-transverse hepatic flexure

20. Hirschsprung’s Disease
laparoscopic Duhamel short rectal pouch – 4 cms rectum everted, closed, replaced anastomosis on dentate line Endo-GIA stapler

21. Hirschsprung’s Disease
laparoscopic Duhamel if stoma present – close at 2 weeks

22. Hirschpsrung’s Disease
what results should be expected?

23. Hirschsprung’s disease
Mishalany, Woolley (1987)
137 patients
62 reviewed
follow up yrs (38 >5yrs)

24. Hirschpsrung’s disease
Mishalany, Woolley (1987)
Soave
Swenson 15
Duhamel 14

25. Hirschsprung’s disease
Mishalany, Woolley (1987)
31 soiling/ incontinent Swenson worst
20 enterocolitis
Duhamel least
manometry abnormal in majority

26. Hirschsprung’s disease
Catto-Smith et al (1995)
60 children (out of 87)
9 yrs post op

27. Hirschsprung’s disease
Catto-Smith et al (1995)
all Soave
home diaries, questionnaires

28. Hirschsprung’s disease
Catto-Smith et al (1995)
38% deficient sensation
80% reported soiling
53% severe soiling
27% less severe soiling
no improvement with age

29. Hirschsprung’s disease
long term complications
constipation
incontinence
enterocolitis
strictures/ fistulae

30. Hirschsprung’s disease
constipation
10-35% in all operations
incontinence
0-50% all operations
most reports <10%

31. Hirschsprung’s disease
enterocolitis
0-34% all operations
Swenson worst
trans-anal now reporting 50%

32. Hirschsprung’s disease
strictures/fistulae
most series % strictures
<5% fistulae
Swenson, Soave

33. Hirschsprung’s disease
total colonic aganglionosis
Escobar et al (2005)
review
36 patients
19% died

34. Hirschsprung’s disease
Escobar et al (2005)
81% continent
highest morbidity Soave
Martin modification
? Kimura patch useful

35. Hirschsprung’s disease
Tsuji et al (1999)
48 patients
6% mortality

36. Hirschsprung’s disease
Tsuji et al (1999)
41(85%)had pull through
38 Duhamel (13 Martin)
3 Soave
6 permanent stoma

37. Hirschsprung’s disease
Tsuji et al (1999)
incontinence at: yrs
82% 57% %
Martin operation troublesome

38. Hirschsprung’s Disease
1983 – new patients 58 neonates 19 total colonic

39. Hirschsprung’s Disease
major complications leak 3 enterocolitis 3 transit. pullthro 2 deaths 2

40. Hirschsprung’s disease
in conclusion
pathophysiology still unclear
results mainly operation dependent?
myriad of operations
most of literature shows no difference
results improve with age

41. Hirschsprung’s disease
recommend
do the procedure which suits you
much to recommend laparoscopy
Duhamel- short pouch/low anastomosis