Presentation is loading. Please wait.

Presentation is loading. Please wait.

Hirschsprung’s Disease: an approach to management

Similar presentations

Presentation on theme: "Hirschsprung’s Disease: an approach to management"— Presentation transcript:

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

Download ppt "Hirschsprung’s Disease: an approach to management"

Similar presentations

Ads by Google