Skip to main content
Archives of Disease in Childhood logoLink to Archives of Disease in Childhood
. 2002 May;86(5):348–351. doi: 10.1136/adc.86.5.348

Hirschsprung's disease: functional and psychological follow up comparing total colonic and rectosigmoid aganglionosis

L Ludman 1, L Spitz 1, H Tsuji 1, A Pierro 1
PMCID: PMC1751105  PMID: 11970929

Abstract

Aims: To compare the long term functional and psychosocial outcomes following surgical treatment for total colonic aganglionosis (TCA) with those in an age and gender matched group of patients with rectosigmoid aganglionosis (RSA).

Methods: Fifteen patients with TCA matched for age and gender with 15 patients with RSA were studied 7–17 years after the definitive operation. The internal and external sphincters were examined using anal endosonography. Functional outcome (faecal continence) was assessed by a surgeon not involved in the patients' care, and by a research psychologist in separate assessment sessions. The behavioural and emotional status of the patients was also assessed.

Results: Based on the surgeon's assessment, 6/15 TCA and 7/15 RSA patients were continent. In comparison, based on the psychological interview, 2/15 TCA and 6/15 RSA patients were continent. The TCA patients reported significantly more behavioural/emotional problems and lower levels of self esteem than the RSA patients. The parent and teacher assessments of psychosocial status revealed no differences between the groups. There was no association between incontinence and psychosocial adjustment in either group. There was no association between the assessments of functional outcome and the endosonographic appearance of the anal sphincters.

Conclusions: The proportion of patients with faecal incontinence 7–17 years after definitive surgery was high in both groups, but no association was found between incontinence and the psychosocial outcome measures. TCA patients perceived themselves as less well adjusted than their matched pairs. Differences between the groups in length of hospitalisation and severity of illness, especially in infancy and early childhood, may account for these differences.

Full Text

The Full Text of this article is available as a PDF (98.6 KB).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Baillie C. T., Kenny S. E., Rintala R. J., Booth J. M., Lloyd D. A. Long-term outcome and colonic motility after the Duhamel procedure for Hirschsprung's disease. J Pediatr Surg. 1999 Feb;34(2):325–329. doi: 10.1016/s0022-3468(99)90201-4. [DOI] [PubMed] [Google Scholar]
  2. Birleson P., Hudson I., Buchanan D. G., Wolff S. Clinical evaluation of a self-rating scale for depressive disorder in childhood (Depression Self-Rating Scale). J Child Psychol Psychiatry. 1987 Jan;28(1):43–60. doi: 10.1111/j.1469-7610.1987.tb00651.x. [DOI] [PubMed] [Google Scholar]
  3. Catto-Smith A. G., Coffey C. M., Nolan T. M., Hutson J. M. Fecal incontinence after the surgical treatment of Hirschsprung disease. J Pediatr. 1995 Dec;127(6):954–957. doi: 10.1016/s0022-3476(95)70036-6. [DOI] [PubMed] [Google Scholar]
  4. Coran A. G., Bjordal R., Eek S., Knutrud O. The surgical management of total colonic and partial small intestinal aganglionosis. J Pediatr Surg. 1969 Oct;4(5):531–537. doi: 10.1016/0022-3468(69)90093-1. [DOI] [PubMed] [Google Scholar]
  5. Diseth T. H., Bjørnland K., Nøvik T. S., Emblem R. Bowel function, mental health, and psychosocial function in adolescents with Hirschsprung's disease. Arch Dis Child. 1997 Feb;76(2):100–106. doi: 10.1136/adc.76.2.100. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Diseth T. H., Egeland T., Emblem R. Effects of anal invasive treatment and incontinence on mental health and psychosocial functioning of adolescents with Hirschsprung's disease and low anorectal anomalies. J Pediatr Surg. 1998 Mar;33(3):468–475. doi: 10.1016/s0022-3468(98)90090-2. [DOI] [PubMed] [Google Scholar]
  7. Diseth T. H., Emblem R. Somatic function, mental health, and psychosocial adjustment of adolescents with anorectal anomalies. J Pediatr Surg. 1996 May;31(5):638–643. doi: 10.1016/s0022-3468(96)90664-8. [DOI] [PubMed] [Google Scholar]
  8. Heij H. A., de Vries X., Bremer I., Ekkelkamp S., Vos A. Long-term anorectal function after Duhamel operation for Hirschsprung's disease. J Pediatr Surg. 1995 Mar;30(3):430–432. doi: 10.1016/0022-3468(95)90048-9. [DOI] [PubMed] [Google Scholar]
  9. Hosie G. P., Spitz L. Idiopathic constipation in childhood is associated with thickening of the internal anal sphincter. J Pediatr Surg. 1997 Jul;32(7):1041–1044. doi: 10.1016/s0022-3468(97)90395-x. [DOI] [PubMed] [Google Scholar]
  10. Ikeda K., Goto S. Total colonic aganglionosis with or without small bowel involvement: an analysis of 137 patients. J Pediatr Surg. 1986 Apr;21(4):319–322. doi: 10.1016/s0022-3468(86)80193-2. [DOI] [PubMed] [Google Scholar]
  11. Kelly J. H. The clinical and radiological assessment of anal continence in childhood. Aust N Z J Surg. 1972 Aug;42(1):62–63. doi: 10.1111/j.1445-2197.1972.tb06743.x. [DOI] [PubMed] [Google Scholar]
  12. Ludman L., Spitz L. Coping strategies of children with faecal incontinence. J Pediatr Surg. 1996 Apr;31(4):563–567. doi: 10.1016/s0022-3468(96)90497-2. [DOI] [PubMed] [Google Scholar]
  13. Ludman L., Spitz L., Kiely E. M. Social and emotional impact of faecal incontinence after surgery for anorectal abnormalities. Arch Dis Child. 1994 Sep;71(3):194–200. doi: 10.1136/adc.71.3.194. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Ludman L., Spitz L. Psychosocial adjustment of children treated for anorectal anomalies. J Pediatr Surg. 1995 Mar;30(3):495–499. doi: 10.1016/0022-3468(95)90065-9. [DOI] [PubMed] [Google Scholar]
  15. Marty T. L., Seo T., Matlak M. E., Sullivan J. J., Black R. E., Johnson D. G. Gastrointestinal function after surgical correction of Hirschsprung's disease: long-term follow-up in 135 patients. J Pediatr Surg. 1995 May;30(5):655–658. doi: 10.1016/0022-3468(95)90682-7. [DOI] [PubMed] [Google Scholar]
  16. Moore S. W., Albertyn R., Cywes S. Clinical outcome and long-term quality of life after surgical correction of Hirschsprung's disease. J Pediatr Surg. 1996 Nov;31(11):1496–1502. doi: 10.1016/s0022-3468(96)90164-5. [DOI] [PubMed] [Google Scholar]
  17. Quinton D., Rutter M. Early hospital admissions and later disturbances of behaviour: an attempted replication of Douglas' findings. Dev Med Child Neurol. 1976 Aug;18(4):447–459. doi: 10.1111/j.1469-8749.1976.tb03684.x. [DOI] [PubMed] [Google Scholar]
  18. Tsuji H., Spitz L., Kiely E. M., Drake D. P., Pierro A. Management and long-term follow-up of infants with total colonic aganglionosis. J Pediatr Surg. 1999 Jan;34(1):158–162. doi: 10.1016/s0022-3468(99)90248-8. [DOI] [PubMed] [Google Scholar]
  19. Verhulst F. C., Althaus M., Berden G. F. The Child Assessment Schedule: parent-child agreement and validity measures. J Child Psychol Psychiatry. 1987 May;28(3):455–466. doi: 10.1111/j.1469-7610.1987.tb01766.x. [DOI] [PubMed] [Google Scholar]

Articles from Archives of Disease in Childhood are provided here courtesy of BMJ Publishing Group

RESOURCES