title: Long-term outcome of internal sphincter myectomy in patients with internal anal sphincter achalasia.
abstract: BACKGROUND
  Internal anal sphincter achalasia (IASA) is a condition with presentation similar to Hirschsprung's disease (HD), but with the presence of ganglion cells on rectal suction biopsy (RSB). The diagnosis is made on anorectal manometry (ARM) by the absence of the rectosphincteric reflex on rectal balloon inflation. Internal sphincter myectomy (ISM) is the treatment of choice for patients with IASA. Recently, botulinum toxin has been used to treat IASA patients. The purpose of this study was to assess the long-term bowel function in patients with IASA following ISM.
  
  
  METHODS
  The medical records of 24 patients with IASA managed by ISM during 1993-2005 were examined. There were 18 boys and 6 girls, aged 2-12 years. All patients presented with intractable constipation with or without soiling. The diagnosis was made by the demonstration of the absence of the rectosphincteric reflex on ARM. HD was excluded by the presence of ganglion cells and normal acetylcholinesterase activity in RSB. Patients were followed 4-14 years later.
  
  
  RESULTS
  Fifteen (62.5%) patients at the time of follow-up had regular bowel motions without the use of laxatives. Six (25%) patients had regular bowel motions, but remained on small doses of laxatives. Two (8.3%) patients who suffered from constipation and soiling required twice weekly enemas to remain clean. One (4.2%) patient required resection of dilated rectosigmoid colon 3 years after myectomy, remains on laxatives, but has normal bowel control. No patients had faecal incontinence following ISM.
  
  
  CONCLUSIONS
  This long-term follow-up study shows that the vast majority of IASA patients have normal bowel control following ISM.
authors.affiliation: Children's Research Centre, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland.
authors.forename: Reshma
authors.initials: R
authors.lastname: Doodnath
authors.forename: Prem
authors.initials: P
authors.lastname: Puri
mesh_terms: D001003:Anal Canal / Q000002:abnormalities* / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D003248:Constipation / Q000209:etiology; D004065:Digestive System Abnormalities / Q000150:complications / Q000601:surgery*; D013505:Digestive System Surgical Procedures / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D020127:Recovery of Function; D012189:Retrospective Studies; D016896:Treatment Outcome
references.citation: Arch Pathol Lab Med. 2003 Sep;127(9):1192-5
references.pmid: 12946224
references.citation: Pediatr Surg Int. 2008 Jul;24(7):779-83
references.pmid: 18443801
references.citation: J Pediatr Gastroenterol Nutr. 1995 Jan;20(1):54-8
references.pmid: 7533833
references.citation: J Pediatr Surg. 2001 May;36(5):736-8
references.pmid: 11329578
references.citation: Pediatr Surg Int. 2001 Sep;17(7):521-3
references.pmid: 11666049
references.citation: Pediatr Surg Int. 1999;15(3-4):192-4
references.pmid: 10370020
references.citation: Pediatr Surg Int. 2005 Feb;21(2):84-7
references.pmid: 15616817
references.citation: Indian J Gastroenterol. 2008 May-Jun;27(3):99-102
references.pmid: 18787278