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Urinary Outcomes in Patients with Down's Syndrome and Hirschsprung's Disease
Alexander Johannes Martinus Dingemans1, Carlos Albert Reck-Burneo2,3, Molly Fuchs2
1Department of Clinical Genetics, Radboudumc, Nijmegen, The Netherlands.
Insights
Children with Hirschsprung
Area of Science:
- Pediatric Surgery
- Urology
- Genetics
Background:
- Hirschsprung's disease (HD) and Down's syndrome (DS) are congenital conditions often requiring surgical intervention.
- Previous research has primarily examined colorectal outcomes in patients with both HD and DS.
- Urinary outcomes in this specific patient population remain under-explored.
Purpose of the Study:
- To investigate and compare urinary outcomes in pediatric patients with Hirschsprung's disease, with and without Down's syndrome.
- To assess the prevalence of urinary symptoms and dysfunctional elimination syndrome in this cohort.
Main Methods:
- Retrospective review of medical records for patients aged five years and older with Hirschsprung's disease.
- Utilized the Vancouver Symptom Score to evaluate dysfunctional elimination syndrome symptoms.
- Compared urinary and fecal symptom prevalence and severity between patients with and without Down's syndrome.
Main Results:
- A significantly higher prevalence of urinary accidents was observed in patients with Hirschsprung's disease and Down's syndrome (44%) compared to those without DS (6%).
- Patients with HD and DS reported more severe urinary symptoms, as indicated by higher Vancouver scores.
- Fecal accidents were also associated with more severe urinary symptoms in the overall HD cohort.
Conclusions:
- Patients with Down's syndrome represent a distinct subgroup of Hirschsprung's disease patients with a notable increase in postoperative urinary issues.
- Enhanced focus on postoperative urinary care is crucial for patients with both HD and DS.
- Improved urinary management can significantly enhance patient quality of life and ease caregiver burden.
Introduction:
Previous research in children with Hirschsprung's disease (HD) and Down's syndrome (DS) has focused on colorectal outcomes. We set out to review urinary outcomes in this patient group.
Materials And Methods:
The medical records of all patients aged five years and older with HD were reviewed, and patients and caregivers filled out the Vancouver Symptom Score at intake, which is designed and validated to diagnose dysfunctional elimination syndrome.
Results:
A total of 104 patients with HD were included in this study. Of these, 16 (15%) patients had DS. There were no significant differences in the prevalence of enterocolitis or colorectal symptoms between patients with or without DS. Five of 88 (6%) patients without DS and 7 of 16 (44%) (p = 0.00001) with DS reported having urinary accidents. Patients with HD and DS scored higher on the Vancouver score (9 vs. 17.5; p = 0.007), indicating more severe urinary symptoms. Patients who also reported fecal accidents scored significantly higher on the Vancouver (12 vs. 9; n = 61; p = 0.016), indicating more problems.
Conclusion:
Patients with DS appear to be a unique subset of HD patients who have a higher prevalence of urinary symptoms after surgery. In the postoperative care of patients with HD and DS, a strong focus should be placed on postoperative urinary care in addition to their bowel care. This could significantly ease care and contribute to the quality of life of the parents and the patient.