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  • Title: Quality of life of patients with Hirschsprung's disease at 5 - 20 years post pull-through operations.
    Author: Niramis R, Watanatittan S, Anuntkosol M, Buranakijcharoen V, Rattanasuwan T, Tongsin A, Petlek W, Mahatharadol V.
    Journal: Eur J Pediatr Surg; 2008 Feb; 18(1):38-43. PubMed ID: 18302068.
    Abstract:
    PURPOSE: The aim of this study was to evaluate the bowel habits and quality of life with respect to faecal continence of patients with Hirschsprung's disease (HD) who had undergone pull-through operations more than 5 years previously. MATERIALS AND METHODS: Four hundred and sixty-seven patients who underwent pull-through operations for HD during the period of 1987 - 1999 were followed up for evaluation. A questionnaire including demographic data and a qualitative clinical scoring method as described by Holschneider was used for the evaluation of faecal continence. The scoring system did not require a physical examination. Outcomes of the 3 major procedures (Swenson, Duhamel and Soave technique) were analysed. The research was undertaken from October 2004 to September 2006. Patients with neurological defects and total colonic aganglionosis (TCA) were excluded from the analysis. Only patients with typical HD were evaluated, and they were divided into 3 groups based on the length of the period since surgery: 5 - 10 years in Group A; 10 - 15 years in Group B; and 15 - 20 years in Group C. RESULTS: Only 204 patients (male to female ratio: 169 : 35) returned to the Queen Sirikit National Institute of Child Health for evaluation. Twenty-six patients were excluded because of TCA in 13, Down's syndrome in 9 and cerebral palsy in 4. The remaining 178 patients were evaluated and divided into Group A (n = 67), Group B (n = 75) and Group C (n = 36). Excellent results (14 points), good results (10 - 13 points) and fair results (5 - 9 points) were noted in Group A in 52.2 %, 34.3 % and 7.5 % of cases respectively, in Group B in 68 %, 28 % and 4 % of cases, respectively, and in Group C in 88.9 %, 11.1 % and 0 % of cases, respectively. Five cases (7.5 %) in Group A and 3 cases (4 %) in Group B with fair results still had problems such as constant soiling and an inability to hold back defecation. They experienced marked limitations in their social life because of their dependence on diapers and frustration because of teasing by their friends. CONCLUSION: Faecal incontinence still remains a problem in some patients with HD at 5 - 15 years after surgical correction. However, nearly all patients who were operated on more than 15 years previously had nearly normal faecal continence and a normal social life.
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