Clinical outcome and long-term quality of life after surgical correction of Hirschsprung's disease

Date
1996
Authors
Moore S.W.
Albertyn R.
Cywes S.
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Abstract
One hundred seventy-eight of 330 patients were recalled after undergoing surgery for histologically proven Hirschsprung's disease (HD). One hundred fifteen were older than 4 years at interview (Mean age, 10 years). This sample appeared to be representative of the whole in terms of demographic features such as ethnic group, sex, length of aganglionic segment, timing of presentation and surgery performed. Anthropomorphic indices for weight and height were comparable to norms, but many younger patients were below expected weight for age. In general, weight and height for age was regained with time. Nine patients had delayed developmental milestones, which were owing to specific causes in four. Nine patients had a poor functional outcome, of which two had neurological impairment. Satisfactory school performance was achieved in all but 19 (26%) of the remaining patients. Long- term functional results were comparable for the Scare and Duhamel procedures with less favorable results noted following the Swenson procedure. Assessment of complications demonstrated a significantly (P < .01) lower incidence of constipation, sexual dysfunction, and micturition disturbance following the Soave procedure when compared with the Duhamel and Swenson procedures. Neurological impairment and length of aganglionic segment beyond the rectosigmoid area appeared to influence functional outcome, as did persisting enterocolitis. Enterocolitis was observed in 16.6% of patients on presentation, but continued in only 6%. Constipation was particularly associated with the Duhamel procedure, and a higher incidence of micturition disturbance, abdominal distension, and cuff stricture was noted following the Swenson procedure. Functional assessment by three different scoring methods showed that 86 (74.7%) of the 115 patients over the age of 4 had excellent anorectal function and appeared to be well adjusted. Twenty-two patients (19.2%) had relatively minor long-term problems but seven (6.1%) had persistent fecal soiling with resulting psychosocial maladjustment.
Description
Keywords
abdominal distension, adolescent, adult, anthropometric parameters, article, child, constipation, demography, developmental disorder, enterocolitis, feces incontinence, hirschsprung disease, human, major clinical study, micturition disorder, neurologic disease, priority journal, psychosocial disorder, quality of life, sexual dysfunction, surgical technique, Anal Canal, Child, Child, Preschool, Defecation, Educational Status, Fecal Incontinence, Follow-Up Studies, Growth, Hirschsprung Disease, Humans, Incidence, Postoperative Complications, Quality of Life, Rectum, Retrospective Studies, Social Adjustment, Treatment Outcome
Citation
Journal of Pediatric Surgery
31
11