Long-term functional outcomes and quality of life in patients with Hirschsprung's disease

Abstract

Background It is unclear whether functional outcomes improve or deteriorate with age following surgery for Hirschsprung's disease. The aim of this cross‐sectional study was to determine the long‐term functional outcomes and quality of life (QoL) in patients with Hirschsprung's disease. Methods Patients with pathologically proven Hirschsprung's disease older than 7 years were included. Patients with a permanent stoma or intellectual disability were excluded. Functional outcomes were assessed according to the Rome IV criteria using the Defaecation and Faecal Continence questionnaire. QoL was assessed by means of the Child Health Questionnaire Child Form 87 or World Health Organization Quality of Life questionnaire 100. Reference data from healthy controls were available for comparison. Results Of 619 patients invited, 346 (55·9 per cent) responded, with a median age of 18 (range 8–45) years. The prevalence of constipation was comparable in paediatric and adult patients (both 22·0 per cent), and in patients and controls. Compared with controls, adults with Hirschsprung's disease significantly more often experienced straining (50·3 versus 36·1 per cent; P = 0·011) and incomplete evacuation (47·4 versus 27·2 per cent; P < 0·001). The prevalence of faecal incontinence, most commonly soiling, was lower in adults than children with Hirschsprung's disease (16·8 versus 37·6 per cent; P < 0·001), but remained higher than in controls (16·8 versus 6·1 per cent; P = 0·003). Patients with poor functional outcomes scored significantly lower in several QoL domains. Conclusion This study has shown that functional outcomes are better in adults than children, but symptoms of constipation and soiling persist in a substantial group of adults with Hirschsprung's disease. The persistence of defaecation problems is an indication that continuous care is necessary in this specific group of patients.

Health-Related Quality of Life in Biliary Atresia Patients with Native Liver or Transplantation

“…To assess HrQoL in children with BA, we used the internationally validated CHQ. 12,13 This questionnaire has been developed to test both physical and psychosocial HrQoL of children aged 5 years and older. 14 The CHQ consists of a 50item parent-proxy form (CHQ-PF50), and an 87-item childreported form (CHQ-CF87) for children aged 10 years or older.…”

Redo surgery with longitudinal resection for dilated bowel in Hirschsprung disease: an illustrative case series

“…Disadvantages of re-resection of the dilated segment without a redo pull-through are further omission of bowel length and vascular supply, as well as jeopardizing the distal segment if the original distal anastomosis remains in place. Simultaneous resection of the distal segment as well carries higher risk of future defecation disorders, especially faecal incontinence [14]. Disadvantage of a permanent colostomy is the time-consuming daily care, as well as the risk of stoma-related complications such as skin problems, prolapse and parastomal herniation [15].…”

Health-Related Quality of Life and Psychosocial Morbidity in Anorectal Malformation and Hirschsprung's Disease

“…Psychosocial function À Reduced emotional and psychosocial functioning 5,27 À Altered physical function impairs psychosocial functioning [24][25][26] À Reduced emotional and social functioning 28,29 À Specific learning disabilities at school age 30 À Altered physical function impairs psychosocial functioning 29 Abbreviations: ARM, anorectal malformation; HD, Hirschsprung's disease; HrQoL, health-related quality of life. Note: Plus (þ) sign indicates no or comparable effect.…”

“…Minus (-) sign indicates a negative impact. 15,29,34 À Psychosocial HrQoL worsens in adolescence, although physical morbidity improves 26,28,35 Gender þ No effect 13,32,33,39,40 þ Comparable HrQoL in infancy for boys and girls 38 À Reduced HrQoL in females from adolescence 34,37,38…”

“…Likewise, the often-addressed fear of being incontinent for feces or gas during sexual intercourse may influence sexual activity and, therefore, indirectly have an impact on the childbirth rate. Parents À QoL is reduced with a chronically ill child 60,61 À Mothers are more anxious and have a lower psychosocial QoL than fathers at the diagnosis 29,59 À Maternal anxiety relieves at school age but increases again in adolescence 29,48,59 Abbreviations: ARM, anorectal malformation; HD, Hirschsprung's disease; QoL, quality of life. Note: Minus (-) sign indicates a negative impact.…”