Hirschsprung's Disease an approach to management

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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
  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
                 - stoma
   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

				
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posted:2/28/2011
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