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Hirschsprung’s disease, the past and the present. Dr.Dávidovics Sándor Petz A.County Teaching Hospital Department of Paediatric Surgery Győr. Hirschsprung’s disease. One in 5000 live births Male-to-female ratio of 4:1 In 90 % are mature newborns In 1-6 % are familial

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hirschsprung s disease the past and the present

Hirschsprung’s disease,the past and the present

Dr.Dávidovics Sándor

Petz A.County Teaching Hospital Department of Paediatric Surgery

Győr

hirschsprung s disease
Hirschsprung’s disease
  • One in 5000 live births
  • Male-to-female ratio of 4:1
  • In 90 % are mature newborns
  • In 1-6 % are familial
  • In 10%-30% are associated anomalies
hirschsprung s disease3
Hirschsprung’s 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
hirschsprung s disease4
Hirschsprung’s 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
hirschsprung s disease5
Hirschsprung’s disease
  • Innervation
  • adrenergic
  • cholinergic
  • Nonadrenergic and noncholinergic

-pepdidergic

-nitrergic

-nerve-supporting cells

-interstitial cells in Cajal

hirschsprung s disease6
Hirschsprung’s disease

Histopathology

  • Absence of ganglionic cells
  • Hypertrophic cholinergic nerve trunks
  • Limited number of adrenergic fiber
hirschsprung s disease7
Hirschsprung’s 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
hirschsprung s disease8
Hirschsprung’s 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 can’t be put easily
  • After irrigation the signs and symptoms return again in a few days
hirschsprung s disease9
Hirschsprung’s 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 tube:explosive expulsion of gas and foul-smelling stools
hirschsprung s disease10
Hirschsprung’s disease

Symptoms in infants

  • Constipation
  • Meteorism
  • Palpable faecaloma
  • Sometimes putrescent diarrhea
  • Ulceration, bleeding
  • Hypoproteinaemia, anaemia
  • Electrolyt disorders
hirschsprung s disease11
Hirschsprung’s 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
hirschsprung s disease13
Hirschsprung’s disease

Examinations

  • Plain abdominal radiographs
  • Contrast enema
  • Anorectal manometry
  • Rectal biopsy,histopathology
hirschsprung s disease14
Hirschsprung’s 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)

hirschsprung s disease17
Hirschsprung’s disease

Anorectal manometry

  • Anorectal pressure is elevated
  • No relaxation of the internal sphincter
  • It can be put through safely in newborn age as well
hirschsprung s disease19
Hirschsprung’s disease

Biopsy and Histology

  • Full-thickness strip-biopsy
  • Suction biopsy
nitrergic innervation pattern light microscopy x100

Nitrergic innervation pattern(light microscopy, x100)

Normal colon Hirschsprung’s disease

nitrergic innervation pattern light microscopy x10023

Nitrergic innervation pattern(light microscopy, x100)

Hirschsprung's disease Total intestinal aganglionosis

hirschsprung s disease24
Hirschsprung’s disease

Treatment:

  • Decompression: introduce a rectal tube and irrigation
  • Colostomy
  • Definitive procedures
  • Closing of the stoma
hirschsprung s disease28
Hirschsprung’s 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
hirschsprung s disease33
Hirschsprung’s disease

Long-term outcome

  • If started in time, under adequate treatment is good.
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

Summary
summary35
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

Summary