Hirschsprung's disease
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Hirschsprung's disease . Clinical picture , investigations , and management .
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Hirschsprung's disease
- 1. HirschsprungHirschsprung ‘‘s diseases disease Sameh ShehataSameh Shehata AlexandriaAlexandria EgyptEgypt 2014Sameh Shehata
- 2. IncidenceIncidence One in 5000 live birthsOne 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 2014Sameh Shehata
- 3. EmbryologyEmbryology 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 birthThe process of maturation continues after the birth 2014Sameh Shehata
- 6. ExtentExtent 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 segmentLong segment Total aganglionosis (large intestine, sometimes aTotal aganglionosis (large intestine, sometimes a part of small intestine) up to 10%part of small intestine) up to 10% 2014Sameh Shehata
- 9. Visible transitional zoneVisible transitional zone 2014Sameh Shehata
- 10. Total colon aganglionosisTotal colon aganglionosis 2014Sameh Shehata
- 13. Clinical symptomsClinical symptoms The disease can considered to be incompleteThe disease can considered to be incomplete colonic obstructioncolonic obstruction The lenght of the aganglionic segment is variableThe lenght of the aganglionic segment is variable The symptoms are variable tooThe symptoms are variable too The symtoms appears in different agesThe symtoms appears in different ages 2014Sameh Shehata
- 14. EnterocolitisEnterocolitis Life-threatening conditionLife-threatening condition Diarrhea: watery offensiveDiarrhea: watery offensive Abdominal distensionAbdominal distension Bile-stained vomitingBile-stained vomiting Fiver and signs of dehydrationFiver and signs of dehydration Rectal tube:explosive expulsion of gas andRectal tube:explosive expulsion of gas and foul-smelling stoolsfoul-smelling stools 2014Sameh Shehata
- 15. Age?Age? Neonate.Neonate. 70%70% InfantInfant 15%15% Child .Child . 8%8% Adolescent .Adolescent . 5 %5 % AdultAdult 2%2% 2014Sameh Shehata
- 16. Classical presentationClassical presentation Delayed passage of meconium .Delayed passage of meconium . Constipated.Constipated. Abdominal distension .Abdominal distension . Failure to thrive.Failure to thrive. Classical “enterocolitis”Classical “enterocolitis” 2014Sameh Shehata
- 17. Late presentationLate presentation Chronic constipation.Chronic constipation. Not responding .Not responding . Should suspect .Should suspect . Do barium enema .Do barium enema . 2014Sameh Shehata
- 18. Symptoms in newbornSymptoms in newborn ageage Fail to pass meconium (in 24 hours ofFail to pass meconium (in 24 hours of life)life) Abdominal distension,Abdominal distension, VomitingVomiting After irrigation the signs andAfter irrigation the signs and symptoms return again in a few dayssymptoms return again in a few days 2014Sameh Shehata
- 19. Symptoms in infantsSymptoms in infants ConstipationConstipation Palpable faecalomaPalpable faecaloma Sometimes putrescent diarrheaSometimes putrescent diarrhea Hypoproteinaemia, anaemiaHypoproteinaemia, anaemia Electrolyt disordersElectrolyt disorders 2014Sameh Shehata
- 20. Symptoms in childhoodSymptoms in childhood Dilated drumlike bellyDilated drumlike belly Long history of constipationLong history of constipation Defecation in 7-10 daysDefecation in 7-10 days Multiple fecal massesMultiple fecal masses 2014Sameh Shehata
- 26. InvestigationsInvestigations Contrast enemaContrast enema Anorectal manometryAnorectal manometry Rectal biopsy,histopathologyRectal biopsy,histopathology 2014Sameh Shehata
- 27. 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) 2014Sameh Shehata
- 29. Barium ContrastBarium Contrast Diagnostic and accurate in 97Diagnostic and accurate in 97 patients (86.3%).patients (86.3%). 2014Sameh Shehata
- 32. Long segmentLong segment 2014Sameh Shehata
- 33. Anorectal manometryAnorectal manometry Anorectal pressure is elevatedAnorectal pressure is elevated No relaxation of the internal sphincterNo relaxation of the internal sphincter It can be put through safely in newbornIt can be put through safely in newborn age as wellage as well 2014Sameh Shehata
- 37. Biopsy and HistologyBiopsy and Histology Full-thickness strip-biopsyFull-thickness strip-biopsy Suction biopsySuction biopsy 2014Sameh Shehata
- 40. HistopathologyHistopathology Absence of ganglionic cellsAbsence of ganglionic cells Hypertrophic cholinergic nerveHypertrophic cholinergic nerve trunkstrunks Limited number of adrenergic fiberLimited number of adrenergic fiber 2014Sameh Shehata
- 43. Histopathology ofHistopathology of Hirschsprung diseaseHirschsprung disease . Enzyme histochemistry showing aberrant acetylcholine esterase. Enzyme histochemistry showing aberrant acetylcholine esterase (AChE)-positive fibres (brown) in the lamina propria mucosae.(AChE)-positive fibres (brown) in the lamina propria mucosae. 2014Sameh Shehata
- 44. Aim of surgeryAim of surgery RResect aganglionic colon .esect aganglionic colon . RResect dilated segmentesect dilated segment above .???above .??? RRestore bowel continuityestore bowel continuity .. 2014Sameh Shehata
- 45. DDecompressionecompression SSuppositoriesuppositories rectal tuberectal tube ColostomyColostomy 2014Sameh Shehata
- 46. StagesStages Three .Three . Two .Two . One .One . 2014Sameh Shehata
- 47. StagesStages Three .Three . Two .Two . One .One . 2014Sameh Shehata
- 48. TechniqueTechnique Swenson’sSwenson’s Soave .Soave . Duhamel .Duhamel . 2014Sameh Shehata
- 49. ApproachApproach Abdominal /transanal .Abdominal /transanal . Lap/transanalLap/transanal Transanal .Transanal . 2014Sameh Shehata
- 50. RecentRecent One stage .One stage . Transanal .Transanal . 2014Sameh Shehata
- 52. Colostomy ?Colostomy ? 2014Sameh Shehata
- 58. The retractorThe retractor 2014Sameh Shehata
- 59. Placement of the tractionPlacement of the traction stitchesstitches 2014Sameh Shehata
- 60. Clear Transitional ZoneClear Transitional Zone 2014Sameh Shehata
- 61. Segment ResectedSegment Resected 23 cm to 53 cm (38cm)23 cm to 53 cm (38cm) 2014Sameh Shehata
- 64. Transanal 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 „safeThe anastomosis is happening in a „safe”” place at the pectinate lineplace at the pectinate line 2014Sameh Shehata
- 65. Transanal Endorectal Pull-TroughTransanal Endorectal Pull-Trough 2014Sameh Shehata
- 66. Transanal Endorectal Pull-TroughTransanal Endorectal Pull-Trough 2014Sameh Shehata
- 67. Transanal Endorectal Pull-TroughTransanal Endorectal Pull-Trough 2014Sameh Shehata
- 68. Transanal Endorectal Pull-TroughTransanal Endorectal Pull-Trough 2014Sameh Shehata
- 69. Thank youThank you 2014Sameh Shehata
- 70. ReferencesReferences Dr.Dávidovics SándorDr.Dávidovics Sándor Petz A.County Teaching HospitalPetz A.County Teaching Hospital Department of Paediatric SurgeryDepartment of Paediatric Surgery GyőrGyőr 2014Sameh Shehata