Intestinal%20Obstruction%20(Hirschsprung

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Intestinal Obstruction(Hirschsprungs Disease
Intussusception)

• Brig Mushahid Aslam

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Hirschsprungs Disease
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Pathophysiology...

• Anatomy
• Embryology
• Congenital Anomalies
• Anorectal Malformations

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Pathophysiology...
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Pathophysiology...

• Aganglionosis
• Cholinergic Hyperinnervation
• Adrenergic Innervation
• Nitregenic Innervation
• Inerstitial Cells of Cajal
• Enteroendocrine Cells
• Smooth Muscles
• Extracellular Matrix

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Pathophysiology...
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Clinical Features
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Presentation

• Failure to pass meconium
• Abdominal distention
• Bilious aspirate
• Constipation
• Diarrhoea- enterocolitis 12- 58

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Clinical Features

• Isolated Trait 70
• Chromosomal Abnormality 12
• Associated Anomalies 18

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Clinical Features
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Congenital Anomalies and Genetic Associations
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Differential Diagnosis
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Radiological Diagnosis
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Radiological Diagnosis
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Radiological Diagnosis
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Functional Diagnosis

• Electromanometry

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Other methods

• Manovolumetry
• Electromyography
• Endosonography
• Transit time studies

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Histopathological Diagnosis

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

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Histopathological Diagnosis

• Hypoplasia Nerve Cells
• If cells are lt 50 size at 3 years
• Desmosis Colon
• Absence of tendinus network between long and
  circ layer
• Displacement of Ganglion cells

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NADPH-Diaphorase Histochemistry

• Difficult to comment on suction biopsy
• Eosin and H. staining
• Def of NOS
• HD
• Hypoganglionosis
• Hyperganglionosis

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Other Inv.

• Immunohistochemistry
• Direct
• Indirect
• Immunoflorescence
• Electronmicroscopy

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Management

• At Birth
• Rectal Biopsy
• Leveling Colostomy
• Chronic constipation
• Ba Enema
• Rectal biopsy
• 10 months, 10 Hb, 10 kgs
• Duhamels Procedure
• Soaves procedure

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Intussuception
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Definition

• telescoping of one segment of bowel into an
  immediately adjacent segment

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Classification.

• Enterocolic(90)
• Colocolic
• Enteroenteric

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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,
  (

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Epidemiology

• 2 per 1000 live births.
• male-to-female ratio is 31.
• 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

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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

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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.

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Presentation

• 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)

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

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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

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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).

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Investigations

• Blood tests
• FBC, UE
• Blood group and cross -match
• Blood glucose

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• 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

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• 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.

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.
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• 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

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• Hydrostatic reduction( air or barium)
• This intervention is both diagnostic and
  therapeutic
• Diagnostic investigation of choice if high level
  of suspicion

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Complications

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

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

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Differential diagnosis

• Gastroenteritis
• Enterocolitis
• Infantile colic
• Incarcerated inguinal hernia
• meckels diverticulum
• HSP
• others polyps, appendicitis

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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.

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• Hydrostatic reduction
• Sucuss rate is 80 in lt24h of intrassusception.
  Only 32 if gt24h.,
• recrrence is 10(most within 24 hr post
  reduction)
• CI peritonitis, perforation, shock
• Complications perforation, reduction of necrotic
  bowel.

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

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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)