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Case presentation (ileal atreasia)

EXAMPLE OF NEONATAL INTESINAL OBSTRUCTION

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Case presentation (ileal atreasia)

  1. 1. Case presentationCase presentation Ileal atresiaIleal atresia Dr.Ashraf HamedDr.Ashraf Hamed Consultant pedia surgeryConsultant pedia surgery
  2. 2. 19-8-24(3pm)19-8-24(3pm)  Full term male baby,9daysFull term male baby,9days old,philipino,born to a motherold,philipino,born to a mother para 1+0 by SVD(nursing staff inpara 1+0 by SVD(nursing staff in B.C.H)B.C.H)  Presented with abdominalPresented with abdominal distention ,vomting of fecaldistention ,vomting of fecal matter and constipation since 6matter and constipation since 6 day.day.
  3. 3. On examinationOn examination  The baby looked ill,lethergicThe baby looked ill,lethergic ,dehydrated with poor,dehydrated with poor perfusion.perfusion.  B.W: 2.7 kgB.W: 2.7 kg  NGT:big amount of fecal matterNGT:big amount of fecal matter
  4. 4. Abdominal examAbdominal exam  Sever abdominal distntion withSever abdominal distntion with erythema of ant. Abd. Wall.erythema of ant. Abd. Wall.  Visible peristaltic waves.Visible peristaltic waves.  Tenderness allover the abd.Tenderness allover the abd.  Absent bowel soundsAbsent bowel sounds  PR.empty rectumPR.empty rectum
  5. 5. Plain x- rayPlain x- ray  Mutiple air fluid levelsMutiple air fluid levels  no pneumoperitoneumno pneumoperitoneum  No contrast studyNo contrast study  The baby shifted to OTThe baby shifted to OT atat 10pm after full stabilization and10pm after full stabilization and resuscitationresuscitation
  6. 6. Operative findingsOperative findings  We found ileal artesia type 3 atWe found ileal artesia type 3 at distal ileum,about 20 cm from ileo-distal ileum,about 20 cm from ileo- cecal valvececal valve  The distal ileum and colon wereThe distal ileum and colon were small and attenuated.small and attenuated.  The proximal bind-ended ileum wasThe proximal bind-ended ileum was hugely dilated ,hypertrophic andhugely dilated ,hypertrophic and oedematousoedematous  There was a mesenteric gapThere was a mesenteric gap
  7. 7. detailsdetails  Excision of both blind ends withExcision of both blind ends with performance of primary end to endperformance of primary end to end anastomosis in two layers with 5/0 vicrylanastomosis in two layers with 5/0 vicryl  The baby passed stool at 3The baby passed stool at 3rdrd post-op daypost-op day  NGT feeding started at 4NGT feeding started at 4thth post-op day(24-8-post-op day(24-8- 24)24)  The wound was clean and stitchesThe wound was clean and stitches removed on 10removed on 10thth post-op daypost-op day  The baby discharged in good G.C withThe baby discharged in good G.C with BW3.1kg at30-8-24BW3.1kg at30-8-24
  8. 8. Neonatal IntestinalNeonatal Intestinal ObstructionObstruction DR.ASHRAF HAMEDDR.ASHRAF HAMED CONS.PEDIA SUREGRYCONS.PEDIA SUREGRY
  9. 9. IntroductionIntroduction  Gastro intestinal obstruction during theGastro intestinal obstruction during the neonatal period classically presents withneonatal period classically presents with a triad of symptoms and signs i.e.a triad of symptoms and signs i.e. feeding difficulties, abdominalfeeding difficulties, abdominal distension and a change in the stoolingdistension and a change in the stooling patternpattern
  10. 10.  Bilious vomiting in newborns is anBilious vomiting in newborns is an urgent condition that requires theurgent condition that requires the immediate involvement of a team ofimmediate involvement of a team of pediatric surgeons andpediatric surgeons and neonatologists for perioperativeneonatologists for perioperative managementmanagement  Bilious vomiting, with or withoutBilious vomiting, with or without abdominal distention, is an initialabdominal distention, is an initial sign of intestinal obstruction insign of intestinal obstruction in newbornsnewborns  WWhen a neonate develops bilioushen a neonate develops bilious vomiting, one should suspect avomiting, one should suspect a surgical conditionsurgical condition
  11. 11.  It is important to keep in mind thatIt is important to keep in mind that extra gastrointestinal conditions such asextra gastrointestinal conditions such as neonatal sepsis, birth trauma,neonatal sepsis, birth trauma, prematurity and transplacental crossingprematurity and transplacental crossing of maternal medication can closelyof maternal medication can closely mimic mechanical intestinal obstructionmimic mechanical intestinal obstruction in the newbornin the newborn
  12. 12. INCIDENCEINCIDENCE  Neonatal intestinal obstruction occurs inNeonatal intestinal obstruction occurs in approximately l/2000 live births. It mayapproximately l/2000 live births. It may result from:result from:  intrinsic developmental defects arisingintrinsic developmental defects arising from disordered embryogenesisfrom disordered embryogenesis  abnormalities of peristalsis and/orabnormalities of peristalsis and/or intestinal contentsintestinal contents  a secondary insult acquired in utero,a secondary insult acquired in utero, after normal intestinal development.after normal intestinal development.
  13. 13. Development of the intestinal tractDevelopment of the intestinal tract  Active growth of the mid-gut andActive growth of the mid-gut and hindgut begins during the 5th -10thhindgut begins during the 5th -10th week. The mid-gut bends ventrally toweek. The mid-gut bends ventrally to form a U-loop, with the yolk sacform a U-loop, with the yolk sac attached at its apex: the superiorattached at its apex: the superior mesenteric artery (SMA) forms themesenteric artery (SMA) forms the axis of this loop and extends to theaxis of this loop and extends to the yolk sac. Rapid mid-gut growth forcesyolk sac. Rapid mid-gut growth forces part of the loop into the umbilicalpart of the loop into the umbilical cord, forming a temporarycord, forming a temporary physiological herniaphysiological hernia
  14. 14. Rotation of the mid-gutRotation of the mid-gut  Frist StageFrist Stage While the developing mid-gut and hindgut areWhile the developing mid-gut and hindgut are lying in the physiological hernia (5th-8thlying in the physiological hernia (5th-8th week), there is a rotation of 90” anticlockwise,week), there is a rotation of 90” anticlockwise, when viewed from the front, which may bewhen viewed from the front, which may be caused by the developing liver. A further 90”caused by the developing liver. A further 90” rotation occurs before the mid-gut moves outrotation occurs before the mid-gut moves out of the umbilical herniaof the umbilical hernia
  15. 15.  Second stage:Second stage: The bowel returns to the abdominalThe bowel returns to the abdominal cavity (10th week). The caecum andcavity (10th week). The caecum and ascending colon reduce last, and come toascending colon reduce last, and come to lie in the right upper quadrant.lie in the right upper quadrant. Subsequent growth of the colon pushesSubsequent growth of the colon pushes the caecum into the right loin; thisthe caecum into the right loin; this further 90” anticlockwise rotation, resultsfurther 90” anticlockwise rotation, results in a total rotation of the mid-gut of 270”in a total rotation of the mid-gut of 270” about the axis of the SMA.about the axis of the SMA.  Third stage:Third stage: The caecum descends to the right iliacThe caecum descends to the right iliac fossa (11 weeks to birth).fossa (11 weeks to birth).
  16. 16.  Fourth stage:Fourth stage: The ascending and descending colonThe ascending and descending colon and the 2nd, 3rd and 4th parts of theand the 2nd, 3rd and 4th parts of the duodenum become fixed to theduodenum become fixed to the posterior abdominal wall. The terminalposterior abdominal wall. The terminal ileum has followed the caecum intoileum has followed the caecum into the right lower abdomen; therefore,the right lower abdomen; therefore, its mesentery attachment is aits mesentery attachment is a diagonal line from the Treitz ligamentdiagonal line from the Treitz ligament to the right iliac fossa. The broad baseto the right iliac fossa. The broad base of the small bowel mesentery providesof the small bowel mesentery provides positional stability.positional stability.
  17. 17. Malrotation and midgutMalrotation and midgut volvulusvolvulus  The incidence of malrotation is aboutThe incidence of malrotation is about l/600 live births. Most errors occur duringl/600 live births. Most errors occur during the second phase of rotation. The 1st andthe second phase of rotation. The 1st and 2nd parts of the duodenum are situated2nd parts of the duodenum are situated normallnormallyy, but the 3rd and 4th parts, but the 3rd and 4th parts descend vertically along the right sidedescend vertically along the right side and the small bowel lies predominantly toand the small bowel lies predominantly to the right of the mid-line. The caecum liesthe right of the mid-line. The caecum lies centrally and the colon, doubled on itself,centrally and the colon, doubled on itself, is on the left side of the abdomenis on the left side of the abdomen
  18. 18. Malrotation and midgutMalrotation and midgut volvulusvolvulus 
  19. 19. 1-81-8  Failure of fixation often occurs. AlthoughFailure of fixation often occurs. Although thickened peritoneal attachments (Ladd’sthickened peritoneal attachments (Ladd’s bands) may pass from the caecum across thebands) may pass from the caecum across the duodenum to the right upper quadrantduodenum to the right upper quadrant posterior abdominal wall, these seldom causeposterior abdominal wall, these seldom cause obstruction. The mid-gut segment isobstruction. The mid-gut segment is predisposed to volvulus because, as a resultpredisposed to volvulus because, as a result of the failure of fixation, the entire mid-gut isof the failure of fixation, the entire mid-gut is suspended in the abdominal cavity by asuspended in the abdominal cavity by a narrow pediclenarrow pedicle
  20. 20. DiagnosisDiagnosis  Volvulus almost always occurs in aVolvulus almost always occurs in a clockwise directionclockwise direction  Abdominal signs other than epigastricAbdominal signs other than epigastric distension are absent initially, anddistension are absent initially, and become obvious only once intestinalbecome obvious only once intestinal strangulation has occurred. The onlystrangulation has occurred. The only evidence may be a history of bile-evidence may be a history of bile- stained vomiting, which is oftenstained vomiting, which is often intermittent.intermittent.
  21. 21. 1-101-10  Contrast enema; the caecum is oftenContrast enema; the caecum is often ‘high’ in neonates and may not be taken‘high’ in neonates and may not be taken up in the volvulus.up in the volvulus.  A barium meal excludes or confirms theA barium meal excludes or confirms the diagnosis.diagnosis.
  22. 22. ManagementManagement  At surgery, the bowel is untwistedAt surgery, the bowel is untwisted anticlockwise, Ladd’s bands and inter-loopanticlockwise, Ladd’s bands and inter-loop adhesions are divided, and the distaladhesions are divided, and the distal duodenum is mobilized to the right, thusduodenum is mobilized to the right, thus broadening the base of the mesentery.broadening the base of the mesentery. Patency of the lumen is checked. The bowelPatency of the lumen is checked. The bowel is then replaced in the abdomen in a non-is then replaced in the abdomen in a non- rotated position. It is unnecessary to fix therotated position. It is unnecessary to fix the bowel. The appendix may be removedbowel. The appendix may be removed
  23. 23. Intestinal atresia and stenosisIntestinal atresia and stenosis  The term ‘atresia’ denotes complete intrinsicThe term ‘atresia’ denotes complete intrinsic occlusion of the intestinal lumen as a result ofocclusion of the intestinal lumen as a result of anomalous development of the intestinal walls.anomalous development of the intestinal walls. The term ‘stenosis’ refers to narrowing of theThe term ‘stenosis’ refers to narrowing of the lumen which causes incomplete obstruction.lumen which causes incomplete obstruction. Approximately 30% of infants presenting withApproximately 30% of infants presenting with neonatal intestinal obstruction have atresia orneonatal intestinal obstruction have atresia or stenosis. Atretic lesions are distributed as follows:stenosis. Atretic lesions are distributed as follows:  duodenum - 45%duodenum - 45%  jejunum - 25%jejunum - 25%  ileum - 15%ileum - 15%  colon - 5%colon - 5%  multiple - 10%multiple - 10%
  24. 24. Pathogenesis:Pathogenesis:  Figure 2Figure 2 Figure 2
  25. 25. Classification of intestinalClassification of intestinal atresiaatresia  StenosisStenosis There is a localised narrowing of the bowel orThere is a localised narrowing of the bowel or a diaphragm (membrane) with a smalla diaphragm (membrane) with a small perforation.perforation.  Atresia type 1Atresia type 1 This is characterised by a thin mucosalThis is characterised by a thin mucosal diaphragm. The bowel is normal in length.diaphragm. The bowel is normal in length.  Atresia type 2Atresia type 2 This characterised by blind ends joined by aThis characterised by blind ends joined by a band. There is seldom a correspondingband. There is seldom a corresponding defect in the mesentery: when this occurs, thedefect in the mesentery: when this occurs, the length of bowel may be subnormallength of bowel may be subnormal
  26. 26.  Atresia type 3Atresia type 3 This is characterised by disconnected blindThis is characterised by disconnected blind ends with a gap in an otherwise normalends with a gap in an otherwise normal mesentery, and often considerably shortenedmesentery, and often considerably shortened small bowel. Type 3 atresia is divided into 2small bowel. Type 3 atresia is divided into 2 subtypessubtypes  Atresia type 4Atresia type 4 Multiple intestinal occlusions occur. ThereMultiple intestinal occlusions occur. There may be 2 or more atresias or stenoses of themay be 2 or more atresias or stenoses of the same or different types, and considerablesame or different types, and considerable shortening of the bowel. Type 4 lesions mayshortening of the bowel. Type 4 lesions may result from intermittent torsion or placentalresult from intermittent torsion or placental emboli. Familial types have also beenemboli. Familial types have also been describeddescribed
  27. 27. 1-1`41-1`4  Associated anomaliesAssociated anomalies Prematurity, growth retardation and co-Prematurity, growth retardation and co- existent malformation are common,existent malformation are common, particularly in patients with duodenal atresias.particularly in patients with duodenal atresias. Almost 50% of duodenal atresias areAlmost 50% of duodenal atresias are associated with some other anomaly (e.g.associated with some other anomaly (e.g. cardiac, genitourinary or anorectal), and up tocardiac, genitourinary or anorectal), and up to 30% of such patients have Trisomy 21. Less30% of such patients have Trisomy 21. Less than 10% of infants with jejuno-ileal atresia,than 10% of infants with jejuno-ileal atresia, however, suffer from coexistent extra-however, suffer from coexistent extra- abdominal malformations.abdominal malformations.
  28. 28. Duodenal atresiaDuodenal atresia  The lumen of the duodenum passes throughThe lumen of the duodenum passes through a solid phase between the 5th and 8th weeksa solid phase between the 5th and 8th weeks of embryonic life in about 30% of fetusesof embryonic life in about 30% of fetuses  Failure of recanalization could, theoretically,Failure of recanalization could, theoretically, cause duodenal atresias.cause duodenal atresias.  Most duodenal atresias occur in the midMost duodenal atresias occur in the mid anatomical 2nd part of the duodenum.anatomical 2nd part of the duodenum. Several types are recognised.Several types are recognised.
  29. 29. 1-141-14  Proximal and distal segments may endProximal and distal segments may end blindly and adjacent or separated by ablindly and adjacent or separated by a gap or fibrous cord which is usuallygap or fibrous cord which is usually ‘filled-in’with pancreatic tissue.‘filled-in’with pancreatic tissue.  A diaphragm, often with a smallA diaphragm, often with a small perforation, is not uncommon; multipleperforation, is not uncommon; multiple diaphragms seldom occur. A thindiaphragms seldom occur. A thin diaphragm which has ballooned distallydiaphragm which has ballooned distally is known as a ‘windsock’is known as a ‘windsock’
  30. 30. 1-151-15  The distal biliary tree is often abnormal:The distal biliary tree is often abnormal: it usually opens at the level of theit usually opens at the level of the diaphragm, via a T-shaped, L-shapeddiaphragm, via a T-shaped, L-shaped or even Y-shaped insertion proximalor even Y-shaped insertion proximal and/or distal to the atresia.and/or distal to the atresia.  Almost 50% of duodenal atresias areAlmost 50% of duodenal atresias are associated with some other anomalyassociated with some other anomaly (e.g. cardiac, genitourinary or(e.g. cardiac, genitourinary or anorectal),anorectal),
  31. 31. DiagnosisDiagnosis  The diagnosis of intestinal obstruction may beThe diagnosis of intestinal obstruction may be suspected during antenatal ultrasoundsuspected during antenatal ultrasound examination. Polyhydramnios may be anexamination. Polyhydramnios may be an indication of high intestinal obstructionindication of high intestinal obstruction  Symptoms and signs of intestinal obstructionSymptoms and signs of intestinal obstruction – vomiting (clear, or bile-stained if obstruction– vomiting (clear, or bile-stained if obstruction occurs distal to Vater’s ampullaoccurs distal to Vater’s ampulla  diagnostic contrast enema is an essential aid,diagnostic contrast enema is an essential aid, therefore, in defining the level and cause oftherefore, in defining the level and cause of more distal obstructions.more distal obstructions.
  32. 32. ManagementManagement  Laparotomy via a transverse abdominalLaparotomy via a transverse abdominal incision above the umbilicus is preferred. Inincision above the umbilicus is preferred. In duodenal obstructions, the atretic area isduodenal obstructions, the atretic area is exposed by mobilizing the ascending andexposed by mobilizing the ascending and transverse colon to the left. In all types oftransverse colon to the left. In all types of obstruction, including stenosis andobstruction, including stenosis and fenestrated diaphragms, the most widelyfenestrated diaphragms, the most widely performed operation is duodeno-performed operation is duodeno- duodenostomy – either end-to-side or aduodenostomy – either end-to-side or a diamond-shaped anastomosis usingdiamond-shaped anastomosis using interrupted fine absorbable sutures.interrupted fine absorbable sutures.
  33. 33. 1-171-17  The technique of neonatal bowelThe technique of neonatal bowel anastomosis i.e. end-toend or end-to-anastomosis i.e. end-toend or end-to- back if lumenal discrepancy is presentback if lumenal discrepancy is present  Anastomosis of dilated bowel andAnastomosis of dilated bowel and collapsed bowel distal to the origin of acollapsed bowel distal to the origin of a ‘windsock’ diaphragm is a well-‘windsock’ diaphragm is a well- described pitfall.described pitfall.
  34. 34. 1-181-18  A primary, one layer end-to-end or modifiedA primary, one layer end-to-end or modified end-to-back anastomosis is used in mostend-to-back anastomosis is used in most patients. Stomas are fashioned only if there ispatients. Stomas are fashioned only if there is gross peritonitis. Tapering of the dilatedgross peritonitis. Tapering of the dilated proximal bowel rather than resection isproximal bowel rather than resection is performed when total bowel length isperformed when total bowel length is deficient. Dilated and hypertrophied boweldeficient. Dilated and hypertrophied bowel which remains above the atresia will bewhich remains above the atresia will be unable to provide effective peristalsis if left inunable to provide effective peristalsis if left in situ.situ.
  35. 35. Meconium ileusMeconium ileus  In meconium ileus, excessively viscousIn meconium ileus, excessively viscous mucus secretion causes the meconium tomucus secretion causes the meconium to stick to the intestinal wall and in l0-15% ofstick to the intestinal wall and in l0-15% of newborn infants with cystic fibrosis, this leadsnewborn infants with cystic fibrosis, this leads to acute intestinal obstruction.to acute intestinal obstruction.  The distal ileum is small and contracted, andThe distal ileum is small and contracted, and contains firm, greyish, pebble-like secretionscontains firm, greyish, pebble-like secretions of meconium.of meconium.  The mid-ileum becomes dilated andThe mid-ileum becomes dilated and hypertrophied, and contains greyish-blackhypertrophied, and contains greyish-black material with a thick, tar-like consistencymaterial with a thick, tar-like consistency
  36. 36. Diagnosis of meconium ileusDiagnosis of meconium ileus  Visible abdominal peristalsis and palpableVisible abdominal peristalsis and palpable bowel loops are evident. Plain radiographs ofbowel loops are evident. Plain radiographs of the abdomen reveal multiple dilated loops ofthe abdomen reveal multiple dilated loops of bowel of varying diameter, but erectbowel of varying diameter, but erect radiographs show a paucity of fluid levels.radiographs show a paucity of fluid levels. The meconium-filled bowel gives a ‘groundThe meconium-filled bowel gives a ‘ground glass’ appearance, and contrast enemaglass’ appearance, and contrast enema shows a minute, ribbon-like colon withshows a minute, ribbon-like colon with meconium pelletsmeconium pellets
  37. 37. Meconium ileusMeconium ileus  ManagementManagement Uncomplicated meconium ileus is effectivelyUncomplicated meconium ileus is effectively treated, in most patients, by gastrografintreated, in most patients, by gastrografin enema. Gastrografin is a contrast mediumenema. Gastrografin is a contrast medium that is hygroscopic, stimulates bowelthat is hygroscopic, stimulates bowel peristalsis, and has a detergent effect.peristalsis, and has a detergent effect. Adequate intravenous fluid should be given,Adequate intravenous fluid should be given, to compensate for intraluminal losses,to compensate for intraluminal losses, because gastro-grafin is hyperosmolarbecause gastro-grafin is hyperosmolar
  38. 38. 1-231-23  When obstruction persists after gastrografinWhen obstruction persists after gastrografin enema, or when complications are evident,enema, or when complications are evident, surgical exploration is necessarysurgical exploration is necessary  In patients with complicated meconium ileus,In patients with complicated meconium ileus, resection and stoma formation is theresection and stoma formation is the preferred treatment; this is either double-preferred treatment; this is either double- barrelled, or the BishopKoop type (barrelled, or the BishopKoop type (seesee
  39. 39. 1-231-23  8Figure 98Figure 9 8 Figure 9
  40. 40. Meconium plug syndromeMeconium plug syndrome  DiagnosisDiagnosis Meconium plug syndrome typically presentsMeconium plug syndrome typically presents as distal obstruction, caused by a cast or plugas distal obstruction, caused by a cast or plug of inspissated meconium in the distal colonof inspissated meconium in the distal colon and rectum. It may occur in infants of diabeticand rectum. It may occur in infants of diabetic mothers, premature infants with intestinalmothers, premature infants with intestinal dysmotility, hypothyroid babies. and infants ofdysmotility, hypothyroid babies. and infants of mothers receiving ganglion blocking agentsmothers receiving ganglion blocking agents or sedatives.or sedatives.
  41. 41. ManagementManagement  Most infants respond to rectal washoutsMost infants respond to rectal washouts with half-strength gastrografin, followingwith half-strength gastrografin, following diagnostic contrast enema.diagnostic contrast enema. Decompression may fail; urgentDecompression may fail; urgent colostomy proximal to the obstructioncolostomy proximal to the obstruction then becomes necessary.then becomes necessary. Hirschsprung’s disease and cysticHirschsprung’s disease and cystic fibrosis must be excludedfibrosis must be excluded
  42. 42. Hirschsprung’s diseaseHirschsprung’s disease  The cause of Hirschsprung’s disease isThe cause of Hirschsprung’s disease is unknown, but failure of completely successfulunknown, but failure of completely successful migration and maturation of the entericmigration and maturation of the enteric ganglion cells, which develop fromganglion cells, which develop from neuroblasts derived from the neural crest, isneuroblasts derived from the neural crest, is most likely. These cells normally migratemost likely. These cells normally migrate caudally along the path of the vagus nervecaudally along the path of the vagus nerve from the 6th week and reach the rectum byfrom the 6th week and reach the rectum by the 12th week of developmentthe 12th week of development
  43. 43.  The incidence is l/5000 live births.The incidence is l/5000 live births. Rectosigmoid disease is more commonRectosigmoid disease is more common in males than females. Familialin males than females. Familial occurrence is more common in females,occurrence is more common in females, and where extended segments of boweland where extended segments of bowel are involved. There are few associatedare involved. There are few associated anomalies; congenital heart diseaseanomalies; congenital heart disease and Down’s syndrome occur in 5% ofand Down’s syndrome occur in 5% of patients.patients.
  44. 44.  In 70% of cases, only the rectosigmoid isIn 70% of cases, only the rectosigmoid is affected, but aganglionosis may extend moreaffected, but aganglionosis may extend more proximally. Affecting the left colon (20% ofproximally. Affecting the left colon (20% of patients), whole colon (5%) or even smallpatients), whole colon (5%) or even small intestine (l-2%).intestine (l-2%).  Obstruction occurs because the affectedObstruction occurs because the affected segments are unable to transmit a peristalticsegments are unable to transmit a peristaltic wavewave 
  45. 45. DiagnosisDiagnosis  Patients present with symptoms fromPatients present with symptoms from the neonatal period with failure or delaythe neonatal period with failure or delay in passing meconium, abdominalin passing meconium, abdominal distension and vomiting, in 80% ofdistension and vomiting, in 80% of cases. Infants with longer segmentcases. Infants with longer segment Hirschsprung’s disease often presentHirschsprung’s disease often present with symptoms suggestive ofwith symptoms suggestive of incomplete small bowel obstructionincomplete small bowel obstruction
  46. 46.  Contrast enema may be suggestive butContrast enema may be suggestive but can look normal, particularly if a digitalcan look normal, particularly if a digital examination has been performed in theexamination has been performed in the preceding 24 hours. A delayedpreceding 24 hours. A delayed radiograph, 24 hours after the enema,radiograph, 24 hours after the enema, will show barium retention. Diagnosis iswill show barium retention. Diagnosis is confirmed by Meier-Ruge histochemicalconfirmed by Meier-Ruge histochemical stain (acetylcholin-esterase) of astain (acetylcholin-esterase) of a suction rectal biopsy.suction rectal biopsy.
  47. 47. ManagementManagement  Priorities are distal bowel decompression byPriorities are distal bowel decompression by rectal washouts and fluid resuscitation, andrectal washouts and fluid resuscitation, and antibiotics for enterocolitis. Once fullantibiotics for enterocolitis. Once full resuscitation is achieved, a temporaryresuscitation is achieved, a temporary defunctioning colostomy is preferred: this isdefunctioning colostomy is preferred: this is placed proximal to the aganglionic bowel andplaced proximal to the aganglionic bowel and guided by frozen section histology ofguided by frozen section histology of seromuscular biopsies. Definitive surgery isseromuscular biopsies. Definitive surgery is usually postponed until after the neonatalusually postponed until after the neonatal period, though earlier definitive surgery hasperiod, though earlier definitive surgery has recently been advocatedrecently been advocated
  48. 48.  Surgery for Hirschsprung’s diseaseSurgery for Hirschsprung’s disease involves placing normal innervatedinvolves placing normal innervated bowel just above the dentate line. Anbowel just above the dentate line. An excellent result is obtained in 90% ofexcellent result is obtained in 90% of patients. The definitive procedure ispatients. The definitive procedure is increasingly undertaken in the neonatalincreasingly undertaken in the neonatal period, without colostomyperiod, without colostomy
  49. 49. Anorectal AnomaliesAnorectal Anomalies  wide spectrum of malformations arewide spectrum of malformations are encountered varying from minor, requiringencountered varying from minor, requiring minimal treatment with excellent results, tominimal treatment with excellent results, to major requiring complex reconstruction withmajor requiring complex reconstruction with limited results. They are commonly dividedlimited results. They are commonly divided into low and high abnormalities depending oninto low and high abnormalities depending on the relationship of the terminal bowel to thethe relationship of the terminal bowel to the pelvic floor muscles and the muscularpelvic floor muscles and the muscular complex i.e. anal sphincters. It is important tocomplex i.e. anal sphincters. It is important to recognise the condition at birth as delayedrecognise the condition at birth as delayed diagnosis can cause bowel ischaemia, sepsisdiagnosis can cause bowel ischaemia, sepsis and mortality.and mortality.
  50. 50.  In low lesions the developing bowelIn low lesions the developing bowel passes through the pelvic floor musclespasses through the pelvic floor muscles and the 3 muscles comprising theand the 3 muscles comprising the muscular complex (anal sphincters).muscular complex (anal sphincters). Male children usually present with anMale children usually present with an absent anus and with meconiumabsent anus and with meconium appearing on the perineum along theappearing on the perineum along the median raphymedian raphy
  51. 51.  In females 3 orifices can be counted –In females 3 orifices can be counted – urethra, vagina and an abnormallyurethra, vagina and an abnormally placed anal orifice anywhere betweenplaced anal orifice anywhere between the vestibule of the introitus and thethe vestibule of the introitus and the normal site of the anus. The surgicalnormal site of the anus. The surgical correction is relatively easy with ancorrection is relatively easy with an anoplasty or cutback in the female.anoplasty or cutback in the female. Occasionally a posterior anal transfer isOccasionally a posterior anal transfer is preferred especially in females.preferred especially in females.
  52. 52.  High lesions on the other hand has the bowelHigh lesions on the other hand has the bowel arrested above the pelvic floor muscles andarrested above the pelvic floor muscles and in the male they present with intestinalin the male they present with intestinal obstruction, absent anus and meconiumobstruction, absent anus and meconium usually being passed transurethral. Theusually being passed transurethral. The female baby presents with either 2 or 1female baby presents with either 2 or 1 perineal orifices and bowel obstruction.perineal orifices and bowel obstruction. Decompression colostomy is usually required,Decompression colostomy is usually required, followed by a complex pullthrough procedurefollowed by a complex pullthrough procedure to place the terminal bowel inside theto place the terminal bowel inside the muscular complex. Long-term prognosis formuscular complex. Long-term prognosis for continence is less than satisfactory.continence is less than satisfactory.

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