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Hirschsprung’s Disease: an approach to management
Edward Kiely Great Ormond Street London
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Hirschsprung’s disease
aim of surgery relieve symptoms no constipation normal bowel habit normal control
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Hirschsprung’s disease
to achieve this multiple operations devised many operations = none perfect
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Hirschsprung’s disease
all operations have specific complications Soave enterocolitis dribbling incontinence Swenson stricture Duhamel faecaloma
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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
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Hirschsprung’s Disease
who gets biopsied? distal obstruction in neonates all meconium plug obstruction when constipation begins <1 yr
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Hirschsprung’s Disease
once the diagnosis is made: washouts or stoma? depends on condition of child
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Hirschsprung’s Disease
if not unwell - try washouts if unwell perforated, enterocolitis - stoma if washouts fail
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Hirschsprung’s Disease
where to site the stoma? frozen section optimal otherwise distal ileum
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Hirschsprung’s Disease
what type of stoma? <1yr loop skin bridge >1yr double barrelled remember to biopsy the stoma
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Hirschsprung’s Disease
washouts usually done with NaCl once/day sufficient in most important to verify that the washouts are succeeding
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Hirschsprung’s Disease
washouts there are potential problems parent compliance long segment may not work enterocolitis perforation
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Hirschsprung’s Disease
when to perform the pullthrough? is there an ideal age? no evidence that younger is better
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Hirschsprung’s Disease
at present surgery in first few months advised improved anaesthesia and supportive care make this a safe approach
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Hirschsprung’s Disease
we would usually operate <3mths except total colonic disease severe enterocolitis premature
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Hirschsprung’s Disease
which operation? Swenson Soave (trans-anal) Duhamel Rehbein
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Hirschsprung’s Disease
choice of operation is surgeon dependent now also driven by patient expectation - no scars
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Hirschsprung’s Disease
preference - laparoscopic Duhamel - one, two or three stage camera RUQ 2 working ports RIF LUQ 3mms instruments
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Hirschsprung’s Disease
laparoscopic Duhamel initial sero-muscular biopsies distal, mid, proximal sigmoid splenic flexure mid-transverse hepatic flexure
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Hirschsprung’s Disease
laparoscopic Duhamel short rectal pouch – 4 cms rectum everted, closed, replaced anastomosis on dentate line Endo-GIA stapler
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Hirschsprung’s Disease
laparoscopic Duhamel if stoma present – close at 2 weeks
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Hirschpsrung’s Disease
what results should be expected?
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Hirschsprung’s disease
Mishalany, Woolley (1987) 137 patients 62 reviewed follow up yrs (38 >5yrs)
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Hirschpsrung’s disease
Mishalany, Woolley (1987) Soave Swenson 15 Duhamel 14
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Hirschsprung’s disease
Mishalany, Woolley (1987) 31 soiling/ incontinent Swenson worst 20 enterocolitis Duhamel least manometry abnormal in majority
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Hirschsprung’s disease
Catto-Smith et al (1995) 60 children (out of 87) 9 yrs post op
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Hirschsprung’s disease
Catto-Smith et al (1995) all Soave home diaries, questionnaires
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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
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Hirschsprung’s disease
long term complications constipation incontinence enterocolitis strictures/ fistulae
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Hirschsprung’s disease
constipation 10-35% in all operations incontinence 0-50% all operations most reports <10%
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Hirschsprung’s disease
enterocolitis 0-34% all operations Swenson worst trans-anal now reporting 50%
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Hirschsprung’s disease
strictures/fistulae most series % strictures <5% fistulae Swenson, Soave
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Hirschsprung’s disease
total colonic aganglionosis Escobar et al (2005) review 36 patients 19% died
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Hirschsprung’s disease
Escobar et al (2005) 81% continent highest morbidity Soave Martin modification ? Kimura patch useful
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Hirschsprung’s disease
Tsuji et al (1999) 48 patients 6% mortality
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Hirschsprung’s disease
Tsuji et al (1999) 41(85%)had pull through 38 Duhamel (13 Martin) 3 Soave 6 permanent stoma
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Hirschsprung’s disease
Tsuji et al (1999) incontinence at: yrs 82% 57% % Martin operation troublesome
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Hirschsprung’s Disease
1983 – new patients 58 neonates 19 total colonic
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Hirschsprung’s Disease
major complications leak 3 enterocolitis 3 transit. pullthro 2 deaths 2
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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
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Hirschsprung’s disease
recommend do the procedure which suits you much to recommend laparoscopy Duhamel- short pouch/low anastomosis
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