-
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
-
adult onset
- fecal soiling rare
- ddx: megacolon
-
rectal biopsy
- full thickness, get atleast m. propria
- <2 cm above dentate line
- suction rectal biopsy, acetylcholinesterase activity in lamina propria and m. mucosae
-
anal manometry
- absent RAIR
- failure of IAS relaxation after balloon inflation
-
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
-
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)
-
-
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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