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

Hirschsprung’s Disease

Pathophysiology... Anatomy Embryology Congenital Anomalies Anorectal Malformations

Pathophysiology...

Pathophysiology... Aganglionosis Cholinergic Hyperinnervation Adrenergic Innervation Nitregenic Innervation Inerstitial Cells of Cajal Enteroendocrine Cells Smooth Muscles 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 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,

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 Immunoflorescence Electronmicroscopy Direct Indirect Immunoflorescence Electronmicroscopy

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

Intussuception

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 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%)

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 mid-abdomen 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 FBC, U&E Blood group and cross -match Blood glucose Cbc for leukocytosis, ue for dehydration

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. ( dilated small bowel, fluid levels, minilmal fecal content of colon

.

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

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)

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. It is very important that this condition is diagnosed and treated early

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) ( please remember)