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Surgical Management of Total Colonic Aganglionosis and Extensive Aganglionosis

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Abstract

Total colonic aganglionosis (TCA) is a rare form of Hirschsprung disease and is defined as aganglionosis extending to the cecum or distal ileum. The mortality rate of this disease declined dramatically recently, but the morbidity rate is still high. Although several operative procedures have been adopted for TCA such as Martin, Duhamel, Soave, Swenson, and colon patch, with some modifications, the optimal procedure is not apparent, and we need to develop a novel treatment strategy to improve the high morbidity rate after pull-through operation.

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References

  1. Sandegard E. Hirschsprung’s disease with ganglion cell aplasia of the colon and terminal ileum: report of a case treated with total colectomy and ileo-anostomy. Acta Chir Scand. 1953;106:369–76.

    CAS  PubMed  Google Scholar 

  2. Laughlin DM, Friedmacher F, Puri P. Total colonic aganglionosis: a systematic review and meta-analysis of long-term clinical outcome. Pediatr Surg Int. 2012;28:773–9.

    Article  PubMed  Google Scholar 

  3. Moore SW. Total colonic aganglionosis and Hirschsprung’s disease: a review. Pediatr Surg Int. 2015;31:1–9.

    Article  CAS  PubMed  Google Scholar 

  4. Moore SW. Total colonic aganglionosis in Hirschsprung disease. Semin Oediatr Surg. 2012;21:302–9.

    Article  Google Scholar 

  5. Taguchi T, Obata S, Ieiri S. Current status of Hirschsprung’s disease: based on a nationwide survey of Japan. Pediatr Surg Int. 2017;33:497–504.

    Article  PubMed  Google Scholar 

  6. Ieiri S, Suita S, Nakatsuji T, Akiyoshi J, Taguchi T. Total colonic aganglionosis with or without small bowel involvement: a 30-year retrospective nationwide survey in Japan. J Pediatr Surg. 2008;43:2226–30.

    Article  PubMed  Google Scholar 

  7. Moore SW, Rode H, Miller AJ, Albertyn R, Cywes S. Familial aspects of Hirschsprung disease. Eur J Pediatr Surg. 1991;1:97–107.

    Article  CAS  PubMed  Google Scholar 

  8. Jamieson DH, Dundas SE, Belushi SA, Cooper M, Blair GK. Does the transition zone reliably delineate aganglionic bowel in Hirschsprung’s disease? Pediatr Radiol. 2004;34:811–5.

    Article  PubMed  Google Scholar 

  9. Stranzinger E, DiPietro MA, Teitelbaum DH, Strouse PJ. Imaging of total colonic Hirschsprung disease. Pediatr Radiol. 2008;38:1162–70.

    Article  PubMed  Google Scholar 

  10. Huang Y, Anupama B, Zheng S, Xiao X, Chen L. The expression of enteric nerve markers and nerve innervation in total colonic aganglionosis. Int J Surg Pathol. 2011;19:303–8.

    Article  Google Scholar 

  11. Cheung ST, Tam YH, Chong HM, Chan KW, Mou WC, Shhoe DYJ, Lee KH. An 18-year experience in total colonic aganglionosis: from staged operation to primary laparoscopic endorectal pull-through. J Pediatr Surg. 2009;44:2352–4.

    Article  PubMed  Google Scholar 

  12. Yagi M, Okuyama H, Oue T, Kubota A, Ohyanagi H. One-stage laparoscopic pull-through operation without stoma for total colon aganglionosis. Pediatr Endosurg Innovative Tech. 2003;7:169–73.

    Article  Google Scholar 

  13. Martin LW. Surgical management of Hirschsprung’s disease involving the small intestine. Arch Surg. 1968;97:183–9.

    Article  CAS  PubMed  Google Scholar 

  14. Kimura K, Nishijima E, Muraji T, Tsugawa C, Matsumoto Y. A new surgical approach to extensive aganglionosis. J Pediatr Surg. 1981;16:840–3.

    Article  CAS  PubMed  Google Scholar 

  15. Nishijima E, Kimura K, Tsugawa C, Muraji T. The colon patch graft procedure for extensive aganglionosis: long-term follow-up. J Pediatr Surg. 1998;33:215–9.

    Article  CAS  PubMed  Google Scholar 

  16. Sauer H, Klos I. A proposal to preserve the ileocecal valve and right colon in total colonic aganglionosis. J Pediatr Surg. 1989;24:457–61.

    Article  CAS  PubMed  Google Scholar 

  17. Sauer H, Fasching G. Preservation of ileocecal valve and right colon in total colonic aganglionosis. J Pediatr Surg. 1993;28:1640–3.

    Article  CAS  PubMed  Google Scholar 

  18. Amerstorfer EE, Fasching G, Till H, Huber-Zeyringer A, Hollwarth ME. Long-term results of total colonic aganglionosis patients treated by preservation of the aganglionic right hemicolon and the ileocecal valve. Pediatr Surg Int. 2015;31:773–80.

    Article  PubMed  Google Scholar 

  19. Hukkinen M, Koivusalo A, Rintala RJ, Pakarinen MP. Restorative proctocolectomy with j-pouch anastomosis for total colonic aganglionosis among neonates and infants. J Pediatr Surg. 2014;49:570–4.

    Article  PubMed  Google Scholar 

  20. Kimura K, Inimata Y, Soper RT. Posterior sagittal rectal myectomy for persistent rectal achalasia after Soave procedure for Hirschsprung disease. J Pediatr Surg. 1993;28:1200–1.

    Article  CAS  PubMed  Google Scholar 

  21. Zhang JS, Li L, Hou WY, Liu SL, Diao M, Zhang J, Ming AX, Cheng W. Transanal rectal mucosectomy and partial internal anal sphincterectomy for Hirschsprung disease. J Pediatr Surg. 2014;49:831–4.

    Article  PubMed  Google Scholar 

  22. Li Q, Li L, Jiang Q, Zhang Z, Xiao P. The mid-term outcomes of TRM-PIAS, proctocolectomy and ileoanal anastomosis for total colonic aganglionosis. Pediatr Surg Int. 2016;32:477–82.

    Article  CAS  PubMed  Google Scholar 

  23. Bischoff A, Levitt MA, Pena A. Total colonic aganglionosis: a surgical challenge. How to avoid complications? Pediatr Surg Int. 2011;27:1047–52.

    Article  PubMed  PubMed Central  Google Scholar 

  24. Ziegler MM, Royal RE, Brandt J, Drasnin J, Martin LW. Extended myectomy-myotomy: a therapeutic alternative for total intestinal aganglionosis. Ann Surg. 1993;218:504–11.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Nakamura H, Henderson D, Puri P. A meta-analysis of clinical outcome of intestinal transplantation in patients with total intestinal aganglionosis. Pediatr Surg Int. 2017;33:837–41.

    Article  PubMed  Google Scholar 

  26. Menezes M, Prato AP, Jasonni V, Puri P. Long-term clinical outcome in patients with total colonic aganglionosis: a 31-year review. J Pediatr Surg. 2008;43:1696–9.

    Article  PubMed  Google Scholar 

  27. Blackburn S, Corbett P, Griffiths DM, Burge D, Beattie RM, Stanton M. Total colonic aganglionosis: a 15-year single center experience. Eur J Pediatr Surg. 2014;24:488–91.

    Article  PubMed  Google Scholar 

  28. Shen C, Song Z, Zheng S, Xiao X. A comparison of the effectiveness of the Soave and Martin procedure for the treatment of total colonic aganglionosis. J Pediatr Surg. 2009;44:2355–8.

    Article  PubMed  Google Scholar 

  29. Hukkinen M, Koivusalo A, Merras-Salmio L, Rintala RJ, Pakarinen MP. Postoperative outcome and survival in relation to small intestinal involvement of total colonic aganglionosis. J Pediatr Surg. 2015;50:1859–64.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, Tokyo, Japan

    Yutaka Kanamori

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  1. Yutaka Kanamori
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Correspondence to Yutaka Kanamori .

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Editors and Affiliations

  1. Department of Pediatric Surgery, Kyushu University, Fukuoka, Fukuoka, Japan

    Tomoaki Taguchi

  2. Department of Pediatric Surgery, St. Luke’s International Hospital, Chuo-Ku, Tokyo, Japan

    Hiroshi Matsufuji

  3. Department of Pediatric Surgery, Kagoshima University, Kagoshima, Kagoshima, Japan

    Satoshi Ieiri

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Kanamori, Y. (2019). Surgical Management of Total Colonic Aganglionosis and Extensive Aganglionosis. In: Taguchi, T., Matsufuji, H., Ieiri, S. (eds) Hirschsprung’s Disease and the Allied Disorders. Springer, Singapore. https://doi.org/10.1007/978-981-13-3606-5_25

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  • DOI: https://doi.org/10.1007/978-981-13-3606-5_25

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  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-13-3605-8

  • Online ISBN: 978-981-13-3606-5

  • eBook Packages: MedicineMedicine (R0)

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