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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. 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

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  1. Hirschsprung’s disease,the past and the present Dr.Dávidovics Sándor Petz A.County Teaching Hospital Department of Paediatric Surgery Győr

  2. 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

  3. 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

  4. 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

  5. Hirschsprung’s disease • Innervation • adrenergic • cholinergic • Nonadrenergic and noncholinergic -pepdidergic -nitrergic -nerve-supporting cells -interstitial cells in Cajal

  6. Hirschsprung’s disease Histopathology • Absence of ganglionic cells • Hypertrophic cholinergic nerve trunks • Limited number of adrenergic fiber

  7. 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

  8. 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

  9. 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

  10. Hirschsprung’s disease Symptoms in infants • Constipation • Meteorism • Palpable faecaloma • Sometimes putrescent diarrhea • Ulceration, bleeding • Hypoproteinaemia, anaemia • Electrolyt disorders

  11. 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

  12. Hirschsprung’s disease

  13. Hirschsprung’s disease Examinations • Plain abdominal radiographs • Contrast enema • Anorectal manometry • Rectal biopsy,histopathology

  14. 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)

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  17. 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

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  19. Hirschsprung’s disease Biopsy and Histology • Full-thickness strip-biopsy • Suction biopsy

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  22. Nitrergic innervation pattern(light microscopy, x100) Normal colon Hirschsprung’s disease

  23. Nitrergic innervation pattern(light microscopy, x100) Hirschsprung's disease Total intestinal aganglionosis

  24. Hirschsprung’s disease Treatment: • Decompression: introduce a rectal tube and irrigation • Colostomy • Definitive procedures • Closing of the stoma

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  28. 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

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  33. Hirschsprung’s disease Long-term outcome • If started in time, under adequate treatment is good.

  34. 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

  35. 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

  36. Thank you for your attention !

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