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Diagnosis of Hirschsprung's Disease by Immunostaining Rectal Suction Biopsies for Calretinin, S100 Protein and Protein Gene Product 9.5
Published on: April 26, 2019
Outcomes at five to eight years of age for children with Hirschsprung's disease
Benjamin Saul Raywood Allin1, Charles Opondo2, Timothy John Bradnock3
1National Perinatal Epidemiology Unit, University of Oxford, Oxford, Oxfordshire, UK benjaminallin@doctors.org.uk.
Insights
This study reveals that many children with Hirschsprung disease (HD) face long-term issues like incontinence and low quality of life. Addressing these core outcomes is crucial for improving patient care.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Clinical Outcomes Research
Background:
- Hirschsprung disease (HD) is a congenital condition affecting the large intestine.
- Long-term outcomes in children with HD require comprehensive evaluation.
Purpose of the Study:
- To describe the core outcomes of Hirschsprung disease (HD) in UK and Irish primary school-aged children.
- To assess functional outcomes, reoperation rates, and quality of life in a UK-wide cohort.
Main Methods:
- Prospective cohort study (2010-2012) involving 28 UK/Irish pediatric surgical centers.
- Data collected from birth up to 5-8 years of age, including clinical outcomes and parent/clinician reports.
- Utilized the NETS 1HD core outcomes framework.
Main Results:
- Of 239 children, 13% had a stoma and 9% were incontinent of urine at 5-8 years.
- 79% maintained bowel movements without intervention, but 63% experienced fecal incontinence.
- 44% required unplanned reoperations, and many reported lower quality of life and psychological well-being scores.
Conclusions:
- This study provides a realistic overview of population-level outcomes for HD in school-aged children.
- High rates of incontinence, reoperations, and reduced quality of life highlight significant challenges.
- Prioritizing management of bladder, bowel, and psychological issues is essential for affected children.
Objective:
This study describes core outcomes of Hirschsprung's disease (HD) in a UK-wide cohort of primary school-aged children.
Design:
A prospective cohort study conducted from 1 October 2010 to 30 September 2012. Outcomes data were collected from parents and clinicians when children were 5-8 years of age, and combined with data collected at birth, and 28 days and 1 year post diagnosis.
Setting:
All 28 UK and Irish paediatric surgical centres.
Participants:
Children with histologically proven HD diagnosed at <6 months of age.
Main Outcome Measures:
NETS1HD core outcomes.
Results:
Data were returned for 239 (78%) of 305 children. Twelve children (5%) died prior to 5 years of age.Of the 227 surviving children, 30 (13%) had a stoma and 21 (9%) were incontinent of urine. Of the 197 children without a stoma, 155 (79%) maintained bowel movements without enemas/washouts, while 124 (63%) reported faecal incontinence. Of the 214 surviving children who had undergone a pull-through operation, 95 (44%) underwent ≥1 unplanned reoperation. 89 unplanned reoperations (27%) were major/complex.Of the 83 children with returned PedsQL scores, 37 (49%) had quality of life scores, and 31 (42%) had psychological well-being scores, that were ≥1 SD lower than the reference population mean for children without HD.
Conclusion:
This study gives a realistic picture of population outcomes of HD in primary school-aged children in the UK/Ireland. The high rates of faecal incontinence, unplanned procedures and low quality of life scores are sobering. Ensuring clinicians address the bladder, bowel and psychological problems experienced by children should be a priority.
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