RESUMEN
Purpose@#Children treated for Hirschsprung disease (HD) are adversely affected by fecal incontinence and soiling. This can be detrimental to their physical, psychosocial quality of life (QoL) and impacts the normal functioning of their family. QoL studies in HD are predominantly from developed countries. We measured general quality of life, impact on family and functional bowel status using validated questionnaires in HD children in a developing country. @*Methods@#Patients with HD, treated in a tertiary paediatric institution in India between 2010 and 2017, were identified. Patients and/or their proxy completed the Pediatric Quality of Life and Family Impact Module questionnaires. Functional outcomes were assessed using Rintala's score. @*Results@#A 86 children and their parents participated in the study. Majority had rectosigmoid disease (67.4%) and underwent Soave's endoanal pull through (74.4%). A 21% of patients had low Rintala score indicating poor functional bowel outcomes. Only 11% of children had poor QoL scores. Family functioning outcomes were also severely affected in the same subgroup of patients. There was statistically significant correlation between Rintala score and QoL scores (p-value<0.001). Disease severity, type of surgery, and duration of follow-up did not have a statistically significant impact on the QoL. @*Conclusion@#QoL in children with HD was comparable to the general population. Bowel dysfunction affects a notable number of children and was the most significant determinant of poor QoL.
RESUMEN
Purpose@#Children treated for Hirschsprung disease (HD) are adversely affected by fecal incontinence and soiling. This can be detrimental to their physical, psychosocial quality of life (QoL) and impacts the normal functioning of their family. QoL studies in HD are predominantly from developed countries. We measured general quality of life, impact on family and functional bowel status using validated questionnaires in HD children in a developing country. @*Methods@#Patients with HD, treated in a tertiary paediatric institution in India between 2010 and 2017, were identified. Patients and/or their proxy completed the Pediatric Quality of Life and Family Impact Module questionnaires. Functional outcomes were assessed using Rintala's score. @*Results@#A 86 children and their parents participated in the study. Majority had rectosigmoid disease (67.4%) and underwent Soave's endoanal pull through (74.4%). A 21% of patients had low Rintala score indicating poor functional bowel outcomes. Only 11% of children had poor QoL scores. Family functioning outcomes were also severely affected in the same subgroup of patients. There was statistically significant correlation between Rintala score and QoL scores (p-value<0.001). Disease severity, type of surgery, and duration of follow-up did not have a statistically significant impact on the QoL. @*Conclusion@#QoL in children with HD was comparable to the general population. Bowel dysfunction affects a notable number of children and was the most significant determinant of poor QoL.