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Long-term results of total colonic agangliosis patients treated by preservation of the aganglionic right hemicolon and the ileo-cecal valve

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Abstract

Purpose

In 1989, Sauer introduced a technique to treat patients with total colonic aganglionosis (TCA) where he preserved the ileo-cecal valve and the right hemicolon to retain water and electrolyte absorption. This report examines the long-term outcome of patients who underwent this technique.

Methods

All TCA patients treated between 1981 and 2005 according to Sauer were invited in 2013 to participate in a questionnaire survey to assess their long-term outcome focusing on bowel function and quality of life (QoL), using standardized scores.

Results

Of eight TCA patients (2 females, 6 males), seven participated in the follow-up survey (median follow-up time 13.5 years (range 8–31.6). Early postoperative complications involving bouts of enterocolitis in 3 and anal strictures in 5 patients ceased with age. Bowel movements ranged from median 3–4 times a day (range 1–8). Bowel-function score (BFS) was reduced in 6 patients (median 16 points, range 8–19, max 20), who also reported soiling incidences with the need for night-time protective aids in two. QoL assessment, however, documented a good outcome with a median score of 10 points (range 7–13, max 13).

Conclusion

This long-term investigation of TCA patients treated according to Sauer’s technique documented a good QoL despite a reduced BFS.

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References

  1. Boix-Ochoa J, Casasa JM, Marhuenda C, Broto J, Gil-Vernet JM (1991) Total colonic aganglionosis: surgical treatment and long-term follow up. Pediatr Surg Int 6:198–201

    Article  Google Scholar 

  2. Martin LW (1968) Surgical management of Hirschprung’s disease involving the small intestine. Arch Surg 97:183–189

    Article  CAS  PubMed  Google Scholar 

  3. Walker A, Kempson R, Ternberg J (1966) Aganglionosis of the small intestine. Surgery 60:444–457

    Google Scholar 

  4. Appelbaum H, Richardson RJ, Wilkinson GA, Warden MJ (1988) Alternative operative procedure for total colonic aganglionosis. J Pediatr Surg 23:49–51

    Article  Google Scholar 

  5. Boley SJ (1984) A new operative approach to total colonic aganglionosis of the colon. Surg Gynecol Obstet 159:481–484

    CAS  PubMed  Google Scholar 

  6. Kimura K, Nishijima E, Muraji T, Tsugawa C, Matsumoto Y (1981) A new surgical approach to extensive aganglionosis. J Pediatr Surg 16:840–843

    Article  CAS  PubMed  Google Scholar 

  7. Martin LW (1982) Total colonic aganglionosis: preservation and utilization of entire colon. J Pediatr Surg 17:635–637

    Article  CAS  PubMed  Google Scholar 

  8. Shandling B (1984) Total colon aganglionosis—A new operation. J Pediatr Surg 19:503–505

    Article  CAS  PubMed  Google Scholar 

  9. Sauer H, Klos I (1989) A proposal to preserve the ileocecal valve and right colon in total colonic aganglionosis. J Pediatr Surg 24:457–461

    Article  CAS  PubMed  Google Scholar 

  10. Stringel G (1986) Extensive intestinal aganglionosis including the ileum: a new surgical technique. J Pediatr Surg 21:667–670

    Article  CAS  PubMed  Google Scholar 

  11. Sauer H, Fasching G (1993) Preservation of the ileocecal valve and right colon in total colonic aganglionosis. J Pediatr Surg 28:1640–1643

    Article  CAS  PubMed  Google Scholar 

  12. Rintala RJ, Lindahl H (1995) Is normal bowel function possible after repair of intermediate and high anorectal malformations? J Ped Surg 30:491–494

    Article  CAS  Google Scholar 

  13. Bai Y, Zhengwei Y, Weilin W, Yaru Z, Huizhen W, Wei W (2000) Quality of life for children with fecal incontinence after surgically corrected anorectal malformations. J Ped Surg 35:462–464

    Article  CAS  Google Scholar 

  14. Kottmeier PK, Jongco B, Velcek FT, Friedman A, Klotz DH (1981) Absorptive function of the aganglionic ileum. J Pediatr Surg 16:275–278

    Article  CAS  PubMed  Google Scholar 

  15. Fonkalsrud EW (1996) Long-term results after colectomy and ileoanal pull-through procedure in children. Arch Surg 131:881–886

    Article  CAS  PubMed  Google Scholar 

  16. Rintala RJ, Lindahl HG (2002) Proctocolectomy and J-pouch ileo-anal anastomosis in children. J Pediatr Surg 37:66–70

    Article  CAS  PubMed  Google Scholar 

  17. Lal DR, Nichol PF, Harms BA, Go LL, Lund DP (2004) Ileo-anal S-Pouch reconstruction in patients with total colonic aganglionosis after failed pull-through procedure. J Pediatr Surg 39:7–9

    Article  Google Scholar 

  18. Escobar MA, Grosfeld JL, West KW, Scherer LR, Rouse TM, Engum SA, Rescorla FJ (2005) Long-term outcomes in total colonic aganglionosis: a 32-year experience. J Pediatr Surg 40:955–961

    Article  PubMed  Google Scholar 

  19. Hoehner JC, Ein SH, Shandling B, Kim PC (1998) Long-term morbidity in total colonic aganglionosis. J Pediatr Surg 33(7):961–965 (discussion 965–966)

    Article  CAS  PubMed  Google Scholar 

  20. Nishijima E, Kimura K, Tsugawa C, Muraji T (1998) The colon patch graft procedure for extensive aganglionosis: long-term follow-up. J Pediatr Surg 33:215–219

    Article  CAS  PubMed  Google Scholar 

  21. Careskey J, Weber TR, Grosfeld JL (1981) Ileocecal valve replacement. Its effect on transit time, survival, and weight change after massive intestinal resection. Arch Surg 116:618–622

    Article  CAS  PubMed  Google Scholar 

  22. Clauss W, Schäfer H, Horch I, Hörnicke H (1985) Segmental differences in electrical properties and Na-transport of rabbit caecum, proximal and distal colon in vitro. Pflugers Arch 403:278–282

    Article  CAS  PubMed  Google Scholar 

  23. Goto S, Gunter M, Scherer LR, Bloch T, Grosfeld JL (1986) Surgical treatment of total colonic aganglionosis: efficacy of aganglionic patch enteroplasty in the rat. J Pediatr Surg 21:601–607

    Article  CAS  PubMed  Google Scholar 

  24. Goto S, Grosfeld JL (1987) Is preserving the entire aganglionic colon reasonable in the surgical treatment of total colonic aganglionosis? J Pediatr Surg 22:623–627

    Article  CAS  PubMed  Google Scholar 

  25. Kunieda T, Hosono Y, Sakata K (1981) Experimental studies on intestinal absorption following Martin’s operation. Jpn J Surg 11:481–484

    Article  CAS  PubMed  Google Scholar 

  26. Yau WM, Makhlouf GM (1975) Comparison of transport mechanisms in isolated ascending and descending rat colon. Am J Physiol 228:191–195

    CAS  PubMed  Google Scholar 

  27. Devroede GJ, Phillips SF, Code CF, Lind JF (1971) Regional differences in rates of insorption of sodium and water from the human large intestine. Can J Physiol Pharmacol 49:1023–1029

    Article  CAS  PubMed  Google Scholar 

  28. Hawker PC, Mashiter KE, Turnberg LA (1978) Mechanisms of transport of Na, Cl, and K in the human colon. Gastroenterology 74:1241–1247

    CAS  PubMed  Google Scholar 

  29. Heath AL, Spitz L, Milla PJ (1985) The absorptive function of colonic aganglionic intestine: are the Duhamel and Martin procedures rational? J Pediatr Surg 20:34–36

    Article  CAS  PubMed  Google Scholar 

  30. Sellin JH, De Soignie R (1987) Ion transport in human colon in vitro. Gastroenterology 93:441–448

    CAS  PubMed  Google Scholar 

  31. Grosfeld JL, Rescorla FJ, West KW (1986) Short bowel syndrome in infancy and childhood. Analysis of survival in 60 patients. Am J Surg 151:41–46

    Article  CAS  PubMed  Google Scholar 

  32. Emslie J, Krishnamoorthy M, Applebaum H (1997) Long-term follow-up of patients treated with ileoendorectal pull-through and right colon onlay patch for total colonic aganglionosis. J Pediatr Surg 32:1542–1544

    Article  CAS  PubMed  Google Scholar 

  33. Ross MN, Chang JH, Burrington JD, Janik JS, Wayne ER, Clevenger P (1988) Complications of the Martin procedure for total colonic aganglionosis. J Pediatr Surg 23(8):725–727

    Article  CAS  PubMed  Google Scholar 

  34. Coran AG (1990) A personal experience with 100 consecutive total colectomies and straight ileoanal endorectal pull-throughs for benign disease of the colon and rectum in children and adults. Ann Surg 212(3):242–247 (discussion 247–248)

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  35. Barrena S, Andres AM, Burgos L, Luis AL, Hernandez F, Martinez L, Lopez-Santamaria M, Lassaletta L, Tovar JA (2008) Long-term results of the treatment of total colonic aganglionosis with two different techniques. Eur J Pediatr Surg 18:375–379

    Article  CAS  PubMed  Google Scholar 

  36. Heij HA, de Vries X, Bremer I, Ekkelkamp S, Vos A (1995) Long-term anorectal function after Duhamel operation for Hirschsprung’s disease. J Pediatr Surg 30:430–432

    Article  CAS  PubMed  Google Scholar 

  37. Laughlin DM, Friedmacher F, Puri P (2012) Total colonic aganglionosis: a systematic review and meta-analysis of long-term clinical outcome. Pediatr Surg Int 28:773–779

    Article  PubMed  Google Scholar 

  38. Menezes M, Pini Prato A, Jasonni V, Puri P (2008) Long-term clinical outcome in patients with total colonic aganglionosis: a 31-year review. J Pediatr Surg 43(9):1696–1699

    Article  PubMed  Google Scholar 

  39. Travassos DV, van der Zee DC (2011) Is complete resection of the aganglionic bowel in extensive total aganglionosis up to the middle ileum always necessary? J Pediatr Surg 46:2054–2059

    Article  PubMed  Google Scholar 

  40. Jarvi K, Laitakari EM, Koivusalo A, Rintala RJ, Pakarinen MP (2010) Bowel function and gastrointestinal quality of life among adults operated for Hirschsprung disease during childhood: a population-based study. Ann Surg 252(6):977–981

    Article  PubMed  Google Scholar 

  41. Blackburn S, Corbett P, Griffiths DM, Burge D, Beattie RM, Stanton M (2014) Total colonic aganglionosis: a 15-year single center experience. Eur J Pediatr Surg 24(6):488–491

    Article  PubMed  Google Scholar 

  42. Bai Y, Chen H, Hao J, Huang Y, Wang W (2002) Long-term outcome and quality of life after the Swenson procedure for Hirschsprung’s disease. J Pediatr Surg 37(4):639–642

    Article  PubMed  Google Scholar 

  43. Ludman L, Spitz L, Tsuji H, Pierro A (2002) Hirschsprung’s disease: functional and psychological follow up comparing total colonic and rectosigmoid aganglionosis. Arch Dis Child 86(5):348–351

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  44. Raboei EH (2008) Long-term outcome of total colonic aganglionosis. Eur J Pediatr Surg 18(5):300–302

    Article  CAS  PubMed  Google Scholar 

  45. Tsuji H, Spitz L, Kiely EM, Drake DP, Pierro A (1999) Management and long-term follow-up of infants with total colonic aganglionosis. J Pediatr Surg 34:158–162

    Article  CAS  PubMed  Google Scholar 

  46. Elhalaby EA, Teitelbaum DH, Coran AG, Heidelberger KP (1995) Enterocolitis associated with Hirschsprung’s disease: a clinical histopathological correlative study. J Pediatr Surg 30(7):1023–1026 (discussion 1026–1027)

    Article  CAS  PubMed  Google Scholar 

  47. Surana R, Quinn FM, Puri P (1994) Evaluating the risk factors in the development of enterocolitis complicating Hirschsprung’s disease. Pediatr Surg Int 9:234–236

    Article  Google Scholar 

  48. Shermata DW, Meller JL (1989) Ideal treatment for total colonic Hirschsprung’s disease. J Pediatr Surg 24:88–91

    Article  Google Scholar 

  49. Myrvold H, Tindel MS, Isenberg HD, Stein TA, Scherer J, Wise L (1984) The nipple valve as a sphincter substitute for the ileocecal valve: prevention of bacterial overgrowth in the small bowel. Surgery 96:42–47

    CAS  PubMed  Google Scholar 

  50. Wilkinson L, Rodning CB (1986) Valva ileocaecalis: preservation during ileocolonic surgery employing a rodent experimental model. Am Surg 52:269–272

    CAS  PubMed  Google Scholar 

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Conflict of interest

This study has no conflict of interest and has received no funding.

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Authors and Affiliations

  1. Department of Pediatric and Adolescent Surgery, Medical University of Graz, Auenbruggerplatz 34, 8036, Graz, Austria

    Eva E. Amerstorfer, Holger Till, Andrea Huber-Zeyringer & Michael E. Höllwarth

  2. Department of Pediatric and Adolescent Surgery, Clinical Center Klagenfurt, Feschnigstrasse 11, 9020, Klagenfurt, Austria

    Günter Fasching

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  1. Eva E. Amerstorfer
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Correspondence to Eva E. Amerstorfer.

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Amerstorfer, E.E., Fasching, G., Till, H. et al. Long-term results of total colonic agangliosis patients treated by preservation of the aganglionic right hemicolon and the ileo-cecal valve. Pediatr Surg Int 31, 773–780 (2015). https://doi.org/10.1007/s00383-015-3743-7

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