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 Soave enterocolitis dribbling incontinence Swenson stricture Duhamel faecaloma

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

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

Hirschsprung’s Disease what type of stoma? <1yr loop skin bridge >1yr double 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 ports RIF 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 1- 30 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 series 5-10% strictures <5% fistulae Swenson, Soave

Hirschsprung’s disease total colonic aganglionosis Escobar et al (2005) 1972-2004 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) 1980-1996 48 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: 5 10 15 yrs 82% 57% 33% Martin operation troublesome

Hirschsprung’s Disease 1983 – 2010 138 new patients 58 neonates 19 total colonic

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

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