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Hirschsprung’s Disease: an approach to management Edward Kiely Great Ormond Street London
Hirschsprung’s disease aim of surgery relieve symptoms no constipation normal bowel habit normal control
Hirschsprung’s disease to achieve this multiple operations devised many operations = none perfect
Hirschsprung’s disease all operations have specific complications Soaveenterocolitis dribbling incontinence Swensonstricture Duhamelfaecaloma
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
Hirschsprung’s Disease who gets biopsied? distal obstruction in neonates all meconium plug obstruction when constipation begins <1 yr
Hirschsprung’s Disease once the diagnosis is made: washouts or stoma? depends on condition of child
Hirschsprung’s Disease if not unwell - try washouts if unwell perforated, enterocolitis - stoma if washouts fail - stoma
Hirschsprung’s Disease where to site the stoma? frozen section optimal otherwise distal ileum
Hirschsprung’s Disease what type of stoma? <1yrloop skin bridge >1yrdouble barrelled remember to biopsy the stoma
Hirschsprung’s Disease washouts usually done with NaCl once/day sufficient in most important to verify that the washouts are succeeding
Hirschsprung’s Disease washouts there are potential problems parent compliance long segment may not work enterocolitis perforation
Hirschsprung’s Disease when to perform the pullthrough? is there an ideal age? no evidence that younger is better
Hirschsprung’s Disease at present surgery in first few months advised improved anaesthesia and supportive care make this a safe approach
Hirschsprung’s Disease we would usually operate <3mths except total colonic disease severe enterocolitis premature
Hirschsprung’s Disease which operation? Swenson Soave (trans-anal) Duhamel Rehbein
Hirschsprung’s Disease choice of operation is surgeon dependent now also driven by patient expectation - no scars
Hirschsprung’s Disease preference - laparoscopic Duhamel - one, two or three stage camera RUQ 2 working portsRIF LUQ 3mms instruments
Hirschsprung’s Disease laparoscopic Duhamel initial sero-muscular biopsies distal, mid, proximal sigmoid splenic flexure mid-transverse hepatic flexure
Hirschsprung’s Disease laparoscopic Duhamel short rectal pouch – 4 cms rectum everted, closed, replaced anastomosis on dentate line Endo-GIA stapler
Hirschsprung’s Disease laparoscopic Duhamel if stoma present – close at 2 weeks
Hirschpsrung’s Disease what results should be expected?
Hirschsprung’s disease Mishalany, Woolley (1987) 137 patients 62 reviewed follow up yrs (38 >5yrs)
Hirschpsrung’s disease Mishalany, Woolley (1987) Soave 33 Swenson 15 Duhamel 14
Hirschsprung’s disease Mishalany, Woolley (1987) 31 soiling/ incontinent Swenson worst 20 enterocolitis Duhamel least manometry abnormal in majority
Hirschsprung’s disease Catto-Smith et al (1995) 60 children (out of 87) 9 yrs post op
Hirschsprung’s disease Catto-Smith et al (1995) all Soave home diaries, questionnaires
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
Hirschsprung’s disease long term complications constipation incontinence enterocolitis strictures/ fistulae
Hirschsprung’s disease constipation 10-35% in all operations incontinence 0-50% all operations most reports <10%
Hirschsprung’s disease enterocolitis 0-34% all operations Swenson worst trans-anal now reporting 50%
Hirschsprung’s disease strictures/fistulae most series5-10% strictures <5% fistulae Swenson, Soave
Hirschsprung’s disease total colonic aganglionosis Escobar et al (2005) review 36 patients 19% died
Hirschsprung’s disease Escobar et al (2005) 81% continent highest morbidity Soave Martin modification ? Kimura patch useful
Hirschsprung’s disease Tsuji et al (1999) patients 6% mortality
Hirschsprung’s disease Tsuji et al (1999) 41 (85%) had pull through 38 Duhamel (13 Martin) 3 Soave 6 permanent stoma
Hirschsprung’s disease Tsuji et al (1999) incontinence at: yrs 82% 57% 33% Martin operation troublesome
Hirschsprung’s Disease 1983 – new patients 58 neonates 19 total colonic
Hirschsprung’s Disease major complications leak3 enterocolitis3 transit. pullthro2 deaths2
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
Hirschsprung’s disease recommend do the procedure which suits you much to recommend laparoscopy Duhamel- short pouch/low anastomosis
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