hirshsprung disease Flashcards
what is wrong in hirschsprungs?
nerve cells of the myenteric plexus are absent in the distal bowel
aganglionic section of the colon does not relax and becomes constricted- proximal to the obstruction the bowel becomes distended
what are some genetic associations with hirschsprungs?
- downs syndrome
- neurofibramatosis
- waardenburg syndrome- pale blue eyes, hearing loss and patches of white hair and skin
- MEN 2
how does hirschsprung present?
Delay in passing meconium (more than 24 hours)
Chronic constipation since birth
Abdominal pain and distention
Vomiting- billious
Poor weight gain and failure to thrive
how is hirschsprung diagnosed?
- abdominal X-ray
- rectal biopsy- bowel histology will demonstrate an absense of ganglionic cells
how common is hirschsprung associated enterocolitis?
- occurs in 20% of neonates with hirschsprung
how does HAEC present?
- 2-4 weeks after birth
- fever
- abdo distension
- bloody diarrhoea
- sepsis
what are complications of HAEC?
- Toxic megacolon
- bowel perforation
how to treat HAEC?
- antibiotics
- fluid resuscitation
- decompression of obstructed bowel
what is difinitive treatement of hirchsprungs?
- surgical removal of aganglionic bowel
