Approximately 340 babies are born with Hirschsprung's disease (HSCR) or an anorectal malformation (ARM) per year in the UK. Most require corrective surgery in the newborn or early infancy period. In both conditions, there is both variability in the severity of the condition and the type of operative technique used. Many children do well following surgery and have good continence in later life. However a significant proportion of children suffer from a lifetime of constipation or incontinence. This has a significant impact on their social and psychological welfare and is a significant burden on healthcare resources.
Due to the variation in practice, there are limited data on long-term outcomes following surgery for children with HSCR or ARMs. Where studies have been performed, they often include small numbers, non-standardised outcome measures and short follow-up periods. It is therefore difficult to ascertain the effectiveness of different management strategies. However, a recent Delphi process has been carried out to establish 10 core measures in HSCR to improve outcome reporting. This research group has a long track record in this research area, specifically in the determination of long-term outcomes of children with HSCR, leading to well cited papers within the literature. In the last 2 decades there has been a significant change in the surgical techniques used in HSCR, however the underlying evidence base for this is still lacking. This study seeks to follow-on from previous studies looking specifically at the long-term outcomes in children with HSCR. Furthermore, the aim is to widen the study to encompass all children treated at Alder Hey and also to use the same methodology to assess long-term outcomes for children with ARMs, as both groups of patients experience similar long term morbidity.
This will provide qualitative and quantitative data aiding counselling parents of children with HSCR/ARM.
All children treated at either center with histologically confirmed Hirschsprung's Disease since diagnosis from 1991
All children diagnosed with anorectal malformation on the basis of position in relation to the external sphincter and size
Patients with non-histologically diagnosed Hirschsprung's Disease
Patients whose initial primary treatment or majority of follow-up for either condition has been external to either center
Patients with funnel anus
Adults who are unable to consent for themselves
Children and adults diagnosed with Hirschsprung's disease or Anorectal Malformation since 1991 cared for in a single centre.
Cohort
Other
Pull through
Procedure/SurgeryChildren diagnosed with Hirschpsrung's Disease or an anorectal malformation, with or without operative intervention will be included
Comparison of the Quality of life of children and adults with HSCR and ARM to pre-defined controls
PedsQL score for children. Compared to pre-defined controls. Score 0 (best)-92 (worst)
Comparison of the Quality of life of children and adults with HSCR and ARM to pre-defined controls
PedsQL score for children. Compared to pre-defined controls. Score 0 (best)-92 (worst)
Comparison of the Quality of life of children and adults with HSCR and ARM to pre-defined controls
PedsQL score for children. Compared to pre-defined controls. Score 0 (best)-92 (worst)
Comparison of the Quality of life of children and adults with HSCR and ARM to pre-defined controls
PedsQL score for children. Compared to pre-defined controls Score 0 (best)-92 (worst)
Comparison of the sexual health of adults with HSCR and ARM compared to pre-defined controls and obstetric health
Descriptive data
Description of the obstetric health of adults with HSCR and ARM
Descriptive data
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