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Internal anal sphincter achalasia

MedGen UID:
1054417
•Concept ID:
CN376533
•
Finding
Synonym: Anal achalasia
 
HPO: HP:6000018

Definition

A disorder of the internal anal sphincter (IAS) in which the IAS fails to relax that manifests clinically as intractable constipation. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVInternal anal sphincter achalasia

Professional guidelines

PubMed

Classification and diagnostic criteria of variants of Hirschsprung's disease.
Friedmacher F, Puri P
Pediatr Surg Int 2013 Sep;29(9):855-72. doi: 10.1007/s00383-013-3351-3. PMID: 23943250
Botox injection treatment for anal outlet obstruction in patients with internal anal sphincter achalasia and Hirschsprung's disease.
Koivusalo AI, Pakarinen MP, Rintala RJ
Pediatr Surg Int 2009 Oct;25(10):873-6. doi: 10.1007/s00383-009-2438-3. PMID: 19662428
Internal anal sphincter achalasia in children: clinical characteristics and treatment with Clostridium botulinum toxin.
Ciamarra P, Nurko S, Barksdale E, Fishman S, Di Lorenzo C
J Pediatr Gastroenterol Nutr 2003 Sep;37(3):315-9. doi: 10.1097/00005176-200309000-00020. PMID: 12960655

Recent clinical studies

Etiology

The not-so-rare absent RAIR: Internal anal sphincter achalasia in a review of 1072 children with constipation undergoing high-resolution anorectal manometry.
Baaleman DF, Malamisura M, Benninga MA, Bali N, Vaz KH, Yacob D, Di Lorenzo C, Lu PL
Neurogastroenterol Motil 2021 Apr;33(4):e14028. Epub 2020 Dec 10 doi: 10.1111/nmo.14028. PMID: 33301220Free PMC Article
Botulinum toxin injection for internal anal sphincter achalasia after pull-through surgery in Hirschsprung disease.
Youn JK, Han JW, Oh C, Kim SY, Jung SE, Kim HY
Medicine (Baltimore) 2019 Nov;98(45):e17855. doi: 10.1097/MD.0000000000017855. PMID: 31702647Free PMC Article
Internal anal sphincter achalasia: data from a nationwide survey of allied disorders of Hirschsprung's disease in Japan.
Obata S, Fukahori S, Yagi M, Suzuki M, Ueno S, Ushijima K, Taguchi T
Surg Today 2017 Dec;47(12):1429-1433. Epub 2017 Apr 28 doi: 10.1007/s00595-017-1532-8. PMID: 28455798
Classification and diagnostic criteria of variants of Hirschsprung's disease.
Friedmacher F, Puri P
Pediatr Surg Int 2013 Sep;29(9):855-72. doi: 10.1007/s00383-013-3351-3. PMID: 23943250
Internal anal sphincter achalasia in children: clinical characteristics and treatment with Clostridium botulinum toxin.
Ciamarra P, Nurko S, Barksdale E, Fishman S, Di Lorenzo C
J Pediatr Gastroenterol Nutr 2003 Sep;37(3):315-9. doi: 10.1097/00005176-200309000-00020. PMID: 12960655

Diagnosis

The not-so-rare absent RAIR: Internal anal sphincter achalasia in a review of 1072 children with constipation undergoing high-resolution anorectal manometry.
Baaleman DF, Malamisura M, Benninga MA, Bali N, Vaz KH, Yacob D, Di Lorenzo C, Lu PL
Neurogastroenterol Motil 2021 Apr;33(4):e14028. Epub 2020 Dec 10 doi: 10.1111/nmo.14028. PMID: 33301220Free PMC Article
Internal anal sphincter achalasia: data from a nationwide survey of allied disorders of Hirschsprung's disease in Japan.
Obata S, Fukahori S, Yagi M, Suzuki M, Ueno S, Ushijima K, Taguchi T
Surg Today 2017 Dec;47(12):1429-1433. Epub 2017 Apr 28 doi: 10.1007/s00595-017-1532-8. PMID: 28455798
Classification and diagnostic criteria of variants of Hirschsprung's disease.
Friedmacher F, Puri P
Pediatr Surg Int 2013 Sep;29(9):855-72. doi: 10.1007/s00383-013-3351-3. PMID: 23943250
Variants of Hirschsprung disease.
Puri P, Gosemann JH
Semin Pediatr Surg 2012 Nov;21(4):310-8. doi: 10.1053/j.sempedsurg.2012.07.005. PMID: 22985836
Internal anal sphincter achalasia.
Doodnath R, Puri P
Semin Pediatr Surg 2009 Nov;18(4):246-8. doi: 10.1053/j.sempedsurg.2009.07.006. PMID: 19782306

Therapy

Botulinum toxin injection for internal anal sphincter achalasia after pull-through surgery in Hirschsprung disease.
Youn JK, Han JW, Oh C, Kim SY, Jung SE, Kim HY
Medicine (Baltimore) 2019 Nov;98(45):e17855. doi: 10.1097/MD.0000000000017855. PMID: 31702647Free PMC Article
Anal sphincter botulinum toxin injection in children with functional anorectal and colonic disorders: A large institutional study and review of the literature focusing on complications.
Halleran DR, Lu PL, Ahmad H, Paradiso MM, Lehmkuhl H, Akers A, Hallagan A, Bali N, Vaz K, Yacob D, Di Lorenzo C, Levitt MA, Wood RJ
J Pediatr Surg 2019 Nov;54(11):2305-2310. Epub 2019 Apr 23 doi: 10.1016/j.jpedsurg.2019.03.020. PMID: 31060739
Ultrasound-guided intrasphincteric botulinum toxin injection relieves obstructive defecation due to Hirschsprung's disease and internal anal sphincter achalasia.
Church JT, Gadepalli SK, Talishinsky T, Teitelbaum DH, Jarboe MD
J Pediatr Surg 2017 Jan;52(1):74-78. Epub 2016 Oct 27 doi: 10.1016/j.jpedsurg.2016.10.023. PMID: 27836361
Internal anal sphincter achalasia in children: clinical characteristics and treatment with Clostridium botulinum toxin.
Ciamarra P, Nurko S, Barksdale E, Fishman S, Di Lorenzo C
J Pediatr Gastroenterol Nutr 2003 Sep;37(3):315-9. doi: 10.1097/00005176-200309000-00020. PMID: 12960655
The treatment of internal anal sphincter achalasia with botulinum toxin.
Messineo A, Codrich D, Monai M, Martellossi S, Ventura A
Pediatr Surg Int 2001 Sep;17(7):521-3. doi: 10.1007/s003830100583. PMID: 11666049

Prognosis

The not-so-rare absent RAIR: Internal anal sphincter achalasia in a review of 1072 children with constipation undergoing high-resolution anorectal manometry.
Baaleman DF, Malamisura M, Benninga MA, Bali N, Vaz KH, Yacob D, Di Lorenzo C, Lu PL
Neurogastroenterol Motil 2021 Apr;33(4):e14028. Epub 2020 Dec 10 doi: 10.1111/nmo.14028. PMID: 33301220Free PMC Article
Internal anal sphincter achalasia: data from a nationwide survey of allied disorders of Hirschsprung's disease in Japan.
Obata S, Fukahori S, Yagi M, Suzuki M, Ueno S, Ushijima K, Taguchi T
Surg Today 2017 Dec;47(12):1429-1433. Epub 2017 Apr 28 doi: 10.1007/s00595-017-1532-8. PMID: 28455798
Ultrasound-guided intrasphincteric botulinum toxin injection relieves obstructive defecation due to Hirschsprung's disease and internal anal sphincter achalasia.
Church JT, Gadepalli SK, Talishinsky T, Teitelbaum DH, Jarboe MD
J Pediatr Surg 2017 Jan;52(1):74-78. Epub 2016 Oct 27 doi: 10.1016/j.jpedsurg.2016.10.023. PMID: 27836361
Botox injection treatment for anal outlet obstruction in patients with internal anal sphincter achalasia and Hirschsprung's disease.
Koivusalo AI, Pakarinen MP, Rintala RJ
Pediatr Surg Int 2009 Oct;25(10):873-6. doi: 10.1007/s00383-009-2438-3. PMID: 19662428
Internal anal sphincter achalasia: outcome after internal sphincter myectomy.
De Caluwé D, Yoneda A, Akl U, Puri P
J Pediatr Surg 2001 May;36(5):736-8. doi: 10.1053/jpsu.2001.22949. PMID: 11329578

Clinical prediction guides

Physical and psychological correlates of somatic symptom in patients with functional constipation: a cross-sectional study.
Zhao Z, Bai B, Wang S, Zhou Y, Yu P, Zhao Q, Yang B
BMC Psychiatry 2024 Feb 16;24(1):134. doi: 10.1186/s12888-024-05559-9. PMID: 38365647Free PMC Article
Botulinum toxin injection for internal anal sphincter achalasia after pull-through surgery in Hirschsprung disease.
Youn JK, Han JW, Oh C, Kim SY, Jung SE, Kim HY
Medicine (Baltimore) 2019 Nov;98(45):e17855. doi: 10.1097/MD.0000000000017855. PMID: 31702647Free PMC Article
Botox injection treatment for anal outlet obstruction in patients with internal anal sphincter achalasia and Hirschsprung's disease.
Koivusalo AI, Pakarinen MP, Rintala RJ
Pediatr Surg Int 2009 Oct;25(10):873-6. doi: 10.1007/s00383-009-2438-3. PMID: 19662428
Comparison of botulinium toxin injection and posterior anorectal myectomy in treatment of internal anal sphincter achalasia.
Foroutan HR, Hosseini SM, Banani SA, Bahador A, Sabet B, Zeraatian S, Banani SJ
Indian J Gastroenterol 2008 Mar-Apr;27(2):62-5. PMID: 18695305

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