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Hirschsprung’s disease,Hirschsprung’s disease,the past and the presentthe past and the present
Dr.Dávidovics Sándor Dr.Dávidovics Sándor Petz A.County Teaching Hospital Petz A.County Teaching Hospital Department of Paediatric SurgeryDepartment of Paediatric Surgery
GyőrGyőr
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Hirschsprung’s diseaseHirschsprung’s disease
One in 5000 live births One in 5000 live births Male-to-female ratio of 4:1Male-to-female ratio of 4:1 In 90 % are mature newbornsIn 90 % are mature newborns In 1-6 % are familialIn 1-6 % are familial In 10%-30% are associated anomaliesIn 10%-30% are associated anomalies
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Hirschsprung’s diseaseHirschsprung’s disease
To rectosigmoid in 75%To rectosigmoid in 75% Short segment (rectum)Short segment (rectum) Ultra short (part of the rectum)Ultra short (part of the rectum) Long segment Long segment Total aganglionosis (large intestine, Total aganglionosis (large intestine,
sometimes a part of small intestine) up to sometimes a part of small intestine) up to 10%10%
SegmentalSegmental
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Hirschsprung’s diseaseHirschsprung’s disease
EmbriologyEmbriology Migration of neuroblasts on the 5-7 g.weekMigration of neuroblasts on the 5-7 g.week On the 12. g.week they reach the rectumOn the 12. g.week they reach the rectum Myenteric plexusMyenteric plexus Submucous plexusSubmucous plexus GanglionsGanglions The process of maturation continues after The process of maturation continues after
the birththe birth
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Hirschsprung’s diseaseHirschsprung’s disease
InnervationInnervation adrenergicadrenergic cholinergiccholinergic Nonadrenergic and noncholinergic Nonadrenergic and noncholinergic -pepdidergic-pepdidergic -nitrergic-nitrergic -nerve-supporting cells-nerve-supporting cells -interstitial cells in Cajal-interstitial cells in Cajal
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Hirschsprung’s diseaseHirschsprung’s disease
HistopathologyHistopathology Absence of ganglionic cellsAbsence of ganglionic cells Hypertrophic cholinergic nerve trunksHypertrophic cholinergic nerve trunks Limited number of adrenergic fiber Limited number of adrenergic fiber
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Hirschsprung’s diseaseHirschsprung’s disease
Clinical symptomsClinical symptoms The disease can considered to be The disease can considered to be
incomplete intestinal obstructionincomplete intestinal obstruction The lenght of the aganglionic segment is The lenght of the aganglionic segment is
variablevariable The symptoms are variable tooThe symptoms are variable too The symtoms appears in different agesThe symtoms appears in different ages
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Hirschsprung’s diseaseHirschsprung’s disease
Symptoms in newborn ageSymptoms in newborn age Fail to pass meconium (in 24 hours of life)Fail to pass meconium (in 24 hours of life) Abdominal distension, but the abdomen is Abdominal distension, but the abdomen is
palpablepalpable VomitingVomiting The rectal tube can’t be put easily The rectal tube can’t be put easily After irrigation the signs and symptoms After irrigation the signs and symptoms
return again in a few daysreturn again in a few days
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Hirschsprung’s diseaseHirschsprung’s disease
Symptoms in newborn age(enterocolitis)Symptoms in newborn age(enterocolitis) Life-threatening conditionLife-threatening condition Diarrhea: it can be an early signDiarrhea: it can be an early sign Toxic megacolonToxic megacolon Abdominal distensionAbdominal distension Bile-stained vomitingBile-stained vomiting Fiver and signs of dehydrationFiver and signs of dehydration Rectal tube:explosive expulsion of gas and foul-Rectal tube:explosive expulsion of gas and foul-
smelling stoolssmelling stools
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Hirschsprung’s diseaseHirschsprung’s disease
Symptoms in infantsSymptoms in infants ConstipationConstipation MeteorismMeteorism Palpable faecalomaPalpable faecaloma Sometimes putrescent diarrhea Sometimes putrescent diarrhea Ulceration, bleedingUlceration, bleeding Hypoproteinaemia, anaemiaHypoproteinaemia, anaemia Electrolyt disordersElectrolyt disorders
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Hirschsprung’s diseaseHirschsprung’s disease
Symptoms in childhoodSymptoms in childhood Gracile limbsGracile limbs Dilated drumlike bellyDilated drumlike belly Long history of constipationLong history of constipation Defecation in 7-10 days Defecation in 7-10 days Multiple fecal massesMultiple fecal masses The stimulus of defecation is missingThe stimulus of defecation is missing Rectum is empty and narrowRectum is empty and narrow
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Hirschsprung’s diseaseHirschsprung’s disease
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Hirschsprung’s diseaseHirschsprung’s disease
ExaminationsExaminations Plain abdominal radiographsPlain abdominal radiographs Contrast enemaContrast enema Anorectal manometryAnorectal manometry Rectal biopsy,histopathologyRectal biopsy,histopathology
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Hirschsprung’s diseaseHirschsprung’s disease
Plain X ray: air/fluid levels,free airPlain X ray: air/fluid levels,free air Contrast enema: Contrast enema:
-Narrow distal segment -Narrow distal segment -Funnel-shaped transition zone-Funnel-shaped transition zone-Dilation of proximal colon-Dilation of proximal colon-After 24-48 hours the contrast material-After 24-48 hours the contrast material
is in the bowelis in the bowel-Mucosal irregularity (enterocolitis)-Mucosal irregularity (enterocolitis)
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Hirschsprung’s diseaseHirschsprung’s disease
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Hirschsprung’s diseaseHirschsprung’s disease
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Hirschsprung’s diseaseHirschsprung’s disease
Anorectal manometryAnorectal manometry Anorectal pressure is elevated Anorectal pressure is elevated No relaxation of the internal sphincterNo relaxation of the internal sphincter It can be put through safely in newborn It can be put through safely in newborn
age as wellage as well
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Hirschsprung’s diseaseHirschsprung’s disease
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Hirschsprung’s diseaseHirschsprung’s disease
Biopsy and HistologyBiopsy and Histology
Full-thickness strip-biopsyFull-thickness strip-biopsy Suction biopsySuction biopsy
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Hirschsprung’s diseaseHirschsprung’s disease
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Hirschsprung’s diseaseHirschsprung’s disease
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Nitrergic innervation patternNitrergic innervation pattern(light microscopy, x100)(light microscopy, x100)
Normal colon Hirschsprung’s disease Normal colon Hirschsprung’s disease
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Nitrergic innervation patternNitrergic innervation pattern(light microscopy, x100)(light microscopy, x100)
Hirschsprung's disease Total intestinal aganglionosisHirschsprung's disease Total intestinal aganglionosis
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Hirschsprung’s diseaseHirschsprung’s disease
Treatment:Treatment: Decompression: introduce a rectal tube Decompression: introduce a rectal tube
and irrigationand irrigation ColostomyColostomy Definitive proceduresDefinitive procedures Closing of the stomaClosing of the stoma
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Hirschsprung’s diseaseHirschsprung’s disease
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Hirschsprung’s diseaseHirschsprung’s disease
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Hirschsprung’s diseaseHirschsprung’s disease
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Hirschsprung’s diseaseHirschsprung’s disease
TreatmentTreatmentTransanal Endorectal Pull-TroughTransanal Endorectal Pull-Trough It can be performed safely in infant as wellIt can be performed safely in infant as well Generally one-stage surgeryGenerally one-stage surgery No abdominal phaseNo abdominal phase The anastomosis is happening in a „safe” The anastomosis is happening in a „safe”
place at the pectinate lineplace at the pectinate line
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Hirschsprung’s diseaseHirschsprung’s disease
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Hirschsprung’s diseaseHirschsprung’s disease
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Hirschsprung’s diseaseHirschsprung’s disease
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Hirschsprung’s diseaseHirschsprung’s disease
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Hirschsprung’s diseaseHirschsprung’s disease
Long-term outcomeLong-term outcome If started in time, under adequate If started in time, under adequate
treatment is good.treatment is good.
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SummarySummary
PastPast Operation in 2 or 3 Operation in 2 or 3
stepssteps Pull-through on the Pull-through on the
afunctional bowelafunctional bowel Strip-biopsyStrip-biopsy Stoma-wearingStoma-wearing Nursing,dilatationsNursing,dilatations Higher possibility of Higher possibility of
complcationscomplcations
PresentPresent Operation in 1 stepOperation in 1 step- Pull-through the - Pull-through the
functional gutfunctional gut Suction biopsySuction biopsy No stomaNo stoma - No dilatation- No dilatation Smaller possibility of Smaller possibility of
complicationscomplications
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SummarySummary PastPast Long hospitalisationLong hospitalisation High input High input
/operations,medicines//operations,medicines/ Free stoma toolsFree stoma tools Travelling supportTravelling support Long-term financial Long-term financial
support of govermentsupport of goverment Harder social integration Harder social integration
/kidergarten,school//kidergarten,school/
PresentPresent Short hospitalisationShort hospitalisation Small inputSmall input
No stomaNo stoma Less controll examinationLess controll examination The support can be The support can be
extinguished earlierextinguished earlier Easy social integrationEasy social integration
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Thank you for your Thank you for your attention !attention !