Long-term outcome and quality of life after the Swenson procedure for Hirschsprung's disease

doi:10.1053/jpsu.2002.31625

Journal of Pediatric Surgery

Volume 37, Issue 4, April 2002, Pages 639-642

https://doi.org/10.1053/jpsu.2002.31625 Get rights and content

Abstract

Background/Purpose: The aim of this study was to investigate long-term outcome and quality of life after the Swenson operation for rectosigmoid Hirschsprung's Disease (HD). Methods: Forty-five patients who underwent the Swenson procedure for HD underwent follow-up for 8 to 16 years. Long-term outcome and quality of life were assessed by interviews and questionnaires including scoring systems. Forty-four healthy children with similar age, sex, and education level distributions used as controls. Results: In 45 patients, 23 (51.1%) had bowel dysfunction. Seventeen patients (37.8%) suffered from fecal soiling. According to the clinical bowel function scoring system, the patients' scores (7.6 ± 2.1) were significantly lower than those of the controls (11.4 ± 0.6; P <.05). Because of poor fecal continence, 25 patients (55.7%) had to restrict their foods. School absence occurred in 6 (13.3%) patients. Seven patients (15.6%) had problems in peer relationships. According to the Quality-of-Life Scoring Criteria, 86.7% patients had good or fair quality of life. The patients' scores (7.7 ± 2.9) were significantly lower than those of the controls (11.6 ± 0.7; P <.05). And the scores of patients who had fecal soiling and incontinence (6.3 ± 2.7) were significantly lower than those of patients without fecal soiling and incontinence (8.4 ± 2.6; P <.05). Conclusions: Although most patients had good or fair quality of life after surgical correction for HD, the long-term outcome and quality of life are not as good as surgeons expected. The bowel function and quality of life of the patients were poorer than those of healthy children. Fecal soiling is very common and affects patients' quality of life. Long-term regular follow-up is indispensable. Close attention should be paid to minimizing bowel dysfunction for patients with HD postoperatively to improve their quality of life. J Pediatr Surg 37:639-642. Copyright 2002, Elsevier Science (USA). All rights reserved.

Section snippets

Materials and methods

Of all the patients operated on from 1984 to 1994 at the second hospital of China Medical University for HD, we were able to obtain clearance to recall 76. To assess the long-term outcome and quality of life, 45 patients included in this report were defined as follows: (1) The patients were from 8 to 16 years old. (2) All patients underwent a modified Swenson procedure. (3) HD was confirmed by an operative rectosigmoid biopsy. (4) All patients had an aganglionic segment confined to the

Stool control

Of 45 patients, 23 patients (51.1%) had long-term bowel dysfunction. Among these, 17 patients (37.8%) had fecal soiling, particularly at night. Three patients (6.7%) suffered from fecal incontinence. Three patients (6.7%) had constipation.

Stool frequency

Twenty-five patients (55.6%) had 1 or 2 bowel movements per day. Eleven patients (24.4%) had frequency of stooling ranging between 3 and 5 bowel movements per day. Three patients had 1 or 2 bowel movements per week. Six (13.3%) patients had increased frequency

Discussion

The functional outcome in patients treated for HD is variable markedly. Many reports showed that the results of surgery for HD were satisfactory. Sherman et al¹ reported outcomes with 880 Swenson procedures. At 5 years' or more follow-up, 89.9% of patients had normal bowel habits; this improved to 93.7% at more than 20 years' follow-up. In a large review by Bourdelat et al,² of 2,430 patients receiving a Duhamel operation, at 15 to 30 years' follow up, the majority of patients achieved good or

References (19)

J Sherman et al.
A 40-year multinational retrospective study of 880 Swenson procedures
J Pediatr Surg
(1989)
S Suita et al.
Long-term outcomes and quality of life after Z-shaped anastomosis for Hirschsprung's disease
J Am Coll Surg
(1998)
AG Catto-Smith et al.
Fecal incontinence after the surgical treatment of Hirschsprung's disease
J Pediatr
(1995)
HA Heij et al.
Long-term anorectal function after Duhamel operation for Hirschsprung's disease
J Pediatr Surg
(1995)
NL Yanchar et al.
Long-term outcome after Hirschsprung's disease: Patients' perspectives
J Pediatr Surg
(1999)
SW Moore et al.
Clinical outcome and long-term quality of life after surgical correction of Hirschsprung's disease
J Pediatr Surg
(1996)
R Reding et al.
Hirschsprung's disease: A 20-year experience
J Pediatr Surg
(1997)
M Heikkinen et al.
Long-term anal sphincter performance after surgery for Hirschsprung's disease
J Pediatr Surg
(1997)
TL Marty et al.
Gastrointestinal function after surgical correction of Hirschsprung's disease: Long-term follow-up in 135 patients
J Pediatr Surg
(1995)

There are more references available in the full text version of this article.

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Clinical symptoms and demographic factors did not predict HRQOL among children with HD, highlighting the need to examine other biopsychosocial factors to understand long term HRQOL. Low parent-child HRQOL agreement in children with HD demonstrates the importance of obtaining parent and child perspectives.
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^☆: Address reprint requests to WeiLin Wang, MD, The Second Clinical College, China Medical University, No. 36, Sanhao St, Heping District, Shenyang, 110004, China.

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Long-term outcome and quality of life after the Swenson procedure for Hirschsprung's disease☆

Abstract

Section snippets

Materials and methods

Stool control

Stool frequency

Discussion

J Pediatr Surg

J Am Coll Surg

J Pediatr

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg