Hirschsprungs disease, the past and the present

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Hirschsprungs disease,the past and the present

• Dr.Dávidovics Sándor
• Petz A.County Teaching Hospital Department of
  Paediatric Surgery
• Gyor

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Hirschsprungs disease

• One in 5000 live births
• Male-to-female ratio of 41
• In 90 are mature newborns
• In 1-6 are familial
• In 10-30 are associated anomalies

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Hirschsprungs disease

• To rectosigmoid in 75
• Short segment (rectum)
• Ultra short (part of the rectum)
• Long segment
• Total aganglionosis (large intestine, sometimes a
  part of small intestine) up to 10
• Segmental

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Hirschsprungs disease

• Embriology
• Migration of neuroblasts on the 5-7 g.week
• On the 12. g.week they reach the rectum
• Myenteric plexus
• Submucous plexus
• Ganglions
• The process of maturation continues after the
  birth

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Hirschsprungs disease

• Innervation
• adrenergic
• cholinergic
• Nonadrenergic and noncholinergic
• -pepdidergic
• -nitrergic
• -nerve-supporting cells
• -interstitial cells in Cajal

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Hirschsprungs disease

• Histopathology
• Absence of ganglionic cells
• Hypertrophic cholinergic nerve trunks
• Limited number of adrenergic fiber

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Hirschsprungs disease

• Clinical symptoms
• The disease can considered to be incomplete
  intestinal obstruction
• The lenght of the aganglionic segment is
  variable
• The symptoms are variable too
• The symtoms appears in different ages

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Hirschsprungs disease

• Symptoms in newborn age
• Fail to pass meconium (in 24 hours of life)
• Abdominal distension, but the abdomen is palpable
• Vomiting
• The rectal tube cant be put easily
• After irrigation the signs and symptoms return
  again in a few days

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Hirschsprungs disease

• Symptoms in newborn age(enterocolitis)
• Life-threatening condition
• Diarrhea it can be an early sign
• Toxic megacolon
• Abdominal distension
• Bile-stained vomiting
• Fiver and signs of dehydration
• Rectal tubeexplosive expulsion of gas and
  foul-smelling stools

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Hirschsprungs disease

• Symptoms in infants
• Constipation
• Meteorism
• Palpable faecaloma
• Sometimes putrescent diarrhea
• Ulceration, bleeding
• Hypoproteinaemia, anaemia
• Electrolyt disorders

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Hirschsprungs disease

• Symptoms in childhood
• Gracile limbs
• Dilated drumlike belly
• Long history of constipation
• Defecation in 7-10 days
• Multiple fecal masses
• The stimulus of defecation is missing
• Rectum is empty and narrow

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Hirschsprungs disease
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Hirschsprungs disease

• Examinations
• Plain abdominal radiographs
• Contrast enema
• Anorectal manometry
• Rectal biopsy,histopathology

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Hirschsprungs disease

• Plain X ray air/fluid levels,free air
• Contrast enema
• -Narrow distal segment
• -Funnel-shaped transition zone
• -Dilation of proximal colon
• -After 24-48 hours the contrast material
• is in the bowel
• -Mucosal irregularity (enterocolitis)

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Hirschsprungs disease
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Hirschsprungs disease
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Hirschsprungs disease

• Anorectal manometry
• Anorectal pressure is elevated
• No relaxation of the internal sphincter
• It can be put through safely in newborn age as
  well

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Hirschsprungs disease
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Hirschsprungs disease

• Biopsy and Histology
• Full-thickness strip-biopsy
• Suction biopsy

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Hirschsprungs disease
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Hirschsprungs disease
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Nitrergic innervation pattern(light microscopy,
x100)

• Normal colon Hirschsprungs
  disease

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Nitrergic innervation pattern(light microscopy,
x100)

• Hirschsprung's disease Total intestinal
  aganglionosis

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Hirschsprungs disease

• Treatment
• Decompression introduce a rectal tube and
  irrigation
• Colostomy
• Definitive procedures
• Closing of the stoma

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Hirschsprungs disease
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Hirschsprungs disease
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Hirschsprungs disease
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Hirschsprungs disease

• Treatment
• Transanal Endorectal Pull-Trough
• It can be performed safely in infant as well
• Generally one-stage surgery
• No abdominal phase
• The anastomosis is happening in a safe place at
  the pectinate line

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Hirschsprungs disease
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Hirschsprungs disease
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Hirschsprungs disease
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Hirschsprungs disease
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Hirschsprungs disease

• Long-term outcome
• If started in time, under adequate treatment is
  good.

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Summary

• Past
• Operation in 2 or 3 steps
• Pull-through on the afunctional bowel
• Strip-biopsy
• Stoma-wearing
• Nursing,dilatations
• Higher possibility of complcations

• Present
• Operation in 1 step
• - Pull-through the functional gut
• Suction biopsy
• No stoma
• - No dilatation
• Smaller possibility of complications

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Summary

• Past
• Long hospitalisation
• High input /operations,medicines/
• Free stoma tools
• Travelling support
• Long-term financial support of goverment
• Harder social integration /kidergarten,school/

• Present
• Short hospitalisation
• Small input
• No stoma
• Less controll examination
• The support can be extinguished earlier
• Easy social integration

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Thank you for your attention !