Urinary Outcomes in Patients with Down's Syndrome and Hirschsprung's Disease
29 January 2018
25 April 2018
17 June 2018 (eFirst)
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.
- 1 Friedmacher F, Puri P. Hirschsprung's disease associated with Down syndrome: a meta-analysis of incidence, functional outcomes and mortality. Pediatr Surg Int 2013; 29 (09) 937-946
- 2 Travassos D, van Herwaarden-Lindeboom M, van der Zee DC. Hirschsprung's disease in children with Down syndrome: a comparative study. Eur J Pediatr Surg 2011; 21 (04) 220-223
- 3 Hackam DJ, Reblock K, Barksdale EM, Redlinger R, Lynch J, Gaines BA. The influence of Down's syndrome on the management and outcome of children with Hirschsprung's disease. J Pediatr Surg 2003; 38 (06) 946-949
- 4 Catto-Smith AG, Trajanovska M, Taylor RG. Long-term continence in patients with Hirschsprung's disease and Down syndrome. J Gastroenterol Hepatol 2006; 21 (04) 748-753
- 5 Koff SA, Wagner TT, Jayanthi VR. The relationship among dysfunctional elimination syndromes, primary vesicoureteral reflux and urinary tract infections in children. J Urol 1998; 160 (3 Pt 2): 1019-1022
- 6 Drzewiecki BA, Thomas JC, Pope IV JC, Adams MC, Brock III JW, Tanaka ST. Use of validated bladder/bowel dysfunction questionnaire in the clinical pediatric urology setting. J Urol 2012; 188 (4, Suppl): 1578-1583
- 7 Kwendakwema N, Al-Dulaimi R, Presson AP. , et al. Enterocolitis and bowel function in children with Hirschsprung disease and trisomy 21. J Pediatr Surg 2016; 51 (12) 2001-2004
- 8 Kitamura A, Kondoh T, Noguchi M. , et al. Assessment of lower urinary tract function in children with Down syndrome. Pediatr Int 2014; 56 (06) 902-908
- 9 de Carvalho Mrad FC, de Bessa Jr J, de Figueiredo AA, Netto JMB. About the article: prevalence of lower urinary tract symptoms in individuals with down syndrome. J Pediatr Urol 2015; 11 (04) 230-231
- 10 Powers MK, Brown ET, Hogan RM, Martin AD, Ortenberg J, Roth CC. Trends in toilet training and voiding habits among children with Down syndrome. J Urol 2015; 194 (03) 783-787
- 11 Niemczyk J, von Gontard A, Equit M, Medoff D, Wagner C, Curfs L. Incontinence in persons with Down syndrome. Neurourol Urodyn 2017; 36 (06) 1550-1556