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

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Presentation on theme: "Hirschsprung’s Disease: an approach to management Edward Kiely Great Ormond Street London."— 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 Soaveenterocolitis dribbling incontinence Swensonstricture Duhamelfaecaloma

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 - stoma

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

10 Hirschsprung’s Disease what type of stoma? <1yrloop skin bridge >1yrdouble 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 portsRIF 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 33 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 series5-10% 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) 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% 33% Martin operation troublesome

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

39 Hirschsprung’s Disease major complications leak3 enterocolitis3 transit. pullthro2 deaths2

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

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