Transcript Choanal Atresia

Intestinal Obstruction
(Hirschsprung’s Disease & Intussusception)
Brig Mushahid Aslam
Hirschsprung’s Disease
Pathophysiology...
 Anatomy
 Embryology
 Congenital Anomalies
 Anorectal Malformations
Pathophysiology...
Pathophysiology...
1. Aganglionosis
2. Cholinergic Hyperinnervation
3.
4.
5.
6.
7.
8.
Adrenergic Innervation
Nitregenic Innervation
Inerstitial Cells of Cajal
Enteroendocrine Cells
Smooth Muscles
Extracellular Matrix
Pathophysiology...
Clinical Features
Presentation
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Failure to pass meconium
Abdominal distention
Bilious aspirate
Constipation
Diarrhoea- enterocolitis
12- 58 %
Clinical Features
 Isolated Trait 70 %
 Chromosomal Abnormality
12%
 Associated Anomalies 18%
Clinical Features
Congenital Anomalies and Genetic
Associations
Differential Diagnosis
Radiological Diagnosis
Radiological Diagnosis
Radiological Diagnosis
Functional Diagnosis
 Electromanometry
Other methods
 Manovolumetry
 Electromyography
 Endosonography
 Transit time studies
Histopathological Diagnosis
 HD
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No ganglion cells
Increased Ach E activity
 Ultrashort HD 13%

Increased Ach E in muscularis mucosae
 Hypoganglionosis 5%
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10 times decrease
LDH reaction imp.
Histopathological Diagnosis
 Hypoplasia Nerve Cells

If cells are < 50 % size at 3 years
 Desmosis Colon
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Absence of tendinus network between long
and circ layer
Displacement of Ganglion cells
NADPH-Diaphorase
Histochemistry
 Difficult to comment on suction biopsy
 Eosin and H. staining
 Def of NOS
 HD
 Hypoganglionosis
 Hyperganglionosis
Other Inv.
 Immunohistochemistry
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Direct
Indirect
 Immunoflorescence
 Electronmicroscopy
Management
 At Birth
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Rectal Biopsy
Leveling Colostomy
 Chronic constipation
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Ba Enema
Rectal biopsy
 10 months, 10 Hb, 10 kgs
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Duhamel’s Procedure
Soave’s procedure
Definition
 telescoping of one segment of bowel into
an immediately adjacent segment
Classification.
 Enterocolic(90%)
 Colocolic
 Enteroenteric
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, (
Epidemiology
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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
Epidemiology
 Most patients recover if treated within 24
hours.
 Mortality with treatment is 1-3%
 untreated this condition is uniformly fatal in 25 days
 Recurrence : 3-11%
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.
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%)

 Classic triad(21% all three, 72% have two)
1-Intermittent abd. Pain(80-95%)
2-Bilious vomiting(75%)
3-Currant-jelly stool(60%)
Examination
 Abdomen:
 Abdominal mass(65%) - sausage
shaped mass in RUQ or midabdomen variably tender
 Abdomen may be soft, non-tender or
distended and tender
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).
Investigations
 Blood tests
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FBC, U&E
Blood group and cross -match
Blood glucose
 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 :

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.
 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

 Hydrostatic reduction( air or barium)
This intervention is both
diagnostic and therapeutic
 Diagnostic investigation of
choice if high level of suspicion

Complications:
 Intestinal hemorrhage
 Intestinal obstruction and dehydration.
 Bowel infarction leading to bowel
resection
 Bowel perforation
 Peritonitis
 Sepsis and shock
 recurrence
 Prognosis
 Prognosis is excellent if diagnosed and
treated early; otherwise, severe complications
and death may occur.
Differential diagnosis
 Gastroenteritis
 Enterocolitis
 Infantile colic
 Incarcerated inguinal hernia
 meckel’s diverticulum
 HSP
 others: polyps, appendicitis
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.
 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.

 Surgical reduction: indicated in:
1-suspected bowel gangrene or perforation.
2 -failure of hydrostatic reduction
3-multible recurrence.
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)