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Factors Affecting Quality of Life of Children and Adolescents With Anorectal Malformations or Hirschsprung Disease

Hartman, Esther E*,†; Oort, Frans J; Sprangers, Mirjam AG; Hanneman, Marianne JG*,†; van Heurn, LW Ernest; de Langen, Zacharias J§; Madern, Gerard C; Rieu, Paul NMA||; van der Zee, David C**; Looyaard, Nic††; van Silfhout-Bezemer, Marina‡‡; Aronson, Daniel C*

Journal of Pediatric Gastroenterology & Nutrition: October 2008 - Volume 47 - Issue 4 - p 463–471
doi: 10.1097/MPG.0b013e31815ce545
Original Articles: Gastroenterology

Objectives: First, to compare the quality of life (QL) and perceived self-competence of children and adolescents with anorectal malformations or Hirschsprung disease with that of reference groups. Second, to identify predictors of QL.

Patients and Methods: A total of 491 patients with anorectal malformations or Hirschsprung disease were sent a questionnaire, which assessed QL (mental, physical), disease-specific functioning (defecation-related), perceived self-competence (self-esteem, athletic competencies, school attitude), and demographic characteristics (sex, age). The clinical characteristics (disease severity, presence of congenital anomalies) were extracted from medical records.

Results: More than 50% (316, 64%) of patients with anorectal malformations or Hirschsprung disease completed the questionnaire. On average, children and adolescents in both patient groups reported no differences in QL domains compared with the reference groups. However, standard deviations revealed considerable individual variation, indicating the presence of patients with high levels of QL as well as patients with low levels of QL. Children and adolescents in both patient groups reported psychosocial problems in all domains, compared with the reference groups. Females, older patients, and those with a severe form of the disease reported lower levels of perceived self-competence and global disease-specific functioning, which in turn predicted QL.

Conclusions: Our results should alert clinicians to patients who are at risk for QL problems and may therefore be in need of extra care. Our findings illustrate the importance of both global disease-specific functioning and perceived psychosocial competencies for enhancing the QL of these patients.

*Pediatric Surgical Centers of Amsterdam, the Netherlands

Division of Medical Psychology, University of Amsterdam, the Netherlands

Departments of Pediatric Surgery at the University Hospitals of Maastricht, the Netherlands

§Groningen, the Netherlands

Rotterdam, the Netherlands

||Nijmegen, the Netherlands

**Utrecht, the Netherlands

††the Patient Societies of Anorectal Malformations, the Netherlands

‡‡Hirschsprung's Disease, Nijverdal and Hilversum, the Netherlands

Received 3 November, 2006

Accepted 29 September, 2007

Address correspondence and reprint requests to Esther Hartman, Pediatric Psychology/Tilburg University, Room P 712, PO Box 90153, 5000 LE Tilburg, The Netherlands (e-mail:

Supported, in part, by a grant from the Netherlands Digestive Diseases Foundation and Doctors for Children.

The authors report no conflicts of interest.

© 2008 Lippincott Williams & Wilkins, Inc.