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Eur J Pediatr Surg 2018; 28(05): 445-454
DOI: 10.1055/s-0037-1604115
Original Article
Georg Thieme Verlag KG Stuttgart · New York

Long-Term Outcomes and Quality of Life in Patients after Soave Pull-Through Operation for Hirschsprung's Disease: An Observational Retrospective Study

Viet Quoc Tran
1   Department of Pediatric Surgery, City Children's Hospital, Ho Chi Minh City, Vietnam
2   Hôpital Universitaire des Enfants Reine Fabiola, Bruxelles, Belgique — Université Libre de Bruxelles (ULB), Brussels, Belgium
,
Tania Mahler
3   Department of Gastroenterology, Hôpital Universitaire des Enfants Reine Fabiola, Bruxelles, Belgique — Université Libre de Bruxelles (ULB), Brussels, Belgium
,
Martine Dassonville
2   Hôpital Universitaire des Enfants Reine Fabiola, Bruxelles, Belgique — Université Libre de Bruxelles (ULB), Brussels, Belgium
,
Dinh Quang Truong
1   Department of Pediatric Surgery, City Children's Hospital, Ho Chi Minh City, Vietnam
,
Annie Robert
4   Faculté de santé publique, Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain (UCL), Brussels, Belgium
,
Philippe Goyens
5   Laboratory of Pediatrics, Université Libre de Bruxelles (ULB), Brussels, Belgium
,
Henri Steyaert
2   Hôpital Universitaire des Enfants Reine Fabiola, Bruxelles, Belgique — Université Libre de Bruxelles (ULB), Brussels, Belgium
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Publikationsverlauf

07. Oktober 2016

28. Mai 2017

Publikationsdatum:
24. Juli 2017 (online)

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Abstract

Introduction Patients after pull-through operation for Hirschsprung's disease (HD) are at high risk of defecation disorders. This study aimed at investigating their long-term outcomes and quality of life (QoL) in comparison with controls.

Patients and Methods Patients older than 5 years operated on for HD were interviewed to complete detailed questionnaires on bowel function. Patients without neurologic impairment were enrolled in a QoL survey to compare with controls matched for sex and age and selected randomly from the general population using sampling set in a ratio of four controls to one case of HD.

Results In total, 53 operated patients were enrolled. Mean age of the patients was 16 ± 8 years, with 68% boys. Rectosigmoid aganglionosis was the most seen form of HD in 38 (72%) cases. Open Soave was performed in 40 (75.5%) cases, and minimally invasive surgery Soave (MIS Soave) in 13 (24.5%) cases. At investigation, prevalence of fecal incontinence and constipation were 22.6 and 13.2%, respectively. Regarding QoL survey, 45 patients and 180 controls were enrolled, excluding 8 patients with neurologic impairment. Thirty-seven (82.2%) patients were classified as having a good QoL (score ≥ 9 points); whereas six had a fair QoL (5–8 points) and two had a poor QoL (< 5 points). QoL score in the cases and the controls were 10.2 ± 2.5 and 11.9 ± 0.4 points, respectively. Long aganglionosis form of HD was significantly associated with a low QoL (score < 8 points), adjusted odds ratio = 9, 95% confidence interval [1.3; 64.1] (p < 0.05). In subscales analyses, the prevalence of each dimension including fecal continence, school absenteeism, unhappiness or anxiety, food restriction, and peer rejection was significantly higher in operated patients than in controls (p <0.001).

Conclusion Although the QoL of patients operated on for HD in general was with good outcomes, fecal incontinence and constipation still are problematic issues and challenges in a high percentage of patients. Therefore, a long-term and multidisciplinary follow-up is essentially required for these patients.

Keywords

Hirschsprung's disease - fecal incontinence - constipation - quality of life - long-term outcomes