hirschprung

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Author:
smaddineni
ID:
93827
Filename:
hirschprung
Updated:
2011-07-12 20:26:09
Tags:
constipation
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Description:
constipation
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  1. childhood sx
    • physiologic intestinal obstxn due to lack of peristalsis in aganglionic segment
    • failure to pass meconium 24-48 h
    • Rx: dre, laxatives, enemas, if long segment then sx
  2. adult onset
    • fecal soiling rare
    • ddx: megacolon
  3. rectal biopsy
    • full thickness, get atleast m. propria
    • <2 cm above dentate line
    • suction rectal biopsy, acetylcholinesterase activity in lamina propria and m. mucosae
  4. anal manometry
    • absent RAIR
    • failure of IAS relaxation after balloon inflation
  5. RX
    • anorectal myectomy (extensive IA sphincterortomy for short segment) w/w/o LAR
    • duhamel w/w/o diverting colostomy
    • swenson or soave pull through
    • LAR with CAA w/w/o intervening pouch
    • IPAA
    • altmeier
    • delorme
  6. anorectal myectomy
    • transverse incision, elevate mucosa
    • from level of dentate in lateral position
    • excise strip of muscularis including IAS as far proximal as possible (6-10 cm)
  7. duhamel
  8. Idiopathic megarectum or colon
    • restorative proctocolectomy
    • defuctioning stoma if medical treatment fails
    • rectal resection and CAA w/w/o colonic pouch for megarectum

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