Skip to main content
SpringerLink
Log in

Outcomes of Surgical Management of Total Colonic Aganglionosis

  • Published:
World Journal of Surgery Aims and scope Submit manuscript

Abstract

Purpose

The purpose of this study was to assess the long-term clinical outcomes and bowel function of patients with total colonic aganglionosis (TCA) after surgery.

Methods

The hospital records of 17 TCA patients treated surgically during 1985 to 2004 were reviewed. Long-term follow-up was done by telephone interviews with the parents.

Results

Primary enterostomy was performed in 13 (76%) patients. In three (17%) patients, TCA was not suspected initially. They underwent conservative surgery primarily, which required a second operation soon after. One had transverse colectomy with ileostomy. By pathologic review, nine (53%) patients had small bowel involvement of aganglionosis. Six (35%) patients died before corrective surgery. They all had extensive small bowel involvement. Among 11 patients who had a corrective operation, 10 were treated with Martin’s procedure. Long-term (mean 74 months) follow-up was available in seven patients, and the mean weight-for-age percentiles was 27.1% (range 5–50%), the frequency of defecation was three to five times a day in four patients (57%), one or two times a day in two patients (28%), and more than five times a day in one patient (15%).

Conclusions

TCA is difficult to diagnose; but once it is diagnosed correctly and treated by corrective surgery, outcomes seem promising. Martin’s operation brought about a good outcome and enabled patients to have acceptable bowel habits. The prognosis is highly dependent on the extent of aganglionosis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Coran AG, Teitelbaum DH (2000) Recent advances in the management of Hirschsprung’s disease. Am J Surg 180:382–387

    Article  PubMed  CAS  Google Scholar 

  2. Suita S, Taguchi T, Kamimura T, et al. (1997) Total colon aganglionosis with or without small bowel involvement: a changing profile. J Pediatr Surg 32:1537–1541

    Article  PubMed  CAS  Google Scholar 

  3. 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  PubMed  CAS  Google Scholar 

  4. Ikeda K, Goto S (1986) Total colonic aganglionosis with or without small bowel involvement: an analysis of 137 patients. J Pediatr Surg 21:319–322

    Article  PubMed  CAS  Google Scholar 

  5. Tsujui H, Spitz L, Kiely EM, et al. (1999) Management of long-term follow-up of infants with total colonic aganglionosis. J Pediatr Surg 34:157–162

    Google Scholar 

  6. Barbara EW, Daniel HT, Arnold GC (2005) Total colonic Hirschsprung’s disease: a 28-year experience. J Pediatr Surg 40:203–207

    Article  Google Scholar 

  7. Jasonni V, Martucciello G (1998) Total colonic aganglionosis. Semin Pediatr Surg 7:174–180

    PubMed  CAS  Google Scholar 

  8. Ratta BS, Kiely EM, Spitz L, et al. (1990) Improvement in the management of total colonic aganglionosis. Pediatr Surg Int 5:30–36

    Article  Google Scholar 

  9. Ziegler MM, Ross AJ III, Bishop H (1987) Total intestinal aganglionosis: a new technique for prolonged survival. J Pediatr Surg 22:82–83

    Article  PubMed  CAS  Google Scholar 

  10. Mauricio AE, Jay LG, Karen WW, et al. (2005) Long-term outcomes in total colonic aganglionosis: a 32-year experience. J Pediatr Surg 40:955–961

    Article  Google Scholar 

  11. Hirschsprung H (1887) Stuhltraegheit neugeborener in folge von dilatation and hypertrophie des colons. Jahrb Kinderh 27:1

    Google Scholar 

  12. Kim WK, Park KW, Lee SC (1987) Hirschsprung’d disease: an analysis of 214 patients. J Korean Surg Soc 32:436–444

    Google Scholar 

  13. Okamoto E, Ueda T (1967) Embryogenesis of intramural ganglia of the gut and its relation to Hirschsprung’s disease. J Pediatr Surg 2:437

    Article  Google Scholar 

  14. Swenson O, Rheinlander HF, Diamond I (1949) Hirschsprung’s disease: a new concept of the etiology—operative results of 34 patients. N Engl J Med 241:51

    Article  Google Scholar 

  15. Zuelzer WW, Wilson JL (1948) Functional intestinal obstruction on a congenital neurogenic basis in infancy. Am J Dis Child 74:40

    Google Scholar 

  16. Lee SK, Kim WK, Park KW, et al. (1988) Total colonic aganglionosis: an analysis of 18 cases. J Korean Surg Soc 35:340–345

    Google Scholar 

  17. Martin LW (1968) Surgical management of Hirschsprung’s disease involving the small intestine. Arch Surg 97:183

    PubMed  CAS  Google Scholar 

  18. Martin LW (1972) Surgical management of total colonic aganglionosis. Ann Surg 176:343

    Article  PubMed  CAS  Google Scholar 

  19. Kimura K, Hishijima E, Muraji T, et al. (1981) A new surgical to extensive aganglionosis. J Pediatr Surg 16:840–843

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Author notes

  1. H. Y. Kim

    Present address: Department of Surgery, Gachon Medical Center, Gachon Medical College, Incheon, Korea

Authors and Affiliations

  1. Department of Surgery, College of Medicine, Seoul National University, 28 Yongon-Dong, 110-744, Seoul, Korea

    E. K. Choe

  2. Department of Pediatric Surgery, Seoul National University Children’s Hospital, 28 Yongon-Dong, 110-744, Seoul, Korea

    S. B. Moon, H. Y. Kim, S. C. Lee, K. W. Park & S. E. Jung

Authors
  1. E. K. Choe
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. S. B. Moon
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. H. Y. Kim
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. S. C. Lee
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. K. W. Park
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. S. E. Jung
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to S. E. Jung.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Choe, E.K., Moon, S.B., Kim, H.Y. et al. Outcomes of Surgical Management of Total Colonic Aganglionosis. World J Surg 32, 62–68 (2008). https://doi.org/10.1007/s00268-007-9270-5

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00268-007-9270-5

Keywords

Search

Navigation