Intestinal ObstructionIntestinal Obstruction(Hirschsprung’s Disease & Intussusception)(Hirschsprung’s Disease & Intussusception)

Brig Mushahid AslamBrig Mushahid Aslam

Hirschsprung’s Disease

Pathophysiology...

Anatomy Embryology Congenital Anomalies Anorectal Malformations

Pathophysiology...

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

Pathophysiology...

Clinical Features

Presentation

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 No ganglion cells Increased Ach E activity

Ultrashort HD 13% Increased Ach E in muscularis mucosae

Hypoganglionosis 5% 10 times decrease LDH reaction imp.

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

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

Other Inv.

Immunohistochemistry Direct Indirect

Immunoflorescence Electronmicroscopy

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

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

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 2-

5 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%)

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

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 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)