1. Intestinal Obstruction (Hirschsprung’s Disease & Intussusception)
Brig Mushahid Aslam

2. Hirschsprung’s Disease

3. Pathophysiology... Anatomy Embryology Congenital Anomalies
Anorectal Malformations

4. Pathophysiology...

5. Pathophysiology... Aganglionosis Cholinergic Hyperinnervation
Adrenergic Innervation
Nitregenic Innervation
Inerstitial Cells of Cajal
Enteroendocrine Cells
Smooth Muscles
Extracellular Matrix

6. Pathophysiology...

7. Clinical Features

8. Presentation Failure to pass meconium Abdominal distention
Bilious aspirate
Constipation
Diarrhoea- enterocolitis %

9. Clinical Features Isolated Trait 70 % Chromosomal Abnormality 12%
Associated Anomalies 18%

10. Clinical Features

11. Congenital Anomalies and Genetic Associations
70 % sporadic, but 12% genetic…long segment aut dominnt, sort segment…aut recessive..Malrotation, Arm, intestinal atresia
Down syndrome 7%, hydrocephalus, dandy walker syndrome, myelomingocele. Hypospadias,udt, hydronephrosis, ureteric duplications,dystrophy plydactyly,

12. Differential Diagnosis

13. Radiological Diagnosis

14. Radiological Diagnosis

15. Radiological Diagnosis

16. Functional Diagnosis
Electromanometry

18. Other methods Manovolumetry Electromyography Endosonography
Transit time studies

19. Histopathological DiagnosisHD
No ganglion cells
Increased Ach E activity
Ultrashort HD 13%
Increased Ach E in muscularis mucosae
Hypoganglionosis 5%
10 times decrease
LDH reaction imp.

20. Histopathological Diagnosis
Hypoplasia Nerve Cells
If cells are < 50 % size at 3 years
Desmosis Colon
Absence of tendinus network between long and circ layer
Displacement of Ganglion cells

21. NADPH-Diaphorase Histochemistry
Difficult to comment on suction biopsy
Eosin and H. staining
Def of NOS HD
Hypoganglionosis
Hyperganglionosis

22. Other Inv. Immunohistochemistry Immunoflorescence Electronmicroscopy
Direct
Indirect
Immunoflorescence
Electronmicroscopy

23. Management At Birth Chronic constipation 10 months, 10 Hb, 10 kgs
Rectal Biopsy
Leveling Colostomy
Chronic constipation
Ba Enema
Rectal biopsy
10 months, 10 Hb, 10 kgs
Duhamel’s Procedure
Soave’s procedure

25. Intussuception

26. Definition
telescoping of one segment of bowel into an immediately adjacent segment

27. Classification.
Enterocolic(90%)
Colocolic
Enteroenteric

29. Causes of intussusception
Idiopathic(90%)
Nonidiopathic. (hypertrophied Peyer patches secondary to infection, adenovirus infection, foreign bodies, parasitic infestation polyps, lipomas, Meckel's diverticulum, intestinal duplication, Henoch-Schönlein purpura, lymphomas, (

30. Epidemiology 2 per 1000 live births. male-to-female ratio is 3:1.
Most common between 3-9 month
most common cause of intestinal obstruction between 6 and 36 months of age
Most episodes occur in otherwise healthy and well-nourished children

31. Epidemiology Most patients recover if treated within 24 hours.
Mortality with treatment is 1-3%
untreated this condition is uniformly fatal in 2-5 days
Recurrence : 3-11%

32. Presentation Abdominal pain(80-95%) :
The child appears to have intermittent abdominal pain( manifest as episodic bouts of crying) which is colicky, severe and may be accompanied by pallor and drawing up of the legs (guarded position)
Episodes typically occur 2-3 times/hour.
Infant may sleep or may appear lethargic or playful between episodes of pain.

33. Presentation classic red currant jelly stool is a late sign (60%)
Vomiting (75%)
is usually a prominent feature
Initially nonbilious but may progress to bilious
Bowel motions
blood and/or mucus
classic red currant jelly stool is a late sign (60%)

36. Classic triad(21% all three, 72% have two)
1-Intermittent abd. Pain(80-95%)
2-Bilious vomiting(75%)
3-Currant-jelly stool(60%)

37. Examination
Abdomen:
Abdominal mass(65%) - sausage shaped mass in RUQ or mid-abdomen variably tender
Abdomen may be soft, non-tender or distended and tender

38. Examination Peristaltic wave may be present.
Absence of bowel contents in RLQ ( Dance sign)
PR: may revealed blood or mass. (PR unnecessary if good evidence of intussusception).

39. Investigations Blood tests FBC, U&E Blood group and cross -match
Blood glucose
Cbc for leukocytosis, ue for dehydration

40. Plain abdominal Xray
Performed to exclude perforation or bowel obstruction
A normal AXR does not exclude intussusception
radiographic signs of intussusception are subtle
Signs of intussusception on a plain Xray include :

41. 1-Target sign - two concentric circular radiolucent lines usually in the right upper quadrant
2-Crescent sign : intussusceptum protruding into a gas filled pocket, which often results in a crescent shaped gas pocket.
3-Signs of obstruction.
( dilated small bowel, fluid levels, minilmal fecal content of colon

42. .

43. Sensitive and specific.
Ultrasound scan :
Useful if there is a suggestive history but no mass palpable or signs on plain AXR
Sensitive and specific.
Its use is limited by diagnostic and therapeutic use of air enema
Donut sign: hyperechoic core surrounded by hypoechoic rim

45. This intervention is both diagnostic and therapeutic
Hydrostatic reduction( air or barium)
This intervention is both diagnostic and therapeutic
Diagnostic investigation of choice if high level of suspicion
Sucuss rate is 80-90%, recrrence is 10%(most within 24 hr post reduction)

47. Complications: Intestinal hemorrhage
Intestinal obstruction and dehydration.
Bowel infarction leading to bowel resection
Bowel perforation
Peritonitis
Sepsis and shock
recurrence

48. Prognosis
Prognosis is excellent if diagnosed and treated early; otherwise, severe complications and death may occur.

49. Differential diagnosis
Gastroenteritis
Enterocolitis
Infantile colic
Incarcerated inguinal hernia
meckel’s diverticulum
HSP
others: polyps, appendicitis

50. Management Initial stabilization: Secure IV access
Most children will require fluid resuscitation with normal saline 20mls/kg IV
Keep nil orally
nasogastric decompression
Surgical consultation.
It is very important that this condition is diagnosed and treated early

51. Hydrostatic reduction
Sucuss rate is 80% in <24h of intrassusception. Only 32% if >24h.,
recrrence is 10%(most within 24 hr post reduction)
CI: peritonitis, perforation, shock
Complications: perforation, reduction of necrotic bowel.

52. Surgical reduction: indicated in:
1-suspected bowel gangrene or perforation.
2 -failure of hydrostatic reduction
3-multible recurrence.

53. Clinical pearls
Intussusception is the most common cause of intestinal obstruction between 3 months and 2 years of age.
high index of suspicion is essential
60% of Intussusception are initially misdiagnosed( GE is commonly confused with it)
( please remember)