intestinal obstruction (hirschsprung’s disease & intussusception) brig mushahid aslam
TRANSCRIPT
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Intestinal ObstructionIntestinal Obstruction(Hirschsprung’s Disease & Intussusception)(Hirschsprung’s Disease & Intussusception)
Brig Mushahid AslamBrig Mushahid Aslam
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Hirschsprung’s Disease
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Pathophysiology...
Anatomy Embryology Congenital Anomalies Anorectal Malformations
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Pathophysiology...
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Pathophysiology...
1. Aganglionosis
2. Cholinergic Hyperinnervation
3. Adrenergic Innervation
4. Nitregenic Innervation
5. Inerstitial Cells of Cajal
6. Enteroendocrine Cells
7. Smooth Muscles
8. 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 < 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 Duhamel’s Procedure Soave’s procedure
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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 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
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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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Abdomen: Abdominal mass(65%) - sausage
shaped mass in RUQ or mid-abdomen variably tender
Abdomen may be soft, non-tender or distended and tender
Examination
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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, U&E 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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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 meckel’s 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 <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.
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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)