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1. ARANSI RILWAN A.

2.  Introduction
 Epidemiology
 Aetiopathogenesis
 Pathophysiology
 Clinical features
 Management
 Complications
 Differential diagnosis
 Conclusion

3. • Hirschsprung’s disease also called “Intestinal
Aganglionosis”.
• Megacolon is a condition in which part or the whole
of the intestine is hypertrophied, dilated and filled
with faeces.
 Their is congenital absence of
parasympathetic ganglion cells normally
found in the intermuscular and submucosal
nerve plexus of the intestinal wall.

4. Incidence is 1 in 5000 births.
Four times as common in males
 However, male preponderance almost
disappears when aganglionosis extends
beyond the recto-sigmoid area.

5.  Congenital absence of ganglion cells
normally found in the intermuscular and
submucosal nerve plexus of the bowel wall
in the distal bowel.
 These ganglion cells coordinate muscular
activity between the extrinsic & intrinsic
inhibitory fibres.

6. The absence of enteric ganglion cells is
probably due to their failure to migrate
caudally from the vagal neural crest
between the 5th & 16th week of fetal life.

7.  This abnormal innervation results in;
 spasticity of the affected bowel segment
 failure of coordinated peristaltic activity
results in functional obstruction to
evacuation of faeces.

8.  The affected aganglionic segment may look
normal or narrow,
 merges proximally into a funnel-shaped
transitional zone which contains only a few
ganglia.
 Above the transitional zone, the bowel is grossly
hypertrophied from increased activity to propel
faeces.

9. The pathophysiology of the disease is
not well understood.
The cause of the spasticity of the
aganglionic segment has not been
satisfactorily explained either.

10.  Theories include;
• Lack of modulatory influence of the ganglia causing imbalance
between the cholinergic motor and adrenergic inhibitory
nerve activity
• Excessive cholinesterase activity coupled with an enhanced
sensitivity of bowel muscle to Acetylcholine
• Deficiency of interstitial cells of Cajal (found in the plexuses
and muscle layers, it generates slow waves in the GIT, act as
pacemakers and control of gut contractions and motility

11. The aganglionosis usually starts at the internal
anal sphincter and then extends proximally to
 Rectosigmoid area in about 75%
 Splenic flexure or transverse colon in 17%
 Whole colon or part of terminal ileum in 8%

12.  Ultra short segment: below rectosigmoid
junction
 Short segment: up to sigmoid colon
 Long segment: up to splenic flexure or
beyond
 Total segment: affects whole colon.

13.  The gross features of HD vary with duration of
untreated disease
 In the neonatal period, the intestine may appear
fairly normal.
 Classically there are 3 zones, macroscopically:
1.Dilated proximal intestine
2.Transition zone
3.Distal undilated bowel

14. 1. Proximal Dilated Bowel
(Normoganglionic)
3. Contracted Distal Bowel
(aganglionic)
2. Transition Zone
(hypoganglionic)

15.  DEPENDS ON AGE AT PRESENTATION
• >80% are diagnosed in the neonatal period
 Delayed passage of meconium after birth
 Constipation
 Gross abdominal distention
 Late onset of bilious vomiting
• In older infants
 Wasting
 Failure to thrive
 Distended abdomen
 Chronic constipation

16. If complicated by enterocolitis
• Infant may present paradoxically with diarrhoea
with profuse amount of offensive, loose watery
stool containing mucus or blood.
• If severe- fever, hypovolaemic shock

17.  On examination
• Gross abdominal distention, visible peristalsis,
flared ribs from abdominal distension causing a
wide subcostal angle.
• Palpable faecal masses in the left flank
• On DRE, narrow, tight and empty anal canal with
small amount of meconium or explosive mixture of
flatus and faeces following DRE

18. HISTORY & EXAMINATION
INVESTIGATIONS
TREATMENT

19. Aim:
Confirm diagnosis
Optimize for treatment.

20. Plain abdominal radiograph:
show dilated loops of bowel with air-
fluid levels.
Occasionally, there may be a small
amount of air in the undistended rectum
and the dilated colon above it.

23. Barium Enema:
 Done on an unprepared colon
 There should not have been a digital rectal
examination or rectal washout for 3 days prior to the
examination.
 The enema will demonstrate the flow of barium from
the undilated rectum through a funnel-shaped
transitional zone into the proximal dilated colon.

25. Anorectal Manometry
 This demonstrates the failure of the internal anal
sphincter to relax when the rectum is distended
- the recto-anal reflex
 The pressure in the anal canal is not reduced on
inflating the balloon in the rectum.
 The test is reliable only in the older child

26. Biopsy Studies
 Is the most accurate way of
 diagnosing Hirschsprung' s disease.
 This biopsy is most often taken from the posterior wall of
the rectum 1, 2, and 3cm above the dentate line under
general anaesthesia.
 The biopsy specimen should be full thickness, containing
mucosa, submucosa and muscle wall.

27. FBC
Elecrtolyte, urea and creatinine
Grouping/ Cross-matching

28. • Depends on mode & age at presentation, length of
the involved segment , severity of symptoms
 Acute intestinal obstruction
 Resuscitation
• IV LINE
• NG TUBE
• URETHRAL CATHETER
• BROAD SPECTRUM ANTIBIOTICS

29. A temporary colostomy is done
Site of colostomy should be as low as
possible in the ganglionated segment.

30.  However, in the abscence of complications, a defunctioning
colostomy to prevent enterocolitis.
 The colostomy is sited in normal bowel usually just above
the transitional zone.
 Definitive surgery is done when the infant is 10kg.
 If a colostomy cannot be done or is unacceptable, regular
saline rectal washouts until the infant is ready for definitive
operation.

31. Older children
Definitive operation is performed in the
older child presenting for the first time if
there is no enterocolitis.

32. Definitive Operations
 Swenson's Operation
The transitional zone is resected with
the aganglionic segment and the normal
bowel pulled down and anastomosed to a
cuff of anus 2-3cm above the anal verge.

33. Duhamel's Operation
Soave's Operation

34.  Intra op
• Anaesthetic complications
• Excessive hemorrhage
 Post op
• Anastomotic leak, stenosis,
• Constipation,
• Faecal incontinence,
• Diarrhoea
• Enterocolitis

35. Meconium ileus
Meconium plug syndrome
Atresias of the distal ileum and
proximal colon
Necrotising enterocolitis
Hypothyroidism

38. Principles and Practice of Surgical
Practice( Badoe).
The American Journal of
Gastroenterology(2005).
Bailey’s & Love’s Short Practice of
Surgery.