hirschsprung’s disease, the past and the present dr.dávidovics sándor petz a.county teaching...

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

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


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


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


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


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


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


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


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


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


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


ExaminationsExaminations Plain abdominal radiographsPlain abdominal radiographs Contrast enemaContrast enema Anorectal manometryAnorectal manometry Rectal biopsy,histopathologyRectal biopsy,histopathology


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)


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


Biopsy and HistologyBiopsy and Histology

Full-thickness strip-biopsyFull-thickness strip-biopsy Suction biopsySuction biopsy

Nitrergic innervation patternNitrergic innervation pattern(light microscopy, x100)(light microscopy, x100)

Normal colon Hirschsprung’s disease Normal colon Hirschsprung’s disease

Nitrergic innervation patternNitrergic innervation pattern(light microscopy, x100)(light microscopy, x100)

Hirschsprung's disease Total intestinal aganglionosisHirschsprung's disease Total intestinal aganglionosis


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


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


Long-term outcomeLong-term outcome If started in time, under adequate If started in time, under adequate

treatment is good.treatment is good.

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

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

Thank you for your Thank you for your attention !attention !

hirschsprung’s disease, the past and the present dr.dávidovics sándor petz a.county teaching...

Documents

aganglionic segment

nitrergic nerve

newborn agefail

narrow distal segment

cells interstitial cells

proximal colon

variablethe symptoms

small intestine