Hirschsprung's disease Flashcards

1
Q

What is Hirschsprung’s disease

A

Congenital condition where nerve cells of the myeteric plexus are absent in the distal bowel and rectum

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2
Q

What is the total colonic aganglionosis

A

When the entire colon is affected so there is no nervous innervation

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3
Q

Associations with Hirschsprung’s disease

A

Usually occurs in isolation, but has been associated with Down’s, neurofibromatosis, Waardenburg syndrome and MEN type 2

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4
Q

Presentation of Hirschsprung’s disease

A

Acute intestinal obstruction after birth or more gradually developing symptoms. Delay in passing meconium, chronic constipation since birth, abdo pain and distension, vomiting, poor weight gain and failing to thrive

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5
Q

What is Hirschsprungs associated enterocolitis

A

Inflammation and obstruction of the intestine occuring in around 20% of neonates with the disease.

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6
Q

What does Hirschsprung’s associated enterocolitis present with

A

Within 2-4 weeks of birth with fever, abdo distension, diarrhoea, and features of sepsis

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7
Q

What can Hirschsprungs associated colitis lead to

A

Toxic megacolon, perforation requiring Abx, fluid resus and decompression of the obstructed bowel

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8
Q

Diagnosis of Hirschsprungs disease

A

Abdo XR for diagnosis and features, rectal biopsy to confirm

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9
Q

Management of Hirschsprungs disease

A

Surgical removal of aganglionic section of bowel

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