EXAMPLE OF NEONATAL INTESINAL OBSTRUCTION

1. Case presentationCase presentation
Ileal atresiaIleal atresia
Dr.Ashraf HamedDr.Ashraf Hamed
Consultant pedia surgeryConsultant pedia surgery

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. 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

5. 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

6. 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

8. 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

10. 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

15. Neonatal IntestinalNeonatal Intestinal
ObstructionObstruction
DR.ASHRAF HAMEDDR.ASHRAF HAMED
CONS.PEDIA SUREGRYCONS.PEDIA SUREGRY

16. 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

17.  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

18.  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

19. 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.

20. 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

21. 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

22.  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).

23.  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.

24. 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

25. Malrotation and midgutMalrotation and midgut
volvulusvolvulus


26. 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

27. 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.

28. 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.

29. 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

30. 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%

32. Pathogenesis:Pathogenesis:
 Figure 2Figure 2
Figure 2

33. 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

34.  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

35. 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.

36. 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.

37. 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’

38. 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),

39. 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.

40. 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.

41. 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.

42. 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.

43. 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

44. 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

45. 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

46. 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

47. 1-231-23
 8Figure 98Figure 9
8 Figure 9

48. 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.

49. 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

50. 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

51.  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.

52.  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


53. 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

54.  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.

55. 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

56.  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

57. 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.

58.  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

59.  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.

60.  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.