Hirschsprung's disease (constipation) Flashcards

1
Q

Define Hirschsprung’s disease

A

Congenital condition characterised by partial or complete colonic functional obstruction associated with absence of ganglion cells 

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2
Q

What is the aetiology of Hirschsprung’s disease

A

Absence of ganglion cells from the myenteric and submucosal plexuses (Meissner’s and Auerbach’s) of the large bowel (parasympathetic)
This results in a narrow and contracted segment of the large bowel 
The region of the normal bowel will extend from the rectum for a variable distance proximally 
The region of abnormal bowel will end up with a normally innervated, dilated colon 
In 75% of cases, the lesion is confined to the rectosigmoid 
In 10% of cases, the entire colon is affected 

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3
Q

What are the symptoms of Hirschsprung’s disease

A

Neonatal intestinal obstruction:
- failure to pass meconium within the first 24h
- abdominal distension
- Bile stained vomiting
- Rectal examination: narrowed segment, withdrawing of the examining finger releases liquid stools and flatus
- Hirschsprung’s enterocolitis (first few weeks of life)

Childhood chronic constipation
- Profound constipation
- Abdominal distension, usually without soiling
- Growth failure

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4
Q

What investigations should be done for Hirschsprung’s disease

A

AXR: Multiple dilated loops of bowel
Suction full thickness rectal biopsy (diagnostic): absence of ganglion cells and presence of large acetylcholinesterase-positive nerve trunks

+/- GI contrast enema: dilated distal segment and narrowed proximal segment
- avoided in those suspected to have Hirschsprung’s who are systemically unwell (?perforation)

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5
Q

What is the management for Hirschsprung’s disease

A
  1. Bowel irrigation (help prevent enterocolitis)
  2. Surgery: Anorectal pull through
    - Colostomy -> anastomosing normally innervated bowel to the anus
    - Definitive surgical treatment done either trans-anally or with laparoscopic assistance 
    - Total colonic agangliosis: initial ileostomy with later corrective surgery 
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6
Q

What are the complications of Hirschsprung’s disease

A

Ischaemic enterocolitis 
Post-operative enterocolitis, constipation or diarrhoea 
Duhamel-related rectal pouch 
Faecal incontinence- surgically-related or pseudo 
Meconium plug syndrome  
Hirschsprung’s enterocolitis (perf colon)  

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7
Q

What is the prognosis for Hirschsprung’s disease

A

Rectosigmoid disease outcome is good
Soiling is usually treatable

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8
Q

What are the risk factors for Hirschsprung’s disease

A

Down’s syndrome
M>F
Caucasian

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9
Q

Describe Hirschsprung’s enterocolitis

A

Marked by proximal colonic dilatation secondary to obstruction
Thinning of the colonic wall, bacterial overgrowth and translocation of gut bacteria
S/S: fever, abdominal distension, blood diarrhoea
Shock and death follows rapidly
Tx: fluid resuscitation, decompression with NG and rectal tubes, broad spectrum Abx -> surgery (definitive)

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